Sickle cell disease (SCD), an inherited blood disorder affecting nearly 100,000 people across the country, is a rare but crippling disease that patients recount make day-to-day living almost impossible to manage.
According to the Centers for Disease Control and Prevention, “SCD occurs among about 1 out of every 365 Black or African American births” with one out of 13 Black or African American babies born with the Sickle Cell trait.
Dr. James Taylor, director of the Howard University Center for Excellence in Sickle Cell Disease, shared that roughly 600 adults are suffering from the disease within the District of Columbia.
Furthermore, on a per capita basis, Washington, D.C., has the highest prevalence of sickle cell anywhere in the country at the state level, with Prince George’s County housing the highest prevalence of the disease in the state of Maryland. But despite the numbers, there are no hematologists in Prince George’s County for adult SCD patients.
The disparity creates major access-to-care issues for those affected unlike those suffering from other chronic diseases where there is a greater infrastructure to provide patients with the care they need.
“I think the country across the board has not made this a priority, but there are always reasons for this. I think the reasons for this are all economic. Patients are underinsured, and if you are underinsured, [often] doctors aren’t looking to see [those] patients,” said Taylor. “While it is rare, it’s a very expensive disease, which is unfortunate. So, you have this terrible disease, you are underinsured, and the hospitals [find themselves] losing money when they do care for these patients.”
Latest Developments for SCD Treatment
Since 2017, the FDA has approved three medications to help patients manage SCD, including; L-glutamine approved in 2017, and two during the latter of 2019 called Voxelotor, and Crizanlizumab. Voxelotor binds the sickle hemoglobin and prevents it from forming the long strands that prevent the formation of sickle cells. While it does not affect pain, it is a great treatment for anemia. Crizanlizumab is a monoclonal antibody that doctors distribute to sickle cell patients once a month, as it prevents painful crises by roughly 60%.
Outside of medications, physicians have additionally found transfusion therapy helpful in maintaining a patient’s health. Typically, once a month the doctor will withdraw blood out with a machine and transfuse new blood into the body, considerably playing as a temporary cure.
“So much has focused on symptomatic treatment with opioids that many patients have given up, and many doctors just think sickle cell opioids. So, now I’m emphasizing to patients that these are five different treatments that can prevent episodes from happening and hopefully put you in a position where you don’t, or just occasionally need opioids for painful episodes,” explained Dr. Taylor.
American singer and international star Tionne “T-Boz” Watkins has made huge efforts bringing national attention to the degenerative disease that she was diagnosed with at 28 years old. She details the crippling experience of a sickle cell crisis in her second book, “A Sick Life,” published in 2017.
“You’d think I’d have it all figured out by now, but if I’m telling the truth, it’s incredibly hard to live with this disease. With sickle cell, you don’t always know when a crisis is coming. One can arise at any time, in any place, totally debilitating you,” Watkins explains in the book.
“Some days I wake up consumed by pain which seems to manifest itself slightly differently each time. It’s like knives stabbing me over and over again in my joints. The invisible knives leave no place except my fingers and toes. It’s impossible to function like that. Usually, when I’m in a lot of pain, I can’t walk. Every breath I take throbs, and each gasp of air comes in with a sharp twinge.”
Managing the Pain
Taylor recommends a group of holistic practices of monitoring one’s mental health, controlling anxiety and stress in your life, and participating in activities that help ease your senses similar to yoga or even acupuncture to help control this element of chronic disease.
“Depression and anxiety can trigger a pain crisis, so, it’s very important to control those elements, and if I could work even closer with other groups of doctors, a psychiatrist would be the number one doctor that I would pick. Not because I think that patients have major mental health issues, but it’s dealing with anxiety and depression,” Taylor said.
“These are symptoms of a chronic disease and it doesn’t matter what [disease] it is,” Taylor continued. “But, the difference is in sickle cell, just the anticipation of a pain crisis can give you pain.”
