|COURTESY TYLER NIVAR
|Tyler Nivar (left) and his fraternal twin brother Thomas both have sickle cell disease. They developed the ailment from their parents, who have the sickle cell trait.
Tyler Nivar has good and bad days living with sickle cell disease.
A good day for the 24-year-old may consist of doing push-ups, playing basketball, or working as a server at Olive Garden.
“I can be active,” he said. “I could play sports even, obviously, knowing my limits and using parameters and things of that nature, using precautions. I’ve had more good days than bad days.”
But sometimes, Nivar has excruciating discomfort, describing it as a “deep bone pain” way worse than muscle soreness. In May, he was hospitalized due to an infection related to sickle cell disease, a rare blood disorder that primarily affects people of African descent.
“The infection with the sickle cell started to attack my liver, my lungs and my kidneys,” Nivar said. “The very next day, a few hours later, I was put in ICU and from there I was intubated, [and] medically induced into a coma for a little over 30 days. I woke up [in] late June.”
Both of Nivar’s parents have the sickle trait, which led to him and his fraternal twin brother Thomas developing sickle cell disease. Sickle cell disease occurs when red blood cells form a sickle or C-shape according to the U.S. Centers for Disease Control and Prevention. These cells become hard, sticky, and stuck inside of small blood vessels, blocking the blood flow and oxygen to different parts of the body.
Sickle cells die faster, causing a shortage of red blood cells, often known as sickle cell anemia.
“[My parents] don’t have the disease,” Nivar said, “but they have the trait, and they were unaware that they had the trait until they were pregnant with my brother and I.”
Sickle cell disease can cause severe pain, organ damage, serious infections, and even stroke.
With treatments slowly developing over the past decade, many people living with the condition have died before age 20.
“When I started [12 years ago], there were exactly two things we could do for patients with sickle cell disease in terms of disease modification which was hydroxyurea, and we could do [a blood] transfusion – and that was it,” said Dr. Payal Desai, director of the adult sickle cell enterprise at Atrium Health Levine Cancer Institute. “Over the past five years, there’s been significant advancements,” adding that in 2018 the “first Food and Drug Administration approval in 25 years of a medication” for the disease.
The FDA approved a powder called L-glutamine in 2017 to reduce damage to sickle red blood cells, followed by two other medications to treat SCD. At the end of 2019, the new drug known as Oxbryta was approved by the FDA but there was a delay in the medication’s uptake because of the pandemic.
Between systemic racism and the lack of funding and treatments, the treatment gap for people of color has grown. People with sickle cell often suffer from pain crises, in which cells get stuck in blood vessels, causing mild to severe pain that can last for any length of time.
“I believe my first pain crisis was when I was like six months old,” Nivar said, adding it was much worse as a kid. “When I was younger, it was more like, [a] ‘why me?’ type of attitude. It can be discouraging.”
Nivar has other health conditions related to SCD such as asthma, and he’s had problems with his heart and left hip. In 2019, his hip was replaced after suffering avascular necrosis, or loss of blood supply to the joint. He also has undergone two heart surgeries.
As a provider, Desai wished more doctors understood the pain of patients with sickle cell and how it takes a toll on their mental health.
“The problem is there’s no test,” she said. “There’s nothing that people can measure to say, ‘oh, this person is telling the truth.’ They’re in a crisis and so you have an illness that can’t be measured.”
A common problem people with SCD face are providers not believing they are in severe pain. Studies have found that Black patients were 22% less likely to receive pain medications than their white peers, according to the Association of American Medical Colleges.
With a history of medical experiments done illegally on African Americans, Black patients may be leery of advancements in treatments.
“I think that it is always challenging to build trust,” Desai said. I think that it is an essential part of taking care of patients. I think knowing and sharing what I know, but also being able to say here’s what I don’t know. But here’s how we’re going to figure this out together.”
There have been times when doctors would question Nivar’s pain level whenever he went to the hospital and told them a certain treatment wasn’t working.
“Some doctors, they’re more apprehensive to attack the pain aggressively just because there might be a higher chance of addiction,” he said. “Or they equate [the need for help to] ‘there’s a black male coming in, he’s just seeking drugs.’”
Nivar receives care at Atrium Health Levine Cancer Institute and takes two daily medications for SCD.
Nivar’s brother, Thomas, has also experienced some complications from SCD, resulting in several hospitalizations and taking infusions once a month.
Another problem is relatively few doctors specialize in sickle cell, further explaining why they don’t understand pain levels among patients or how to treat people with the condition.
Although there is a need for more research and funding, gene therapy could possibly be a treatment for SCD.
“Gene therapy is not one entity, but rather different modalities,” Desai said. “They’re trying to optimally change that gene, fix it in some way, or increase that production and put that back into the patient. There’s different ways to fix the problem, we will say ‘cure’ in quotation marks because we don’t know how long the effects of this will last and if it will take away all of the complications or only some of them. We just don’t have enough data to know.”
At Atrium Health, they refer to SCD patients as sickle cell warriors. As one of them, Nivar describes it as a “journey.”
“There are going to be some high points and there are going to be some low points, some mountain tops, and some valleys, but there is light at the end of the tunnel,” he said. “Surround yourself with a good support group. For me, it’s my family, my faith — that is definitely my biggest coping mechanism.”
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