Sickle cell disease (SCD) is a hereditary blood disorder that primarily affects people of African descent, causing immense physical and emotional challenges. Although advancements have been made in treatment, many sickle cell warriors still face difficulties accessing appropriate care.
Understanding the Disease Beyond Pain
Sickle cell impacts nearly every organ in the body due to the sickling of red blood cells that restricts oxygen supply. This lack of oxygen results in chronic pain, organ damage, and reduced life expectancy. However, many healthcare providers still focus primarily on managing acute pain crises, rather than addressing the full scope of the disease, which includes complications such as kidney failure, heart disease, and pulmonary hypertension.
Bert Bruce, Pfizer US President, Rare Disease emphasizes that sickle cell is not just about pain crises; it is a comprehensive disease requiring a systemic approach to care.
“Sickle cell disease, to simplify, is a condition where the red blood cells lose their ability to carry oxygen efficiently. This impacts every part of the body, including the brain, heart, and lungs,” Bruce says. “Since it’s a comprehensive disease, it requires a comprehensive care solution. This care isn’t just about treating the crises that occur but involves managing the entire body.”
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The Transition to Adult Care
One of the most significant issues SCD patients face is the gap in care during the transition from pediatric to adult healthcare. Pediatric care tends to be comprehensive, with a multidisciplinary approach to managing the various complications associated with the disease. However, once patients turn 18, they often struggle to find adequate adult care.
“There are challenges in transitioning to adult care because, historically, patients didn’t live to adulthood. So internal medicine training didn’t prepare doctors to treat adult sickle cell patients. Things are improving, but many non-hematologists and general practitioners still don’t learn how to manage sickle cell unless they’re pediatricians. The medical establishment mostly sees sickle cell patients when they’re in pain, and it’s severe,” Dr. Lisbon shares.
Because of this, many patients lose access to regular care and only re-engage with the healthcare system when they experience a crisis, often ending up in the emergency room
“This reactive rather than proactive approach can lead to more severe health outcomes,” Bruce adds.
To improve care, Bruce advocates for replicating the comprehensive pediatric model in adult care, ensuring that patients have consistent access to specialists who understand the full scope of sickle cell disease.
“The preparation for that transition should start early—ideally when the patient is in high school. Just as students spend four years preparing for college, patients should spend time preparing for the transition to adult care. Healthcare providers can give them a sort of ‘test’ to see what gaps exist in their knowledge and self-management skills, and then work to close those gaps before they transition out of pediatric care,” Bruce shares.
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Addressing Stigma and Bias in Sickle Cell Care
One of the major challenges SCD patients face, especially as adults, is the stigma surrounding their disease. Many patients report being labeled as “drug seekers” when they present to emergency rooms during a pain crisis.
“These patients understand their disease well and know exactly what they need. When they communicate that to emergency room staff, they’re often met with skepticism. The color of their skin and the fact that they don’t always appear to be in visible pain contribute to this misperception,” Bruce says. “This is a common experience for adult patients, but if you ask a 12- or 14-year-old, they might not know what I’m talking about. Pediatric patients are often treated in specialized centers where their care is well-understood, and crises are managed effectively.”
Because of this, many pediatricians even extend care beyond the age of 18 because they don’t want to transition their patients to adult care systems that are ill-equipped to handle the disease.
Dr. Lisbon also mentions the difficulty healthcare providers have in understanding the chronic nature of the disease beyond pain crises. This bias leads to delayed care, with patients often waiting hours for treatment.
“Living with the disease means knowing that organ damage is happening over time, and you might not even feel it. When patients do encounter the medical system, the expertise on how to care for them is often lacking,” Dr Lisbon adds. “For example, a patient might be in crisis and have pulmonary hypertension, which puts them at increased risk for acute chest syndrome. They could be fine at the start of the day, but by the end, they might need to be in the ICU because they suddenly can’t breathe. This acute-on-chronic situation is a challenge. On the provider side, many don’t fully understand the pathophysiology of sickle cell or focus on preventive care to help avoid these crises.”
Since sickle cell affects every organ, Dr. Lisbon encourages healthcare providers to be extra aware of symptoms.
“A system-wide symptom approach would go through each organ system, starting with the head and moving down. For example, if a sickle cell patient has a headache, we need to determine whether they’re at risk for a stroke, particularly an ischemic stroke caused by decreased blood flow due to vaso-occlusion,” she notes.
The Need for Increased Research and Funding
Another key issue is the lack of sufficient funding and resources for sickle cell research. Bruce acknowledges that while there are ongoing clinical trials, the disease remains “massively underfunded” compared to other conditions.
“There are several reasons for this, one being a lack of awareness about the economic consequences of not addressing the disease. Sickle cell disease affects over 100,000 individuals in the U.S. alone, yet it doesn’t get the same attention as other diseases. There’s no major telethon for sickle cell like there is for muscular dystrophy, nor a high-profile advocate like Ronald Reagan was for Alzheimer’s. These catalysts, which help raise awareness and funding in other areas, haven’t emerged for sickle cell,” he notes.
The Role of Community-Based Organizations
Community organizations play a crucial role in supporting sickle cell patients and advocating for better care.
“Community-based organizations play a crucial role. Some are formal advocacy groups, while others are made up of passionate parents who’ve developed relationships within the healthcare system. They advocate for patients, help raise awareness, and provide education about managing sickle cell disease. Their voices are incredibly powerful when it comes to engaging with legislators and stakeholders to influence policy and funding,” Bruce adds. “For example, policies that incentivize hospitals to educate their staff about sickle cell or improve triage procedures would make a huge difference. Community-based organizations can help ensure that hospitals don’t just discharge patients after a crisis without providing follow-up care.”
Moving Toward Systemic Solutions
Both Dr. Lisbon and Bruce agree that the key to improving sickle cell care lies in systemic changes. These include replicating the comprehensive pediatric care model for adults, increasing awareness and education among healthcare providers, and addressing the stigma that SCD patients face.
“No two patients are the same, just like no two fingerprints are the same. We need systemic solutions that benefit all patients while respecting that patients are the experts in their own disease. They’ve lived with it all their lives and know what they need. If healthcare providers can adopt that mindset, the care process will be faster and more effective,” Bruce notes.
As Dr. Lisbon and Bert Bruce have highlighted, the future of sickle cell care depends on systemic solutions that address not only the medical aspects of the disease but also the social and emotional challenges that patients face. With greater awareness, funding, and advocacy, we can ensure that patients with sickle cell disease receive the care and support they need to lead healthier, fuller lives.
