Sickle-Cell Anemia

1. Sickle-Cell Anemia • Inherited defect in formation of Hgb • African American population mostly, but found in Caribbean, Arabs, Greeks, Maltese & Sicilians • RBC’s sickle (crescent shaped) • Diagnosed by chorionic villa sample @ birth

2. Assessments • Fever • Enlarged liver, spleen (eventually atrophies) • Pain - analgesics • Edema – need accurate I & O • Think: stasis of blood flow........ • Acute chest syndrome...leading cause of death – clumping of cells in lungs =↓gas exchange=hypoxia • See Nursing care plan pg 629 (Leifer, 2011)

3. Management • Pain relief - aggressive • Adequate hydration – oral & IV* • Oxygenation* • Transfusion (occasionally) • Avoid infection • Immunizations – • *Important*-flu & pneumococcal vaccine/ Hep A & B • No contact sports • High risk surgery-

4. Health Teaching • Avoid getting overtired • Extra stress & exposure to cold may lower resistance • Overheating may lead to dehydration • Avoid flying in unpressurized plane or exercising @ high altitudes because O2 concentration in their blood is already low • Oral penicillin prior to dental surgery or any invasive treatment

5. References Canadian Hemophilia Society. (2014). Educational material. Retrieved from http://www.hemophilia.ca/en/educational-material/ Leifer, G. (2011). Introduction to maternity & pediatric nursing. (6th ed.). St. Louis, MO: Elsevier. Sickle Cell Disease Association of Canada. (2013). Education. Retrieved from http://www.sicklecelldisease.ca/education/