Sickle Cell, Know Your Status and it's Benefits.

A group of disorders that cause red blood cells to become misshapen and break down.

With sickle cell disease, an inherited group of disorders, red blood cells contort into a sickle shape. The cells die early, leaving a shortage of healthy red blood cells (sickle cell anaemia) and can block blood flow causing pain (sickle cell crisis).

Types of Sickle Cell Disease

There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle Hemoglobin-C Disease (SC), Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia.

Sickle Cell Anemia (SS): When a child inherits one substitution beta globin genes (the sickle cell gene) from each parents, the child has Sickle Cell Anemia (SS). Populations that have a high frequency of sickle cell anemia are those of African and Indian descents.

Sickle Hemoglobin- C Disease (SC): Individuals with Sickle Hemoglobin-C Disease (SC) have a slightly different substitution in their beta globin genes that produces both hemoglobin C and hemoglobin S. Sickle Hemoglobin-C disease may cause similar symptoms as sickle cell anemia but less anemia due to a higher blood count level. Populations that have a high frequency of Sickle Hemoglobin-C disease are those of West African, Mediterranean and Middle Eastern descents.

Sickle Beta-Plus Thalassemia: Individuals with Sickle Beta Thalassemia (SB) disease also contain substitutions in both beta globin genes. The severity of the disease varies according to the amount of normal beta globin produced. When no beta globin is produced, the symptoms are almost identical to sickle cell anemia, with severe cases needing chronic blood transfusions. Populations that have a high frequency of Sickle Beta Thalassemia are those of Mediterranean and Caribbean descents.

Sickle Hemoglobin-D Disease: Through research, hemoglobin D, which is a different substitution of the beta globin gene, has been found to interact with the sickle hemoglobin gene. Individuals with Sickle Hemoglobin-D disease (SD) have moderately severe anemia and occasional pain episodes. Populations that have a high frequency of Sickle Hemoglobin-D disease are those of Asian and Latin American descents.

Sickle Hemoglobin-O Disease: Hemoglobin O, another type of substitution in the beta globin gene, also interacts with sickle hemoglobin. Individuals with Sickle Hemoglobin- O disease (SO) can have symptoms of sickle cell anemia. Populations that have a high frequency of Sickle Hemoglobin-O disease are those of Arabian, North African and Eastern Mediterranean descents.

Symptoms

Signs and symptoms of sickle cell anemia usually appear around 5 months of age. They vary from person to person and change over time. Signs and symptoms can include:

Anemia. Sickle cells break apart easily and die, leaving you with too few red blood cells. Red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells (anemia).

Without enough red blood cells, your body can't get enough oxygen, causing fatigue.

Episodes of pain. Periodic episodes of pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain can also occur in your bones.

The pain varies in intensity and can last for a few hours to a few weeks. Some people have only a few pain crises a year. Others have a dozen or more pain crises a year. A severe pain crisis requires a hospital stay.

Some adolescents and adults with sickle cell anemia also have chronic pain, which can result from bone and joint damage, ulcers, and other causes.

Swelling of hands and feet. The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.

Frequent infections. Sickle cells can damage your spleen, leaving you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.

Delayed growth or puberty. Red blood cells provide your body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.

Vision problems. Tiny blood vessels that supply your eyes can become plugged with sickle cells. This can damage the retina — the portion of the eye that processes visual images — and lead to vision problems.