Sickle cell anemia

• A
• B
• C
• D
• E
• F
• G
• H
• I
• J
• K
• L
• M
• N
• O
• P
• Q
• R
• S
• T
• U
• V
• W
• X
• Y
• Z

Blood cells	Formed elements of blood	Red blood cells, multiple sickle cells	Red blood cells, normal
Red blood cells, sickle and pappenheimer	Red blood cells, sickle cell	Red blood cells, sickle cells

Anemia - sickle cell; Hemoglobin SS disease (Hb SS); Sickle cell disease

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 ).

Prenatal diagnosis of sickle cell anemia 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, maintaining proper activity levels, and receiving proper vaccinations.

PREVENTING CRISES

Parents whose children have sickle cell 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 tissue deoxygenation, avoid the following:

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

Review Date: 04/13/2005
Reviewed By: Reviewed By: A.D.A.M. editorial. Previously reviewed by Jacqueline A. Hart, M.D., Department of Internal Medicine, Newton-Wellesley Hospital, Harvard University