Sickle cell anemia is an inherited blood disorder most common among those of Hispanic, African, Middle Eastern, or Mediterranean descent. It is passed to children when both parents have the sickle cell trait: a single abnormal sickle gene. Though a single gene does not produce sickle cell anemia, inheriting a sickle cell gene from each parent results in the disorder.
Sickle cell anemia affects red blood cells responsible for distributing oxygen throughout the body via a protein called hemoglobin (HbA). Normal, healthy red blood cells are rounded and soft, moving easily through the blood vessels. People with sickle cell anemia have misshapen hemoglobin (HbS) that causes the red blood cells to become crescent-shaped, or sickle-like. HbS also causes cells to become rigid and 'sticky.'
This combination of factors causes abnormal sickle cells to "hang up" on one another, or cluster together. Vessels then become clogged, reducing blood flow, which results in lowered oxygen levels in body tissues and organs. This is not only unhealthy, but can be painful. Additionally, sickle cells break down in just 10-20 days, compared to healthy cells that live about 140 days. This accelerated loss of red blood cells can lead to anemia, hence the full name of the disorder: sickle cell anemia.
Sickle cell anemia is not contagious or infectious, and can only be inherited. Since persons carrying the sickle trait will not know unless tested, couples at risk are urged to see a doctor before having children. If both parents carry the sickle gene, children can receive proper treatment from birth. This can be vital since sickle cell anemia can go undiagnosed or misdiagnosed for years; it's symptoms are often sporadic or similar to those of other diseases.
Personal symptoms of sickle cell anemia vary widely and can be mild or severe, with some sufferers requiring frequent hospitalization. Anemia in general causes fatigue and shortness of breath, and can affect a child's rate of development. Sickle cell anemia might also cause jaundice, or yellowing of the eyes and skin from the accelerated breakdown of red blood cells. High blood pressure and pulmonary hypertension are often related side effects.
Sickle cell anemia is a serious disorder that affects millions of people worldwide, including a reported 75,000 or so Americans. Though there is currently no cure, bone marrow transplants are sometimes successful. Effective management of sickle cell anemia has also improved over recent years.
This article provides general information about sickle cell anemia but is not medical advice or a replacement for a medical diagnosis. If you or someone you know feels unwell or suspects a medical problem, see a doctor for professional care.
