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

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.

Disease burden and quality of life in children with sickle cell disease in Italy: time to be considered a priority

“…Nevertheless, a single center study investigating parental stress in relationship to chronic hematological disorders, included also parents of children with SCD and reported higher stress among parents of children with SCD compared to those of children with Immune Trombocytopenic Purpura [23]. Italian patients and caregivers participated also in the International Sickle Cell World Assessment Survey (SWAY) in which emotional and wellbeing were accessed globally through a dedicated survey: significant impact of SCD on clinical, emotional, psychological and social aspects of life were reported [24].…”

“…Moreover, Italian teenagers and adults with SCD as well as guardians were among the global population of patients completing the International Sickle Cell World Assessment Survey (SWAY) [24] in which the improvement of QoL was ranked as the first top treatment goal in the 6-11, 12-16 and 17-18 year old age groups. Adolescents with SCD living in Italy have also demonstrated a high completion rate of the daily electronic patient-reported outcome (PRO) diary in the international DOVE trial [29] further underscoring their interest in being involved to report PROs.…”

Patient‐reported neurocognitive symptoms influence instrumental activities of daily living in sickle cell disease

“…13 In a robust international sample, more than a third of individuals with SCD reported that the disease impacted their participation in daily household chores and social activities. 14 Medication adherence, another aspect of IADL skills, is often poor among youth and adults with SCD. 15 Workforce and educational engagement represent another marker of IADL skills.…”

Fluid overload due to intravenous fluid therapy for vaso‐occlusive crisis in sickle cell disease: incidence and risk factors

“…Acute painful vaso‐occlusive crisis (VOC) is one of the clinical hallmarks of sickle cell disease (SCD) 1 . VOCs account for the vast majority of SCD‐related hospital admissions and are associated with significant morbidity and mortality, and negatively impact quality of life 2–5 . Despite the improvements made in our understanding of the pathophysiology of VOC, its treatment still primarily consists of supportive care 6 .…”

“…1 VOCs account for the vast majority of SCD-related hospital admissions and are associated with significant morbidity and mortality, and negatively impact quality of life. [2][3][4][5] Despite the improvements made in our understanding of the pathophysiology of VOC, its treatment still primarily consists of supportive care. 6 Besides treatment of a potential underlying cause, extra [intravenous (IV)] fluids, (opioid) analgesics, and occasionally oxygen support are part of the treatment of VOC.…”