http://www.raredr.com/news/nhlbi-guidelines-sickle-cell-disease-published
New NHLBI Guidelines for Managing Sickle Cell Disease Published


RDR Staff


The National Heart, Lung, and Blood Institute (NHLBI) published a new evidence-based expert panel report on the management of sickle cell disease.  The report (http://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines/) is a thorough, systemic review of the evidence available to manage patients with sickly cell anemia

Some of the topics covered include:

Health Maintenance for People With Sickle Cell Disease
Managing Acute Complications of Sickle Cell Disease Managing Chronic Complications of Sickle Cell Disease
Hydroxyurea Therapy in the Management of Sickle Cell Disease

Blood Transfusion in the Management of Sickle Cell Disease
Recommendations for Acute and Chronic Transfusion Therapy
 
The report is also endorsed by the American Society of Hematology (ASH) who wrote in a press release that the NHLBI report is “based on a systematic review of available evidence and consensus of an expert panel as well as published, vetted guidelines by other organizations when evidence was unavailable or insufficient. Included with each recommendation are descriptions of the strength of the recommendation and the quality of supporting evidence.”

While the guidelines are much improved from earlier ones, ASH President Linda J. Burns, MD, of the University of Minnesota conceded that more work needs to be done.  “There are a number of areas where there is clinical consensus; however, many recommendations are not based on strong evidence,” said Dr Burns, adding, “This report is an important statement of what is known about how to care for our sickle cell patients. However, the gaps in evidence are striking and should be a call to redouble our efforts to continue our search for a cure and, in the meantime, provide the best possible care to patients with sickle cell disease.”

Sickle cell disease is a rare genetic condition that affects 90,000 to 100,000 Americans, mostly African Americans.  A person with sickle cell disease have red blood cells that are hard, sticky, and C-shaped (like the farm tool the "sickle"). These cells clog smaller blood vessels resulting in pain as well as increased risk for infection, acute chest syndrome and stroke. Because sickle cell disease is a complex illness that affects multiple organ systems, few physicians specialize in sickle cell disease or can assume primary responsibility for a patient’s care.

Since multiple clinicians are often involved in patients with sickle cell disease, the  NHLBI sickle cell disease expert panel report is designed to provide guidance for all clinicians (from primary care physicians to specialists including hematologists).


Cover image obtained from NHLBI.
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