Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease
Sickle cell anemia can result only when two carriers with sickle cell trait have a child together. Therefore, genetic counseling is recommended for all carriers of sickle cell trait. About 1 in 12 African-Americans has sickle cell trait. Diagnosis of sickle cell anemia during pregnancy is also available.
Prompt treatment of infections, adequate oxygenation, and preventing dehydration may prevent sickling of red blood cells. Antibiotics and vaccinations may prevent infections.
The patient should have regular physical exams every 3 - 6 months to ensure the patient is getting adequate nutrition, maintains proper activity levels, and receives proper vaccinations. Regular eye exams are also recommended.
PREVENTING INFECTIONS
PREVENTING CRISES
Parents whose children have sickle cell disease should encourage their children to lead normal lives. However, in order to decrease the occurrence of sickle cell crises, consider the following precautions:
To prevent loss of oxygen from tissues, avoid the following:
To promote proper hydration:
To avoid sources of infection:
Be aware of the effects that chronic, life-threatening illnesses can have on siblings, marital relationships, parents, and the child.
Lee MT, Piomelli S, Granger S, et al. Stroke Prevention Trial in Sickle Cell Anemia (STOP): extended follow-up and final results. Blood. Aug 1, 2006;108(3):847-52.
Mehta SR, Afenyi-Annan A, Byrns PJ, Lottenberg R. Opportunities to improve outcomes in sickle cell disease. Am Fam Physician. 2006 Jul;15;74(2):303-10.
Saunthararajah Y, Vichinsky EP, Embury SH. Sickle cell disease. In: Hoffman R, Benz Jr. EJ, Shattil SJ, Furie B, Cohen HJ, eds. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa: Churchill Livingston;2005;chap 37.
U.S. Preventive Services Task Force. Screening for Sickle Cell Disease in Newborns: U.S. Preventive Services Task Force Recommendation Statement. Agency for Healthcare Research and Quality, Rockville, MD. Sep 2007:AHRQ Publication No. 07-05104-EF-2.