Disorders of the Blood

1. Leukemia
   1. Introduction: Known as “cancer of the blood,” leukemia is the most common form of childhood cancer, with an incidence of 4/100,000. Acute leukemia is basically a malignant proliferation of white blood cell (WBC) precursors triggered by an unknown cause and affecting all blood - forming organs and systems throughout the body. The onset is typically insidious, and the disease is most common in preschoolers (age 2-6 year old), occurring more frequently in boys.
   2. Assessment:
      1. Major problem - leukopenia: decreased WBC/ increased blasts (overproduction of immature, poorly functioning white blood cells).
      2. Bone marrow dysfunction results in:
         1. Neutropenia: multiple prolonged infections.
         2. Anemia: pallor, weakness, irritability, shortness of breath.
         3. Thrombocytopenia: bleeding tendencies (petechiae, epistaxis, bruising).
      3. Infiltration of reticuloendothelial system (RES): hepatosplenomegaly, abdominal pain, lymphadenopathy.
      4. Leukemic invasion of CNS: increased intracranial pressure/leukemic meningitis.
      5. Leukemic invasion of bone: pain, pathological fractures, hemarthrosis.
   3. Analysis/nursing diagnosis:
      1. Risk for infection related to neutropenia.
      2. Risk for injury related to thrombocytopenia.
      3. Altered nutrition, less than body requirements, related to loss of appetite, vomiting, mouth ulcers.
      4. Pain related to disease process and treatments (e.g., hemarthrosis, bone pain, bone marrow aspiration).
      5. Activity intolerance related to infection and anemia.
      6. Self-esteem disturbance related to disease process and treatments (e.g., loss of hair with chemotherapy, moon face with prednisone).
      7. Anticipatory grieving related to life - threatening illness.
      8. Knowledge deficit related to diagnosis, treatment, prognosis.
   4. Nursing care plan/implementation:
      1. Goal: maintain infection - free state.
         1. Standard precautions to prevent infection
         2. Use good hand - washing technique.
         3. Ongoing evaluation of sites for potential infection, e.g., gums.
         4. Provide meticulous oral hygiene.
         5. Keep record of vital signs, especially temperature.
         6. Provide good skin care.
         7. Screen staff and visitors (especially children) - restrict anyone with infection.
         8. Protective isolation/reverse isolation to minimize exposure to potentially lifethreatening infection.
         9. Discharge planning: return to school, but isolate from chickenpox or known communicable diseases.
      2. Goal: prevent injury.
         1. Avoid IMs/IVs if possible, due to bruising and bleeding tendencies.
         2. Do not give aspirin or medications containing aspirin, which will interfere with platelet formation, thus increasing the risk of bleeding.
         3. Use soft toothbrush to avoid trauma to gums, which may cause bleeding and infection.
         4. Avoid “per rectum” suppositories, due to probable rectal ulcers.
         5. Supervise play/activity carefully to promote safety and prevent excessive bruising or bleeding.
      3. Goal: promote adequate nutrition.
         1. Diet: high calorie, high protein, high iron.
         2. Encourage extra fluids to prevent constipation or dehydration.
         3. I&O, daily weights, to monitor fluid and nutritional status.
         4. Allow child to be involved with food selection/preparation; allow child almost any food he or she tolerates, to encourage better dietary intake.
         5. Thoroughly wash/peel fresh fruits and vegetables, if child is neutropenic (or avoid eating these foods).
         6. Serve frequent, small snacks to increase fluid and caloric consumption.
         7. Offer dietary supplements to increase caloric intake.
         8. Encourage local anesthetics such as dextromethorphan throat lozenges (Chloraseptic) before meals to allow child to eat without pain from oral mucous membrane ulcers.
      4. Goal: relieve pain.
         1. Offer supportive alternatives: extra company, back rub. Offer complementary therapies: meditation, visualization (if age appropriate).
         2. Administer medications, regularly, before pain becomes excessive.
         3. Use bean bag chair for positional changes.
         4. Avoid excessive stimulation (noise, light), which may heighten perception of pain.
      5. Goal: promote self - esteem.
         1. Stress what child can still do to keep the child as independent as possible.
         2. Encourage performance of ADL as much as possible to foster a sense of independence.
         3. Provide diversion/activity as tolerated.
         4. Give lots of positive reinforcement to enhance a sense of accomplishment.
         5. Provide realistic feedback on child’s appearance; offer suggestions, such as a wig or cap to cover alopecia secondary to chemotherapy.
         6. Encourage early return to peers/school to avoid social isolation.
      6. Goal: prevent complications related to leukemia/prolonged immobility/treatments.
         1. Inspect skin for breakdown, especially over bony prominences, due to poor nutritional intake and limited mobility due to bone pain.
         2. Anticipate need for and administer (per physician’s order) multiple transfusions of platelets, packed RBCs, etc.
         3. Check for hemorrhagic cystitis; push fluids (especially with cytoxan).
         4. Check for constipation or peripheral neuropathy (especially with vincristine).
      7. Goal: assist child and parents to cope with lifethreatening illness.
         1. Teach rationale for repeated hospitalizations, multiple invasive tests/treatments, long - term follow - up care.
         2. Encourage compliance with all aspects of therapy, to increase chances of survival.
         3. Support family and their coping mechanisms.
         4. Offer factual information regarding ultimate prognosis (“70% cure” for acute lymphocytic leukemia).
         5. If death appears imminent, assist family to cope with dying and death.
   5. Evaluation/outcome criteria:
      1. Child is maintained in infection - free state.
      2. Injuries are prevented, or kept to a minimum.
      3. Adequate nutrition is maintained.
      4. Child is free from pain or can live with minimum level of pain.
      5. Child’s self - esteem is maintained; child is treated as living (not dying).
      6. Complications are prevented or kept to a minimum.
      7. Child and family use positive coping mechanisms to deal with illness.


2. Sickle cell anemia
   1. Introduction: Sickle cell anemia is a congenital hemolytic anemia resulting from a defective hemoglobin molecule (hemoglobin S). It is most common in African Americans (8% have sickle cell trait) and in people of Mediterranean, Hispanic, and Middle Eastern descent. The diagnosis is usually made during the toddler or preschool years, during the first crisis episode following an infection. There is also the need to differentiate between sickle cell trait (Sickledex test) and sickle cell anemia (hemoglobin electrophoresis). Sickle cell anemia has no known cure.
   2. Assessment:
      1. Increased susceptibility to infection (cause: unknown; most common cause of death in children under 5).
      2. Inherited as autosomal recessive disorder.
      3. Precipitated by conditions of low oxygen tension, dehydration, vascular obstruction, increased blood viscosity.
      4. Signs of anemia:
         1. Pallor (in dark - skinned children, do not rely on pallor alone - check hemoglobin and hematocrit).
         2. Jaundice, due to excessive hemolysis.
         3. Irritability, lethargy, anorexia, malaise.
      5. Vaso-occlusive crisis: severe pain (variable sites, e.g., chest, back, abdomen), fever, swelling of hands and feet, joint pain and swelling, all related to hypoxia, ischemia, and necrosis at the cellular level. Most common; usually non - life threatening.
      6. Splenic sequestration crisis: blood is sequestered (pooled) in spleen; precipitous drop in hemoglobin levels and blood pressure, increased pulse rate, shock, and ultimately death from profound anemia and cardiovascular collapse.
   3. Analysis/nursing diagnosis:
      1. Altered tissue perfusion related to anemia and occlusion of vessels.
      2. Pain related to vaso-occlusion.
      3. Impaired physical mobility related to pain, immobility.
      4. Knowledge deficit related to disease process and treatment (e.g., prevention of sickling or infection; genetic counseling).
   4. Nursing care plan/implementation:
      1. Goal: prevent sickling.
         1. Avoid conditions of low oxygen tension (hypoxia), which causes RBCs to assume a sickled shape.
         2. Provide continuous extra fluids to prevent dehydration, which causes sluggish circulation. Avoid temperature extremes.
         3. Avoid activities that may result in overheating, to prevent dehydration; suggest appropriate clothes; limit time in sun.
         4. If dehydrated due to acute illness, supplement with IV fluids and additional oral fluids to reestablish fluid balance.

2. 4. 2. Goal: maintain infection - free state.
         1. Standard precautions to prevent infection.
         2. Use good hand - washing technique.
         3. Evaluate carefully, check continually for potential infection sites, which may either lead to death due to sepsis or precipitate sickle cell crisis.
         4. Teach importance of prevention of sickle cell crisis: adequate nutrition; frequent medical checkups; keep away from known sources of infection.
         5. Stress need to report early signs of infection (Acute Chest Syndrome) promptly to physician.
         6. Need to balance prevention of infection with child’s need for a “normal” life.
         7. Administer pneumococcal and influenza vaccines to prevent infection (ages 2, 6, and every 10 years).
      3. Goal: provide supportive therapy during crisis.
         1. Provide bed rest/hospitalization during crisis to decrease the body’s demand for oxygen.
         2. Relieve pain due to infarction of tissues by administering pain medications as ordered; handle gently and use proper positioning techniques.
         3. Apply heat (never cold) to affected painful areas to increase blood flow (vasodilation) and oxygen supply.
         4. Administer oxygen, as ordered, to relieve hypoxia and prevent further sickling.
         5. Administer blood transfusions, as ordered, to correct severe anemia. Complication: If hypertransfusion programs are implemented, child may develop “iron overload.” Desferal chelates the iron so that it can be excreted through the urine or bile to help reduce this complication.
         6. Standard meds: hydroxyurea, folic acid.
         7. Monitor fluid and electrolyte balance: I&O, weight, electrolytes.
         8. Perform ADL for child if unable to care for own needs; encourage self-care as soon as possible to promote independence. Avoid stress and fatigue.
      4. Goal: teach child and family about sickle cell anemia.
         1. Provide factual information based on child’s developmental level.
         2. When asked, offer information regarding prognosis (no known cure).
         3. Encourage child to live as normally as possible.
         4. Genetic screening and counseling.
   5. Evaluation/outcome criteria:
      1. Sickling is prevented or kept to a minimum.
      2. Child is maintained in infection-free state.
      3. Child/family verbalize that they can cope adequately with crisis.
      4. Child/family verbalize their understanding about disease, its management, and its prognosis.


3. Hemophilia
   1. Introduction: Hemophilia is a bleeding disorder inherited as a sex - linked (X - linked) recessive trait; i.e., it occurs only in males but is transmitted by symptom-free female carriers. Hemophilia results in a deficiency of one or more clotting factors, and it is necessary to determine which clotting factor is deficient and to what extent. Classic hemophilia (hemophilia A), a lack of clotting factor VIII, accounts for 75% of all cases of hemophilia.
   2. Assessment:
      1. Major problem is bleeding.
         1. In newborn male: abnormal bleeding from umbilical cord, prolonged bleeding from circumcision site.
         2. In toddler male: excessive bruising, possible intracranial bleeding, prolonged bleeding from cuts or lacerations.
         3. General: hemarthrosis, petechiae, epistaxis, frank hemorrhage anywhere in body, anemia.
      2. Need to determine which clotting factor is deficient/missing and extent of deficiency:
         1. Mild: child has 5-50% of normal amount of clotting factor.
         2. Moderate: child has 1-5% of normal amount of clotting factor.
         3. Severe: child has less than 1% of normal amount of clotting factor.
      3. Definitive test: PTT
   3. Analysis/nursing diagnosis:
      1. Risk for injury related to bleeding tendencies.
      2. Pain related to hemarthrosis.
      3. Impaired physical mobility related to bleeding and pain.
      4. Knowledge deficit related to home care and follow - up.
   4. Nursing care plan/implementation:
      1. Goal: prevent injury and possible bleeding.
         1. Provide an environment that is as safe as possible, e.g., toys with no sharp edges, child’s safety scissors.
         2. Use soft toothbrush to prevent trauma to gums.
         3. When old enough to shave, use only electric razor (no straight - edge razors).
         4. Avoid IMs/IVs - but when absolutely necessary, treat as arterial puncture; that is, apply direct pressure to the site for at least 5 minutes after withdrawing needle.
         5. Do not use aspirin or medication containing aspirin (prolongs bleeding/clotting time).

5. 4. 2. Goal: control bleeding episodes when they occur.
         1. Local measures: apply direct pressure, elevate, apply ice (vasoconstriction), keep immobilized during acute bleeding episodes only. For epistaxis: child should sit up and lean slightly forward.
         2. Systemic measures: administer clotting antihemophilic factor (Factor VIII or DDVAP) via IV infusion. Note: These are blood products, so a transfusion reaction is possible.
         3. Note: cryoprecipitate (a blood product) is no longer used due to risk of transmission of hepatitis and HIV.
      3. Goal: prevent long-term disability related to joint degeneration.
         1. Keep immobilized during period of acute bleeding and for 24-48 hours afterward to allow blood to clot and to prevent dislodging the clot.
         2. Administer prescribed pain medications before physical therapy sessions.
         3. Begin prescribed exercise program, starting with passive range of motion (ROM) and gradually advancing to active ROM, then full exercise program, as tolerated, to maintain maximum joint function.
         4. Avoid: prolonged immobility, braces, splints - which can lead to permanent deformities and loss of mobility.
      4. Goal: promote independence in management of own care.
         1. Encourage child to assume responsibility for choosing safe activities.
         2. Encourage child to attend regular school as much as possible; provide support through school nurse.
         3. Advise child to wear Medic Alert bracelet.
         4. Caution parents to avoid overprotecting child.
         5. Offer child chance to self-limit activities within appropriate limits (parents can offer guidance).
         6. Assist child to cope with life-threatening disorder with no known cure.
      5. Goal: health teaching.
         1. Between 9 and 12 years of age: child can be taught to self-administer clotting factor IV (before this, family can perform).
         2. As child enters adolescence: begin to discuss issues such as realistic vocations, insurance coverage, genetic transmission.
   5. Evaluation/outcome criteria:
      1. Serious injuries are prevented; bleeding is kept to a minimum.
      2. Episodes of bleeding controlled by prompt, effective intervention.
      3. There are no long-term disabilities.
      4. Child is able to manage own care independently, with minimum supervision.