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This article discusses a study conducted to assess the quality of life (QoL) of siblings of adolescents with severe haemophilia. The study found that the QoL of these siblings was not significantly different from that of the affected adolescents, and it was actually higher than that of the age- and sex-matched French general population. The study also identified several determinants of QoL, including age, gender, anxiety symptoms, coping strategies, prosocial behavior, and emotional intelligence. The findings suggest that siblings of individuals with haemophilia generally have good QoL, but there is a need to address potential psychological distress and behavioral problems. The study was conducted with the participation of various medical professionals, researchers, and representatives from the French Association for People with Hemophilia. The data used in the study was provided by the FranceCoag registry. The study was funded by grants from Bayer Consumer Care AG and the University Hospital of Marseille. The authors declare no conflicts of interest. The data from the study is not publicly available due to privacy and ethical restrictions. The study was approved by the Ethical Research Committees and conducted in accordance with applicable privacy requirements and ethical principles. [Extracted from the article]

Introduction: Parents of persons with profound intellectual and multiple disabilities (PIMD) play a major and often lifelong role in the care and support of their child. A better understanding of parents' perspectives regarding their experiences of parenting their child with PIMD is essential to support them more effectively. Although this topic has been explored extensively in Anglo‐Saxon and Northern European countries, little is known about the experience of these parents in a highly institutionalized context such as that in France. Objective: We explored parents' experiences of the activities they performed to care for their child with PIMD (namely, the 'parenting work') in the French context. Method: Qualitative semistructured interviews were conducted by telephone with 34 parents of persons with PIMD aged 8–35. The resulting data were analyzed using thematic analysis. Results: The analysis highlighted the diversity of activities performed by parents as well as the influence of context on the forms of this parenting work. Five themes were developed: (1) navigating the challenges of obtaining medical recognition; (2) negotiating a concealed domain and becoming an expert; (3) unfolding medical and medicosocial care management; (4) navigating the challenges of daily living and (5) shaping one's child's possibilities. Conclusion: This study offers a better understanding of the challenges, levers and expectations of parents of children with PIMD in France. Contextual factors such as the lack of knowledge of PIMD among health professionals, access to knowledge and know‐how associated with care management, the administrative complexity of access to care and equipment, institutional issues (e.g., professional turnover) and societal ableism (e.g., access to infrastructures, interpersonal discrimination) shape the work parents perform to support their child's needs. It is necessary to consider contextual aspects to better support these parents and their children. Suggestions for applications are provided in the discussion. Patient or Public Contribution: One of the researchers, a parent of a child with PIMD, supported the research design and provided feedback on the study's procedures and manuscript. [ABSTRACT FROM AUTHOR]

Introduction: It is necessary to gain insights into adherence to healthcare in people with severe haemophilia (PwSH), especially during the transition from paediatric to adult care, which is an important phase in lives of young people with childhood chronic disease. This adherence can be considered as a marker of successful transition. Objectives: The main objective of the quantitative phase of the TRANSHEMO project was to compare the adherence to healthcare between adolescents and young adults (YAs) with severe haemophilia. The secondary objective was to identify the determinants (facilitators and barriers) of this adherence and associations between these determinants. Methods: A multicentre, observational, cross‐sectional study was conducted in 2017–2019 on PwSH aged between 14 and 17 years (adolescents) or between 20 and 29 years (YAs), included in the FranceCoag registry and having completed the questionnaires. The adherence to healthcare (treatment regimens and clinical follow‐up) was compared between adolescents and YAs using the chi‐squared test. The determinants of this adherence were analysed by structural equation modelling. Results: There were 277 participants, 107 adolescents, and 170 YAs. The rate of adolescents adhering to healthcare was 82.2%, while the rate of YAs was 61.2% (p <.001). The barriers to the adherence to healthcare were being YA, having repeated at least one school grade and presenting mental health concerns. Conclusion: Adolescents had better adherence to healthcare than YAs. According to the determinants enlightened in this project, targeted supportive strategies and adapted therapeutic education programs can be developed for young PwSH to facilitate their adherence to healthcare. [ABSTRACT FROM AUTHOR]

Introduction: Health of people with severe haemophilia (PwSH) improves thanks to the advancements in haemophilia care, giving them more opportunities in occupational integration. However, there is little literature on the occupational integration of PwSH. Objectives: The main objective of our study was to assess the occupational integration of PwSH and to compare it with that of the general population. The secondary objective was to study the association between individual characteristics (sociodemographic, clinical and psycho‐behavioural) and occupational integration of PwSH. Methods: A multicentre, non‐interventional, cross‐sectional study was conducted in 2018–2020 on PwSH, aged over 18 and under 65 years and included in the FranceCoag registry. Measurements included indicators of occupational integration, sociodemographic, clinical and psycho‐behavioural characteristics. The indicators of occupational integration were compared with those of the general population, using indirect standardization. The data of the general population were available from the National Institute of Statistics and Economic Studies (INSEE). Determinants of occupational integration were explored using structural equation modelling. Results: Of 1262 eligible people, 588 were included. PwSH had a lower employment rate than the general population (standardized ratio,.85; 95% CI,.77–.94). There were more PwSH at tertiary education level than expected (standardized ratio, 1.38; 95% CI, 1.17–1.61). HIV infection, poor physical health and mental health concerns were associated with a higher risk of unemployment in PwSH. Conclusion: Employment rate of PwSH is lower than that of the general population despite their higher education level. Target interventions focusing on determinants of difficult occupational integration could be helpful for PwSH. [ABSTRACT FROM AUTHOR]

Background: The healthcare pathway of persons with complex developmental disabilities differs by country, depending both on the specificities of the associated healthcare system and the related societal views. In 2017, the French National Organization of Solidarity for Autonomy entrusted to a group of French experts with a specific mission to analyze three European experiences. Specific Aim The aim of this article is to report the similarities and differences in the healthcare pathways of persons with complex developmental disabilities between France and two other European countries, Italy and Norway. The three countries present different health policies and different ideologies for the care management of persons with complex developmental disabilities. Method: A group of six French experts (including a neuro‐pediatrician, an occupational therapist, a specialist of physical medicine and rehabilitation, a responsible member of a residential facility, a family caregiver representative, and the administrative coordinator of the French Polyhandicap Federation) traveled throughout Italy and Norway (3 and 4 days, respectively). Standardized forms were used to collect information, including dedicated structures, care delivery, dedicated physicians and other healthcareworkers, human and financial aid, place of the caregivers, and preventive and therapeutic actions. Findings Law and health policies, funding, care coordination, and education differ between the three countries (France, Italy, and Norway) and in their effect on the lives of patients and their families. Discussion The three different society models for modalities of care for persons with complex developmental disabilities may have consequences on the lives of patients and their families. This work may help to optimize the level of care according to both patient and family characteristics. [ABSTRACT FROM AUTHOR]

Background and purpose: Patients with a chronic illness, such as multiple sclerosis (MS), and their natural caregivers have a specific experience of healthcare and health services. These experiences need to be assessed to evaluate the quality of care. Our objective was to develop a French‐language questionnaire to evaluate the quality of care as experienced by MS patients and their natural caregivers. Methods: Eligible patients had been diagnosed with MS according to the McDonald criteria. Eligible caregivers were individuals designated by the patients. The MusiCare questionnaire was developed in two standard phases: (i) item generation, based on interviews with patients and caregivers; and (ii) validation, consisting of validity, reliability, external validity, reproducibility, and responsiveness measures. Results: In total, 1088 patients (n = 660) and caregivers (n = 488) were recruited. The initial 64‐item version of MusiCare was administered to a random subsample (n = 748). The validation process generated a 35‐item questionnaire. Internal consistency and scalability were satisfactory. Testing of the external validity revealed expected associations between MusiCare scores and sociodemographic and clinical data. The questionnaire showed good reproducibility and responsiveness. Conclusions: The availability of a reliable and validated French‐language self‐report questionnaire probing the experience of the quality of care for MS will allow the feedback of patients and caregivers to be incorporated into a continuous healthcare quality‐improvement strategy. [ABSTRACT FROM AUTHOR]

Context: [18F]FDG PET/CT improves adrenal tumour characterization. However, there is still no consensus regarding the optimal imaging biomarkers of malignancy. Objectives: To assess the performance of Tumour standardized uptake value (SUV)max:Liver SUVmax for malignancy‐risk and to build and evaluate a prediction model. Design/Methods: The cohort consisted of consecutive patients with adrenal masses evaluated by [18F]FDG PET/CT. The gold standard for malignancy was based on histology or a multidisciplinary consensus in nonoperated cases. The performance of the previously reported cut‐off for Tumour SUVmax:Liver SUVmax (>1.5) was evaluated in this independent cohort. Additionally, a predictive model of malignancy was built from the training cohort (previous study) and evaluated in the validation cohort (current study). Results: Sixty‐four patients were evaluated; 28% of them had a Cushing's syndrome. Fifty‐four adrenal masses were classified as benign and 10 as malignant (including 7 adrenocortical carcinomas). Compared to benign masses, malignant lesions were larger in size, had higher unenhanced densities and higher [18F]FDG uptake. CT‐derived anthropometric parameters did not differ between benign and malignant masses. A tumour SUVmax:Liver SUVmax > 1.5 showed a good diagnostic performance: Se = 90.0%/Sp = 92.6%/PPV = 69.2%/NPV = 98.0% and accuracy = 92.2%. A predictive model based on tumour size and tumour‐to‐liver uptake SUVmax ratio for malignancy‐risk was validated and provides a complementary approach to the ratio. Conclusions: Tumour SUVmax:Liver SUVmax uptake ratio is a useful biomarker for diagnosis of adrenal masses. Another tactic would be to calculate with the model an individual risk of malignancy and integrate this information into a shared decision‐making process. [ABSTRACT FROM AUTHOR]

Background: The use of artificial nutrition, defined as a medical treatment that allows a non‐oral mechanical feeding, for cancer patients with limited life expectancy is deemed nonbeneficial. High‐quality evidence about the use of artificial nutrition near the end of life is lacking. This study aimed (a) to quantify the use of artificial nutrition near the end‐of‐life, and (b) to identify the factors associated with the use of artificial nutrition. Methods: This was a retrospective cohort study of decedents based on data from the French national hospital database. The study population included adult cancer patients who died in hospitals in France between 2013 and 2016 and defined to be in a palliative condition. Use of artificial nutrition during the last 7 days before death was the primary endpoint. Results: A total of 398 822 patients were included. The median duration of the last hospital stay was 10 (interquartile range, 4‐21) days. The artificial nutrition was used for 11 723 (2.9%) during the last 7 days before death. Being a man, younger, having digestive cancers, metastasis, comorbidities, malnutrition, absence of dementia, and palliative care use were the main factors associated to the use of artificial nutrition. Conclusion: This study indicates that the use of artificial nutrition near the end of life is in keeping with current clinical guidelines. The identification of factors associated with the use of artificial nutrition, such as cancer localization, presence of comorbidities or specific symptoms, may help to better manage its use. [ABSTRACT FROM AUTHOR]

Background: Efforts to improve the quality of end-of-life (EOL) care depend on better knowledge of the care that children, adolescents, and young adults with cancer receive, including high-intensity EOL (HI-EOL) care. The objective was to assess the rates of HI-EOL care in this population and to determine patient- and hospital-related predictors of HI-EOL from the French national hospital database.Methods: This was a population-based, retrospective study of a cohort of patients aged 0 to 25 years at the time of death who died at hospital as a result of cancer in France between 2014 and 2016. The primary outcome was HI-EOL care, defined as the occurrence of ≥1 chemotherapy session <14 days from death, receiving care in an intensive care unit ≥1 time, >1 emergency room admission, and >1 hospitalization in an acute care unit in the last 30 days of life.Results: The study included 1899 individuals from 345 hospitals; 61.4% experienced HI-EOL care. HI-EOL was increased with social disadvantage (adjusted odds ratio [AOR], 1.30; 95% confidence interval [CI], 1.03-1.65; P = .028), hematological malignancies (AOR, 2.09; 95% CI, 1.57-2.77; P < .001), complex chronic conditions (AOR, 1.60; 95% CI, 1.23-2.09; P = .001) and care delivered in a specialty center (AOR, 1.70; 95% CI, 1.22-2.36; P = .001). HI-EOL was reduced in cases of palliative care (AOR, 0.31; 95% CI, 0.24-0.41; P < .001).Conclusion: A majority of children, adolescents, and young adults experience HI-EOL care. Several features (eg, social disadvantage, cancer diagnosis, complex chronic conditions, and specialty center care) were associated with HI-EOL care. These findings should now be discussed with patients, families, and professionals to define the optimal EOL. [ABSTRACT FROM AUTHOR]

Objective: Researchers are interested in studying whether the quality of life (QoL) of cancer patients and caregivers is influenced by internal psychobehavioral processes (temporality and coping strategies) and the personality traits that they or their relatives experience. We examined these associations in a sample of patient-caregiver dyads by using the actor-partner interdependence model.Methods: This cross-sectional study involved 156 cancer patient-caregiver dyads. The self-reported data included QoL (Short-Form 36), coping strategies (Brief Coping Orientation to Problems Experienced Scale), time perspectives (Zimbardo Time Perspective Inventory), and personality (Big Five Inventory). The actor-partner interdependence model was used to test the dyadic effect individualizing actor (degree to which the individual's characteristics were associated with their QoL) and partner (degree to which the individual's characteristics were associated with the QoL of the other dyad member) effects.Results: Actor effects were found for patients and caregivers: The use of positive thinking and future/present-hedonistic perspectives were associated with higher QoL; the use of avoidance and past-negative perspective were associated with lower QoL. Partner effects were also found highlighting the specific mechanisms of the interconnections in the patient-caregiver dyad. The patient's QoL was higher when the caregiver used social support and experienced openness. The caregiver's QoL was lower when the patient used social support and avoidance strategies and experienced future perspective.Conclusions: The examination of the relationships between individuals' QoL and their internal psychobehavioral processes and personality traits will have several applications in the routine clinical management. Individual-level and dyad-level interventions should be proposed: cognitive-rehabilitation, emotional and cognitive self-regulation for time perspectives, and personality constructs. [ABSTRACT FROM AUTHOR]

Aim: Individuals with severe and complex disabilities, defined by a combination of profound intellectual impairment and serious motor deficit resulting in extreme dependence, often remain in hospital or at residential facilities. The aim of this study was to identify the determinants of quality of life (QoL) of 238 health care workers (HCWs) caring for individuals with severe and complex disabilities.Method: We conducted a cross-sectional study. The recruitment of the HCWs was performed in five French centres specializing in patients with severe and complex disabilities. The selection criteria were age above 18 years, being an institutional referent HCW (a resource person coordinating various issues for or about the patient), and agreeing to participate. Sociodemographic, health, professional variables, and psycho-behavioural (QoL, burn-out, and coping strategies) data were collected.Results: Of the 362 eligible HCWs, 65.7% returned the questionnaires. The scores of the physical and social dimensions of QoL were significantly lower, and the score of the psychological dimension significantly higher, than those of a comparison group. The main factors modulating QoL were age, financial difficulties, nature of coping strategy, and burn-out.Interpretation: This research provides preliminary evidence that caring for patients with severe and complex disabilities affects the QoL of HCWs. These results support the need for optimization of the work environments for HCWs. [ABSTRACT FROM AUTHOR]