COLUMBIA, Mo. ― September is National Sickle Cell Disease Awareness Month, and health professionals at University of Missouri Health Care want people who are most at risk of carrying the disease’s genetic trait to understand the importance of education and comprehensive care.
“Sickle cell is an inherited disease that causes abnormally formed red blood cells,” said Thomas Loew, M.D., medical director of the Children’s Blood Disorders and Cancer Unit at Children’s Hospital. “Normal red blood cells are soft and round, allowing them to flow easily through blood vessels. Sickle cell disease produces red blood cells that are rigid and half-moon shaped. These abnormally shaped cells disrupt normal blood flow and cause health complications.”
Individuals with the disease experience symptoms such as increased susceptibility to bacterial infections, increased risk of stroke, severe pain caused by the difficult passage of the abnormally shaped blood cells through the body’s tissue, and damage to internal organs such as the kidneys, liver and spleen.
Treatment for sickle cell anemia usually is aimed at avoiding crises, relieving symptoms and preventing complications.
“Antibiotics and pain management are typical methods we use to care for someone with sickle cell disease,” said Elizabeth Gunier, R.N., a coordinator at Children’s Hospital’s Blood Disorders and Cancer Unit and Ellis Fischel Cancer Center’s dedicated adult sickle cell clinic. “However, in some instances, blood transfusions or bone marrow transplants may be necessary. It is important for patients to make regular visits to their health care providers so red blood cell counts and overall health can be monitored to reduce the risk of complications.”
In the United States, sickle cell disease affects African-Americans mostly, and some Latinos. Some forms of the disease may also occur in people with other ethnic backgrounds, such as those whose ancestors came from East India or Mediterranean countries such as Turkey, Greece and Italy.
All babies born in Missouri are tested for sickle cell disease, but Loew recommends that those at risk or with a family history of sickle cell also get tested for the genetic trait before having children.
“It’s important for people to know that parents who carry the genetic trait but who don’t have the disease still can pass the disease on to their children,” he said.
When both parents have the sickle cell trait, there’s a 25 percent chance that their child will inherit sickle cell disease. But when one parent carries the trait and the other actually has the disease, the odds increase to 50 percent that parents will pass the disorder on to their child.
“Awareness is the key with managing sickle cell disease,” said Gunier. “The most critical time is the first few years of life, which is why prenatal care, early diagnosis and comprehensive treatment are so important.”
For more information or to make an appointment with a sickle cell specialist, please contact Gunier at (573) 882-3961.