ALBERT

Description: Introduction Sickle cell disease (SCD) is the most common inherited blood disorder in Jamaica. SCD is associated with significantly shortened lifespans globally. SCD has serious health complications and individuals with SCD often experience poor quality of life (QoL) and high levels of perceived stigma as a result. Stigma acts as a barrier to self-management, and affects morbidity, mortality, and QoL in individuals with SCD. Sources and perceptions of stigma depend largely on culture, society, and environment. The purpose of this study is to describe health-related QoL, perceived stigma, and measure the relationships between these concepts in adults with SCD in Jamaica. Methods Inclusion criteria were 〉18 years of age and self-reported diagnosis of a SCD genotype. Participants were recruited from the Sickle Cell Unit at The University of the West Indies in Kingston, Jamaica. Participants (n=50) completed interviewer-administered surveys including demographic/clinical characteristics; SCD Health Related Stigma Scale (SCD-HRSS) subscales: family, general public, doctors, nurses (scores range 10-60, total 40-240); the Measure of Sickle Cell Stigma (MoSCS) subscales: disclosure concerns, expected discrimination, social exclusion, internalized stigma (scores range 3-18, 2-12, 3-18, 3-18 respectively; total 11-66); and the Adult Sickle Cell Quality of Life Measurement System (ASCQ-Me) subscales: Emotional Impact, Sleep Impact, Social Functioning Impact, Stiffness Impact, Pain Impact, and Pain Episode Frequency and Severity. ASCQ-Me uses a T-score metric where 50 is the mean of the reference population and 10 is the standard deviation. The value of 50 indicates the health score of the average field test respondent during testing of the ASCQ-Me in the United States. Higher scores indicate healthier status for all subscales except pain episode frequency and severity. Scores were determined by using the online scoring system at Healthmeasures.net as recommended. The MoSCS and SCD-HRSS are both scored by obtaining summing the mean score of the subscales; higher scores indicate higher perceived stigma. Descriptive statistics were used to report scores per subscale. To identify a relationships between QoL and stigma a set of Spearman point-biserial correlation analyses were performed. Results Fifty individuals (average age 34.4 +/- 11.4; 94% Black) were recruited, 22 (44%) males and 28 (56%) females. The majority of the sample reported low disease severity. Participants reported average to healthier status on the ASCQ-Me domain in comparison to the normative United States population. Low to moderate levels of stigma were reported. See Table I for ASCQ-Me, SCD-HRSS, and MoSCS results. There were weak but significant relationships identified between the QoL measures and the stigma scales, according to the lenient p ≤0.10 significance level that was used for this exploratory study. A positive correlation was identified between pain frequency and stigma (SCD-HRSS: r=0.303, p: 0.043; MoSCS: r=0.379 p: 0.008), indicating that individuals who report experiencing more frequent pain may also report higher stigma. Negative correlations were identified between stigma and both emotional impact (SCD-HRSS: r=-0.335, p=0.025; MoSCS: -0.331, p=0.021) and social functioning (MoSCS: r=-0.397; p=0.005); suggesting that individuals who report less emotional impact, as a result of having SCD, and better social functioning also experience less perceived stigma. Conclusion It is important to note that while participants in this Jamaican sample of adults with SCD reported average to healthier status on the QoL measures than the normative population of individuals with SCD, this does not signify better QoL than the general population. A previous study found that in comparison to the general population, individuals with SCD reported QoL levels most similar to those undergoing hemodialysis. Our sample reported low levels of stigma, which is also consistent with also having lower disease severity. Correlational analysis revealed relationships that indicate that greater disease severity (pain frequency) could result in higher stigma levels, while experiencing less psychosocial (social functioning and emotional) impacts could result in lower stigma levels. Disclosures Bulgin: NIH NINR: Research Funding; Jonas Nurse Leaders Scholar Program: Other: Education Funding ; Duke Global Health Institute Field Work Grant: Research Funding. Tanabe:Alliant Health: Consultancy; NIH and AHRQ: Research Funding; Duke University: Employment.

Description: Introduction Pregnancy in sickle cell disease (SCD) is associated with an exacerbation of SCD-related complications and an increased risk of maternal complications. The increased risk is partly due to physiologic adaptations in pregnancy, which include increased metabolic demands and a hypercoagulable state. The maternal death rate for SCD is 629 per 100,000 deliveries, compared to 12 per 100,000 deliveries in black women and 6 per 100,000 deliveries in the general population (Raider et al., 2016). Studies on maternal and perinatal outcomes of patients with SCD present inconsistent and conflicting results. Some studies have reported an increase in maternal complications such as pre-eclampsia, acute chest syndrome and thromboembolic events, while other studies have reported no significant risk in adverse maternal outcomes. The inconsistent findings reported in prior studies may be attributed to small sample sizes and single-centered sites. Our study aims to determine the prevalence and predictors of maternal morbidity among participants enrolled in the SCD Implementation Consortium (SCDIC) registry, which is the largest, most geographically diverse SCD participant sample in the United States. Methods This cross-sectional study included women enrolled in the SCDIC registry who had at least one pregnancy event. The SCDIC is composed of eight academic SCD centers across the United States and one data-coordinating center. Participants were enrolled in the SCDIC registry if they were 18 to 45 years of age and had a confirmed diagnosis of SCD. Enrolled participants completed a series of surveys that collected sociodemographic information, SCD and pregnancy history and data abstractions of participants' medical records was completed. Medical complications queried during pregnancy included: vaso-occlusive episodes, acute chest syndrome, blood transfusion requirement, preeclampsia, maternal diabetes and deep venous thrombosis. Descriptive analysis of sociodemographic, clinical and maternal characteristics was conducted. Bivariate analysis was performed using Chi-Square test, Mann-Whitney U test, t-test, and logistic regressions, as appropriate. A p-value of ≤ 0.05 was considered statistically significant for all analysis. Results The study sample included 743 women who had at least one pregnancy event, and a total of 1066 live births. Almost all women (96.3%) were African American, with a median age of 21 years (inter-quartile range of 19 to 23 years) at first birth. The majority had Hb SS SCD genotype (69.5%; 513 of the 738 with SCD genotype data). Of all reported pregnancies, participants did not use hydroxyurea during conception (78%), and pregnancy (84.5%). Only 2.7 % of the women reported using fertility drugs or assisted reproductive procedures. Seventy five percent of the pregnancies that ended in live births had maternal complications. The leading complications were vaso-occlusive episodes (61.2%), pregnancy requiring blood transfusion(s) (33.2%), preeclampsia (15.4%), deep venous thrombosis (5.6%) and acute chest syndrome (7.7%). When the pregnancies were stratified by SCD genotype, women with Hb SS had a higher occurrence of acute chest syndrome (63.4% vs. 26.7%), transfusion requirement (70.8% vs. 21%) and preeclampsia (66.7% vs 22.4%). In the univariate logistic regressions, multiparous women, with a history of adverse maternal outcomes in a previous pregnancy, had higher odds of vaso-occlusive episodes (OR: 3.42; 95% CI: 2.42-4.94) acute chest syndrome (OR:4.99; 95% CI:2.56- 9.48), transfusion requirement (OR:3.86; 95% CI:2.64- 5.69), and pre-eclampsia (OR:3.36; 95% CI:2.05-5.45). Conclusion In this large multicenter registry, we found pregnant women with SCD have significant maternal complications. Early antenatal care by healthcare providers knowledgeable about risk factors for adverse maternal outcomes in SCD is essential improve maternal and fetal outcomes and reduce the maternal death rate for SCD. Disclosures Hankins: Novartis: Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; MJH Life Sciences: Consultancy, Patents & Royalties; UptoDate: Consultancy; National Heart, Lung, and Blood Institute: Honoraria, Research Funding; LINKS Incorporate Foundation: Research Funding; American Society of Pediatric Hematology/Oncology: Honoraria. Treadwell:Global Blood Therapeutics: Consultancy; UpToDate: Honoraria. King:Amphivena Therapeutics: Research Funding; Bioline: Consultancy; Celgene: Consultancy; Cell Works: Consultancy; Incyte: Consultancy; Magenta Therapeutics: Membership on an entity's Board of Directors or advisory committees; Novimmune: Research Funding; RiverVest: Consultancy; Tioma Therapuetics: Consultancy; WUGEN: Current equity holder in private company. Gordeuk:CSL Behring: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; Imara: Research Funding; Ironwood: Research Funding; Novartis: Consultancy. Kanter:SCDAA Medical and Research Advisory Board: Membership on an entity's Board of Directors or advisory committees; AGIOS: Membership on an entity's Board of Directors or advisory committees; BEAM: Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy; GLG: Honoraria; Jeffries: Honoraria; Cowen: Honoraria; Wells Fargo: Honoraria; NHLBI Sickle Cell Advisory Board: Membership on an entity's Board of Directors or advisory committees; Medscape: Honoraria; Guidepoint Global: Honoraria; bluebird bio, inc: Consultancy, Honoraria; Sanofi: Consultancy. Glassberg:Pfizer: Research Funding; Global Blood Therapeutics: Consultancy; Eli Lilly and Company: Research Funding. Shah:Novartis: Consultancy, Research Funding, Speakers Bureau; Alexion: Speakers Bureau; CSL Behring: Consultancy; Bluebird Bio: Consultancy; Global Blood Therapeutics: Consultancy, Research Funding, Speakers Bureau.

Description: Introduction Sickle cell disease (SCD) is the most common inherited blood disorders in the United States. The disease predominantly affects African Americans with 1 out of every 365 individuals born with SCD. The disease is characterized by vascular inflammation and vaso-occlusion leading to numerous complications and multi-organ dysfunction. Previous studies have shown women with SCD tend to outlive their male counterparts. Other than the increased life expectancy, sex-based clinical outcome differences in SCD remain largely unknown. To better characterize sex-based differences in SCD, we assessed sociodemographic characteristics, 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 academic and 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. Participants were eligible for the enrollment in the SCDIC registry if they were 15 to 45 years of age and had a confirmed diagnosis of SCD. Participants were excluded if they had sickle cell trait or had a successful bone marrow transplant. Enrolled participants completed surveys. Data were also abstracted from the participants' medical records. Data were entered into a REDCap database and analyzed using SAS version 9.4 (SAS Institute; Cary, NC). Categorical variables were presented as frequencies and percentages, continuous variables were presented as medians and interquartile ranges (IQR) or means and standard deviations. Categorical variables were analyzed using Chi-Square or Fisher exact tests when appropriate. Continuous variables were compared using the Mann-Whitney U test or independent sample t-tests depending on the distribution. A two-sided p-value less than 0.05 was deemed significant. Results A total of 2,124 participants were included in the study. The mean (SD) age of our participants was 27.8 (7.9) years. Almost all (95.6%) were Africa American, female (56%) and had hemoglobin SS (68.2%) SCD genotype. More males (55.4 % vs. 44.6%, p

Description: 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