Management of Pain in Sickle Cell Disease: ASH releases new guidelines

WASHINGTON -- The American Society of Hematology (ASH), has released 2020 Guidelines for Sickle Cell Disease. The guidelines have been published in the journal Blood Advances.

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:

• Cardiopulmonary and Kidney Disease
• Transfusion Support
• Cerebrovascular Disease
• Management of Acute and Chronic Pain
• Transplantation (anticipated 2020)

"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.

For further reference 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.