Sickle cell is an often misunderstood disease—especially when it comes to adult patients, say advocates for patients in Louisville.
The Sickle Cell Association of Kentuckiana is trying to bring attention to the disease and the people who live with it ahead of World Sickle Cell Awareness Day on Friday.
The association provides education, support and advocacy for people with sickle cell disease and their families.
Sickle cell is a hereditary blood disease where red blood cells are misshapen into a crescent shape, making it difficult to carry oxygen throughout the body. It is most common in people of African descent.
Daphne Matthews Johnson, who was born with sickle cell disease, is the president of the association. She said there are many misconceptions about the disorder, including the idea that patients can't live productive lives and die at a young age.
"With adequate treatment and substantial health care, all patients can live healthy, successful lives as long as they monitor the things that they need to monitor—like their eating habits, taking medications, staying up to date on their shots and immunizations," said Johnson, 33.
It is estimated that more than 70,000 Americans have sickle cell disease and about 1,000 babies are born with the disease each year in the United States, according to the Sickle Cell Disease Association of America.
And while there is much emphasis on treating babies and children with the disorder, many young adults fall through the cracks, said Johnson.
"We're noticing that a lot of them are not living long lives because they aren't getting adequate treatment, because there's a lack of doctors and professionals who can treat them and take care of them just like there was for the children side," she said.
Dr. Ashok Raj, pediatric hematologist oncologist with University of Louisville Physicians, said adult care is fragmented. Unlike other chronic diseases, children are not necessarily ready to transition to adult care when they turn 18, so doctors try to assess the readiness of each patient.
"We try to tell them that they have to empower themselves with knowledge of sickle cell disease," he said.
"Know what the complications of the disease are and the need to see a variety of specialists, need to keep appointments with the radio specialists, need to take medications on a regular basis—adherence to all the treatment regimens that have been outlined for them."
Johnson said adult patients in the area have been known to travel to Cincinnati for treatment.
Raj said Louisville would benefit from an adult center where all patients can be treated. For now, through the transition program patients are at least under the care of at least one dedicated sickle cell hematologist who knows them well rather than going to emergency rooms for care.