Michelle Parr’s sickle cell disease went undiagnosed for 25 years. Her diagnosis came when she was pregnant with her first daughter.
“Dealing with all the things that I've dealt with in my life living with sickle cell and the years that I didn't even know that I had sickle cell disease, it brought me to a place where I want to be an advocate, to let people know that it's first and foremost to get tested to see if you are living with a trait,” Parr said.
Access to consistent, quality care for the disease allowed her to defy the odds.
“I have been blessed to be on this earth for 60 years,” she said. “The average life expectancy for an African American living with sickle cell in America is 42 if you're a male and 48 if you're a female. It's indeed a blessing to still be here. I feel as though I have much more work to do.”
Parr’s role as a community health worker at the Children’s Sickle Cell Foundation, Inc., based in Pittsburgh, allows her to help others manage their diagnosis. She answers calls on the foundation’s newly launched toll-free hotline to connect unaffiliated patients to care.
What is sickle cell disease?
Sickle cell disease occurs when red blood cells are crescent- or sickle-shaped instead of disc-shaped, according to The National Heart, Lung and Blood Association. This means that the red blood cells can not bend or move easily and can block blood flow.
Pain crises are common with sickle cell disease as the blocked blood flow can cause severe pain that often requires medical attention. Individuals living with sickle cell disease may also have other serious health issues, such as chronic pain, stroke, lung issues, eye issues, infections and kidney disease.
The disease is a lifelong illness that results from a gene mutation and is often diagnosed at birth.
More than 100,000 Americans live with sickle cell disease. The disease is most prevalent in Americans with African descent or Americans that identify as Black.
According to the Sickle Cell Disease Association of America, treatment of complications caused by the disease often include antibiotics, pain management, intravenous fluids, blood transfusions, surgery and psychological support.
And that care should come from a specialist, according to Dr. Sophie Lanzkron, a professor of medicine and the Division Director of Hematology at the Sydney Kimmel Medical College of Thomas Jefferson University and a co- principal investigator on the Health Resources and Service Administration (HRSA) funded grant at Sickle Cell Improvement in the Northeast Region through Education, or SiNERGe, a collaborative of community-based sickle cell organizations and clinical sites in the Northeast United States.
“I cannot express the point enough that everyone living with sickle cell disease should be being seen by a sickle cell expert,” said Lanzkron.
How the sickle cell hotline works
A partnership between the Children’s Sickle Cell Foundation, Inc. and SiNERGe produced the hotline. It’s funded by an HRSA grant.
The pilot launched this summer in Pennsylvania, ahead of September, which is National Sickle Cell Awareness Month. Organizers hope it will spread to neighboring states if this trial phase goes well in the Commonwealth.
Patients can call 1-855-SICKLE8 (1-855-742-5538) or text “sickle” to 66866 to connect with community health workers like Parr. Community health workers help patients find care and develop treatment plans.
Toll-free sickle cell hotline
1-855-SICKLE8 (1-855-742-5538)
text “sickle” to 66866
Unaffiliated sickle cell patients
The hotline’s target demographic is unaffiliated patients.
“The unaffiliated patient is someone who has seen their hematologist or their primary care physician in more than a year, and we really want them to call and to connect with a community health worker who has been trained to answer the questions, get them into care in the closest comprehensive sickle cell center to them,” said Andrea Matthews, founder of the Children’s Sickle Cell Foundation, Inc.
Matthews’ son lives with sickle cell disease. She knows the importance of regular, high- quality care and of having a support system.
“We noticed that there were a lot of supports for kids with cancer in the same hematology oncology unit, but there were very few supports for children with sickle cell disease, and so that didn't sit well with me. There was a gap in services at that time. There were a lot of services for adults with sickle cell, and not any for children or families,” said Matthews, who is from Pittsburgh.
Her son Jonathan is now 25 and as she says, “living well with sickle cell.”
But, the first few years of his life put strain on the family. The disease made him very sick from the ages of two to six.
“We were in the hospital every couple of months for six days at a time, it was very difficult on me as a caregiver, because I had to leave work and be with him,” she said.
Young people are most likely to fall into the unaffiliated patient category, according to Lanzkron.
“The period of transition from pediatric to adult care is a time in which patients fall out of care,” Lanzkron said. “In fact, they probably fall out of care before they reach transition so in the teenage years, particularly people who have less severe disease, who don't need that intensive sort of follow up, are the ones who are most likely to fall out of care during the late teenage years, early adulthood right before transition.”
Going without care for sickle cell disease is dangerous for any patient, but especially for the younger demographic.
“The highest rate of mortality and morbidity is between 18 and 26. They need to know that they are in charge of their care,” Matthews said.
Community health workers offer knowledge and resources, but often a support system as well. Most of the workers live with sickle cell disease themselves.
“Having first hand experience, living with the pain and the fatigue and other complications that I face, I can better connect with that person who is dealing with the disease,” Parr said. “I find once I reveal to the person that I'm speaking to that I too, suffer from sickle cell, they feel more comfortable in talking to me, and they're able to feel as though, ‘Hey, this woman has it. She's doing okay. I'll be okay too.’”
Matthews’ mission is personal. She sees the hotline as a way to help people like her son and families like hers.
“Until there is a universal cure, we need to ensure that people are gaining access to care, continuing with their care, and staying connected so that they can partake in clinical trials,” she said.
