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Objective: Sickle cell disease (SCD) is associated with complications during pregnancy and can negatively influence maternal outcomes. Our study aimed to determine the prevalence and predictors of maternal morbidity among participants enrolled in an eight-site SCD Implementation Consortium (SCDIC) registry., Methods: We conducted a cross-sectional analysis of female registry participants, aged 15-45 years, with a confirmed diagnosis of SCD. Participants completed a survey of self-reported pregnancies and outcomes., Results: Seven hundred and thirty-eight individuals had at least one pregnancy event, with 1076 live births. Twenty percent reported a pregnancy loss or fetal demise. Of the 1076 live births, 75% involved at least one complication. The most prevalent complications were pain crises (61.1%) and pregnancy requiring blood transfusion(s) (33.0%). Multiparous individuals with a prior occurrence of a complication in a previous pregnancy had higher odds of recurrence of the same complication in subsequent pregnancies (i.e., previous acute crisis was associated with subsequent acute pain events odds ratio [OR]: 3.13; 95% confidence interval [CI]: 2.06-4.76) and prior transfusion requiring another transfusion (OR: 3.22; 95% CI: 2.01-5.16)., Conclusion: Individuals reported a high prevalence of pregnancy loss and maternal complications. Our findings underscore the importance of preconception counseling and early initiation of perinatal care in SCD., (© 2024 International Federation of Gynecology and Obstetrics.)

Abstract: To address acute vaso-occlusive episodes (VOEs), the leading cause of emergency department (ED) visits among individuals with sickle cell disease (SCD), we conducted the clinical study, ALIGN (An Individualized Pain Plan with Patient and Provider Access for Emergency Department care of SCD), across 8 sites. We hypothesized an improvement of 0.5 standard deviations in perceived quality of ED pain treatment of a VOE after implementing individualized pain plans (IPPs) accessible to both patients and providers. Patients with SCD were aged 18 to 45 years, owned a cell phone, and had an ED VOE visit within 90 days prior. Patients completed the perceived quality of care surveys at baseline and within 96 hours after an ED VOE visit. Providers completed surveys regarding comfort managing VOEs at baseline and after managing an enrolled patient. Most of the 153 patients were African American (95.4%), female (64.7%), and had Hb SS/Sβ0 genotype (71.9%). The perceived quality of ED pain treatment was high at both baseline and after implementation of IPPs; our primary outcome hypothesis was not met, because no statistically significant change in the patient-perceived quality of ED treatment occurred. A total of 135 providers completed baseline and follow-up surveys. On a scale of 1 to 7, with 7 being extremely comfortable managing VOEs, 60.5% reported a score ≥6 after IPP implementation vs 57.8% at baseline. Almost all (97.6%) ordered the recommended medication, and 94.7% intended to use IPPs. In this implementation protocol, all sites successfully implemented IPPs. Patients and ED providers both endorsed the use of IPPs. This trial was registered at www.ClinicalTrials.gov as # NCT04584528., (© 2024 by The American Society of Hematology. Licensed under Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International (CC BY-NC-ND 4.0), permitting only noncommercial, nonderivative use with attribution. All other rights reserved.)

Objective: Sickle cell disease (SCD), the most common inherited blood disorder in the United States, is associated with severe psychoneurological symptoms. While epigenetic age acceleration has been linked to psychoneurological symptom burden in other diseases, this connection is unexplored in SCD. This study aimed to assess the association between epigenetic age acceleration and psychoneurological symptom burden in SCD. Methods: In this cross-sectional study, emotional impact, pain impact, sleep impact, social functioning, and cognitive function were assessed in 87 adults living with SCD. DNA methylation data were generated from blood specimens and used to calculate epigenetic age using five clocks (Horvath, Hannum, PhenoAge, GrimAge, & DunedinPACE). Associations between epigenetic age acceleration and symptoms were assessed. Results: The sample ( N = 87) had a mean (SD) chronologic age was 30.6 (8.1) years. Epigenetic age acceleration was associated with several symptom outcomes. GrimAge age acceleration (β = -0.49, p = .03) and increased DunedinPACE (β = -2.23, p = .004) were associated with worse emotional impact scores. PhenoAge (β = -0.32, p = .04) and the GrimAge (β = -0.48, p = .05) age acceleration were associated with worse pain impact scores. Increased DunedinPACE (β = -2.07 p = .04) were associated with worse sleep impact scores. Increased DunedinPACE (β = -2.87, p = .005) was associated with worse social functioning scores. We did not find associations between epigenetic age acceleration and cognitive function in this sample. Conclusion: Epigenetic age acceleration was associated with worse symptom experiences, suggesting the potential for epigenetic age acceleration as a biomarker to aid in risk stratification or targets for intervention to mitigate symptom burden in SCD., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Background: This study aimed to capture the implementation process of the ALIGN Study, (An individualized Pain Plan with Patient and Provider Access for Emergency Department care of Sickle Cell Disease). ALIGN aimed to embed Individualized Pain Plans in the electronic health record (E-IPP) and provide access to the plan for both adult patients with sickle cell disease (SCD) and emergency department providers when a person with SCD comes to the emergency department in vaso-occlusive crises., Methods: Semi-structured interviews were conducted with research teams from the 8 participating sites from the ALIGN study. Seventeen participants (principal investigators and study coordinators) shared their perspectives about the implementation of ALIGN in their sites. Data were analyzed in three phases using open coding steps adapted from grounded theory and qualitative content analysis., Results: A total of seven overarching themes were identified: (1) the E-IPP structure (location and upkeep) and collaboration with the informatics team, (2) the role of ED champion, (3) the role of research coordinators, (4) research team communication, and communication between research team and clinical team, (5) challenges with the study protocol, (6) provider feedback: addressing over-utilizers, patient mistrust, and the positive feedback about the intervention, and (7) COVID-19 and its effects on study implementation., Conclusions: Findings from this study contribute to learning how to implement E-IPPs for adult patients with SCD in ED. The study findings highlight the importance of early engagement with different team members, a champion from the emergency department, study coordinators with different skills and enhancement of communication and trust among team members. Further recommendations are outlined for hospitals aiming to implement E-IPP for patients with SCD in ED., (© 2023. The Author(s).)

Introduction: Sex-based clinical outcome differences in sickle cell disease (SCD) remain largely unknown despite evidence that female sex is associated with an increased lifespan. To better characterize sex-based differences in SCD, we assessed pain, treatment characteristics, laboratory measures and complications among males and females currently enrolled in the Sickle Cell Disease Implementation Consortium (SCDIC) registry., Methods: The SCDIC consists of eight comprehensive SCD centers and one data coordinating center that received funding from the National Heart Lung and Blood Institute to improve outcomes for individuals with SCD. Eligibility criteria included: 15 to 45 years of age and a confirmed diagnosis of SCD. Self-report surveys were completed and data were also abstracted from the participants' medical records., Results: A total of 2,124 participants were included (mean age: 27.8 years; 56% female). The majority had hemoglobin SS SCD genotype. Females had worse reports of pain severity (mean (SD) T-score 51.6 (9.6) vs 49.3 (10), p<0.001), more vaso-occlusive episodes (p = 0.01) and a higher occurrence of 3 or more hospital admissions in the past year (30.9% vs. 25.5, p = 0.03). On multivariable analysis, males had higher odds of acute chest syndrome (odds ratio (OR) 1.4, p = 0.002), cardiovascular (OR 1.70, p<0.001) and musculoskeletal (OR 1.33, p = 0.0034) complications and lower odds of depression (OR 0.77, p = 0.0381). Females had higher fetal hemoglobin levels with and without hydroxyurea use (9.6% vs 8.5%, p = 0.03 and 3% vs 2.2%, p = 0.0005, respectively)., Conclusion: Our data suggests that sex differences in clinical outcomes do occur among individuals with SCD. Future research needs to explore the mechanisms underlying these differences., Competing Interests: Jane S. Hankins receives consultancy fees from Forma Therapeutics, Global Blood Therapeutics and bluebird bio. All other authors have declared that no competing interests exist.

BACKGROUND Sickle cell disease (SCD) is a complex disease associated with many complications and a shortened lifespan. In 2016, the National Heart, Lung and Blood Institute funded 8 centers in the United States to form the Sickle Cell Disease Implementation Consortium, with the goal of improving SCD care. The aim of our study was to describe SCD self-efficacy, pain interference, and barriers to care from the perspective of persons with SCD in the North Carolina center. METHODS Persons with SCD, aged 15 and older were recruited from a large SCD center in North Carolina. Surveys, focus groups, and interviews were completed. RESULTS Fifty-one people completed a survey, and 14 people completed an interview or focus group. Barriers identified in the survey included self-care barriers, misconceptions related to hydroxyurea (an oral medication that reduces rates of pain crisis), limited provider knowledge, and stigma. Concerning self-efficacy, participants reported that they were able to manage their pain symptoms most of the time. Pain interfered most with the ability to participate in social and day-to-day activities.Common themes from the focus groups and interviews included misconceptions about hydroxyurea, pain, provider knowledge, stigma, co-management, transportation, and insurance. Recommendations to improve care included the use of case managers, utilization of treatment guidelines, individualized pain protocols, and effective co-management by providers. LIMITATIONS Participants were recruited from 1 SCD center and may not be representative of the entire SCD population in North Carolina. CONCLUSIONS Participants described many perceived barriers to care, and their responses suggest a need for improvements in patient hydroxyurea education, provider knowledge, and care coordination., (©2021 by the North Carolina Institute of Medicine and The Duke Endowment. All rights reserved.)

Sickle cell disease (SCD) is a genetic disorder predominantly affecting people of African descent and is associated with significant morbidity and mortality. To improve SCD outcomes, the National Heart Lung and Blood Institute funded eight centers to participate in the SCD Implementation Consortium. Sites were required to each recruit 300 individuals with SCD, over 20 months. We aim to describe recruitment strategies and challenges encountered. Participants aged 15-45 years with confirmed diagnosis of SCD were eligible. Descriptive statistics were used to analyze the effectiveness of each recruitment strategy. A total of 2432 participants were recruited. Majority (95.3%) were African American. Successful strategies were recruitment from clinics (68.1%) and affiliated sites (15.6%). Recruitment at community events, emergency departments and pain centers had the lowest yield. Challenges included saturation of strategies and time constraints. Effective recruitment of participants in multi-site studies requires multiple strategies to achieve adequate sample sizes.

Importance: Sickle cell disease (SCD) is the most common inherited red blood cell disorder in the United States, and previous studies have shown that individuals with SCD are affected by multiple health disparities, including stigmatization, inequities in funding, and worse health outcomes, which may preclude their ability to access quality health care. This needs assessment was performed as part of the Sickle Cell Disease Implementation Consortium (SCDIC) to assess barriers to care that may be faced by individuals with SCD., Objective: To assess the SCD-related medical care experience of adolescents and adults with SCD., Design, Setting, and Participants: This one-time survey study evaluated pain interference, quality of health care, and self-efficacy of 440 adults and adolescents (aged 15 to 50 years) with SCD of all genotypes and assessed how these variables were associated with their perceptions of outpatient and emergency department (ED) care. The surveys were administered once during office visits by trained study coordinators at 7 of 8 SCDIC sites in 2018., Results: The SCDIC sites did not report the number of individuals approached to participate in this study; thus, a response rate could not be calculated. In addition, respondents were not required to answer every question in the survey; thus, the response rate per question differed for each variable. Of 440 individuals with SCD, participants were primarily female (245 [55.7%]) and African American (428 [97.3%]) individuals, with a mean (SD) age of 27.8 (8.6) years. The majority of participants (306 of 435 [70.3%]) had hemoglobin SS or hemoglobin S β0-thalassemia. Most respondents (361 of 437 [82.6%]) reported access to nonacute (usual) SCD care, and the majority of respondents (382 of 413 [92.1%]) noted satisfaction with their usual care physician. Of 435 participants, 287 (66.0%) reported requiring an ED visit for acute pain in the previous year. Respondents were less pleased with their ED care than their usual care clinician, with approximately half (146 of 287 [50.9%]) being satisfied with or perceiving having adequate quality care in the ED. Participants also noted that when they experienced severe pain or clinician lack of empathy, this was associated with a negative quality of care. Age group was associated with ED satisfaction, with younger patients (<19 vs 19-30 and 31-50 years) reporting better ED experiences., Conclusions and Relevance: These results suggested that a negative perception of care may be a barrier for patients seeking care. These findings underscore the necessity of implementation studies to improve access to quality care for this population, especially in the acute care setting.

Introduction: Scale up of anti-retroviral therapy has enabled millions of children infected with HIV to survive into adulthood, requiring transition of care to the adult HIV clinic. This transition period is often met with anxiety and reluctance. Youth who fail to transition may create strain on capacity in the pediatric and adolescent clinics or result in individuals dropping out of care entirely. This study examined challenges and facilitators to the transition among young adults living with HIV in Moshi, Tanzania., Methods: From April to June 2017, in-depth interviews were conducted with young adults aged 18 to 27 years living with HIV in order to capture the spectrum of experiences from pre-transitioning youth to those who successfully transitioned to adult care. Young adults were purposively recruited based on prior study enrollees and recommendations from healthcare staff. Recruitment occurred in the adolescent, adult HIV and the prevention of mother to child transition clinics at Kilimanjaro Christian Medical Centre. Two separate in-depth interviews were conducted with eligible participants. Medical records were reviewed retrospectively to collect information on HIV-related outcomes., Results: In-depth interviews were held with 19 young adults. Participants mean age was 23.8 years (interquartile range 22.2 to 26.3 years); 53% were female. Most (78.9%) participants had been receiving anti-retroviral therapy for nearly a decade and 72.2% were virologically suppressed (HIV RNA <200 copies/mL). Barriers to transition included fear of losing peer networks formed in the adolescent clinic, the abrupt manner in which young adults were asked to transition, stigma, financial constraints and a lower quality of care in the adult clinic. Facilitators of transition included family and social support, positive perspectives on living with HIV and maintenance of good health. Recommendations for transition included transition preparation, transition as a group and adoption of desirable aspects of the adolescent clinic (peer networks and education) in the adult clinic., Conclusions: Transition is a complex process influenced by many factors. As the number of young adults living with HIV continues to grow, it is vital to develop a transition protocol that addresses these challenges and is feasible to implement in low-resource settings., (© 2019 The Authors. Journal of the International AIDS Society published by John Wiley & Sons Ltd on behalf of the International AIDS Society.)

Background: Sickle cell disease (SCD) is an inherited blood disorder associated with acute pain crisis and other complications that lead to frequent emergency department (ED) visits. To improve outcomes, the National Heart, Lung and Blood Institute (NHLBI) published recommendations for management of acute pain crisis. NHLBI also funded eight centers across the United States to participate in the Sickle Cell Disease Implementation Consortium. This six-year effort consists of two phases. Phase one involved conducting needs assessments of barriers and facilitators to SCD care. The aim of this study was to describe challenges and facilitators to caring for SCD from the perspective of ED providers in central North Carolina (NC)., Methods and Findings: We conducted a needs assessment survey with ED providers throughout NC. We also conducted focus groups and an interview with ED providers from three healthcare facilities in central NC. One hundred and eleven surveys (60.6% physicians, 26% registered nurses, 13.5% physician assistants) were completed and 13 providers participated in 3 focus groups and 1 interview. Slightly more than half (50. 4%) utilized individualized dosing protocols to treat sickle cell pain. Only 32.4% of the providers were aware of the NHLBI SCD recommendations. Barriers to care from the survey included: patient behavior (67.57%), the opioid epidemic (67.57%), overcrowding (64.86%), and concern about addiction (49.55%). Perceived barriers to care identified in the focus groups and interview included: high patient volumes, lack of SCD care protocols, poor communication among providers and stigma. Facilitators to care included: individualized pain plans, comfort prescribing opioids and electronic medical records., Conclusion: SCD care is influenced by many factors. Our results illuminate the need for increased use of the NHLBI SCD recommendations, individualized pain protocols, and use of electronic medical records and other care-interventions, specifically geared towards improving provider knowledge and mitigating provider bias., Competing Interests: The authors have declared that no competing interests exist.