Clinical effects of dexamethasone among patients with sickle cell disease hospitalized with COVID-19: Outcomes from a single academic health system.

Background: Dexamethasone is a steroid used in the treatment of hospitalized patients with severe COVID-19. However, the effect of dexamethasone in patients with SCD remains unclear given that steroids may precipitate vaso-occlusive crisis (VOC) in patients with SCD.

Methods And Findings: We performed a retrospective analysis of patients with SCD who were hospitalized at Johns Hopkins Health System between June 1, 2020 and June 26, 2022.

Attitudes and Beliefs Regarding Pain and Discrimination Among Black Adults with Sickle Cell Disease: A Mixed Methods Evaluation of an Adapted Chronic Pain Intervention.

Purpose: This study sought to adapt a chronic pain group curriculum for adults with sickle cell disease (SCD). Given the association of experiences of racism and discrimination with health outcomes, this study also explored how such experiences for patients with SCD impact their interactions with clinicians and health-care systems.

Patients And Methods: This mixed methods study recruited patients (aged ≥18 years) in a sickle cell treatment program; all self-identified as Black or African American.

Late effects of hemopoietic stem cell transplant for sickle cell disease: monitoring and management.

Introduction: Allogeneic hemopoietic stem cell transplantation (HSCT) is a curative therapy for sickle cell disease (SCD). Exposure to both SCD and HSCT conditioning regimens is associated with late health effects.

Areas Covered: This review addresses post-HSCT outcomes and late health effects among individuals with SCD exposed to allogeneic HSCT regimens, summarizes recommendations for long-term care, and identifies future survivorship research needs.

The use of abstract animations and a graphical body image for assessing pain outcomes among adults with sickle cell disease.

Painimation, a novel digital pain assessment tool, allows patients to communicate their pain quality, intensity, and location using abstract animations (painimations) and a paintable body image. This study determined the construct validity of painimations and body image measures by testing correlations with validated pain outcomes in adults with sickle cell disease (SCD). Analyses used baseline data from a multisite randomized trial of 359 adults with SCD and chronic pain.

Genetic contributions to pain modulation in sickle cell: A focus on single nucleotide polymorphisms.

Background: Despite recent advances in our knowledge of genetic contributions to the highly variable sickle cell disease (SCD) phenotype, our understanding of genetic factors associated with pain sensitivity in SCD remains limited. Previous studies investigated specific variants in single candidate genes and their association with SCD pain variability. The primary aim of the current study was to expand the genes and polymorphisms tested to discover new risk genes (polymorphisms) associated with central sensitization for individuals with SCD.

Chronic automated red cell exchange therapy for sickle cell disease.

Background: The data to support chronic automated red cell exchange (RCE) in sickle cell disease (SCD) outside of stroke prevention, is limited, especially in adults.

Study Design And Methods: A retrospective analysis was conducted of patients with SCD who were referred for chronic RCE at our institution over a 10-year period. Data that were evaluated included patient demographics, referral indications, and procedural details (e.

COVID-19 mRNA vaccination responses in individuals with sickle cell disease: an ASH RC Sickle Cell Research Network Study.

Children and adults with sickle cell disease (SCD) have increases in morbidity and mortality with COVID-19 infections. The American Society of Hematology Research Collaborative Sickle Cell Disease Research Network performed a prospective COVID-19 vaccine study to assess antibody responses and analyze whether messenger RNA (mRNA) vaccination precipitated any adverse effects unique to individuals with SCD. Forty-one participants received 2 doses of the Pfizer-BioNTech vaccine and provided baseline blood samples before vaccination and 2 months after the initial vaccination for analysis of immunoglobulin G (IgG) reactivity against the receptor binding domain (RBD) of the severe acute respiratory syndrome coronavirus 2 spike protein.

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).

Short-term air pollution levels and sickle cell disease hospital encounters in South Carolina: A case-crossover analysis.

Background: Sickle cell disease (SCD) is a genetic disorder and symptoms may be sensitive to environmental stressors. Although it has been hypothesized that exposure to outdoor air pollution could trigger acute SCD events, evidence is limited.

Methods: We obtained SCD administrative data on hospital encounters in South Carolina from 2002 to 2019.

Disparities in pediatric hospital use during transition to adult healthcare for young adults with childhood-onset chronic conditions.

Background: Young adults (YA) with childhood-onset chronic conditions-particularly YA with cystic fibrosis (CF), congenital heart disease (CHD), and sickle cell disease (SCD)-continue to have pediatric hospital admissions. Factors associated with this continued pediatric hospital use remain underexplored.

Objective: To determine if pediatric hospital use by YA differed (1) across condition and (2) within each condition by sociodemographic factors.

Sleep Disruption Moderates the Daily Dynamics of Affect and Pain in Sickle Cell Disease.

Persons with sickle cell disease (SCD) often experience pain that can interfere with quality of life and daily activities. Pain can modulated by affect and sleep continuity; however, few studies have explored how these factors complementarily influence pain in adults with SCD. The study aims were to investigate 1) whether pain levels were heightened on days characterized by low positive affect and high negative affect, and 2) whether the relationship between affect and pain was intensified following nights of disrupted sleep.

Managing sickle cell disease and related complications in pregnancy: results of an international Delphi panel.

Data to guide evidence-based management of pregnant people with sickle cell disease (SCD) are limited. This international Delphi panel aimed to identify consensus among multidisciplinary experts for SCD management during pregnancy. The 2-round Delphi process used questionnaires exploring 7 topics (antenatal care, hydroxyurea use, transfusion, prevention of complications, treatment of complications, delivery and follow-up, and bottlenecks and knowledge gaps) developed by a steering committee.

Mental health, pain and likelihood of opioid misuse among adults with sickle cell disease.

Depressive symptoms are prevalent in individuals living with sickle cell disease (SCD) and may exacerbate pain. This study examines whether higher depressive symptoms are associated with pain outcomes, pain catastrophizing, interference and potential opioid misuse in a large cohort of adults with SCD. The study utilized baseline data from the 'CaRISMA' trial, which involved 357 SCD adults with chronic pain.

Blood pressure thresholds for the diagnosis of hypertensive disorders of pregnancy in sickle cell disease.

In this retrospective cohort study of singleton pregnancies in people with sickle cell disease (SCD) delivered at two academic centres between 1990 and 2021, we collected demographic and SCD-related data, pregnancy outcomes, and the highest systolic and diastolic blood pressure (SBP and DBP) at seven time periods. We compared the characteristics of subjects with new or worsening proteinuria (NWP) during pregnancy to those without. We then constructed receiver operating characteristic (ROC) curves to determine the blood pressure (BP) that best identifies those with NWP.

Euvolemic automated transfusion to treat severe anemia in sickle cell disease patients at risk of circulatory overload.

Background: Red blood cell (RBC) transfusion remains a major treatment for sickle cell disease (SCD). Patients with SCD have a high prevalence of renal impairment and cardiorespiratory disease, conferring risk of transfusion-associated circulatory overload (TACO).

Study Design And Methods: We describe an approach, titled euvolemic automated transfusion (EAT), to transfuse SCD patients with severe anemia who are at risk of TACO.

Natural history of blood pressure in sickle cell disease pregnancy.

In this retrospective cohort study of singleton pregnancies in people with sickle cell disease (SCD) delivered at two academic centres between 1990 and 2021, we collected demographic and SCD-related data, pregnancy outcomes, and the highest systolic and diastolic blood pressure (SBP and DBP) at seven time periods. We compared blood pressure values and trajectories in the composite cohort and in each genotype group to control values in a non-SCD pregnancy dataset. There were 290 pregnancies among 197 patients with SCD.

"Buprenorphine, It Works so Differently": Adults with Sickle Cell Disease Describe Transitioning to Buprenorphine for Treatment of Chronic Pain.

A growing body of literature describes the use of buprenorphine for the treatment of chronic pain in people with sickle cell disease. The experiences of people with sickle cell disease who have tried buprenorphine have not yet reported. This qualitative descriptive study was conducted to explore perspectives on buprenorphine for chronic pain in sickle cell disease.

"The Patient Should Have a Choice": Adults with Sickle Cell Disease Advise Integration of Telemedicine into the Comprehensive Sickle Cell Disease Care Model.

Background: Adults with sickle cell disease (SCD) constitute a unique and vulnerable patient population with complex healthcare needs including routine follow-up visits and acute care evaluations. The COVID-19 pandemic accelerated healthcare systems' transition to providing telemedicine care. The purpose of this qualitative study was to elicit the perspectives of adults with SCD about their experience with telemedicine during the COVID-19 pandemic and to understand their preferences with respect to future telemedicine care.