Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. Approximately 10 percent of people with SCD in the US live in New York State, with the majority residing in New York City.
A new study led by researchers at NYU School of Global Public Health finds that SCD care is unevenly distributed across regions in New York State. New York City has the largest proportion of hospitalizations and Long Island has the highest charges for care, but hospital stays are longer in other areas with less access to specialized care, according to the study published in JAMA Network Open .
SCD affects the body's red blood cells. A genetic mutation, which is most prevalent among people of African descent, causes cells to become crescent-shaped. These cells block blood vessels and can lead to excruciating pain and progressive organ damage. Despite effective treatments, like the use of the medication hydroxyurea that can prevent pain crises, individuals with SCD still face frequent hospitalizations.
"A range of factors can influence care for sickle cell disease—whether people are experiencing multiple health conditions at once, whether hospitals have a dedicated sickle cell disease team with hematologists with specialized training, or have protocols in the emergency department for people who present in crisis," said study author Emeka Iloegbu, a public health scientist who recently earned his DrPH at the NYU School of Global Public Health.
"Understanding regional differences in sickle cell disease can help us to identify and address gaps in care," said study author Emmanuel Peprah, associate professor of global and environmental health and director of the Implementing Sustainable Evidence-based interventions through Engagement (ISEE) Lab at NYU School of Global Public Health.
SCD patterns in New York
To assess regional differences in SCD hospitalizations across New York State, the researchers used publicly available data from the Statewide Planning and Research Cooperative System (SPARCS), an administrative database that captures inpatient discharge records from healthcare facilities. Analyzing data from 42,271 SCD hospitalizations in New York State from 2009 to 2022, the researchers looked at the length of hospital stays, total charges, total costs, severity of illness, and risk of death.
Statewide, most people hospitalized for SCD were Black (83 percent), consistent with the racial distribution of SCD in the US. Young adults (18-29 years old) made up the largest proportion of hospitalizations (40 percent) followed by those aged 30-49 years (32 percent). These patterns align with prior studies showing higher healthcare use and elevated risk of death among young adults with SCD, which may, in part, reflect shortcomings in the transition from pediatric to adult care.
In addition, hospitalizations for severe illness grew over time; those classified as "major severity" increased from 13 percent in 2009 to 27 percent in 2022, while those classified as "major risk of mortality" increased from 3 percent to 13 percent over the same period. These patterns coincided with the COVID-19 pandemic, and research shows that people with SCD experienced disruptions in care and higher rates of severe COVID-19 complications and hospitalization compared with the general population.
Differences across the state
The researchers found significant variation in SCD hospitalizations across regions in New York State. Central New York had the longest average length of stay (6.3 days) followed by the Hudson Valley (6.2 days).
New York City, which had a shorter average length of stay (5.5 days), accounted for the largest volume and proportion of hospitalizations in the state, but had fewer patients (5 percent) at major risk of death. In contrast, Long Island had the highest proportion of hospitalizations with a major risk of death (10 percent) and the highest total charges for hospital stays.
"This suggests a high sickle cell disease burden on Long Island," noted Iloegbu. "Both Long Island and New York City had high costs, which may suggest that these regions are managing clinically complex cases, or have differences in specialized care or care-delivery practices."
Better care and outcomes
These insights about regional inequities in SCD can inform tailored approaches to improve care and outcomes.
"Geographic differences in hospitalizations and outcomes for sickle cell disease point to the need for region-specific strategies," said Peprah.
At the hospital level, improving care could look like strengthening access to specialists, smoothing the transition from pediatric to adult care, and educating providers about effective therapies, all of which would reduce the reliance on emergency departments.
However, the researchers note that addressing these disparities will likely require broader systemic reforms, including improving the availability of effective therapies, prioritized funding for SCD research and care, and updated public health policies. For instance, New York was the first state in the country to implement a newborn screening program for SCD—more than 50 years ago. But as migration patterns have shifted, people are coming to New York from countries with high burdens of sickle cell disease but were not screened as newborns.
"Instead of being screened and monitored, the entry into the health system for these individuals is the emergency department," said Iloegbu. "Knowing that certain regions of New York have increased severity or longer lengths of stay could contribute to the need for improved surveillance, especially at the patient level."
Additional study authors include Jonathan Odumegwu of NYU School of Global Public Health and Baylor University; Joyce Gyamfi, John Patena, John Patena, Xinyu Wang, Etornam Amesimeku, and Prince Amegbor of NYU School of Global Public Health; Andrew Campbell and Folasade Ogunlesi of George Washington University School of Medicine & Health Sciences; and Uju Ozoh of the University of Lagos.
