When COVID-19 arrived in New York City, Tartania Brown became scared.
She had good reason: Brown is immuno-compromised due to sickle cell disease, a genetic blood disorder that affects about 100,000 Americans, most of them Black.
By July, Brown had heard of two local friends with the disease who had contracted COVID-19. One was still recovering after two months in the intensive care unit at NewYork-Presbyterian Hospital in Manhattan. The other had passed away.
“I’m still scared,” said Brown, who is a palliative care physician at the Metropolitan Jewish Health System in Brooklyn.
Her concerns have only recently been acknowledged by the U.S. Centers for Disease Control and Prevention (CDC). In late June, the CDC added sickle cell to a growing list of underlying conditions that put people at a higher risk for severe COVID-19-related complications.
Months before, hematologists had warned of overlap cases being potentially fatal.
The New York State Department of Health does not track sickle cell cases and their outcomes. But recently published crowdsourced data has corroborated those fears, suggesting heightened fatality rates for sickle cell patients who contract COVID-19.
The issues go much further, advocates, doctors and patients say, for the roughly 10,000 people living with sickle cell in New York State, the second-highest cluster of the disease of any U.S. state behind Florida. Roughly 85% of those with sickle cell in New York live in the city, experts say.
Across the city, sickle cell patients without COVID-19 have also been struggling to refill life-saving prescriptions and get needed transfusions while in quarantine. Others find themselves avoiding medical offices to lessen the chances of coronavirus infection.
Layers of Risk
Early data, collected nationwide by a collaboration of hematologists at the Medical College of Wisconsin in Milwaukee, indicate a 7% mortality rate among patients with sickle cell who contract COVID-19. Roughly 20% experienced “severe” to “critical” versions of the disease.
The data was published in a July 8 study, whose authors called the findings “alarming,” particularly in comparison to case-fatality rates in the population-at-large.
Patients with the sickle cell can suffer from periods of intense pain when malformed blood cells move throughout the body. These “sickling” episodes can also cause multisystem organ damage over a lifetime. As a result, the disorder predisposes patients to other underlying conditions on the CDC’s list, such as chronic kidney disease.
One group of hematologists expressed “significant concern” of increased complications should sickled blood cells flow into lungs affected by COVID-19. The event, known as Acute Chest Syndrome (ACS), can be fatal on its own.
“In theory, it should be deadly,” said Dr. Thomas Moulton, a hematologist who has long advocated for greater awareness of the blood disorder in New York City. “Sudden death has always been a part of sickle cell disease because of Acute Chest Syndrome.”
The CDC has yet to comment publicly on the timing of the update to its list, which already included other preconditions like serious heart conditions, obesity and Type 2 diabetes. The CDC did not respond to THE CITY’s requests for comment.
Montefiore Medical Center in the Bronx has treated 40 patients — the majority of them adults — who had both COVID-19 and sickle cell, said Dr. Caterina Minniti, director of Albert Einstein College of Medicine and Montefiore Health System’s Sickle Cell Program.
Four of those patients died at Montefiore. A larger dataset compiled by Montefiore that includes cases reported from Mount Sinai as well as hospitals in Boston, Detroit and Chicago bears out a similar mortality rate — close to 10%. None of them, Minniti said, has been taking Hydroxyurea, a drug that reduces both frequency of painful crises and the need for blood transfusions.
Increase in Deaths Reported
Whether a sickle cell patient dies from COVID-19 may also depend on whether they have access to doctors experienced with the disorder’s many side effects.
“You have to consider, this is a rare disease. So what happens is that a smaller hospital — their knowledge is going to be much less, just by default,” Minniti said. “I do think that smaller hospitals may have difficulty in providing comprehensive and tailored care patients with sickle cell disease need.”
Minniti also noted a higher mortality rate in sickle cell patients overall. By June, Montefiore doctors had already seen as many sickle cell deaths as they normally see in a full year, she said.
‘Could it be that they had painful episodes and then they just never called us?’
The correlation is still unclear, but doctors like Minniti are uneasy with what they still don’t know, in part because of a decrease in visits by sickle cell patients since the pandemic erupted.
For blood disorders like sickle cell, an inability to receive a transfusion can be dangerous on its own: The blood of patients who postpone transfusions can turn anemic, making them susceptible to a heart attack or stroke.
Dr. Abena Appiah-Kubi, a pediatric hematologist at Cohen’s Children’s Medical Center in Queens, said that many hospitals had noticed a drop in appointments made by their sickle cell patients.
“I don’t know if there were patients that could have had [COVID-19] and we just never knew about them,” Appiah-Kubi said. “Could it be that they had painful episodes and then they just never called us?”
Called a ‘Faker’
Ginger Davis, who has sickle cell and works with the Sickle Cell Thalassemia Patients Network in Brooklyn, said that sickle cell patients were “paranoid” when the pandemic arrived in New York.
“We spent a lot of time trying to soothe people, trying to calm them down,” said Davis, who called the CDC’s early failure to add sickle cell to the underlying conditions list “egregious.”
Patients were already wary of hospitals, Davis said. Many report experiencing discrimination by hospital staff who suspect them of faking their pain.
“I’m never gonna forget the first time I was called a faker. I was still in pediatrics,” said Aisha Braimah, 33, who lives in The Bronx.
In April, she experienced “unbearable” chest pain, which turned out to be coronavirus. Braimah was treated for 11 days at Albert Einstein College of Medicine, one of the few hospitals she trusts, she said.
“We wait and wait and wait — and we refuse to go to the hospital because we don’t wanna be mistreated,” Braimah said. “I honestly believe it’s one of the reasons why we die so much.”
‘A Very Scary Time’
Both diseases have disproportionately impacted New York’s Black communities. Black people are dying of COVID-19 at twice the rate of white people in the city and they are 24 times more likely to suffer from sickle cell disease compared to white people.
“I don’t even think people remember us,” said Anthony Guobadia, who is Black and has sickle cell disease.
“It’s one of the most prominent diseases affecting the Black community,” added the retiree, who was a doctor in Haiti and a physician assistant in the U.S. “They just, as usual, just ignore it. It’s a kind of racism when it comes to sickle cell.”
Tartania Brown said she struggled to get a blood transfusion for months, relying on a cache of extra pain medication. In June, she received her first transfusion since the pandemic began.
“It was like we were left in the wind,” Brown said. “Through March you were stuck. The doctors weren’t really trying to see us — they shut the hospitals down unless you had COVID. People were struggling — really bad — with the disease, with managing pain and complications.
“It was a very scary time.”
Has the coronavirus pandemic made it difficult for you or someone you know with sickle cell to access treatment? Reach out to us at email@example.com, or by phone at 203-543-1094.
Luca Powell is a J-Corps fellow from the Craig Newmark Graduate School of Journalism at CUNY, which is participating in “MISSING THEM,” THE CITY’s ongoing collaborative project to remember every New Yorker killed by COVID-19.