Sickle cell anemia

Alternative Names

Prevention

Sickle cell anemia can only result 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.

General health visits with a physician are recommended to ensure the patient is getting adequate nutrition, maintains proper activity levels, and receives proper vaccinations.

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:

1. Demanding physical activity, especially if the spleen is enlarged
2. Emotional stress
3. Environments with low oxygen content (high altitudes, non-pressurized airplane flights)
4. Known sources of infection

To promote proper hydration:

1. Recognize signs of dehydration
2. Avoid too much exposure to the sun
3. Provide access to fluids, both at home and away

To avoid sources of infection:

1. Keep child properly immunized as recommended by the health care provider
2. Consider having the child wear a Medic Alert Bracelet
3. Share above information with teachers and other caretakers as appropriate

Be aware of the effects that chronic, life-threatening illnesses can have on siblings, marital relationships, parents, and the child.

References

Goldman L, Ausiello D. Cecil Textbook of Medicine. 22nd ed. Philadelphia, Pa: WB Saunders; 2004:1030-1039

Hoffman R, Benz Jr. EJ, Shattil SJ, et al., eds. Hematology: Basic Principles and Practice. 4th ed. Philladelphia, Pa: Churchill Livingston; 2005:591-634.