June 19 is World Sickle Cell Awareness Day, and to raise awareness, the American Society of Hematology (ASH) has announced the launching of an effort to develop clinical practice guidelines for management of the disease, among other new initiatives.
Sickle cell disease is hereditary, and is characterized by stiff, sticky sickle-shaped red blood cells that cling onto blood vessels and block the blood flow of patients. The chronic disorder affects an estimated 100,000 Americans, and has no widely-available cure. At present, there is only one U.S. Food and Drug Administration (FDA)-approved treatment; hydroxyurea
The guidelines will be compiled with valuable opinions from both physicians and patients to address the unique needs of this patient population. They will be based on a systematic review of accessible evidence and established according to standards suggested by the National Academy of Medicine.
"These guidelines will help specialists provide optimal care for patients throughout their lives," said Chair of the ASH Sickle Cell Disease Guideline Coordination PanelRobert 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 the result of a group effort, as 5 panels of hematologists, other clinicians, people living with sickle cell disease, and experts in evidence-based guideline production will all contribute. One panel has already been approached to tackle the usage of blood transfusions to improve red blood cell counts and mitigate complications, while another has been asked to better the understanding of the only current cure for SCD: stem cell transplantation.
The ASH guidelines will also consider new evidence available since the publication of the National Heart, Lung, and Blood Institute (NHLBI) guidelines and will be recurrently restructured to present the most innovative, up-to-date information as it pertains to optimal care strategies.
The guidelines are a piece of a much larger commitment from ASH to overcome sickle cell disease around the world. Publication of the guidelines is expected in 2019.