Adults with Sickle Cell Report Poor Health-Related Quality of Life Scores

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Based on the 36-item Short Form Survey questionnaire, adults with SCD in France report low physical and mental health summary scores.

Adult patients living with major sickle cell disease (SCD) reported poor physical and mental health-related quality of life (HRQoL) scores, according to new findings from the DREPatient study in France.1

The quality of life scores, in both physical and mental domains, were particularly worse in female participants, those of older age, and those who had been hospitalized for vaso-occlusive crises (VOCs) or acute chest syndrome (ACS) within the last 12 months.

“These findings suggest paying greater attention to HRQoL in adults with SCD, in terms of SCD management strategies,” wrote the investigative team, led by Issifou Yaya, PhD, Patient-Reported Outcomes Research, URC-ECO, Hôpital Hôtel-Dieu.

In the past two decades, the global prevalence of SCD has increased by more than 40%, affecting millions worldwide.2 SCD is characterized by the experience of a wide range of clinical symptoms, as well as disease complications, including VOC episodes, invasive infections, ACS, stroke, and chronic pulmonary hypertension.3

Prior evidence has shown a patient’s HRQoL in SCD to be impacted by numerous complex factors, including stigma, discrimination, family life and work issues, and social isolation.4 In this study, Yaya and colleagues sought to evaluate French adults' HRQoL with SCD and the associated factors influencing these outcomes.1

DREPatient is a large-scale survey designed to assess HRQoL in SCD. This cross-sectional, multi-center study was primarily conducted in mainland France and multiple overseas territories where SCD remains prevalent, from June 2020 to April 2021.

A questionnaire was completed online–participants self-reported HRQoL using the French version of the 36-Item Short Form Survey (SFS). The SFS’ eight domains were aggregated into two main scores, ranging from 0 to 100 (best): the Physical Component Summary score (PCS) and the Mental Component Summary score (MCS).

Among 1088 adults and children recruited for DREPatient, 570 adults with major SCD were included for analysis. Participants were mostly women (68.9%) and a mean age of 33.3 years. Nearly half of the participants (48.8%) had already been hospitalized for a VOC or ACS.

Upon analysis, the highest mean score was identified in the Physical Functioning domain (67.5 ± 21.8), while the General Health domain had the lowest mean score (37.7 ± 20.3). Overall, the mean score of the PCS and MSC in the SF-36 summary scores was 40.6 ± 8.9 and 45.3 ± 9.8, respectively.

In multivariable analysis, Yaya and colleagues identified the factors associated with worse PCS scores in individuals with SCD:

  • Oxygen therapy (β, –3.20; 95% CI, –5.56 to –0.85)
  • History of femoral osteonecrosis (β, –3.09; 95% CI, –4.64 to –1.53)
  • Hospitalization for VOCs or ACS (β, –2.58; 95% CI, –3.93 to –1.22)
  • Chronic complications (β, –2.33; 95% CI, –4.04 to –0.62)
  • Female participants (β, –2.17; 95% CI, –3.65 to –0.69)
  • Psychological follow-up (β, –2.13; 95% CI, –3.59 to –0.67)
  • Older age (β, –1.69; 95% CI, –3.28 to –0.09)
  • Painkiller use (β, –1.61; 95% CI, –3.16 to –0.06).

Meanwhile, those who had completed secondary or high school (β, 4.36; 95% CI, 2.41 - 6.31) and those with stable financial situations (β, 2.85; 95% CI, 0.94 - 4.76) reported better PCS scores.

Further multivariable analysis showed individuals with psychological follow-up (β, –2.54; 95% CI, –4.28 to –0.80) and those hospitalized for VOC/ACS in the last 12 months (β, –2.38; 95% CI, –3.99 to –0.77) reported worse MCS scores. On the other hand, those with relative support (β, 5.27; 95% CI, 1.92 - 8.62) and those in stable financial situations (β, 4.95; 95% CI, 2.65 - 7.65) reported better MCS scores.

Based on these data, Yaya and colleagues noted higher education attainment, in combination with socioeconomic status, could be a proxy for health literacy. Efforts to help patients with SCD improve their capacity for self-management of the disease could reduce complications, including VOC episodes.

“Efforts to promote education and health literacy, alleviate poverty, and provide equitable access to healthcare are essential components of public health initiatives aiming to reduce health inequalities,” they wrote.


  1. Yaya I, Pourageaud A, Derbez B, et al. Predictors of health-related quality of life in a large cohort of adult patients living with sickle cell disease in France: the DREPAtient study. Front Public Health. 2024;12:1374805. Published 2024 May 20. doi:10.3389/fpubh.2024.1374805
  2. GBD 2021 Sickle Cell Disease Collaborators. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: a systematic analysis from the Global Burden of Disease Study 2021 [published correction appears in Lancet Haematol. 2023 Aug;10(8):e574. doi: 10.1016/S2352-3026(23)00215-6]. Lancet Haematol. 2023;10(8):e585-e599. doi:10.1016/S2352-3026(23)00118-7
  3. Shah N, Bhor M, Xie L, Paulose J, Yuce H. Sickle cell disease complications: Prevalence and resource utilization. PLoS One. 2019;14(7):e0214355. Published 2019 Jul 5. doi:10.1371/journal.pone.0214355
  4. O'Brien JA, Hickman RL Jr, Burant C, Dolansky M, Padrino S. Health Literacy, Perceived Stigma, Self-Efficacy, and HRQOL in Sickle Cell Disease. West J Nurs Res. 2023;45(4):335-343. doi:10.1177/01939459221135331