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What is sickle cell anemia?
Q: My sister’s 4-week-old baby was diagnosed with sickle cell anemia. My sister and her husband are healthy, so how could she have gotten this? I’m planning to take care of her when my sister goes back to work. What do I need to know about sickle cell?
Shanika Miami
A: Shanika, sickle cell anemia (also known as sickle cell disease) is an abnormality of the hemoglobin proteins in the baby’s red blood cells. This causes the red blood cells to be stiffer and more fragile than usual. As a result, the red blood cells break down more frequently, which can cause anemia or low red blood cell count. The blood cells can also get stuck in the small blood vessels in different parts of the body, which can cause painful crises and other medical problems.

Sickle cell disease is a genetic or inherited condition. It’s most common in people of African descent, but is also seen in people of Indian, Middle Eastern, Mediterranean, Latin American and other backgrounds. Your niece inherited the sickle cell genes from both her mother and father. Each parent has two copies of a gene and passes down one of them to the baby. If both her parents are healthy, it means that each parent probably has a normal hemoglobin gene and a sickle gene, also known as a sickle cell trait. Each of them handed down their copy of the sickle cell gene to the baby, so she got two abnormal hemoglobin genes.

If they have another baby, there’s a 25 percent chance that the baby will have sickle cell disease, and a 75 percent chance that the baby will be healthy.

Babies with sickle cell disease are usually healthy for the first few months of life. Then the symptoms of anemia and circulation problems can begin. The sickle cell crises are more likely to occur when the baby has an illness, a fever or dehydration. Babies can develop episodes of painful and swollen hands and feet or severe abdominal pain. They can also get an enlarged and damaged spleen, which makes them more vulnerable to catching infections. Children with sickle cell are also susceptible to pneumonia or lung infection, stroke, slow growth and other medical problems.

The key to keeping your niece as healthy as possible is to have close medical care with a doctor skilled in treating children with sickle cell. Some regions have a local medical centre that provides comprehensive care for children with sickle cell. Many illnesses can be prevented by making sure your niece gets her immunizations on time. Babies with sickle cell are also prescoted daily penicillin to prevent common bacterial infections, vitamins to help regenerate blood cells and sometimes other medications to help prevent sickling crises.

To prepare for caring for your niece, you might want to attend one of her medical visits. Be sure to ask the doctor for written instructions about caring for the baby and preventing sickle cell complications. This might include instructions about keeping the baby well hydrated, learning to recognize the signs of possible medical emergencies (fever, illness or pain) and when you need to contact your sister immediately and take the baby to the emergency room.

Karen Sokal-Gutierrez M.D., M.P.H. Pediatrician