Sickle cell disease may be something you’ve heard of, but it’s likely a disease that you haven’t heard much about. That’s because, as awful as the disease is, it’s lacked headlines even while it’s claimed the lives of its youngest patients. Since its discovery, the absence of a suitably robust public dialogue around the disease has further contributed to the unfortunate circumstances of its patients.
Sickle cell disease was first discovered in 1910 when doctors examined the blood of a patient suffering from severe pain and anemia under a microscope and were startled by the sickle-shaped blood cells they saw. However, while research over the century since has slowly, but surely, given us a solid understanding of the disease, the national and global communities have essentially tread water on sickle cell treatment.
The Forces Working Against Sickle Cell Patients
In the past ten years, the life expectancy for sickle cell patients has declined. However, even prior to the mid-2000s, the mortality rate for adults with sickle cell had steadily risen by 1% each year since 1979, in contrast to substantially improved life expectancies and health outcomes for many other rare diseases during this time. All while the annual treatment costs for the disease have risen comfortably past $1 billion.
It’s shocking that a disease affecting 100,000 Americans could be moving in reverse, but it’s even more shocking to look at it relative to other rare diseases. Cystic fibrosis, which affects only 30,000 people in the US, receives 7 to 11x more funding per patient than sickle cell. Similarly, only about 16,000 patients in the US have ALS, but the disease received just 20% less funding than sickle cell disease, which affects over five times as many patients.
So why has sickle cell been left behind in the race for cures and the calls for awareness? For one, sickle cell demographics may play a substantial role. An estimated 60 to 80% of sickle cell patients are African-American, and much of the remaining 20 to 40% are Hispanic or from other underrepresented communities. It’s hard to believe that a lack of investment in this kind of disease could be a simple oversight in a society with a dark history of racism, both institutionalized and spoken.
But sickle cell’s symptoms have also complicated patients’ ability to receive treatment. During acute episodes of the disease, patients experience excruciating pain that easily tips the far end of the 1-to-10 pain scale. However, it causes few other physical symptoms, and the presence of the disease can only be confirmed through certain blood tests — but undergoing those blood tests too frequently can place patients’ lives at risk.
Meanwhile, the United States is decades into an opioid crisis that has shown no signs of letting up, further complicating sickle cell patients’ treatment. Physicians are trained to be suspicious of patients who ask for opioids by name, make multiple visits for the same complaint, report symptoms “out of proportion” with what a physical exam shows, and who seek treatment at an ER.
These behaviors may be characteristic of opioid addicts, but they also describe sickle cell patients in the midst of an episode — and contribute to numerous pervasive, false beliefs about sickle cell patients. For example, 63% of nurses responded to a surveyindicating that they believe many patients with sickle cell are addicted to opioids, when in fact, their rate of addiction is certainly no higher, and likely lower, than that of the general population.
Along the same vein, studies have found that black Americans receive less pain treatment than white Americans — even though Caucasians make up the majority of opioid addicts. That’s likely why one extensive study of ER waiting rooms found that sickle cell patients wait 60% longer to get pain medication than other patients who reported less severe pain. These misplaced beliefs represent one more substantial barrier preventing sickle cell patients from getting the care they deserve.
Sickle Cell Treatment, and a New Direction
Sickle cell patients can often reduce the frequency of their episodes and their time spent in the ER with regular treatment — but there are obstacles to receiving regular treatment as well. For one, a 2014 survey of more than 30,000 family physicians showed that just 20% are comfortable caring for sickle cell anemia patients, representing yet another reason why these patients frequently seek care in ERs — and only in the midst of an acute sickle cell episode. And for patients who do receive regular treatment — approximately 1 in 4 — the existing treatment options are inadequate, containing potentially serious side effects, and not always covered by insurance.
It’s clear that sickle cell disease patients are facing an uphill battle: against their disease, against low research funding, against their insurance companies, and sometimes even against the doctors that should be providing their care. However, many of the problems that these chronically ill patients are facing can be attributed to a simple lack of awareness.
Medical professionals need to be made aware of sickle cell symptoms and how to treat them. Insurance companies need to be made better-aware of how sickle cell treatments interact with and benefit from each other. And sickle cell patients need to be empowered to manage their own care. Awareness of their treatment options, where to find them, and how to pay for them is a good start, but driving change to bridge the trust gap that exists between patients and medical providers will be key, especially as breakthrough new treatments begin to enter the market.
It’s disheartening and frustrating to understand the complex obstacles that stand in the way of treating, and eventually curing, sickle cell disease. Still, the recent growth in the conversation is nice to see, especially working at an agency that prides itself on empowering patients. It’s our job to get the right message, in front of the right people, at the right time — and that’s exactly what needs to happen to move the needle on sickle cell treatment.
Jenn Renoe, Associate Media Director, is an experienced strategist with ten years planning both traditional and digital campaigns on a local, regional, and national level.
Outside of the office she’s an advocate for redefining healthcare for underserved and underrepresented audiences. She moonlights as a podcaster, writer, speaker, and aspiring author.
Connect with Jenn on LinkedIn.
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