What is Sickle Cell Disease?

Sickle cell disease is an inherited blood disorder that affects the red blood cells which can cause bouts of pain, damage to vital organs. The effects of sickle cell vary greatly from one person to another and from one time to another in the same person. Most persons with sickle cell disease enjoy reasonable good health most of the time.

Oxygen carrying blood cells are normally round and flexible. Under certain conditions, the red blood cells of a person with sickle cell anemia may change into crescent or sickle-shaped and have trouble passing through the small blood vessels. Sickle-shaped cells tend to become trapped in the spleen and elsewhere and are destroyed. This results in a shortage of red blood which when severe can cause the patient to be pale, short of breath and easily tired. Patients may be prone to certain infections, which can worsen their condition and their physical growth and development are often slower than normal.

Sometimes certain conditions may worsen a patient’s anemia. This can happen when the red blood cells are either destroyed or not produced in sufficient amounts, or are removed from circulation. Infections and other problems can speed up the destruction of red blood cells. A tell-tale sign of this is the yellowing of the whites of patients’ eyes. Reduced red blood cell production can be caused by a viral infection or a vitamin deficiency, especially during pregnancy. Large numbers of red blood cells may be trapped by the spleen. This occurs primarily in young children.

What is Sickle Cell Trait?

Sickle cell trait is an inherited carrier state affecting the red blood cells. Both hemoglobin A and S are produced in the red blood cells. Some of the red blood cells are distorted in shape, but, as a rule, anemia and symptoms are absent and persons with the trait require no treatment. Sickle cell trait is not a disease and persons with sickle cell trait are generally as healthy as non-carriers, rarely having any health problems related to the trait. They cannot later develop sickle cell disease. However, when two persons who both have trait have a child, their child may inherit two sickle cell genes and develop the disease.

What is Sickle Cell Crisis?

At times sickle cells become stuck in tiny blood vessels. When other red blood cells pile up behind this jam, they lose oxygen and this causes them to sickle and the vessels become totally blocked. These sickling crises usually occur in bones and organs of abdomen or chest. They deprive the tissues of oxygen. They are often very painful and if long-lasting, can destroy areas of tissue. Crises damage the vital organs such as the brain, lungs, and kidneys and may lead to disability or even death.

Can I Catch Sickle Cell From Anyone Who Has It?

No, the disease is inherited and is not contagious. To develop the disease a child must inherit two sickle cell genes – one from each parent who “carries” a single sickle cell gene.

Do We All Have The Same Chance of Inheriting Sickle Cell Disease?

No, in the United States, most cases of sickle cell disease occur among blacks and Hispanics and people of Caribbean ancestry. About one in every 400 to 600 blacks and one in every 1000 to 1500 Hispanics inherit sickle cell disease. The disease also affects some people of Arabian, Greek, Maltese, Sicilian, Sardinian, Turkish and Southern Asian ancestry.

Are There Other Forms Of Sickle Cell Disease?

Some people have a combination of one sickle cell gene plus any one of several other abnormal genes that can affect red blood cells. These combinations can cause anemia and sickling, but in general they are less troublesome than sickle cell anemia.

The most common types are: Sickle cell anemia (SS) Sickle-Hemoglobin C Disease (SC) Sickle Beta-Plus Thalassemia Sickle Beta-Zero Thalassemia

What Are The Chances That Parents With Sickle Cell Trait Will Pass It On To Their Children?

The chances are two in four that each child of a couple who both carry a sickle cell gene will have the trait like their parents. There is a one in four chance that the child will develop sickle cell disease. There is also a one in four chance that the child will have neither the trait nor the disease.