How health care fails sickle cell patients

CJ Smith was propped up in a hospital bed in May, arguing with a doctor, hot pain flashing through blood vessels jammed with sticky, crescent-shaped red blood cells.

CJ had spent the night in agony after the doctor prescribed much less than the usual dose of painkiller. This came as no surprise to a person with decades of experience being treated for sickle cell disease, someone who could have written the appropriate prescription to the milliliter. The next morning, with a reporter in the room, CJ complained to a patient advocate at Ascension Providence Hospital in Southfield. Now the doctor wanted to talk.

“How are you?” he asked.

“Hello,” CJ said quietly, not meeting his eye.

Standing five feet away, the doctor made his case. He said CJ, at age 34, was receiving far too much painkiller and spending too much time in the hospital. He warned of bed sores, infection, muscle loss and blood clots.

“Six days of IV dilaudid every month?” he asked, his tone incredulous. If he agreed to continue that regimen, he said, “Now I’m negligent.”

CJ pushed back, insisting that no one would come to the hospital unless they were in extreme pain. Anyway, the doctor hadn’t even visited CJ’s room before cutting the prescription below the usual amount, and he didn’t seem aware that CJ, like many sickle cell patients, had developed a high tolerance for opioids. The doctor interrupted before CJ finished, and soon they were speaking at the same time, their voices getting louder and angrier.

“My spine is on fire,” CJ said, cheeks wet with tears. “And you’re not listening, and it’s pissing me off. I have respected you.”

The patient advocate, who’d walked in during the interaction, touched the doctor’s arm and asked him to step out of the room.

CJ stewed, still crying with the frustration of having one’s pain disbelieved.

“They think that all sickle cell patients are the same, and we’re not,” CJ said later. “It’s terrible that we’ve got to put on a show just for us to get the right pain medication. They want us to be kicking and screaming and crying and all that, but I’m not doing that because it won’t do anything but make it worse. They don’t believe our pain. Because they don’t see it.”

Ascension, which operates 140 hospitals across the U.S., declined to respond to questions about CJ’s experience specifically.

“Patient safety is our highest priority, and we strive to provide all patients with compassionate, personalized care,” said Dr. Douglas J. Apple, chief clinical officer for Ascension Michigan, in a statement. “Decisions regarding patient care are carefully made by an interdisciplinary team, including physicians, in conjunction with each patient to ensure that we provide safe and appropriate care.”

CJ knew the problems with sickle cell care went beyond one stubborn doctor.

Sickle cell disease is the most common genetic blood disorder in the world. More than 100,000 Americans navigate its brutal symptoms, including upwards of 4,000 people in Michigan, most of whom, like CJ, live in the Detroit area.

The sickle cell community made national headlines this year after federal regulators OK’d a new gene editing treatment for the disease, the first-ever approval for a treatment using cutting-edge CRISPR technology. But local doctors say the astronomical cost and complexity of the gene editing process will limit the technology’s reach to a handful of patients for the foreseeable future.

Meanwhile, this large group of people suffering from a predictable, well-understood illness receives haphazard, uncoordinated care. Patients have little choice but to rely on emergency rooms to manage their most dangerous symptoms, putting themselves in the hands of doctors who are often unfamiliar with the best practices of sickle cell care and skeptical, in particular, about the high doses of painkillers that are a standard tool for treating a sickle cell crisis.

Sickle cell patients frequently “have to make the decision, do I want to die at home or do I want to go into the emergency room?” said Eric Scott, a professor of psychology at the University of Michigan who studies sickle cell care. “That is one wicked-bad choice.”

A turn for the worse

The problem is especially acute for young adults with sickle cell, who are more likely to visit the ER and more likely to die than patients in other age groups.

This is at least partly because sickle cell patients in this age group were a rarity just a few decades ago.

When pioneering doctor Charles Whitten opened Detroit’s first sickle cell clinic in the 1960s, its patients were considered lucky to reach age 20. Over the years, improvements in treatment slowly boosted life expectancy. Today, it’s in the early 50s for people with the most common form of the disease.

Dr. Wanda Whitten-Shurney, Whitten’s daughter, watched that change happen in real time. She grew up around the Detroit clinic before following her father into medicine.

As more of her pediatric sickle cell patients survived to adulthood, she noticed they took a sharp turn for the worse when they entered adult care.

• Nationally, mortality rates for young adults with SCD more than doubled as they transitioned from pediatric to adult care
• In 2020 , young adult Michiganders with sickle cell visited the ER nearly four times on average, far more than their peers in other age groups. They also had the highest death rate
• 38% of young adults with SCD in Michigan reported not having access to reliable transportation, making it harder for them to get to the hospital or doctor appointments in a timely way

“We are losing far too many patients we’ve brought all this way for the past four decades to a medical system unprepared to receive them,” said Whitten-Shurney, CEO and medical director of the Sickle Cell Association of America Michigan Chapter.

Sickle cell does its damage in a straightforward way: The blood fails to carry oxygen and other nutrients where they need to go.

The symptoms, though, are often maddeningly complex. Every system in the body can go haywire in the absence of steady blood flow, requiring specialized care. In addition to a primary care doctor and a hematologist — a blood specialist — patients may need the expertise of a heart, lung and kidney specialist, to name a few. And that’s to say nothing of the physical therapy and counseling essential to coping with the relentless challenge of living with the disease, but that too few adult patients receive.

All of that complexity is managed for patients at the pediatric sickle cell center at Children’s Hospital of Michigan in Detroit. The center attends to young patients’ needs holistically, and schedulers help them make necessary appointments.

Once patients age out of pediatric care, they are effectively left on their own to navigate the complexities of specialty care in their early 20s.

Unable to stay ahead of their symptoms, patients often seek care in emergency rooms — the U.S. medical system’s option of last resort. A 2020 survey of more than 500 adolescents and adults with sickle cell found 46% felt that emergency room doctors “didn’t care” about them.

Many of Whitten-Shurney’s former pediatric patients keep her on speed dial, giving her an inside look at the system’s failure to provide them with basic care.

“Sometimes when they call me from the hospital, I can’t get the doctor to listen or understand or do anything different,” she recalled. Almost always, the issue is pain management. Some doctors, not realizing that a high tolerance to painkillers is common among people with sickle cell, balk at the large doses that patients know they need.

“I’ve gotten out of bed and gone to the emergency room and been unable to get them to do the right thing,” Whitten-Shurney said. “We’ve got to get them out of the emergency room and into routine maintenance care, so we can prevent some of these complications.”

A poster child for medical inequity

D’Juan Johnson comes from a large family that knows the ruthless nature of sickle cell all too well. He’s one of ten kids. Five were born with sickle cell, including him. Three died of the disease.

In 2017, his older brother Chauncelle, 32, walked into a Detroit emergency room in a sickle cell crisis.

This was a life-threatening situation, for which D’Juan believes his brother should have been admitted for observation and treatment.

That’s not what happened.

Chauncelle, whom the family called “Choo-choo,” had taken pain medication before arriving at the hospital.

“They put medicine on top of that and then sent him home,” said D’Juan, 35. “They did not check to see the amount of medicine he had taken before he got there.”

He died at home of a preventable opioid overdose.

Drawing from his own experience with emergency rooms, D’Juan thinks he knows why the doctors were so quick to turn his brother away.

“They were looking at him like, ‘Oh, he’s just here for pain medicine,’” he said. “But he was actually going through a crisis.”

D’Juan’s sense of injustice at his brother’s death has deep roots in the history of sickle cell disease in the U.S., which is marred by racism at every stage — from drug development to patient-doctor interactions.

The genetic mutation that causes sickle cell disease originated in Africa and the Middle East, where it apparently evolved as a natural defense against malaria.

American complicity in the slave trade brought the gene across the Atlantic, and it’s still common among descendants of enslaved Africans. In Michigan today, around 90% of people with sickle cell disease are Black.

In the wake of the Civil Rights Movement, SCD became an early poster child of medical inequity. Charles Whitten and Dr. Roland Scott , a Black researcher and pediatrician at Howard University, led a successful campaign to push the federal government to support sickle cell research and treatment. His main argument: Far less private and public research funding was dedicated to improving care for sickle cell compared with less-common diseases that mostly affected white people, notably cystic fibrosis.

Racial inequity was the only possible explanation for the difference, he argued. Racial gaps in economic and political power all but ensured that the sickle cell community could not attract the same kind of investment as white patients.

Scott’s work helped bring about the first federal legislation to help the sickle cell community, funding the first medical centers focused on the disease in the 1970s. But disparities persisted.

By 2006, combined public and private research funding for cystic fibrosis was eight times higher than for sickle cell disease. In 2020, a separate study using a different methodology found that public funding for the two diseases was about even, despite the fact that sickle cell is three times more prevalent than cystic fibrosis.

The underinvestment in sickle cell treatment left patients with few options besides opioids to treat their symptoms. Because sickle cell is a fairly rare disease, pharmaceutical companies had little incentive to develop new drugs to treat it. Decades passed without any new treatments targeting the disease. (Hydroxyurea, which since the 1990s has been viewed as the best drug for reducing sickle cell symptoms, was developed to treat cancer.)

Because of that underinvestment, many sickle cell patients learned to expect few options when they saw a doctor.

That’s if they could even find one. Sickle cell specialists have always been — and still are — difficult to find, particularly for adults.

The scarcity of doctors is also linked to race — in this case by way of the nation’s massive racial wealth gap. About 87% of sickle cell patients in Michigan are insured by Medicaid, the federal program for people with low incomes. And in the U.S., caring for Medicaid patients tends to be less lucrative for doctors.

“This is a trend nationally that there is just a shortage of individuals that are willing to treat people that have sickle cell disease,” Scott said. “Among hematologists, benign hematology is the area that covers sickle cell, and it’s not as well … compensated.”

When patients can’t find a specialist to help establish a preventive care routine, they’re more likely to end up in the ER, where many patient complaints arise. This is unsurprising, given that hospitals aren’t required to follow established best practices for emergency sickle cell care. They don’t even have to collect data about the quality of care adult sickle cell patients receive, in part because there is no established definition of what that would look like.

Data collected about pediatric sickle cell care shows “the quality of care that kids receive is horrific,” said Sarah Reeves, an epidemiologist at the University of Michigan who helped establish a list of quality measures for children with sickle cell. “And it’s so sobering because it’s even worse for adults. This is all borderline criminal, what goes on with sickle cell.”

Reeves noted that Michigan has been proactive about improving sickle cell care.

Last year’s state budget included $2.5 million for a new sickle cell center at Henry Ford Hospital in Detroit, which would provide coordinated care and daybeds for adult patients, allowing them to avoid the emergency room. Another $200,000 in grant funding went to five hospital systems around the state for projects — such as patient focus groups — aimed at improving sickle cell care.

The state also granted lifetime coverage to sickle cell patients under the Children’s Special Health Care Services, which covers the costs of some specialty services. The program previously covered sickle cell patients until age 26.

Those programs amount to progress, but alone they can’t solve the problems of sickle cell care. The new clinic at Henry Ford, for instance, is only serving about 180 patients. Dr. Asif Alavi, the director, said they plan to expand.

Mistreatment and mistrust

Recent steps forward in sickle cell treatment have only just begun to address the long history of marginalization that follows a patient like CJ Smith into the emergency room.

This history is present in the lack of treatment options. It’s there in CJ’s interactions with doctors, who tend to take Black people’s pain less seriously than white people’s, and who have likely been trained in the disparaging notion that patients such as CJ tend to go to hospitals in search of painkillers.

If not for this history, CJ might not be in the emergency room at all. Over years of working with a dedicated specialist, CJ and the doctor would have explored various treatments, including drugs and physical therapy, to minimize hospitalization. If a crisis did arise, CJ would receive appropriate pain treatment at a dedicated center staffed by doctors and nurses trained to care for people with sickle cell disease.

That world looks a lot like CJ’s childhood as a patient at the pediatric sickle cell center at Children’s Hospital.

But it seems a long way off today, more than a decade after CJ aged out of pediatric care.

“It’s tough because you’ve got to find someone willing to treat you, and treat you appropriately,” said April Smith, CJ’s stepmom and a registered nurse. That’s why CJ has “bounced around a lot” among providers, she said.

Trapped in a cycle of mistreatment and mistrust, CJ is skeptical of the available treatment options and relies primarily on painkillers for relief, which do nothing to address the underlying cause of the pain.

So when CJ’s doctor at Ascension in Southfield allowed the consultation to devolve into a shouting match, the result wasn’t just emotional distress, though that would be bad enough for a patient experiencing physical pain. The doctor further fractured CJ’s trust in the medical system — a trust that plays an outsized role in keeping sickle cell patients alive.

CJ was still in bed, mentally replaying the sarcasm and disbelief in the doctor’s voice, when the doctor walked back into the room and offered a “compromise.” He’d increase the painkiller dose above what he’d previously suggested, but not up to CJ’s usual amount.

CJ remained stone-faced as the doctor left, unwilling to give him the satisfaction of gratitude, or even agreement.

Later that day, a different doctor took over CJ’s care and reinstated the usual dose. CJ’s thoughts eventually shifted to an idea for a podcast for other sickle cell patients, particularly young adults preparing to transition out of pediatric care.

The message? “Adulthood is terrible,” CJ said.

How health care fails sickle cell patients · Outlier Media