Sickle cell disease patients at Long Island hospitals had highest risk of dying, study finds

People hospitalized for sickle cell disease on Long Island are more likely to be admitted with life-threatening symptoms than any other New Yorkers, a study released Friday shows, and public health experts said that indicates there is not enough patient and physician awareness in the region of treatments that can significantly improve patients’ lives.

“What it suggests to us is there are not a lot of specialty services on Long Island to address the sickle cell disease population,” said Emmanuel Peprah, an associate professor in New York University’s School of Global Public Health and senior author of the study, which was published in the journal JAMA Network Open.

About 10% of people hospitalized with sickle cell on Long Island between 2009 and 2022 faced a “major risk of mortality,” by far the highest proportion of any region and nearly twice the statewide percentage, researchers found.

Long Island also had the highest average hospital charges, nearly $60,000, compared with under $39,000 statewide. That, researchers said, could be in part because hospitals were handling more complex cases.

Sickle cell disease, a genetic mutation that causes red blood cells to become crescent-shaped and block blood flow, has been transformed in the past 50 years from a condition in which most patients didn’t live past 10 years old to one in which many people have vibrant, active lives and normal life expectancies, said Dr. Edward Donnell Ivy, chief medical officer for the Sickle Cell Disease Association of America, which was not involved in the study. The primary reason life expectancy is still, on average, according to federal statistics, more than 20 years lower than typical is a lack of awareness of and access to appropriate treatments, Ivy said.

“With access to proper care, the majority of sickle cell patients can live a long and productive life,” he said.

Without it, Ivy said, people with sickle cell often endure the type of severe pain flare-ups and long hospitalizations that he had growing up, until in 1995 when he was prescribed the then-experimental medication hydroxyurea that dramatically reduced his pain and revolutionized his life

Other symptoms and complications of sickle cell can include chronic pain, fatigue, blood clots, swollen joints and severe damage to multiple organs. About 100,000 Americans have sickle cell disease. More than 90% are Black.

Some people with the disease have been cured with bone marrow transplants or, more recently, gene therapies.

But, Ivy said, “If a sickle cell patient isn't interacting with a doctor that's aware that this therapy is available as a treatment, they're not going to be offered that as a treatment. And so we have to figure out, ‘How do we ensure that more sickle cell patients have access to knowledgeable providers?’”

Despite the advances in sickle cell treatment, there was a spike in patients hospitalized with “major severity of illness,” from less than 13% statewide in 2009 to more than 27% in 2022, the study found. Part of the reason was COVID-19, which can exacerbate sickle cell symptoms and which, early on, caused patients to delay seeking care, Peprah said. But the upward trend, which started before the pandemic, also may be rooted in other factors, including access to care, he said.

Although New York has required sickle cell screening for newborns for more than 50 years, and many children receive high-quality care, there often is a fall-off in treatment once a patient becomes an adult, Ivy said. In addition, he said, many immigrants come from countries where there is no screening, so they may not be diagnosed early in life, arriving at the hospital with advanced symptoms. Long Island has a large population of immigrants from Haiti, where about 1 in 120 newborns has sickle cell and screening is uncommon.

In areas where there are fewer sickle cell patients — only about 2% of the hospitalizations examined in the study were on Long Island — there typically are fewer providers with sickle cell expertise, Peprah said.

In addition, Long Island’s sickle cell population is on average older, researchers found. Older patients tend to have more complications, because the disease often damages organs over time, which likely contributes to them arriving at hospitals with more severe symptoms, said Dr. Banu Aygun, who heads the sickle cell program at Northwell Health’s Cohen Children’s Medical Center in New Hyde Park.

Even when adult patients have access to physicians with sickle cell expertise, they are more likely to lead healthier lives if they can get care in a dedicated sickle cell center, Peprah said in a Zoom and email interview. Sickle cell patients typically see multiple doctors and nurses, and they do better when those providers coordinate care, he said.

Northwell has long had a specialized sickle cell center at Cohen, which treats patients through age 21. In August, Northwell opened an adult sickle cell center in Rego Park, Queens, that serves primarily New York City and Long Island patients.

Since the center opened, there has been a more deliberate effort to ensure a smooth transition from pediatric to adult care, said Dr. Douglas Gladstone, chief of classical hematology, which includes sickle cell disease, and malignant hematology at the Northwell Cancer Institute. In addition, Dr. Edward Zhou, a hematologist and oncologist at the center, said he has traveled to different Northwell hospitals to talk with doctors, including in emergency departments, about sickle cell disease, including how to appropriately identify and manage symptoms.