Contributions
Sickle Cell Trait and the Increased Risk of Stillbirth
Pregnant people with sickle cell trait (SCT) have increased risk of stillbirth, according to a study published in JAMA Network Open. Silvia P. Canelón, Ph.D., from the University of Pennsylvania in Philadelphia, and colleagues examined the potential association between SCT and stillbirth outcome in a retrospective cohort study including data on deliveries occurring between Jan....
Women With Sickle Cell Disease Pain Receive Fewer Opioids
Past trends have shown that men are more likely than women to receive opioids in the emergency department for the management of acute pain. This cross-sectional study, published in The American Journal of Emergency Medicine, examined recent data to see if this disparity persists among men and women admitted to the emergency department for sickle cell...
Adolescent Sickle Cell Disease and Health Literacy
In the United States, patients with sickle cell disease (SCD) are often Black, poor, and publically insured, leaving them vulnerable to barriers in care. In addition, adolescent patients with SCD are especially vulnerable when transitioning from pediatric to adult care. A patient’s level of health literacy is thought to influence the success of this transition. ...
ASH Guidelines on HSCT for Sickle Cell Disease
In a 2021 clinical guideline issued by the American Society of Hematology and published in Blood Advances, eight conditional recommendations are presented for the use of hematopoietic stem cell transplantation (HSCT) in sickle cell disease (SCD). Julie Kanter, M.D., from the University of Alabama Birmingham, and colleagues developed evidence-based guidelines for the use of HSCT...
Drug Might Reverse Heart Damage Linked to Sickle Cell Anemia
Heart issues are common among people with sickle cell disease. Among them are enlargement of the heart and an impaired ability to relax the heart, a condition called diastolic dysfunction that can lead to heart disease, heart failure, and death. Long-term treatment with hydroxyurea, however, seems to improve and even reverse some of these abnormalities,...
Sickle Cell Disease and Kidney Transplant Disparity
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...
Pain Crises and Lack of Access to Infusion Centers
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 Disease and Poor COVID-19 Outcomes
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 in the Annals of Internal Medicine. Ashley Kieran Clift, M.B.B.S., from the University of Oxford in the United Kingdom, and colleagues assessed the risk for COVID-19-related...
Treating Sickle Cell Disease With Genetic Silencing
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...