Sickle-cell disease medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]
Overview
Treatment
Febrile illness
Children with fever are screened for bacteremia i.e. complete blood count, reticulocyte count and blood culture taken. Younger children (varies from center to center) are admitted for intravenous antibiotics while older children with reassuring white cell counts are managed at home with oral antibiotics. Children with previous bacteremic episodes should be admitted.
Zn administration
Zinc is given as it stablises cell membrane.
Painful (vaso-occlusive) crises
Most people with sickle cell disease have intensely painful episodes called vaso-occlusive crises. The frequency, severity, and duration of these crises, however, vary tremendously. Painful crises are treated symptomatically with analgesics; pain management requires opioid administration at regular intervals until the crisis has settled. For milder crises a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen). For more severe crises most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting. Diphenhydramine is effective for the itching associated with the opioid use.
Acute chest crises
Management is similar to vaso-occlusive crises with the addition of antibiotics (usually a quinolone or macrolide, since wall-deficient ["atypical"] bacteria are thought to contribute to the syndrome),[1] oxygen supplementation for hypoxia, and close observation. Should the pulmonary infiltrate worsen or the oxygen requirements increase, simple blood transfusion or exchange transfusion is indicated. The latter involves the exchange of a significant portion of the patients red cell mass for normal red cells, which decreases the percent hemoglobin S in the patient's blood.
Hydroxyurea
The first approved drug for the causative treatment of sickle cell anemia, hydroxyurea, was shown to decrease the number and severity of attacks in a study in 1995 (Charache et al) [2] and shown to possibly increase survival time in a study in 2003 (Steinberg et al) [3]. This is achieved, in part, by reactivating fetal hemoglobin production in place of the hemoglobin S that causes sickle cell anemia. Hydroxyurea's clinical benefits can actually precede the induction of fetal hemoglobin, however. Hydroxyurea had previously been used as a chemotherapy agent, and there is some concern that long-term use may be harmful, but it is likely that the benefits outweigh the risks.
Contraindicated medications
Contraindicated medications
Sickle-cell disease is considered an absolute contraindication to the use of the following medications:
References
- ↑ Aldrich TK, Nagel RL. (1998). "Pulmonary Complications of Sickle Cell Disease.". In Bone RC et al., editors. Pulmonary and Critical Care Medicine (6th edition ed.). St. Louis: Mosby. pp. pp.1–10.
- ↑ Charache, Samuel (1995). "Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell anemia". NEJM. 332 (20): 1317&ndash, 1322. PMID 7715639. Retrieved 2007-04-15. Unknown parameter
|month=ignored (help); Unknown parameter|coauthors=ignored (help) - ↑ Steinberg, Martin H (2003). "Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment". JAMA. 289 (13): 1645&ndash, 1651. PMID 12672732. Retrieved 2007-04-15. Unknown parameter
|month=ignored (help); Unknown parameter|coauthors=ignored (help)