Predictors of health-related quality of life in a large cohort of adult patients living with sickle cell disease in France: the DREPAtient study.

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 (ß = -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.

Early Strokes Are Associated with More Global Cognitive Deficits in Adults with Sickle Cell Disease.

This study sought to link neurocognitive profiles in sickle cell disease (SCD) patients with clinical characteristics. We conducted a prospective cohort study of adults with SCD who underwent comprehensive neuropsychological assessment at the UMGGR clinic at Henri Mondor Hospital, Créteil (France). A cluster analysis was performed based on neuropsychological testing scores. The association between clusters and clinical profiles was assessed. Between 2017 and 2021, 79 patients with a mean age of 36 [range 19-65] years were included. On principal component analysis, a 5-factor model presented the best fit (Bartlett's sphericity test [χ2 (171) = 1345; p < 0.001]), explaining 72% of the variance. The factors represent distinct cognitive domains and anatomical regions. On hierarchical classification, three clusters emerged. Cluster 1 (n = 24) presented deficits in all five factors compared to Cluster 3 (n = 33). Cluster 2 (n = 22) had deficits in all factors, but to a lesser extent than Cluster 1. MoCA scores mirrored the severity of these cognitive deficits. Age, genotype and stroke prevalence did not differ significantly between clusters. However, the time of first stroke occurrence differed significantly between Cluster 1 and 2-3: 78% of strokes occurred during childhood, whereas 80% and 83% occurred during adulthood in Clusters 2 and 3, respectively. Educational attainment was also reduced in Cluster 1. SCD patients with childhood stroke seem to be at increased risk of a global cognitive deficit profile. In addition to existing methods of primary and secondary stroke prevention, early neurorehabilitation should be prioritized in order to reduce the long-term cognitive morbidity of SCD.

Psychometric validation of a 7C-model of antecedents of vaccine acceptance among healthcare workers, parents and adolescents in France.

Support for vaccine decision-making requires a tailored approach taking into account psychological antecedents of vaccine acceptance. We aimed at validating an extended 7C-model of antecedents in three different target population groups (healthcare workers [n = 3870], parents [n = 2002] and adolescents [n = 7118]) and two vaccinations (COVID-19, HPV) in France. We performed a secondary analysis of questionnaires collecting sociodemographic characteristics, attitudes and knowledge on vaccination, and vaccine status and intention. We used standard psychometric techniques to validate a first and second order latent structure, and evaluated their association with vaccine intentionality in three levels (refusal, indecision, acceptance). In all populations, the 7C-model yielded a very good model fit (CFI and TLI > 0.90) and, in comparison with non-nested and nested 5C-models, significantly improved the model performance (Ω2, p < 0.05; Wald's test, p < 0.05). The resulting vaccine readiness score was strongly associated with vaccine intentionality (acceptance vs. indecision: ßHCW = 2.93, ßParents = 2.41, ßAdolescents = 1.34; refusal vs. indecision: ßHCW = - 1.68, ßParents = - 0.16, ßAdolescents = - 0.89.). The addition of confidence in the system and social conformism among antecedents of vaccine acceptance allowed a finer understanding of the continuum moving from refusal to indecision and acceptance. To work with these antecedents in interventional research, appropriate questionnaire items should be developed for various vaccines and target populations.

How can we improve the acceptability of vaccination against Human Papillomavirus (HPV) in France? An original qualitative study with focus groups comprising parents and school staff, interviewed separately.

BACKGROUND: It has been proven that vaccination is effective against Human Papillomavirus (HPV) infections, genital warts, and pre-cancerous and cancerous cervical lesions. Nevertheless, the HPV vaccine coverage of 37.4 % in 2021 in France is one of the lowest in Europe. To explore and understand the reason why French population is so late compared to its neighbours, we carried out focus groups with mothers and with National Education school staff. We aimed to identify knowledge and perceptions of HPV in both study populations, as well as factors influencing HPV vaccination. METHODS: Between January 2020 and March 2021, we performed a qualitative study using an inductive approach with a thematic content analysis (TCA). We conducted semi-structured focus groups with 29 people including 15 mothers of adolescents in middle schools and 14 school staff from the national education system. RESULTS: Different factors influenced the decision-making process of parents and school staff: knowledge and perceptions of HPV and its vaccine, sources of information about HPV and vaccination. Mothers' discourses differed from those of school staff. They mentioned the importance of gynaecological monitoring and the negative image of pharmaceutical companies, and questioned internet as a reliable source of information. For their part, school staff mentioned cultural and/or religious affiliation, municipalities' role to inform the population, and ethical dilemma or logistical challenges regarding HPV vaccination in schools. CONCLUSION: The results of these focus groups provided information on which elements may harm or help HPV vaccination. Identified perceptions, beliefs, knowledge, barriers, and facilitators will help us to build an intervention program focus on general practitioners (GP), school staff, parents, and adolescents.

Psychometric characteristics of the Revised Illness Perception Questionnaire (IPQ-R) in adults with sickle cell disease.

OBJECTIVE: Sickle cell disease (SCD) is the most frequent monogenic disease worldwide. Psychological and behavioural factors are often reported as playing a significant role in predicting SCD health outcomes. When focusing on adaptation to a specific health condition and its treatment, the Common Sense Model of Health and Illness (CSM) has proven to be of heuristic value. In other health conditions, illness outcomes are directly influenced by illness perception. Therefore, the aim of this study is to explore the psychometric proprieties of the Revised Illness Perception Questionnaire (IPQ-R). DESIGN: We performed a cross-sectional assessment on 517 adult patients with sickle cell disease and collected the results of 406 IPQ-R. With these data, we verified the factor structure of the Belief scale and proposed modifications to improve its fit to the data with a confirmatory factor analysis. In addition, we explored the factorial structure of the Causal attribution scale with an exploratory factor analysis. RESULTS: The initial model showed poor fit with the data. After structural modifications, elimination of two items with a low loading (model 2), covariance added between items (model 3) and items reallocation (model 4), the last model proposed presented a correct fit with the data. Before doing this model specification, we reviewed and compiled the nine studies that explored the psychometric properties of the IPQ-R in order to highlight all the modifications made by the other authors who have adapted the IPQ-R to a specific population and to allow a comparison with our own modifications. CONCLUSION: Considering previous findings, this research suggests further work is needed on the structure of the dimensions of the IPQ-R.

Depression in adults with sickle cell disease: a systematic review of the methodological issues in assessing prevalence of depression.

BACKGROUND: Sickle cell disease (SCD) as other chronic medical conditions is commonly complicated by depression or other psychiatric symptoms. Results reported in studies present a large variation. Thus, synthetic data are needed to understand impact of depression in adults with SCD. The aim of this literature review is to analyse the methodology used in the studies assessing depression and discuss the different prevalence levels reported. METHODS: Studies involving adults with SCD from 1999 to 2018 were included when providing data on prevalence of depression. It was defined by a psychometric assessment, a structured interview, or a medical record review. PRISMA recommendations were followed. RESULTS: 36 studies are included accordingly to our methodology. Prevalence variation is large, from 0% to more than 85%. We find that the type of assessment tool used plays a major role in this between studies variation. Also, methodological issues arise with respect to psychometric assessment. Moreover, differences emerge between continents, setting of recruitment or time of assessment. CONCLUSION: All these issues are discussed to provide insight on depression in adults with sickle cell disease. TRIAL REGISTRATION: PROSPERO Registration CRD42018100684.