Tionne “T-Boz” Watkins was born in Des Moines, Iowa. She is of African American and Native American descent. As a child, Watkins was diagnosed with sickle-cell anemia; her parents both have the homozygous recessive trait which one in 12 people of African descent have. Since the age of seven, Watkins has been in and out of hospitals due to her condition. Watkins’ family moved from Des Moines to Atlanta, Georgia, when she was nine years old. Because of her disease Watkins was not expected to have children and live past her 30s.
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 changes the shape of red blood cells, especially when the cells are exposed to low oxygen levels. The red blood cells become shaped like crescents or sickles.
The fragile, sickle-shaped cells deliver less oxygen to the body’s tissues. They can also get stuck more easily in small blood vessels, and break into pieces that interupt healthy blood flow.
Sickle cell anemia is inherited from both parents. If you inherit the hemoglobin S gene from one parent and normal hemoglobin (A) from your other parent, you will have sickle cell trait. People with sickle cell trait do not have the symptoms of sickle cell anemia.
Sickle cell disease is much more common in people of African and Mediterranean descent. It is also seen in people from South and Central America, the Caribbean, and the Middle East.
Symptoms usually don’t occur until after age 4 months.
Almost all patients with sickle cell anemia have painful episodes (called crises), which can last from hours to days. These crises can affect the bones of the back, the long bones, and the chest.
Some patients have one episode every few years. Others have many episodes per year. The crises can be severe enough to require a hospital stay.
Common symptoms include:
- Attacks of abdominal pain
- Bone pain
- Delayed growth and puberty
- Rapid heart rate
- Ulcers on the lower legs (in adolescents and adults)
- Yellowing of the eyes and skin (jaundice)
Tests commonly performed to diagnose and monitor patients with sickle cell anemia include:
- Complete blood count (CBC)
- Hemoglobin electrophoresis
- Sickle cell testThe goal of treatment is to manage and control symptoms, and to limit the number of crises.Patients with sickle cell disease need ongoing treatment, even when they are not having a painful crisis.Folic acid supplements should be taken. Folic acid is needed to make red blood cells.Treatment for a sickle cell crisis includes:
- Blood transfusions (may also be given regularly to prevent stroke)
- Pain medicines
- Plenty of fluids
Other treatments for sickle cell anemia may include:
- Hydroxyurea (Hydrea), a medicine that may help reduce the number of pain episodes (including chest pain and difficulty breathing) in some people
- Antibiotics to prevent bacterial infections, which are common in children with sickle cell disease
Treatments for complications of sickle cell anemia may include:
- Kidney dialysis or kidney transplant for kidney disease
- Drug rehabilitation and counseling for psychological complications
- Gallbladder removal in those with gallstone disease
- Hip replacement for avascular necrosis of the hip
- Treatments, including surgery, for persistent, painful erections (priapism)
- Surgery for eye problems
- Wound care, zinc oxide, or surgery for leg ulcers
Bone marrow or stem cell transplants can cure sickle cell anemia. However, they are current not an option for most patients. Sickle cell anemia patients are often unable to find well-matched donors.