Sickle cell disease (SCD) is an
inherited blood disorder. This means it is passed down from a parent’s genes. It causes
the body to make abnormal hemoglobin. This is the protein in red blood cells that
carries oxygen to all parts of your body. When you have SCD, your body’s tissues and
organs don’t get enough oxygen.
Healthy red blood cells are round and move easily all over the body. With SCD, the red blood cells are hard and sticky. They are shaped like the letter C (and like a farm tool called a sickle). These damaged red blood cells (sickle cells) clump together. They can’t move easily through the blood vessels. They get stuck in small blood vessels and block blood flow. This blockage stops the movement of healthy oxygen-rich blood. This blockage can cause pain. It can also damage major organs.
Sickle cells die sooner than healthy cells. Normally the spleen helps filter infections out of the blood. But sickle cells get stuck in this filter and die. Having fewer healthy red blood cells causes anemia. The sickle cells can also damage the spleen. This puts you at greater risk for infections.
Sickle cell is an inherited disease caused by a defect in a gene.
Having a family history of sickle cell disease increases your risk for the disease. SCD mainly affects people whose families came from Africa, and Hispanics whose families are from the Caribbean. But the gene has also been found in people whose families are from the Middle East, India, Latin America, and Mediterranean countries. It has also been found in Native American Indians.
Each person’s symptoms may vary. They may be mild or severe. Symptoms may include:
The symptoms of SCD may look like other blood disorders or health problems. Always see your healthcare provider for a diagnosis.
Your healthcare provider will take
your health history and give you a physical exam. You may also have blood tests and
Many states routinely screen newborns for sickle cell. This allows treatment to begin as soon as possible. Early diagnosis and treatment can reduce the risk of problems.
A blood test called hemoglobin electrophoresis may be done. It can tell if you are a carrier of sickle cell. It can also tell if you have any of the diseases linked with the sickle cell gene.
Treatment will depend on your symptoms, age, and general health. It
will also depend on how severe the condition is.
Early diagnosis and preventing
further problems is critical in treating SCD. Treatment goals include preventing organ
damage (including strokes), preventing infection, and treating symptoms. Treatment may
SCD can affect any major organ. The liver, heart, kidneys, gallbladder, eyes, bones, and joints can all be damaged. They suffer damage from the abnormal function of the sickle cells and their inability to flow through the small blood vessels correctly. Problems may include:
SCD is an ongoing (chronic) condition. You may not be able to fully prevent the complications of this disease. But living a healthy lifestyle can reduce some of the problems. This includes doing things such as:
Stay away from things that may
trigger a crisis, such as:
Prevent infections by:
Tips to help you get the most from a visit to your healthcare provider:
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