Sickle cell disease (SCD) causes a reduction in quality of life, greatly affecting patients’ emotional wellbeing. A cross-sectional survey of the international Sickle Cell World Assessment Survey (SWAY) published in the American Journal of Hematology reaffirmed that quality of life is a primary treatment goal for patients.
SCD leads to chronic hemolytic anemia, organ damage, and vaso-occlusive crises (VOCs). In high-resource countries like the United States, the life expectancy of people with SCD has improved, but those improvements haven’t been seen equally in low-resource countries.
SCD negatively affects patients’ physical and mental health, social life, and work and school performance. Before SWAY, quality of life surveys weren’t specific to SCD and were performed in individual countries. SWAY aimed to provide real-world global insights into the lives of patients with SCD.
A total of 2,145 patients were surveyed from 16 countries. Of these, 60% of patients said they felt SCD affects their emotional wellbeing and 41% said they felt SCD affected their social or family life. SCD also had a negative effect on employment for many people surveyed. Of the respondents, 53% who were currently or previously employed said they had to reduce hours and 32% had lost their jobs because of SCD.
Similarly, SCD affected people’s performance at school by decreasing motivation (30%) and reducing school attendance (46%).
SWAY also surveyed SCD symptoms in the month before and VOCs in the 12 months before survey completion. Patients who reported symptoms were more likely to also report a high impact of SCD on their daily life, school, employment, and emotional wellbeing. Fatigue, bone aches, and headaches were the most commonly reported symptoms, with many respondents rating their symptoms as severe. More than one-third of patients also reported depression and anxiety.
A total of 91% of patients experienced 1 or more VOCs in the 12 months before survey completion, with a mean of 5.3 and a median of 3.0 VOCs per patient. Patients who had more VOCs also reported a high impact of SCD on their quality of life.
Of the respondents, 55% of patients selected that their treatment goal was to improve quality of life, followed by prevent worsening of disease (43%), reduce severe pain crises (30%), improve overall symptoms (29%), and improve long-term survival (28%).
The results of SWAY are consistent with country-specific surveys of patients with SCD. The burden of VOCs shows that there’s a need globally to improve management of these painful events. Almost a quarter of VOCs were managed at home in the 12 months before the survey, primarily because of a prior poor experience in an emergency room.
The global data and specificity for SCD were strengths of the study. However, it was a self-completed survey, with more than half of patients recruited by their health care provider, and therefore may not fully represent the general SCD population.
Disclosures: Some authors have declared affiliations with or received grant support from the pharmaceutical industry. Please refer to the original study for a full list of disclosures.
Osunkwo I, Andemariam B, Minniti CP, et al. 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). Am J Hematol. 2021;96(4):404-417. doi:10.1002/ajh.26063