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New Recommendations Aim to Increase Capacity for Specialized Care for Adults Living with Sickle Cell Disease

Paper is the first to define the requirements for establishment of dedicated adult sickle cell disease treatment centers

(WASHINGTON, August 12, 2020) — Across the United States, adults with sickle cell disease (SCD) lack access to comprehensive care to manage their condition, help them prevent emergency department visits, and avoid serious complications. Today, directors of top SCD programs in the United States published the first set of recommendations for establishing SCD centers aimed at improving access to care for adults living with this devastating, lifelong blood disease. The recommendations, which resulted from a study done in preparation for an American Society of Hematology (ASH) workshop in 2019, outline the essential components that must be in place to establish an adult SCD treatment center.

SCD is the most common inherited red blood cell disorder in the United States, affecting an estimated 100,000 people. According to the Centers for Disease Control and Prevention, SCD affects one out of every 365 Black or African American births and one out of every 16,300 Hispanic American births. Individuals with SCD suffer from acute pain episodes and chronic pain, and may be affected by an array of other organ complications, which can cause disability or even death. Since the universal adoption of newborn screening in the United States in 2006, care for children living with SCD has improved. However, their access to appropriate, quality care often suffers in the transition to adulthood because of a shortage of specialized providers (adults with SCD are more likely to receive care in community hospitals, where SCD-specific experience is less common) and gaps in coordination among the various primary care physicians and specialists who might treat an individual living with SCD. Other barriers to high-quality care for adults living with SCD include challenges relating to health insurance cost and coverage, socioeconomic status, and co-existing conditions. In fact, two years ago individuals living with SCD, along with their families, health care providers, and others working to advance care for SCD, reported there is room for improvement in access to care via the 2018 State of SCD report card. An updated report card will be released in September.

One of the reasons many adults with SCD cannot access specialized care is a relative lack of investment in SCD as compared to other conditions. “There are dedicated treatment centers for hemophilia and cystic fibrosis patients, as well as large cancer centers. There is no such thing as a sickle cell disease center of excellence, and we’re trying to change that,” said workshop co-chair and paper co-author Wally Smith, MD, of Virginia Commonwealth University.

“I have taken care of numerous people who never even knew there was a sickle cell specialist. They were seeing family medicine, primary care, emergency room, or community oncologist doctors whose primary goals are not SCD care,” said Julie Kanter, MD, of University of Alabama, Birmingham, co-chair of the workshop as well as principal author of the paper. “This is a clear disparity. To improve care for individuals living with SCD, we first needed to define that care.”

The recommendations, published in Blood Advances, codify the required components of establishing SCD adult care centers. These requirements include:

  • Multidisciplinary, team-based, evidence-guided care that is coordinated throughout the institution
  • The SCD center as the recognized authority for managing SCD within the institution
  • A physician lead who is considered an SCD specialist
  • One or more social workers, a patient coordinator, and dedicated nursing staff
  • The ability to offer acute and chronic pain management, transfusion, and access to specialists

The paper makes recommendations for additional important, but not required, personnel such as clinic managers, behavioral health staff and psychologists, physical and occupational therapists, and pharmacists. Altogether, the recommendations define comprehensive care for SCD as team-based, holistic, and tailored to the unique needs of individuals with SCD.

In recognition that care for individuals living with SCD can vary greatly by location and institution, the paper also defines several feasible models of care. In addition to the classic model, in which there is a dedicated SCD clinical space and staff, there are also options including the embedded model (in which the SCD center is within another clinical area or department) as well as models blending specialty and primary care centers. “Different centers have different abilities to provide care, depending on amount of space or types of providers. We defined a minimum standard, and suggested some options for meeting that standard,” said Dr. Kanter.

Establishing adult SCD programs with these recommendations could result in better health outcomes, fewer acute care visits and hospitalizations, and improved quality of life for individuals with SCD. Dedicated SCD centers are financially feasible since they can reduce the need for costly acute care in hospitals and emergency departments. It is estimated that there are more than 100,000 SCD-related hospital stays in the United States each year, and comprehensive care would reduce hospitalization for individuals with SCD.

Dr. Smith expressed hope that in addition to benefiting patients, adult SCD centers could benefit health care providers. “They should see SCD care as a normal, profitable, academically engaging, and productive life for them as a professional. We now have four therapeutic agents specifically targeted for SCD in the United States. We have bone marrow transplant. We have clinical trials for gene therapy. The time has come for training programs to tell their trainees that the up-and-coming place to be is sickle cell disease.”

In November 2019, ASH hosted the inaugural SCD Centers Workshop, led by directors of adult sickle cell treatment programs. Attendees from 11 institutions seeking to develop new adult care programs learned core elements of centers and strategized ways to implement them. Since the workshop, attendees have been mentored and given resources such as monthly webinars as they work to establish their centers.

The ASH SCD Centers Workshop stems from a multifaceted initiative ASH launched in 2016 to address the global burden of SCD. The ASH Sickle Cell Disease Initiative includes development of SCD clinical practice guidelines, expansion of education and training efforts, focused federal policy efforts, and establishment of the Sickle Cell Disease Coalition. In addition to these efforts, the ASH Research Collaborative (ASH RC) SCD Clinical Trials Network and Data Hub were developed with the mission to improve outcomes for individuals with SCD by expediting SCD therapy development and scientific discovery and facilitating innovation in clinical trial research.

Establishing SCD centers is an important step to improving access to care, but care cannot be sustained without adequate financial support. Therefore, ASH is complementing these efforts to outline requirements and models of comprehensive care by advocating for a Medicaid demonstration program which aims to expand federal reimbursement to states for the care of individuals with SCD. The demonstration focuses on improving comprehensive and quality outpatient preventive, primary, and specialized care for individuals with SCD.

“All of our efforts have to ensure that the patients are empowered to understand their condition and make the best decisions for themselves,” said Dr. Kanter. “Everybody deserves access to care.”

The American Society of Hematology (ASH) (www.hematology.org) is the world’s largest professional society of hematologists dedicated to furthering the understanding, diagnosis, treatment, and prevention of disorders affecting the blood. For more than 60 years, the Society has led the development of hematology as a discipline by promoting research, patient care, education, training, and advocacy in hematology. ASH publishes Blood (www.bloodjournal.org), the most cited peer-reviewed publication in the field, and Blood Advances (www.bloodadvances.org), an online, peer-reviewed open-access journal.

Leah Enser, American Society of Hematology
[email protected]; 202-552-4927