Impact of spinal fusion on severity health status in scoliotic adolescents with polyhandicap.

BACKGROUND: Scoliosis constitutes a prevalent comorbidity in adolescents with polyhandicap and frequently leads to other severe impairments, impacting abilities and requiring complex caregiving strategies. Therefore, spinal fusion surgeries are commonly performed to alleviate pain and provide more comfort. However, spine stabilization has not previously been proven to improve the severity health status of adolescents with polyhandicap according to specific clinical scales. OBJECTIVE: This study describes and compares the severity health status of adolescents with polyhandicap before and after they underwent spinal fusion. METHODS: A monocentric retrospective observational study was conducted in the university hospital centre of Saint-Etienne, France. We included between 2009 to 2020, 30 scoliotic adolescents with polyhandicap who underwent spinal fusion performed with the same surgical technique and the same surgeon. The main outcome was the variation in the Polyhandicap Severity Scale (PSS) score after surgery. Secondary outcomes were variations in PSS subscores, quality of life scores, fronto-sagittal X-ray parameters, and measures of surgical complication rates and lengths of stay. RESULTS: Among 30 adolescents, 27 PSS analyses were performed. We found a significant improvement between pre- and postoperative PSS scores, mainly for pain and respiratory, digestive, and skin disabilities. These improvements were accompanied by significant reductions in pelvic obliquity, in frontal and sagittal curves. The mean hospital length of stay was 45 days. During postoperative period, patients received a personalized postoperative rehabilitation procedure with spasticity and pain treatments, physiotherapy, and verticalization (wheelchair sitting and positioning devices such as contoured seat intended to increase postural stability). The mortality rate was estimated at 7%. At least 1 complication per patient occurred. CONCLUSIONS: We show that spinal fusion surgeries confer a significant improvement in the severity health status in scoliotic adolescents with polyhandicap.

Health care management adequacy among French persons with severe profound intellectual and multiple disabilities: a longitudinal study.

BACKGROUND: The care organization of persons with profound intellectual and multiple disabilities (PIMD) varies by country according to the health care system. This study used a large sample of French individuals with severe PIMD/polyhandicap to assess: 1) the adequacy of care setting over a 5-year period and 2) health care consumption. METHODS: The longitudinal study used data from the French EVALuation PoLyHandicap (EVAL-PLH) cohort of persons with severe PIMD/polyhandicap who were receiving managed in specialized care centres and residential facilities. Two assessments were performed: wave 1 (T1) in 2015-2016 and wave 2 (T2) in 2020-2021. The inclusion criteria were as follows: age > 3 years at the time of inclusion; age at onset of cerebral lesion younger than 3 years old; and severe PIMD. The adequacy of the care setting was based on the following: i) objective indicators, i.e., adequacy for age and adequacy for health status severity; ii) subjective indicators, i.e., self-perception of the referring physician about medical care adequacy and educational care adequacy. Health care consumption was assessed based on medical and paramedical care. RESULTS: Among the 492 persons assessed at the 2 times, 50% of individuals at T1 and 46% of individuals at T2 were in an inadequate care setting based on age and severity. Regarding global subjective inadequacy, the combination of medical adequacy and educational adequacy, 7% of individuals at T1 and 13% of individuals at T2 were in an inadequate care setting. At T2, a majority of individuals were undermonitored by medical care providers (general practitioners, physical medicine rehabilitation physicians, neurologists, orthopaedists, etc.). Important gaps were found between performed and prescribed sessions of various paramedical care (physiotherapy, occupational therapy, psychomotor therapy, etc.). CONCLUSIONS: This study revealed key elements of inadequate care management for persons with severe PIMD/polyhandicap in France. Based on these important findings, healthcare workers, familial caregivers, patients experts, and health decision-makers should develop appropriate care organizations to optimize the global care management of these individuals. TRIAL REGISTRATION: NCT02400528, registered 27/03/2015.

Development and initial validation of a screening tool for visual ability/performance of people with polyhandicap.

BACKGROUND: Visual impairments are common in people with polyhandicap although they are poorly assessed. However, evaluation of the visual abilities of these people is critical to determining treatment for impairments. OBJECTIVES: To develop and validate an easy-to-use visual-behavioural scale for assessing the visual abilities of people with polyhandicap. METHODS: The development of the Visual Assessment for People with Polyhandicap (VA-PLH) involved 2 steps: i) construction of the scale and ii) field validation. Participant selection criteria were aged > 3 years, age at onset of cerebral lesion < 3 years, a combination of motor impairment and profound intellectual impairment associated with restricted mobility (Gross Motor Function Classification System levels [GMFCS] III, IV or V), and everyday life dependence (Functional Independency Measure [FIM] <55). Vision assessment by both an orthoptist and an ophthalmologist was the reference against which were analysed the items of the scale completed by local health care workers. Acceptability, validity, and reliability were analysed. RESULTS: Amongst the 232 participants included, 217 had a complete assessment, and 33% were < 18 years of age. Ocular abnormalities were reported in 83% of participants. Visual ability was altered or insufficient in 60% of participants. The final version of the VA-PLH included 3 items related to visual reaction (Area Under Curve Receiver Operating Characteristic = 0.83). Participants were considered at-risk if they had at ≥ 1 of 3 signs present (sensitivity 83% and specificity 73%). The scale's reliability was satisfactory CONCLUSION: The VA-PLH scale provides an easy-to-use, reliable and valid measure of visual status for people with polyhandicap and may be used both in clinical practice and clinical research. In addition, this study provides an overview of the diversity of visual impairments in a large population of people with polyhandicap, showing that most experience visual challenges.

Parents' experiences of parenting a child with profound intellectual and multiple disabilities in France: A qualitative study.

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.

Polyhandicap, profound intellectual multiple disabilities : Concept and definition of a highly specific public health issue.

OBJECTIVES: The concept of polyhandicap first emerged in the late '60s in France, with actually a consensus on its definition. This consensus has yet to be reached internationally. The absence of an international consensus on a definition and name for persons with polyhandicap limits progress in research and health planning for these people. METHODS: This article describes the history of the emergence of the concept of polyhandicap in France and internationally. RESULTS: The emergence of the concept and definition of polyhandicap is part of the history of the development of special education and care for children with disabilities started at the end of the 19th century and during the first half of the 20th century. In France, between 1970 and 2002, working groups composed of professionals and family associations gradually developed and refined the definition of polyhandicap, differentiating it from other clinical entities such as cerebral palsy. Internationally, the term polyhandicap is used in 4 European countries: in France where it first appeared, in Italy, in French-speaking Belgium, and in French-speaking Switzerland but also outside the EU. Various terms may be used around the world to describe clinical entities similar to polyhandicap; the most frequently used in the literature is the term Profound Intellectual and Multiple Disabilities (PIMD) or PIMD Spectrum which does not systematically refer to an early brain injury. DISCUSSION: We are currently in the process of internationalizing the concept and definition of polyhandicap, and hopefully, as was the case for cerebral palsy in the 2000s, the various research teams working on this subject around the world will create collaborations and research networks targeting this specific population. CONCLUSION: A consensus around a precise definition of polyhandicap is important to ensure that these people are recognized for their uniqueness and specific qualities and to provide them adapted care.

The French EVAL-PLH cohort of persons with polyhandicap.

Polyhandicap is characterized by a combination of profound intellectual disability and serious motor deficit, resulting in the extreme restriction of autonomy and communication. The aim of the EVAL-PLH (EVALuation PoLyHandicap) study is to identify the impact of socioeconomic, environmental, and epidemiological determinants on the health status of the persons with polyhandicap and the daily lives of their caregivers. EVAL-PLH is a prospective cohort study. The study involved persons with severe polyhandicap (who were cared for at reeducation centers, residential facilities, and one specialized pediatric/neurological department of a university hospital), their familial caregivers and the institutional caregivers. Data collection included sociodemographics, heath status, and psychocomportemental information. Data have been collected at 2 points (2015-2016 and 2020-2021). The French EVAL-PLH cohort is the first cohort study focusing on persons with polyhandicap, their families, and the health care workers caring for them. The sustainability of the device is essential to assist patients, families, clinicians, and health decision-making authorities in the optimization of care management.

Impact of severe polyhandicap cared for at home on French informal caregivers' burden: a cross-sectional study.

OBJECTIVES: Polyhandicap (PLH), defined by a combination of profound intellectual impairment and serious motor deficits, is a severe condition with complex disabilities. In France, care of the large majority of PLH individuals is managed in specialised rehabilitation centres or residential facilities, but some of PLH individuals are cared for at home. The aims of this study were to assess the self-perceived burden among informal caregivers of PLH individuals and to identify potential determinants of this burden. DESIGN: Cross-sectional study (Neuropaediatric Department, Trousseau Hospital, Paris, France). SETTINGS: PLH children were recruited from a specialised paediatric/neurological department. PARTICIPANTS: The selection criteria of caregivers were age above 18 years and being the PLH individual's next of kin. OUTCOMES MEASURES: From March 2015 to December 2016, data were collected from the caregivers, including sociodemographical data, health status, psycho-behavioural data (quality of life, mood disorders and coping) and self-perceived burden. In addition, the health status of the PLH individual was collected. Relationships between the burden scores and potential determinants were tested (correlations coefficients, Mann-Whitney tests, generalised estimating equations models). RESULTS: Eighty-four children were eligible; 77 families returned their questionnaire. The informal caregivers of PLH children experienced a high level of perceived burden (scores ranged from 55±20 to 81±12). Eighty per cent of them had more than 5 hours of daily caregiving and 51% of them had to get up more than twice during the night. The main factors associated with caregiver burden were age, financial issues, health status, daily care and coping strategies. The patients' health status was not associated with caregiver burden. CONCLUSIONS: Some of the caregiver burden determinants might be modifiable. These findings should help healthcare workers and health-decision makers implement specific and appropriate interventions. TRIAL REGISTRATION NUMBER: NCT02400528.

Polyhandicap and aging.

BACKGROUND: Knowledge of the health status and care management of elderly individuals with polyhandicap* is lacking; however, a better understanding of the natural course of ageing in persons with severe and complex disability would help optimize preventive and curative care management strategies. OBJECTIVES: To describe persons with severe and complex disability aged 18-68 years by providing i) a description of their health status and ii) a description of their medications, medical devices and rehabilitation procedures. METHODS: This was an 18-month cross-sectional study including people aged 18-68 years with a combination of severe motor deficiency and profound intellectual impairment. They were recruited from 4 specialized rehabilitation centres, 9 residential facilities, and a neurological department. The following data were collected: aetiology of severe and complex disability, health status, medical devices, and rehabilitation procedures. RESULTS: A total of 474 persons with severe and complex disability were included (N = 219 [18-34 years], N = 151 [35-49 years], N = 104 [50-68] years). The aetiology of severe and complex disability was unknown for 13%-17% of persons with severe and complex disability across the 3 age classes. Behavioural disorders and pain were more frequent in the oldest age classes. Elderly persons with severe and complex disability had more severe but less unstable severe and complex disability. Their neurodevelopmental was close to that of a 4-month-old child without progression across age. Gastrostomy was the most frequent device needed by the persons with severe and complex disability. CONCLUSIONS: The longevity of persons with severe and complex disability is improving; some of these persons, among whom are the least unstable and with less comorbidity, can survive for more than 50 years due to the improvement of preventive actions and supportive care.

Impact of severe polyhandicap on parents' quality of life: A large French cross-sectional study.

BACKGROUND: Polyhandicap (PLH) is a condition of severe and complex disabilities and is defined by a combination of profound intellectual impairment and serious motor deficits. Parents of PLH individuals are chronically confronted with stressful situations. The aims of this study are i) to assess and compare the quality of life (QoL) of a large panel of parents of PLH individuals with age- and gender-matched controls and ii) to identify potential determinants of parents' QoL. METHOD: We conducted a cross-sectional study. Parents were recruited from 4 specialized rehabilitation centres, 9 residential facilities, and a specialized paediatric/neurological department. The selection criteria were age above 18 years and being the mother/father of a PLH individual. The data collected from the parents included sociodemographic, health status, and psycho-behavioural data (including QoL); additionally, the health status of the PLH individuals was collected. RESULTS: The QoL scores of all dimensions were significantly lower for parents than for controls. The main factors modulating parents' QoL were financial issues, health status, and coping strategies. The PLH individuals' health status was not associated with parents' QoL. CONCLUSIONS: Some QoL determinants might be amenable. These findings should help health care workers and health decision makers to implement specific and appropriate interventions.

Adequacy of care management of patients with polyhandicap in the French health system: A study of 782 patients.

BACKGROUND: The aims of this study were 1) to describe the health profiles and care management of polyhandicapped patients according to 2 modalities, specialized rehabilitation centers (SRC) and residential facilities (RF), and 2) to estimate the adequacy of care management of these patients. METHODS: This was an 18-month cross-sectional study including patients with a combination of severe motor deficiency and profound intellectual impairment. The patients were from 4 SRC and 9 RF. The following data were collected: sociodemographics, health status, care management, and adequacy of care management. RESULTS: A total of 782 patients were included: 410 (52%) were cared for in SRC and 372 (48%) in RF. Global objective adequacy (health severity and age category) was higher for patients cared for in SRC compared with patients cared for in RF (57 vs. 44%, p< = 10-3). Global subjective adequacy (self-perception of the referring physician and request of change in structure) was higher for patients cared for in SRC in comparison with patients cared for in RF (98 vs. 92%, p< = 10-3). CONCLUSIONS: This study provides key elements of adequacy of care management modalities for polyhandicapped patients in France. TRIAL REGISTRATION: ClinicalTrials.gov NCT02400528.

Exploring the perceptions of physicians, caregivers and families towards artificial nutrition and hydration for people in permanent vegetative state: How can a photo-elicitation method help?

The question of withdrawing artificial nutrition and hydration from people in a permanent vegetative state sparks considerable ethical and legal debate. Therefore, understanding the elements that influence such a decision is crucial. However, exploring perceptions of artificial nutrition and hydration is methodologically challenging for several reasons. First, because of the emotional state of the professionals and family members, who are facing an extremely distressing situation; second, because this question mirrors representations linked to a deep-rooted fear of dying of hunger and thirst; and third, because of taboos surrounding death. We sought to determine the best method to explore such complex situations in depth. This article aims to assess the relevance of the photo-elicitation interview method to analyze the perceptions and attitudes of health professionals and families of people in a permanent vegetative state regarding artificial nutrition and hydration. The photo-elicitation interview method consists in inserting one or more photographs into a research interview. An original set of 60 photos was built using Google Images and participants were asked to choose photos (10 maximum) and talk about them. The situations of 32 patients were explored in 23 dedicated centers for people in permanent vegetative state across France. In total, 138 interviews were conducted with health professionals and family members. We found that the photo-elicitation interview method 1) was well accepted by the participants and allowed them to express their emotions constructively, 2) fostered narration, reflexivity and introspection, 3) offered a sufficient "unusual angle" to allow participants to go beyond stereotypes and habits of thinking, and 4) can be replicated in other research areas. The use of visual methods currently constitutes an expanding area of research and this study stressed that this is of special interest to enhance research among populations facing end-of-life and ethical issues.

Impact of caring for patients with severe and complex disabilities on health care workers' quality of life: determinants and specificities.

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.

Evaluation of quality of life in individuals with severe chronic motor disability: A major challenge.

Diverse conditions causing a very heavy and chronic motor disability, such as an advanced amyotrophic lateral, advanced form of multiple sclerosis, high spinal cord injury or a locked-in syndrom, are now getting better medical care and benefit of life support technology with consequent prolonged survival. Quality of life (QoL) assessment is being considered increasingly important to globally apprehend their general well-being. However, the motor disability that affects them appears as a substantial limitation for the assessment of their QoL and consequently a major challenge for all the community that carries an interest for them. This review discussed several avenues to provide to patients and caregivers, clinicians and researchers, and health decision making authority: i) elements to determine the most appropriate QoL measure with regard to the interest of patient's point of view, the QoL instruments suitable for this category of patients and their acceptability, ii) some arguments of the clinical relevance and accuracy of QoL assessment: interpretations of the questionnaires, QoL determinants, particularity of QoL evaluation for individuals with cognitive impairment and the caregivers perceptions of patients QoL. In conclusion, evaluation of QoL in patients with severe chronic motor handicap is a challenge of major interest, with major ethical issues. It needs to use adapted QoL scales and longitudinal following because of adaptive phenomena to the degree of handicap.

Quality of life in patients with locked-in syndrome: Evolution over a 6-year period.

BACKGROUND: Improved knowledge of the quality of life (QoL) of locked-in syndrome (LIS) patients have implications for managing their care, and assists clinicians in choosing the most appropriate interventions. We performed a survey of a population of LIS patients to describe the course of the QoL of LIS patients over a 6-year period and to determine the potential predictive factors of QoL changes over time. METHOD: This is a study performed over a 6-year period in patients with a LIS diagnosis. Questionnaires were sent in 2007 and 2013. The following data were recorded: i) sociodemographic data; ii) clinical data related to LIS, physical/handicap status, psychological status; iii) self-reported QoL: Anamnestic Comparative Self-Assessment (ACSA); iv) Integration in life: French Reintegration to Normal Living Index (RNLI). RESULTS: Among the 67 patients included in 2007, 39 (58 %) patients returned their questionnaire in 2013. The LIS etiology was stroke in 51 individuals. The QoL of the patients was relatively satisfactory compared to populations in other severe conditions. Twenty-one (70 %) individuals reported a stable/improved QoL between 2007 and 2013. The physical/handicap statuses in 2007 and 2013 were not related to the QoL 6 years later, with the exception of one communication parameter: the individuals who used yes-no code reported significantly lower QoL levels than those who did not in 2013. DISCUSSION: In opposition to a widespread opinion, LIS persons report a relatively satisfactory QoL level that stays stable over time, suggesting that life with LIS is worth living. Preservation of autonomy and communication may help them to live as normal life as possible.

Evaluation of quality of life in complete locked-in syndrome patients.

BACKGROUND: There are few studies where quality of life (QOL) and contributive factors are assessed in locked-in syndrome (LIS) patients with complete physical and functional disability and dependence on caregivers. OBJECTIVE: The study's objective was evaluation of QOL in LIS patients. DESIGN: We have compared QOL in LIS patients with QOL in healthy controls (control group). SETTING/SUBJECTS: We have included 19 LIS patients, most of them with vascular etiologies or terminal course of ALS, and 20 healthy controls comparable with LIS patients for age; none of them was in a caregiving position. MEASUREMENTS: Administered to patients and controls were the McGill Quality of Life Single Item Scale (MQOL-SIS) Part A; Short Form survey (SF-36), Mental Component Summary (MCS) and Physical Component Summary (PCS); Beck Depression Inventory-II (BDI-II); and Toronto Alexithymia Scale (TAS). RESULTS: Mean MQOL-SIS and MCS SF-36 were not significantly different between the LIS group and healthy controls. PCS SF-36 score was significantly higher in controls. There were no significant differences between the two groups for TAS scores. Frequency of depressive symptoms was significantly higher in LIS patients than in controls. CONCLUSION: In our study, QOL in LIS patients was not significantly altered compared to control subjects in MQOL-SIS and in the MCS SF-36 scale; these results match previous studies published in the literature. Several factors may have an impact on QOL in LIS patients, such as family support and patient-computer communication devices; these may have contributed to improve QOL in LIS patients in this study.

Quality of life of ALS and LIS patients with and without invasive mechanical ventilation.

There are very few studies where quality of life (QOL) is assessed in patients with complete physical and functional disability and dependence to invasive mechanical ventilation (IV). We compared QOL of amyotrophic lateral sclerosis (ALS) and locked-in-syndrome (LIS) patients with invasive mechanical ventilation to ALS and LIS patients without mechanical invasive ventilation. Thirty-four patients, 27 with ALS and seven with LIS (vascular or tumoral aetiology) were included in the study. Twelve had invasive ventilation, 22 had non-invasive ventilation, and in the non-invasive ventilation group, five of them had ventilation via mask. The following scales were used for patients: ALS Functional Rating Scale (ALSFRS), McGILL, Short-Form 36 (SF36), Beck Depression Inventory-II, the Toronto Alexithymia Scale and the anxiety inventory of Spielberger. Mean ALSFRS scores were significantly lower in the invasive ventilation group (IV) than in the non-invasive ventilation group. McGILL and SF36 were not significantly different between the IV group and the non-invasive ventilation group; there were no significant differences between the two groups for others scales either. Comparison between IV group and LIS without invasive mechanical ventilation revealed no significant difference for SF36 and McGILL QOL scores. QOL was not significantly different between the IV and not invasively ventilated patients, but ALSFRS was significantly lower in the IV group, and comparison of QOL scores between non-ventilated LIS patients who had the same score of dependence that invasively ventilated patients did not show any difference. Invasive mechanical ventilation for patients who accept tracheotomy allows life prolongation and their QOL is not affected; medical teams should be aware of that.

Valproic acid-induced encephalopathy in very long course treated patients.

PRIMARY OBJECTIVE: In patients receiving valproate (VPA) treatment, valproate induced-encephalopathy (VIE) is among the most serious adverse side-effects and hyperammonaemia is a frequent and well-recognized event. This report evaluates adult patients receiving VPA for several years. RESEARCH DESIGN: This study was conducted in adult patients receiving VPA for several years. Many studies have evaluated blood ammonia levels and VPA-related encephalopathy in patients whose treatment was initiated for a few months. Most studies were conducted in children. METHODS AND PROCEDURES: This study looked at retrospectively occurrences of VIE in 63 patients who were receiving VPA for a minimum duration of at least 2 years. At the beginning of the study basal ammonaemia was measured for all patients. MAIN OUTCOMES AND RESULTS: This study reports a rather frequent onset of VIE. Long duration of VPA treatment was not correlated with encephalopathy onset. In seven cases temporary administration of lactulose alone was effective and VPA was continued. CONCLUSIONS: This study confirms that, under VPA treatment, hyperammonaemia is a current event; 25.4% patients presented isolated elevated ammonaemia when receiving VPA for very long durations. VPA therapy should be monitored closely and clinicians must be warned of VPA encephalopathy signs.