1. 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.
      2. 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.
      3. 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.
      4. 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.
      5. 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).
      6. 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.
    2. 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.