Trends in Sickle Cell Disease Focus on Therapeutic Treatments, Care Transitions and Global Health

Posted April 07, 2016 by Cindy Hutter

Dr. Suzette Oyeku
Suzette Oyeku
Since NICHQ was named the National Coordinating Center for the Sickle Cell Disease Treatment Demonstration Program (SCDTDP) in 2010 there have been increases in the number of providers prescribing disease modifying therapies, such as hydroxyurea to treat patients with sickle cell disease (SCD), better care in emergency departments, along with increases in the number of patients receiving regular care with providers knowledgeable about treating SCD. As the project enters into a new phase, we recently sat down with Suzette Oyeku, MD, MPH, medical director for NICHQ’s sickle cell work to learn more about the project’s evolution and learnings.

Tell us about the current SCDTDP and how it has evolved in the past six years?
Overall, we’re trying to get individuals with SCD into a patient-centered medical home and connected to ongoing specialty care, along with increasing the use of hydroxyurea, the only Food and Drug Administration approved treatment for SCD. The major change in the current SCDTDP is the shift to a regional focus. When we first started, we were working with nine institution-based programs in separate states. In this new iteration of the SCDTDP, it’s focused on regional and statewide initiatives that are trying to spread lessons learned and optimize access to care. There are four regional leads that are serving as regional coordinating centers, which work with several states. Many regional coordinating centers have taken the lessons learned from NICHQ’s learning collaborative in the earlier version of the project and applied it to launch their own regional learning collaboratives. As the national coordinating center, NICHQ is providing a lot of technical assistance around data collection and connecting participants with others doing similar work across the country.

What is Sickle Cell Disease?

Sickle cell disease (SCD) is a group of inherited red blood cell disorders that affects approximately 100,000 people in the United States, largely individuals of African ancestry, but increasingly in Latino and other populations as well. Individuals with SCD experience painful episodes when their red blood cells morph into a crescent (“sickle”) shape and get stuck in small blood vessels. This blockage inhibits blood flow, which deprives tissues of oxygen and causes severe pain and tissue damage. SCD is characterized by chronic anemia, unpredictable episodes of pain and end-organ damage.

What are some of the accomplishments in this work that make you most proud?
The work that focused on improving acute care management. It wasn’t originally a main focus of the last iteration of the project that ended in 2014, but by listening to the voices of families and those affected with SCD, we heard that optimizing acute care management was important. It’s wonderful to start hearing that other programs are using some of the innovations that came out of our work, such as using intranasal fentanyl to deliver rapid pain relief for patients, and other strategies for shortening time to treatment in the emergency room. Another is the healthcare transition toolkit and curriculum that was developed, which has broad application for the SCD community.

What’s the challenge when it comes to access to care?
For children with SCD, there is not really a shortage of pediatric hematologists and the idea of having regular checkups with a primary care pediatrician is entrenched. Since individuals with SCD are living longer and into adulthood, there is still a lot of work to be done regarding access to primary care and specialty care for adults with SCD. There are very few adult SCD specialists across the country and limited numbers of primary care providers who feel comfortable caring for individuals with SCD. The SCDTDP regional coordinating centers are trying some innovative ideas about how to address this, including leveraging Project ECHOExternal Link as a way to provide virtual/telementoring support to practices that may not have access to a SCD specialist.

What are some trends right now in the sickle cell community?

We see a continued focus on new therapeutic options to manage pain. This is an ongoing area of study because pain management is a hallmark of the disease.

We are also seeing more of a focus on dissemination and implementation—more specifically, how we insure that the strategies and treatments we know work well are being translated into clinical practice and communities. That is important for the field because there is a gap between what we know to be effective and what is happening in practice. For a patient who may not be getting treatment at a SCD center, ensuring that they are getting high-quality care, even if in a resource poor setting, is essential.

Care transitions are also getting a lot of attention. Current efforts are focused on ensuring the transfer from pediatric to adult care occurs smoothly and people are integrated into appropriate care settings with providers capable for caring for individuals with SCD.

Lastly, global health is gaining increased attention. The burden of SCD is significantly higher outside the U.S. Some leaders in the SCD community have begun to develop partnership with institutions across West Africa and South America and the Caribbean with the hopes of translating lessons learned in the U.S. to these institutions as well as adapt effective treatment approaches to a broader population.

What keeps you up at night?

Being able to get accurate data that is telling us what is happening at institutions and state levels so we get an accurate picture of the state of SCD care across the U.S. More specifically, how can we develop a system that will track healthcare utilization and patient experience of care? We have a lot of insights about the type of metrics, but we have more work to do to assess the best strategies to consistently and accurately collect patient-level data and data from sickle cell community-based organizations on an ongoing basis. This information will ultimately help us better understand improvement opportunities related to SCD care. Our overarching goal is for individuals with SCD to get the care they need consistently.

If there was one thing you could change tomorrow about SCD care or management, what would it be?
Improving the coordination of care and making it more patient-and-family centered. Right now, care is fragmented and people have to go to multiple different places. Adjusting healthcare financing could help to facilitate a shift towards more coordinated, integrated patient-and-family-centered care for individuals with SCD.


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