ASH to Develop Clinical Guidelines to Improve Care for People with Sickle Cell Disease

(WASHINGTON, June 19, 2017) — This World Sickle Cell Day, the American Society of Hematology (ASH) is working on several projects to conquer sickle cell disease (SCD) worldwide. Among them, the Society has launched an effort to develop clinical practice guidelines on the management of SCD. An expansion of ASH’s commitment to identify and confront unmet needs in SCD patient care, these guidelines will be created with input from physicians and patients to address the unique needs of this underserved patient population.

An inherited, lifelong chronic disorder affecting nearly 100,000 Americans, SCD is characterized by rigid, sickle-shaped red blood cells that stick to blood vessels, blocking blood flow. With only one FDA-approved treatment and no widely available cure, life is extremely challenging for people with SCD. Pain — both chronic and acute — is common and results in tremendous suffering and difficulty continuing daily activities like work or school. Brain injury, organ damage, stroke, and death are all devastating complications associated with SCD.

“These guidelines will help specialists provide optimal care for patients throughout their lives,” said Chair of the ASH Sickle Cell Disease Guideline Coordination Panel Robert Liem, MD, Director of the Comprehensive Sickle Cell Program at the Ann & Robert H. Lurie Children’s Hospital of Chicago. “We are eager to work with other hematologists, pediatricians, emergency room physicians, and people with SCD to improve patients’ overall quality of life.”

The guidelines will be based on a systematic review of available evidence and developed according to standards recommended by the National Academy of Medicine (formerly the Institute of Medicine). ASH has contracted with the Mayo Clinic Evidence-Based Practice Research Center to conduct the systematic evidence reviews and to support the guideline development process. Publication of the guidelines is expected in 2019.

These state-of-the-art guidelines will be produced and maintained by five panels of hematologists, other clinicians, people living with SCD, and experts in evidence-based guideline production. The panels will address the care and management of acute and chronic complications of SCD, including pain caused by the disease, stroke prevention and treatment, and prevention and treatment of cardiopulmonary and kidney diseases. One panel has been called to address the use of blood transfusions to improve red blood cell count and mitigate complications of SCD, while another has been convened to improve the understanding of stem cell transplantation, currently the only cure for SCD.

The ASH SCD guidelines will expand on guidelines published by the National Institutes of Health’s National Heart, Lung, and Blood Institute (NHLBI). The NHLBI’s EvidenceBased Management of Sickle Cell Disease: Expert Panel Report, 2014 provides useful guidance and information for primary care, emergency, and other clinicians who may be unfamiliar with this rare disease. The ASH guidelines will provide clinical recommendations for hematologists and other specialists. The ASH guidelines will also take into account new evidence available since the publication of the NHLBI guidelines and will be continually updated to present the latest in optimal care strategies.

These guidelines are part of a broader ASH commitment to conquer SCD worldwide. Last fall, ASH launched a call to action on SCD by publishing a report card that identified the areas of care most in need of improvement and announced the formation of a new Sickle Cell Disease Coalition. The Society also issued the State of Sickle Cell Disease: 2016Report, which was endorsed by 24 other organizations. 

The Society is in the midst of developing a consortium of sub-Saharan African countries to address newborn screening and early intervention. The consortium would introduce standard-of-care practices for screening and early intervention therapies at participating institutions, with the goal of decreasing childhood mortality rates for SCD.

Additionally, ASH continues to work with partners to support legislation that will improve access to SCD care, treatments, and cures. This month, the Society co-hosted a briefing on Capitol Hill to educate policymakers on advancements in SCD research, and has advocated for federal funding to expand surveillance of this population that will help us better understand health outcomes and health care system utilization patterns of people living with SCD.

ASH continues to increase its educational resources through the development of webinars and in-person training opportunities and will also host a broad array of sessions about SCD at the 59th ASH Annual Meeting in Atlanta.

“There are many unique challenges that people with SCD face,” said ASH President-Elect Alexis Thompson, MD, MPH. “From enabling patients to find local health care providers with the knowledge and expertise to provide proper care, to supporting efforts to bring newborn screening and early intervention to infants and children with sickle cell disease in sub-Saharan Africa, there is enormous opportunity to transform the way we care for people suffering from SCD. ASH is ready to tackle this effort on all fronts.”

The American Society of Hematology (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 50 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, which is available weekly in print and online. In 2016, ASH launched Blood Advances (www.bloodadvances.org), an online, peer-reviewed open-access journal.

Contact:
Stephen Fitzmaurice, American Society of Hematology
[email protected]; 202-552-4927