Impact of sickle cell disease on patients' daily lives, symptoms reported, and disease management strategies: Results from the international Sickle Cell World Assessment Survey (SWAY)

• Type of Material: Article
• Authors: Ifeyinwa Osunkwo, Atrium Health; Biree Andemariam, University of Connecticut Health Center; Caterina P. Minniti, Albert Einstein College of Medicine; Baba P. D. Inusa, Guys and St. Thomas NHS Foundation Trust; Fuad El Rassi, Emory University; Beverley Francis-Gibson, Sickle Cell Disease Association of America; Alecia Nero, University of Texas Southwestern; Cassandra Trimnell, Sickle Cell 101; Miguel R. Abboud, American University of Beirut; Jean-Benoît Arlet, University of Paris; Raffaella Colombatti, Azienda Ospedale‐Università di Padova; Mariane de Montalembert, Hôpital Universitaire Necker‐Enfants Malades; Suman Jain, Thalassemia and Sickle Cell Society; Wasil Jastaniah, Umm Al‐Qura University; Erfan Nur, Academic Medical Center; Marimillia Pita, Laureate University‐UAM; Laurie DeBonnett, Novartis Pharmaceuticals Corporation; Nicholas Ramscar, Novartis Pharma AG; Tom Bailey, Adelphi Real World; Olivera Rajkovic-Hooley, Adelphi Real World; John James, Sickle Cell Society
• Language: English
• Date: 2021-02-25
• Publisher: WILEY
• Publication Version: Final Published Version
• Copyright Statement: © 2020 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.
• License: Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1002/ajh.26063
• Title of Journal or Parent Work: AMERICAN JOURNAL OF HEMATOLOGY
• Volume: 96
• Issue: 4
• Start Page: 404
• End Page: 417
• Grant/Funding Information: This survey was sponsored by Novartis Pharmaceuticals Corporation. Medical writing assistance was provided by Liz Pearce MBChB, from Mudskipper Business Ltd, funded by Novartis Pharmaceuticals Corporation.; Novartis provided sponsorship and was involved in running the survey. Adelphi Real World (ARW) designed the survey in collaboration with the SWAY steering committee and Novartis. Funding was provided by Novartis Pharmaceuticals Corporation to ARW for the survey design, data collection, and data analysis, and Mudskipper Business Ltd, Bollington, Macclesfield, UK for medical writing support.
• Keywords: Hematology; Life Sciences & Biomedicine; Science & Technology
• Research Categories: Health Sciences, Epidemiology; Biology, Genetics; Health Sciences, Medicine and Surgery

Abstract

Sickle cell disease (SCD) is a genetic disorder, characterized by hemolytic anemia and vaso-occlusive crises (VOCs). Data on the global SCD impact on quality of life (QoL) from the patient viewpoint are limited. The international Sickle Cell World Assessment Survey (SWAY) aimed to provide insights into patient-reported impact of SCD on QoL. This cross-sectional survey of SCD patients enrolled by healthcare professionals and advocacy groups assessed disease impact on daily life, education and work, symptoms, treatment goals, and disease management. Opinions were captured using a Likert scale of 1-7 for some questions; 5-7 indicated “high severity/impact.” Two thousand one hundred and forty five patients (mean age 24.7 years [standard deviation (SD) = 13.1], 39% ≤18 years, 52% female) were surveyed from 16 countries (six geographical regions). A substantial proportion of patients reported that SCD caused a high negative impact on emotions (60%) and school achievement (51%) and a reduction in work hours (53%). A mean of 5.3 VOCs (SD = 6.8) was reported over the 12 months prior to survey (median 3.0 [interquartile range 2.0-6.0]); 24% were managed at home and 76% required healthcare services. Other than VOCs, fatigue was the most commonly reported symptom in the month before survey (65%), graded “high severity” by 67% of patients. Depression and anxiety were reported by 39% and 38% of patients, respectively. The most common patient treatment goal was improving QoL (55%). Findings from SWAY reaffirm that SCD confers a significant burden on patients, epitomized by the high impact on patientsʼ QoL and emotional wellbeing, and the high prevalence of self-reported VOCs and other symptoms.