Hematology and Oncology Disorders



Hematology and Oncology Disorders



Objectives



1. Define the vocabulary terms listed


2. Recommend four food sources of iron for an infant with iron-deficiency anemia


3. Discuss how sickle cell disease is inherited


4. Describe the care of hemarthrosis in a child with hemophilia


5. List four symptoms of acute lymphoid leukemia


6. Demonstrate comfort measures for a child undergoing chemotherapy


7. Discuss pre- and postoperative care of a child with Wilms tumor


8. Compare and contrast osteosarcoma and Ewing sarcoma


9. List and describe the four stages of Hodgkin disease



Key Terms











Hematology


imagehttp://evolve.elsevier.com/Price/pediatric/


Hematology is the study of blood and blood-forming tissues. The bone marrow, blood, spleen, and lymph system are included. The red blood cells (erythrocytes), white blood cells (leukocytes), and platelets (thrombocytes) are produced in the bone marrow. The spleen produces red and white blood cells during fetal development; filters red blood cells; and stores lymphocytes, monocytes, and platelets. The lymph system contains the lymph and lymph nodes, which are responsible for fighting infection in the body.



Iron-Deficiency Anemia


The most common nutritional deficiency of children in the United States today is anemia caused by insufficient amounts of iron in the body. The incidence rate has decreased in infants because of the use of iron-fortified formulas and cereals. Toddlers and adolescent girls remain at risk because of rapid growth and inadequate iron intake. Anemia (an, without; emia, blood) is a condition in which there is a reduction in the amount and size of the red blood cells or in the amount of hemoglobin, or both. The clinical features are related to the decrease in the oxygen-carrying capacity of the blood. Iron is needed for the manufacture of red blood cells. Iron-deficiency anemia may be caused by severe hemorrhage, the child’s inability to absorb the iron received, rapid growth requirements, or an inadequate dietary intake. It is also known that whole cow’s milk can precipitate gastrointestinal (GI) bleeding in some babies, which, if left unchecked, can lead to anemia.


Prevention of iron-deficiency anemia begins with good prenatal care to ensure that the mother has a suitable intake of iron during pregnancy. For the first few months after birth, the newborn infant relies on iron that was stored during fetal development. Iron is obtained late in the prenatal period, which has an effect on the infant’s iron stores. Premature infants can deplete their iron stores by as early as 2 months of age. The normal term infant who receives unfortified formula depletes iron stores by about age 4 months. Breastfed infants should be supplemented with oral iron from 4 months of age until iron-rich foods (e.g., iron-fortified cereals) are introduced (AAP Clinical Report, 2010).


The highest incidence of this type of anemia occurs from 9 to 24 months of age. During this period of rapid growth, the baby outgrows the limited iron reserve that was in the body; in addition, iron-fortified formula and infant cereals may have been eliminated from the diet. Poorly planned meals or feeding problems also contribute to this deficiency. The mother may rely too much on bottle feedings to avoid conflict at meals. Unfortunately, cow’s milk contains very little iron. It is important to remember that cow’s milk should not be given to children until they are over the age of 1. Instead, the amounts of solid food should be increased and the milk decreased. Boiled egg yolk; liver; green leafy vegetables; iron-fortified cereal; dried fruits (apricots, peaches, prunes, and raisins); cooked, dried beans; crushed nuts; and whole-grain bread are good sources of iron. Iron-fortified cereals eaten out of the box provide a nutritious snack.


Upon diagnosis, the child’s hemoglobin level is usually less than 10 g/dL. Children may have much lower hemoglobin levels before they show signs and symptoms. Typically, blood tests are done for hemoglobin, hematocrit, morphological changes in red blood cells, and iron concentration. A dietary history is also important in the diagnosis.




Treatment and Nursing Care


Iron-deficiency anemia responds well to treatment. The physician must first differentiate it from other types of anemia. The prescribed iron dosage is given orally two or three times a day between meals. Ferrous sulfate is usually prescribed; ferrous fumerate (Feostat) and ferrous gluconate (Fergon) are additional drugs that may be prescribed. Vitamin C aids in the absorption of iron; therefore juice that is enriched with vitamin C or that naturally contains vitamin C is a good choice. Strawberries, tomatoes, and orange slices are also high in vitamin C. Some liquid preparations are taken through a straw to prevent temporary discoloration of the teeth. Teeth brushing after administration of the drug may help also. Calcium interferes with the absorption of iron; therefore milk should not be given during iron supplement administration. Antacids should be avoided as well. Intramuscular iron is given in cases of malabsorption and when noncompliance with the oral route is a problem. Most children can tolerate the oral drug, and parents should be educated about the importance of compliance so the painful injections can be avoided. The injectable drug is an iron-dextran mixture (Imferon) that must be injected deep in a large muscle, with Z-track technique to minimize staining and irritation. Postinjection massage is not done with Z-track injections. Follow-up evaluation is important. Treatment with the iron preparation is recommended for 6 to 8 weeks after the laboratory values return to normal levels.



imageNursing Brief


Avoid iron poisoning in children by keeping preparations well out of reach. Educate parents about this hazard.



Parent Education. 

Parents need explicit instructions regarding the proper foods for the infant or child. The nurse stresses the importance of using iron-fortified formula throughout the first year of life. Whole milk should not be used before 12 months of age. Infants older than 6 months of age receiving formula should not take more than 32 ounces per day. For children older than 1 year of age drinking whole milk, the amount should be less—about 16 to 24 ounces per day. Infants should be started on solid foods by 6 months of age. Review solid food intake, and suggest specific iron-enriched nutrients including rice cereal. Consider financial, ethnic, and family preferences in discussions.


The stools of babies who are taking iron are tarry green in color. Absence of this finding may indicate poor compliance with therapy by the parents. It is important to emphasize that both dietary changes and supplemental iron therapy are necessary to eradicate iron-deficiency anemia. Dietary changes may need to be lifelong in order to maintain good health and to prevent recurrence. It is important that parents finish the prescribed medication. The main side effect of iron therapy is constipation. Reinforce to the family and child that if constipation occurs, increase water intake, bulk, and activity as permitted. Parents are encouraged to return for periodic evaluation of the child’s blood status. During discussions, nurses should attempt to support parents, who usually have guilt feelings or believe they are not successful parents. It may be comforting for the nurse to reiterate that most babies are in the process of catching up on iron supplies and that the condition is not uncommon.



Sickle Cell Anemia


Sickle cell anemia is one of a group of diseases in which normal Hgb (HgbA) is partly or completely replaced by abnormal sickle Hgb (HbS). Sickle cell disease (SCD) is an inherited defect in the formation of hemoglobin. It occurs mainly in populations of African descent but is also carried by some people of Arabian, Greek, Maltese, and Sicilian descent or other Mediterranean groups. Sickling caused by decreases in blood oxygen may be triggered by dehydration, infection, physical or emotional stress, or exposure to cold. Laboratory examination of the affected child’s blood shows that the red blood cell has changed its shape to resemble that of a sickle blade, from which the name of the disorder is derived (Figure 21-1). These cells contain an abnormal form of hemoglobin, termed hemoglobin S (the sickling type). The membranes of these cells are fragile and easily destroyed. Their crescent shape makes it difficult for them to pass through the capillaries, causing a pile-up of cells in the small vessels. This clumping together may lead to a thrombosis (clot) and cause an obstruction. Infarcts, or areas of dead tissue, may result when the tissue is denied proper blood supply. These generally develop in the spleen but may also be seen in other areas of the body, such as the brain, heart, lungs, GI tract, kidneys, and bones. The child feels pain in the affected area.


image
FIGURE 21-1 Peripheral blood smear from a child with hemoglobin with SCD. Note the sickle cells.

There are two types of sickle cell disorders: an asymptomatic (a, without; symptoma, symptom) version, referred to as sickle cell trait, and a much more severe form requiring intermittent hospitalization termed sickle cell disease. There are a variety of screening methods. Electrophoresis and high-performance liquid chromatography (HPLC) are most commonly used.



Sickle Cell Trait


This form of the disease occurs in about 10% of the African-American population in the United States. The blood of the child contains a mixture of normal (hemoglobin A) and sickle (hemoglobin S) hemoglobins. The proportions of hemoglobin S are low because the disease is inherited from only one parent. The physician can distinguish sickle cell trait from the more severe form by studying the child’s red blood cells and hemoglobin. In sickle cell trait, the hemoglobin and red blood cell counts are normal. Although there is no need for treatment of the mild form, the child is a carrier and genetic counseling is important. Advice might be sought from a family physician, pediatrician, or genetic specialist. The nurse encourages and supports such efforts made by the parents. The importance of regular visits to a well-child clinic or family-centered clinic is stressed.



Sickle Cell Disease


This severe form of the disease results when the child inherits the abnormal gene from each parent (Figure 21-2). Each offspring has one chance in four of inheriting the disease (not one of four children). The incidence rate is about 1 in 600 African Americans. The symptoms generally do not appear until the last part of the first year of life, although they may occur as early as 4 months (fetal hemoglobin inhibits sickling). The first symptom may be an unusual swelling of the fingers and toes called hand-foot syndrome or dactylitis (Figure 21-3). Damage to the kidney can result, affecting the kidney’s ability to concentrate urine. This can lead to increased urination in children. Small children with SCD are difficult to toilet-train and may wet the bed for several years. When this is explained to parents as a side effect of the disease, they may be more able to accept the problem. Teenagers and adults with SCD may develop painful, slow-healing ulcers on the lower legs, particularly on the ankles.


image
FIGURE 21-2 Inheritance patterns for autosomal genes. A, When both parents have a trait, offspring have a 25% chance of being normal, a 25% chance of having the disease, and a 50% chance of having the trait. B, When one parent has the disease and the other parent has the trait, offspring have a 50% chance of having the trait and a 50% chance of having the disease. C, When one parent has the disease and othe other parent is normal, all offspring will have the trait. A, Gene for normal hemoglobin; X, gene for abnormal hemoglobin.

image
FIGURE 21-3 Hand-foot syndrome (dactylitis) in a 3-year-old child with SCD. This syndrome is primarily seen in infants and toddlers and is less frequent in the older child.

Chronic anemia is present, which is why the disease may be referred to as sickle cell anemia. The hemoglobin level ranges from 6 to 9 g/dL or lower. The child is pale, tires easily, and loses appetite. These manifestations of anemia are complicated by what is termed the sickle cell crisis, which can be fatal. A number of types of crises have been defined. They differ in pathology and may require somewhat different treatment (Table 21-1). Unfortunately, in some cases, the sickle cell crisis is the first evidence of the condition. For this reason, all 50 states screen all newborn infants. Parents are contacted immediately if there is a positive state screening result. Regularly scheduled health visits, penicillin prophylaxis, and immunizations including influenza, pneumococcal, and meningococcal vaccine should be stressed with the parents.



In a sickle cell crisis, the child appears acutely ill with severe abdominal pain. Muscle spasms, leg pains, or painful swollen joints may be seen. Fever, vomiting, hematuria, convulsions, stiff neck, coma, or paralysis can result, depending on the organs involved. The child may be jaundiced. Cardiac enlargement and murmurs are not uncommon. Priapism is a painful penile erection that occurs in male children, adolescents, and adults. This can be a medical emergency if it is prolonged. According to Pack-Mabien and Haynes (2009), cholelithiasis (formation of gallstones) and retinopathy (disorder of the retina) resulting in blindness are additional complications to monitor for in children with sickle cell disease. The sickle cell crises recur periodically throughout childhood; however, they tend to decrease with age. Between episodes, children should be kept in good health. They should refrain from becoming overly tired. They also should avoid situations such as flying in an unpressurized airplane or exercising at high altitude because oxygen concentrations are already reduced in the blood. Added stress and exposure to cold may lower resistance, causing additional problems. Overheating, which can lead to dehydration, should also be avoided.



imageNursing Brief


During crises, anticipate a child’s need for hydration, rest, protection from infection, pain control, blood transfusions, and emotional support for life-threatening illness.



Treatment and Nursing Care


When the infant or child is hospitalized during a crisis, the treatment is supportive and symptomatic. The child is often confined to bed. Heat is soothing, but cold is not used because it causes sickling and vasoconstriction. Oxygen is used short term with respiratory difficulty; however, it is not used routinely or long term with SCD. Blood transfusions may be given for anemia, but they must be given conservatively to avoid iron overload. If iron overload becomes a problem, chelation therapy with desferrioxamine is begun. Antimicrobials are given to all children with fever. Infection is the most common risk for infants with SCD, and this has prompted many practitioners to use penicillin prophylactically. Starting at 2 months of age, this preventive therapy has significantly reduced both morbidity and mortality from pneumococcal infections and streptococcal pneumonia. Fluid intake is increased above the maintenance level for the child’s age. Analgesics are given for relief of pain. Children in a severe pain crisis should receive a continuous intravenous narcotic infusion, and morphine is the drug of choice. Meperidine (Demerol) is not routinely prescribed for children.


The nurse observes the overall appearance of the child and assesses the developmental stage, body proportions, and the relation of height and weight to age. Facial expressions, degree of restlessness, and areas of pain are noted and recorded. Signs and symptoms that may be observed and require immediate attention are signs of dehydration possibly detected by an elevated temperature; a rapid, weak pulse; a sunken fontanel in infants younger than 18 months of age; weight loss; poor tissue turgor; dry skin, lips, and mucous membranes; and a decrease in urination. If vomiting occurs, appropriate oral hygiene is provided. The nurse observes and records infusions according to unit policy. An accurate record of intake and output is kept. Careful attention is given to the skin. Jaundice (icterus) can be detected by observing whether the skin (palms and soles) and the whites of the eyes have taken on a yellowish tinge. The child’s body position is changed gently due to the pain in the joints.


Because SCD can affect muscle tone, any rigidity of the muscles should be reported. Observe eye movements, swallowing, or sucking. Note whether the child is uncomfortable when the neck is flexed to have the gown changed. Watch for twitching in the face or elsewhere.


Neurologic complications such as stroke, hemiparesis, transient ischemic attack (TIA), or seizures are possible. Children should be monitored regularly. The transcranial Doppler (TCD) is effective in screening for increased blood velocity and narrowing of cerebral vessels and should be done yearly.


The prognosis is guarded. Death may result from severe anemia or secondary infection. Pregnancy may increase mortality. There is also an increased likelihood of miscarriage, premature births, and stillbirths in women with SCD. Ideally, all African-American women should be screened for the disease before pregnancy. The sickling test (Sickledex) is commonly used for screening purposes.



Surgery. 

The approach to splenectomy in children with SCD has been conservative. Recurrence of acute splenic sequestration becomes less likely after 5 years of age. Routine splenectomy is not recommended because the spleen generally atrophies on its own because of fibrotic changes that take place in children with SCD. However, splenectomy is indicated in selected children with multiple splenic events. Because no form of prophylaxis is foolproof and because the duration of treatment is controversial, the child should continue to be carefully observed for signs of infection. Parents should be educated on how to palpate for an enlarged spleen and monitor for signs of infections (fever).


Before elective surgery, a sickle cell screening test should be performed on all African-American children because general anesthesia places these persons at greater risk for hypoxia. With the stress of surgery and hypoxia from anesthesia, a sickle cell crisis can be precipitated.



Medication. 

The FDA has designated the use of hydroxyurea, an antineoplastic drug, for the palliative treatment of SCD in adults. This drug increases the production of hemoglobin F (HbF; fetal hemoglobin). HbF has a higher affinity for oxygen. Erythropoietin, which stimulates the production of red blood cells, may be used to enhance the effects of hydroxyurea. A reduction in episodes of painful vaso-occlusive crisis, fewer hospitalizations, and fewer blood transfusions have been seen. Because this is an antineoplastic drug and could possibly result in mutation of genes, childbearing issues must be considered. Trials have been done with children between 5 and 15 years of age. These studies have positive results, but hydroxyurea use in children should be supervised by a pediatric hematologist (Hilliard and Howard, 2006).


New advances with stem cell transplantation have produced exciting results for the child with SCD. Ongoing investigation is continuing with stem cell and umbilical cord blood transplantation.



Hemophilia


Hemophilia is one of the oldest hereditary diseases known. It has been called “the disease of kings” because of its occurrence in children of several royal families in Russia and Western Europe. In hemophilia, the blood does not clot normally, and even the slightest injury can cause severe bleeding. The clotting disorder is the result of a deficiency in specific blood clotting factors. A sex-linked genetic pattern causes most cases of hemophilia, although it can rarely occur with no family history of the disease. Most hemophiliacs are male. Affected males inherit the bleeding disorder from their mothers, who are the carriers. It is possible to determine the level of factor VIII in the blood with a test called the partial thromboplastin time (PTT). This aids in the diagnosis and assessment of the child’s condition. Women who are carriers and affected fetuses can be identified.


Two types of hemophilia constitute the highest incidence of the disorder. Factor VIII deficiency, or hemophilia A, is approximately four times more common than factor IX deficiency, hemophilia B. For our purposes, this discussion is limited to classic hemophilia, or hemophilia A, which accounts for about 80% of cases.


The severity of hemophilia A depends on the level of factor VIII in the plasma of the child’s blood. Hemophilia is classified as severe, moderate, or mild. In mild hemophilia, bleeding is usually only a problem after surgery or major trauma. The child with moderate hemophilia can expect bleeding episodes after trauma. Children with severe hemophilia may bleed without apparent cause. The degree of severity tends to remain constant within a given family.



Signs and Symptoms


Hemophilia often is not apparent in the newborn infant unless abnormal bleeding occurs at the umbilical cord, at sites of initial injections, or after circumcision. As the child grows older and becomes more subject to injury, the slightest bruise or cut can induce extensive bleeding. Normal blood clots in about 3 to 6 minutes. In a child with severe hemophilia, the time necessary for clotting may be an hour or more. Anemia, leukocytosis, and a moderate increase in platelets may be seen in the hemorrhaging child, who may show signs of shock. Hematuria is occasionally seen. Parents may notice that it takes a long time to stop bleeding from a cut. Death can result from excessive bleeding anywhere in the body but particularly when hemorrhage occurs into the brain or neck.


An injured knee, elbow, or ankle presents particular problems because of hemorrhage into the joint cavity (hemarthrosis) and is a cardinal sign in children with hemophilia. The earliest joint hemorrhages appear most commonly in the ankle, from instability of this joint as the toddler assumes an upright posture. Many children with severe hemophilia develop a “target” joint where repetitive bleeding episodes occur (Kliegman et al., 2007). The affected joint is stiff, warm, red, and swollen. Joint limitation occurs. Repeated hemorrhages may cause permanent deformities that could disable the child.



Treatment and Nursing Care


The mechanism of blood formation is complex. Defects in the synthesis of protein may lead to deficiencies in any of the factors in blood plasma needed for clotting to occur. The treatment of each type of hemophilia consists of replacing the deficient factor to ensure clotting. The nursing care for all types is similar.


The child’s family history is of particular importance in the diagnosis of hemophilia. When hemophilia is present in the family and the child has had periods of abnormal bleeding from early childhood, the determination is relatively easy. However, in many cases the family history may be vague or unobtainable. In some instances, even careful scrutiny produces no evidence of the disease in the family.


Visible bleeding is treated immediately with the application of a cold pack and continuous, firm pressure. When possible, elevate the area above heart level to decrease blood flow. Parents should have ice packs available at all times. Nosebleeds can be controlled by tilting the head forward and applying firm pressure to the nose for 15 to 30 minutes. A nasal pack may be necessary. Mouth bleeding is usually minor, but if it cannot be controlled, an antifibrinolytic agent such as Amicar may be used to promote clot formation.


Hemarthrosis occurs most frequently in the knees, elbows, and ankles. Regardless of its location, the deficient factor should be given and the joint immobilized. Bleeding is also treated with rest, ice, compression, and elevation of the affected part (RICE). Cold packs are applied to decrease the pain, and analgesics such as acetaminophen can be given as ordered. Aspirin and nonsteroidal antiinflammatory drugs (NSAIDs), such as ibuprofen, should be avoided because they have a depressive effect on platelet function. The joint might be placed in a splint for immobilization. Therapy for muscle injuries is essentially the same. When the bleeding has ceased, the child can begin active exercises under the supervision of the physical therapist.



imageNursing Brief


Instruct parents of the child with hemophilia to avoid medications that inhibit platelet function, such as aspirin and ibuprofen. Explain the importance of contacting a physician or pharmacist before giving any over-the-counter drugs to the child.


Current treatment of hemophilia includes the administration of highly purified or recombinant factor VIII concentrates to treat bleeding episodes or anticipated bleeding episodes (surgery, tooth extraction). These concentrates are in powder form and may be kept at room temperature or in the refrigerator. They are reconstituted with sterile water before IV administration. In the past, the risk for hepatitis and HIV was high in this population because concentrates were made from a pool of as many as 20,000 blood donors and were not adequately treated to eliminate viruses. Careful screening of blood donors and new techniques to make concentrate have reduced the risk for children newly diagnosed with hemophilia. Treatment ranges from four times a week (preventive regime) to three times a month.


Parents can be taught at home how to administer the factor VIII concentrate to their child. Home care and management of bleeding episodes have improved the prognosis and quality of life for children with hemophilia. The newer treatment options have also reduced the cost of treatment and decreased the risk for psychological trauma for the child. Detailed teaching is done by the physician and nurse in a specialty clinic. Instruction includes an exact explanation of the illness, with emphasis on the signs and treatment of any active bleeding. Specific procedures taught include the storage and preparation of replacement factors, venipuncture, transfusion management and possible reactions, and record keeping. Signs of complications are reviewed, and emergency numbers and other protocols are spelled out. One advantage of home treatment is its immediate availability. The earlier hemarthrosis is treated, the less severe the consequences. The goal is for the child to become independent and for the health care center to be available as a backup when a need arises. Older school-age children are able to learn self-care. Many children learn more about their disease at hemophilia camp.


Desmopressin (DDAVP) has been found to increase factor VIII levels in children with hemophilia. The increase is not enough to manage hemarthrosis or severe bleeding episodes, but it can be used to treat mild hemophilia. It is less expensive and less invasive than administration of factor VIII concentrate.


Preventing bleeding episodes is an important part of comprehensive care. When the child with hemophilia is an infant, the crib sides require padding and all toys must be checked for sharp edges. Active toddlers and preschoolers need a safe environment and close supervision in which to practice newly learned gross-motor skills. The use of protective equipment such as helmet and joint padding is essential for active children. The older child should avoid contact sports and other activities that have a high risk for injury. Swimming is an excellent competitive sport that also helps strengthen muscles and maintain joint mobility. Walking and bicycle riding are also good ways to exercise. An active, regular exercise program is beneficial. Strong muscles support joints and reduce the numbers of bleeds.


The nurse should teach the parent to carefully observe the skin at bath time for bruises or hematomas. The child’s nails should be kept short. Good oral hygiene is essential. Select a toothbrush with soft bristles. The dentist needs to be consulted early in the preschool years to establish a program of preventive oral health.


The child needs well-balanced meals. Excessive weight gain should be avoided because it places additional strain on the joints. A regular exercise program strengthens muscles surrounding the joints, thus decreasing the potential for tissue injury. If the child is receiving medication via injection, or if blood work has been ordered, pressure is applied to the site immediately afterward. The site is carefully observed by the parent or nurse to ensure that all is well. The child’s stools and urine are observed for blood. Vital signs are taken routinely to detect concealed bleeding. Children and adolescents are instructed to wear a Medic Alert bracelet. All children should receive routine childhood immunizations as recommended (see the Appendix on Evolve for more on immunizations).



imageNursing Brief


Consult the child’s physician regarding immunizations and intramuscular injections as these can cause bleeding into the muscle.

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Dec 22, 2016 | Posted by in NURSING | Comments Off on Hematology and Oncology Disorders

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