Andrew D. Campbell

Background: COVID‐19 had a disproportionate impact on the health of individuals with sickle cell disease (SCD). While numerous studies have documented that patients with SCD were at increased risk for morbidity and mortality, limited research has examined its effects on social determinants of health (SDOH) among families of children with SCD. Procedure: The study was divided into two phases: early pandemic and late pandemic. Caregivers completed surveys about food insecurity, housing, and employment status. Results: Fifty‐nine caregivers were surveyed during the early pandemic period; 61 were surveyed during the late pandemic period. Caregivers were significantly more likely to report food insecurity in the late pandemic period as compared to the early pandemic period (OR = 4.47; 95% CI = 1.20, 20.46). Those with lower monthly income ($1000–1999) were more likely to report food insecurity, as compared to caregivers within the monthly income range of $4000 or more. Caregivers who were born outside the United States and who had lower income were significantly more likely to report food insecurity as compared to caregivers who were born in the United States or had an income within the range of $4000. No significant differences were noted between the two periods in terms of housing, employment, or financial insecurity. Conclusions: We found significant associations between demographic characteristics and SDOH. While some aspects of food insecurity worsened during the late pandemic, others improved. We did not observe significant changes in housing or employment insecurity across pandemic periods, potentially suggesting either resilience or sustained hardship.

Pain is a leading cause of morbidity and healthcare utilization in sickle cell disease (SCD), and opioids remain central to treating vaso-occlusive and chronic pain. Yet opioid response varies widely, raising the question of whether pharmacogenetic testing should inform opioid prescribing. Our review examined the PubMed literature on pharmacogenomics and opioid efficacy in SCD. We focus on CYP2D6 as the clearest current pharmacogenetic signal for codeine and tramadol, and assess SCD-specific implementation studies, preemptive testing, and African pharmacoequity. The current evidence supports targeted CYP2D6-informed prescribing in selected contexts rather than universal testing for all opioids, while highlighting the need to integrate genotype with pain phenotype, drug–drug interactions, liver function, and clinically grounded implementation studies, and better characterize African and African-ancestry pharmacogene variation.

Objectives: To determine the spectrum of ocular complications of sickle cell disease (SCD) and age of onset of proliferative sickle cell retinopathy (PSR) in children attending the pediatric clinic at a tertiary hospital in southern Ghana. Methods: A cross-sectional study of all children with SCD in steady state, ages 2 to 16 years. Demographic and clinical data were recorded using predesigned forms. The ocular manifestations of SCD were classified as either proliferative or non-proliferative based on the presence or absence of neovascularization, respectively. Results: Included in this study were 294 children, (53.4% males), mean age 9.2 ± 3.7 years and majority had HbSS-199 (67.9%) and HbSC-74 (25.3%) genotypes. Comma-shaped conjunctiva vessels were the commonest anterior segment manifestation occurring in 85% of children. Non-proliferative posterior segment signs included: venous tortuosity 69 (23.5%), salmon-patch hemorrhages 4 (1.4%), iridescent spots 38 (12.9%), and black sunburst 24 (8.2%). Prevalence of PSR of any stage among children with HbSS was 2.5% (5/199) and 8.1% (6/74) among those with HbSC (P = .0366). The earliest age PSR was detected in this cohort was 8 years and for PSR stage 3, 13 years. No child was blind from eye complications of SCD or any other cause. Conclusion: Comma-shaped conjunctiva vessels were the commonest anterior segment manifestation of SCD, and retinal venous tortuosity was the most common posterior segment manifestation. Proliferative retinopathy was more common in HbSC genotype.

Analysis of the placebo cohort in the STArT trial shows that patient characteristics and hospital site strongly influence time-to-crisis-resolution and total opioid use in children and young adults with sickle cell disease, highlighting the need to account for these factors in the design of future clinical trials.

Background: Sickle cell anaemia (SCA) is associated with a significantly increased risk of stroke, primarily assessed using transcranial Doppler ultrasound (TCD). However, access to TCD and haemoglobin F (HbF) testing remains limited in resource-constrained settings. Our preliminary findings identified the platelet-neutrophil ratio (PNR) as a promising biomarker for stroke risk stratification. This updated study evaluates the predictive value of PNR while adjusting for HbF levels and transfusion history. Methods : We conducted a retrospective study of 248 HbSS children and young adults at Children’s National Hospital, Washington DC. Demographic, clinical, and hematologic parameters—including PNR, HbF levels, and transfusion status—were analysed in relation to TCD results. Logistic regression and ROC curve analyses were performed. Results: Subjects with abnormal TCD values had significantly lower PNR (53.05 vs. 87.65, pConclusion: PNR remains a cost-effective and accessible biomarker for stroke risk stratification, retaining its predictive strength even after adjusting for HbF and transfusion status.

Rapid treatment and frequent reassessment of pain are key components of treatment guidelines for acute sickle cell disease (SCD) pain. Few studies, however, report the associations between emergency department (ED) pain scores, number of ED opioid doses, receipt of an opioid prescription, ED visit disposition, or ED return visits. This seven‐site retrospective cohort study analyzed 4983 ED visits by children with SCD pain using electronic health record data from the Pediatric Emergency Care Applied Research Network Registry. ED pain scores included initial, last, and change in scores (initial minus last), measured on a 0–10 scale. Dispositions of discharge and hospital admission were included. Modified Poisson regression and the Cochran–Armitage test of trend were used for analysis. The median (IQR) initial pain score was 8.0 (6–10); last pain score was 5.0 (2–8); and median decrease was 2.0 (0–5). In multivariable analysis, last pain score was the best predictor of disposition. For the return visit analyses, of the 2377 visits discharged at index ED visit, 29% returned within 14 days. Higher initial and last ED pain scores were associated with increased return visits. Children with no opioid discharge prescription and ≥3 ED opioid doses had a return visit rate of 36% compared to 22% if the child received an opioid prescription and only one ED opioid. Increasing discharge opioid prescriptions and targeting interventions for those who receive multiple ED opioid doses could decrease return visits.