First global guideline of the World Health Organization on pregnancy care in sickle cell disease: Balancing maternal equity and ethical accountability

Health Equity in Sickle Cell Research and Access to Therapy

Patient outcomes for sickle cell disease with support staff-driven interventions: A scoping review

HIV and sickle cell disease (SCD) are significant causes of morbidity and mortality in sub-Saharan Africa. Given their separate roles in immune dysregulation, our objective was to characterise the impact that SCD has on the presentation and progression of paediatric HIV. The study was a retrospective cohort study (study period 2004-2018). Cases of HIV+and SCD-afflicted patients (HIV+/SCD+) were obtained via electronic chart review from a paediatric HIV clinic in Kampala, Uganda and matched 1:3 with HIV+controls without SCD (HIV+/SCD-). Thirty-five HIV+/SCD+subjects and 95 HIV+/SCD- controls were analysed (39% female (51/130), age 3.6years (SD3.9)). At baseline, WHO clinical stage (64% total cohort Stage III/IV) and nutritional status (9.4% severe acute malnutrition) were similar for both groups, whereas HIV+/SCD+had higher though non-significant baseline CD4 count (1036 (SD713) vs 849 (SD638) cells/microlitre, P=0.20, two-tailed t-test). There were 19 deaths, 6 (17%) HIV+/SCD+and 13 (14%) HIV+/SCD-, with unadjusted/adjusted models showing no significant difference. Nutritional progression and clinical stage progression showed no significant differences between groups. Kaplan-Meier analysis showed a slower rate of treatment failures in the HIV+/SCD+cohort (P=0.11, log-rank survival test). Trajectory analysis showed that in the time period analysed, the HIV+/SCD+cohort showed a more rapid rise and higher total CD4 count (P=0.012, regression analysis). The study suggests that SCD does not adversely affect the progression of HIV in patients on ART. Further, HIV+/SCD+achieved higher CD4 counts and fewer HIV treatment failures, suggesting physiological effects due to SCD might mitigate HIV progression.

Examining the Role of Sickle Cell Disease Patients As Teachers in the Emergency Department: Patient Directed Physician Education

“We Have to Change How We Interact with the Patient”: Comprehensive Sickle Cell Care Is the Anchor for Inpatient Care Complexity

Letter to the Editor: Physicians' Opinions of COVID-19 Ambulatory Care Constraints: A Survey of Sickle Cell Clinicians.

High Rate of Attendance at a Young Adult Clinic Embedded in an Adult Sickle Cell Center: If You Build It, They Will Come

Dismantling systemic barriers in sickle cell disease care: Design and rationale of a municipal public health initiative in New York City

Novel Electronic Consultation for Sickle Cell Disease Inpatient Management

BACKGROUND Transitioning from pediatric to adult sickle cell disease (SCD) care is challenging for emerging adults (17-25 years old). This period is marked by a seven-fold increase in mortality rates, and has the highest rates of hospitalizations, emergency room visits, and hospital readmissions compared to children living with SCD. These challenges are exacerbated by fragmented care coordination, difficulty navigating adult healthcare systems, and increased self-management responsibilities. OBJECTIVE This study aims to compare the effectiveness of two interventions designed to support emerging adults living with SCD during this transition: a mobile health (mHealth) application and community health worker (CHW) support to standard care. METHODS The Community Health Workers and Mobile Health for Emerging Adults Transitioning Sickle Cell Disease Care (COMETS Trial) is an ongoing multicenter, three-arm, open-label randomized controlled trial (RCT); 375 emerging adults (17 to 25 years old) are being enrolled and randomized 1:1:1 to (1) a 6-month CHW intervention focused on self-management skills, symptom tracking, care coordination, and transition planning, (2) a 6-month mHealth self-management program (enhanced iManage application) with tailored SMS texting (THRIVE 2.0), or (3) enhanced usual care (control). Participants are followed for 18 months. The primary outcome is the change in self- reported health-related quality of life (HRQOL) assessed using the PedsQL Sickle Cell Disease Module. Secondary outcomes include acute care utilization (hospitalizations, ED visits), patient activation, self-management behavior, and successful transfer to adult hematology care. RESULTS The Institutional Review Board (IRB) at Children’s Hospital of Philadelphia approved this study in June 2018. We have enrolled a total of 405 participants. CONCLUSIONS This trial addresses a critical gap in transition intervention research for young adults with SCD. It will provide evidence on the comparative effectiveness of two promising interventions (CHW and mHealth) and inform the development of scalable and sustainable transition support programs. Findings will have implications for improving HRQOL, reducing acute care utilization, and promoting successful transition to adult-centered SCD care for this vulnerable population. CLINICALTRIAL ClinicalTrials.gov NCT03648710: https://clinicaltrials.gov/study/NCT034648710

Health Care Disparities in Sickle Cell Disease : A Population-Based Study of Los Angeles County.

Transition Experiences of Adolescents and Young Adults (AYA) with Sickle Cell Disease at the Ghana Institute of Clinical Genetics (GICG), Korle Bu in Accra, Ghana

Background Sickle cell disease (SCD) is a significant public health concern in New York City, home to much of the state’s estimated 10,000 individuals with SCD, with the highest burden in the Bronx and Brooklyn. Despite advances in treatment, individuals with SCD continue to experience disparities in healthcare access, quality of care, and health outcomes. New York City Department of Health and Mental Hygiene (DOHMH) has led in SCD care, pioneering the first effective newborn screening program for SCD and sickle cell trait (SCT). Building on this legacy, the NYC DOHMH is improving equitable healthcare, enhancing provider education about SCD and SCT, and addressing systemic challenges. This abstract highlights the department’s role in SCD surveillance, provider education, policy development, and patient-centered interventions to improve health outcomes. Methods A review of NYC DOHMH policies, advisories, research initiatives, and partnerships was conducted. This included an analysis of: • Health advisories and statements on SCD • Surveillance data on SCD hospitalizations, prevalence, and health disparities. • DOHMH-supported research initiatives examining comorbidities in children with SCD. • Collaborations with NYC Health + Hospitals and other institutions Results NYC DOHMH has implemented multiple interventions to support individuals living with SCD, including: 1. Provider Education and Health Advisories: In 2024, the department issued Health Advisory #15, which urged providers to utilize evidence-based pain management strategies and adopt neutral language to reduce stigma in the emergency department (ED). The department also hosted a webinar to address providers’ concerns regarding pain management in the ED and sent a “Dear Colleague” letter to educate providers about sickle cell trait. 2. Surveillance & Data Collection: Recent analyses indicate that NYC had over 5,000 SCD-related hospitalizations annually, which are about evenly distributed among women and men, though there are significant geographic disparities. Tracking discharges against medical advice as a tool to understand systemic changes. The department began tracking DAMA rates as an indicator of systemic gaps in SCD care. Tailored interventions are being piloted to improve retention. 3. Research on Comorbidities: The department investigated potential environmental risk factors, such as the association between lead poisoning and SCD complications. 4. Community Engagement & Care Coordination: DOHMH has collaborated with hospitals and advocacy groups to improve providers and community understanding of SCD and SCT. Conclusions The New York City Department of Health and Mental Hygiene plays a pivotal role in addressing sickle cell disease through policy advocacy, surveillance, provider education, and research initiatives. While progress has been made, continued public health efforts are needed to improve comprehensive SCD care, reduce health disparities, and address workforce training. Lessons from NYC’s approach may be replicable in other urban centers facing similar SCD-related challenges.

Background Hydroxyurea (HU) is safe and effective oral therapy for sickle cell disease (SCD). Its use significantly reduces complications and improves survival and quality of life. In 2014 the National Heart, Lung, and Blood Institute recommended HU for all children with homozygous SCD and sickle beta-zero thalassemia. However, despite these guidelines and HUs proven efficacy, use remains low. Barriers include access to comprehensive care, social economic challenges, mistrust and misinformation. Recognizing these gaps, the Office of Minority Health (OMH) awarded NYC Health+Hospitals (H+H) a grant 2020 to increase HU use among eligible children. To address this problem, we used a multifaceted approach, combining provider education system enhancements, financial incentive, and community-based partnership s. These interventions increased HU prescribing and adherence rates and decreased emergency department visits. This study evaluates the role CBO -hospital partnerships in improving uptake and identifies key structural and policy changes necessary for lasting impact. Methods Health Research Inc. Our independent evaluator conducted a mixed-methods evaluation to assess the contributions of CBOs in supporting patients and families. Key data sources included: 1) interviews with healthcare providers, CHWs, and administrators, 2) provider surveys measuring engagement and perceived effectiveness of CBO partnerships, and 3) tracking CBO referrals and social support interventions. Results Our findings indicated that Community-Based Organizations (CBOs) played a vital role in improving adherence to health interventions (HU) and reducing Emergency Department visits. They accomplished this by addressing healthcare gaps, enhancing patient engagement, tackling social determinants of health, and providing culturally competent education and support. Specifically, their contributions included:1. Providing wraparound support: They connected families with essential resources such as food security, housing assistance, and help with navigating insurance, often in collaboration with Community Health Workers (CHWs). 2. Enhancing patient and caregiver education: They organized awareness sessions about health interventions, established peer support networks, and offered culturally relevant educational materials to dispel myths and improve adherence.3. Bridging healthcare gaps:—CHWs, in partnership with CBOs, facilitated referrals and follow-ups, which improved communication between providers and patients and ensured continuity of care. Conclusions Our findings support the transformative potential of CBO-hospital partnerships in improving hydroxyurea (HU) uptake and comprehensive sickle cell disease (SCD) care. By integrating CBOs into hospital-based care models, these partnerships bridge critical gaps in access, patient engagement, and social support, leading to improved adherence and reduced emergency department utilization.To sustain these gains, health systems, and policymakers must prioritize the formalization of CBO-hospital collaborations through:• Structured referral pathways that ensure patients receive consistent support beyond clinical settings.• Sustainable funding models that move beyond grant-based support, embedding CBO partnerships into long-term healthcare financing and policy frameworks. By fostering coordinated partnerships between hospitals and CBOs, healthcare systems can build trust, improve outcomes, and advance health equity for individuals with SCD. Expanding and institutionalizing these collaborations will ensure lasting improvements in HU adherence, patient-centered care, and overall health system effectiveness.

Role efficiency in psychosocial care of sickle cell disease: A national survey of social workers