• Ifeyinwa Osunkwo, Biree Andemariam, Caterina P Minniti, InusaBaba P DBPD0000-0003-2643-765XEvelina Children's Hospital, Guy's and St Thomas' NHS Foundation Trust, London, UK., Fuad El Rassi, Beverley Francis-Gibson, Alecia Nero, Cassandra Trimnell, Miguel R Abboud, Jean-Benoît Arlet, Raffaella Colombatti, Mariane de Montalembert, Suman Jain, Wasil Jastaniah, Erfan Nur, Marimilia Pita, Laurie DeBonnett, Nicholas Ramscar, Tom Bailey, Olivera Rajkovic-Hooley, and John James.
• Sickle Cell Disease Enterprise, The Levine Cancer Institute, Atrium Health, Charlotte, North Carolina, USA.
• Am. J. Hematol. 2021 Apr 1; 96 (4): 404-417.

AbstractSickle 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 patients' QoL and emotional wellbeing, and the high prevalence of self-reported VOCs and other symptoms.© 2020 The Authors. American Journal of Hematology published by Wiley Periodicals LLC.

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