Questions & Answers
1. What causes the pain in sickle cell disease?
The hallmark of sickle cell disease is the sickle cell crisis, which is an episode of pain due to decreased blood flow and oxygen to a certain area of the body. Pain most commonly occurs in the lower back, leg, hip, abdomen or chest, often in two or more locations. Episodes usually recur in the same areas.
2. Can a woman with sickle cell disease have a healthy pregnancy?
Yes. However, many women with sickle cell disease experience more frequent pain crises and other sickle cell-related complications during pregnancy. The increased frequency of crises, particularly during the latter half of pregnancy, likely results from heightened metabolic requirements in pregnancy, decreased blood flow and the increased tendency for blood to clot during pregnancy.
3. Will taking iron supplements help a person who has sickle cell disease?
Not necessarily. Patients with sickle cell disease who receive multiple blood transfusions are cautioned against taking iron even though they have anemia because they are at risk for storing too much iron and developing iron overload. Patients should take daily folic acid and vitamin B-12 and B-6 supplements. Vitamin B-6 may have specific anti-sickling properties.
4. Is sickle cell disease curable?
Sickle cell disease is not curable at the moment. Bone marrow transplantation is a potential cure but remains an experimental treatment at this point.
5. Can people with sickle cell disease play competitive sports?
It depends. One generally accepted precaution in the prevention of pain crises is to avoid excessive demands on the body that would increase oxygen needs, such as physical overexertion and stress. Patients should consult their doctor about any specific exercise or sports program.
6. Are there precautions that people with sickle cell disease should follow?
Although it is not possible to prevent sickle cell crises or long-term complications of sickle cell disease, advances in treatment coupled with taking certain precautions have allowed patients to have a longer, better-quality life.
Examples are:
• Have regular physical examinations every two to 12 months depending on age.
• Get yearly eye examinations and flu shots.
• Get sufficient rest and fluids, and avoid extremes in temperature to reduce oxygen loss and the risk for dehydration.
• Avoid conditions or activities that demand increased need for oxygen, such as competitive sports, physical overexertion or even stress. Exercise is recommended, but know your limits. Low impact exercises, such as leg lifts, that increase or maintain strength are helpful. It is wise to consult your doctor before starting any exercise program.
• Avoid high altitudes if possible. When flying make sure that the airline can provide oxygen.
• Do not smoke and avoid exposure to second-hand smoke. Both active and passive smoking may promote acute chest syndrome in patients with sickle cell disease.
7. If a person with sickle cell disease has a baby with a person with normal red blood cells, is it possible to pass the disease to their offspring?
No. Each child born to those parents will have sickle cell trait and will be a “carrier” of sickle cell disease. People inherit a pair of genes — one from each parent — that regulate hemoglobin, a protein that is responsible for transporting oxygen in red blood cells. If two sickle cell genes are inherited, a person will have sickle cell disease. If one normal hemoglobin gene and one sickle cell gene are inherited, a person will have sickle cell trait. People with the trait are healthy and do not develop sickle cell disease, but they are can pass their defective gene on to their children.
8. Why are people with sickle cell disease prone to infections?
The sickled red blood cells tend to stick together and slow the blood flow to certain areas. The decreased blood flow decreases the oxygen content, which provides a more supportive environment in which bacterial and viruses can thrive.
9. Can sickle cell trait become sickle cell disease?
No. People who inherit just a single gene are referred to as having the sickle cell trait. The trait cannot transform into sickle cell disease.
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Lisa Michelle Owens, M.D.
Medical Director
Brigham Primary Physicians at
Faulkner Hospital
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