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Sickle Cell Anemia – What to Expect

Sickle cell anemia is an inherited disease of the red blood cells that affects people from African, Mediterranean and Middle Eastern countries. In the United States, about 1,000 babies are born with the disorder each year, and 1 in 12 African-Americans possess the sickle cell trait.

How it occurs: When a person is born with one sickle cell gene from each parent, they may have a deficiency in the production of normal hemoglobin, a protein in red blood cells that carries oxygen throughout the body. With more abnormal hemoglobin, the red blood cells change from being flexible and disk-shaped to curving into a jagged crescent that resembles a sickle. Normal red blood cells can travel easily through the bloodstream, but sickle-shaped cells clump together and clog the blood vessels, affecting the delivery of oxygen and threatening the tissue in the body’s organs.

Symptoms: Some symptoms of sickle cell anemia include:

o Hypoxia: The heart must work harder to pump anemic blood, causing a rapid heart rate, fatigue, weakness, dizziness, and other complications. Hypoxia can lead not only to a risk of heart failure, but to more sickling of the blood because of further oxygen shortages.

o Jaundice: When red blood cells break down too rapidly, the liver cannot dispose of their waste products. The buildup in the bloodstream can cause skin, mucous membranes and the whites of the eyes to take on a yellowish tint. Permanent liver damage may occur.

o Pain crises: The common first sign of the disease in infants is hand-foot syndrome, causing pain and swelling in those organs. Pain can occur at all ages in the arms, legs, hips, shoulders, back, muscles, and joints, and can vary in frequency. Some episodes last a few hours while others can continue for weeks. Acute chest syndrome results in severe chest and abdominal pain as well as fever, cough, and difficulty breathing.

o Increased risk of infection: Damage to the spleen caused by a buildup of red blood cells can lower a person’s resistance to viral infections, respiratory infections such as pneumonia, and osteomyelitis, which affects the bones.

o Stunted growth and vision problems: Some children may be underweight, have short trunks, and a delay in reaching puberty. Sickle cells building up in the blood vessels to the eyes can lead to blindness.

Diagnosis and treatment: A blood test known as hemoglobin electrophoresis, performed on newborns in more than 40 US states, can detect sickle hemoglobin as well as the sickle cell trait.
No cure for sickle cell anemia has been found. Individuals rely on bed rest and painkillers for pain crises and fluids to prevent dehydration. Penicillin is given to young children to avoid infection. Blood transfusions can increase the number of normal red blood cells to prevent more serious complications. The anticancer drug hydroxyurea has alleviated pain crises and acute chest syndrome in some adults. Regular medical visits, a healthy diet, and moderate exercise can increase life expectancy and quality of life.

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