Spotlight on Sickle Cell Disease: Life and Hope from an Advocate

What is it really like to live with sickle cell disease (SCD)? “Sweet and sour,” says Golie, who has lived with the disease for 42 years. “It does take things away from you … but as an adult, it’s helped me face daily life challenges from a new perspective.”?

People like Golie living with SCD face a double bind. Not only do they live each day with an inherited, life-long condition that affects their quality of life – but they must also navigate a system of inconsistent care that is not well-prepared to treat them, exacerbating an already difficult medical challenge.??

Golie, an SCD advocate from Houston, has experienced both sides of the equation. “Some days you have all the things everyone else manages – going to work, taking care of the kids, doing the shopping – all the while dealing with SCD. You are tired, lethargic, and in pain.”??

And then there is the system of care, which Golie describes as “difficult.” While some patients have access to a hematologist who specializes in SCD, more often patients encounter physicians who are not experts or hospitals that follow their own protocols. Many individuals living with SCD, especially young adults, are unable to find specialists to care for them or primary care physicians that understand the disease. “That can throw off the approach taken in your care,” Golie says. “First, SCD does not respond to a one-size-fits-all approach. And second, people need consistency in their care.”?

On top of that, many patients cannot access pain management when going to a hospital in crisis because of addiction concerns. This need for consistency, expertise, and stigma-free care is so critical and hard to come by that many, like Golie, stay with their pediatrician as long as they can rather than transition to adult care.??

What Golie describes is the case for many with SCD: a lack of viable treatment options compounded by a general lack of disease understanding, all the while having a condition where their personal experience is often discredited.??

While there is increased attention on the promise of new treatments, fundamental change needs to happen first. That’s why ASH undertook a systemic approach to improving the care landscape for SCD in conjunction with real people in the SCD community. The Office of Minority Health at the U.S. Department of Health and Human Services awarded ASH a two-year grant in 2020 for $2 million to support the creation of the Sickle Cell Disease Learning Community. The SCD Learning Community includes people living with SCD and their families, clinicians, researchers, and quality improvement specialists to ensure the patient experience is continuously improving.??

There are currently ten pilot hospital sites in the SCD Learning Community, and their focus is on improving the reliable use of disease-modifying therapies and increasing the use of co-developed pain management plans. A new effort is underway to focus on helping SCD patients transition from pediatric to adult care.???

These efforts are making strides in adopting evidence-informed practices, sharing best practices, standardizing care across participating sites, and creating a community of quality improvement leaders, with the ultimate goal of fostering a better and more reliable network of care for individuals living with SCD.?

“It’s been like this for way too long,” says Golie. “If the SCD system of care were a private company, it wouldn’t survive.” He says he has hope that as a result of bringing people from the community together with industry experts and physicians to change the system, living with SCD won’t be the double bind so many are caught in. Changing the system is a big challenge, but as Golie says, “one thing you learn having to deal with the challenge of SCD on a regular basis is that obstacles are there to be overcome.”?

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