Information From Your Health Care Provider

SICKLE CELL ANEMIA & SICKLE CELL TRAIT

Basic information

description

Sickle cell anemia is an inherited blood disorder. It causes chronic anemia, periods of severe pain, high risk of infections, and general poor health. It usually begins at about 6 months of age and is a lifelong disorder. People with the sickle cell trait usually have no symptoms of the disorder, but may pass it onto their children.

FREQUENT SIGNS & SYMPTOMS

Swollen hands or feet, fever, and paleness.
Shortness of breath; rapid heartbeat; fatigue.
Painful episodes (crises) in a joint or an organ that can last hours or days.
Frequent infections (e.g., pneumonia). Eye problems.
Delayed growth and development in children.
Jaundice (yellow skin and/or eyes).

causes

A gene that causes defective hemoglobin (blood cells). The gene occurs mostly in African Americans (about 8% are carriers) and some Hispanics. Defective hemoglobin changes blood cells from smooth and round to a stiff, sickle shape. These abnormal cells cause a blockage of blood flow in blood vessels. This leads to the symptoms and sickle crisis. A chronic short supply of red blood cells causes anemia.

risk increases with

Family history of sickle cell anemia. If both parents have the gene, their child may have sickle cell anemia. If one parent has the gene, the child will not get the disorder, but may carry the sickle cell trait.

PREVENTIVE MEASURES

If you have a family history of sickle cell anemia, ask for testing. If the condition is present, obtain genetic counseling before starting a family.
Early pregnancy test to see if the baby has inherited the double-dose gene (both parents are carriers).

expected outcomes

There is no cure for sickle cell anemia. Life span is reduced, but has gradually increased to over 40 years. More effective treatments are helping.

possible complications

Infections, other medical problems, stroke, and death.

diagnosis & treatment

general measures

Blood tests can diagnose sickle cell anemia. Screening tests are done on newborns in most states. If the screening test shows sickle hemoglobin, a second test is done to confirm diagnosis.
A health care provider with special knowledge of this disorder may be recommended for ongoing health care.
Home treatment involves good nutrition and hygiene, rest as needed, and steps to avoid infection and stress. Encourage a child to lead as normal a life as possible.
Some things may worsen symptoms. These include an injury or infection, pregnancy, surgery, dehydration, traveling to high altitudes (as in driving up a mountain, or an airplane trip).
See your health care and dental care providers on a regular basis. Keep all vaccines and flu shots up to date.
Wear a medical alert type bracelet or pendant to identify the medical disorder.
Counseling may be helpful in adapting to this condition, especially for children. Support groups may help.
Hospital care may be required at times of severe attacks for intravenous (IV) therapy and oxygen therapy and, sometimes, blood transfusions.
Bone marrow transplants may be an option for some.
To learn more: Sickle Cell Disease Association of America, 231 East Baltimore St., Ste. 800, Baltimore, MD 21202; (800) 421-8453; website: www.sicklecelldisease.org.

medications

No drugs are effective for the disease. Drugs are prescribed to treat symptoms and prevent complications.
Use nonsteroidal anti-inflammatory drugs or acetaminophen for pain.
Penicillin started in infancy helps prevent infection.
Adults should be treated promptly for infections.
Hydroxyurea helps to reduce the frequency of painful crisis. It may be prescribed for certain patients.
Daily vitamin supplements are often recommended.

activity

Avoid strenuous exercise. Avoid being out in very hot or cold temperatures. Rest in bed during acute attacks.
Activity may be somewhat limited due to chronic anemia and poor muscular development.

diet

Eat a healthy diet. Drink plenty of fluids.

notify our office if

After diagnosis, any signs of infection occur, pain increases, or symptoms develop that cause concern.
You want to know if you have the sickle cell gene.