- Course objectives .
- Definition .
- Epidemiology .
- Pathology .
- Clinical presentation.
Sickle Cell Disease:
- A genetic hemoglobinopathy
- Results in deformation, increased rigidity, and destruction of RBCs
- Chronic multiorgan system disease
- Severe morbidity and early mortality
- 200,000 children born yearly in Africa with Sickle Cell Disease
- Areas of Prevalence
- Seen commonly irn sub-Saharan Africa but also occur in the Mediterranean, India, and the Arabian Peninsula
- Corresponds to the distribution of malaria
- Highest rates in Ghana, Nigeria, Uganda In Uganda,
- Highest rates in Bundibugyo, in the west.
Sickling Mechanism De-oxygenation of Hgb S results in polymers which precipitate in RBCs as long, rigid fibers resulting into RBCs taking on a sickle shape.
Chronic organ damage
- • Gall bladder: Cholelithiasis due to bilirubin gallstones
- • Lungs: Pulmonary venous hypertension
- • Eyes: Retinopathy
- • Kidney: Renal failure
- • Spleen: Autosplenectomy
- • Extremities: Chronic leg ulcerations
- • Heart: Myocardial infarction and cardiomyopathy
Maintenance of health
- • Prevent infection
- — Antimalarial prophylaxis, ITN
- — Daily oral Penicillin V, monthly parenteral benzathine penicillin
- — Vaccinate against S. pnuemoniae and H. influenza
- • Treat pain
- — Provide paracetamol for home use
• Supplement nutrition
— Daily folate and zinc supplementation
• Prevent painful crises — Hydroxyurea reduces frequency of painful crises and Improves quality of life.
• Family and patient education
• Join sickle-cell societies