as of November 21, 2009
Sickle Cell Anemia Corner
OVERVIEW
Sickle cell anemia is a disease passed down through families in which red blood cells are an abnormal crescent shape. (Red blood cells are normally shaped like a disc.)
CAUSES/RISK FACTORS
Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen. Hemoglobin S, however, distorts the red blood cells' shape. The fragile, sickle-shaped cells deliver less oxygen to the body's tissues, and can break into pieces that disrupt blood flow.
Sickle cell anemia is inherited as an autosomal recessive trait. This means it occurs in someone who has inherited hemoglobin S from both parents. Sickle cell disease is much more common in certain ethnic groups, affecting approximately one out of every 500 African Americans. Someone who inherits hemoglobin S from one parent and normal hemoglobin (A) from the other parent will have sickle cell trait. Someone who inherits hemoglobin S from one parent and another type of abnormal hemoglobin from the other parent will have another form of sickle cell disease, such as thalassemia.
Although sickle cell disease is present at birth, symptoms usually don't occur until after 4 months of age. Sickle cell anemia may become life threatening. Blocked blood vessels and damaged organs can cause acute painful episodes, or "crises." There are several types of crises:
Hemolytic crisis occurs when damaged red blood cells break down
Splenic sequestration crisis is when the spleen enlarges and traps the blood cells
Aplastic crisis results when an infection causes the bone marrow to stop producing red blood cells
These painful crises, which occur in almost all patients at some point in their lives, can last hours to days, affecting the bones of the back, the long bones, and the chest. Some patients have one episode every few years, while others have many episodes per year. The crises can be severe enough to require admission to the hospital for pain control and intravenous fluids.
Repeated crises can cause damage to the kidneys, lungs, bones, eyes, and central nervous system.
SYMPTOMS
Common symptoms include:
-Attacks of abdominal pain
-Bone pain
-Breathlessness
-Delayed growth and puberty
-Fatigue
-Fever
-Jaundice
-Paleness
-Rapid heart rate
-Susceptibility to infections
-Ulcers on the lower legs (in adolescents and adults)
The patient may also have:
-Bloody urine (hematuria)
-Chest pain
-Excessive thirst
-Frequent urination
-Painful erection (priapism; this occurs in 10 - 40% of men with the disease)
-Poor eyesight/blindness
CARE/TREATMENT
Patients with sickle cell disease need continuous treatment, even when they are not having a painful crisis. Supplementation with folic acid, an essential element in producing red blood cells, is required because of the rapid red blood cell turnover.
The purpose of treatment is to manage and control symptoms, and to try to limit the frequency of crises.
During a sickle crisis, certain treatments may be necessary. Painful episodes are treated with analgesics and enough liquid intake. Treatment of pain is critical. Non-narcotic medications may be effective, but some patients will require narcotics.
Bone marrow transplants can cure the disease, but it is only recommended in a minority of patients. This is mostly due to the high risk of the procedure (the drugs needed to make the transplant possible are highly toxic) and the difficulty in finding suitable donors. Also, bone marrow transplants are much more expensive than other treatments.
PREVENTION
Sickle cell anemia can only result when two carriers with sickle cell trait have a child together. Therefore, genetic counseling is recommended for all carriers of sickle cell trait. About 1 in 12 African Americans has sickle cell trait. Diagnosis of sickle cell anemia during pregnancy is also available.
Prompt treatment of infections, adequate oxygenation, and preventing dehydration may prevent sickling of red blood cells. Antibiotics and vaccinations may prevent infections.
General health visits with a physician are recommended to ensure the patient is getting adequate nutrition, maintains proper activity levels, and receives proper vaccinations.
Real Stories, Expert Advice
Health Corner Videos: Sickle Cell Anemia
Curing Blood Diseases: How Cord Blood Saves Lives
Eight-year-old Joseph Davis Jr. is a healthy boy – but at birth he was diagnosed with sickle cell anemia, a chronic blood disorder that deprives cells of proper oxygen, causes periodic painful episodes and reduces life expectancy. Only a bone...
Resources
American Sickle Cell Anemia Association
National Heart, Lung and Blood Institute
Sickle Cell Disease Association of America
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