Expert consensus on the management of infusion-related reactions (IRRs) in patients with sickle cell disease (SCD) receiving crizanlizumab: a RAND/UCLA modified Delphi panel.

Crizanlizumab, a monoclonal antibody against P-selectin, has been shown to reduce vaso-occlusive crises (VOCs) compared to placebo in patients ≥ 16 years with sickle cell disease (SCD). However, there have been rare reports of patients experiencing severe pain and subsequent complications within 24 hours of crizanlizumab infusions. These events are defined as infusion-related reactions (IRRs). Informed by current literature and clinical experience, a group of content experts developed clinical guidelines for the management of IRRs in patients with SCD. We used the RAND/University of California, Los Angeles (UCLA) modified Delphi panel method, a valid, reproducible technique for achieving consensus. We present our recommendations for managing IRRs, which depend on patient characteristics including: prior history of IRRs to other monoclonal antibodies or medications, changes to crizanlizumab infusion rate and patient monitoring, pain severity relative to patient's typical SCD crises, and severe allergic symptoms. These recommendations outline how to evaluate and manage IRRs in patients receiving crizanlizumab. Future research should validate this guidance using clinical data and identify patients at risk for these IRRs.

COVID-19 in hospitalized adult patients with sickle cell disease: A 2020 US cohort using Cerner Real-World Data™ (CRWD).

We used Cerner Real-World Data™, representing hospital admission records from 2020, to examine patients with co-occurring sickle cell disease and COVID-19 by discharge disposition grouped as death/hospice versus transfers to other facilities, returned home, or left against medical advice. Among the death/hospice group, we found older age and higher rates of congestive heart failure and diabetes. There were also significant differences in tachypnea, mechanical ventilation, minimum O2 saturation, and length of stay with higher rates in the death/hospice group. Awareness of such factors and associated mortality risks for this population may aid in patient care.

Health Care Utilization by Adolescent/Young Adult Patients With Sickle Cell Disease in Delaware.

Patients with sickle cell disease transition from the pediatric to the adult health care system during a vulnerable time in their lives, resulting in increased morbidity and mortality during this adolescent/young adult (AYA) period. The purpose of this study is to examine the health care utilization of a cohort of adolescent/young adult patients with sickle cell disease in the main adult health care system in Delaware. Analysis of an electronic health record (EHR) data set of emergency department encounters and inpatient admissions for all patients with sickle cell disease between July 2016 and June 2017 was performed. This revealed significant variability in health care utilization by adolescent/young adult patients with sickle cell disease. There was a small cohort of high utilizer patients with multiple emergency department visits and inpatient admissions. These high-utilizing patients might benefit from targeted interventions and a primary care medical home. By analyzing health care utilization and identifying the needs of this chronic disease cohort, a comprehensive care program specifically for adolescents/young adults could be developed to address the needs of the patients and to correct the gaps in the current system.