The man we know as Kier “Junior” Spate, a comedian best known for his role on The Steve Harvey Morning Show, was not always a famous face. As a child, he was sick and his family didn’t know why. At seven years old, he was diagnosed with Sickle Cell Disease (SCD) and given about four more years to live. Kier talked about how that diagnosis—and that life expectancy—was just the first of many times hope was stolen from him recently at the Second Annual Expo for A Cause: Sickle Cell Disease, in Greenville, NC. He described how he defied the odds and went on to be a star running back at his high school in Texas—only to have a teammate report his status as living with SCD, which sidelined him from the team.
Kier, like many of the attendees at Friday’s Expo, has continued doing great things, but wonders what might have been different had he not so often been shut down due to a lack of knowledge or treatment options for SCD. His early struggles did create in him an intolerance for petty problems—a trait he shares with the expo’s founder, Travis Robinson, a nurse at Roanoke Chowan Community Health Center.
Robinson, LPN, and his mother Julia Robinson kicked off the day by remembering their brother and son, respectively, Ti’Rell Montae’ Robinson, who passed away from complications of the disease on Christmas day when he was only 14 years old. Ms. Robinson struggled to talk in front of the conference attendees as she remembered her son and paused. “Take your time,” someone gently told her. As a result of the family’s experiences with Ti’Rell’s care, Travis and Julia Robinson founded The Robinson’s Sickle Cell Foundation, which works to educate both health providers and everyday North Carolinians about SCD.
Though nearly 100,000 Americans and many others across the world live with SCD therapeutics that do anything more than treat symptoms are only now being developed, often through gene therapy. Before, one of the only treatments was hyndroxyurea.
Edward Donnell Ivy, MD, MPH, was born in NC and lives with SCD. When he was young, he was hospitalized with acute chest syndrome and wound up being put on a ventilator. It was so severe that his doctors thought he might not live, until he met a hematologist who prescribed hydroxyurea. Because this drug transformed his life, Dr. Ivy talked about the benefits of hydroxyurea for those living with severe sickle cell crises and hospitalizations. He also talked about how access affects people’s ability to be treated. If only hematologists prescribe a medication and someone has no transportation and lives in rural NC, they may never be exposed to the possibility of the lifesaving treatment. He has seen this treatment’s availability increase beyond just the offices of hematologists and encouraged physicians and those with SCD to learn more about it.
Hydroxyurea wasn’t the only medication that attendees learned about. They also found out about Oxbryta, which curves the oxygen to prevent cells sickling, allowing hemoglobin levels to hold on to oxygen. And they learned that L-glutamine can help cells maintain their shape with the same outcome of holding onto oxygen better.
Several speakers noted that health disparities for African Americans are not just around SCD, but also cardiovascular health. Having a medical home is one way to reduce health disparities. Community Health Centers strive to be medical homes for most of their patients. With SCD as with so many chronic diseases, maintenance of health and regular visits with a doctor who knows you can reduce emergency room visits.
Another recommendation is that people living with SCD learned how to advocate as a patient. It’s also helpful to have an ED backpack that contains:
- Pain meds to take while in crisis until you are processed at your visit.
- A blanket to stay warm (EDs are often cold, which is especially uncomfortable for those with SCD).
- A narrative about your own disease and symptoms in writing so that you don’t have to repeat it while feeling very bad.
- Other items that might make you more comfortable while waiting.
One participant asked, “Ok if I am going to advocate, who do I advocate to?”
Dr. Ivy replied, “Start with community-based organizations, hospitals that are great for sickle cell disease. In North Carolina, there are several of these, but patients in other states may have to do some research to find out where the best facilities are for them… I know you may be very frustrated. But don’t get combative and shut people down. Try to work with folks where they are. Ask them to partner with you to make it better.”
Advocacy to friends and neighbors is also essential. According to the American Red Cross, only 3% of Americans donate blood. People living with SCD often benefit from donated blood, and one in three African Americans donors are a blood match for people living with SCD.
Having both patients and providers learning about SCD treatments and medications as well as how to advocate for themselves are just the kinds of outcomes founder Travis Robinson hoped for as he planned this event last year and again this year. “As a sickle cell warrior and a nurse. Sickle Cell disease education and awareness is very important. Increasing knowledge about SCD can help decrease stereotypes and false assumptions,” Robinson explained. “So, one can have an open mind. It can help decrease health disparities, so one can know about the disease process and the symptoms that come with this debilitating disease. Increased knowledge can also help prevent and intervene when a sickle cell warrior is in crisis and infections can be caught sooner to help decrease the time in the hospital and decrease ER visits.”