Sickle cell disease is an inherited blood disorder found prominently in people of African descent, though it can occur in anyone. In this disease, the hemoglobin in red blood cells is deformed, resulting in characteristic sickle-shaped cells that get stuck in blood vessels and cannot carry sufficient oxygen to tissues. The disease is also called sickle cell anemia, a reflection of the primary symptom of the illness. The malformed red blood cells are created in the bone marrow, so the only cure is a bone marrow transplant.
Anemia
The deformed red blood cells of sickle cell patients last only 10 to 20 days in the body instead of the normal 120 days or so. This results in severe anemia. Individuals suffering from anemia may report feeling extremely tired.
Pain Crises
Pain crises are regular incidents of extreme pain, generally in the lungs, abdomen, bones or joints. The pain occurs when sickle cells form clumps that get caught in blood vessels. Patients with sickle cell anemia experience pain crises that may vary in both intensity and frequency; the most extreme forms are chronic pain that can last for months or acute bouts of pain up to 15 times yearly.
Jaundice
Because the liver can't keep up with the breakdown and disposal of the malformed red blood cells present in sickle cell anemia, the skin and the whites of the eyes become yellow.
Swollen Hands and Feet
In many sickle cell patients, the hands and feet swell if the blood vessels that lead to those extremities are blocked. This is also called hand-foot syndrome and is frequently the first visible symptom in babies who have inherited the disease.
Vision Problems
If the blood vessels leading to the eyes become clogged by sickle cells, vision problems can be the result. The retina can become damaged and eventually destroyed if the blood vessels serving it cannot get enough red blood cells through to the tissues because they are clogged with sickle cells.
Spleen Shrinkage
A shrinking of the spleen, also termed a splenic crisis, can occur when the spleen, which normally filters abnormal blood cells out of the blood and fights infections, becomes clogged with sickle cells. The affected spleen shrivels and stops working.
Frequent Infections
Because sickle cell anemia takes a heavy toll on the spleen, frequent infections are common in those who have the disease. Babies are particularly affected, since their immune system is still developing. Infections common in sickle cell patients include pneumonia, meningitis, influenza and hepatitis.
Delayed Growth
In children, the presence of too few working red blood cells can result in slow growth. It can also cause a delay in puberty among adolescent sufferers. Adults who have sickle cell disease may be smaller than normal due to the delays in growth during childhood and adolescence.
