Last November, just as the novel coronavirus was beginning its deadly trajectory through China, the sickle cell community in the U.S. was celebrating. Two potentially transformative drugs for sickle cell had just been approved by the FDA and clinical trials involving cutting-edge gene therapies were well underway.
“One of my colleagues called it ‘The November to Remember,’” recalled Biree Andemariam, chief medical officer of the Sickle Cell Disease Association of America and founding director of the New England Sickle Cell Institute at the University of Connecticut. “Literally, just before the pandemic hit, we had infused our first patient with one of the new drugs. And then everything stopped.”
While the coronavirus is affecting everyone, it has hit those with sickle cell particularly hard. Not only did the pandemic temporarily stop clinical trials and the introduction of new drugs, it made accessing care for sickle cell — which can be challenging even in ordinary times — far more difficult. Because sickle cell damages the spleen, people with the disease are considered immunocompromised and at high risk for serious complications from Covid-19.
As Covid-19 quickly grew into a public health crisis, trips to the emergency room and hospital for severe pain crises suddenly became terrifying. For many, they remain so.
“If I get sick, I don’t want to get admitted,” said Marvin Banks, 50, a radio host and DJ from Red Wing, Minn., whose sickle cell triggered hospital stays about three times a year before the pandemic. These days, Banks is doing everything in his power to avoid a hospital stay. “I’ve been sick for two days but I’m going to stay home and fight this,” he said.
Many physicians are concerned that fear is keeping patients like Banks from seeking the care and pain management they need. With a disease as serious as sickle cell, that can lead to dire consequences.
“It was concerning to us that people were not coming in,” said Kim Smith-Whitley, who directs the comprehensive sickle cell center at Children’s Hospital of Philadelphia. “People were trying to stay home and manage pain or manage cough and fever, which is not wise.”
Others were trying, and failing, to get to medical facilities. “I had one family trying to get to the clinic and the bus wouldn’t stop for them because they didn’t have masks,” Smith-Whitley said. “They were devastated.”
Some clinicians have started relying heavily on telemedicine, hoping to keep patients out of emergency rooms where those with sickle cell often seek care — but where they also might be exposed to the virus. Others have helped patients arrange safe transport to clinics or set up ways for them to bypass waiting rooms.
“We don’t want them not getting care because they’re afraid,” said E. Leila Jerome Clay, a pediatrician who directs the sickle cell program at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida.
From the start, physicians were worried about increased hospitalization and mortality from Covid-19 early on because people with sickle cell disease have a higher mortality from the common flu and because symptoms of one of the leading causes of death in sickle cell disease, acute chest syndrome, overlap closely with those of Covid-19.
Andemariam was on the alert for her patients early on, partly because of regular global calls she holds with sickle cell physicians in other countries, including Italy. “We got the on-the-ground description of what they were seeing, that otherwise healthy kids were coming in sick and dying,” said Andemariam, who advocated for patients to wear masks long before the Centers for Disease Control and Prevention did.
It appeared, by many accounts, that patients with sickle cell were avoiding much-needed medical care — and in some cases, were falling dangerously ill as a consequence. Jerome Clay said the pandemic has caused her to be more aggressive and proactive with treatment, particularly when patients appear with acute chest syndrome, which can look like Covid-19 on a chest scan.
“If they come in with acute chest, there’s no reason to wait to have a transfusion,” she said. Many of the deaths of sickle cell patients that occurred during the outbreak in hot spots, she said, may have occurred because patients weren’t treated aggressively early on, or because Covid infections went undetected because they appeared to be episodes of acute chest syndrome.
Physicians are still trying to determine whether patients with sickle cell are dying at higher rates from Covid. A registry of Covid-19 cases in sickle cell patients found a 7% mortality rate, alarming given that the average age of patients in the registry was below 40. (Many pediatricians told STAT their patients who have contracted Covid have generally done well; many were asymptomatic and had Covid-19 infections detected during routine testing.)
As the evidence comes in, patients and caregivers remain on high alert. Demetria Germaney Hampton, whose 12-year-old son, Jeramyah, has sickle cell disease, said she watched with fear as the virus spread worldwide in “a domino effect.”
“I pulled him out of school before they even closed the schools here,” said Germaney Hampton, who lives in Eau Claire, Wis. “I said, I’m sorry, but we’re chained to the house.”
When the coronavirus started tearing through the Northeastern U.S. in March, Dima Hendricks immediately locked down, not leaving her home for a month and a half. “I told my physician, ‘Look, I’m not coming in,’ and she said, ‘That’s fine because I’m not coming in either.’”
For Hendricks, a 38-year-old therapist who has a history of needing to be hospitalized about four times a year for bouts of pneumonia and acute chest syndrome, telehealth has been a godsend.
“I feel like it’s easier to make appointments and get refills. It’s more responsibility, but I also feel more involved,” she said.
The fact that telehealth visits are now reimbursed may be one of the great legacies of the pandemic, Andemariam said. “It’s a major improvement when patients don’t have to take one, two, three buses to come in to see me,” she said.
But physicians say changes in health care related to the pandemic are also exacerbating an existing gap in sickle cell care. “Those who were already getting good care are getting better care, and those who were without care are suffering more,” Andemariam said.
Clinicians are most concerned about patients without medical homes, including those who live in rural areas without experienced sickle cell providers. “Where we are, they honestly don’t know about sickle cell at all,” said Germany Hampton of Eau Claire. “When I took Jeremyah to the local hospital for pain, the nurse literally had to look up sickle cell on Google.” (The family now travels about two hours to Minneapolis for treatment that can’t be conducted remotely or when Jeremyah needs to be in the hospital or ICU.)
Also of deep concern: young adults who have aged out of sickle cell treatment programs for children, and are having trouble finding care as adults. The pandemic stands to make connecting with a new provider all the more difficult.
“Those patients are at highest risk of death,” said Mohammed Sani Bukari, an assistant clinical professor at UCSF Fresno and hematologist oncologist who recently received a $75,000 grant allowing him to open an adult sickle cell clinic in California’s Central Valley; previously, the area’s more than 500 adult sickle cell patients had to drive hours to Los Angeles or San Francisco for care.
Bukari said he continues to see anxiety and reluctance to seek care among patients, even as new protocols make it safer for patients to return. “I have noticed more patients trying to manage their care at home. I tell them, don’t think because of coronavirus, you can’t go to the ER,” he said. “The last thing I want is for them to not go and have complications.” (Andemariam agreed it is important for patients to get to an ER for emergency care or if they can’t reach their provider directly; the disease can cause sudden and unexpected death.)
Bukari, whose patients often have to drive hours to see him, is relieved he can use telemedicine to see patients and prescribe narcotics remotely for pain crises. “We want them to have what they need,” he said. But he’s also glad to see an uptick in in-person visits.
“I had a patient come in who was not as bubbly as she usually is. She had lost a friend to Covid. In-person visits allow you to assess for things like depression,” he said.
Like so many in this pandemic economy, sickle cell patients and caregivers are losing work and livelihoods too. Germany Hampton lost her job as a customer service representative in December, before the pandemic took hold. She hasn’t been able to find a replacement job. “Every job here is for essential workers,” she said. “I can’t put myself in the line of fire and then come home.”
Marvin Banks still works as a radio host for an online radio station, but he’s stopped taking the DJ gigs that were a major part of his income. “I don’t want to be around big crowds,” he said.
Angie Uko, 21, who lives near Minneapolis, left her job as a care aide in a nursing home in June when it became too stressful. Uko did end up getting infected with Covid in July, though she’s not sure how she was exposed. Luckily her case was relatively mild: She had pain, fever, a sore throat, and then panic attacks.
“With my sickle cell, I knew if I didn’t take care of it, I could have gotten worse and ended up in the hospital,” she said.
Uko is still looking for a job, while also trying to find a new physician since she’s aged out of the pediatric care program she was in. “I was supposed to have a new doctor by now, but I haven’t been able to do that. I haven’t been able to get my blood drawn,” she said. “Covid is setting a lot of things back.”
The pandemic, Bukari said, has amplified the many problems his patients already had, from not having reliable transportation to medical visits, to losing their insurance or having difficulty getting approvals for treatment. “If you lose anything now, it just takes longer,” he said.
Those challenges add to other long-standing barriers to care for patients with sickle cell, who are largely of African descent.
A legacy of structural racism has left many sickle cell patients with insecure housing, poverty, and fewer employment options that carry health insurance, experts note. Many say structural racism is also apparent in the inadequate research funding for the disease: While cystic fibrosis affects one-third as many patients as sickle cell, it has received far more research funding, been the topic of more published studies, and been the target of more FDA-approved drugs.
“There may be no population of patients whose health care and outcomes are more affected by racism than those with sickle cell disease,” physicians Alexandra Power-Hays and Patrick McGann wrote in a recent editorial in the New England Journal of Medicine.
Many patients seeking relief for sickle cell crises say the last thing they need during the pandemic is to be turned away still in pain because providers see them as drug seekers. “That’s what we deal with. They think we’re seeking a drug high,” said Banks, the radio host. “It’s clearly discrimination. If it was someone who came in with cancer, they’d give them pain treatment.”
At his rural ER, Banks said he has been turned away and rushed out so often without getting proper relief, he now travels to a different facility where the staff has more experience with — and more compassion for — those with sickle cell.
“I drive an hour,” he said. “While I am in pain.”
In some communities that saw protests over the killing of George Floyd and other Black people by police, a painful and unexpected outcome has been the loss of pharmacies that tend to stock medications, like hydroxyurea and dilaudid, that sickle cell patients rely on. As Smith-Whitley, who works in Philadelphia, put it: “When communities went up in flames, so did the pharmacies that had the medications people needed.”
With the pandemic forcing people to settle into a new type of routine, many patients are finding ways to resume their medical care and get started with the newly approved drugs, Oxbryta and Adakveo. Clinical trials are back underway too, said Andemariam, though they are slowed by having to adjust enrollment and protocols due to the pandemic, especially for treatments that temporarily compromise immune systems.
Meanwhile, fall is ushering in a new host of worries and hardships. Many parents of children with sickle cell, like Germany Hampton, are choosing to keep their children at home instead of sending them to school or day care. “I don’t want to cut him off from the world,” she said of her son. “But the government has no idea what they’re doing, and they’ve opened up all the bars and restaurants, so I have to do my own thing.”
Some parents aren’t able to do so, which introduces another set of challenges.
“Can you imagine sending your toddler with sickle cell to day care?” said Jerome Clay. “But these parents have to work, and they are really afraid.”
She and other physicians are bracing for what happens next as well. “Having asthma, sickle cell, and Covid, and adding the flu, and then the winter brings on pain crises,” she said. “I am a little bit apprehensive about what happens when winter comes.”
But patients with sickle cell also said the pandemic seems to be ushering in more compassion. “The discrimination I was feeling before, the racism, I’m not feeling it now,” Hendricks said. “When I was sick before, people used to say, ‘You’re faking it.’ Now they say, ‘Why don’t you go get some rest?’”
Hendricks said she sees “able-bodied privilege” as being akin to white privilege. “Before Covid, people who were able-bodied and walking in wellness didn’t understand what it was like to walk in sickness,” she said. “I really feel like the pandemic has shined a light on all of this.”
Andemariam agrees. “The pandemic began to more openly validate the feelings that have been described by the sickle cell community for so long that have gone unheard,” she said. “It’s a disease where there is so much stigma, so much isolation, and questions like what will the future look like, and how early will I die? For once, people with sickle cell feel exactly the same way as everyone else.”