Sickle cell disease (SCD) has a significant effect on patient quality of life, with a global commonality in unmet treatment needs, disease burden, and effects on daily life, according to results from an international survey published in American Journal of Hematology.

SCD is an inherited disorder that affects millions of people around the world. Patients with SCD experience anemia, organ damage and painful vaso-occlusive crises (VOCs). Previous surveys have found that SCD contributes to poor health-related quality of life, but most of those surveys were generic and limited to 1 country.

The Sickle Cell World Assessment Survey (SWAY) surveyed patients on the impact of the disease on their daily lives. A total of 2145 patients from 16 countries completed the survey from April 3 to October 4, 2019. The survey included patients from high-income (HI) and low-middle-income (LMI) countries, and used a 7-point Likert scale, with scores of 5, 6, or 7 indicating high impact or high severity.

Of the participants surveyed, the majority indicated that SCD affected their emotional wellbeing (60%). Many patients also reported feeling frustrated with putting ip with symptoms (58%), and worries about worsening disease (58%). In addition, the majority of patients reported avoidance of intense physical activity (62%).

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A total of 53% of patients felt that SCD limited them to certain careers, 44% reported that SCD has prevented them from attending work, and 46% reported that SCD reduced attendance at school.

Fatigue, bone aches, and headaches were the 3 most commonly reported symptoms (41%, 38%, and 25%, respectively). Patients who experienced these symptoms were more likely to respond that the disease had a high impact on their lives or wellbeing. Fatigue was the most common symptom in patients before the survey (65%). Almost all patients experienced at least 1 VOC in the 12 months before the survey. Usually, VOCs were managed by an overnight hospital stay.

Survey results confirm previous studies finding that patients with SCD experience poorer quality of life across the globe and that VOCs are highly debilitating. The results also suggested the need for improvement in VOC management. Nearly 25% of patients reported managing their VOCs at home, and 94% of patients were receiving ongoing treatment for SCD, with folic acid being the most common. The majority of patients saw an SCD specialist and felt confident in the care they received by their healthcare provider.

The burden of SCD seemed to be greater for patients in HI countries, which may be due to older patient age in these countries. Disease burden worsens with age, and patients in HI countries have a longer life expectancy than those in LMI nations. SWAY results can help providers understand how SCD affects patients to tailor management strategies to their unique needs.

“Further analyses of SWAY will also fully explore how patient demographic factors (eg, age, gender), VOC and symptom/complication frequency, and treatment use (eg, opioids) influence self-reported impact of SCD on patients’ daily lives.”

Disclosure: 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, Inusa BPD, 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. Published online December 2, 2020. doi:10.1002/ajh.26063