SICKLE CELL DISEASE

Sickle cell disease is a worldwide health problem affecting many races, countries, and ethnic groups. Sickle cell disease is an inherited disorder that affects red blood cells. People with sickle cell disease have red blood cells that become hard and pointed instead of soft and round. Sickle cells cause anemia, pain and many other problems. Sickle cells block small blood vessels and prevent oxygen from getting to tissues and organs. Almost any system of the body can be affected and can be severely damaged.

Sickle cell is the most common disease passed through families. The defect can be passed from parents to their children even though the parent does not have the disease. Children must inherit the gene from both parents to inherit the full-blown disease. It is most common in people who are African, African-American, Mediterranean (Italian or Greek), middle Eastern, East Indian, Caribbean, and Central or South American. In the United States, one of every 12 African American newborns carry the sickle cell trait in his or her genes.

Sickle cell disease should be diagnosed as early as possible, preferably at birth. Most states in the United States require that newborns be screened for sickle cell disease while still in the hospital.

The signs and symptoms of the disease vary depending on the organ or body system involved. Pain is the most common symptom. Most children and adults with sickle cell disease have periods of no symptoms (called remissions) between crises. Crises are times when the abnormal red blood cells form clots.

Sickle cell crises are painful episodes that occur with varying frequency and severity in different individuals. They may start suddenly and last from a few days to several weeks. Sickle cell crises may be brought on by an illness, traveling to high altitudes, or situations that increase the body's demand for oxygen. Sometimes, a sickle cell crisis occurs for no apparent reason. Most people can manage these episodes at home. Some people have severe disease and may require frequent hospitalization.

Long-term effects include heart problems, lung damage, stroke, and kidney and liver disorders. Swollen joints, misshapen bones, and leg ulcers may develop. Affected persons are also more likely to have infections.

Treatment begins in infancy with antibiotics to prevent infections. If the condition worsens rapidly, the person receives transfusions of packed red blood cells. A severe crisis requires a hospital stay. During a painful crisis, the person should get bed rest in a sitting position and use warm compresses and blankets.

To help prevent future crises, conditions that lower blood oxygen should be avoided, such as:

strenuous exercise
cold
high altitude
tight clothing or
drugs that constrict blood vessels

It's important for people with sickle cell problems to have regular physical exams, eat a balanced diet with 5 to 9 daily servings of fruits and vegetables, and drink as much water as possible. Getting immunizations on time, good wound care, good dental care and a balanced diet help prevent infections. Recently, research on bone marrow transplants has shown promise as a potential treatment. Treatments for complications of sickle cell disease have prolonged the lives of many individuals who now live into adulthood.

Because sickle cell anemia is inherited, it can be prevented if couples who both carry the sickle cell trait gene do not have children. When both parents are carriers, each child has a 25 percent risk of having sickle cell anemia and a 50 percent risk of being a carrier. Screening programs are available to identify sickle cell trait carriers. If you are thinking about having a child and have sickle cell anemia or are a carrier, it is a good idea to seek genetic counseling.

For more information, contact the Sickle Cell Disease Association of America at (800) 421-8453 or on the Internet at www.sicklecelldisease.org.

Disclaimer: This content is reviewed periodically and is subject to change as new health information becomes available. The information provided is intended to be informative and educational and is not a replacement for professional medical evaluation, advice, diagnosis or treatment by a healthcare professional.

HIL File GENE4594.rf2 VRS# 4594 Data Version 7.0