The U.S. Department of Health and Human Services (HHS) held the inaugural Sickle Cell Disease (SCD) summit on Sept. 24, designed to educate the masses and connect various parts of the SCD community.
The landmark event themed “Empowered and Thriving SCD Warriors and Communities,” provided sickle cell warriors (those surviving the crippling condition) health professionals, and advocates alike, an opportunity to network and align goals and initiatives to accelerate developments in research, SCD care, and an improved quality of life.
“I hope you’re here because you believe that there is a chance that we can actually change this paradigm when it comes to sickle cell,” HHS Secretary Xavier Becerra said to the attending audience. “I hope you’re here to send a message to the scientific community, [to] keep going. We’re going to figure out how to make your life saving therapy available to people, if you just partner with us and make sure others can afford it.”
The summit’s program was packed with an array of panel discussions featuring several resilient ‘SCD warriors,’ leading healthcare providers, members of Congress, and community advocates. Each discussion examined a bevy of information, highlighting the latest research and innovative treatments, while discussing the economic, social, and healthcare challenges encountered by individuals with SCD.
The inherited blood disorder that causes red blood cells to become rigid and sickle-shaped, can cause arduous health challenges including excruciating pain crises – a major symptom of sickle cell anemia – as well as blood clots, anemia, stroke, and other complications that often impede one’s ability to maintain work, school, or demanding life responsibilities.
The event’s keynote speaker Dr. Edward Donnel Ivy, vice chief medical officer for the Sickle Cell Disease Association of America (SCDAA), is also a sickle cell warrior who weathered severe pain crises and complications throughout his childhood and adult years.
Beginning his federal career at the National Health, Lung, and Blood Institute, Ivy helped develop some of the most critical SCD clinical treatment guidelines that exist today. During the summit, he shared his first visit with a hematologist being the most pivotal moment of his bout with SCD, introducing him to a groundbreaking medication called Hydroxyurea.
“[While at UNC Chapel Hill], I spent weeks in ICU recovering from acute chest syndrome, and it was at that time that I was able to meet Dr. Gene Oringer [who] was also an investigator on the Hydroxyurea study, and he would talk to me often about [the treatment],” Ivy shared. “I’m talking to him and saying, ‘I don’t know if I want to take Hydroxyurea. I don’t want to be a guinea pig. I’ve heard of the Tuskegee experiment. Or hey, what if this gives me cancer?”
After heavy consideration, Ivy started taking Hydroxyurea, which turned out to be what he calls ‘a game changer.’ He went from having three to four crises a year that required hospitalization to not having any crises for years, allowing him the stable health and ability to complete his undergraduate studies.
Ivy’s continued studies in pediatrics highlighted the vast disparities in patients who were not exposed to Hydroxyurea, often due to fear of fatal side effects from the medication unfamiliar to many, along with community barriers, like the lack of providers prescribing the drug. His treatment has encouraged his efforts to improve clinical trial guidelines and access to quality care for people living with SCD.
“I’ll never forget what Dr.Oringer said to me. He said, ‘Donnell, I can’t guarantee you that you won’t get cancer if you take Hydroxyurea, but, chances are, if you had one episode of acute chest syndrome (ACS), you’re more likely to have another. So, you could not take Hydroxyurea and develop ACS in six months to a year and die from that,’” Ivy said. “That helped me to realize that sickle cell is a very severe disease, that there is risk to not treating sickle cell disease, just like there are risks to some of the therapies that exist.”
Why the Summit Matters: The Widespread Impact of Sickle Cell Disease
Sickle cell disease is the most common inherited blood disorder in the United States, according to the National Heart, Lung, and Blood Institute.
“It affects approximately 100,000 Americans and more than 20 million people worldwide,” the National Heart, Lung, and Blood Institute reports.
Further, there is a genetic disparity shown throughout persons with the disease, as the Centers for Disease Control and Prevention reports that over 90% of people with the SCD in the U.S. are non-Hispanic Black or African Americans across the country.
Among the many experiencing the most challenging complications from the disease, there are more than roughly 2 million people in the United States who live with the sickle cell trait. But while people with only sickle cell trait typically experience no symptoms or health complications in their daily lives, their lifestyle and decisions are still impacted by their genetic makeup.
People like Nigerian-born, British actress Wunmi Mosaku, of “Lovecraft Country,” “Deadpool” and “Wolverine,” spoke volumes to the lesser known effects of having SCD trait that impacted her life when she received her diagnosis in 2020.
“I just didn’t realize how many decisions were being made because of SCD. The jobs, travel, the climate they choose to live in. I have friends who have told me that every decision they make in their life, Sickle cell is a part of that decision making,” Mosaku said. “Someone I know said they just don’t get into relationships because relationships are stressful, and stress brings on crises for them.”
For Mosaku, whose husband also discovered he too is a trait carrier, the confirmed blood testing unexpectedly changed the trajectory of her future maternity journey, leading her to carefully consider motherhood with the potential of birthing a child with SCD – an experience that many with the SCD trait can relate to.
“I spoke to a few mothers of [SCD] warriors, and I heard the stories about changing from pediatric care to adult care, prejudices that come into play with adult care. I knew that as a parent I could be there for a child, but [when they become an] adult, you’re not always around,” she said. “So just hearing how people live, the struggle, pain, and decisions that warriors were having to make because of their disease, [including] carriers, I felt like because I have the knowledge, I shouldn’t gamble [those possibilities].”