-Author name in bold denotes the presenting author
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1732 Sex Based Differences in Sickle Cell Disease

Program: Oral and Poster Abstracts
Session: 114. Hemoglobinopathies, Excluding Thalassemia—Clinical: Poster II
Hematology Disease Topics & Pathways:
Adult, sickle cell disease, Diseases, Hemoglobinopathies, Young Adult, Study Population, Clinically relevant
Sunday, December 6, 2020, 7:00 AM-3:30 PM

Rita V Masese, MSc, MD1*, Dominique Bulgin, BSN, RN2*, Mitchell Knisely, PhD, RN1*, Liliana Preiss3*, Eleanor Stevenson, PhD, RN1*, Jane S. Hankins, MD, MS4, Marsha Treadwell, PhD5,6*, Allison A. King, MD, MPH, PhD7,8,9,10,11, Victor R. Gordeuk, MD12,13,14, Julie Kanter, MD15,16,17,18,19,20,21,22, Robert Gibson, MD23*, Jeffrey A. Glassberg, MD24,25, Paula Tanabe, PhD, RN26 and Nirmish Shah, MD27,28,29,30,31

1School of Nursing, Duke University, Durham, NC
2Duke University School of Nursing, Durham, NC
3RTI International, Durham, NC
4Department of Hematology, St. Jude Children's Research Hospital, Memphis, TN
5Children's Hospital and Research Center, Oakland, Oakland, CA
6UCSF Benioff Children's Hospital Oakland, Oakland, CA
7Washington University, St. Louis, Clayton, MO
8Program in Occupational Therapy, Washington University in St. Louis, School of Medicine, Clayton, MO
9Washington University in St. Louis, Clayton, MO
10Washington University, Saint Louis, MO
11Washington University School of Medicine, St. Louis, MO
12Sickle Cell Center, Division of Hematology and Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, IL
13University of Illinois at Chicago, Chicago, IL
14Department of Medicine, University of Illinois at Chicago, Chicago, IL
15Director, Adult Sickle Cell Program, Comprehensive Sickle Cell Center,, Birmingham, AL
16Division of Hematology–Oncology, University of Alabama, Birmingham, AL
17Hematology/Oncology, Comprehensive Sickle Cell Center, University of Alabama at Birmingham, Birmingham, AL
18University of Alabama, Director of the UAB Adult Sickle Cell Clinic Associate Professor in the Division of Hematology and Oncology, Birmingham, AL
19University of Alabama at Birmingham, Birmingham, AL
20Division of Pediatrics, Medical University of South Carolina, Charleston, SC
21Division of Hematology-Oncology, University of Alabama-Birmingham, Birmingham, AL
22Medical University Of South Carolina, Charleston, SC
23Medical College of Georgia, Augusta University, Augusta, GA
24Division of Hematology and Oncology, Department of Medicine and Department of Emergency Medicine, Mount Sinai Medical Center, New York, NY
25Division of Hematology Oncology, Icahn School of Medicine at Mount Sinai, New York, NY
26School of Nursing/ School of Medicine, Duke University, Durham, NC
27Division of Hematology, Duke University School of Medicine, Durham, NC
28Duke University Medical Center, Durham, NC
29Duke University Health System, Durham, NC
30Duke University, Cary, NC
31Duke Adult Comprehensive Sickle Cell Center, Durham, NC


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.


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.


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 <0.0001) were taking hydroxyurea. Females had significantly worse reports of pain frequency and severity (p=0.0002 and <0.0001 respectively), 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). Males had significantly more skin ulcers and respiratory, musculoskeletal, genitourinary and cardiovascular complications while females had more anxiety, depression and autoimmune conditions. Males also had significantly higher creatinine, blood urea nitrogen, albumin and liver enzymes (alkaline phosphatase, aspartate and alanine aminotransferases). Females had higher fetal hemoglobin levels with and without hydroxyurea use. There were no statistical differences in ethnicity, marital and employment status.


Key differences in SCD presentation and occurrence of complications exist among males and females. Females had higher rates of depression and anxiety while males had more chronic end-organ complications that are life threatening. Our findings emphasize the need for stratification of data analysis by sex in future SCD studies.

Disclosures: Hankins: Global Blood Therapeutics: Consultancy, Research Funding; National Heart, Lung, and Blood Institute: Honoraria, Research Funding; LINKS Incorporate Foundation: Research Funding; American Society of Pediatric Hematology/Oncology: Honoraria; MJH Life Sciences: Consultancy, Patents & Royalties; UptoDate: Consultancy; Novartis: Research Funding. Treadwell: UpToDate: Honoraria; Global Blood Therapeutics: Consultancy. King: Tioma Therapuetics: Consultancy; WUGEN: Current equity holder in private company; RiverVest: Consultancy; Celgene: Consultancy; Cell Works: Consultancy; Incyte: Consultancy; Magenta Therapeutics: Membership on an entity's Board of Directors or advisory committees; Bioline: Consultancy; Novimmune: Research Funding; Amphivena Therapeutics: Research Funding. Gordeuk: CSL Behring: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; Imara: Research Funding; Ironwood: Research Funding; Novartis: Consultancy. Kanter: BEAM: Membership on an entity's Board of Directors or advisory committees; Medscape: Honoraria; Guidepoint Global: Honoraria; Sanofi: Consultancy; Novartis: Consultancy; bluebird bio, inc: Consultancy, Honoraria; AGIOS: Membership on an entity's Board of Directors or advisory committees; SCDAA Medical and Research Advisory Board: Membership on an entity's Board of Directors or advisory committees; NHLBI Sickle Cell Advisory Board: Membership on an entity's Board of Directors or advisory committees; Wells Fargo: Honoraria; Cowen: Honoraria; Jeffries: Honoraria; GLG: Honoraria. Glassberg: Eli Lilly and Company: Research Funding; Pfizer: Research Funding; Global Blood Therapeutics: Consultancy. Shah: Bluebird Bio: Consultancy; Novartis: Consultancy, Research Funding, Speakers Bureau; Global Blood Therapeutics: Consultancy, Research Funding, Speakers Bureau; CSL Behring: Consultancy; Alexion: Speakers Bureau.

*signifies non-member of ASH