A lucky break may have finally arrived for people with sickle cell disease, but 22 year-old Detroiter Raymond Taylor is hard-pressed to see it that way.

Sickle cell disease, a painful genetic blood disorder that mostly affects Black people, has an outsized impact in Detroit, which has one of the nation’s largest Black populations.

More new drugs aimed at improving the lives of people living with sickle cell have been authorized by federal officials in the last five years than in the previous 50. A cutting-edge gene editing procedure with curative potential could be approved by federal officials before the end of the year.

But for Taylor and other patients, who tend to call each other warriors, it can feel as if the battle against sickle cell is grinding on unchanged. Earlier this fall, at the Sickle Cell Matters Walk fundraiser in Detroit, several people with sickle cell disease told Outlier Media they’d been hospitalized just a few days before with a pain crisis.

Newer treatments can come with serious side effects, and they are often priced unaffordably. Meanwhile, many adult patients in Detroit still lack access to basic quality care for the disease.

Gene editing treatment faces skepticism

The most heralded of the new treatments, which would infuse patients with a genetically modified version of their own blood cells, has eliminated sickle cell symptoms entirely in some trial patients. The treatment is also risky with potentially deadly side effects, and projected to be eye-poppingly expensive.

Since 2017, the U.S. Food and Drug Administration (FDA) has approved three new sickle cell-specific drugs. Pharmaceutical companies were apparently spurred on by government incentives and, as some advocates believe, by growing public awareness of racial inequities in American medicine. The new drugs help reduce pain episodes and lower the risk of stroke. The FDA hopes to decide in early December whether two new sickle cell drugs based on gene-editing technology would be among the first of their kind to be approved for use in humans.

“They’re brave, they’re inspiring. They’re also skeptical.”

Dr. Wanda Whitten-Shurney, CEO and medical director of the Sickle Cell Association of America Michigan Chapter

After enduring decades of mistreatment and disregard from American medicine, many sickle cell patients — most of whom are Black and use Medicaid — aren’t ready to believe the hype or take the risk, assuming they could even access the pricey treatment.

That includes Taylor, who is two years removed from a double hip replacement caused by sickle cell. “I don’t like to just put stuff in my body” without being sure it will help, he said.

He’s happy to be on his feet and building strength, and he’d be content to stay out of the hospital and try to join the workforce, maybe as a physical therapist. He’ll pass, at least for now, on the experimental gene editing treatments.

Sickle cell patients “use that phrase a lot — guinea pig,” said Dr. Wanda Whitten-Shurney, CEO and medical director of the Sickle Cell Association of America Michigan Chapter. “They’re brave, they’re inspiring. They’re also skeptical.”

The gene that causes the disease first evolved in Africa, and most people with the disease are descendents of Africans. There are an estimated 100,000 people with sickle cell disease in the U.S. and millions more around the world. The history of the disease’s treatment in the U.S. is an extreme example of American medicine’s well established dismissal of Black people’s pain.

For decades, the pharmaceutical industry invested next to nothing in sickle cell treatments. Patients were left with few options to manage the pain caused by their crescent shaped red blood cells clumping together and blocking blood vessels besides going to emergency rooms for powerful pain medications. Patients frequently report facing long waits for treatment, poor care and suspicions that they were there to feed a drug addiction, not because of pain.

“We’re doing our best and building momentum, but we’re still dealing with a lot of health disparities, and we’re still being considered drug seekers,” said Clifton Kirkman II, the social media specialist for SCDAAMI and a sickle cell patient.

New treatments don’t eliminate challenges in basic care

Most of the marchers on Woodward Avenue in Detroit wore red, the color favored by advocates for sickle cell awareness.

Organizers of the event said they are just as interested in the average quality of care for sickle cell patients as the cutting edge of medicine.

Bone marrow transplants can cure sickle cell disease, but the procedure is risky and requires a very specific donor: simply having the same blood type as the patient is not enough. The new gene editing treatments, which are effectively a transplant of the patient’s own genetically modified stem cells, could make the procedure more accessible by eliminating the need for a donor. But chemotherapy is required to make space for the new cells, which can cause dangerous infections, infertility or death, among other side effects. And the estimated price tag of $2 million per person could push the gene editing treatment out of reach even for patients inclined to take those risks.

The new science is reason for optimism, but it is not an excuse to ignore the failures of basic care for sickle cell patients in Detroit, Whitten-Shurney said.

Whitten-Shurney previously worked for 30 years as a pediatrician at the nationally renowned center for sickle cell disease at Children’s Hospital in Detroit, and she says she’s watched many of her former patients struggle to find adequate medical care as adults.

Sickle cell disease affects every system in the body, and patients often need to consult with a small army of therapists. Coordinating such complicated care can be overwhelming, and  Whitten-Shurney says patients often wait to seek care until they have a pain crisis.

”They wait so long because they know that when they get to the emergency room for the most part they’re not going to be treated well,” she said. “I really want to have a place for them to go where they can be believed.”

Shurney wants to help build a new center for adult sickle cell care at Henry Ford Hospital where people living with sickle cell could get specialized care and see primary care physicians, physical therapists, and social workers. That plan got a jump-start this year when the state lawmakers included a $2.5 million grant for the center in the state budget.

After the walk ended, a few dozen people gathered in a light-filled room inside The Charles H. Wright Museum of African American History. The Motown tunes blasting outside felt far away as the group stood in a circle to talk about the worst days of their lives.

One speaker lost a child to sickle cell in April. Another lost a son last year. Others had lost siblings and grandchildren. Their stories touched on struggle, pain, resilience, faith. People hugged each other and cried.

Taylor, meanwhile, was not in the room. He was outside, helping event organizers clean up, still on his feet.

Koby (he/him) believes that love drives people to fight for their communities, and that curiosity is food for love. He enjoys the many moods of the Detroit River.