AUTHOR=Yaya Issifou , Pourageaud Adrien , Derbez Benjamin , Odièvre Marie-Hélène , Oudin Doglioni Damien , Podevin Marieke , Thomas Gaëlle , Yombo-Kokule Lisa , Godart Christian , Lepetit Maryannick , Cassubie-Mercier Tania , Galacteros Frederic , Chassany Olivier , DREPAtient study group , Podevin Marieke , Thomas Gaëlle , Chassany Olivier , Yaya Issifou , Yombo-Kokule Lisa , Galactéros Frédéric , Doglioni Damien Oudin , Marie-Hélène Odièvre-Montanié , Derbez Benjamin , Godart Christian , Lepetit Maryannick , Cassubie-Mercier Tania , Cassubie-Mercier Tania , Pavan Sonia , Aguilar-Martinez Patricia , Arlet Jean-Benoït , Cannas Giovanna , Chamouine Abdourahim , Etienne-Julan Maryse , Guitton Corinne , Le Jeune Sylvain , Loko Gylna , Pondarre Corinne TITLE=Predictors of health-related quality of life in a large cohort of adult patients living with sickle cell disease in France: the DREPAtient study JOURNAL=Frontiers in Public Health VOLUME=12 YEAR=2024 URL=https://www.frontiersin.org/journals/public-health/articles/10.3389/fpubh.2024.1374805 DOI=10.3389/fpubh.2024.1374805 ISSN=2296-2565 ABSTRACT=Background

Sickle cell disease (SCD) is an inherited autosomal recessive disorder exhibiting a range of symptoms and acute and/or chronic complications that affect the quality of life. This study aimed to assess health-related quality of life (HRQoL) and to identify the associated factors in adult patients with SCD in France.

Methods

DREPAtient is a cross-sectional, multicenter study conducted from June 2020 to April 2021 in France and in certain French overseas territories where SCD is highly prevalent. Sociodemographic and clinical data were collected online. HRQoL was assessed by the French version of the 36-Item Short Form Survey (SF-36) questionnaire. HRQoL determinants were identified using multivariable linear regression analysis.

Results

In total, 570 participants were included, mostly women (68.9%), with a mean age of 33.3 (±10.7) years. The highest mean score HRQoL was found in the Physical functioning domain (67.5 ± 21.8) and the lowest mean score in the General Health perception domain (37.7 ± 20.3). The mean score of the physical composite (PCS) and mental composite (MCS) of SF-36 summary scores was 40.6 ± 8.9 and 45.3 ± 9.8, respectively. Participants receiving oxygen therapy (β = −3.20 [95%CI: −5.56; −0.85]), those with a history of femoral osteonecrosis (−3.09 [−4.64; −1.53]), those hospitalized for vaso-occlusive crisis (VOC) or acute chest syndrome (ACS) (−2.58 [−3.93; −1.22]), those with chronic complications (−2.33 [−4.04; −0.62]), female participants (−2.17 [−3.65; −0.69]), those with psychological follow-up (−2.13 [−3.59; −0.67]), older participants (−1.69 [−3.28; −0.09]), and those receiving painkillers (−1.61 [−3.16; −0.06]) reported worse PCS score. By contrast, those who had completed secondary or high school (4.36 [2.41; 6.31]) and those with stable financial situation (2.85 [0.94, 4.76]) reported better PCS scores. Worse MCS scores were reported among participants with psychological follow-up (−2.54 [−4.28; −0.80]) and those hospitalized for VOC/ACS in the last 12 months (−2.38 [−3.99; −0.77]), while those who had relatives’ support (5.27 [1.92; 8.62]) and those with stable financial situation (4.95 [2.65; 7.26]) reported better MCS scores.

Conclusion

Adults with major SCD reported poor physical and mental HRQoL scores. Hospitalization for VOC/ACS, chronic complications, use of painkillers, perceived financial situation, and support from relatives are important predictors of HRQoL in SCD patients. Interventions to improve HRQoL outcomes SCD should be considered.