PHILADELPHIA — “We need a spray bottle, to spray her like a bad cat,” one of the emergency department workers said as Tolulope Afolabi protested that she was too woozy from pain medication to be hustled out of the hospital.

Afolabi, a 29-year-old Philadelphia nursing student, had come to Roxborough Memorial Hospital hours earlier that night by ambulance, delirious with a body-breaking pain that she described as like an SUV rolling over her back. She was met by emergency room staff who, after treating her with IV pain medication, tried to discharge her before she was even able to walk straight.

By the time of this March 2019 encounter, Afolabi had had so many similar encounters in hospitals that she discreetly captured it on an Instagram livestream, so friends would know how to find her if she needed help and so no one could say it hadn’t happened.

Afolabi was born with sickle cell disease, a blood disorder that affects every organ of the body and leads to unpredictable pain crises. Despite some 100,000 people in the U.S. living with sickle cell disease, the condition — which is most common in but not exclusive to people of sub- Saharan African ancestry — is under- resourced and misunderstood.

Until infant screening for the disease became routine in the 1980s, few people born with the disorder lived past childhood.

With early diagnosis and treatment, patients are increasingly living into adulthood, but when they age out of the pediatric system, they encounter a system that can be so harsh and unsupportive, some do all they can to avoid seeking care until their pain is unbearable. They struggle to book appointments with the few sickle cell specialists for adults, and are often accused of seeking opioids when they get emergency care.

Sickle cell patients and advocates say their suffering is largely the result of the systemic racism in society generally and health care specifically.

About 1 out of every 365 Black babies are born with sickle cell disease, which occurs when round red blood cells take on the C-shape of a farming sickle and grow hard and sticky, making them unable to carry adequate oxygen to vital organs.

Episodes of extreme pain, called a sickle cell crisis, require hospital treatment and are the hallmark of the disease. But many people with the disease are in constant pain or discomfort. Young adults often need joint replacements to repair bone deteriorated by the disease.

Children’s Hospital of Philadelphia’s sickle cell program has grown from about 325 patients in the early 1990s, before universal newborn screening, to over 1,000 patients. The sickle cell program’s hematologists work with primary care providers, social workers and specialists in eye, lung and heart care — organs most commonly affected by the disease.

But after childhood, sickle cell patients feel like they’re stepping into a health care void.

Or, as the director of Penn Medicine’s sickle cell program, Farzana Sayani, puts it, “And then everything falls apart when they become adults.”

Young adults may begin to experience different symptoms as they mature. They may be learning how to manage their health, without help from parents. At the same time, they are transitioning into an adult health care system that lacks wraparound services such as social workers and nurses who check in periodically.

After months of searching, Terri Booker, 37, found a hematologist she likes, and has a good care plan. But even so, she has constant pain in her legs, like they’re being squeezed by someone with an unrelenting grip. When the sensation climbs up her arms and her body starts to feel heavy, she knows a pain crisis is coming.

Booker, a Philadelphia lawyer, waits until she can bear it no longer to go to the emergency department. Too often providers don’t believe her, don’t listen and don’t know the right way to help, she said.

“It’s the physical, but also the mental anguish of knowing people don’t want to treat you, they don’t care what happens to you. The mental toll it takes on your mind and body is devastating,” Booker said. “Stress for a sickle cell patient is another trigger. If you’re constantly stressed out, you can’t get better, and then it makes you sicker.”

Booker has more mild symptoms than other people she knows, she said, but the disease has taken a personal and professional toll. Days after passing the Pennsylvania bar exam in 2012, Booker had a pain crisis that landed her in the hospital for a month and required another six months of recovery. She felt compelled to explain to prospective employers why her job search had been delayed and worried that divulging her medical condition might bias firms against her.

Earlier this year, Booker was laid off from her job when she was in the hospital for five days during a pain crisis. Even worse, she also lost her employer- sponsored health insurance.

“The first thing I did, I didn’t try to collect unemployment, I didn’t look for a new job, I applied for Medicaid,” she said. “Not having health insurance as a sickle cell patient is unimaginable. It’s not an option.”

Sayani joined Penn in 2013 to build up a comprehensive program for adult sickle cell patients. The program added 40 patients last year, about half of whom transitioned from pediatric care. The other half were adults in the community who were not regularly seeing a doctor, which she said speaks to the demand for more support.

One important change Sayani pushed for was an ER protocol, so that the sickle cell team is alerted when one of their patients comes to the emergency department. The new protocol has raised awareness about sickle cell in the health system’s emergency departments, but patients may still encounter skepticism if they go to a hospital that doesn’t know them.

Improving sickle cell programs could be a starting place for health systems to make measurable change in addressing health disparities and racism, said Alexandra Power-Hays, a pediatrician in Chicago who specializes in sickle cell disease and has written about health disparities.

“It’s really hard to ‘study’ racial disparities because a lot of the things are nebulous, but because sickle cell disease is a diagnosis there are objective measures,” such as how long patients wait for pain medication, she said. “It can be a good starting point to help shine that light overall on racism in medicine.”