For three excruciating hours, Merlene Smith-Sotillo witnessed her daughter Cathyann writhe in agony in a hospital emergency room. The pain, her daughter later told her, felt as if a truck was rolling over her body, crushing every bone.
Cathyann was in her late 20s at the time, and the sickle cell anemia pain crisis she was enduring should have been attended to immediately. The hospital staff, however, did not think it was important enough to treat her ahead of other patients. Instead, they kept asking her to enumerate her pain level on a scale of 1 to 10.
Smith-Sotillo and her husband, a Trinidadian couple living in Queens, didn’t know that they carried the sickle cell trait until their children began grappling with the genetic condition. Cathyann, now 52, has survived a life-long battle with the disease. Her brother Syd, on the other hand, died at age 30, after experiencing four major organ failures associated with the disease. Unlike his sister, Syd was rarely in hospitals. When he passed, he was in the process of completing a degree in Business Administration at the Interboro Institute. He’d also started a company to help others launch businesses through prepaid legal support, grant writing and other services.
Syd, in fact, helped his mother launch the Sickle Cell Awareness Foundation Corp. Inc. . Since his death, Smith-Sotillo has made it her mission to get the State of New York to introduce comprehensive centers for sickle cell patients. But legislators’ support for the bill has been lagging for almost 10 years.
Sickle cell disease is an inherited blood disorder that affects an estimated 100,000 Americans, approximately 10 percent of whom reside in New York. It strikes communities of color almost exclusively, with the impacted majority being of African American descent.
The disease causes frequent and severe pain crises, along with numerous life-threatening health complications like strokes, organ failures and infections. Sickle cell is also the most expensive disease to New York State Medicaid. Each year, the state spends $15,000 per patient – almost 50 percent more than what is spent on an HIV patient. A 2021 report found that, nationwide, Medicaid covered 66 percent of hospitalizations in 2004.
Thomas Moulton, a pediatric hematology and oncology specialist who took care of sickle cell patients for over 30 years in the Bronx and who now works at a pharmaceutical company, noted that New York State does not collect any statistics on sickle cell disease, despite being the second most populous state for the condition. North Carolina, which ranks eighth in sickle cell population, has allocated $4 million to sickle cell research and treatment. California, which ranks sixth, has allocated $15 million to improve sickle cell care. Funding in New York State, in contrast, has decreased over the last 20 years, from $500,000 to $170,000.
Moulton compared the sickle cell budget to that of cystic fibrosis, a similarly life-threatening genetic disease that primarily affects white people. Cystic fibrosis has less than one third of the sickle cell patients nationwide but receives 3.5 times the National Institutes of Health funding and 400 times the private support, revealing a racial disparity in healthcare resources.
“If these were white people who died consistently by the age of 40, you bet they would be fighting tooth and nail to find the funding,” he said.
Moulton, who also sits on the Sickle Cell Thalassemia Patients Network advisory board in Brooklyn, helped craft the 2012 Sickle Cell Treatment Act with the input of sickle cell patients and their families, including Smith-Sotillo, who originated a similar measure in 2010. The bill, first developed by Smith-Sotillo in 2010, would provide $3 million to fund eight comprehensive sickle cell centers and one coordinating center throughout New York. The legislation, however, has not passed. Most recently, government officials divided it into three separate bills in hopes of making it more passable for the 2021-2022 session.
So far, only one bill has passed, which requires hospitals to distribute informative pamphlets to patients at risk of developing sickle cell disease. Still with New York State are the other two bills. These would establish the Sickle Cell Treatment Act, the core demand of the sickle cell community, and create a sickle cell education program. If implemented, the state would designate five sickle cell centers of excellence, with a team of specialists to treat symptoms, and ten outpatient treatment centers.
Pain from sickle cell crises is often underestimated, as was the case when Cathyann was in the emergency room. With limited access to specialized providers, almost all patients rely on the emergency room to relieve their pain, where they often wait 25 percent longer than other patients to get attention. Once they finally do, healthcare professionals are quick to dismiss patients’ pain, as many workers also lack the education about the rare disease.
Too often, sickle cell patients are accused of seeking drugs or labeled addicts by nurses or doctors because they require pain medication. This stigma is worsened by the opioid crisis, which caused ER doctors to be extremely reluctant to prescribe pain pills. This can result in inadequate care and pain management for sickle cell patients.
To avoid stigma and mistreatment, many sickle cell patients avoid going to the hospital to seek the care they need, which will inevitably worsen their condition, said Moulton.
Some sickle cell patients are afraid of the “Black disease” label, rooted in its disproportionate impact on Black Americans. In New York, 89 percent of babies born with sickle cell disease are of African American descent, the Center for Disease Control said in a 2008 report.
The “Black disease” characterization, however, is misleading. The affliction is also found in people of Latino, Indian, Middle Eastern and Mediterranean heritage.
Donnette Carroll, the former president of the Sickle Cell Thalassemia Patients Network and a sickle cell patient herself, has seen families from Indian, Italian and Latino backgrounds choose not to acknowledge they had sickle cell disease or hid their condition due to the negativity and stigma surrounding it.
“I guess they did not want to be associated with a so-called ‘Black disease,’” said Carroll, “but that’s where the disparity comes in – it’s because of this label.”