Monica Brown is used to hearing people say she looks young for her age. She’s 41, but feels decades older – mainly because of her struggle with sickle cell disease, a genetic disorder that affects her blood.
“People say, ‘Oh you look so good,’ but they do not know what’s going on inside,” she said.
Brown’s chronic pain lands her in the emergency room every few weeks. A few years ago, she moved from California to Fort Worth, Texas to be closer to family. For three years, she spent hours on the phone nearly everyday looking for a sickle cell specialist within driving distance. No one was close by, and even doctors hours away told her they were booked out for years or didn't treat sickle cell disease.
“I was losing hope. I didn’t want to live,” she said.
Brown is one of an estimated 100,000 people living with sickle cell disease in the U.S. The exact number of sickle cell patients in the U.S. is unknown, because data on the genetic disorder is lacking.
Most states don’t have basic information like the number of sickle cell patients, the counties where they live or the type of health insurance they have. This means these patients’ needs aren’t taken into account when it comes to federal and state-level public health planning and budgeting.
The Sickle Cell Data Collection Program aims to address that data gap. The program is supported by a combination of federal funding and charitable donations to the U.S. Centers for Disease Control and Prevention Foundation.
The program, which began collecting data on California in 2015, has enlisted 11 states to start collecting aggregate data on sickle cell disease patients. So far, only two states have data available on the CDC website: California and Georgia. Indiana, Michigan, Wisconsin and Minnesota are among nine other states for which full data will be coming soon.
Initial findings from the Indiana program shared by the Indianapolis nonprofit Martin Center Sickle Cell Initiative in early August show that 1,659 residents live with sickle cell disease. A little more than half – 52 percent – are female, and 87 percent are Black. And 69 percent of sickle cell disease patients in Indiana are on Medicaid.
The CDC aims to expand the data collection program to determine how many people live with the disease, how the disease affects patients’ health and how researchers can improve medical treatments to extend and improve the lives of people living with sickle cell.
Dr. Julie Kanter, a sickle cell specialist in Alabama, said that having national data is crucial for improving patient outcomes.
“If we don't understand the disease, we're just not going to be able to make headway,” said Kanter, who also serves as president of the National Alliance of Sickle Cell Centers, which collaborates with the CDC on the data program.
Uninformed doctors can’t give patients the care they need
Sickle cell disease causes a patient’s red blood cells to change from their typical doughnut shape into a banana or sickle shape. Pain is the hallmark of this complex, multi-system disease. Brown described it like someone is stabbing her with a blunt knife all over her body nonstop for hours or sometimes days.
But there is a nationwide shortage of sickle cell specialists, and many non-specialized health care providers don’t know how to treat sickle cell disease. Patients like Brown who can’t find a specialist to treat their disease holistically instead rely on emergency department visits for relief during pain crises.
But patients can come to be labeled “drug-seeking,” which Brown saw written in the patient notes included with her medical records.
“If you don't have a hematologist it's really hard, if you're dealing with doctors who are like, ‘I'm gonna do this because that’s how I treat all of my sickle cell patients,’” Brown said. “We are all different. What works for this person might not work for me.”
Brown had close friends with sickle cell disease who chose to stay home and suffer through a crisis rather than go to the hospital and risk being mistreated. The majority of sickle cell patients in the U.S. are Black, and patients and researchers say systemic racism impacts the care patients receive.
The lack of national data contributes to inequities within the health care system. For example, sickle cell receives far less research funding compared to other illnesses, which slows progress in the development of new treatments and medical guidelines that physicians – including ER doctors and non-specialists – reference when making decisions regarding patient care. These are all problems the Sickle Cell Data Collection Program aims to address.
Access to data takes away ‘educated guesswork’
For other diseases like hemophilia – a genetic blood disorder that is less common than sickle cell disease and affects majority White patients – there are national registries, databases and clear guidelines that inform patient care.
Dr. Brandon Hardesty, a hematologist at the Indiana Hemophilia and Thrombosis Center which is one of the organizations working on the CDC data collection program, treats both sickle cell and hemophilia patients.
Hardesty said a national data system provides doctors with a bigger pool of health data so that when a patient shows up with less common symptoms, providers are more likely to find information that helps them determine the best treatment for that patient.
“So if a patient with hemophilia has a heart attack, you're able to look at the overall data from the system and say, ‘How common are heart attacks in hemophilia?’ and ‘What kinds of risk factors are present in this small portion [of people] that are having heart attacks?’” Hardesty said.
There is no database for sickle cell patients. So Hardesty said he has to do a lot of “educated guesswork” to provide the best care for his patients.
The CDC program will also collect information on the transitional period from pediatric to adult sickle cell care. There are more than 160 comprehensive care centers for children across the country, but once patients age out of pediatric care, they face a sickle cell care desert.
Kanter of the National Alliance of Sickle Cell Centers said some experts suspect that people born with sickle cell disease who’ve had a specific complication called dactylitis, which causes inflammation to the toes and fingers by age 2, will likely have more severe disease. But data is not available to explore this hypothesis. The CDC’s data program could help researchers find the answer.
“And the reason to ask a question like that is so you could say, OK, this is the group of patients that might need to consider stem cell transplant early on, or this is the group of patients that need more intensive therapies,” Kanter said.
Farah’s reporting on sickle cell disease is supported by a grant from the USC Annenberg Center for Health Journalism’s 2022 Impact Fund for Reporting on Health Equity and Health Systems.
This story comes from a reporting collaboration that includes the Indianapolis Recorder and Side Effects Public Media, a public health news initiative based at WFYI. Contact Farah at firstname.lastname@example.org. Follow on Twitter: @Farah_Yousrym.