Disparities in funding and access to care

Sickle cell disease patients are often unable to access the care they need for their debilitating disease.

And government and private resources dedicated to it pale in comparison to other diseases.

Sickle cell disease is often compared to cystic fibrosis – another genetic illness that’s similarly complex, but not as common as sickle cell disease. An estimated 30,000 people in the U.S. have cystic fibrosis, compared to about 100,000 sickle cell disease patients. The majority of cystic fibrosis patients are White. Federal funding for research on cystic fibrosis is nearly 10 times higher per patient compared to sickle cell disease.

There’s only one explanation for this federal funding disparity: systemic racism, says Marsha Treadwell, a researcher and a pediatric psychologist specializing in sickle cell disease at Benioff Children’s Hospital at the University of California San Francisco.

She suspects the disparity in philanthropic funding for the two diseases is even greater, because Black people in the U.S. typically have less social capital. One peer-reviewed study found funding from charitable foundations for cystic fibrosis is 75 times greater per patient than for sickle cell disease.

Black Americans have been handed the short end of the stick for generations when it comes to educational opportunities and wealth accumulation. Those who have sickle cell disease on top of that experience a double whammy. The disease is debilitating, making it hard for many of them to complete school, especially under-resourced, majority-Black public schools that often lack proper accommodations. As a result, they are less likely to land well-paying jobs and more likely to end up unemployed due to the unexpected nature of the disease.

“When you think about Blacks and African Americans, you have to understand the relationship between wealth, employment, health and the criminal justice system,” Treadwell said. “It is a very complex relationship in this country. And it does come into play for people with sickle cell disease.”

More than half of all sickle cell patients are on some kind of government insurance programs, like Medicaid and Medicare, which tend to pay health care providers less for services than private insurers

For this reason, fewer doctors choose to specialize in treating sickle cell disease, says Dr. Sophie Lanzkron, the director of the sickle cell center for adults at Johns Hopkins University.

When she seeks reimbursement from Medicaid for care provided to sickle cell patients, Lanzkron said she is paid less than 40 percent of what she bills.

“You can’t just put up a shingle and open your door and say, ‘I’m a sickle cell doc’ and expect to make a living. You can’t,” Lanzkron said.

It creates a situation where only large academic centers like Hopkins can afford to sustain comprehensive sickle cell disease programs. Compare that, again, to cystic fibrosis: There are more than 100 comprehensive care centers for cystic fibrosis, and fewer than half that many centers for sickle cell disease. That leaves comprehensive care out of reach for most sickle cell patients in the U.S.

The way we treat sickle cell patients today is like telling someone with cancer to get care from a non-specialist or the emergency department – instead of an oncologist.

Lanzkron called it “inadequate and unethical.”

Black pain is discredited and neglected

Teanika Hoffman, 35, has first-hand experience with inadequate care. She’s Black and has sickle cell disease.

When Hoffman was in graduate school, midterm season hit and the pressure and stress of wanting to ace her exams and land on the Dean’s list triggered a pain crisis. She was overcome by pain in her legs.

This was her first sickle cell pain crisis away from her family, navigating care alone as an adult. She managed to get to a nearby hospital in Philadelphia. Things quickly unraveled.

“I was basically ignored in excruciating pain. My health deteriorated really quickly,” Hoffman said. “I could barely move [and felt] super weak. My family had to drive up from Maryland to discharge me against medical advice.”

Hoffman said her family rushed her to another hospital where they “nursed her back to life.” But by that point, her hip bone had died, leaving her unable to walk for a month.

Because Hoffman has sickle cell disease SC (a type of sickle cell disease), her hemoglobin levels are often high compared to other sickle cell patients. During pain crises that land her in the hospital, she said staff would do blood tests, see her hemoglobin levels and start to question if she even has sickle cell disease.

“It’s like taking your finger and like slamming it in your car door repeatedly, right? And then going to the hospital and being told, ‘you’re not in pain, you’re a liar, you’re a drug addict,’ and then sitting hours in excruciating pain and then hoping that someone will take pity on you,” she said.

“This is the story I’ve noticed throughout my life, you know, being discharged from one hospital to another hospital.”

The onus was on her to prove her sickness — something she suspects she wouldn’t have to do if she were White.

In health care settings, clinical guidelines help inform doctors on the proper care and management of patients with complex illnesses. Some guidelines exist for sickle cell disease, but they’re not consistently followed, said Treadwell, the UCSF psychologist.

A dearth in funding and providers means less research and less data are available. Unlike cystic fibrosis and hemophilia — another less-common genetic blood disorder affecting mostly White patients — sickle cell does not have a national registry that supports nationwide clinical research studies and aggregates findings that help inform guidelines.

This means that even when sickle cell guidelines are updated with new data and information, providers are not always aware of them or inclined to follow them.

“The health care system says, ‘We need evidence for these guidelines that you’re putting forward.’ But you can’t generate the evidence without proper funding,” she said. “So you have this cycle, again, where there’s a demand for evidence before the guidelines are pulled into play, but yet, there’s no way to generate the evidence because you just don’t have enough funding behind it.”

For diseases like cystic fibrosis and hemophilia, providers in emergency departments have clear guidelines to follow, and specialists are often called on for help.

“When you have cystic fibrosis and you show up in the emergency room in the middle of nowhere, the first phone call is to [your] pulmonologist,” Lanzkron said. “[ER staff] are terrified to do the wrong thing for that person with cystic fibrosis.”

But it’s a different story for sickle cell disease.

Oftentimes when sickle cell patients show up in crisis, they have other complications that can go unrecognized because there is no sickle cell disease expert involved in their care – and specialists are not brought in to help.

Some of these patients end up dying or are left with irreversible damage.

“I’ve had patients of mine who have had really bad outcomes because no one bothered to pick up the phone,” Lanzkron said.

This story comes from a reporting collaboration that includes the Indianapolis Recorder and Side Effects Public Media, a public health news initiative based at WFYI. Contact Farah at fyousry@wfyi.org. Follow on Twitter: @Farah_Yousrym.

Farah’s reporting on sickle cell disease is supported by a grant from the USC Annenberg Center for Health Journalism’s 2022 Impact Fund for Reporting on Health Equity and Health Systems.