Five updates for World Sickle Cell Day
In recognition of World Sickle Cell Day, the American Society of Hematology launched an effort to develop clinical practice guidelines on the management of sickle cell disease.
The guidelines — which will expand on the NHLBI’s 2014 “Evidence-based management of sickle cell disease: Expert panel report,” and are expected in 2019 — will be based on a systematic review of available evidence conducted by the Mayo Clinic Evidence-Based Practice Research Center. Panels composed of clinicians and patients will address the care and management of sickle cell disease, including pain, stroke prevention and treatment, and prevention and treatment of cardiopulmonary and kidney diseases.
“These guidelines will help specialists provide optimal care for patients throughout their lives,” Robert Liem, MD, chair of the ASH Sickle Cell Disease Guideline Coordination Panel and director of the Comprehensive Sickle Cell Program at the Ann & Robert H. Lurie Children’s Hospital of Chicago, said in a press release. “We are eager to work with other hematologists, pediatricians, emergency room physicians and people with sickle cell disease to improve patients’ overall quality of life.”
In conjunction with World Sickle Cell Day, HemOnc Today presents five updates in the treatment and outcomes associated with sickle cell disease.
- The Oncologic Drugs Advisory Committee of the FDA voted 10-3 in favor of Emmaus Medical’s new drug application for oral L-glutamine powder for chronic use in adults and children age 5 years and older with sickle cell disease. Read more.
- Patients with sickle cell disease who did not have asthma experienced pain reduction after inhaling corticosteroids. Read more.
- Patients with sickle cell anemia experience a faster decline in the glomerular filtration rate and an increased prevalence of hyperfiltration, although no association between the two was observed. Read more.
- LentiGlobin BB305 (Bluebird Bio) — a self-inactivating lentiviral vector that encodes the human HBB variant BetaA-T87Q — showed promise in a 13-year-old boy with sickle cell disease who showed no signs of the disease and resumed normal activities 15 months after an infusion of the gene therapy. Read more.
- Harvoni, a fixed-dose combination of ledipasvir and sofosbuvir (Gilead Sciences), safely and effectively treated hepatitis C virus infection in patients with sickle cell disease. Read more.