Sickle Cell Disease

In a study conducted at the Johns Hopkins School of Medicine, data were collected from 1998 to 2017 on two cohorts of patients with kidney failure. The first cohort, a dialysis cohort, included 1,970 patients with sickle cell disease and approximately 2 million patients with other conditions. The second cohort, the “transplant waitlist cohort,” consisted...

A prospective observational study showed that adults with sickle cell disease who had experienced an uncomplicated vaso-occlusive crisis had better outcomes when treated at specialty infusion centers than emergency departments (EDs). Patients treated at infusion centers received pain medication faster than patients treated at EDs (62 vs. 132 minutes). Additionally, patients treated at infusion centers...

Sickle cell trait also associated with increased risk for COVID-19-related hospitalization and death, but to a lesser extent Sickle cell disease is associated with a fourfold increased risk for COVID-19-related hospitalization and a more than twofold increased risk for COVID-19-related death, according to a research letter published online July 20 in the Annals of Internal...

Sickle cell disease (SCD) is associated with complications that increase the risk of early death. In recent decades of SCD research, multiple interventions have proven successful in reducing premature mortality in infants and children with SCD. However, adults with SCD still have a high risk of early mortality, with an average life expectancy of 54...

Clinical manifestations of sickle cell disease, such as hemolytic anemia, pain, and progressive organ damage, can be alleviated by high levels of erythrocyte fetal hemoglobin (HbF). The BCL11A gene represses γ-globin expression and adult erythrocyte fetal hemoglobin production. This study, published in the New England Journal of Medicine, focuses on the genetic silencing of BCL11A, resulting...

Voxelotor is a hemoglobin oxygen-affinity modulator that is used to treat sickle cell disease. This study, published in Annals of Pharmacotherapy, conducted a comprehensive literature review on the safety, efficacy, and affordability of voxelotor. Ultimately, it was found that voxelotor is generally well-tolerated and significantly increases mean hemoglobin level compared to placebo. Commonly noted adverse...

Hydroxyurea use does not impact development of serious COVID-19 or need for hospitalization in children or adults A history of severe pain episodes and coexisting organ conditions may increase the risk for severe COVID-19 illness and hospitalization in individuals living with sickle cell disease (SCD), according to a study published online July 1 in Blood...

People with sickle cell disease who have a history of severe pain episodes and coexisting organ conditions have an increased risk for severe COVID-19 illness, a new study suggests. “This study tells us that all individuals with sickle cell disease are not at equal levels of risk,” said study author Dr. Lana Mucalo, of the...

It’s been more than six months since Brandy Compton last landed in a hospital emergency room. That’s an amazing medical achievement, brought about by scientific breakthroughs that have been unfortunately overshadowed by the coronavirus pandemic, experts say. Compton, 31, was born with sickle cell disease, a genetic condition that primarily affects people of African descent....