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 min after withdrawing needle.
         5. Do not use aspirin or medication containing aspirin (prolongs bleeding/clotting time).