9. Discuss the management and prognosis of sickle cell anemia.

Management:
- hydration, thorough evaluation for underlying causes (such as infection) and
aggressive analgesia administered by a standing order and/or patient-controlled
analgesia (PCA) pump.
- Morphine (0.1-0.15 mg/kg every 3-4h) should be used to control severe pain.
- Bone pain may respond as well to ketorolac (30-60 mg initial dose, then 15-30mg
every 6-8h).
- Inhalation of nitrous oxide can provide short-term pain relief, but great care must be
exercised to avoid hypoxia and respiratory depression.
- Nasal oxygen should be employed as appropriate to protect arterial saturation. Use
of blood transfusion should be reserved for extreme cases.
- Nonsteroidal anti-inflammatory agents are often effective for sickle cell arthropathy.
- If acute chest syndrome is present, hydration should be monitored carefully to avoid
the development of pulmonary edema, and oxygen therapy should be especially
vigorous for protection of arterial saturation.
- Hydroxyurea (10-30mg/kg per day increases fetal hemoglobin and may also exert
beneficial effects in the RBC hydration, vascular wall adherence, and suppression of
the granulocyte and reticulocyte counts.
- Hydroxyurea should be considered in patients experiencing repeated episodes for
acute chest syndrome.
- Agents blocking RBC dehydration or vascular adhesion, such as clotrimazole or
magnesium, may have value as an adjunct to hydroxyurea therapy.