Physician: Sickle Cell Disease

What you should know.

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What do I, as an adoptive parent, need to know about sickle cell disease? Is it a serious condition, and can it be managed safely here in the USA?

Sickle cell anemia is a genetic disease that causes abnormal red blood cells.  In the US, the frequency of sickle cell anemia among African Americans is about 1 in every 400 births. One of every twelve African American newborns carries the sickle cell trait in his or her genes. Therefore, we have a lot of experience with sickle cell disease.

Children with sickle cell disease can vary from being very sick and in the hospital all the time to hardly ever sick.

Sickle cell disease definitely can affect the longevity of the affected person. Lifespan depends on associated factors which are impossible to predict without further information about the child.

What makes sickle cell bad? People with sickle cell disease have abnormally shaped and abnormally functioning red blood cells (RBC’s). These RBC’s are what our bodies use to transport oxygen from the lungs to the rest of our body. When these cells do not function, it causes almost every organ in the body to have low supplies of oxygen, which cause the organs to malfunction and to accumulate damage, which leads in part to high mortality among people with the disease. It also leads to a poorer immune system, predisposing the person to severe infections.

People with sickle cell disease are prone to attacks known as crises. These crises come in many forms. What they have in common is that they are precipitated when there is a decrease in oxygen to a body part. The most common crisis is a pain crisis where the individual has pain, usually in an extremity from lack of oxygen.

Persons afflicted with sickle cell disease are not necessarily always in pain or incredibly sick all the time. They have “remissions” where they can appear asymptomatic. They usually have episodes of pain or other crises during a viral illness that they will not be able to cope with the way other children do.

Over time almost every organ has been described as being damaged in a person with sickle cell disease. This damage manifests in varied ways, including an increased chance of having strokes, heart disease, ulcers kidney disease, and gallstones.

People with sickle cell anemia are more likely to have serious infections ranging from flu to pneumonia. Prevention of serious infections is an important part of the treatment in sickle cell patients because infections cause a crisis.

There is a cure for sickle cell disease, and that is bone marrow transplantation. This is a very technically difficult procedure with a high mortality rate even if a compatible donor can be found and the child is relatively healthy. Some patients are managed effectively with frequent blood transfusions, but these have substantial risks. One of these risks is from known and unknown viruses. Physicians manage most patients with medicines and increased observation.

In summary, sickle cell disease is a horrible genetic abnormality, which often leads to significant decrease in quality of life and in the length of life. Caring for a child with this condition can be challenging and should not be gone into lightly. But, as with any adoption, it can be very rewarding.

For more information:
Sickle Cell Disease Association of America
(800) 421-8453

by James Reilly, M.D.

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