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

What makes sickle cell bad ? The problem with people with sickle cell disease is this; they have abnormally shaped/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 and leads in part to the mortality. 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 are precipitated when there is a decrease in oxygen to a body part. The most common crises 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 usually 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. 

As we stated before over time almost every organ has been described as being damaged in a person with sickle cell disease. This can be as varied as 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 can be very rewarding.

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

James Reilly M.D. of Adoptiondoctors.com. He is a General Pediatrician and works at RBKPediatrics.com

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