Sickle cell disease is an inherited disorder that causes the red blood cells to be abnormally shaped and get stuck inside the blood vessels, making it hard to deliver oxygen throughout the body. This causes intense pain and can lead to infection and severe complications like organ damage and stroke. Here are five things leading physicians in the field say you should know about the disease.
1. Sickle cell disease is the most common genetic disorder in the United States. About 100,000 Americans are thought to be living with sickle cell disease, and every year another 1,000 babies are born with the disease. “That compares to about 50,000 people living with cystic fibrosis,” says Dr. Clinton H. Joiner, director of the division of hematology at Cincinnati Children’s Hospital Medical Center. “It’s much more common than people realize.” Worldwide it is thought to affect more than 500,000 babies a year.
2. People are born with sickle cell disease; it does not develop in adulthood, and it is not contagious.Sickle cell disease is a genetic disorder that is passed down from parent to child. To have the disease, both sets of parents must carry what is known as the sickle cell trait. If both parents have this trait, there is a 25 percent chance that their child will have sickle cell disease. In the United States, as many as two million people carry this trait.
3. Sickle cell disease is chronic but treatable and is not a death sentence. Twenty years ago, children with sickle cell disease rarely lived to become adults. Today, the outlook for patients with the disease has improved significantly. Thanks to new treatments and therapies, many people with the disease are now living into their 40s and 50s. Even though sickle cell disease is a very debilitating chronic illness, “people can lead very productive lives with the disease,” says Dr. Lanetta Jordan, chief medical officer for the Sickle Cell Disease Association of America.
4. Sickle cell disease affects people of many different races. Sickle cell disease is often thought of as a disease affecting blacks, and a majority of patients — researchers estimate 60 to 80 percent — are African-American, but other races are affected. People of Indian, Middle Eastern, Hispanic and Mediterranean heritage are also commonly affected. “People are often surprised to learn that this is not an African-American disease,” says Dr. Jordan.
5. Patients with sickle cell disease require comprehensive care. Since sickle cell disease is a chronic illness that affects many systems in the body, it is essential that children and adults have a system of care that includes primary care physicians, hematologists, pediatricians and social workers. “A lot of day-to-day care can be done by a generalist,” says Dr. George Buchanan, professor of pediatrics at UT Southwestern Medical Center at Dallas. “But it is important to have a team of experts who work together and are in ongoing consultation with a large institution or center that specializes in sickle cell disease.”