Sickle cell disease is the most common inherited blood disorder in the United States. The Centers for Disease Control and Prevention estimate that 100,000 people are living with sickle cell in the US and an additional 1 to 3 million people carry the sickle cell trait.[1,2] While sickle cell is found most commonly in people with African ancestry (approximately 1 out of 365 African American births), it is also common in people with ancestry from Central and South America, the Middle East, Asia, India and the Mediterranean.
In sickle cell disease, red blood cells become rigid and form a crescent or moon shape, like a sickle. Sickle-shaped cells break apart easily, clump together, and stick to the walls of blood vessels, preventing oxygen from distributing throughout the body. This leads to individuals living with sickle cell to experience many health complications, from mild to severe. Common complications include: stroke, pain - both chronic and episodic, anemia, acute chest syndrome, kidney disease, and venous thromboembolism.
Pain is the most common reason that people with sickle cell seek emergency care.
A recent survey of adolescents and adults in the U.S. conducted by the Sickle Cell Disease Implementation Consortium found that most respondents had negative experiences in acute care settings even though they were pleased with their primary care provider.[3] Pain is the most common reason that people with sickle cell seek emergency care. Respondents were so dissatisfied with their care that many reported intentionally delaying seeking help.
Sadly these findings are in line with a shocking report from STAT exposing several compelling stories from patients living with sickle cell that struggled to receive adequate care at emergency rooms in the US and were often labeled as opioid drug seekers.[4] These patients knowingly avoided care during the night to avoid negative judgements and dressed up to look professional before heading to the emergency room - all to help combat the misconceptions many sickle cell patients face.
The FDA has approved four medications to treat sickle cell disease including hydroxyurea and voxelotor, targeting hemoglobin-S polymerization, and L-glutamine and crizanlizumab, targeting vaso-occlusion.[5] Additional pharmacological strategies like targeting the underlying inflammation in sickle cell disease that contributes to end organ damage are also being explored in clinical studies. Beyond drug interventions, stem cell transplantation has shown some successes in individuals, offering a long-term cure, though the procedure is not widely available. Multiple gene therapy strategies are in early stages of development. Regular blood transfusion may also relieve some symptoms, delivering oxygen to the body and unblocking blood vessels.
PicnicHealth is excited to contribute to the sickle cell research community. Since 2020, PicnicHealth has enrolled nearly 1000 patients into a novel medical-records based research study. We’re giving people living with sickle cell the opportunity to easily, securely, and anonymously share their medical data. Our real-world data cohort is helping our research partners better understand patients’ day-to-day challenges of living with sickle cell - answering questions like how frequently do pain crises occur and how do patients treat crises at home and within formal care settings.
“If I had a medical emergency I experienced extended wait times while explaining my condition...Now with PicnicHealth, all of my data is located in my cellular device. I can hold the power in the palm of my hand,” said Dima, a PicnicHealth Sickle Cell research participant.
Explore findings from a preliminary analysis including a subset of 500 patients contributing more than 6 years retrospective medical records data. Study participants visited a median number 4 care sites and saw a median total of 36 providers. It’s understandable that patients will struggle to organize their medical data and repeating their story to each new provider can place significant barriers during a pain crisis or when seeking care in response to other disease complications.
Through our sickle cell research program we continue to empower patients to take ownership of their personal medical journey. We want to arm sickle cell disease warriors with the confidence to advocate for their own care within all settings during healthy times and during crises.
Interested in learning more about PicnicHealth’s Sickle Cell RWD cohort? Contact our team today.
References:
1. https://www.cdc.gov/ncbddd/sicklecell/data.html. Accessed 10/22/21
2. https://www.hematology.org/education/patients/anemia/sickle-cell-trait. Accessed 10/22/21
3. Kanter J, Gibson R, Lawrence R, et al. Perceptions of US Adolescents and Adults With Sickle Cell Disease on Their Quality of Care. JAMA Netw Open. 2020 May 1;3(5):e206016. Published 2020 May 29. doi:10.1001/jamanetworkopen.2020.6016
4. https://www.statnews.com/2017/09/18/sickle-cell-pain-treatment/ Accessed 10/22/21
5. Salinas CG, Thein SL. Recent Advances in the Treatment of Sickle Cell Disease. Front Physiol. 2020;11:435. Published 2020 May 20. doi:10.3389/fphys.2020.00435
