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ASH Releases New Clinical Practice Guidelines on Management of Pain in Sickle Cell Disease

Pain guideline is the latest in Society’s evidence-based series of clinical practice guidelines for sickle cell disease aiming to improve and personalize care for individuals with this complex condition

(WASHINGTON, June 19, 2020) – Today, the American Society of Hematology (ASH), the world’s largest professional society dedicated to furthering the understanding, diagnosis, treatment, and prevention of blood disorders, announced the publication of the ASH 2020 Guidelines for Sickle Cell Disease Management of Acute and Chronic Pain, the new installment of the Society’s evidence-based practice guidelines on the disease.

Pain is the most common complication of SCD and it significantly decreases daily quality of life. Severe pain is the leading cause of emergency department visits and hospitalizations for people with SCD. Individuals with pain from SCD often don’t get the care they need because comprehensive information about medications and therapies had not been readily available. This guideline and others in the collection, published in ASH’s journal Blood Advances, address clinical challenges by providing the first evidence-based recommendations to help individuals with SCD and their providers make the most informed decisions for personalized care.

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 Blacks or African Americans and one out of every 16,300 Hispanic Americans. In individuals with SCD, the red blood cells, which are normally round, become crescent or sickle shaped. These abnormally shaped cells break apart easily, clump together, and stick to the walls of blood vessels, blocking the flow of blood and causing excruciating pain. In addition, individuals with SCD can experience joint and organ damage, stroke, and reduced life expectancy. On top of the immense physical burden of the disease, individuals often cannot access the care they need because SCD is largely misunderstood by medical professionals. In fact, 20% of family physicians report feeling comfortable caring for individuals with SCD.

“We’ve seen remarkable advances in the therapies available for sickle cell disease and its complications, with two new FDA-approved therapies last year alone and many more in development. Yet care is often the responsibility of primary care, family, and emergency room physicians and nurses, and the community has long expressed a strong need for guidance that can help them deliver the best care for their patients,” said Robert Liem, MD, chair of the ASH Sickle Cell Disease Guideline Coordination Panel and director of the Comprehensive Sickle Cell Disease Program at the Ann & Robert H. Lurie Children’s Hospital of Chicago. “There is no one-size-fits-all approach to treating sickle cell disease, and these guidelines provide evidence-based recommendations about how to individualize care in accordance with individuals’ preferences and values.”

In partnership with the Evidence-Based Practice Research Program at Mayo Clinic, the ASH Guidelines on SCD were developed using the GRADE methodology to ensure the highest standards for trustworthiness. ASH brought together 61 clinical experts, five methodologists, and 10 patient representatives to identify best practices for the management of acute and chronic complications of SCD and ways to improve the quality of care for patients.

Five total guidelines cover SCD across areas where there has previously been little guidance and where there is significant uncertainty or variation in clinical practice:

“Supporting research and improving care for all people living with sickle cell disease is a chief priority for ASH,” said ASH President Stephanie Lee, MD, MPH, of Fred Hutchinson Cancer Research Center. “Creating and disseminating a comprehensive set of SCD treatment guidelines can improve the care of people with SCD by identifying the best evidence-based practices to be used by the many health care professionals who treat the disease. The future will hold better treatment options for patients, but for now, we can and need to improve care using tools we have available now.”

The ASH Guidelines on SCD are part of a larger guideline development initiative for ASH. ASH is committed to the timely update of existing guidelines and the development of new guidelines for a range of hematologic conditions.

ASH also has resources for treating SCD in the COVID-19 pandemic, including frequently asked questions about SCD and COVID and a checklist for caring for individuals with sickle cell disease who go to emergency departments with COVID-like symptoms.

Visit www.hematology.org/SCDguidelines for more information about the ASH Guidelines on SCD, including fact sheets with key takeaways from each guideline, infographics, clinical teaching slide sets, and patient stories.

About ASH’s Work in Sickle Cell Disease

In 2016, ASH launched a multifaceted initiative to address the burden of disease both in the United States and globally. ASH has since developed clinical guidelines for SCD management and care, expanded education and training efforts, and advocated with policymakers to enhance and expand federal SCD programs that include efforts to address pain management in individuals with SCD (learn more about ASH’s advocacy efforts here). Currently, ASH is working with members of Congress to create a pilot program for better access to health care for people living with SCD. In 2016, the Society also founded the Sickle Cell Disease Coalition which has grown to more than 85 members. In addition to these efforts, the ASH Research Collaborative (ASH RC) Data Hub and SCD Clinical Trials Network was developed with the mission to improve outcomes for individuals with SCD by expediting SCD therapy development and facilitating innovation in clinical trial research. It provides the infrastructure for identifying patient cohorts for trials, matching trial sponsors with sites, facilitating recruitment of eligible patients, and ensuring optimally designed trials and an efficient, coordinated approach. Through patient engagement and optimized clinical trial execution, the Clinical Trials Network will help to bring new and more effective therapies to individuals with SCD.

American Society of Hematology

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.

Amanda Szabo, American Society of Hematology 
[email protected]; 202-552-4914