26 The Child With Hematologic or Immunologic Dysfunction

ROSALIND BRYANT

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RELATED TOPICS and ADDITIONAL RESOURCESIN TEXT

Administration of Medication, Ch. 22

Anaphylaxis, Ch. 25

Bone Marrow Aspiration or Biopsy, Ch. 22

Family-Centered Care of the Child During Illness and Hospitalization, Ch. 21

Immunizations, Ch. 10

Impact of Chronic Illness, Disability, or Death on the Child and Family, Ch. 18

Infection Control, Ch. 22

Lumbar Puncture, Ch. 22

Pain Assessment; Pain Management, Ch. 21

Physical Examination, Ch. 7

Preparation for Procedures, Ch. 22

CD COMPANION

Critical Thinking Exercises—Bleeding; HIV Testing in Children; Bone Marrow Test

Case Studies—Sickle Cell Anemia; Acute Lymphoid Leukemia

Guidelines—Handling Chemotherapeutic Agents; Calculating the Absolute Neutrophil Count

Clinical Manifestations—Anemia; Sickle Cell Anemia; Hemophilia

Nursing Care Plans—The Child With Sickle Cell Anemia; The Child With HIV Infection; The Child With Cancer

NCLEX-Style Review Questions

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WONG'S CLINICAL MANUAL OF PEDIATRIC NURSING, 6/E

Community and Home Care Instructions—Caring for a Venous Access Device; Administering Medications to Children

Nursing Care Plan—The Child With Anemia

LEARNING OBJECTIVESOn completion of this chapter the reader will be able to:

• Distinguish between the various categories of anemia.

• Describe the prevention of and care of the child with iron-deficiency anemia.

• Compare sickle cell anemia and β-thalassemia major in relation to pathophysiology and nursing care.

• Describe the mechanisms of inheritance and nursing care of the child with hemophilia.

• Relate the pathophysiology and clinical manifestations of leukemia.

• Demonstrate an understanding of the rationale of therapies for neoplastic disease.

• Outline a plan of care for the child with neoplastic disease and the family.

• Contrast the pathophysiology and management of the immunodeficiency disorders.

• List nursing precautions and responsibilities during blood transfusion.

• Describe the types of hematopoietic stem cell transplants.

HEMATOLOGIC/IMMUNOLOGIC DYSFUNCTION

ASSESSMENT OF HEMATOLOGIC FUNCTIONSeveral tests can be performed to assess hematologic function, including additional procedures to identify the cause of the dysfunction. The following discussion is limited to a description of the most common and one of the most valuable tests, the complete blood cell count (CBC). Other procedures, such as those related to iron, coagulation, and immune status, are discussed throughout the chapter as appropriate. The nurse should be familiar with the significance of the findings from the CBC (Table 26-1) and aware of normal values for age, which are listed in Appendix E.

TABLE 26-1 Tests Performed as Part of the Complete Blood Cell Count

As with any disorder, the history and physical examination are essential to identification of hematologic dysfunction, and the nurse is often the first person to suspect a problem based on

information from these sources. Comments by the parent regarding the child's lack of energy, food diary of poor sources of iron, frequent infections, and bleeding that is difficult to control offer clues to the more common disorders affecting the blood. A careful physical appraisal, especially of the skin, can reveal findings (e.g., pallor, petechiae, bruising) that may indicate minor or serious hematologic conditions. Nurses need to be aware of the clinical manifestations of blood diseases to assist in recognizing symptoms and establishing a diagnosis.

FYIA common term used in describing an abnormal CBC is shift to the left, which refers to the presence of immature neutrophils in the peripheral blood from hyperfunction of the bone marrow, as seen during a bacterial infection.

RED BLOOD CELL DISORDERS

ANEMIAThe term anemia describes a condition in which the number of red blood cells (RBCs) or the hemoglobin (Hgb or Hb) concentration is reduced below normal values for age. As a result of this decrease, the oxygen-carrying capacity of the blood is diminished, causing a reduction in the oxygen available to the tissues. Anemia is the most common hematologic disorder of infancy and childhood and is not a disease itself but an indication or manifestation of an underlying pathologic process.

ClassificationAnemias are classified in relation to (1) etiology or physiology, manifested by erythrocyte or Hgb depletion, and (2) morphology, the characteristic changes in RBC size, shape, or color (Box 26-1). Although the morphologic classification is more useful in terms of laboratory evaluation of anemia, the etiologic approach provides direction for planning nursing care. For example, anemia with reduced Hgb concentration may be caused by a dietary depletion of iron, and the principal intervention is replenishing iron stores. The classification of anemias is found in Fig. 26-1.

BOX 26-1 Red Blood Cell (RBC) Morphology Characteristics of RBCsSIZE (CELL SIZE)

Variation in RBC sizes (anisocytosis)

Normocytes (normal cell size)

Microcytes (smaller than normal cell size)

Macrocytes (larger than normal cell size)

SHAPE (IRREGULAR SHAPE)

Variation in RBC shapes (poikilocytes)

Spherocytes (globular cells)

Drepanocytes (sickle-shaped cells)

Numerous other irregular-shaped cells

COLOR (STAINING CHARACTERISTICS)

Variation in hemoglobin concentration in the RBC

Normochromic (sufficient or normal amount of hemoglobin per RBC)

Hypochromic (reduced amount of hemoglobin per RBC)

Hyperchromic (increased amount of hemoglobin per RBC)FIG. 26-1 Classifications of anemias.

Consequences of AnemiaThe basic physiologic defect caused by anemia is a decrease in the oxygen-carrying capacity of blood and consequently a reduction in the amount of oxygen available to the cells. When the anemia has developed slowly, the child usually adapts to the declining Hgb level.

The effects of anemia on the circulatory system can be profound. Because the viscosity of blood depends almost entirely on the concentration of RBCs, the resulting hemodilution of severe anemia decreases peripheral resistance, causing greater quantities of blood to return to the heart. The increased circulation and turbulence within the heart may produce a murmur. Because the cardiac workload is greatly increased, especially during exercise, infection, or emotional stress, cardiac failure may ensue.

Children seem to have a remarkable ability to function quite well despite low levels of Hgb. Cyanosis (the result of the quantity of deoxygenated Hgb in arterial blood) is typically not evident. Growth retardation, resulting from decreased cellular metabolism and coexisting anorexia, is a common finding in chronic severe anemia and is frequently accompanied by delayed sexual maturation in the older child.

Diagnostic Evaluation

In general, anemia may be suspected from findings on the history and physical examination, such as lack of energy, easy fatigability, and pallor, but unless the anemia is severe, the first clue to the disorder may be alterations in the CBC, such as decreased RBCs, and decreased Hgb and hematocrit (Hct) levels (see Fig. 26-1). Although anemia is sometimes defined as an Hgb level below 10 or 11 g/dL, this arbitrary cutoff is inappropriate for all children, because Hgb levels normally vary with age (see Table 26-1 and Appendix E).

Other tests specific to a particular type of anemia are employed to determine the underlying cause of anemia. These are discussed in relation to the particular disorder.

Therapeutic ManagementThe objective of medical management is to reverse the anemia by treating the underlying cause and to make up for any deficiency of blood, blood component, or substance the blood needs for normal functioning. For example, blood or blood cells are replaced after hemorrhage; in nutritional anemias the specific deficiency is replaced.

In patients with severe anemia, supportive medical care may include oxygen therapy, bed rest, and replacement of intravascular volume with intravenous (IV) fluids. The prognosis for anemia depends on the correction of the cause.

Nursing ConsiderationsThe assessment of anemia includes the basic techniques that are applicable to any condition. The age of the infant or child provides some clues regarding the possible etiology of the anemia. For example, iron deficiency anemia occurs more frequently in the toddler between 12 and 36 months of age and during the growth spurt of adolescence.

Racial or ethnic background is significant. For example, the anemias related to abnormal Hgb levels are found in Southeast Asians and persons of African or Mediterranean ancestry. These same groups may be genetically deficient in the enzyme lactase after the period of infancy. Affected individuals are unable to

tolerate lactose in the diet, with consequent intestinal irritation and chronic blood loss.

Special emphasis is placed on a careful history to elicit any information that might help identify the cause of the anemia. For example, a statement such as "My child drinks lots of milk" is a frequent finding in toddlers with iron deficiency anemia. An episode of diarrhea may have precipitated a temporary lactose intolerance in a young child.

Stool examination for occult (microscopic) blood (Hemoccult test) can identify chronic intestinal bleeding that results from a primary or secondary lactase deficiency. It is also important to understand the significance of various blood tests (see Table 26-1).

Prepare Child and Family for Laboratory Tests.Usually, several blood tests are ordered, but because they are generally done sequentially rather than at one time, the child is subjected to multiple finger or heel punctures or venipunctures. Laboratory technicians frequently are not aware of the trauma that repeated punctures represent to a child. However, these invasive procedures need not be painful (see Blood Specimens, Chapter 22). For example, the topical application of EMLA or Elamax before needle punctures can eliminate any pain (see Pain Management, Chapter 21). Therefore the nurse is responsible for preparing the child and family for the tests by (1) explaining the significance of each test, particularly why the tests are not done at one time; (2) encouraging parents or another supportive person to be with the child during the procedure; and (3) allowing the child to play with the equipment on a doll or participate in the actual procedure (e.g., by cleansing the finger with an alcohol swab). Older children may appreciate the opportunity to observe the blood cells under a microscope or in photographs. This experience is an especially important consideration if a serious blood disorder, such as leukemia, is suspected, because it serves as a foundation for explaining the pathophysiology of the disorder.

Bone marrow aspiration is not a routine hematologic test but is essential for definitive diagnosis of the leukemias, lymphomas, and certain anemias.

NURSING TIPThe following are suggested explanations for teaching children about blood components:

•Red blood cells—Carry the oxygen you breathe from your lungs to all parts of your body

•White blood cells—Help keep germs from causing infection

•Platelets—Small parts of cells that help make bleeding stop; platelets help your body stop bleeding by forming a clot (scab) over the hurt area

•Plasma—The liquid portion of blood; has clotting factors that help make bleeding stop

Decrease Tissue Oxygen Needs.Because the basic pathology in anemia is a decrease in oxygen-carrying capacity, an important nursing responsibility is to assess the child's energy level and minimize excess demands. The child's level of tolerance for activities of daily living and play is assessed, and adjustments are made to allow as much self-care as possible without undue exertion. During periods of rest the nurse takes vital signs and observes behavior to establish a baseline of nonexertion energy expenditure. During periods of activity the nurse repeats these measurements and observations to compare them with resting values.

! NURSING ALERTSigns of exertion include tachycardia, palpitations, tachypnea, dyspnea, shortness of breath, hyperpnea, breathlessness, dizziness, light-headedness, diaphoresis, and change in skin color. The child looks fatigued (sagging, limp posture; slow, strained

movements; inability to tolerate additional activity; difficulty sucking in infants).

Diversional activities are planned that promote rest but prevent boredom and withdrawal. Because short attention span, irritability, and restlessness are common in anemia and increase stress demands on the body, appropriate activities are planned, such as listening to music; using a tape recorder; watching television; reading or listening to stories or comics; continuing a favorite hobby, such as stamp collecting, coloring, or drawing; playing board and card games; or being wheeled in a carriage or chair. Choosing the appropriate roommate, such as a child of similar age with a diagnosis that also requires restricted activity, is a helpful intervention.

If infants or young children are hospitalized, the importance of preventing separation from parents must be considered. Crying and fretfulness place increased stress demands on the body, which increases oxygen needs. Parents need help in understanding the importance of their presence, even though the child may be less responsive than usual. The nurse also explains the reason for mood changes and the necessity of allowing the child's dependency.

Prevent Complications.Children who are so severely anemic that they are hospitalized may require oxygen to prevent or reduce tissue hypoxia. Because these children are susceptible to infection, every effort is expended to prevent exposure to infectious agents. All the usual precautions are taken to prevent infection, such as practicing thorough handwashing, selecting an appropriate room in a noninfectious area, restricting visitors or hospital personnel with active infection, and maintaining adequate nutrition. The nurse also observes for signs of infection, particularly temperature elevation and leukocytosis.

IRON-DEFICIENCY ANEMIAAnemia caused by an inadequate supply of dietary iron is the most prevalent nutritional disorder in the United States and the most common mineral disturbance. Children 12 to 36 months of age are at risk for anemia as a result of cow's milk being a major staple of

the child's diet (Segel, Hirsh, and Feig, 2002). The prevalence of iron deficiency anemia has decreased, probably in part because of families' participation in the Women, Infants, and Children (WIC) program, which provides iron-fortified formula for the first year of life and routine screening of Hgb levels during early childhood (Bogen, Krause, and Serwint, 2001). Premature infants are especially at risk because of their reduced fetal iron supply. Adolescents are also at risk because of their rapid growth rate combined with poor eating habits.

PathophysiologyIron deficiency anemia can be caused by any number of factors that decrease the supply of iron, impair its absorption, increase the body's need for iron, or affect the synthesis of Hgb. Although the clinical manifestations and diagnostic evaluation are quite similar regardless of the cause, the therapeutic and nursing considerations depend on the specific reason for the iron deficiency. The following discussion is limited to iron deficiency anemia resulting from inadequate iron in the diet.

During the last trimester of pregnancy, iron is transferred from mother to fetus. Most of the iron is stored in the circulating erythrocytes of the fetus, with the remainder stored in the fetal liver, spleen, and bone marrow. These iron stores are usually adequate for the first 5 to 6 months in a full-term infant but for only 2 to 3 months in premature infants or multiple births. If dietary iron is not supplied to meet the infant's growth demands after the fetal iron stores are depleted, iron deficiency anemia results. Physiologic anemia should not be confused with iron deficiency anemia resulting from nutritional causes.

Although most toddlers with iron deficiency anemia are underweight, many infants are overweight because of excessive milk ingestion (known as milk babies). These children become anemic for two reasons: milk, a poor source of iron, is given almost to the exclusion of solid foods, and 50% of iron-deficient infants fed cow's milk have an increased fecal loss of blood.

Therapeutic ManagementAfter the diagnosis of iron deficiency anemia is made, therapeutic management focuses on increasing the amount of

supplemental iron the child receives. This is usually done through dietary counseling and the administration of oral iron supplements.

In formula-fed infants the most convenient and best sources of supplemental iron are iron-fortified commercial formula and iron-fortified infant cereal. Iron-fortified formula provides a relatively constant and predictable amount of iron and is not associated with an increased incidence of gastrointestinal (GI) symptoms, such as colic, diarrhea, or constipation. Infants younger than 12 months of age should not be given fresh cow's milk because it may increase the risk of GI blood loss occurring from allergy to the milk protein or from GI mucosal damage resulting from a lack of cytochrome iron (heme protein) (Segel, Hirsh, and Feig, 2002). If GI bleeding is suspected, the child's stool should be guaiac tested on at least four or five occasions to identify any intermittent blood loss.

Dietary addition of iron-rich foods is usually inadequate as the sole treatment of iron deficiency anemia, because the iron is poorly absorbed and provides insufficient supplemental quantities of iron. If dietary sources of iron cannot replace body stores, oral iron supplements are prescribed for approximately 3 months. Ferrous iron, more readily absorbed than ferric iron, results in higher Hgb levels. Ascorbic acid (vitamin C) appears to facilitate absorption of iron and may be given as vitamin C enriched foods and juices with the iron preparation.

If the Hgb level fails to rise after 1 month of oral therapy, it is important to assess for persistent bleeding, iron malabsorption, non compliance, improper iron administration, or other causes for the anemia. Parenteral (IV or intramuscular [IM]) iron administration is safe and effective, but painful, expensive, and occasionally associated with regional lymphadenopathy or allergic reaction (Andrews, 2003). Therefore parenteral iron is reserved for children who have iron malabsorption or chronic hemoglobinuria. Transfusions are indicated for the most severe anemia and in cases of serious infection, cardiac dysfunction, or surgical emergency when anesthesia is required. Packed RBCs (2 to 3 cc/kg), not whole blood, are used to minimize the chance of circulatory overload. Supplemental oxygen is administered when tissue hypoxia is severe.

Prognosis.The prognosis for a child with this condition is very good. However, there is some evidence that if the iron deficiency anemia is severe and long-standing, cognitive, behavioral, and motor impairment may result (Halterman and others, 2001; Andrews, 2003).

Nursing ConsiderationsAn essential nursing responsibility is instructing parents in the administration of iron. Oral iron should be given as prescribed in two divided doses between meals, when the presence of free hydrochloric acid is greatest, because more iron is absorbed in the acidic environment of the upper GI tract. A citrus fruit or juice taken with the medication aids in absorption.

NURSING TIPCow's milk contains substances that bind the iron and interferes with absorption. Iron supplements should not be administered with milk or milk products (Carley, 2003).

An adequate dosage of oral iron turns the stools a tarry green color. The nurse advises parents of this normally expected change and inquires about its occurrence on follow-up visits. Absence of the greenish black stool may be a clue to poor administration of iron, either in schedule or in dosage. Vomiting or diarrhea can occur with iron therapy. If the parents report these symptoms, the iron can be given with meals and the dosage reduced and then gradually increased until tolerated.

Liquid preparations of iron may temporarily stain the teeth. If possible, the medication should be taken through a straw or given through a syringe or medicine dropper placed toward the back of the mouth. Brushing the teeth after administration of the drug lessens the discoloration.

If parenteral iron preparations are prescribed, iron dextran must be injected deeply into a large muscle mass using the Z-tract method. The injection site is not massaged after injection to minimize skin staining and irritation. Because no more than 1 mL

should be given in one site, the IV route should be considered to avoid multiple injections. Careful observation is required because of the risk of adverse reactions, such as anaphylaxis, with IV administration. A test dose is recommended before routine use.

Diet.A primary nursing objective is to prevent nutritional anemia through family education. Because breast milk is a poor iron source after 5 months of lactation, the nurse must reinforce the importance of administering iron supplementation in the exclusively breast-fed infant by 4 to 6 months of age (Andrews, 2003; Griffins and Abrams, 2001).

In the formula-fed infant, the nurse discusses with parents the importance of using iron-fortified formula in and the introduction of solid foods at the appropriate age during the first year of life. Traditionally, cereals are one of the first semisolid foods to be introduced into the infant's diet at approximately 4 months of age (Davidsson, 2003). The best solid-food source of iron is commercial iron-fortified cereals. It may be difficult at first to teach the infant to accept foods other than milk. The same principles are applied as those for introducing new foods (see Nutrition, Chapter 10), especially feeding the solid food before the milk. Predominantly milk-fed infants rebel against solid foods, and parents are cautioned about this and the need to be firm in not relinquishing control to the child. It may require intense problem solving on the part of both the family and the nurse to overcome the child's resistance.

A difficulty encountered in discouraging the parents from feeding milk to the exclusion of other foods is dispelling the popular myth that milk is a "perfect food." Many parents believe that milk is best for the infant and equate the weight gain with a "healthy child" and "good mothering." The nurse can also stress that overweight is not synonymous with good health.

Diet education of teenagers is especially difficult, especially because teenage girls are particularly prone to following weight-reduction diets. Emphasizing the effect of anemia on appearance (pallor) and energy level (difficulty maintaining

popular activities) may be useful. (See Chapter 11—Mineral Disturbances and Table 11-2—for sources of iron-rich foods.)

SICKLE CELL ANEMIASickle cell anemia (SCA) is one of a group of diseases collectively termed hemoglobinopathies, in which normal adult hemoglobin (hemoglobin A [HbA]) is partly or completely replaced by abnormal sickle hemoglobin (HbS). Sickle cell disease (SCD) includes all those hereditary disorders whose clinical, hematologic, and pathologic features are related to the presence of HbS. Even though SCD is sometimes used to refer to SCA, this use is incorrect. Other correct terms for SCA are SS and homozygous sickle cell disease.

The following are the most common forms of SCD in the United States:

Sickle cell anemia, the homozygous form of the disease (HbSS or SS).

Sickle cell-C disease, a heterozygous variant of SCD, including both HbS and HbC (SC).

Sickle cell-hemoglobin E disease, a variant of SCD in which glutamic acid has been substituted for lysine in the number-26 position of the β-chain (SE).

Sickle thalassemia disease, a combination of sickle cell trait and β-thalassemia trait (Sβthal). β+ refers to the ability to still produce some normal HbA. β0 indicates that there is no ability to produce HbA.

Of the SCDs, SCA is the most common form in African Americans, followed by sickle cell-C disease and sickle β-thalassemia.

SCA is found primarily in African Americans, Hispanics, and other ethnic groups. SCA occurs infrequently in Caucasians (especially those of Mediterranean descent). The incidence of the disease varies in different geographic locations. Among African Americans the incidence of sickle cell trait is about 8%. In West Africa the incidence is reported to be as high as 40% among native Africans. The high incidence of sickle cell trait in West Africans is believed

by some to be the result of selective protection afforded trait carriers against one type of malaria.

The gene that determines the production of HbS is situated on an autosome and, when present, is always detectable and therefore dominant. Heterozygous persons who have both normal HbA and abnormal HbS are said to have sickle cell trait. Persons who are homozygous have predominantly HbS and have sickle cell anemia. The inheritance pattern is essentially that of an autosomal recessive disorder (see Appendix B). Therefore, when both parents have sickle cell trait, there is a 25% chance with each pregnancy of producing an offspring with SCA.

Although the defect is inherited, the sickling phenomenon is usually not apparent until later in infancy because of the presence of fetal hemoglobin (HbF). As long as the child has predominantly HbF, sickling does not occur because there is less HbS. The newborn with SCA is generally asymptomatic because of the protective effect of Hgb F (60% to 80% HbF), but this rapidly decreases during the first year, so the child is at risk for sickle cell-related complications (Dover and Platt, 2003).

PathophysiologyThe clinical features of SCA are primarily the result of (1) obstruction caused by the sickled RBCs and (2) increased RBC destruction (Fig. 26-2). The entanglement and enmeshing of rigid sickle-shaped cells with one another intermittently block the microcirculation, causing vaso-occlusion. The resultant absence of blood flow to adjacent tissues causes local hypoxia, leading to tissue ischemia and infarction (cellular death). Most of the complications seen in SCA can be traced to this process and its impact on various organs of the body. The effect of sickling and infarction on organ structures occurs in the following sequence (see also consequences in Box 26-2):

1. Stasis with enlargement

2. Infarction with ischemia and destruction

3. Replacement with fibrous tissue (scarring)

FIG. 26-2 Differences between A, normal and B, sickled red blood cells on circulation with related complications.

BOX 26-2 Clinical Manifestations of Sickle Cell AnemiaGENERAL

Possible growth retardation

Chronic anemia (Hgb 6 to 9 g/dL)

Possible delayed sexual maturation

Marked susceptibility to sepsis

VASO-OCCLUSIVE CRISIS

Pain in area(s) of involvement

Manifestations related to ischemia of involved areas:

Extremities—painful swelling of hands and feet (sickle cell dactylitis, or "hand-foot syndrome"), painful joints

Abdomen—severe pain resembling acute surgical condition

Cerebrum—stroke, visual disturbances

Chest—symptoms resembling pneumonia, protracted episodes of pulmonary disease

Liver—obstructive jaundice, hepatic coma

Kidney—hematuria

Genital—priapism (painful penile erection)

SEQUESTRATION CRISIS

Pooling of large amounts of blood:

Hepatomegaly

Splenomegaly

Circulatory collapse

EFFECTS OF CHRONIC VASO-OCCLUSIVE PHENOMENA

Heart—cardiomegaly, systolic murmurs

Lungs—altered pulmonary function, susceptibility to infections, pulmonary insufficiency

Kidneys—inability to concentrate urine, progressive renal failure, enuresis

Liver—hepatomegaly, cirrhosis, intrahepatic cholestasis

Spleen—splenomegaly, susceptibility to infection, functional reduction in splenic activity progressing to autosplenectomy

Eyes—intraocular abnormalities with visual disturbances, sometimes progressive retinal detachment and blindness

Extremities—skeletal deformities such as lordosis and kyphosis, avascular necrosis of hip/shoulder, chronic leg ulcers, susceptibility to osteomyelitis

Central nervous system—hemiparesis, seizures

Clinical ManifestationsThe clinical manifestations of SCA vary greatly in severity and frequency. The most acute symptoms of the disease occur during periods of exacerbation called crises. There are several types of episodic crises: vaso-occlusive, acute splenic sequestration, aplastic, hyperhemolytic, cerebrovascular accident (stroke), chest syndrome, and infection. The crises may occur individually or concomitantly with one or more other crises. The episode may be a vaso-occlusive crisis, preferably called a "painful episode," characterized by distal ischemia and pain; sequestration crisis, a pooling of blood in the liver and spleen with decreased blood volume and shock; aplastic crisis, diminished RBC production resulting in profound anemia; or hyperhemolytic crisis, an accelerated rate of RBC destruction characterized by anemia, jaundice, and reticulocytosis. This complication frequently suggests other coexisting conditions, such as viral illness or glucose-6-phosphate dehydrogenase (G6PD) deficiency.

Another serious complication is acute chest syndrome, which is clinically similar to pneumonia. It is the presence of a new pulmonary infiltrate and is associated with chest pain, fever, cough, tachypnea, wheezing and hypoxia. A cerebrovascular accident (CVA, stroke) is a sudden and severe complication, often with no related illnesses. Sickled cells block the major blood vessels in the brain, resulting in cerebral infarction, which causes variable degrees of neurologic impairment. Repeat CVAs causing progressively greater brain damage occur in approximately 70% of untreated children who have already experienced one stroke (Dover and Platt, 2003).

Diagnostic Evaluation

Newborn screening for SCA is mandatory in most of the United States so that infants can be identified before symptoms occur. At birth the infant has up to 80% of HbF, which does not carry the defect. Because levels of HbS are low at birth, Hgb electrophoresis or other tests that measure Hgb concentrations are indicated. Early diagnosis (before 3 months of age) enables initiation of appropriate interventions to minimize complications. The family is taught to administer prophylactic antibiotics and identify early signs of infection to seek medical therapy as soon as possible.

If SCA is not diagnosed in early infancy, it is likely to manifest symptoms during the toddler and preschool years. SCA is occasionally first diagnosed during a crisis that follows an acute respiratory or GI infection. Routine hematologic tests are done to evaluate the anemia. Several specific tests detect the presence of the abnormal Hbg in the heterozygote or the homozygote. For screening purposes the sickle-turbidity test (Sickledex) is frequently used, because it can be performed on blood from a finger stick and yields accurate results in 3 minutes. However, if the test is positive, Hgb electrophoresis is necessary to distinguish between those children with the trait and those with the disease. Hemoglobin electrophoresis ("finger printing" of the protein) is an accurate, rapid, and specific test for detecting the homozygous and heterozygous forms of the disease, as well as the percentages of the various types of Hgb.

Therapeutic ManagementThe aims of therapy are (1) to prevent conditions that enhance sickling phenomena, which are responsible for the pathologic sequelae; and (2) to treat the medical emergencies of sickle cell crisis. Prevention consists of maintaining hemodilution. The successful implementation of this goal depends more often on nursing interventions than on medical therapies. Research is investigating hydroxyurea with and without erythropoietin, which may increase the concentration of fetal hemoglobin and ultimately reduce complications (Ferster and others, 2001; Charache and others, 1996). Hematopoietic stem cell transplantation with stabilization of prior organ damage is a possible cure for SCD (Karayalcin, 2000). Limiting factors include proper patient selection as well as the availability of suitable donors (Karayalcin, 2000). This technology raises many ethical

issues regarding patient access and availability of therapy (Simon and others, 1999; Platt and Guinan, 1996).

Medical management of a crisis is usually directed at supportive and symptomatic treatment. The main objectives are to provide (1) rest to minimize energy expenditure and oxygen use; (2) hydration through oral and IV therapy; (3) electrolyte replacement, because hypoxia results in metabolic acidosis, which also promotes sickling; (4) analgesics for the severe pain from vaso-occlusion; (5) blood replacement to treat anemia and hydration to reduce the viscosity of the sickled blood; and (6) antibiotics to treat any existing infection. (See Ethical Case Study.)

ETHICAL CASE STUDY

Administration of pneumococcal and meningococcal vaccines is recommended for these children because of their susceptibility to infection as a result of a functional asplenia. In addition to routine immunizations, the child with SCD should receive a yearly influenza vaccination. (See Immunizations, Chapter 10.) Oral penicillin prophylaxis is also recommended by 2 months of age (American Academy of Pediatrics, 2002; National Institutes of Health, National Heart, Lung, and Blood Institute [NIH/NHLBI], 2002; Segal and others, 2002).

Short-term oxygen therapy may be helpful if a child has symptoms of respiratory difficulty. Severe hypoxia must be prevented because this causes massive systemic sickling that

can be fatal. Although oxygen may prevent more sickling, it usually is not effective in reversing sickling, because the oxygen is unable to reach the enmeshed sickled erythrocytes (Perkins, 2001; Chiocca, 1996) in clogged vessels. In addition, prolonged administration can depress bone marrow, further aggravating the anemia (Khoury and Grimsley, 1995).

Exchange transfusion, which reduces the number of circulating sickle cells and slows down the vicious circle of hypoxia, thrombosis, tissue ischemia, and injury, has been successful. The procedure is sometimes advocated as a possible preventive technique. A transcranial Doppler (TCD) test identifies the child with SCD who is high-risk for developing a CVA by monitoring the intracranial vascular flow (American Academy of Pediatrics, 2002; Segal and others, 2002). The TCD is performed yearly on children from 2 to 16 years of age. If the TCD is abnormal, then magnetic resonance imaging of the brain is done to detect cerebral arterial stenosis or ischemia. The recommended treatment for a confirmed abnormal TCD is chronic transfusion therapy (Segal and others, 2002). However, multiple transfusions carry the risk of transmission of viral infection, hyperviscosity, transfusion reactions, alloimmunization, and hemosiderosis (Orkin and Nathan, 2003; Karayalcin, 2000). After a CVA has occurred, blood transfusions are usually given every 3 to 4 weeks to help prevent a repeat stroke. To reduce iron overload, home subcutaneous chelation therapy may be started (see p. 950).

In children with recurrent life-threatening splenic sequestration, splenectomy may be a lifesaving measure. However, because the spleen usually atrophies on its own through progressive fibrotic changes (functional asplenia), routine splenectomy is not recommended because of the risk of overwhelming infection. Any procedure that requires anesthesia has increased risk for these children. Painful priapism (continual or intermittent erection) may be treated by aspiration of the corpora cavernosum. This complication is particularly frequent in vaso-occlusive crises.

The most frequent problem for patients with SCA is vaso-occlusive pain (Fig. 26-3). The chronic nature of this pain can greatly affect the child's development. A multidisciplinary approach is best for its management. When mild to moderate pain is reported, ibuprofen or acetaminophen is used initially. If

these drugs are not effective alone, codeine can be added. The dosages of both drugs are titrated (adjusted) to a therapeutic level. Opioids such as immediate- and sustained-release morphine, oxycodone, hydromorphone (Dilaudid), and methadone are administered intravenously or orally for severe pain and are given around the clock. Patient-controlled analgesia (PCA) has been used successfully for sickle cell-related pain. PCA reinforces the patient's role and responsibility in managing the pain and provides flexibility in dealing with pain, which may vary in severity over time. The use of high-dose IV methylprednisolone has decreased the duration of severe pain in children (Dover and Platt, 2003; Griffin, McIntire, and Buchanan, 1994). (See Pain Management, Chapter 21.)FIG. 26-3

Drawing of sickle cell pain by a 17-year-old boy. When asked what message he would like to give health professionals about treating pain, he stated, "Tell them to listen to the patient and family. They know about the pain."

! NURSING ALERTMeperidine (pethidine [Demerol]) is not recommended. Normeperidine, a metabolite of meperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it

accumulates with repetitive dosing. Patients with SCD are particularly at risk for normeperidine-induced seizures (NIH/NHLBI, 2002; American Pain Society, 1999).

Prognosis.The prognosis varies. Most of the time, children are without symptoms and participate in normal activities without restrictions. The greatest risk is usually in children younger than 5 years of age, and the majority of deaths in these children are caused by overwhelming infection. Consequently, SCA is a chronic illness with a potentially terminal outcome.

Individuals with higher levels of HbF are more likely to have fewer complications than those with lower levels (Steinberg and others, 2003; Segal and others, 2002). Long-term follow up of patients taking hydroxyurea alone revealed a 40% reduction in mortality (Steinberg and others, 2003).

Physical and sexual maturation are delayed in adolescents with SCA. Although adults achieve normal height, weight, and sexual function, the delay may present problems to the adolescent (Dover and Platt, 2003; Gribbons, Zahr, and Opas, 1995). Hematopoietic stem cell transplantation offers the hope of a cure for some children, although the mortality rate is approximately 8% and the graft failures after transplantation range from 9% to 14% (Dover and Platt, 2003) (see p. 959).

Nursing ConsiderationsEducate family and child. Family education begins with an explanation of the disease and its consequences. After this explanation, the most important issues to teach the family are to (1) seek early intervention for problems, such as fever of 38.5° C (101.5° F) or greater; (2) give penicillin as ordered; (3) recognize signs and symptoms of splenic sequestration, as well as respiratory problems that can lead to hypoxia; and (4) treat the child normally. The nurse tells the family that the child is normal but can get sick in ways that other children cannot.

NURSING TIP

One simple yet graphic way to demonstrate the effect of sickling is to roll rounded objects, such as marbles or beads, through a tube to simulate normal circulation and then roll pointed objects, such as screws or jacks, through the tube. The effect of sickling and clumping of the pointed objects is especially noticeable at a bend or slight narrowing of the tube.

The nurse emphasizes the importance of adequate hydration to prevent sickling and to delay the stasis-thrombosis-ischemia cycle in a crisis. It is not sufficient to advise parents to "force fluids" or "encourage drinking." They need specific instructions on how many daily glasses or bottles of fluid are required. Many foods are also a source of fluid, particularly soups, flavored ice pops, ice cream, sherbet, gelatin, and puddings.

Increased fluids combined with impaired kidney function result in the problem of enuresis. Parents who are unaware of this fact frequently employ the usual measures to discourage bed-wetting, such as limiting fluids at night, and may resort to punishment and shame to force bladder control. Enuresis is treated as a complication of the disease, such as joint pain or some other symptom, to alleviate parental pressure on the child.

Promote Supportive Therapies During Crises.The success of many of the medical therapies relies heavily on nursing implementation. Management of pain is an especially difficult problem and often involves experimenting with various analgesics, including opioids, and schedules before relief is achieved. Unfortunately, these children tend to be undermedicated, resulting in their "clock watching" and demands for additional doses sooner than might be expected. Often this incorrectly raises suspicions of drug addiction, when in fact the problem is one of improper dosage (see Family Focus box). In choosing and scheduling analgesics, the goal should be prevention of pain.

FAMILY FOCUS: Fear of AddictionAlthough pain is usually severe and opioids are warranted, many families fear that their child will become addicted to the

narcotic. Unfortunately, misinformed health professionals may foster this unfounded fear, resulting in needless suffering. Few, if any, children who receive opioids for severe pain become behaviorally addicted to the drug (NIH/NHLBI, 2002). Families and older children, especially adolescents, need to be reassured that opioids are medically indicated, high doses may be needed, and addiction is rare.

! NURSING ALERTAdvise parents to be particularly alert to situations in which dehydration may be a possibility, such as hot weather, and to recognize early signs of reduced intake, such as decreased urine output (e.g., fewer wet diapers) and increased thirst.

Any pain program should be combined with psychologic support to help the child deal with the depression, anxiety, and fear that may accompany the disease. This includes regular visits with the child to discuss any concerns during the hospitalization and positive reinforcement of coping skills, such as successful methods of dealing with the pain and compliance with treatment prescriptions. To reduce the negative connotation associated with the term "crisis," it is best to say "pain episode."

Frequently, heat to the affected area is soothing. Cold compresses are not applied to the area because this enhances sickling and vasoconstriction. Bed rest is usually well tolerated during a crisis, although actual rest depends greatly on pain alleviation and organized schedules of nursing care. Some activity, particularly passive range-of-motion exercises, is beneficial to promote circulation. Usually the best course of action is to let children dictate their activity tolerance.

If blood transfusions or exchange transfusions are given, the nurse has the responsibility of observing for signs of transfusion reaction (see Table 26-5). Because hypervolemia from too-rapid transfusion can increase the workload of the heart, the nurse also is alert to signs of cardiac failure.

In splenic sequestration the size of the spleen is gently measured by abdominal palpation (see Abdomen, Chapter 7).

The nurse should be aware of spleen size because an increasing splenomegaly is an ominous sign. A decreasing spleen size denotes response to therapy. Vital signs and blood pressure are also closely monitored for impending shock. Anemia is typically not a presenting complication in vaso-occlusive crises but is a critical problem in other types of crises. The nurse monitors for evidence of increasing anemia and institutes appropriate nursing intervention (see p. 946). Oxygen is not beneficial in vaso-occlusive episodes unless hypoxemia is present (Karayalcin, 2000; Chiocca, 1996). It does not reverse sickled RBCs, and if used in the nonhypoxic patient, it will decrease erythropoiesis (Khoury and Grimsley, 1995). Because prolonged use of oxygen can aggravate the anemia, signs of lack of therapeutic benefit, such as restlessness, increased pallor, and continued pain, are reported.

Intake, especially of IV fluids, and output are recorded. The child's weight should be taken on admission, because it serves as a baseline for evaluating hydration. Because diuresis can result in electrolyte loss, the nurse also observes for signs of hypokalemia and should be familiar with normal serum electrolyte values to report changes.

Recognize Other Complications.Nurses also need to be aware of the signs of chest syndrome and CVA, both potentially fatal complications.

! NURSING ALERTReport signs of the following immediately:

Chest syndrome:

Severe chest, back, or abdominal pain

Fever of 38.5° C (101.5° F) or higher

Very congested cough

Dyspnea, tachypnea

Retractions

Declining oxygen saturation (oximetry)

Cerebrovascular accident (CVA, stroke):

Severe, unrelieved headaches

Severe vomiting

Jerking or twitching of the face, legs, or arms

Seizures

Strange, abnormal behavior

Inability to move an arm or leg

Stagger or an unsteady walk

Stutter or slurred speech

Weakness in the hands, feet, or legs

Changes in vision

Support Family.Families need the opportunity to discuss their feelings regarding transmitting a potentially fatal, chronic illness to their child. Because of the widely publicized prognosis for children with SCA, many parents express their prevalent fear of the child's death. Three manifestations of SCD that may appear in the first 2 years of life (dactylitis, severe anemia, leukocytosis) can be predictors of disease severity (Miller and others, 2000). However, nursing care for the family should be the same as for any family with a child with a life-threatening illness. Particular emphasis is placed on the siblings' reactions, the stress on the marital relationship, and the childrearing attitudes displayed toward the child (see Chapter 18). Several resources are available to the family with a sickling disorder.*

The nurse advises parents to inform all treating personnel of the child's condition. The use of medical identification, such as a bracelet, is another way of ensuring awareness of the disease.

If family members have the SCD trait or SCA, genetic counseling is necessary. A primary goal is informing parents who carry the trait, in language they can understand, of the 25% chance with each pregnancy of having a child with the disease.

* National Association for Sickle Cell Disease, Inc., 3345 Wilshire Boulevard, Suite 1106, Los Angeles, CA 90010-1880; (800) 421-8453; website: http://www.sicklecelldisease.org; Center for Sickle Cell Disease, Howard University, 2121 Georgia Avenue NW, Washington, DC 20059; (202) 806-7930; National Heart, Lung, and Blood Institute, 9000 Rockville Pike, Building 31, Room 4A-21, Bethesda, MD 20892; (301) 496-4236; website: http://www.nhlbi.nih.gov/nhlbi/nhlbi.htm. The Agency for Healthcare Research Quality (AHRQ) (formerly AHCPR) has published three booklets on sickle cell disease: Sickle Cell Disease: Comprehensive Screening, Diagnosis, Management, and Counseling in Newborns and Infants, Clinical Practice Guideline no 6, pub no AHCPR-0562; Sickle Cell Disease: Comprehensive Screening and Management in Newborns and Infants, quick reference guide for clinicians no 6, pub no AHCPR-0563; and Sickle Cell Disease in Newborns and Infants: A Guide for Parents, pub no AHCPR 93-0564. They are available from the AHCPR Publications Clearinghouse, PO Box 8547, Silver Spring, MD 20907; (800) 358-9295; website: http://www.ahcpr.gov. Guideline for the Management of Acute and Chronic Pain in Sickle-Cell Disease is available from the American Pain Society, 4700 W Lake Avenue, Glenview, IL 60025-1485; (847) 375-4715; fax: (847) 375-6315; email: info@ampainsoc.org; website: http://www.ampainsoc.org. Clinical Reference Guide for Health Care Providers; Sickle Cell Related Pain: Assessment and Management—A Guide for Patients and Parents is available from the New England Regional Genetics Groups (NERGG), No. 28 Clarendon Street, Newton, MA 02460; (617) 243-3033, email: maryaten@mediaone.net; website: http://www.acadia.net/NERGG. A video, Sickle Cell Disease Is More Than Pain Management, is available from Maxishare, PO Box 2041, Milwaukee, WI 53201; (800) 444-7747. Information is also available from the Sickle Cell Disease Association of America, Inc., 200 Cooperate Pointe, Suite 495, Culver City, CA 90230-7633.

β-THALASSEMIA (COOLEY ANEMIA)The term thalassemia, which is derived from the Greek word thalassa, meaning "sea," is applied to a variety of inherited blood disorders characterized by deficiencies in the rate of production of specific globin chains in Hgb. The name appropriately refers to descendants of or those people living near the Mediterranean sea, who have the highest incidence of the disease, namely Italians, Greeks, and Syrians. Evidence suggests that the high incidence of the disorders among these groups is a result of selective advantage of the trait to malaria, as is postulated in sickle cell disease. However, the disorder has a wide geographic distribution, probably as a result of genetic migration through intermarriage or possibly as a result of spontaneous mutation.

β-Thalassemia is the most common of the thalassemias and occurs in four forms: two heterozygous forms: thalassemia minor, an asymptomatic silent carrier, and thalassemia trait, which produces a mild microcytic anemia; thalassemia intermedia, which is manifested as splenomegaly and moderate to severe anemia; and a homozygous form, thalassemia major (also known as Cooley anemia), which results in a severe anemia that would lead to cardiac failure and death in early childhood without transfusion support.

PathophysiologyNormal postnatal Hgb is composed of 2 α- and 2 β-polypeptide chains. In β-thalassemia there is a partial or complete deficiency in the synthesis of the β-chain of the Hgb molecule. Consequently, there is a compensatory increase in the synthesis of α-chains, and γ-chain production remains activated, resulting in defective Hgb formation. This unbalanced polypeptide unit is very unstable; when it disintegrates, it damages RBCs, causing severe anemia.

To compensate for the hemolytic process, an overabundance of erythrocytes is formed unless the bone marrow is suppressed by transfusion therapy. Excess iron from hemolysis of supplemental RBCs in transfusions and from the rapid destruction of defective cells is stored in various organs (hemosiderosis).

Diagnostic Evaluation

The onset of thalassemia major may be insidious and not recognized until the latter half of infancy. The clinical effects of thalassemia major are primarily attributable to (1) defective synthesis of HbA, (2) structurally impaired RBCs, and (3) shortened life span of erythrocytes (Box 26-3).

BOX 26-3 Clinical Manifestations of β-ThalassemiaANEMIA (BEFORE DIAGNOSIS)

Pallor

Unexplained fever

Poor feeding

Enlarged spleen/liver

WITH PROGRESSIVE ANEMIA

Signs of chronic hypoxia

Headache

Precordial and bone pain

Decreased exercise tolerance

Listlessness

Anorexia

OTHER FEATURES

Small stature

Delayed sexual maturation

Bronzed, freckled complexion (if not chelated)

BONE CHANGES (OLDER CHILDREN IF UNTREATED)

Enlarged head

Prominent frontal and parietal bosses

Prominent malar eminences

Flat or depressed bridge of the nose

Enlarged maxilla

Protrusion of the lip and upper central incisors and eventual malocclusion

Generalized skeletal osteoporosis

Hematologic studies reveal the characteristic changes in RBCs (i.e., microcytosis, hypochromia, anisocytosis, poikilocytosis, target cells, and basophilic stippling of various stages). Low Hgb and Hct levels are seen in severe anemia, although they are typically lower than the reduction in RBC count because of the proliferation of immature erythrocytes. Hgb electrophoresis confirms the diagnosis, and radiographs of involved bones reveal characteristic findings.

Therapeutic ManagementThe objective of supportive therapy is to maintain sufficient Hgb levels to prevent bone marrow expansion and the resulting bony deformities, and to provide sufficient RBCs to support normal growth and normal physical activity. Transfusions are the foundation of medical management. Recent studies have evaluated the benefits of maintaining the child's Hgb level above 9.5 g/dL, a goal that may require transfusions as often as every 3 to 5 weeks. The advantages of this therapy include (1) improved physical and psychologic well-being because of the ability to participate in normal activities, (2) decreased cardiomegaly and hepatosplenomegaly, (3) fewer bone changes, (4) normal or near-normal growth and development until puberty, and (5) fewer infections.

One of the potential complications of frequent blood transfusions is iron overload. Because the body has no effective means of eliminating the excess iron, the mineral is deposited in body tissues. To minimize the development of hemosiderosis, deferoxamine (Desferal), an iron-chelating agent, is given with oral supplements of vitamin C. Vitamin C should be used

only in patients who are ascorbate depleted and only while deferoxamine is being administered. As ferritin levels decrease toward normal, the role of vitamin C in increasing iron excretion disappears (Orkin and Nathan, 2003). Deferoxamine is given intravenously or subcutaneously, often at home using a portable infusion pump, over 8 to 24 hours (usually during sleep) for 5 to 7 days a week. It is also given intravenously over 4 hours at the time of blood transfusion in many centers. Creative strategies such as behavioral contracting have been used to assist the child in complying with the deferoxamine regimen.

In some children with severe splenomegaly who demonstrate increased transfusion requirements, a splenectomy may be necessary to decrease the disabling effects of abdominal pressure and to increase the life span of supplemental RBCs. Over time, the spleen may accelerate the rate of RBC destruction and thus increase transfusion requirements. After a splenectomy, children generally require fewer transfusions, although the basic defect in Hgb synthesis remains unaffected. A major postsplenectomy complication is severe and overwhelming infection. Therefore these children continue to receive prophylactic antibiotics with close medical supervision for many years and should receive the pneumococcal and meningococcal vaccines in addition to the regularly scheduled immunizations (see Immunizations, Chapter 10).

! NURSING ALERTEnsure that the family/patient understands the need to notify the health professional of all fevers of 38.5° C (101.5° F) or greater because of the risk of sepsis in a child with asplenia.

Prognosis.Most children treated with blood transfusion and early chelation therapy survive well into adulthood. The most common cause of death is iron-induced heart disease, multiple organ failure, postsplenectomy sepsis, liver disease, and malignancy (Paley, 2000). Hematopoietic stem cell transplantation has the best results in the least symptomatic patients with a 75% rate of complication-free survival (Orkin and Nathan, 2003; Paley, 2000).

Nursing ConsiderationsThe objectives of nursing care are to (1) promote compliance with transfusion and chelation therapy, (2) assist the child in coping with the anxiety-provoking treatments and the effects of the illness, (3) foster the child's and family's adjustment to a chronic illness, and (4) observe for complications of multiple blood transfusions. Basic to each of these goals is explaining to parents and older children the defect responsible for the disorder, its effect on RBCs, and the potential effects of untreated iron overload (such as diabetes and heart disease). Because the prevalence of this condition is high among families of Mediterranean descent, the nurse also inquires about the family's previous knowledge about thalassemia. All families with a child with thalassemia should be tested for the trait and referred for genetic counseling.

As with any chronic illness, the needs of the family must be met for optimum adjustment to the stresses imposed by the disorder (see Chapter 18). Sources of information for the family include the Cooley's Anemia Foundation* and the Thalassemia Action Group.† Genetic counseling for the parents and fertile offspring is mandatory, and both prenatal diagnosis using amniocentesis at 20 weeks of gestation or fetal blood sampling at 10 weeks and screening for thalassemia trait are available.

* 129-09 26th Avenue, Suite 203, Flushing, NY 11354; (718) 321-2873 or (800) 522-7222; fax: (718) 321-3340; website: http://www.thalassemia.org.† 129-09 26th Avenue, Suite 203, Flushing, NY 11354; (718) 321-2873 or (800) 522-7222; fax: (718) 321-3340; website: http://www.cooleysanemia.org/sections.php?sec=2&tab=83.

APLASTIC ANEMIAAplastic anemia (AA) refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. The peripheral blood smear demonstrates pancytopenia or the triad of profound anemia, leukopenia, and thrombocytopenia. Hypoplastic anemia is characterized by a profound depression of RBCs, but normal or slightly decreased white blood cells (WBCs) and platelets.

EtiologyAplastic anemia can be primary (congenital, or present at birth) or secondary (acquired). The best-known congenital disorder of which aplastic anemia is an outstanding feature is Fanconi syndrome, a rare hereditary disorder characterized by pancytopenia, hypoplasia of the bone marrow, and patchy brown discoloration of the skin resulting from the deposit of melanin and associated with multiple congenital anomalies of the musculoskeletal and genitourinary systems. The syndrome appears to be inherited as an autosomal recessive trait with varying penetrance; therefore, affected siblings may demonstrate several different combinations of defects.

Several etiologic factors contribute to the development of acquired hypoplastic anemia; however, about 70% of all cases are considered idiopathic (Box 26-4). Acquired aplastic anemia is classified as either severe acquired aplastic anemia or moderate acquired aplastic anemia. The following discussion focuses on severe acquired aplastic anemia, which carries a poorer prognosis and follows a more rapidly fatal course than the primary types.

BOX 26-4 Common Causes of Acquired Aplastic Anemia

Infection with the human parvovirus (HPV), hepatitis, or overwhelming infection

Irradiation

Drugs such as the chemotherapeutic agents and several antibiotics, one of the most notable being chloramphenicol

Industrial and household chemicals, including benzene and its derivatives, which are found in petroleum products, dyes, paint remover, shellac, and lacquers

Infiltration and replacement of myeloid elements, such as in leukemia or the lymphomas

Idiopathic, in which no identifiable precipitating cause can be found

Diagnostic EvaluationThe onset of clinical manifestations, which include anemia, leukopenia, and decreased platelet count, is usually insidious. Definitive diagnosis is determined from bone marrow aspiration, which demonstrates the conversion of red bone marrow to yellow, fatty bone marrow. Severe AA is defined as less than 25% bone marrow cellularity with at least two of the following findings: granulocyte count <500/mm3; platelet count <20,0000/mm3; and reticulocyte count <40,000/mm3 (Shimamura and Guinam, 2003; Shende, 2000). Moderate AA is defined as more than 25% bone marrow cellularity with at least two of the following findings: granulocyte count >500/mm3; platelet count >20,000/mm3; and reticulocyte count >40,000/mm3 (Shimamura and Guinam, 2003; Shende, 2000).

Therapeutic ManagementThe objectives of treatment are based on the recognition that the underlying disease process is failure of the bone marrow to carry out its hematopoietic functions. Therefore therapy is directed at restoring function to the marrow and involves two main approaches: (1) immunosuppressive therapy to remove the presumed immunologic functions that prolong aplasia or (2) replacement of the bone marrow through transplantation. Bone marrow transplantation is the treatment of choice for severe aplastic anemia when a suitable donor exists (see p. 959).

Antilymphocyte globulin (ALG) or antithymocyte globulin (ATG) is the principal drug treatment used for aplastic anemia. The rationale for using ATG is based on the theory that aplastic anemia may be a result of autoimmunity. ATG and cyclosporine suppress T cell-dependent autoimmune responses but does not cause bone marrow suppression. Cyclosporine is administered orally for several weeks to months. The optimum schedule for ATG administration is still under investigation. It is usually given intravenously over 12 to 16 hours for 4 days, after a test dose to check for hypersensitivity. A course may be repeated, depending on the reduction in circulating lymphocytes and the patient's response. Because of the hypersensitivity response associated with ATG (i.e., fever, chills, myalgias), methylprednisolone is given intravenously to prevent these side effects. Colony-stimulating factor (CSFs), and granulocyte-macrophage colony-

stimulating factor (GM-CSF) given parenterally, may be used to enhance bone marrow production. Androgens may be used with ATG to stimulate erythropoiesis if the AA is nonresponsive to initial therapies. Cyclosporine may be administered in children who fail to respond to ATG, and success has also been achieved using high-dose methylprednisolone.

Hematopoietic stem cell transplantation should be considered early in the course of the disease if a compatible donor can be found. Transplantation is more successful when performed before multiple transfusions have sensitized the child to leukocyte and human leukocyte antigens (HLA). Hematopoietic stem cell transplantation is associated with an 85% survival rate in untransfused patients compared with a 70% survival rate in transfused patients (Shende, 2000).

Nursing ConsiderationsThe care of the child with aplastic anemia is similar to that of the child with leukemia (see p. 958)—specifically, preparing the child and family for the diagnostic and therapeutic procedures, preventing complications from the severe pancytopenia, and emotionally supporting them in terms of a potentially fatal outcome. Information and support are available from the Aplastic Anemia and MDS International Foundation, Inc.*

Because each of these nursing considerations is discussed in the section on leukemia, only the exceptions are presented here. The drug ATG is usually administered by way of a central vein. If not, vigilant care must be directed to the IV infusion to prevent extravasation. Meticulous care of the venous access is essential because of the child's susceptibility to infection. CSFs are usually given by subcutaneous injection over several days. Chemotherapeutic agents have been reported in the treatment of the relapsed patient with AA after ATG/CSF therapy. Many of the side effects associated with chemotherapy such as nausea and vomiting, alopecia, and mucositis are experienced by children receiving treatment for AA. Specialized care is required for children who have hematopoietic stem cell transplantation (HSCT) (see p. 959).

* PO Box 613 Annapolis, MD 21404-0613; (800) 747-2828; fax (410) 867-0240; website: http://www.aamds.org.

DEFECTS IN HEMOSTASISHemostasis is the process that stops bleeding when a blood vessel is injured. Vascular and plasma clotting factors, as well as platelets, are required. A complex system of clotting, anticlotting, and clot breakdown (fibrinolysis) mechanisms exists in equilibrium to ensure clot formation only in the presence of blood vessel injury and to limit the clotting process to the site of vessel wall injury. Dysfunction in these systems will lead to bleeding or abnormal clotting. Although the coagulation process is complex, clotting depends on three factors: (1) vascular influence, (2) platelet role, and (3) clotting factors.

HEMOPHILIAThe term hemophilia refers to a group of bleeding disorders in which there is a deficiency of one of the factors necessary for coagulation of the blood. Although the symptomatology is similar regardless of which clotting factor is deficient, the identification of specific factor deficiencies allows definitive treatment with replacement agents.

In about 80% of all cases of hemophilia, the inheritance pattern is demonstrated as X-linked recessive (see Appendix B). The two most common forms of the disorder are factor VIII deficiency (hemophilia A, or classic hemophilia) and factor IX deficiency (hemophilia B, or Christmas disease). von Willebrand disease (vWD) is another hereditary bleeding disorder characterized by a deficiency, abnormality, or absence of the protein called von Willebrand factor (vWF) and a deficiency of factor VIII. Unlike hemophilia, vWD affects both males and females. The following discussion is primarily concerned with factor VIII deficiency, which accounts for 80% to 85% of all hemophilia cases.

PathophysiologyThe basic defect of hemophilia A is a deficiency of factor VIII (antihemophilic factor [AHF]). AHF is produced by the liver and is necessary for the formation of thromboplastin in phase I of blood coagulation. The less AHF found in the blood, the more severe the disease. Individuals with hemophilia have two of the three factors required for coagulation: vascular influence and

platelets. Therefore they may bleed for longer periods, but not at a faster rate.

Bleeding into subcutaneous and intramuscular tissue are common. Hemarthrosis, which is bleeding into a joint space, is the most frequent type of internal bleeding. Bony changes and crippling deformities occur after repeated bleeding episodes over several years. Signs of hemarthrosis are swelling, warmth, redness, pain, and loss of movement. Bleeding in the neck, mouth, or thorax is serious, because the airway can become obstructed. Intracranial hemorrhage can have fatal consequences and is one of the major causes of death. Hemorrhage anywhere along the GI tract can lead to anemia, and bleeding into the retroperitoneal cavity is especially hazardous because of the large space for blood to accumulate. Hematomas in the spinal cord can cause paralysis.

Diagnostic EvaluationOvert, prolonged hemorrhage is readily apparent; bleeding into tissues is less apparent (Box 26-5). The diagnosis is usually made from a history of bleeding episodes, evidence of X-linked inheritance (only one third of the cases are new mutations), and laboratory findings. The tests specific for hemophilia plasma depend on specific factors for a reaction to occur, such as the partial thromboplastin time (PTT). Specific determination of factor deficiencies requires assay procedures normally performed in specialized laboratories. Carrier detection is possible in classic hemophilia using DNA testing and is an important consideration in families in which female offspring may have inherited the trait.

BOX 26-5 Clinical Manifestations of Hemophilia

Prolonged bleeding anywhere from or in the body

Hemorrhage from any trauma—loss of deciduous teeth, circumcision, cuts, epistaxis, injections

Excessive bruising—even from a slight injury, such as a fall

Subcutaneous and intramuscular hemorrhages

Hemarthrosis (bleeding into the joint cavities), especially the knees, ankles, and elbows

Hematomas—pain, swelling, and limited motion

Spontaneous hematuria

Therapeutic ManagementThe primary therapy for hemophilia is replacement of the missing clotting factor. The products available are factor VIII concentrate from pooled plasma or a genetically engineered recombinant, to be reconstituted with sterile water immediately before use, and DDAVP (1-deamino-8-d-arginine vasopressin), a synthetic form of vasopressin that increases plasma factor VIII and vWF levels, is the treatment of choice in mild hemophilia and vWD if the child shows an appropriate response. DDAVP is not effective in the treatment of severed hemophilia A, severe vWD, or any form of hemophilia B. Vigorous therapy is instituted to prevent chronic crippling effects from joint bleeding.

Other drugs may be included in the therapy plan, depending on the source of the hemorrhage. Corticosteroids are given for hematuria, acute hemarthrosis, and chronic synovitis. Nonsteroidal antiinflammatory drugs (NSAIDs), such as ibuprofen, are effective in relieving pain caused by synovitis; however, they must be used with caution because they inhibit platelet function (National Hemophilia Foundation, 2003; Dragone and Karp, 1996). Oral administration or local application of epsilon-aminocaproic acid (Amicar) prevents clot destruction; however, its use is limited to mouth or trauma surgery, and a dose of factor concentrate must be given first.

A regular program of exercise and physical therapy is an important aspect of management. Physical activity within reasonable limits strengthens muscles around joints and may decrease the number of spontaneous bleeding episodes.

Treatment without delay results in more rapid recovery and a decreased likelihood of complications; therefore, most children are treated at home. The family is taught the technique of venipuncture and to administer the AHF to children older than 2 to 3 years of age. The child learns the procedure for self-

administration at 8 to 12 years of age. Home treatment is highly successful, and the rewards, in addition to the immediacy, are less disruption of family life, fewer school or work days missed, and enhancement of the child's self-esteem and independence.

Primary prophylaxis in hemophilia patients has been practiced for many years in European countries (Fischer and others, 2001) and has proved to be effective in preventing arthropathy. Primary prophylaxis involves the infusion of factor VIII concentrate on a regular basis before the onset of joint damage. Secondary prophylaxis involves the infusion of factor VIII concentrate on a regular basis after the child experiences his first joint bleed. The infusions are given three times a week. Aggressive factor replacement may be a cost-effective alternative to primary prophylaxis. This involves the infusion of a high dose of factor VIII concentrate when a joint bleed occurs, followed by 2 days of more standard doses of factor VIII concentrate with consideration of additional treatment every other day for one week (Montgomery and others, 2003; Nolan and others, 2003).

Prognosis.Although there is no cure for hemophilia, its symptoms can be controlled and its potentially crippling deformities greatly reduced or even avoided. Today many children with hemophilia function with minimal or no joint damage. They are normal children with an average life expectancy in every respect but one: they have a tendency to bleed, which is a significant inconvenience but not necessarily a life-threatening event.

Unfortunately, those individuals with hemophilia who were treated before current purification techniques for factor VIII concentrate (between 1979 and 1985) may have been exposed to the human immunodeficiency virus (HIV). It is estimated that more than 50% of these patients have seroconverted to HIV-positive status (Butler and others, 2003). As these individuals become sexually active, the issue of sexual transmission of HIV becomes increasingly important. The adolescent must be knowledgeable regarding safe sexual behavior. Individuals with hemophilia diagnosed and treated with factor concentrates since 1985 are at virtually no risk for developing HIV infection

from treatment. Current manufacturing techniques have also greatly reduced the risk of hepatitis transmission.

Gene therapy may prove to be a treatment option in the future. This therapy involves introducing a working copy of the factor VIII gene into a patient who has a flawed copy of the gene. Problems exist with appropriate selection of the vector, identification of the cell for gene expression, and control of side effects (Montgomery and others, 2003).

Nursing ConsiderationsThe earlier a bleeding episode is recognized, the more effectively it can be treated. Signs that indicate internal bleeding are especially important to recognize. Children are aware of internal bleeding and are very reliable in telling the examiner where an internal bleed is. In addition to the manifestations described (see Box 26-5), the nurse maintains a high level of suspicion when a child with hemophilia demonstrates signs such as headache, slurred speech, loss of consciousness (from cerebral bleeding), and black tarry stools (from GI bleeding).

Prevent Bleeding.The goal of prevention of bleeding episodes is directed toward decreasing the risk of injury. Prevention of bleeding episodes is geared mostly toward appropriate exercises to strengthen muscles and joints and to allow age-appropriate activity. During infancy and toddlerhood the normal acquisition of motor skills creates innumerable opportunities for falls, bruises, and minor wounds. Restraining the child from mastering motor development can herald more serious long-term problems than allowing the behavior. However, the environment should be made as safe as possible, with close supervision maintained during playtime, to minimize incidental injuries.

For older children the family usually needs assistance in preparing for school. A nurse who knows the family can be instrumental in discussing the situation with the school nurse and in jointly planning an appropriate schedule of activity. Because almost all persons with hemophilia are boys, the physical limitations in regard to active sports may be a difficult adjustment, and activity restrictions must be tempered with

sensitivity to the child's emotional, as well as physical, needs. Use of protective equipment, such as padding and helmets, is particularly important, and noncontact sports, especially swimming, walking, jogging, tennis, golf, fishing and bowling are encouraged (National Hemophilia Foundation, 2003).

To prevent oral bleeding, some readjustment in terms of dental hygiene may be needed to minimize trauma to the gums, such as use of a water irrigating device, softening the toothbrush in warm water before brushing, or using a sponge-tipped disposable toothbrush. A regular toothbrush should be soft bristled and small in size.

Because any trauma can lead to a bleeding episode, all persons caring for these children must be aware of their disorder. These children should wear medical identification, and older children should be encouraged to recognize situations in which disclosing their condition is important, such as during dental extraction or injections. Health personnel need to take special precautions to prevent the use of procedures that may cause bleeding, such as IM injections. The subcutaneous route is substituted for IM injections whenever possible. Venipunctures for blood samples are usually preferred for these children. There is usually less bleeding after the venipuncture than after finger or heel punctures. Neither aspirin nor any aspirin-containing compound should be used. Acetaminophen (Tylenol) is a suitable aspirin substitute, especially for use during control of pain at home.

Recognize and Control Bleeding.As noted, the earlier a bleeding episode is recognized, the more effectively it can be treated. Factor replacement therapy should be instituted according to established medical protocol, and supportive measures may be implemented, such as RICE, which is (1) rest, (2) ice, (3) compression, and (4) elevation. When parents and older children are taught such measures beforehand, they can be prepared to initiate immediate treatment. Plastic bags of ice or cold packs should be kept in the freezer for such emergencies. However, such measures do not take the place of factor replacement.

Prevent Crippling Effects of Bleeding.

As a result of repeated episodes of hemarthrosis, incompletely absorbed blood in the joints, and limitation of motion, bone, and muscle changes occur that result in flexion contractures and joint fixation. During bleeding episodes the joint is elevated and immobilized. Active range-of-motion exercises are usually instituted after the acute episode. This allows the child to control the degree of exercise and discomfort. If an exercise program is instituted in the home, a physical therapist or public health nurse may need to supervise compliance with the regimen. Rarely, orthopaedic intervention, such as casting, application of traction, or aspiration of blood, may be necessary to preserve joint function. Diet is also an important consideration, because excessive body weight can increase the strain on affected joints, especially the knees, and predispose to hemarthrosis. Consequently, calories need to be supplied in accordance with energy requirements.

Support Family and Prepare for Home Care.Genetic counseling is essential as soon as possible after diagnosis. Unlike many other disorders in which both parents carry the trait, the feeling of responsibility for this condition usually rests with the mother. Without an opportunity to discuss her feelings, the marital relationship can suffer. Technology is now available to identify carriers in approximately 80% of cases and may reduce the anxiety regarding childbearing in females who may be at risk of carrying the defective gene, such as sisters or maternal aunts of an affected male. The discovery of factor concentrates has greatly changed the outlook for these children. Bleeding can be minimized, and the child can live a much more normal, unrestricted life. Children are taught to take responsibility for their disease at an early age. They learn their limitations and other preventive measures, as well as self-administration of the prophylactic AHF.

The needs of families who have children with hemophilia are best met through a comprehensive team approach of physicians (pediatrician, hematologist, orthopedist), nurse practitioner, nurse, social worker, and physical therapist. Parent-group discussions are beneficial in meeting those needs often best met by similarly affected families. For example, with

the improved prognosis for these children, parents and adolescents with hemophilia are faced with vocational and financial problems, in addition to concern over future childbearing. After children reach 21 years of age, many insurance companies will no longer carry them. This can be disastrous in terms of the cost of treatment. The National Hemophilia Foundation* and the Canadian Hemophilia Society† provide numerous services and publications for both health providers and families. Financial support is particularly important. A person with severe hemophilia may require factor replacement therapy and other medical treatments that cost in excess of $70,000 to $90,000 a year.

Children who have become infected with HIV through transfusions and factor replacement products are faced with the consequences of this dreaded disease. Consequently, they need the support of health professionals, especially in the areas of safe sexual practices to avoid disease transmission and public education regarding acquired immunodeficiency syndrome (AIDS) and ways to deal with public reactions to persons who have AIDS (see p. 978).

* National Hemophilia Foundation, 116 West 43rd Street, 11th Floor, New York, NY 10001; (800) 42HANDI or (212) 328-3700; fax (212) 328-3777; email: info@hemophilia.org; website: http://www.hemophilia.org.† Canadian Hemophilia Society, 625 President Kennedy, Suite 1210, Montreal, Quebec H3A 1K2; (514) 848-0503; e-mail: chs@odysee.net.

IDIOPATHIC THROMBOCYTOPENIC PURPURAIdiopathic thrombocytopenic purpurea (ITP) is an acquired hemorrhagic disorder characterized by (1) thrombocytopenia, excessive destruction of platelets, (2) purpura, a discoloration caused by petechiae beneath the skin, and (3) a normal bone marrow with normal or increased number of immature platelets (megakaryocytes) and eosinophils. Although the cause is unknown, it is believed to be an autoimmune response to disease-related antigens. It is the most frequently occurring thrombocytopenia of childhood. The greatest frequency of occurrence is between 2 and 10 years of age.

The disease occurs in one of two forms: an acute, self-limiting course or a chronic condition (greater than 6 months' duration). The acute form is most often seen after upper respiratory infections; after the childhood diseases measles, rubella, mumps, and chickenpox; or after infection with parvovirus B19.

Diagnostic EvaluationThe diagnosis is suspected on the basis of clinical manifestations (Box 26-6). In ITP the platelet count is reduced to below 20,000 mm3; therefore, tests that depend on platelet function, such as the tourniquet test, bleeding time, and clot retraction, are abnormal. Although there is no definitive test on which to establish a diagnosis of ITP, several are usually performed to rule out other disorders in which thrombocytopenia is a manifestation, such as systemic lupus erythematosus, lymphoma, or leukemia.

BOX 26-6 Clinical Manifestations of Idiopathic Thrombocytopenic Purpura

Easy bruising

Petechiae

Ecchymoses

Most often over bony prominences

Bleeding from mucous membranes

Epistaxis

Bleeding gums

Internal hemorrhage evidenced by:

Hematuria

Hematemesis

Melena

Hemarthrosis

Menorrhagia

Hematomas over lower extremities

Therapeutic ManagementManagement of ITP is primarily supportive, because the course of the disease is self-limited in the majority of cases. Activity is restricted at the onset while the platelet count is low and while active bleeding or progression of lesions is occurring. Treatment for acute presentation is symptomatic and has included prednisone, IV immune globulin (IVIG), and anti-D antibody. These are not curative therapies. Some experts suggest that no therapy is necessary for the asymptomatic patient, with no difference in the recovery of platelet counts over time. Anti-D antibody is a relatively new therapy for ITP. Infusion of anti-D antibody causes a transient hemolytic anemia in the patient. Along with the clearance of antibody-coated RBCs, there is prolonged survival of platelets resulting from the blockade of the Fc receptors of the reticuloendothelial cells. The platelet count does not increase until 48 hours after an infusion of anti-D antibody; therefore, it is not appropriate therapy for patients who are actively bleeding. The benefits of choosing anti-D antibody therapy over prednisone or IVIG is that anti-D antibody can be given in one dose over 5 to 10 minutes and is significantly less expensive than IVIG. Historically, patients who are treated with prednisone must first undergo a bone marrow examination to rule out leukemia. Therefore the use of anti-D antibody alleviates the need for a bone marrow examination. Patients must meet certain criteria before the administration of anti-D antibody (Box 26-7). Premedication with acetaminophen (such as Tylenol) 5 to 10 minutes before infusion is recommended.

BOX 26-7 Criteria for Anti-D Antibody Therapy

Children between age 1 year and 19 years

Rh(D)-positive blood type

Normal white blood cell count and hemoglobin for age: platelets <30,000/µL

No active mucosal bleeding

No prior history of reaction to plasma products

No patient known to be immunoglobulin A deficient

No concurrent infection

No patient with Evans syndrome (characterized by the combination of idiopathic thrombocytopenia purpura and autoimmune hemolytic anemia)

No patient with suspected lupus or other collagen/vascular disorder

! NURSING ALERTAfter administration of anti-D antibody, observe the child for a minimum of 1 hour and maintain a patent IV line. Obtain baseline vital signs before the infusion and again 5, 20, and 60 minutes after beginning the infusion. Fever, chills, and headache may occur during or shortly after the infusion. If fever, chills, or headache occurs, diphenhydramine (Benadryl) and Solu-Cortef should be given and the patient should be observed for an additional hour.

Splenectomy is reserved for those patients in whom ITP has persisted for 1 year or longer. It is the only treatment associated with long-term remission for 60% to 90% of children. Splenectomy removes the risk of hemorrhage, but increases the risk of septicemia (Wilson, 2003; Bell, 2002; Chu and others, 2000). Before considering splenectomy, it is generally recommended to wait until the child is older than 5 years of age because of the increased risk of bacterial infection. Pneumococcal and meningococcal vaccines are recommended before splenectomy (see Immunizations, Chapter 10). The child also receives penicillin prophylaxis after splenectomy. The length of prophylactic therapy is controversial, but in general, a minimum of 3 years is recommended.

Prognosis.

The majority of children have a self-limited course without major complications. Some children will develop chronic ITP and require ongoing therapy. A splenectomy may modify the disease process, and the child will be asymptomatic.

Nursing ConsiderationsNursing care is largely supportive and should include teaching regarding possible side effects of therapy and limitation in activities while the child's platelet count is 50,000 to 100,000/mm3. Children with ITP should not participate in any contact sports, bike riding, skateboarding, in-line skating, gymnastics, climbing, or running. Parents are encouraged to engage their children in quiet activities and to prevent any injuries to the child's head. The harmful effects of using aspirin and NSAIDs to control pain are critical for these children; therefore, salicylate substitutes (such as acetaminophen) are always used. As in any condition with an uncertain outcome, the family needs emotional support.

DISSEMINATED INTRAVASCULAR COAGULATIONDisseminated intravascular coagulation (DIC), also known as consumption coagulopathy, is characterized by diffuse fibrin deposition in the microvasculature, consumption of coagulation factors, and endogenous generation of thrombin and plasmin. DIC is a secondary disorder of coagulation that occurs as a complication of a number of pathologic processes, such as hypoxia, acidosis, shock, and endothelial damage. It can result from many severe systemic diseases, such as congenital heart disease, necrotizing enterocolitis, gram-negative bacterial sepsis, rickettsial infections, and some severe viral infections.

PathophysiologyDIC occurs when the first stage of the coagulation process is abnormally stimulated. Although no well-defined sequence of events occurs, two distinct phases can be identified. First, when the clotting mechanism is triggered in the circulation, thrombin is generated in greater amounts than can be neutralized by the body. Consequently, there is rapid conversion of fibrinogen to

fibrin, with aggregation and destruction of platelets. If local and widespread fibrin deposition in blood vessels takes place, obstruction and eventual necrosis of tissues occur. Second, the fibrinolytic mechanism is activated, causing extensive destruction of clotting factors. With a deficiency of clotting factors the child is vulnerable to uncontrollable hemorrhage into vital organs. An additional complication is damage and hemolysis of RBCs (Fig. 26-4).FIG. 26-4 Effects of disseminated intravascular coagulation.

Diagnostic EvaluationDIC is suspected when the patient has an increased tendency to bleed (Box 26-8). Hematologic findings include prolonged prothrombin time (PT), PTT, and thrombin time (TT). There is a profoundly depressed platelet count, fragmented RBCs, and depleted fibrinogen.

BOX 26-8 Clinical Manifestations of Disseminated Intravascular Coagulation

Petechiae

Purpura

Bleeding from openings in the skin

Venipuncture site

Surgical incision

Bleeding from umbilicus, trachea (newborn)

Evidence of gastrointestinal bleeding

Hypotension

Organ dysfunction from infarction and ischemia

Therapeutic ManagementTreatment of DIC is directed toward control of the underlying or initiating cause, which in most instances stops the coagulation problem spontaneously. Platelets and fresh-frozen plasma may be needed to replace lost plasma components, especially in the child whose underlying disease remains uncontrolled. The extremely ill newborn infant may require exchange transfusion with fresh blood. The IV administration of heparin to inhibit thrombin formation is most often restricted to patients who have not responded to treatment of the underlying disease or replacement of coagulation factors and platelets.

Nursing ConsiderationsThe goals of nursing care are to be aware of the possibility of DIC in the severely ill child and to recognize signs that might indicate its presence. The skills needed to monitor IV infusion and blood transfusions and to administer heparin are the same as for any child receiving these therapies (see p. 978). (See Chapter 18 for care of the child with a life-threatening illness.)

EPISTAXIS (NOSEBLEEDING)Isolated and transient episodes of epistaxis, or nosebleeding, are common in childhood. The nose, especially the septum, is a highly vascular structure, and bleeding usually results from direct trauma, including blows to the nose, foreign bodies, and nose picking, or from mucosal inflammation associated with allergic rhinitis and upper respiratory infections. The bleeding ordinarily stops spontaneously or with minimum pressure and requires no medical evaluation or therapy.

Recurrent epistaxis and severe bleeding may indicate an underlying disease, particularly vascular abnormalities, leukemia, thrombocytopenia, and clotting factor deficiency diseases (e.g., hemophilia, von Willebrand disease). Nosebleeds are sometimes associated with administration of aspirin, even in normal amounts. Persistent episodes of epistaxis require medical evaluation.

Nursing ConsiderationsIn the event of a nosebleed, an essential intervention is to remain calm. Otherwise, the child will become more agitated, the blood pressure will increase, and the child will not cooperate. Although in most instances a nosebleed is not serious, it can be very upsetting to family members as well. They need reassurance that the loss of blood is not serious and that the bleeding usually stops within 10 to 15 minutes.

To control the bleeding, the child is instructed to sit up and lean forward (not to lie down) to avoid aspiration of blood. Most of the nosebleeding originates in the anterior part of the nasal septum and can be controlled by applying pressure to the soft lower portion of the nose with the thumb and forefinger (see Emergency Treatment box). During this time the child breathes through the mouth.

EMERGENCY TREATMENT: EpistaxisHave child sit up and lean forward (not lying down).

Apply continuous pressure to nose with thumb and forefinger for at least 10 minutes.

Insert cotton or wadded tissue into each nostril, and apply ice or cold cloth to bridge of nose if bleeding persists.

Keep child calm and quiet.

In the event that hemorrhage continues, the child should be evaluated by a practitioner, who may pack the nose with epinephrine-soaked gauze. After a nosebleed, petroleum or water-soluble jelly can be inserted into each nostril to prevent crusting of old blood and to lessen the likelihood of the child's picking at the nose and restarting the hemorrhage. If a child has numerous nosebleeds, factors believed to increase the likelihood of bleeds are eliminated, such as discouraging nose picking or altering the household humidity by placing a cool-mist humidifier in the child's room. Repeated bleeding episodes lasting longer than 30 minutes may be an indication to refer the child for evaluation for the possibility of a bleeding disorder.

NEOPLASTIC DISORDERSNeoplastic disorders are the leading cause of death from disease in children past infancy, and almost half of all childhood cancers involve the blood or blood-forming organs. Leukemias and lymphomas are discussed here. Malignant solid tumors of childhood are discussed elsewhere in relation to the tissues or organs involved.

LEUKEMIASLeukemia, cancer of the blood-forming tissues, is the most common form of childhood cancer. The annual incidence is 3 to 4 cases per 100,000 white children younger than 15 years of age (Margolin, Steuber, and Poplack, 2002). It occurs more frequently in males than in females after age 1 year, and the peak onset is between 2 and 6 years of age. It is one of the forms of cancer that has demonstrated dramatic improvements in survival rates. Current long-term disease-free survival for children with acute lymphoid leukemia approaches 80% (Redner, 2000), whereas acute nonlymphoid leukemia has a 45% to 50% survival rate (Redner, 2000). (See also Prognosis, see p. 959)

Classification

Leukemia is a broad term given to a group of malignant diseases of the bone marrow and lymphatic system. Research has revealed that it is a complex disease of varying heterogeneity. Consequently, classification has become increasingly complex, sophisticated, and essential, because identification of the subtype of leukemia has therapeutic and prognostic implications. The following is a brief overview of the major classification systems currently being used.

Morphology.Two forms are generally recognized in children: acute lymphoid leukemia (ALL) and acute nonlymphoid (myelogenous) leukemia (ANLL or AML). Synonyms for ALL include lymphatic, lymphocytic, lymphoblastic, and lymphoblastoid leukemia. Usually the terms stem cell or blast cell leukemia also refer to the lymphoid type of leukemia. Synonyms for the AML type include granulocytic, myelocytic, monocytic, myelogenous, monoblastic, and monomyeloblastic.

Cytochemical markers. Several chemical stains aid in differentiation between ALL and ANLL. For instance, terminal deoxynucleotidyl transferase (TdT) is able to differentiate between ALL and non-ALL (Margolin, Steuber, and Poplack, 2002).

Chromosome studies. Chromosome analysis has become an important tool in the diagnosis of acute lymphoblastic leukemia. For example, children with trisomy 21 have 20 times the risk of other children for developing ALL. Children with more than 50 chromosomes on the leukemic cells (hyperdiploid) have the best prognosis (Margolin, Steuber, and Poplack, 2002). Translocations of chromosomes also found on the leukemic cells can denote good prognosis, as in the trisomies 4 and 10, or a poor prognosis, as in the t(9:22) or Philadelphia chromosome.

Cell-surface immunologic markers. Cell-surface antigens have permitted differentiation of ALL into three broad classes: non-T, non-B ALL; B-cell ALL; and T-cell ALL. Children with non-T, non-B ALL have the best prognosis, especially if they have the common acute lymphocytic leukemia antigen,

known as CALLA positive, on their cell surfaces (Margolin, Steuber, and Poplack, 2002).

PathophysiologyLeukemia is an unrestricted proliferation of immature WBCs in the blood-forming tissues of the body. Although not a "tumor" as such, the leukemic cells demonstrate the same neoplastic properties as solid cancers. Therefore the resulting pathologic condition and clinical manifestations are caused by infiltration and replacement of any tissue of the body with nonfunctional leukemic cells. Highly vascular organs, such as the spleen and liver, are the most severely affected.

To understand the pathophysiology of the leukemic process, it is important to clarify two common misconceptions. First, although leukemia is an overproduction of WBCs, most often in the acute form the leukocyte count is low (thus the term leukemia). Second, these immature cells do not deliberately attack and destroy the normal blood cells or vascular tissues. Cellular destruction takes place by infiltration and subsequent competition for metabolic elements (Table 26-2).

TABLE 26-2 Pathology and Related Clinical Manifestations of Leukemia

In all types of leukemia the proliferating cells depress the production of formed elements of the blood in bone marrow by

competing for and depriving the normal cells of the essential nutrients for metabolism. The most frequent presenting signs and symptoms of leukemia are a result of infiltration of the bone marrow. The three main consequences are (1) anemia from decreased RBCs, (2) infection from neutropenia, and (3) bleeding from decreased platelet production. The invasion of the bone marrow with leukemic cells gradually causes a weakening of the bone and a tendency toward fractures. As leukemic cells invade the periosteum, increasing pressure causes severe pain.

The spleen, liver, and lymph glands demonstrate marked infiltration, enlargement, and eventually fibrosis. Hepatosplenomegaly is typically more common than lymphadenopathy. The next most important site of involvement is the central nervous system (CNS) secondary to leukemic infiltration, which may cause increased intracranial pressure (see Box 28-1).

Leukemic cells may also invade the testes, kidneys, prostate, ovaries, GI tract, and lungs. With long-term survivors becoming more common, such sites of leukemia invasion, especially the testes, are becoming more important clinically.

Diagnostic EvaluationLeukemia is usually suspected by the history, physical manifestations (see Table 26-2), and a peripheral blood smear that contains immature forms of leukocytes, frequently combined with low blood counts. Definitive diagnosis is based on flow cytometry of the cells obtained in the bone marrow aspiration or biopsy. Flow cytometry identifies the specific type of blast cell. Typically, the bone marrow is hypercellular, with primarily blast cells. After the diagnosis is confirmed, a lumbar puncture is performed to determine if there is any CNS involvement. A few of the children will have CNS involvement at diagnosis, although most are asymptomatic.

Therapeutic ManagementTreatment of leukemia involves the use of chemotherapeutic agents, with or without cranial irradiation, in four phases: (1) induction therapy, which achieves a complete remission or

less than 5% leukemic cells in the bone marrow; (2) CNS prophylactic therapy, which prevents leukemic cells from invading the CNS; (3) intensification therapy (consolidation), which eradicates residual leukemia cells, followed by delayed intensification, which prevents emergence of resistant leukemic clones; and (4) maintenance therapy, which serves to maintain the remission phase. Although the combination of drugs and radiation may vary according to institutions, the prognostic or risk characteristics of the patient, and the type of leukemia being treated, the following general principles for each phase are quite consistently employed.

Remission induction. Almost immediately after confirmation of the diagnosis, induction therapy is begun and lasts for 4 to 6 weeks. The principal drugs used for induction in ALL are corticosteroids, vincristine, and L-asparaginase, with or without doxorubicin. Recent clinical trials have substituted dexamethasone for prednisone because of its effectiveness in crossing the blood-brain barrier and reducing CSF relapse (Colby-Graham, and Chordas, 2003). However, the toxicities of dexamethasone continue to be evaluated. Drug therapy for AML includes doxorubicin or daunorubicin (daunomycin) and cytosine arabinoside; various other drugs such as etoposide or thioguanine may be used.

Because many of the drugs also cause myelosuppression of normal blood elements, the period immediately after a remission can be critical. The body is defenseless against and highly susceptible to infection and spontaneous hemorrhage. Consequently, supportive therapy during this time is essential.

CNS prophylactic therapy. Treatment of the CNS consists of prophylactic therapy using intrathecal chemotherapy with methotrexate, cytarabine, and hydrocortisone. Sometimes methotrexate, as well as cytarabine, may be given as single agents intrathecally. Because of the concern regarding late effects of cranial irradiation, this treatment is reserved for high-risk patients and those with CNS disease.

Intensification or consolidation therapy. After complete remission is obtained, a period of intensified treatment is administered to eradicate residual leukemic cells; this is followed by delayed intensification to prevent emergence of resistant leukemic clones. Intrathecal along with systemic

chemotherapy, including L-asparaginase, high-dose or intermediate-dose methotrexate, cytarabine, vincristine, and mercaptopurine, is administered over a period of several months.

Maintenance therapy. Maintenance therapy is begun after completion of successful induction and consolidation therapy to preserve the remission and further lessen the number of leukemic cells. Combined drug regimens, including daily mercaptopurine, weekly methotrexate, and periodic intrathecal therapy, are administered over the remaining 2-year period. Also, during maintenance therapy, periodic CBCs are taken to evaluate the marrow's response to the drugs.

Reinduction after relapse. The presence of leukemic cells in the bone marrow, CNS, or testes constitutes a relapse. Therapy for the child who has relapsed includes reinduction with prednisone and vincristine, along with a combination of other drugs not previously used. CNS preventive therapy and maintenance therapy follow as outlined previously, after remission occurs.

Hematopoietic stem cell transplantation (HSCT). HSCT has been used successfully for treating children who have ALL and AML. HSCT is not recommended for children with ALL during the first remission because of the excellent results possible with chemotherapy. Because of the poorer prognosis in children with AML, recent studies support HSCT during first remission. From 60% to 70% of children with AML who undergo HSCT experience long-term remission (Colby-Graham, and Chordas, 2003).

Bone marrow used for HSCT may not only be from antigen-matched related donors, but also from matched unrelated donors or mismatched donors. Peripheral blood stem cell transplants are capable of differentiating into specialized cells of the hematologic system and can be obtained from related or unrelated donors or from umbilical cord blood (see also p. XXX). Regardless of the type of transplant, it is accompanied by significant morbidity and mortality, including graft-versus-host disease (GVHD), overwhelming infection, or severe organ damage.

PrognosisThe most important prognostic factors for determining long-term survival for children with ALL (in addition to treatment) are (1) the initial WBC count, (2) the child's age at the time of diagnosis, (3) the type of cell involved, (4) the sex of the child, and (5) karyotype analysis. Children with a normal or low WBC count and who have non-T, non-B ALL and are CALLA positive have a much better prognosis than those with a high count or other cell types. Children diagnosed between 2 and 9 years of age have consistently demonstrated a better outlook than those diagnosed before 2 or after 10 years of age, and girls appear to have a more favorable prognosis than boys. Children with a deoxyribonucleic acid (DNA) index greater than 1.16 (hyperdiploid) and translocation of chromosomes 4 and 10 have a better prognosis (Margolin, Steuber, and Poplack, 2002).

Late Effects of TreatmentAlthough vigorous treatment of childhood cancers has resulted in dramatically improved survival rates, increasing concern surrounds late effects–adverse changes related to treatment modalities—and recurrence of the disease process. Almost no organ is exempt, and almost every antineoplastic agent, including and especially irradiation, is responsible for some adverse effect.

The most devastating late effect is development of a second malignancy. Children who received cranial irradiation at age 5 years or younger are most susceptible to developing brain tumors (Silverman and Sallan, 2003). Treatment with anthracycline is associated with cardiomyopathy; cranial irradiation and intrathecal chemotherapy are associated with cognitive and neuropsychologic deficits, which are just a few of the long-term sequelae. Consequently, close monitoring for late effects is essential, especially with the advent of additional clinical trials.

Nursing ConsiderationsNursing care of the child with leukemia is directly related to the regimen of therapy. General psychologic interventions during each phase of therapy are discussed in Chapter 18.

AssessmentThe history and physical examination often yield the first clues to the presence of neoplastic disease. Vague complaints such as fatigue, pain in a limb, night sweating, lack of appetite, headache, and general malaise may be the earliest clues of leukemia.

Nursing DiagnosesMany nursing diagnoses become apparent after an assessment of the child with leukemia and the family. Some are considered in the Nursing Care Plan on pp. 966 to 970. Others will be identified in specific situations.

PlanningThe goals of nursing care of the child with leukemia and the family include:

1. Child will receive appropriate primary health care.

2. Child and family will be prepared for diagnostic and therapeutic procedures.

3. Child will experience minimal complications of myelosuppression.

4. Problems of irradiation and drug toxicity will be managed.

5. Child and family will receive adequate support and education.

ImplementationNursing care of the child with leukemia is directly related to the regimen of therapy. Nurses working with families of children with cancer have a significant supportive role in helping them understand the various therapies, preventing or managing expected side effects or toxicities, observing for late effects of treatment, and helping the child and family live as normal a life as possible and cope with the emotional aspects of the disease. Education is a constant feature of the nursing role, especially in

terms of clinical trials and home care. Diagnosis of leukemia tends to generate anxiety in families and patients. The nurse is instrumental in providing support and reassurance, as well as accurate explanation regarding diagnostic tests, procedures, and treatment plans.

Prepare Child and Family for Diagnostic and Therapeutic Procedures.From the time before diagnosis to cessation of therapy, children must undergo several tests; the most traumatic are bone marrow aspiration or biopsy and lumbar punctures. Multiple finger sticks and venipunctures for blood analysis and drug infusion are common occurrences. Therefore the child needs an explanation of each procedure and what can be expected. In addition, effective pharmacologic measures, including conscious and unconscious sedation, and nonpharmacologic strategies are used to reduce discomfort associated with these painful procedures.

Relieve Pain.The effective use of analgesia is especially important when the malignant process is uncontrolled and causes acute pain. Dosages of opioids (narcotics) are adjusted or titrated to the child's needs and administered around the clock for optimal pain control. Nonpharmacologic strategies should be implemented as needed but are not substitutes for pharmacologic management. The reader is encouraged to review the principles of pain assessment and management presented in Chapter 21 and Preparation for Procedures in Chapter 22 when caring for a child with leukemia.

Prevent Complications of Myelosuppression.The leukemic process and most of the chemotherapeutic agents cause myelosuppression. The reduced numbers of blood cells result in secondary problems of infection, bleeding tendencies, and anemia. Supportive care involves both medical and nursing management. Because these are so

closely linked, they are discussed together rather than separately.

Infection.A frequent complication of treatment for childhood cancer is overwhelming infection secondary to neutropenia. The child is most susceptible to overwhelming infection during three phases of the disease: (1) at the time of diagnosis and relapse when the leukemic process has replaced normal leukocytes, (2) during immunosuppressive therapy, and (3) after prolonged antibiotic therapy that predisposes to the growth of resistant organisms. However, the use of granulocyte colony-stimulating factor (GCSF) has reduced the incidence and duration of infection in children receiving treatment for cancer.

The first defense against infection is prevention. When the child is hospitalized, the nurse employs all measures to control transfer of infection. These typically include the use of a private room, restriction of all visitors and health personnel with active infection, and strict handwashing technique with an antiseptic solution. In some research centers, special germ-free environments are available during complete myelosuppression from intensive chemotherapy or for bone marrow transplant.

! NURSING ALERTBecause the usual viral infections of childhood are particularly dangerous, the child is not immunized against these diseases (measles, rubella, mumps, and polio) until the immune system is capable of responding appropriately to the vaccine. If given when the immune system is depressed, the attenuated virus can result in an overwhelming infection. The child can receive the Salk (inactivated) vaccine for poliomyelitis. Children with cancer should not routinely receive the varicella vaccine. Siblings and other family members can receive the varicella vaccine without risk to the child with cancer (American Academy of Pediatrics, 2003) (Chapter 10).

The child is evaluated for potential sites of infection (e.g., mucosal ulceration; skin abrasion; skin tear, such as a hangnail) and observed for any elevation in temperature. To identify the source of infection, chest radiographs and blood, stool, urine, and nasopharyngeal cultures are taken. IV antibiotics are administered, and if this therapy is prolonged, a venous access device, such as a peripherally inserted central catheter (PICC), intermittent infusion device (saline lock or PRN adaptor), catheter, or implanted infusion port, is used to maintain IV access.

Prevention of infection continues to be a priority after discharge from the hospital. Ordinarily, the child is allowed to return to school when the WBC count is at a satisfactory level, usually an absolute neutrophil count (ANC) greater than 500/mm3 (see Guidelines box). At all times, family members are encouraged to practice good handwashing to prevent introducing pathogens into the home. The child may need to be isolated from school contacts in the event of an outbreak of a childhood disease, especially chickenpox.

GUIDELINES: Calculating the Absolute Neutrophil Count

Determine the total percent of neutrophils ("polys" or "segs" and "bands").

Multiply white blood cell (WBC) count by percent of neutrophils.

Example:

WBC = 1000, neutrophils = 7%, nonsegmented neutrophils (bands) = 7%

Step 1: 7% + 7% = 14%

Step 2: 0.14 × 1000 = 140 absolute neutrophil count (ANC)

Nutrition is another important component of infection prevention. An adequate protein-caloric intake provides the child with better host defenses against infection and increased tolerance to chemotherapy and irradiation.

However, providing optimum nutrition during periods of anorexia and vomiting from chemotherapy is a tremendous challenge (see Feeding the Sick Child, Chapter 22).

Hemorrhage.Before the use of transfused platelets, hemorrhage was a leading cause of death in patients with leukemia. Now most bleeding episodes can be prevented or controlled with the administration of platelet concentrates or platelet-rich plasma.

Because infection increases the tendency toward hemorrhage, and because bleeding sites become more easily infected, skin punctures are avoided whenever possible. When finger sticks, venipunctures, IM injections, and bone marrow aspirations are performed, aseptic technique must be employed, as well as continued observation for bleeding. Meticulous mouth care is essential, because gingival bleeding with resultant mucositis is a frequent problem. Because the rectal area is prone to ulceration from various drugs, feces and urine are removed immediately, and the perianal area is washed. Using rectal temperatures are avoided to prevent trauma. Children are advised to avoid activities that might cause injury or bleeding, such as riding bicycles or skateboards, climbing trees or playground equipment, and playing contact sports.

Platelet transfusions are generally reserved for active bleeding episodes that do not respond to local treatment and that may occur during induction or relapse therapy. Epistaxis and gingival bleeding are the most common. The nurse teaches parents and older children measures to control nosebleeding (see p. 957). Pressure at the site without disturbing clot formation is the general rule.

During bleeding episodes the parents and child need much emotional support. Often parents will request a platelet transfusion, unaware of the need for trying local measures first. The nurse can be instrumental in allaying anxiety by acknowledging the feelings of the child and family and explaining the reason for delaying a platelet transfusion until absolutely necessary.

Anemia.Initially, anemia may be profound from complete replacement of the bone marrow by leukemic cells. During induction therapy, blood transfusions may be necessary. The usual precautions in caring for the child with anemia are instituted (see p. 941).

Use Precautions in Administering and Handling Chemotherapeutic Agents.Many chemotherapeutic agents are vesicants (sclerosing agents) that can cause severe cellular damage if even minute amounts of the drug infiltrate surrounding tissue. Only nurses experienced with chemotherapeutic agents should administer vesicants. Guidelines are available* and must be followed exactly to prevent tissue damage to patients. Interventions for extravasation vary, but each nurse should be aware of the institution's policies and implement them at once.

In addition to extravasation, a potentially fatal complication is anaphylaxis, especially from L-asparaginase, teniposide (VM-26), etoposide (VP-16), bleomycin, and cisplatin. Nursing responsibilities include prevention of, recognition of, and preparation for serious reactions. Prevention begins with a careful history for known allergy.

In addition to the many responsibilities nurses must have in regard to the child and family, they must also use safeguards to protect themselves. Handling chemotherapeutic agents may present risks to handlers and to their offspring, although the exact degree of risk is not known.

Some children have a venous access device, which facilitates administration of IV drugs. During treatment and remission, many drugs are taken orally at home. Compliance with the medication schedule is essential, and nurses play an important role in educating the family about the drugs and encouraging adherence to the plan.

! NURSING ALERT

Chemotherapeutic drugs must be given through a free-flowing IV line. The infusion is stopped immediately if any sign of infiltration (pain, stinging, swelling, or redness at the cannulation site) occurs.

! NURSING ALERTWhen chemotherapeutic and immunologic agents are given, the child must be observed for 20 minutes after the infusion for signs of anaphylaxis (cyanosis, hypotension, wheezing, severe urticaria). Emergency equipment (especially blood pressure monitor and bag-valve-mask) and emergency drugs (especially oxygen, epinephrine, antihistamine, aminophylline, corticosteroids, and vasopressors) must be available. If a reaction is suspected, the drug is discontinued, the IV line is flushed with saline, and the child's vital signs and subsequent responses are monitored.* Cancer Chemotherapy Guidelines can be obtained from the Oncology Nursing Society, 501 Holiday Drive, Pittsburgh, PA 15220-2749; (412) 921-7373; website: http://www.ons.org.

Manage Problems of Drug Toxicity.Chemotherapy presents several nursing challenges. The complexity of the treatment protocols is often overwhelming to families. In addition, each therapy is associated with a number of predictable side effects. Nurses must be aware of these side effects and use judgment in recognizing actions, as well as toxicities (Box 26-9).

BOX 26-9 Summary of Selected Chemotherapeutic Agents Used in the Treatment of Childhood Leukemias and Lymphomas*BLEOMYCIN (BLENOXANE)

Administration

IV, IM, SC

Side Effects and Toxicity

Allergic reaction—fever, chills, hypotension, anaphylaxis

Fever (nonallergic)

N/V (mild)†

Stomatitis

Cumulative dose effects include:

Skin—rash, hyperpigmentation, thickening, ulceration, peeling, nail changes, alopecia

Lungs—pneumonitis with infiltrate that can progress to fatal fibrosis

Comments and Specific Nursing Considerations

Should give test dose (SC) before therapeutic dose is administered

Have emergency drugs at bedside

Hypersensitivity occurs with first one to two doses

May give acetaminophen before drug to reduce likelihood of fever

Concentration of drug in skin and lungs accounts for toxic effects

Perform pulmonary function tests at baseline, during and following therapy

CYCLOPHOSPHAMIDE (CYTOXAN, CTX, NEOSAR)

Administration

PO, IV, IM

Side Effects and Toxicity

N/V (3 to 4 hours later) (severe at high doses)

BMD (10 to 14 days later)

Alopecia

Hemorrhagic cystitis

Severe immunosuppression

Stomatitis (rare)

Hyperpigmentation

Transverse ridging of nails

Infertility

Cardiac toxicity

SIADH

Comments and Specific Nursing Considerations

BMD has platelet-sparing effect

Give dose early in day to allow adequate fluids afterward

Force fluids before administering drug and for 2 days after to prevent chemical cystitis; encourage frequent voiding even during night

Warn parents to report signs of burning on urination or hematuria to practitioner

Mesna is given to prevent hemorrhagic cystitis

CYTOSINE ARABINOSIDE (ARA-C, CYTOSAR, CYTARABINE, ARABINOSYL CYTOSINE)

Administration

IV, IM, SC, IT

Side Effects and Toxicity

Alopecia

N/V (mild)

BMD (7 to 14 days later)

Mucosal ulceration

Immunosuppression

Hepatitis (usually subclinical)

Fever, conjunctivitis and maculopapular rash with high doses

Comments and Specific Nursing Considerations

Crosses blood-brain barrier

Use with caution in patients with hepatic dysfunction

Administer steroid eye drops to prevent conjunctivitis with high doses

CORTICOSTEROIDS (HORMONES)

Administration

PO, IT; IM or IV rarely used

Side Effects and Toxicity, Short-Term

For short-term use, no acute toxicity

Usual side effects are mild: moon face, fluid retention, weight gain, mood changes, increased appetite, gastric irritation, insomnia, susceptibility to infection

Comments and Specific Nursing Considerations

Explain expected effects, especially in terms of body image, increased appetite, and personality changes

Monitor weight gain

Recommend moderate salt restriction

Administer with antacid and early in morning (sometimes given every other day to minimize side effects)

May need to disguise bitter taste (crush tablet and mix with syrup, jam, ice cream, or other highly flavored substance; use ice to numb tongue before administration; place tablet in gelatin capsule if child can swallow it)

Observe for potential infection sites; usual inflammatory response and fever are absent

Side Effects and Toxicity, Long-Term

Long-term effects of chronic steroid administration are mood changes, hirsutism, trunk obesity (buffalo hump), thin extremities, muscle wasting and weakness, osteoporosis, poor wound healing, bruising, potassium loss, gastric bleeding, hypertension, diabetes mellitus, growth retardation

Comments and Specific Nursing Considerations

Same as for short-term use; in addition, encourage foods high in potassium (bananas, raisins, prunes, coffee, chocolate)

Test stools for occult blood

Monitor blood pressure

Test blood for sugar and urine for acetone

Observe for signs of abrupt steroid withdrawal: flulike symptoms, hypotension, hypoglycemia, shock

DACARBAZINE (DTIC-DOME)

Administration

IV

Side Effects and Toxicity

N/V (especially after first dose) (severe)

BMD (7 to 14 days later)

Alopecia

Flulike syndrome

Burning sensation in vein during infusion (not extravasation)

Comments and Specific Nursing Considerations

Vesicant (less sclerosive)

Must be given cautiously in patients with renal dysfunction

Decrease IV rate or use warm, moist towels on IV site to decrease burning

DAUNORUBICIN (DAUNOMYCIN, RUBIDOMYCIN) AND DOXORUBICIN (ADRIAMYCIN, DOXORUBICIN)

Administration

IV

Side Effects and Toxicity

N/V (moderate)

Stomatitis

BMD (7 to 14 days later)

Fever, chills

Local phlebitis

Alopecia

Cumulative-dose toxicity includes:

Cardiac abnormalities

Electrocardiographic changes

Heart failure

Comments and Specific Nursing Considerations

Vesicant‡ (extravasation may not cause pain)

Use only sterile distilled water as a diluent

Observe for any changes in heart rate or rhythm and signs of failure

Cumulative dose must not exceed 375 mg/m2 (less with radiation)

Warn parents that drug causes urine to turn red (for up to 12 days after administration); this is normal, not hematuria

L-ASPARAGINASE (ELSPAR)

Administration

SQ, IV

Side Effects and Toxicity

Allergic reactions (including anaphylactic shock)

Fever

N/V (mild)

Anorexia

Weight loss

Arthralgia

Toxicity:

Liver dysfunction

Hyperglycemia

Renal failure

Pancreatitis

Coagulation abnormalities

Comments and Specific Nursing Considerations

Have emergency drugs at bedside

Record signs of allergic reaction, such as urticaria, facial edema, hypotension, or abdominal cramps

Check weight daily

Normally, blood urea nitrogen (BUN) and ammonia levels rise as a result of drug; not evidence of liver damage

Check urine for sugar and blood amylase

Observe for thrombotic events

MECHLORETHAMINE (NITROGEN MUSTARD, MUSTARGEN)

Administration

IV

Side Effects and Toxicity

N/V (30 minutes to 8 hours later) (severe)

BMD (2 to 3 weeks later)

Alopecia

Local phlebitis

Comments and Specific Nursing Considerations

Vesicant

MERCAPTOPURINE (6-MP, PURINETHOL)

Administration

PO, IV

Side Effects and Toxicity

N/V (mild)

Diarrhea

Anorexia

Stomatitis

BMD (4 to 6 weeks later)

Immunosuppression

Dermatitis

Less often may be hepatic dysfunction

Comments and Specific Nursing Considerations

6-MP is an analog of xanthine; therefore, allopurinol (Zyloprim) delays its metabolism and increases its potency, necessitating a lower dose (one-third to one-quarter) of 6-MP

METHOTREXATE (MTX, AMETHOPTERIN)

Administration

PO, IV, IM, IT

May be given in conventional doses (mg/m2) or high doses (g/m2)

Side Effects and Toxicity

N/V (severe at high doses)

Diarrhea

Mucosal ulceration (2 to 5 days later)

BMD (10 days later)

Immunosuppression

Dermatitis

Photosensitivity

Alopecia (uncommon)

Toxic effects include:

Hepatitis (fibrosis)

Osteoporosis

Nephropathy

Pneumonitis (fibrosis)

Neurologic toxicity with IT use—pain at injection site, meningismus (signs of meningitis without actual inflammation), especially fever and headache; potential sequelae—transient or permanent hemiparesis, seizures, dementia, death

Comments and Specific Nursing Considerations

Side effects and toxicity are dose-related

Potency and toxicity are increased by reduced renal function, salicylates, sulfonamides, and aminobenzoic acid; avoid use of these substances, such as aspirin

Avoid exposure to sun and use sun block

High-dose therapy:

Citrovorum factor (folinic acid or leucovorin) decreases cytotoxic action of MTX; used as an antidote for overdose and to enhance normal cell recovery after high-dose therapy; avoid use of vitamins containing folic acid during MTX therapy unless prescribed by physician

IT therapy:

Drug must be mixed with preservative-free diluent

Report signs of neurotoxicity immediately

PROCARBAZINE (MATULANE)

Administration

PO

Side Effects and Toxicity

N/V (moderate)

BMD (3 to 4 weeks later)

Lethargy

Dermatitis

Myalgia

Arthralgia

Less often:

Stomatitis

Neuropathy

Alopecia

Diarrhea

Amenorrhea

Comments and Specific Nursing Considerations

Central nervous system (CNS) depressants (phenothiazines, barbiturates) enhance CNS symptoms

Monoamine oxidase (MAO) inhibition sometimes occurs; therefore all other drugs are avoided unless medically approved; red wine, fava beans, and broad bean pods are avoided

VINCRISTINE (ONCOVIN) AND VINBLASTINE (VELBAN)

Administration

IV

Side Effects and Toxicity

Neurotoxicity (less severe with vinblastine)—paresthesia (numbness); ataxia; weakness; footdrop; hyporeflexia;

constipation (dynamic ileus); hoarseness (vocal cord paralysis); abdominal, chest, and jaw pain; mental depression

Fever

N/V (mild)

BMD (minimal; 7 to 14 days later)

Alopecia

Syndrome of inappropriate antidiuretic hormone excretion (SIADH)

Comments and Specific Nursing Considerations

Vesicant

Report signs of neurotoxicity because may necessitate cessation of drug

Individuals with underlying neurologic problems may be more prone to neurotoxicity

Monitor stool patterns closely; administer stool softener

Excreted primarily by liver into biliary system; administer cautiously to anyone with biliary disease

Maximum vincristine dose is 2 mg

IV, Intravenous; IT, intrathecal; PO, by mouth; IM, intramuscular; SC, subcutaneous; BMD, bone marrow depression.

Nausea and vomiting.The nausea and vomiting that occur shortly after administration of several of the drugs and from cranial or abdominal radiation can be profound. The serotonin-receptor antagonists (e.g., ondansetron [Zofran]) are effective in the control of nausea and vomiting occurring after emetogenic chemotherapy and radiation therapy. When combined with dexamethasone, these agents are the treatment of choice in

the prevention of cisplatin-induced delayed emesis (Bryant, 2003).

The most beneficial regimen for antiemetic control has been the administration of the antiemetic before the chemotherapy begins. The goal is to prevent the child from ever experiencing nausea or vomiting, thus preventing development of anticipatory symptoms (the conditioned response of developing nausea and vomiting before receiving the drug).

Anorexia.Loss of appetite is a direct consequence of the chemotherapy or irradiation. It is a major problem for parents because it is the one area they feel responsible for, particularly when so many other facets of care are outside their control. There are no universally successful techniques for encouraging a sick child to eat. However, the guidelines in Chapter 22 can be helpful during the anorexic period and can prevent additional problems during the remission.

Some children still do not eat despite these approaches. When loss of appetite and weight persist, the nurse should investigate the family situation to determine if any factors (e.g., conditioned aversion to food, environmental stress related to eating, controlling behavior, anger) might be contributing to the problem. Nasogastric tube feedings or total parenteral nutrition may be implemented for children with significant nutritional problems.

Mucosal ulceration.One of the most distressing side effects of several drugs is GI mucosal cell damage, which can produce ulcers anywhere along the alimentary tract. Oral ulcers greatly compound anorexia because eating is extremely uncomfortable, but the following interventions may be helpful: (1) provide a bland, moist, soft diet appropriate for the child's age and preferences; (2) use a soft sponge toothbrush (Toothettes)* or cotton-tipped applicator; (3) provide frequent mouthwashes with normal saline (using a solution of 1 teaspoon of table salt and 1 pint of water) or sodium

bicarbonate mouth rinses (using a solution of 1 teaspoon of baking soda in 1 quart of water); and(4) use local anesthetics (e.g., Chloraseptic lozenges) or nonprescription preparations without alcohol (e.g., Orabase, Ulcerease, Benadryl/Maalox solution). Although local anesthetics are effective in temporarily relieving the pain, many children dislike the taste and numb feeling they produce.

! NURSING ALERTViscous lidocaine is not recommended for young children; if applied to the pharynx, it may depress the gag reflex, increasing the risk of aspiration. Seizures have been rarely associated with the use of oral viscous lidocaine (Cho, Cheng, and Cheng, 2000).

Other preparations that may be used to prevent or treat mucositis include chlorhexidine gluconate (Peridex) because of its dual effectiveness against candidal and bacterial infections, antifungal troches (lozenges) or mouthwash, and lip balm (e.g., Aquaphor) to keep the lips moist. Agents that should not be used include lemon glycerin swabs (irritate eroded tissue and can decay teeth), hydrogen peroxide (delays healing by breaking down protein), and milk of magnesia (dries mucosa).

Stomatitis may cause such difficulty with eating that the child may require hospitalization for hydration, parenteral nutrition, and pain control (often with IV morphine). The child will usually choose the foods that are best tolerated, and the nurse should encourage parents to relax any eating pressures. Because the stomatitis is a temporary condition, the child can resume good food habits after the ulcers heal. Dental hygiene can become a serious problem for children with orthodontic appliances. Sometimes it may be necessary to remove the braces to allow chemotherapy to continue.

Rectal ulcers are managed by meticulous toilet hygiene, warm sitz baths after each bowel movement, and use of an occlusive ointment or dressing applied to the ulcerated area to promote epithelialization. Stool softeners are necessary to prevent further discomfort. Parents are advised to record

bowel movements, because the child may voluntarily avoid defecation to prevent discomfort. Rectal thermometers and suppositories are contraindicated because insertion may further traumatize the area.

* Manufactured by Halbrand, Inc., Willoughby, Ohio.

Neuropathy.Vincristine and, to a lesser extent, vinblastine can cause various neurotoxic effects. Nursing interventions for management of these effects include (1) administering stool softeners or laxatives for severe constipation caused by decreased bowel innervation; (2) maintaining good body alignment and, if on bed rest, using a footboard or high-top shoes to minimize or prevent footdrop; (3) carrying out safety measures during ambulation because of weakness and numbing of the extremities, which may cause difficulty in walking or fine hand movement; and (4) providing a soft or liquid diet for severe jaw pain.

Hemorrhagic cystitis.Sterile hemorrhagic cystitis, a side effect of chemical irritation to the bladder from cyclophosphamide, can be decreased and often prevented by (1) a liberal fluid intake (at least one and a half times the recommended daily fluid requirement); (2) frequent voiding immediately after feeling the urge, before bed, and after arising; (3) administering the drug early in the day to allow for sufficient oral intake and voiding; and (4) administering mesna (an agent that provides protection to the bladder) as ordered. If oral home administration is prescribed, the family needs specific instructions regarding exactly how much fluid the child must have.

! NURSING ALERTIf signs of cystitis occur, such as burning or bleeding on urination, prompt medical evaluation is needed.

Alopecia.

Hair loss is a common side effect of several chemotherapeutic drugs and cranial irradiation, although not all children lose their hair during drug therapy. It is better to warn children and parents of this side effect than to allow them to think that it is only a remote possibility. A soft cotton cap is the most comfortable head wear for children. Polyester increases perspiration and causes itching. Other options include scarves, hats, or a wig.

NURSING TIPIf the child chooses to wear a wig, encouraging a child to select one similar to the child's own hairstyle and color before the hair falls out is helpful in fostering later adjustment to hair loss.

The nurse should also inform the family that hair regrows in 3 to 6 months and may be of a different color and texture. Frequently the hair is darker, thicker, and curlier than before. If the child chooses not to wear a wig, attention to some type of head covering, especially in cold climates and during exposure to sun, and scalp hygiene are important. The scalp should be washed like any other body part.

Moon face.Short-term steroid therapy produces no acute toxicities and produces two beneficial reactions: increased appetite and a sense of well-being. However, it does produce alterations in body image, which, although not clinically significant, can be extremely distressing to older children. One of these is moon face, in which the child's face becomes rounded and puffy. It is not unusual for other children to make fun of the child with such remarks as "Miss Piggy," "Porky Pig," or "fat face." It is helpful to reassure children who experience such name-calling that after cessation of the drug the facial changes will return to normal. Unlike hair loss, little can be done to camouflage this obvious change. If the child resumes activity early in the course of treatment, the change may be less noticeable to peers than after a long absence.

Mood changes.

Shortly after beginning steroid therapy, children experience a number of mood changes that range from feelings of well-being and euphoria to depression and irritability. If parents are unaware of these drug-induced changes, they may become unduly concerned. Therefore the nurse should warn them of the reactions and encourage them to discuss the behavioral changes with each other and the child.

Provide continued physical care and emotional support. Because of the improved survival of these children, continued monitoring of physical and intellectual growth and development is essential. Nurses should stress the importance of regular follow-up care.

An important aspect of continued emotional support involves the prognosis. Although leukemia is no longer invariably fatal, it must be remembered that survival statistics are only average estimates and apply to those children treated with the latest protocols since diagnosis. For the low-risk child the chances may be better, but for the high-risk child they may be significantly poorer. Of those who do survive after discontinuing therapy, some will relapse. Therefore, at present, only the passage of time is positive confirmation of the child's being ultimately "cured" of the disease. Remission, even in excess of 5 years, cannot be equated with a cure. With increasing concern regarding late effects of treatment, continued surveillance of the child's health status is needed. The nurse who is working with family members must individualize information regarding the "numbers" and the potential risks. An understanding of each member's emotional needs, as well as competent care of physical ones, is essential to the positive, growth-promoting support of the family. Comprehensive emotional support for the family of the child with a potentially fatal illness is discussed in Chapter 18.* Includes principal drugs used in the treatment of childhood leukemias and lymphomas. Several other conventional and investigational chemotherapeutic agents may be employed in the treatment regimen.† N/V, Nausea and vomiting. Mild, 20% incidence; moderate, 20% to 70% incidence; severe, 0.75% incidence.‡ Vesicants (sclerosing agents) can cause severe cellular damage if even minute amounts of the drug infiltrate surrounding tissue.

EvaluationThe effectiveness of nursing interventions is determined by continual reassessment and evaluation of care based on the following observational guidelines:

1. Compare number of visits for primary health with recommended schedule of health supervision.

2. Monitor growth, development, and other aspects of regular health assessment; check mouth for adequacy of dental hygiene; review immunization record for age-appropriate vaccines and use of non-live virus preparations.

3. Interview child and family regarding their understanding of treatments and diagnostic tests.

4. Employ pain assessment techniques for procedural pain.

5. Make careful observations of physical status.

a. Take vital signs regularly.

b. Observe for evidence of bleeding, infection, neuropathy, cystitis, and mucosal ulceration.

c. Observe and record intake and output.

6. Interview child and family and observe behaviors as a result of complications of therapies.

7. Interview child and family and observe behaviors that provide clues to their response to the disease, its therapy, and nursing interventions.

The expected outcomes are described in the Nursing Care Plan on pp. 966 to 970.

NURSING CARE PLAN: The Child With CancerNURSING DIAGNOSIS: Risk for injury related to malignant process, treatment

PATIENT GOAL 1: Will experience partial or complete remission from disease

NURSING INTERVENTIONS/RATIONALES

*Administer chemotherapeutic agents as prescribed

Assist with radiotherapy as ordered

Assist with procedures for administration of chemotherapeutic agents (e.g., lumbar puncture for intrathecal administration)

†Prepare child and family for surgical procedure if appropriate

EXPECTED OUTCOME

Child achieves a partial or complete remission from disease

PATIENT GOAL 2: Will not experience complications of chemotherapy

NURSING INTERVENTIONS/RATIONALES

Follow guidelines for administration of chemotherapeutic agents

Observe for signs of infiltration at intravenous (IV) site: pain, stinging, swelling, redness

Immediately stop infusion if any sign of infiltration occurs to prevent severe tissue damage

Implement policies of institution to treat infiltration

Obtain careful history for known allergies to prevent anaphylaxis

Observe child for 20 minutes after infusion for signs of anaphylaxis (cyanosis, hypotension, wheezing, severe urticaria)

Stop infusion of drug and flush IV line with normal saline if reaction is suspected

Have emergency equipment (especially blood pressure monitor and manual resuscitation bag and mask) and emergency drugs (especially oxygen, epinephrine, antihistamine, aminophylline, corticosteroids, and vasopressors) readily available to prevent delay in treatment

EXPECTED OUTCOMES

Child will not experience complications of chemotherapy

Child will receive prompt, appropriate treatment of complications

NURSING DIAGNOSIS: Risk for infection related to depressed body defenses

PATIENT GOAL 1: Will experience minimized risk of infection

NURSING INTERVENTIONS/RATIONALES

Place child in private room to minimize exposure to infective organisms

Advise all visitors and staff to use good handwashing technique to minimize exposure to infective organisms

Screen all visitors and staff for signs of infection to minimize exposure to infective organisms

Use scrupulous aseptic technique for all invasive procedures

Monitor temperature to detect possible infection

Evaluate child for any potential sites of infection (e.g., needle punctures, mucosal ulceration, minor abrasions, dental problems)

Provide nutritionally complete diet for age to support body's natural defenses

Avoid giving live attenuated virus vaccines (e.g., measles, mumps, rubella, oral poliovirus) to child with depressed immune system because these vaccines can result in overwhelming infection

*Give inactivated virus vaccines (e.g., varicella [chickenpox], Salk polio, influenza) as prescribed and indicated to prevent specific infections

Administer antibiotics as prescribed

*Administer granulocyte colony-stimulating factor (GCSF) as prescribed

EXPECTED OUTCOMES

Child does not come in contact with infected persons or contaminated articles

Child consumes diet appropriate for age (specify)

Child does not exhibit signs of infection

NURSING DIAGNOSIS: Risk for injury (hemorrhage, hemorrhagic cystitis) related to interference with cell proliferation

PATIENT GOAL 1: Will exhibit no evidence of bleeding

NURSING INTERVENTIONS/RATIONALES

Use all measures to prevent infection, especially in ecchymotic areas, because infection increases tendency toward bleeding

Use local measures (e.g., apply pressure, ice) to stop bleeding

Restrict strenuous activity that could result in accidental injury

Involve child in responsibility for limiting activity when platelet count drops to encourage compliance

Avoid skin punctures when possible to prevent bleeding

Observe for bleeding after procedures such as venipuncture, bone marrow aspiration

Turn frequently and use pressure-reducing or pressure-relieving mattress to prevent decubitus ulcers

Teach parents and older child measures to control nosebleeding

Prevent oral and rectal ulceration because ulcerated skin is prone to bleeding

Avoid aspirin-containing medications because aspirin interferes with platelet function

*Administer platelets as prescribed to raise platelet count

EXPECTED OUTCOME

Child exhibits no evidence of bleeding

PATIENT GOAL 2: Will exhibit no evidence of hemorrhagic cystitis

NURSING INTERVENTIONS/RATIONALES

Observe for signs of cystitis (e.g., burning and pain on urination)

Report signs of cystitis to practitioner, because prompt medical evaluation is needed

Give liberal (3000 mL/m2/day) fluid intake (meters squared is calculated from West nomogram; see Administration of Medication, Chapter 22)

Encourage frequent voiding, including during nighttime, to minimize metabolites' contact with bladder mucosa

Administer drugs irritating to bladder early in the day to allow for sufficient fluid intake and voiding

EXPECTED OUTCOMES

Child voids without discomfort

No hematuria is present

PATIENT GOAL 3: Will experience minimal effects of anemia

NURSING INTERVENTIONS/RATIONALES AND EXPECTED OUTCOMES

See Nursing Care Plan: The Child With Anemia

NURSING DIAGNOSIS: Risk for fluid volume deficit related to nausea and vomiting

PATIENT GOAL 1: Will experience no nausea or vomiting

NURSING INTERVENTIONS/RATIONALES

*Administer initial dose of antiemetic before chemotherapy begins to prevent child from ever experiencing nausea and vomiting, thus preventing an anticipatory response

*Administer antiemetic around the clock for as long as nausea and vomiting typically last to prevent any episodes from occurring

Assess child's response to antiemetic, because no antiemetic drug is uniformly successful

Avoid foods with strong odors that may induce nausea and vomiting

Uncover hospital food tray outside of child's room to reduce food odors that may induce nausea

Encourage frequent intake of fluids in small amounts, because small portions are usually better tolerated

*Administer IV fluid, as prescribed, to maintain hydration

EXPECTED OUTCOMES

Child retains food and fluid

Child does not experience nausea or vomiting

NURSING DIAGNOSIS: Altered mucous membranes related to administration of chemotherapeutic agents

PATIENT GOAL 1: Will not develop oral mucositis

NURSING INTERVENTIONS/RATIONALES

Inspect mouth daily for oral ulcers; report evidence of ulcers to practitioner for early treatment

Avoid oral temperatures to prevent trauma

Institute meticulous oral hygiene as soon as a drug is used that causes oral ulcers

Use soft sponge toothbrush, cotton-tipped applicator, or gauze-wrapped finger to avoid trauma

Administer frequent (at least every 4 hours and after meals) mouthwashes (normal saline with or without sodium bicarbonate solution) to promote healing

Apply local anesthetics to ulcerated areas before meals and as needed to relieve pain

Avoid using viscous lidocaine for young children, because if applied to pharynx, it may depress gag reflex, increasing risk of aspiration, and may cause seizures

Apply lip balm to keep lips moist and prevent cracking or fissuring

Serve bland, moist, soft diet; offer food best tolerated by child

Encourage fluids; use a straw to help bypass painful areas

Encourage parents to relax any eating pressures, because stomatitis is a temporary condition

Avoid juices containing ascorbic acid and hot or cold or spicy foods if they cause further discomfort

Avoid using lemon glycerin swabs (irritate eroded tissue and can decay teeth), hydrogen peroxide (delays healing by breaking down protein), and milk of magnesia (dries mucosa)

Explain to parents that child may require hospitalization for hydration, parenteral nutrition, and pain control (often with IV morphine) if stomatitis interferes with food and fluid intake

*Administer antiinfective medication as ordered to prevent or treat mucositis

*Administer analgesics, including opioids, to control pain

EXPECTED OUTCOMES

Mucous membranes remain intact

Ulcers show evidence of healing

Child reports or exhibits no evidence of discomfort

PATIENT GOAL 2: Will not develop rectal ulceration

NURSING INTERVENTIONS/RATIONALES

Wash perianal area after each bowel movement to lessen irritation

Use warm sitz baths or tub baths to promote healing

Expose reddened but not ulcerated areas to air to keep skin dry

Apply protective skin barriers (transparent film dressings, occlusive ointment) to perineal area to protect skin from direct contact with urine or feces and to promote healing

Observe for constipation resulting from child's voluntary refusal to defecate or from chemotherapy

Record bowel movements; use stool softener to prevent constipation; may need stimulants for evacuation

Avoid rectal temperatures and suppositories to prevent rectal trauma

EXPECTED OUTCOMES

Rectal mucosa remains clean and intact

Ulcerated areas heal without complications

Child has regular bowel movements

NURSING DIAGNOSIS: Altered nutrition: less than body requirements related to loss of appetite

PATIENT GOAL 1: Will receive adequate nutrition

NURSING INTERVENTIONS/RATIONALES

Encourage parents to relax pressures placed on eating; explain that loss of appetite is a direct consequence of nausea and vomiting and chemotherapy

Allow child any food tolerated; plan to improve quality of food selections when appetite increases

Explain expected increase in appetite from steroids to prepare child and parents for this change

Take advantage of any hungry period: serve small "snacks," because small portions are usually better tolerated

Fortify foods with nutritious supplements, such as powdered milk or commercial supplements, to maximize quality of intake

Allow child to be involved in food preparation and selection to encourage eating

Make food appealing

Remember usual food practices of children in each age-group, such as food jags in toddlers or normal occurrence of physiologic anorexia, to distinguish these expected changes from actual refusal to eat

Assess family for additional problems (e.g., use of food by child as a control mechanism if appetite does not improve despite improved physical status) to identify areas that require intervention

EXPECTED OUTCOME

Nutritional intake is adequate

NURSING DIAGNOSIS: Impaired skin integrity related to administration of chemotherapeutic agents, radiotherapy, immobility

PATIENT GOAL 1: Will maintain skin integrity

NURSING INTERVENTIONS/RATIONALES

Provide meticulous skin care, especially in mouth and perianal regions, because they are prone to ulceration

Change position frequently to stimulate circulation and relieve pressure

Encourage adequate caloric-protein intake to prevent negative nitrogen balance

EXPECTED OUTCOME

Skin remains clean and intact

PATIENT GOAL 2: Will experience minimal negative effects of therapy

NURSING INTERVENTIONS/RATIONALES

Select loose-fitting clothing over irradiated area to minimize additional irritation

Protect area from sunlight and sudden changes in temperature (avoid ice packs, heating pads) during radiotherapy or administration of methotrexate

EXPECTED OUTCOME

Child and family comply with suggestions (specify)

NURSING DIAGNOSIS: Impaired physical mobility related to neuromuscular impairment (neuropathy)

PATIENT GOAL 1: Will experience minimal negative effects of peripheral neuropathy

NURSING INTERVENTIONS/RATIONALES

Encourage ambulation when child is able

Alter activity, including school attendance, to prevent injuries if weakness occurs

Use footboard or high-top shoes to prevent footdrop

Provide fluids and soft foods to lessen chewing movements with jaw pain

EXPECTED OUTCOME

Child ambulates without incident or difficulty

NURSING DIAGNOSIS: Body-image disturbance related to loss of hair, moon face, debilitation

PATIENT/FAMILY GOAL 1: Will exhibit positive coping behaviors

NURSING INTERVENTIONS/RATIONALES

Introduce idea of wig before hair loss

Encourage child to select a wig similar to child's own hairstyle and color before hair falls out to foster later adjustment to hair loss

Provide adequate covering during exposure to sunlight, wind, or cold, because natural protection is lost

Suggest keeping thin hair clean, short, and fluffy to camouflage partial baldness

Explain that hair begins to regrow in 3 to 6 months and may be a slightly different color or texture to prepare child and family for changes in appearance of new hair

Explain that alopecia during a second treatment with same drug may be less severe

Encourage good hygiene, grooming, and sex-appropriate items (e.g., wig, scarves, hats, makeup, attractive sex-appropriate clothing) to enhance appearance

EXPECTED OUTCOMES

Child verbalizes concern regarding hair loss

Child helps determine methods to reduce effects of hair loss and applies these methods

Child appears clean, well groomed, and attractively dressed

PATIENT GOAL 2: Will exhibit adjustment to altered facial appearance

NURSING INTERVENTIONS/RATIONALES

Encourage rapid reintegration with peers to lessen contrast of changed facial appearance

Stress that this reaction is temporary to provide reassurance that usual appearance will return

Evaluate weight gain carefully (with weight gain resulting from administration of steroids, extremities remain thin)

Encourage visits from friends before discharge to prepare child for reactions and questions

EXPECTED OUTCOMES

Family demonstrates understanding of consequences of therapies

Child resumes former activities and relationships within capabilities

PATIENT GOAL 3: Will express feelings

NURSING INTERVENTIONS/RATIONALES

Provide opportunities for child to discuss feelings and concerns

Provide materials for nonverbal expression (e.g., play, art)

EXPECTED OUTCOME

Child expresses feelings regarding altered body in words, play, art (specify)

NURSING DIAGNOSIS: Pain related to diagnosis, treatment, physiologic effects of neoplasia

PATIENT GOAL 1: Will experience no pain or reduction of pain to level acceptable to child

NURSING INTERVENTIONS/RATIONALES

Whenever possible, make use of procedures (e.g., noninvasive temperature monitoring, venous access device) to minimize discomfort

Assess need for pain management (see Chapter 21)

Evaluate effectiveness of pain relief with degree of alertness vs sedation to determine need for change in dosage, time of administration, or drug

Implement appropriate nonpharmacologic pain reduction techniques as adjunct to analgesics

*Administer analgesics as prescribed

Avoid aspirin or any of its compounds (e.g., other nonsteroidal antiinflammatory agents) because aspirin increases bleeding tendency

*Administer drugs on preventive schedule (around the clock) to prevent pain from recurring

Monitor effectiveness of therapy on pain assessment record

EXPECTED OUTCOME

Child rests quietly, reports or exhibits no evidence of discomfort, and verbalizes no complaints of discomfort

NURSING DIAGNOSIS: Fear related to diagnostic test, procedures, treatments

PATIENT GOAL 1: Will exhibit reduced fear related to diagnostic procedures and tests

NURSING INTERVENTIONS/RATIONALES

Explain procedure carefully at child's level of understanding to reduce fear of the unknown

Explain what will take place and what child will feel, see, and hear to increase sense of control

Use recall of each step as method of distraction

Explain special requests of child (e.g., need to remain motionless during test or radiotherapy) to encourage cooperation

Provide child with some means for involvement with procedure (e.g., holding a piece of equipment, such as bandage or tape, counting with the operator, answering questions) to promote sense of control, encourage cooperation, and support child's coping skills

Implement distracting techniques and pain reduction techniques as indicated

See also Preparation for Procedures, Chapter 22

EXPECTED OUTCOMES

Child readily responds to verbal directives

Child repeats information accurately

NURSING DIAGNOSIS: Fear related to diagnosis, prognosis

See Nursing Care Plan: The Child Who Is Terminally Ill or Dying, Chapter 18

NURSING DIAGNOSIS: Diversional activity deficit related to restricted environment (private room)

PATIENT GOAL 1: Will have opportunity to participate in diversional activities

NURSING INTERVENTIONS/RATIONALES

Provide age-appropriate toys that can be properly cleaned to provide diversion without risk of infection

Involve child-life specialist or other supportive services in planning diversional activities

EXPECTED OUTCOMES

Child engages in activities appropriate for age and interests

Suitable toys are provided

NURSING DIAGNOSIS: Altered family processes related to having a child with a life-threatening disease

PATIENT/FAMILY GOAL 1: Will demonstrate knowledge about diagnostic/therapeutic procedures

NURSING INTERVENTIONS/RATIONALES

Explain reason for each test and procedure

Explain reason for radiotherapy, chemotherapy

Explain operative procedure honestly (if appropriate)

Avoid overemphasis on benefits, which may not be immediately evident (applies primarily to brain tumors), to avoid unrealistic expectations

See also Preparation for Procedures, Chapter 22

EXPECTED OUTCOME

Child and family demonstrate understanding of procedures (specify learning and manner of demonstration)

PATIENT (FAMILY) GOAL 1: Will receive adequate support

NURSING INTERVENTIONS/RATIONALES

Teach parents about disease process

Explain all procedures that will be done to child

Schedule time for family to be together, without interruptions from staff, to encourage communication and expression of feelings

Help family plan for future, especially for helping child live a normal life, to promote child's optimum development

Encourage family to discuss feelings regarding child's course before diagnosis and child's prognosis

Discuss with family how they will tell child about outcome of treatment and need for additional treatment (if appropriate) to maintain open and honest communication

Refer to local chapter of American Cancer Society or other organizations

EXPECTED OUTCOMES

Family demonstrates knowledge of child's disease and treatments (specify methods of learning and evaluation)

Family expresses feelings and concerns and spends time with child

See also:

Nursing Care Plan: The Child in the Hospital, Chapter 21

Nursing Care Plan: The Family of the Ill or Hospitalized Child, Chapter 21

NURSING DIAGNOSIS: Altered family processes related to a child undergoing therapy

PATIENT (FAMILY) GOAL 1: Will demonstrate understanding of side effects or complications of treatment

NURSING INTERVENTIONS/RATIONALES

Advise family of expected side effects versus toxicities; clarify which demand medical evaluation (mucosal ulceration, hemorrhagic cystitis, peripheral neuropathy, evidence of infection or dehydration) to prevent delay in treatment

Reassure family that such reactions are not caused by return of cancer cells to minimize undue concern

Interpret prognostic statistics carefully, realizing family's temporary need to interpret them as they see necessary, to present a realistic, but hopeful, future

Prepare family for expected mood changes from steroids

Interpret mood changes based on drugs or reactions to disease/treatment to prevent any unwarranted negative reaction to child (e.g., punishment)

EXPECTED OUTCOMES

Family demonstrates knowledge of instructions (specify methods of learning and evaluation)

Family demonstrates understanding of behavior changes

PATIENT GOAL 2: Will receive adequate support during treatment

NURSING INTERVENTIONS/RATIONALES

Explain reason for antibiotics or transfusions, particularly why platelets are reserved for acute, uncontrolled bleeding episodes

Observe for signs of transfusion reaction (see Table 26-6)

Record appropriate time for hemostasis to occur after administration of platelets to determine if transfusions are becoming less effective

EXPECTED OUTCOME

Child demonstrates understanding of procedures and tests (specify methods of learning and evaluation)

PATIENT (FAMILY) GOAL 3: Will be prepared for home care

NURSING INTERVENTIONS/RATIONALES

Teach preventive measures at discharge (e.g., handwashing, isolation from crowds) to prevent infection

Stress importance of isolating child from any known cases of chickenpox or other childhood diseases; work with school nurse and physician to determine optimum time for school reattendance to prevent unnecessary absences or risk of infection

Teach home care instructions specific to child's needs

EXPECTED OUTCOME

Family demonstrates ability to provide home care for child (specify)

NURSING DIAGNOSIS: Anticipatory grief related to perceived potential loss of a child

PATIENT (FAMILY) GOAL 1: Will acknowledge and cope with possibility of child's death

NURSING INTERVENTIONS/RATIONALES

Provide consistent contact with family to establish a trusting relationship that encourages communication

Clarify, refocus, and supply information as needed

Help family plan care of child, especially at terminal stage (e.g., extent of extraordinary lifesaving measures) to ensure that their wishes are implemented

Provide or arrange for hospice care if family desires it

Arrange for spiritual support in accordance with family's beliefs or affiliations

EXPECTED OUTCOMES

Family remains open to counseling and nursing contact

Family and child discuss their fears, concerns, needs, and desires at terminal stage

Family investigates hospice care

Appropriate religious representative is contacted (specify)

PATIENT (FAMILY) GOAL 2: Will receive adequate support

NURSING INTERVENTIONS/RATIONALES AND EXPECTED OUTCOMES

See Nursing Care Plan: The Child Who Is Terminally Ill or Dying (Chapter 18)

* Dependent nursing action.† Indicates content that is specific to a particular malignancy.

LYMPHOMAS

Pediatric lymphomas are the third most common group of malignancies in children and adolescents. The lymphomas, a group of neoplastic diseases that arise from the lymphoid and hemopoietic systems, are divided into Hodgkin disease and non-Hodgkin lymphoma (NHL). These diseases are further subdivided according to tissue type and extent of disease. NHL is more prevalent in children younger than 14 years of age, whereas Hodgkin disease is prevalent in adolescence and the young adult period, with a striking increase between ages 15 and 19 years.

HODGKIN DISEASEHodgkin disease is a neoplastic disease that originates in the lymphoid system and primarily involves the lymph nodes. It predictably metastasizes to nonnodal or extralymphatic sites, especially the spleen, liver, bone marrow, and lungs, although no tissue is exempt from involvement (Fig. 26-5). It is classified according to four histologic types: (1) lymphocytic predominance, (2) nodular sclerosis, (3) mixed cellularity, and (4) lymphocytic depletion. Accurate staging of the extent of disease is the basis for treatment protocols and expected prognoses.FIG. 26-5 Main areas of lymphadenopathy and organ involvement in Hodgkin disease.

The Ann Arbor staging system (Box 26-10) assigns stage based on the number of sites of lymph node involvement, presence of extranodal disease, and history of any symptoms. Patients are classified as A if asymptomatic and as B if they have the following symptoms: temperature of 38° C (100.4° F) or higher for 3 consecutive days, drenching night sweats, or unexplained loss of body weight (10% or more) over the preceding 6 months (Hudson and Donaldson, 2002).

BOX 26-10 Stages of Hodgkin DiseaseStage I: Lesions are limited to one lymph node area or only one additional extralymphatic site (IE), such as the liver, lungs, kidney, or intestines.

Stage II: Two or more lymph node regions on the same side of the diaphragm or one additional extralymphatic site or organ (IIE) on the same side of the diaphragm is involved.

Stage III:Lymph node regions on both sides of the diaphragm are involved, or one extralymphatic site (IIIE), spleen (IIIS), or both (IIISE).

Stage IV:Cancer has metastasized diffusely throughout the body to one or more extralymphatic sites with or without involvement of associated lymph nodes.

Asymptomatic enlarged cervical or supraclavicular lymphadenopathy is the most common presentation of Hodgkin disease (Box 26-11). Other systemic symptoms, including fever, weight loss, and night sweats, as well as cough, abdominal discomfort, anorexia, nausea, and pruritus, may be manifested. Because multiple organs may be involved, diagnosis is based on several tests and the extent of metastatic disease. Tests include a CBC, erythrocyte sedimentation rate, serum copper, ferritin level, fibrinogen, immunoglobulins, uric acid level, liver function tests, T-cell function studies, and urinalysis. Radiographic tests include computed tomography (CT) scans of the neck, chest, abdomen, and pelvis; a gallium scan (identifies metastatic/recurrent disease); a chest x-ray film; and, if clinically indicated, a bone scan to identify metastatic disease. With the advent of CT and gallium scans, a lymphangiogram may not be needed, although elimination is controversial.

BOX 26-11 Clinical Manifestations of Hodgkin Disease

Painless enlargement of the lymph nodes:

Enlarged, firm, nontender, movable nodes in the cervical area are most common

"Sentinel" node located near the left clavicle may be first enlarged node

Axillary and inguinal lymph nodes less frequently

Other signs and symptoms of lymphadenopathy:

Enlarged mediastinal nodes cause persistent nonproductive cough

Enlarged retroperitoneal nodes produce unexplained abdominal pain

Systemic symptoms (usually indicate advanced involvement):

Low-grade or intermittent fever

Anorexia

Nausea

Weight loss

Night sweats

Pruritus

Although used rarely, lymphangiography may be performed. This is visualization of the lymphatic circulation of the lower extremities, groin, ileopelvic and abdominal-aortic regions, and thoracic duct by way of a radiopaque medium injected in the feet or hands.

A lymph node biopsy is essential to establish histiologic diagnosis and staging. The presence of Reed-Sternberg cells is characteristic of Hodgkin disease. These large cells, which are multilobed and nucleated with abundant cytoplasm and a typically halolike clear zone around the nucleolus, are often described as having an "owl's eyes" appearance (Hudson and Donaldson, 2002). A bone marrow aspiration or biopsy is usually performed. With the advent of CT and gallium scans to identify metastatic disease and multiagent chemotherapy to eradicate metastatic disease, a laparotomy without splenectomy is avoided except in a few selected cases.

Therapeutic ManagementThe primary modalities of therapy are radiation and chemotherapy. Each may be used alone or in combination based on the clinical staging. Radiation may involve only the involved field (IF), an extended field (EF) (involved areas plus adjacent

nodes), or total nodal irradiation (TNI), depending on the extent of involvement.

An effective combination of chemotherapy widely used is MOPP (mechlorethamine, vincristine [Oncovin], procarbazine, prednisone) or ABVD (Adriamycin, bleomycin, vinblastine, dacarbazine). However, this therapy combination caused severe late effects, especially secondary malignancies. At present, use of ABVD with COPP (cyclophosphamide, vincristine, prednisone, procarbazine) as a substitute for MOPP has minimized late effects.

Follow-up care of children no longer receiving therapy is essential to identify relapse and secondary cancers. In children with splenectomy resulting from laparotomy or splenic irradiation, prophylactic antibiotics are administered for an indefinite period. Also, immunizations against pneumococci and meningococci are recommended before the splenectomy.

Prognosis.Long-term survival for all stages of Hodgkin disease is excellent. Early-stage disease can have survival rates greater than 90%, with advanced stages having rates between 65% and 75%.

Nursing ConsiderationsNursing care involves the same objectives as for patients with other types of cancer—specifically, (1) preparation for diagnostic and operative procedures, (2) explanation of treatment side effects (see Box 26-9), and (3) child and family support (see Chapter 18). Because this is most often a disease of adolescents and young adults, the nurse must have an appreciation of their psychologic needs and reactions during the diagnostic and treatment phases (see Nursing Care Plan, pp. 966 to 970).

After the child is hospitalized for suspected Hodgkin disease, a battery of diagnostic tests is ordered. The family needs an explanation of why each test is performed, because many of them, such as bone marrow aspiration and lymph node biopsy are invasive procedures.

The most common side effect of irradiation is fatigue. This is particularly difficult for active, outgoing school-age children and adolescents, because it prevents them from keeping up with their peers. Sometimes adolescents will push themselves to the point of physical exhaustion rather than admit and succumb to the decreased activity tolerance. The nurse cautions parents to observe for behavior such as extreme fatigue at the end of the day, falling asleep at the dinner table, inability to concentrate on homework, or an increased susceptibility to infection. A regular bedtime and scheduled rest periods are important for these children, especially during chemotherapy, when myelosuppression increases the risk of infection and debilitation. Before discharge the nurse should discuss a feasible school schedule with the parents and child.

An area of concern for adolescents is the high risk of sterility from irradiation and chemotherapy. Both drugs, particularly procarbazine and alkylating agents, and irradiation to the gonads can lead to infertility. Adolescents should be informed of these side effects early in the course of the diagnosis and treatment. Sperm banking is now offered at many cancer centers before the initiation of treatment in adolescent boys. Sexual function is not altered, although the appearance of secondary sexual characteristics and menstruation may be delayed in the pubescent child. Delayed sexual maturation may be an extremely sensitive and stressful issue for children (see Chapter 17).

NON-HODGKIN LYMPHOMANon-Hodgkin lymphoma (NHL) occurs more frequently in children than Hodgkin disease. NHL is diagnosed in approximately 750 to 800 children each year in the United States (Link and Donaldson, 2003). Histologic classification of childhood NHL is strikingly different from that of Hodgkin disease, as demonstrated in the following statements:

• The disease is usually diffuse rather than nodular.

• The cell type is either undifferentiated or poorly differentiated.

• Dissemination occurs early, more often, and rapidly.

• Mediastinal involvement and invasion of meninges are common.

NHL exhibits a variety of morphologic, cytochemical, and immunologic features, not unlike the diversity seen in leukemia. Classification is based on the histologic pattern: (1) lymphoblastic, (2) Burkitt or non-Burkitt, or (3) large cell. Immunologically these cells are also classified as T cells; B cells; or non-T, non-B cells (lacking immunologic properties).

The clinical staging system used in Hodgkin disease is of little value in NHL, although it has been modified and other systems have been developed.

Diagnostic EvaluationBecause the clinical presentation of most children with NHL is widespread disseminated disease, thorough pathologic staging is unnecessary. Clinical manifestations depend on the anatomic site and extent of involvement. These manifestations include many of those seen in Hodgkin disease and leukemia, as well as organ symptoms related to pressure from enlargement of adjacent lymph nodes, such as intestinal or airway obstruction, cranial nerve palsies, and spinal paralysis.

Recommendations for staging include a surgical biopsy of an enlarged node, histopathologic confirmation of disease with cytochemical and immunologic evaluation, bone marrow examination, radiographic studies (especially tomograms of the lungs and GI organs), and lumbar puncture.

Therapeutic ManagementThe treatment protocols for NHL include aggressive use of irradiation and chemotherapy. Similar to leukemic therapy, the protocols include induction, consolidation, and maintenance phases, some with intrathecal chemotherapy. Several antineoplastic agents used in the treatment of NHL include vincristine, prednisone, L-asparaginase, methotrexate, 6 mercaptopurine, cytarabine, cyclophosphamide, anthracyclines, and teniposide or etoposide (Link and Donaldson, 2003).

Prognosis.

The prognosis is excellent for children with localized disease, and long-term remissions are possible in many patients, even in those with disseminated disease. Because relapse after 2 years is rare, survival after 24 months is considered a cure.

Nursing ConsiderationsNursing care of the child with NHL is very similar to that required for children with leukemia. Many of the same drugs are employed, although the schedules differ. Because of the intense chemotherapy, nursing care is primarily directed toward managing the side effects of these agents and providing supportive care to the child and family (see Nursing Care Plan, pp. 966 to 970).

IMMUNOLOGIC DEFICIENCY DISORDERSA number of disorders can cause profound, often life-threatening alterations within the body's immune system. The most serious are those conditions that completely depress immunity, such as severe combined immunodeficiency disease. However, the one disorder that generates the most anxiety, within both the family and the community at large, is HIV infection/AIDS.

Several classifications of immune dysfunction exist. AIDS, severe combined immunodeficiency syndrome (SCID), and Wiskott-Aldrich syndrome are syndromes wherein the body is unable to mount an immune response. The immune response can also be misdirected. In autoimmune disorders, antibodies, macrophages, and lymphocytes attack healthy cells.

HUMAN IMMUNODEFICIENCY VIRUS INFECTION AND ACQUIRED IMMUNODEFICIENCY SYNDROMESince the first cases of AIDS were identified in the early 1980s, HIV infection has generated intense medical investigation. Research has led to early diagnosis of and improved medical treatments for HIV infection, changing this disease from a rapidly fatal one to a chronic, but terminal, disease of childhood.

EpidemiologyThe first AIDS case in the pediatric population in the United States was identified in children born to HIV-infected mothers, or in children who received blood products. More than 90% of these children acquired the disease perinatally from their mothers. Smaller numbers of children were infected through the transfusion of contaminated blood/blood products before 1985 or were infected through sexual abuse. In contrast, sexual activity and IV drug use are major sources of HIV infection in adolescents.

The estimated number of children with perinatally acquired AIDS peaked during 1992; subsequent years have seen significant declines. This trend is a result of implementation of recommended HIV counseling and voluntary testing practices and the use of zidovudine therapy to prevent perinatal transmission. Zidovudine therapy in HIV-infected pregnant women, and subsequently in their infants, has significantly reduced the transmission of HIV (Lyall, 2002; Ioannidis and others, 2001). The effectiveness of other HIV drugs such as Nevirapine to prevent perinatal transmission is being studied (Meldrum, 2003; Merchant and Keshavarz, 2001). Routine HIV counseling and voluntary testing for pregnant women are recommended (American Academy of Pediatrics, 2000), and guidelines for the use of antiretroviral drugs in HIV-infected pregnant women to reduce perinatal transmission are available (Lyall, 2002).

EtiologyHIV is a retrovirus that is transmitted by lymphocytes and monocytes. It is found in the blood, semen, vaginal secretions, and breast milk. It has an incubation period of months to years (Ezekowitz and Stockman, 2003). There are different strains of HIV. HIV-2 is prevalent in Africa, whereas HIV-1 is the dominant strain in the United States and elsewhere. Horizontal transmission of HIV occurs through intimate sexual contact or parenteral exposure to blood or body fluids containing visible blood. Perinatal (vertical) transmission occurs when an HIV-infected pregnant woman passes the infection to her infant. There is no evidence that casual contact between infected and uninfected individuals can spread the virus.

PathophysiologyThe HIV virus primarily infects a specific subset of T lymphocytes, the CD4+ T cells. The virus takes over the machinery of the CD4+ lymphocyte, using it to replicate itself, rendering the CD4+ cell dysfunctional. The CD4+ lymphocyte count gradually decreases over time, leading to progressive immune deficiency. The count eventually reaches a critical level below which there is substantial risk of opportunistic illnesses followed by death.

Clinical ManifestationsCommon clinical manifestations of HIV infection in children are varied (Box 26-12). The diagnosis of AIDS is associated with certain illnesses or conditions. The most common AIDS-defining conditions observed among American children are listed in Box 26-13. Other problems in these children may include short stature, malnutrition, and cardiomyopathy. CNS abnormalities resulting from HIV infection may include neuropsychologic deficits; developmental disabilities; and deficits in motor skills, communication, and behavioral functioning.

BOX 26-12 Common Clinical Manifestations of HIV Infection in Children

Lymphadenopathy

Hepatosplenomegaly

Oral candidiasis

Chronic or recurrent diarrhea

Failure to thrive

Developmental delay

Parotitis

BOX 26-13 Common AIDS-Defining Conditions in Children

Pneumocystis carinii pneumonia (PCP)

Lymphoid interstitial pneumonitis (LIP)

Recurrent bacterial infections

Wasting syndrome

HIV encephalopathy

Candidal esophagitis

Cytomegalovirus disease

Mycobacterium avium-intracellulare complex infection

Severe herpes simplex infection

Pulmonary candidiasis

Cryptosporidiosis

Diagnostic EvaluationFor children 18 months of age and older, the HIV enzyme-linked immunosorbent assay (ELISA) and Western blot immunoassay are performed to determine HIV infection. In infants born to HIV-infected mothers, these assays will be positive because of the presence of maternal antibodies derived transplacentally. Maternal antibodies may persist in the infant up to 18 months of age. Therefore other diagnostic tests are employed, most commonly the HIV polymerase chain reaction (PCR) for detection of proviral DNA. With this technique, more than 95% of infected infants can be diagnosed by 1 month of age (Ezekowitz and Stockman, 2003).

The Centers for Disease Control and Prevention (CDC) (1994) has developed a classification system to describe the spectrum of HIV disease in children (Table 26-3). The system indicates the severity of clinical signs and symptoms and the degree of immunosuppression. Mild signs and symptoms include lymphadenopathy, parotitis, hepatosplenomegaly, and recurrent or persistent sinusitis or otitis media. Moderate signs and symptoms include lymphoid interstitial pneumonitis (LIP) and a

variety of organ-specific dysfunctions or infections. Severe signs and symptoms include AIDS-defining illnesses with the exception of LIP. Children with LIP have a better prognosis than those with other AIDS-defining illnesses. In children whose HIV infection is not yet confirmed, the letter E (vertically exposed) is placed in front of the classification. The immune categories are based on CD4+ lymphocyte counts and percentages. Age adjustment of these numbers is necessary because normal counts, which are relatively high in infants, decline steadily until 6 years of age, when they reach adult norms (Table 26-4).

Table 26-3 Pediatric HIV Classification*

From Centers for Disease Control and Prevention: 1994 Revised classification system for human immunodeficiency virus infection in children less than 13 years of age, MMWR 43(RR-12):1–10, 1994.

* Children whose HIV infection status is not confirmed are classified by using the above table with the letter E (for perinatally exposed) placed before the appropriate classification code (e.g., EN2).

TABLE 26-4 Immunologic Categories Based on Age-Specific CD4

+ T-Lymphocyte Counts and Percent of Total Lymphocytes

From Centers for Disease Control and Prevention: 1994 Revised classification system for human immunodeficiency virus infection in children less than 13 years of age, MMWR 43(RR-12):1–10, 1994.

Therapeutic ManagementThe goals of therapy for HIV infection include slowing the growth of the virus, preventing and treating opportunistic infections, and providing nutritional support and symptomatic treatment. Antiretroviral drugs work at various stages of the HIV life cycle to prevent reproduction of functional new virus particles. Although not a cure, these drugs can suppress viral replication, preventing further deterioration of the immune system, and thus delay disease progression. Classes of antiretroviral agents include nucleoside reverse transcriptase inhibitors (e.g., zidovudine, didanosine, stavudine, lamivudine, abacavir), nonnucleoside reverse transcriptase inhibitors (e.g., nevirapine, delavirdine, efavirenz), nucleotide reverse transcriptase inhibitors (e.g., adefovir), protease inhibitors (e.g., indinavir, saquinavir, ritonavir, nelfinavir, amprenavir), and adjunctive antiretrovirals (e.g., hydroxyurea). Combinations of these drugs are used to forestall the emergence of drug resistance. Antiretroviral therapy regimens and guidelines are continually evolving. Therapy is lifelong, making adherence difficult. Laboratory markers (CD4+ lymphocyte count, viral load) assist in monitoring both disease progression and response to therapy.

Pneumocystis carinii pneumonia (PCP) is the most common opportunistic infection of children infected with HIV. It occurs most frequently between 3 and 6 months of age. All infants born to HIV-infected women should receive prophylaxis during the first year of life according to guidelines set by the CDC (1995) and American Academy of Pediatrics (2000). After 1 year of age, the need for prophylaxis is determined by the presence of severe immunosuppression or a history of PCP (National Institute of Allergy and Infectious Diseases/NIH, 2003; CDC, 1995). Trimethoprim-sulfamethoxazole (TMP-SMZ) is the agent of choice. If adverse effects are experienced with TMP-SMZ, dapsone or pentamidine can be used.

Prophylaxis is often employed for other opportunistic infections, such as disseminated Mycobacterium avium-intracellulare complex (MAC), candidiasis, and herpes simplex. IV immunoglobulin has been helpful in preventing recurrent or serious bacterial infections in some HIV-infected children.

Immunization against common childhood illnesses is recommended for all children exposed to and infected with HIV (American Academy of Pediatrics, 2000). Varicella (chickenpox) vaccine and measles-mumps-rubella (MMR) vaccine can be administered if there is no evidence of severe immunocompromise close contacts. The pneumococcal and influenza vaccines are recommended. Because antibody production to vaccines may be poor or decrease over time, immediate prophylaxis after exposure to several vaccine-preventable diseases (e.g., measles, varicella) is warranted. It should be recognized that children receiving IV gamma globulin prophylaxis may not respond to the MMR vaccine (Morbidity and Mortality Weekly Report [MMWR], 2003).

HIV infection often leads to marked failure to thrive and multiple nutritional deficiencies. Nutritional management may be difficult because of recurrent illness, diarrhea, and other physical problems. Intensive nutritional interventions should be instituted when the child's growth begins to slow or weight begins to decrease.

Prognosis.Early recognition and improved medical care have changed HIV disease from a rapidly fatal illness to a chronic disease. After the introduction of combination antiretroviral therapy, the numbers of new AIDS cases and deaths declined substantially. Between 1995 and 1998, the annual number of AIDS cases declined by 38% and deaths declined by 63% (MMWR, 2003). The annual number of AIDS cases and deaths have remained stable since 1998 (CDC, 2001). The number of children with AIDS, attributed to perinatal HIV transmission, peaked in 1992 at 954 cases and declined by 89% to 101 cases in 2001 (CDC, 2001).

Nursing Considerations

Education concerning transmission and control of infectious diseases, including HIV infection, is essential for children with HIV infection and anyone involved in their care. The basic tenets of standard precautions should be presented in an age-appropriate manner, with careful consideration of the educational levels of the individuals (see Infection Control, Chapter 22). Safety issues, including appropriate storage of special medications and equipment (e.g., needles and syringes), are emphasized. Unfortunately, relatives, friends, and others in the general public may be fearful of contracting HIV infection, and criticism and ostracism of the child and family may occur. In an effort to protect the child, the family may limit the child's activities outside the home. Although certain precautions are justified in limiting exposure to sources of infections, they must be tempered with concern for the child's normal developmental needs. Both the family and the community need ongoing education about HIV to dispel many of the myths that have been perpetuated by uninformed persons.*

Prevention is a key component of HIV education. Educating adolescents about HIV is essential in preventing HIV infection in this age-group. Education should include the routes of transmission, the hazards of IV and other recreational drug use, and the value of sexual abstinence and safe sex practices. Such education should be a part of anticipatory guidance provided to all adolescent patients. Nurses can also encourage adolescents at risk to undergo HIV counseling and testing. In addition to identifying infected teenagers and getting them into care, such counseling affords adolescents an opportunity to learn about, and possibly change, their risk behaviors.

The nurse's role in the care of the child with HIV is multifaceted (see Nursing Care Plan, pp. 976 and 977). The nurse serves as educator, direct care provider, case manager, and advocate. As with all chronic illnesses, these children will have much involvement with the health care system. The need for HIV medications is lifelong. Nurses are instrumental in encouraging and empowering these children (and their caretakers) to adhere to their medication regimens. Clinic visits and hospitalizations may become frequent as the disease progresses. The physiologic care of the child is directed at minimum exposure to infections; nutritional support; comfort measures, including pain management; and assessment and recognition of changes in status that may indicate new complications. The scope of nursing

care will change with new symptoms, changes in treatment, and disease progression. The unpredictability of the course of pediatric HIV infection is a continual source of stress to these children and their caretakers. Psychologic interventions will vary with the unique circumstances of each child and family.

The multiple complications associated with HIV disease are potentially painful (Ezekowitz and Stockman, 2003; Sullivan and Woda, 2003). Aggressive pain management is essential for these children to have an acceptable quality of life. Their pain may be due to infections (e.g., otitis media, dental abscess), encephalopathy (e.g., spasticity), adverse effects of medications (e.g., peripheral neuropathy), or an unknown source (e.g., deep musculoskeletal pain). Sources of pain are related not only to disease processes, but also to various treatments these children often undergo, including venipunctures, lumbar punctures, biopsies, and endoscopies. Ongoing assessment of pain is crucial and is most easily accomplished in older children who are able to communicate. Nonverbal and developmentally delayed children are more difficult to assess. Be alert for other signs of pain: emotional detachment, lack of interactive play, irritability, and depression. Effective pain management depends on the appropriate use of pharmacologic agents, including EMLA cream, acetaminophen, NSAIDs, muscle relaxants, and opioids. Tolerance to opioids may indicate increased dosing; monitored use ensures safety. Nonpharmacologic interventions (guided imagery, hypnosis, relaxation and distraction techniques) are useful adjuncts.

Common psychosocial concerns include disclosure of the diagnosis to the child, making custody plans when the parent is infected, and anticipating the loss of a family member. Other stressors may include financial difficulties, HIV-associated stigma, striving to keep the diagnosis secret, other infected family members, and the multiple losses associated with HIV. Most mothers of these children are single mothers who are also HIV-infected. As primary caretakers, they often attend to the needs of their child first, neglecting their own health in the process (see Family Focus box). The nurse can encourage the mother to receive regular health care. Family members are often involved in the care of the child, particularly if the mother has symptomatic illness. After the death of the mother, a grandparent or other relative typically assumes responsibility for the care of the child. Nursing can provide support and

encouragement for the new surrogate parent, particularly during the transition phase. If no family member is available, the child may be placed in a foster or group home. Nursing is an integral part of the multidisciplinary team necessary for the successful management of the complex medical and social problems of these families.

FAMILY FOCUS: Caregivers and the Infant With HIV InfectionUnlike other fatal pediatric diseases, HIV infection is associated with special family alterations. The infant infected in utero faces multiple physical and parental problems. Because the mother is infected, she may be ill or dying and therefore unable to care for the child. If possible, grandparents or other relatives may assume care. Foster care is often difficult to arrange because of the nature of the disease, especially in relation to the social stigma and the child's multiple medical needs. These children may require frequent hospitalizations with progression of their HIV disease. When children remain in the hospital, the importance of consistent caregivers, especially primary nurses, who attend to the youngsters' physical, developmental, and emotional needs cannot be overemphasized. However, primary nurses may face the risk of overinvolvement and must be aware of the boundaries of a therapeutic relationship.

Children with HIV infection attend day care centers and schools. It is well established that the risk of HIV transmission in these settings is minimal. These institutions are required to follow CDC and Occupational Safety Health Administration (OSHA) guidelines for infection control measures. Standard precautions describing proper management of blood and body fluids should also be followed. It is recommended that school personnel receive current HIV information and include it in the health education curriculum for kindergarten through twelfth grade (American Academy of Pediatrics, 2000; American Academy of Pediatrics, 1999). School nurses play a vital role in educating the school staff, students, and parents. They are also invaluable in monitoring the needs of known affected children.

Confidentiality is a major issue in day care or school attendance. Parents and legal guardians have the right to decide whether they inform these agencies of their child's HIV diagnosis.

Unfortunately, myths about HIV infection continue to exist, and the family often wishes to avoid any potential criticism or ostracism of the child.

Nursing care of the child with HIV infection is summarized in the Nursing Care Plan on pp. 976 and above.

NURSING CARE PLAN: The Child and Adolescent With HIV InfectionNURSING DIAGNOSIS: Risk for infection related to impaired body defenses, presence of infective organisms

PATIENT GOAL 1: Will experience minimized risk of infection

NURSING INTERVENTIONS/RATIONALES

Use thorough handwashing technique to minimize exposure to infective organisms

Advise visitors to use good handwashing technique to minimize exposure to infective organisms

Place child in room with noninfectious children or in private room

Restrict contact with persons who have infections, including family, other children, friends, and members of staff; explain that child is highly susceptible to infection to encourage cooperation and understanding

Observe medical asepsis as appropriate to decrease risk of infection

Encourage good nutrition and adequate rest to promote body's remaining natural defenses

Explain to family and older child importance of contacting health professional if exposed to childhood illnesses (e.g., chickenpox, measles) so that appropriate immunizations can be given

*Administer appropriate immunizations as prescribed to prevent specific infections

*Administer antibiotics as prescribed

EXPECTED OUTCOMES

Child does not come in contact with infected persons or contaminated articles

Child and family apply good health practices

Child exhibits no evidence of infection

PATIENT GOAL 2: Will not spread disease to others

NURSING INTERVENTIONS/RATIONALES

Implement and carry out standard precautions to prevent spread of virus (see Infection Control, Chapter 22)

Instruct others (e.g., family, members of staff) in appropriate precautions; clarify any misconceptions about communicability of virus, because this is a frequent problem and may interfere with use of appropriate precautions

Teach affected children protective methods to prevent spread of infection (e.g., handwashing, handling genital area, care after using bedpan or toilet)

Endeavor to keep infants and small children from placing hands and objects in contaminated areas

Place restrictions on behaviors and contacts for affected children who bite or who do not have control of their bodily secretions

Assess home situation and implement protective measures as feasible in individual circumstances

EXPECTED OUTCOME

Others do not acquire the disease

NURSING DIAGNOSIS: Altered nutrition: less than body requirements related to recurrent illness, diarrheal losses, loss of appetite, oral candidiasis

PATIENT GOAL 1: Will receive optimum nourishment

NURSING INTERVENTIONS/RATIONALES

Provide high-calorie, high-protein meals and snacks to meet body requirements for metabolism and growth

Provide foods child prefers to encourage eating

Fortify foods with nutritional supplements (e.g., powdered milk, commercial supplements) to maximize quality of intake

Provide meals when child is most likely to eat well

Use creativity to encourage child to eat (see Feeding the Sick Child, Chapter 22)

Monitor child's weight and growth so that additional nutritional interventions can be implemented if growth begins to slow or weight drops

*Administer antifungal medication as ordered to treat oral candidiasis

EXPECTED OUTCOME

Child consumes a sufficient amount of nutrients (specify)

NURSING DIAGNOSIS: Impaired social interaction related to physical limitations, hospitalizations, social stigma toward HIV infection

PATIENT GOAL 1: Will participate in peer-group and family activities

NURSING INTERVENTIONS/RATIONALES

Assist child in identifying personal strengths to facilitate coping

Educate school personnel and classmates about HIV infection so that child is not unnecessarily isolated

Encourage child to participate in activities with other children and family

Encourage child to maintain phone contact with friends during hospitalization to lessen isolation

EXPECTED OUTCOME

Child participates in activities with peer group and family

NURSING DIAGNOSIS: Altered sexuality patterns related to risk of disease transmission

PATIENT GOAL 1: Will exhibit healthy sexual behavior

NURSING INTERVENTIONS/RATIONALES

Educate adolescent about the following so that adolescent has adequate information to identify safe, healthy expressions of sexuality:

Sexual transmission

Risks of perinatal infection

Dangers of promiscuity

Abstinence, use of condoms

Avoidance of high-risk behaviors

Encourage adolescent to talk about feelings and concerns related to sexuality to facilitate coping

EXPECTED OUTCOMES

Adolescent exhibits a positive sexual identity

Adolescent does not infect other individuals

NURSING DIAGNOSIS: Chronic pain related to disease process (i.e., encephalopathy, treatments)

PATIENT GOAL 1: Will exhibit minimal or no evidence of pain or irritability

NURSING INTERVENTIONS/RATIONALES

Assess pain (see Pain Assessment, Chapter 21)

Use nonpharmacologic strategies to help child manage pain

For infants, may try general comfort measures (i.e., rocking, holding, swaddling, reducing environmental stimuli [may or may not be effective because of encephalopathy])

Use pharmacologic strategies (see Pain Management, Chapter 21)

Plan preventive schedule if analgesics are effective in relieving continuous pain

Encourage use of premedication for painful procedures to minimize discomfort (i.e., use of EMLA)

Child may benefit from use of adjunctive analgesics (e.g., antiepileptics) that are effective against neuropathic pain

Use pain assessment record to evaluate effectiveness of pharmacologic and nonpharmacologic interventions

EXPECTED OUTCOME

Child exhibits absence of or minimal evidence of pain or irritability

NURSING DIAGNOSIS: Interrupted family processes related to having a child with a dreaded and life-threatening disease

PATIENT (FAMILY) GOAL 1: Will receive adequate support and will be able to meet needs of child

NURSING INTERVENTIONS/RATIONALES AND EXPECTED OUTCOMES

See Nursing Care Plan: The Family of the Child Who Is Ill or Hospitalized, Chapter 21

NURSING DIAGNOSIS: Anticipatory grief related to having a child with a potentially fatal illness

See Nursing Care Plan: The Child Who Is Terminally Ill or Dying, Chapter 18

* Additional information is available from the AIDS Hotline: (800) 342-2437 (AIDS); and from the National Pediatric and Family HIV Resource Center, 30 Bergen Street, ADMC 4, Newark, NJ 07103; (973) 972-0410 or (800) 362-0071; website: http://www.pedhivaids.org.* Dependent nursing action.

SEVERE COMBINED IMMUNODEFICIENCY DISEASESCID is a defect characterized by absence of both humoral and cell-mediated immunity. The terms Swiss-type lymphopenic agammaglobulinemia (an autosomal recessive form of the disease) and X-linked lymphopenic agammaglobulinemia have been used to describe this disorder, which, as the names imply, can follow either mode of inheritance.

Susceptibility to infection occurs early in life, most often in the first month of life. The child suffers from chronic infection, fails to completely recover from an infection, is frequently reinfected, and is infected with unusual agents. Failure to thrive is a consequence of the persistent illnesses.

Diagnosis is usually based on a history of recurrent, severe infections from early infancy; a familial history of the disorder; and specific laboratory findings, which include lymphopenia, lack of lymphocyte response to antigens, and absence of plasma cells in the bone marrow. Documentation of immunoglobulin (Ig) deficiency is difficult during infancy because of the normally delayed response of infants in producing their own immunoglobulins and material transfer of IgG.

Therapeutic ManagementThe only definitive treatment for SCID is HSCT from a histocompatible donor (usually a sibling), a haplo-identical (usually a parent), or a match-unrelated donor. IVIG infusions and PCP prophylaxis are used to augment the humoral immunity until the transplant is performed. Several investigators are attempting gene therapy with some success, but there is a

potential complication of insertional mutagenesis (Buckley, 2002).

Nursing ConsiderationsNursing care focuses on the prevention of infection and supporting the child and family. The care is consistent with that needed for HSCT for any condition (see p. 959). Because the prognosis for SCID is very poor if a compatible bone marrow donor is not available, nursing care is directed at supporting the family in caring for a child with a life-threatening illness (see Chapter 18). Genetic counseling is essential because of the modes of transmission in either form of the disorder.

WISKOTT-ALDRICH SYNDROMEThe Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder characterized by a triad of abnormalities: (1) thrombocytopenia, (2) eczema, and (3) immunodeficiency of selective functions of B lymphocytes and T lymphocytes. A defective gene has been identified and designated the WAS protein (Bonilla and Geha, 2003). At birth the presenting symptoms may be bloody diarrhea as a result of thrombocytopenia. As the child grows older, recurrent infection and eczema become more severe, and the bleeding becomes less frequent.

Eczema is typical of the allergic type and easily becomes superinfected. Chronic infection with herpes simplex is a frequent problem and may lead to chronic keratitis of the eye with loss of vision. Chronic pulmonary disease, sinusitis, and otitis media result from repeated infections. In those children who survive the bleeding episodes and overwhelming infections, malignancy presents an additional risk to survival.

Medical treatment involves (1) counteracting the bleeding tendencies with platelet transfusions, (2) using IV gamma globulin to provide passive immunity, and (3) administering prophylactic antibiotics to prevent and control infection. Splenectomy alone or HSCT may extend the survival to adulthood (Bonilla and Geha, 2003; Champi, 2002).

Nursing ConsiderationsBecause of the poor prognosis for these children, the main nursing consideration is supporting the family in the care of a fatally ill child (see Chapter 18). Physical care is directed at controlling the problems imposed by the disorder. The measures used to control bleeding are similar to those for hemophilia and vWD (see previous discussions). Another major goal is prevention or control of infection. Because eczema is a troublesome problem, nursing measures specific to this condition are especially important (see Chapter 30). The genetic implications of this X-linked recessive disorder differ little from those of any other X-linked disorder.

TECHNOLOGIC MANAGEMENT OF HEMATOLOGIC/IMMUNOLOGIC DISORDERS

BLOOD TRANSFUSION THERAPYTechnologic advances in blood banking and transfusion medicine enable the administration of only the blood component needed by the child, such as packed RBCs in anemia or platelets for bleeding disorders. However, regardless of the blood component infused, all transfusions have some risks. Therefore nurses need to be aware of the possible complications and the appropriate interventions. Table 26-5 summarizes the major hazards of transfusions, the signs and symptoms typically associated with each, and nursing responsibilities. General guidelines that apply to all transfusions include:

• Take vital signs, including blood pressure, before administering blood to establish baseline data for intratransfusion and posttransfusion comparison, then every 15 minutes for 1 hour while blood is infusing.

• Check the identification of the recipient with the donor's blood group and type, regardless of the blood product used.

• Administer the first 50 mL of blood or 20% of the volume (whichever is smaller) slowly and stay with the child.

• Administer with normal saline on a piggyback setup or have normal saline available.

• Administer blood through an appropriate filter to eliminate particles in the blood and prevent the precipitation of formed elements; gently shake the container frequently.

• Use blood within 30 minutes of its arrival from the blood bank; if it is not used, return to the blood bank—do not store in the regular unit refrigerator.

• Infuse a unit of blood (or the specified amount) within 4 hours. If the infusion will exceed this time, the blood should be divided into appropriately sized quantities by the blood bank, and the unused portion refrigerated under controlled conditions.

• If a reaction of any type is suspected, take vital signs, stop the transfusion, maintain a patent IV line with normal saline and new tubing, notify the practitioner, and do not restart the transfusion until the child's condition has been medically evaluated.

TABLE 26-5 Nursing Care of the Child Receiving Blood Transfusions

Although hemolytic reactions are rare, ABO incompatibility remains the most common cause of death from blood transfusion, and human error is usually responsible (administration of the wrong type to the patient or mislabeling of the blood product) (Norville and Bryant, 2002). Hemolysis can also cause the release of large quantities of phospholipids, which are capable of stimulating disseminated intravascular coagulation (see p. 956). Acute kidney shutdown and eventual renal failure are a result of renal vasoconstriction from antigen-antibody complexes derived from the RBC surface.

Blood is usually administered to children by infusion pump; therefore, the usual precautions and management related to pumps apply. When the blood is started with a standard transfusion set, the filter chamber is filled to allow the total filter to be used. The drip chamber is partially filled with blood to permit counting of the drops. In adjusting the flow rate, it is important to remember that blood administration sets do not use microdrops (60 drops/mL) but regular drops (usually 10 or 15 drops/mL). Therefore this must be considered when calculating the flow rate.

HEMATOPOIETIC STEM CELL TRANSPLANTATIONHSCT is used to establish healthy hematopoiesis in both malignant and nonmalignant disease. Candidates for transplantation are children who have disorders that are unlikely to be cured by other means. Most HSCT patients undergo ablative therapy that is intensive using high-dose combination chemotherapy with or without total body irradiation (Ryan and others, 2002). after the body is free of cells and the immune system is suppressed to prevent rejection of the transplanted marrow, the stem cells harvested from the bone marrow, peripheral blood, or the umbilical vein of the placenta are given to the patient by IV transfusion. The newly transfused stem cells will begin to repopulate the ablative bone marrow. In essence, a new blood-forming organ will be accepted by the recipient.

The selection process of a suitable donor and the potential complications in transplantation are related to the HLA system complex. Some of the major HLA antigens are A, B, C, D, and DR. There is a wide diversity for each of these HLA loci. There are

more than 20 different HLA-A antigens that can be inherited and more than 40 different HLA-B antigens.

The genes are inherited as a single unit or haplotype. A child inherits one unit from each parent; thus, a child and each parent have one identical and one nonidentical haplotype. Because the possible haplotype combinations among siblings follow the laws of mendelian genetics, there is a 1-in-4 chance that two siblings have two identical haplotypes and are perfectly matched at the HLA loci.

The importance of HLA matching is to prevent the serious complication known as GVHD. Because the child's immune system is essentially rendered nonfunctional, there is little difficulty with bone marrow rejection by the recipient. However, the donor's marrow may contain antigens not matched to the recipient's antigens, which begin attacking body cells. The more closely the HLA systems match, the less likely GVHD is to develop. However, it can occur even with a perfect HLA match, because there are as yet unidentified and thus unmatched histocompatibility antigens (Guinan, Krance, and Lehmann, 2002).

Different types of BMT are now performed in children with cancer. Allogeneic BMT involves the matching of a histocompatible donor with the recipient. However, allogeneic BMT is limited by the presence of a suitable marrow donor.

Because of the limited numbers of patients having HLA-identical siblings, other types of allogeneic transplants have evolved. Umbilical cord blood stem cell transplantation is an established, rich source of hematopoietic stem cells for use in children with cancer (Ryan and others, 2002). Because stem cells can be found with high frequency in the circulation of newborns, cord blood transplantation has become an alternative for some children (Ryan and others, 2002). The benefit of using umbilical cord blood is the blood's relative immunodeficiency at birth, allowing for partially matched unrelated cord blood transplants to be successful, with a lower risk of GVHD-related problems (Ryan and others, 2002).

Autologous BMTs use the patient's own marrow that was collected from disease-free tissue, frozen, and sometimes treated to remove malignant cells. Children with solid tumors such as neuroblastoma, Hodgkin disease, NHL, rhabdomyosarcoma, Ewing

sarcoma, and Wilms tumor have been treated with autologous BMTs.

Peripheral stem cell transplants (PSCTs) are also used in children with cancer. PSCT, a type of autologous transplant, differs in the way stem cells are collected from the patient. CSF is first given to stimulate the production of many stem cells (Ryan and others, 2002). After the WBC count is high enough, the stem cells are collected by an "apheresis" machine. This machine filters out peripheral stem cells from whole blood, returning the remainder of the blood cells and plasma to the child. Stem cells have been collected in very small children without problems (Guinan, Krance, and Lehmann, 2002). The peripheral stem cells are then frozen until the patient is ready for the PSCT.

Nursing ConsiderationsThe care of children undergoing BMT is similar to that of any child receiving chemotherapy and radiotherapy. The hospitalization is typically 3 to 6 weeks in an isolated environment, during which time the child is subjected to numerous procedures and side effects of therapy. Throughout this long ordeal there is the family's concern for successful engraftment and fear of fatal complications (see Family Focus box). Consequently, nurses involved with the child and family need to provide sensitive care and maintain a supportive attitude during the many crises that may arise. If the procedure is not successful, the care needed by these families is consistent with that required by the family of any child with a life-threatening disorder (see Chapter 18).

FAMILY FOCUS: The Decision for a Hematopoietic Stem Cell TransplantA family's decision for a child to undergo hematopoietic stem cell transplantation (HSCT) may be fraught with challenges. Often the child is facing certain death from the malignancy. The preparation of the child for the transplant also places the patient at great medical risk.

After the preparatory regimen is begun and the child's immune system is destroyed, there is no turning back. Unlike kidney transplantation, HSCT does not have a "rescue" procedure,

such as dialysis, for supportive therapy. If the donor is a sibling, the issue of his or her marrow "saving" the brother or sister can be a concern, especially if the transplant fails. Parents often must leave the home to stay at the transplant center and encounter additional stressors such as arranging child care, taking a leave from work, and managing finances. The patient faces the greatest stress—fear of HSCT failure or life-threatening complications.

APHERESISApheresis is the removal of blood from an individual, separation of the blood into its components, retention of one or more of these components, and reinfusion of the remainder of the blood into the individual. Apheresis is most often used to remove large quantities of platelets from healthy adult donors. These transfusion products have greatly prolonged the survival of patients with hematologic and oncologic diseases.

This technique is used to remove peripheral blood stem cells (PBSCs) from children before they receive HSCT or high-dose chemotherapy or radiation therapy, which is severely toxic to the bone marrow. These PBSCs can then be used to restore the child's bone marrow. Apheresis is also used as a therapeutic modality. The blood component that is diseased or toxic is separated from the blood, and the remainder is returned to the individual. Therapeutic apheresis is considered part of standard therapy for many diseases. Plasma is selectively removed from individuals with hyperviscosity, life-threatening complications of myasthenia gravis, Guillain-Barré syndrome, thrombotic thrombocytopenic purpura, and certain drug overdoses. WBCs are removed from individuals with high-WBC-count leukemia.

Nursing ConsiderationsDifficult venous access and small blood volume can limit the ability to use this therapy in the infant and young child. Education of the family and child focuses on the purposes of the therapy, as well as the technology.

Specially trained individuals perform the apheresis procedure. Attention focuses on rate of removal, blood component separation, and reinfusion of blood into the child. Vital signs are

monitored, and the child is continuously observed for any adverse reactions secondary to the circulatory volume changes and the anticoagulant used.

When apheresis components are infused, nursing measures will differ if the product is autologous (blood component from the child) or allogeneic (blood component from another individual). Autologous components are the child's own blood; therefore, a major precaution is proper identification to ensure the correct component. The rate of infusion should be adjusted to the child's tolerance. If the product is allogeneic, all precautions for blood transfusions apply.

KEY POINTS• Anemia is defined as reduction of red blood cells or hemoglobin concentration to levels below normal for age; disorders are classified either by etiology/physiology or by morphology.

• The role of the nurse in treatment of anemia is to assist in establishing a diagnosis, prepare the child for laboratory tests, administer prescribed medications, decrease tissue oxygen needs, implement safety precautions, and observe for complications.

• The main nursing goal in prevention of nutritional anemia is parent education regarding correct feeding practices.

• Sickle cell anemia is a hereditary hemoglobinopathy caused by adult hemoglobin (Hgb A) being partly or completely replaced by sickle hemoglobin (Hgb S).

• Nursing care of the child with sickle cell anemia is focused on teaching the family how to prevent and recognize sickle cell problems, manage pain during crises, and help the child and parents adjust to a lifelong, chronic disease.

• Nursing care of the child with thalassemia includes observing for complications of multiple blood transfusions, assisting the child in coping with the effects of illness, and fostering parent-child adjustment to long-term illness.

• Causes of acquired aplastic anemia include irradiation, drugs, industrial and household chemicals, infections, infiltration and

replacement of myeloid elements; however, the majority of the causes are idiopathic.

• Clotting depends on three processes: vascular spasm, platelet aggregation, and coagulation and clot formation.

• Nursing care of the child with hemophilia involves preventing bleeding by decreasing the risk of injury, recognizing and managing bleeding with factor replacement, preventing the crippling effects of joint degeneration, and preparing and supporting the child and family for home care.

• Goals in the care of the child with leukemia are to prepare the family for diagnostic and therapeutic procedures, prevent complications of myelosuppression, manage problems of irradiation and drug toxicity, and provide continued emotional support.

• The lymphomas include Hodgkin and non-Hodgkin lymphoma and are disorders involving the lymphoid system.

• Immunodeficiency disorders render the affected individual unable to fight infectious organisms.

• HIV infection is primarily acquired in infants from a parent with HIV infection and in adolescents from engaging in high-risk behaviors.

• Blood transfusions supply needed blood components.

• Hematopoietic stem cell transplantation replaces the diseased or malfunctioning bone marrow with viable blood stem cells.

• Apheresis is the selective removal of a blood component. It can be used to supply cellular elements needed for therapy (i.e., platelets or stem cells) or to remove diseased components.

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