Sickle cell disease


Sickle cell disease

Description, causes and Risk Factors:

Alternative Names: Sickle cell anemia, drepanocytic anemia, vasoocclusive crisis.

ICD-9-CM: 282.60.

Abbreviation: SCD.

Sickle cell disease is an inherited disorder in which red blood cells (RBCs) are abnormally shaped. This abnormality can result in painful episodes, serious infections, chronic anemia, and damage to body organs. This is an inherited disorder that leads to the production of hemoglobin S (Hb S or Hgb S), an abnormal hemoglobin variant.

These complications can, however, vary from person to person depending on the type of sickle cell disease each has. Some people are relatively healthy and others are hospitalized frequently.

The different forms of sickle cell disease are determined by the genes inherited from the person's parents. Someone who inherits a sickle cell gene from each parent has hemoglobin SS disease, also called sickle cell anemia.

A person can also inherit a sickle cell gene from one parent and a different kind of abnormal gene from the other and end up with a different form of sickle cell disease, such as hemoglobin SC disease or hemoglobin S beta thalassemia. Someone who inherits only one sickle cell gene and a normal gene from the other parent will have the sickle cell trait, but not the disease.

Sickle cell anemia is common among people whose ancestors come from sub-Saharan Africa, Spanish speaking regions of the world (South America, Cuba, and Central America), Saudi Arabia, India, and Mediterranean countries such as Turkey, Sicily, Greece, and Italy.

Once thought to be a rare disease, research now shows that in the United States, sickle cell anemia occurs in about 1 in every 500 African-American children born and in 1 in every 900 Hispanic American children born. In the United States, hemoglobin SS disease (sickle cell anemia) affects mostly African Americans. However, forms of sickle cell disease may occur in people with different ethnic backgrounds, such as those whose ancestors came from Mediterranean countries (including Turkey, Greece, and Italy), East India, or Middle Eastern countries.

Symptoms:

Symptoms include pain, anemia, eye problem, delayed growth, stroke, infections, and acute chest syndrome.

Diagnosis:

Sickle cell disease usually is diagnosed at birth with a blood test during routine newborn screening tests. If a child tests positive on the screening test, a second blood test called hemoglobin electrophoresis should be performed to confirm the diagnosis.

Because kids with sickle cell disease are at an increased risk of infection and other health complications, early diagnosis and treatment to prevent problems is important.

Treatment:

Pain from sickle cell anemia is treated with pain-killing drugs and intravenous fluids. Most complications are treated as they occur. The anticancer drug hydroxyurea can reduce the frequency of painful sickle cell crises and of acute chest syndrome. Droxia, the prescription form of hydroxyurea, was approved by the FDA in 1998 and is now available for adult patients with sickle cell anemia.

Bone marrow transplant is the only known cure for sickle cell disease. Transplants are complex and risky procedures and currently are an option only for a carefully selected subset of patients with severe complications.

To be eligible, a child would need bone marrow or stem cells from a "matched" donor with a low risk of rejection. Even then, the procedure has significant risks and there's always the chance of rejection of the transplanted marrow.

But even without a cure, kids with sickle cell disease can lead relatively normal lives. Medicines are available to help manage the pain, and immunizations and daily doses of penicillin (an antibiotic) can help prevent infection.

Most kids will require two daily doses of penicillin, as prescribed by their doctors, until they're at least 5 years old (and often older). They also should be fully immunized with the regular childhood vaccinations, as well as the pneumococcal vaccine and influenza and meningococcal vaccines. Folic acid supplements can help them continue to produce new RBCs.

Your child should receive regular care from your primary care doctor as well as a hematologist (a blood specialist) or a sickle cell specialty clinic. It's important to share your concerns and discuss any new symptoms or complications with your child's health care team.

Your child also should drink lots of fluids, get plenty of rest, and avoid extreme temperatures.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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