Weekly Journal of Sickle Cell Disease Publication Update
Stay current with the latest sickle cell research, regulatory updates, and guidelines.
Preliminary observations on cerebral blood flow and pain sensitivity in adults with SCD
To explore the relationship between cerebral blood flow (CBF) and pain sensitivity in adults with sickle cell disease (SCD).
An international multicenter cohort shows sickle cell disease is no longer uniformly associated with low body weight, with persistent undernutrition alongside increasing overweight and obesity across age groups, genotypes, and countries.
To characterize body mass index (BMI) distribution and associated clinical factors in individuals with sickle cell disease across diverse global settings.
Elevated ambient nitrogen dioxide (NO₂) levels were associated with greater odds of abnormal pulmonary function tests in children and young adults with sickle cell disease (SCD), highlighting an environmental factor that may influence respiratory health in this population.
To evaluate whether ambient air pollution exposures, particularly criteria pollutants, are associated with abnormal pulmonary function tests (PFTs) among young people living with SCD.
A 5-year-old girl with sickle cell disease (Hb SS) and poor hydroxyurea adherence developed concurrent warm autoimmune hemolytic anemia (wAIHA), splenic sequestration, and hyperhemolysis/delayed hemolytic transfusion reaction after transfusion, illustrating the complexity of immune-mediated hemolysis and the role of multidisciplinary, phenotype-matched intervention.
To present a clinical case illustrating the diagnostic and therapeutic challenges when multiple severe hemolytic complications of SCD converge in a single pediatric patient.
In chronically transfused adults with sickle cell disease (SCD), a retrospective analysis found that a substantial subgroup experienced sleep-associated hemoglobin desaturation (SpO₂ ≤ 88% for ≥5 minutes), which was associated with markers of hemolysis and inflammation, underscoring the need to consider sleep-related hypoxemia in comprehensive SCD care.
To characterize the prevalence and clinical correlates of nocturnal hemoglobin desaturation (NHD) in adults with SCD managed with chronic red blood cell exchange transfusion.
A pooled analysis of Phase 2 and Phase 3 clinical trials provides a detailed safety profile of crizanlizumab 5.0 mg/kg in sickle cell disease (SCD), showing predominantly mild to moderate adverse events alongside infrequent but notable serious infections and bleeding events.
To evaluate the safety of crizanlizumab 5.0 mg/kg by pooling adverse-event data across multiple clinical trials in individuals with SCD.
A retrospective analysis of Medicaid-insured children with sickle cell disease (SCD) found that those with longer continuous coverage had significantly better adherence to oral Hydroxyurea (HU), underscoring the role of stable insurance and continuity in optimizing SCD therapy.
To investigate whether the length of continuous Medicaid enrollment (12- vs. 24-month coverage) affects adherence to hydroxyurea among children with SCD.
Advanced neuroimaging in adults with sickle cell disease (SCD) revealed elevated cerebral blood flow (CBF), reduced cortical amplitude of low-frequency fluctuation (ALFF), increased white-matter ALFF, and altered functional connectivity — patterns associated with slower cognitive processing and higher pain sensitivity.
To characterize CBF, ALFF, and resting-state functional connectivity in adults with SCD compared with matched healthy controls, and relate these measures to cognition and pain sensitivity.
This study describes 46 children and adolescents with SCD who met criteria for high-impact chronic pain (HICP) at the time of hematopoietic cell transplant (HCT) consultation, highlighting significant pain burden, healthcare utilization, and clinical complexity within this referred subgroup.
To characterize the clinical phenotype of HICP among children and adolescents with SCD referred for HCT consultation, including pain frequency, healthcare utilization (HCU), analgesic exposure, and comorbidities such as AVN.
A preclinical study in humanized sickle-cell mice found that older animals exhibited heightened evoked and non-evoked pain-related behaviors along with age-associated gene-expression changes data that may help inform future research on aging and pain in SCD.
To examine how increasing age influences pain-related behaviors and associated gene-expression patterns in a humanized sickle cell disease mouse model (HbSS-BERK).