Sickle Cell Anemia

Sickle cell anemia is a congenital hemolytic disease that results from a defective hemoglobin molecule (hemoglobin S) that causes red blood cells (RBCs) to become sickle shaped. Such cells impair circulation, resulting in chronic ill health (fatigue, dyspnea on exertion, swollen joints), periodic crises, long-term complications, and premature death.

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.

In the United States, sickle cell anemia occurs in about 1 in every 500 African-American children born and in 1 in every 1,000-1,400 Hispanic American children born.

Causes

Sickle cell anemia is cause by a genetic change in hemoglobin, the oxygen-carrying protein inside the red blood cells. This causes the cells to take on a sickle-shape and have a shorter life span, which can cause anemia. Sickle cells are also less flexible and stickier than normal red blood cells, and can become trapped in small blood vessels preventing blood flow and starving tissues and organs. This compromises the delivery of oxygen, which may result in damage to associated tissues and organs. The disease is often inherited.

Symptoms

There are many symptoms for sickle cell. Individuals with sickle cell may not experience all of these symptoms. The symptoms include: family history of sickle cell anemia, fatigue, breathlessness, rapid heart rate, delayed growth and puberty, susceptibility to infections ulcers on the lower legs (in adolescents and adults) jaundice, attacks of abdominal pain, weakness, joint pain, fever , vomiting, bloody (hematuria) urination, excessive thirst, excessive penis pain, priapism, chest pain and decreased fertility.

Symptoms may not appear until 4 months after birth and are most severe during an acute episode.

Diagnostic tests

Most states in the U.S. perform a simple blood test on all babies born to detect sickle cell anemia. If the test shows the abnormal hemoglobin is present, a second blood test is done to confirm the diagnosis.

Treatment

Although sickle cell anemia can't be cured, treatments can alleviate symptoms and prevent painful crises. Certain vaccines, such as polyvalent pneumococcal and Haemophilus influenzae B vaccine; anti-infectives, such as low-dose oral penicillin; and chelating agents, such as deferoxamine, can minimize complications resulting from the disease and from transfusion therapy.

Other medications, such as analgesics, may help to relieve the pain of vaso-occlusive crisis. Iron supplements may be given if folic acid levels are low. A good antisickling agent isn't yet available; the most commonly used drug, sodium cyanate, has many adverse effects.

Treatment begins before age 4 months with prophylactic penicillin. If the patient's hemoglobin level decreases suddenly or if his condition deteriorates rapidly, hospitalization is needed for transfusion of packed RBCs.

In an acute sequestration crisis, treatment may include sedation and administration of analgesics, blood transfusion, oxygen therapy, and large amounts of oral or I.V. fluids. Despite the effectiveness of transfusions, some clinicians limit them because they increase blood viscosity and the risk of vascular occlusion.

Prevention

With the right precautions, teens with sickle cell disease can do most of the stuff other teens do. To stay as healthy as possible, people with sickle cell anemia should take these steps:

• Eat a balanced, healthy diet.
• Take vitamins, including folic acid supplements, as prescribed.
• Avoid places low in oxygen. (For example, it's not a good idea to go hiking at high altitudes or spend lots of time swimming under water.)
• Drink plenty of water to prevent dehydration.
• Get plenty of rest.
• Avoid alcohol , drugs , and smoking , which can aggravate sickle cell disease and its symptoms. Some people with sickle cell disease are prone to lung problems, so smoking is particularly risky.

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