What is Sickle Cell Anemia?

Sickle Cell Anemia (SCA) was first described in Chicago in 1910 by James Herrick as an inherited condition that results in a decrease in the ability of red blood cells to carry oxygen throughout the body. Sickle red blood cells become hard and irregularly shaped (resembling a sickle). These sickled cells become clogged in the small blood vessels and therefore do not deliver oxygen to the tissues. This lack of tissue oxygenation can cause excruciating pain, damage to body organs and even death.

What are the different types of Sickle Cell?
The symbol for the normal hemoglobin gene is "A" and for the sickle gene"S". Individuals with SCA have two "S" genes; one "S" gene inherited from each parent. Sickle Cell Trait is a carrier state, and carriers have both "A" and "S" genes (AS). Other forms of sickle cell disease include Sickle- hemoglobin C disease (SC), Sickle Beta-Thalassemia (S-Beta-Thal), Sickle - hemoglobin D Pujab, etc.

Who has Sickle Cell?
SCA is seen most often in Africans and African Americans. However, persons who originated from East Asia, Southern Italy, Saudi Arabia, India, Egypt, South and Central American, Cuba, Greece, and Iran, and Eastern Jews have also been found to have a form of this illness.

Among African - Americans:
1 in 10 have Sickle Cell Trait (Hb AS) 1 in 600 have Sickle Cell Anemia (Hb SS) 1 in 350 have Sickle Cell Disease (Hb SS, SC, S-Beta-Thal) 1 in 1500 have Sickle C Disease (Hb SC)

Among Latinos:
1 in 1,000 have Sickle Cell Disease (Hb SS, SC, S-Beta-Thal) 1 in 172 have Sickle Cell Trait (Hb AS)

Early Symptoms
Symptoms usually begin to appear during an infant's first year, as early as six months. The first symptom seen are dactylitis and fever (usually age 6months - 2 years of age). Other symptom include pain in the chest, abdomen, limbs and joints; enlargement of the heart, liver and spleen; nosebleeds; and frequent upper respiratory infections.

Other Symptoms
As patients grow older they can develop symptoms of anemia - fatigue, irritability, jaundice (yellowish discoloration of the skin and eyes), pain, delayed puberty, leg sores, eye problems, and gum disease.

During A Crisis
Patients also encounter episodes of pain known as sickle cell crises. During a crisis, patients experience shortness of breath, nausea, low-grade fever, jaundice and severe pain in the fingers, toes, arms, joints, legs, back, abdomen, and bones.

Other Symptoms of A Crisis
During a crisis, blockage of blood, and lack of oxygen to the chest and lungs may lead to acute chest syndrome. Damage to the lungs causes severe pain and fever, and produces an abnormal chest X-ray. The lungs' airways narrow, further reducing oxygen delivery. All this leads to an increased risk of infections, which can be fatal.

Some Male Symptoms
Male patients may also suffer from priapism - painful and prolonged erections.

Sickle Cell Today
Presently there is no cure for Sickle Cell Anemia, but with improved care, most sickle cell anemia patients are living long and very productive lives. Sickle Cell disorders are recognized as disabilities and affected individuals are protected under the ADA (The Americans with Disabilities Act).