General Adult Health Information
Sickle Cell Disease
This information is about Sickle Cell Disease. You'll find out what Sickle Cell Disease is, how it's treated, and resources your family can use.
Sickle Cell Disease affects about 50,000 people in the United States and there are about 1,500 new infants born with this disease each year. Most, but not all, of the patients with Sickle Cell Disease are black.
In Sickle Cell Disease, the oxygen- carrying part of the red blood cells, or hemoglobin, is defective. This causes the red cells to be deformed and take on a so-called sickle shape. These sickle-shaped red cells get caught in the small blood vessels of various organs causing the symptoms of Sickle Cell Disease.
Children with Sickle Cell Disease may have many problems related to anemia, infection, and blocking of the blood vessels causing damage or death of the surrounding tissues. This last condition is commonly called "sickle cell crisis."
In addition to experiencing the symptoms of anemia, paleness, weakness, shortness of breath and increased heart beat, the unique symptoms of Sickle Cell Disease include pain in the bones and abdomen especially during a sickle cell crisis.
Sickle Cell Disease varies from person to person depending on the specific type of hemoglobin the individual inherits. The disease results from inheriting one gene for sickle hemoglobin from one parent and another gene for either sickle hemoglobin or another abnormal hemoglobin from the other parent. The most severe type occurs when a child inherits sickle hemoglobin from both parents.
During the first years of life, severe infections may result in pneumonia, blood infections, meningitis, or bone infections. These conditions may even result in death if not medically attended to rapidly.
Very young children, usually less than five years of age, are commonly treated with daily low dose penicillin to prevent a number of these infections that may have killed children in the past.
In adolescence and early adult life, there tends to be less infections and more sickling episodes. These episodes cause either painful "crises" or changes in various organs that may result in pulmonary disease, kidney problems, strokes, arthritis or even heart failure.
Sickle Cell Disease is not curable, but the symptoms can be managed. When close medical care is provided to the child or teen, along with family training and vigilance on the part of the parents, most individuals with Sickle Cell Disease may lead normal lifestyles.
Please remember these key points: Sickle Cell Disease can cause severe illness in young children through teens if left untreated. Adults suffer more often from sickling episodes that are painful, and can cause changes in various organs. Sickle Cell Disease can be managed and most individuals can participate in normal lifestyle activities.
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