Dystrophin Genotype and Risk of Neuropsychiatric Disorders in Dystrophinopathies: A Systematic Review and Meta-Analysis.

BACKGROUND: Dystrophinopathies are associated with neuropsychiatric disorders due to alterations in dystrophin/DMD expression. OBJECTIVE: The objective was to estimate the association of developmental disorders, autism spectrum disorders (ASD), attention deficit hyperactivity disorder (ADHD), depression, anxiety disorders, and obsessive-compulsive disorder with the dystrophin/DMD genotype in population with dystrophinopathies. METHODS: Systematic searches of Medline, Scopus, Web of Science, and Cochrane Library were performed from inception to September 2022. We included observational studies in the population with Becker or Duchenne muscular dystrophies (BMD, DMD) that estimated the prevalence of these disorders according to Dp140 and/or Dp71 genotype. Meta-analysis of the prevalence ratio (PR) of genotype comparisons was conducted for each disorder. RESULTS: Ten studies were included in the systematic review. In BMD, Dp140+ vs. Dp140- and Dp71+ vs. Dp71- were associated with developmental disorders with a PR of 0.11 (0.04, 0.34) and 0.22 (0.07, 0.67), respectively. In DMD, Dp140+/Dp71+ vs. Dp140- /Dp71- had a PR of 0.40 (0.28, 0.57), and Dp71+ vs. Dp71- had a PR of 0.47 (0.36, 0.63) for ADHD. However, there was no association of genotype with ASD, only a trend was observed for Dp71+ vs. Dp71-, with a PR of 0.61 (0.35, 1.06). Moreover, the data showed no association of these isoforms with emotional-related disorders. CONCLUSIONS: In BMD, Dp140 and Dp71 could be associated with developmental disorders, while ADHD might be associated with the Dp71 genotype in DMD. Further research is needed regarding Dp140 and Dp71, especially in DMD for ASD.

A patient-focused survey to assess the effects of the COVID-19 pandemic and social guidelines on people with muscular dystrophy.

INTRODUCTION/AIMS: In this study, we examined the social and health impacts of the coronavirus disease 2019 (COVID-19) pandemic and social guidelines on people with muscular dystrophies. METHODS: A prospective de-identified electronic survey was distributed to adults with self-reported facioscapulohumeral muscular dystrophy (FSHD), myotonic dystrophy (DM), and limb-girdle muscular dystrophy (LGMD) enrolled in national registries or with patient advocacy groups. The COVID-19 Impact Survey was developed by muscular dystrophy experts in association with patient collaborators and advocacy groups. The Perceived Stress Scale was used to measure perceived stress. RESULTS: Respondents (n = 774: 56% FSHD; 35% DM, and 9% LGMD) were mostly women and middle-aged (range 19-87 y). Rates of COVID-19 infections were low (<1%), compliance with local social distancing guidelines and policies high (98%). Major challenges reported during the pandemic included: obtaining treatment (40%), managing stress (37%), social distancing (36%), and obtaining essentials (34%). The majority reported a slight worsening in their disease state. Respondents reported moderate stress levels (stress score = 15.4; range = 0-35), with higher stress levels reported by women and those under age 30 y. Three-quarters of participants who participated in telemedicine visits were satisfied with the encounters; however, most reported a preference for in-person visits. DISCUSSION: People with muscular dystrophy reported moderate stress and challenges during the COVID-19 pandemic. Interventions such as exercise and stress-coping strategies, including strategies specific to women or individuals <30 y, may be important. Further investigation is needed into the role of telemedicine in the care of individuals with muscular dystrophy.

Psychological parameters impact health-related quality of life in mental and physical domains in adults with muscular dystrophy.

The impacts of potentially treatable psychological parameters on quality of life are relatively unreported in adults with Facioscapulohumeral, Becker and Limb-girdle muscular dystrophy. The purpose of this study was to compare quality of life, psychological parameters, and physical function between adults with muscular dystrophy and controls, and to examine relationships among these parameters in muscular dystrophy. Twenty-one adults with muscular dystrophy (n = 7 Becker, n = 8 Facioscapulohumeral, n = 6 Limb-girdle) and ten age-matched controls participated. Outcome measures were health-related quality of life, depressive symptoms, trait anxiety, self-esteem, physical self-worth and six-minute walk distance. Quality of life scores were lower in the muscular dystrophy groups than the control (p < .05). Depressive symptoms had the greatest association with quality of life in the Mental Health domain (r= -0.89, p < .001). Depressive symptoms also had the most associations with quality of life (7 of 10 domains), followed by trait anxiety (6 of 10 domains), physical self-worth (5 of 10 domains), self-esteem (4 of 10 domains) and six-minute walk distance (3 of 10 domains). Psychological parameters and, to a lesser extent, physical function impact quality of life in muscular dystrophy. This study provides a rationale to include psychological assessment and treatment within muscular dystrophy healthcare.

Association of Initial Maximal Motor Ability With Long-term Functional Outcome in Patients With COL6-Related Dystrophies.

OBJECTIVE: To accurately categorize the phenotypes of individuals with collagen VI-related dystrophies (COL6-RDs) during the first years of life to predict long-term motor function and pulmonary function, to provide phenotype-specific anticipatory care, and to improve clinical trial readiness. METHODS: This retrospective, multicenter, international study analyzed the relationship of long-term motor and pulmonary function with the initial maximal motor ability achieved in individuals with COL6-RD. RESULTS: We studied 119 patients with COL6-RD from Spain (n = 54) and the United States (n = 65). The early maximal motor milestones of ability to rise from the floor unassisted and ability to climb 4 steps without holding onto a railing demonstrated reliability in distinguishing between 3 COL6-RD phenotypic subgroups: (1) Ullrich congenital muscular dystrophy, (2) intermediate COL6-RD, and (3) Bethlem myopathy. Long-term motor function and pulmonary function are strongly correlated with the maximal motor ability achieved during the first years of life. Maximal motor capacity can predict other disease-relevant events such as the age at loss of ambulation and the need for the initiation of nocturnal noninvasive ventilation. CONCLUSION: This work proposes a prospective phenotypic classification for COL6-RDs that will enable an accurate prediction of a patient's COL6-RD phenotype during the first years of life. The ability to establish a patient's COL6-RD phenotypic classification early will enable a more accurate prognosis of future motor and pulmonary function, thus improving anticipatory clinical care, and it will be instrumental in aiding the design of future clinical trials by allowing early stratification of trial cohorts.

Health-Related Quality of Life and Emotional Distress Among Mothers of Sons With Muscular Dystrophy as Compared to Sex- and Age Group-Matched Controls.

The health-related quality of life and emotional distress among mothers of sons with Duchenne or Becker muscular dystrophies (n = 82) were compared to sex- and age group-matched controls (n = 26). Participants self-reported health-related quality of life for themselves and their son(s), emotional distress, and mood/anxiety-related medication. Mothers reported poorer health-related quality of life across all domains of their health-related quality of life, as well as higher levels of emotional distress. Clinically elevated symptoms of anxiety were reported by 39% of mothers. Mothers' report of poorer health-related quality of life for their son(s) was a significant predictor of worse health-related quality of life and emotional distress for themselves across most domains. Additionally, older age of mothers predicted greater energy/less fatigue and lower levels of anxiety. Results highlight the need for screening emotional distress among mothers, as well as consideration for accessible interventions to improve the psychosocial functioning among these families.

Inappropriate/d bodies: Reorganizing the terms of life and death.

The aim of this article is to offer insight into the different meanings of death that pierce the lives of people with disabilities and to discuss how those meanings are formed through a close connection with their bodies. To do that, I take an anthropological approach to trace the life paths of two individuals from a southern Latin American metropolis, exploring their embodied experiences of disability. Based on their accounts, I look at how their bodies are affected by specific conditions of stigma, dispossession, and social death, but also how, as "inappropriate/d" bodies, they rise above the logic of difference and move from a state of "absence" to a state of "presence" in the social world.

Mental health and coping strategies in families of children and young adults with muscular dystrophies.

BACKGROUND: Living with a progressive disease as muscular dystrophy (MD) can be challenging for the patient and the entire family from both emotional and practical point of view. We aimed to extend our previously published data about mental health in patients with MDs, also investigating coping profiles of both themselves and their parents. Furthermore, we wanted to verify whether psychological adaptation of patients can be predicted by coping strategies, taking also into account physical impairment, cognitive level and socioeconomic status. METHODS: 112 patients with MDs, aged 2-32 were included. Their emotional and behavioural features were assessed through parent- and self-report Achenbach System for Empirically Based Assessment questionnaires and Strength and Difficulties Questionnaires. Development and Well-Being Assessment or Autism Diagnostic Observation Schedule were administered to confirm suspected diagnoses. Coping profile of both parents and patients was assessed through the self-administered New Italian Version of the Coping Orientation to the Problems Experienced questionnaire and its relationship with emotional/behavioural outcome was examined in linear regression analyses. RESULTS: High prevalence of intellectual disability and autism spectrum disorders was confirmed in Duchenne MD. Despite the high rate of internalizing symptomatology, we did not report higher rate of psychopathological disorders compared to general population. Parents tend to rely more on positive reinterpretation and less on disengagement coping. Avoidance coping, whether used by parents or patients, and ID, predicted increased emotional/behavioural problems. CONCLUSIONS: Psychosocial interventions should address problems of anxiety and depression that people with MDs frequently experience, even through fostering parents' and childrens' engagement coping over disengagement coping.

Swallowing with Noninvasive Positive-Pressure Ventilation (NPPV) in Individuals with Muscular Dystrophy: A Qualitative Analysis.

The purpose of the study is to describe experiences of swallowing with two forms of noninvasive positive-pressure ventilation (NPPV): mouthpiece NPPV (M-NPPV) and nasal bilevel positive airway pressure (BPAP) in people with muscular dystrophy. Ten men (ages 22-42 years; M = 29.3; SD = 7.1) with muscular dystrophy (9 with Duchenne's; 1 with Becker's) completed the Eating Assessment Tool (EAT-10; Ann Otol Rhinol Laryngol 117(12):919-924 [33]) and took part in semi-structured interviews. The interviews were audio recorded, transcribed, and verified. Phenomenological qualitative research methods were used to code (Dedoose.com) and develop themes. All participants affirmed dysphagia symptoms via responses on the EAT-10 (M = 11.3; SD = 6.38; Range = 3-22) and reported eating and drinking with M-NPPV and, to a lesser extent, nasal BPAP. Analysis of interview data revealed three primary themes: (1) M-NPPV improves the eating/drinking experience: Most indicated that using M-NPPV reduced swallowing-related dyspnea. (2) NPPV affects breathing-swallowing coordination: Participants described challenges and compensations in coordinating swallowing with ventilator-delivered inspirations, and that the time needed to chew solid foods between ventilator breaths may lead to dyspnea and fatigue. (3) M-NPPV aids cough effectiveness: Participants described improved cough strength following large M-NPPV delivered inspirations (with or without breath stacking). Although breathing-swallowing coordination is challenging with NPPV, participants reported that eating and drinking is more comfortable than when not using it. Overall, eating and drinking with NPPV delivered via a mouthpiece is preferred and is likely safer for swallowing than with nasal BPAP. M-NPPV (but not nasal BPAP) is reported to improve cough effectiveness, an important pulmonary defense in this population.

Resilience and Function in Adults With Chronic Physical Disabilities: A Cross-Lagged Panel Design.

BACKGROUND: Resilience is a psychological construct referring to one's positive adaptation in response to adversity. Evidence suggests that resilience may contribute to various function domains in adults with chronic physical disabilities. PURPOSE: To test hypothesized temporal associations between resilience and four function domains (anxiety, depression, social role satisfaction, and physical function) in individuals with chronic physical disabilities. METHODS: Participants were 1,574 adults with one of four chronic physical disabilities (spinal cord injury, muscular dystrophy, multiple sclerosis, or postpolio myelitis syndrome) who were participating in a large, ongoing USA-based longitudinal survey study. Three surveys were mailed on an approximately yearly basis. Resilience was assessed using the Connor-Davidson Resilience Scale 10-item (CDRSC-10) and each function domain was assessed using the respective Patient Reported Outcome Measurement System (PROMIS) short-form. RESULTS: Cross-lagged path models evidenced statistically significant reciprocal relationships between resilience and each function domain except physical function. The standardized lagged coefficients corresponding to resilience predicting social role satisfaction (T1-T2 = 0.09, T2-T3 = 0.09) had similar effect sizes as those corresponding to social role satisfaction predicting resilience (T1-T2 = 0.11, T2-T3 = 0.04), although resilience was a slightly stronger predictor in the second lag. In models assessing psychological function, resilience was a stronger predictor of later psychological function (resilience-to-anxiety, T1-T2 = -0.15, T2-T3 = -0.11; resilience-to-depression, T1-T2 = -0.21, T2-T3 = -0.13) than the inverse (anxiety-to-resilience, T1-T2 = -0.11, T2-T3 = -0.06; depression-to-resilience, T1-T2 = -0.12, T2-T3 = -0.05). CONCLUSIONS: The study findings suggest that resilience is a significant prospective predictor of psychological and social function over time in individuals with chronic physical disabilities.

Goal Pursuit, Goal Adjustment, and Pain in Middle-Aged Adults Aging With Physical Disability.

Objective: Aging with physical disability disrupts one's ability to achieve valued goals due to changes in symptoms and function. It is unclear how to cope optimally in this context. This study examined whether two possible strategies-tenacious goal pursuit (TGP) and flexible goal adjustment (FGA)-were associated with reduced pain interference and depressive symptoms and greater well-being, and protected against pain intensity, and FGA was more protective with increasing age and worse physical function. Method: Middle-aged adults with muscular dystrophy, multiple sclerosis, post-polio syndrome, or spinal cord injury (N = 874; MAGE = 58.3 years, range = 46-68; MDISEASEDURATION = 26.2 years, range = 2-67) completed two questionnaires, a year apart. Results: TGP and FGA use was associated with greater well-being. FGA use predicted decreased depressive symptoms. Concurrent use of both predicted decreased pain interference. Discussion: Adults with disability employ a variety of goal management strategies. Findings support TGP and FGA as potential intervention targets for healthy aging with disabilities.

Changes in perceived social support predict changes in depressive symptoms in adults with physical disability.

BACKGROUND: Perceived social support has been found to be associated with depression, subjective well-being and psychological health in cross-sectional studies in people with physical disabilities. No longitudinal studies have been conducted to examine these associations over time using a comprehensive measure of social support. OBJECTIVE/HYPOTHESIS: We hypothesized that: (1) the amount of perceived social support would be similar across individuals with different diagnoses often associated with disability (i.e., multiple sclerosis [MS], spinal cord injury [SCI], muscular dystrophy [MD]) but (2) lower among men, relative to women, and (3) changes over time in perceived social support would be negatively associated with changes in depressive symptoms across diagnostic groups. METHODS: A survey-based six-year longitudinal study. Depressive symptoms were measured with the PROMIS Depression scale. General (Total Scale), Friend, Family and Significant Other social support were measured with the Multi-dimensional Scale of Perceived Social Support (MSPSS). RESULTS: The findings indicated no significant differences in any of the perceived social support scales as a function of diagnosis or sex. However, over the course of six years, those reporting increases in social support reported decreases in depressive symptoms, while those reporting decreases in social support reported increases in depressive symptoms. CONCLUSIONS: Changes in perceived social support are inversely related with the changes in depressive symptoms in adults with MS, SCI, and MD. Social support would be a reasonable treatment target in interventions seeking to improve psychological wellbeing in individuals with conditions that are often associated with disabilities.

Anxiety, depression, and function in individuals with chronic physical conditions: A longitudinal analysis.

OBJECTIVE: This longitudinal study examined the unique relationship between anxiety, symptoms (pain intensity, sleep disturbance, fatigue severity), and function domains (self-reported cognitive function, physical function, satisfaction with social roles) in individuals with chronic physical conditions, independent of depressive symptoms. METHOD: Three surveys were mailed on an approximately yearly basis to community-dwelling adults with one of four chronic physical conditions (spinal cord injury, multiple sclerosis, muscular dystrophy, postpoliomyelitis syndrome). The first survey was completed by 1594 individuals (T1). Of these, 1380 completed the second survey (T2), and 1272 completed the third survey (T3). RESULTS: Mixed growth curve models evidenced significant concurrent and longitudinal associations between anxiety and each symptom and function domain, independent of depression severity. The largest unique association found was between anxiety and self-reported cognitive function; inclusion of the measure of anxiety in the model improved model fit substantially over depression alone, χ²[2] = 104.40, p < .001. Both anxiety and depression exhibited similar effect sizes in their unique relationships with each symptom and function domain measure. However, depression was more strongly associated with satisfaction with social roles and physical function than was anxiety. CONCLUSIONS: The findings showed that anxiety assessed at one point in time demonstrated significant and unique associations with concurrent and subsequent symptom and function domains. Given that anxiety has been inadequately studied (and perhaps evaluated and treated) in rehabilitation populations, the current findings suggest that researchers and clinicians should broaden their scope when assessing and treating psychological distress to incorporate anxiety and related disorders. (PsycINFO Database Record (c) 2018 APA, all rights reserved).

A Qualitative Approach to Health Related Quality-of-Life in Congenital Muscular Dystrophy.

Congenital muscular dystrophies (CMD) cause progressive muscle weakness resulting in severe motor disabilities. Previous studies focused on the effects of motor disability. Here, we explore other factors affecting health related quality-of-life (HRQOL) in CMD. Qualitative interviews were conducted with participant-parent dyads to identify symptoms having the greatest impact on HRQOL. Symptoms were classified into themes and domains representing physical, mental, social health, and disease specific issues. Social role limitations and specific activity impairment were frequently mentioned. A greater understanding of symptoms impacting HRQOL will provide a framework for improved clinical care and patient centered outcomes as new therapies are developed.

Factors affecting the health-related quality of life of caregivers of patients with muscular dystrophy.

Muscular dystrophy (MD) is a group of progressive muscle weakness diseases. The caregiver burden, increasing as the disease progresses, can be associated with impaired health-related quality of life (HRQOL). The aims of this study were to investigate the HRQOL in caregivers of patients with MD and identify the factors associated with HRQOL. A cross-sectional assessment of caregiver HRQOL was performed with the Short Form-36 and compared with norms. The factors affecting HRQOL were investigated by patient and caregiver characteristics. The Muscular Dystrophy Functional Rating Scale was used to assess the functional status (mobility, basic activities of daily living, arm function, and impairment) of patients. The demographic data and social interaction activities of caregivers were assessed. Caregivers (n = 62) had poor HRQOL. Caregiver HRQOL was associated with the patient's functional status, especially in the domains of Vitality and Mental Health. Numerous visits by neighbors and close friends of the caregiver family indicated better HRQOL (in the body pain, general health, vitality, role emotion and mental health domains). Caregiver HRQOL was associated with caregiver education level, while patient age, caregiver age, length of caregiving, and family income were not. These findings demonstrate that caregivers have poor HRQOL, and the mental domain of quality of life is associated with the patient's functional status, social interaction, and caregiver education level. We suggest that rehabilitation programs focus on caregiver HRQOL, promote the patient's functional status with assistive technology, enhance professional caring techniques, and encourage participation in social groups to improve caregiver HRQOL.

Clinical features of children and adults with a muscular dystrophy using powered indoor/outdoor wheelchairs: disease features, comorbidities and complications of disability.

PURPOSE: To describe the clinical features of electric powered indoor/outdoor wheelchair users with a muscular dystrophy, likely to influence optimal prescription; reflecting features of muscular dystrophies, conditions secondary to disability, and comorbidities impacting on equipment provision. METHODS: Cross-sectional retrospective case note review of recipients of electric powered indoor/outdoor wheelchairs provided by a specialist regional wheelchair service. Data on demography, diagnostic/clinical, and wheelchair prescription were systematically extracted. RESULTS: Fifty-one men and 14 women, mean age 23.7 (range 10-67, s.d. 12.95) years, were studied. Forty had Duchenne muscular dystrophy, 22 had other forms of muscular dystrophy, and three were unclassified. Twenty-seven were aged under 19. Notable clinical features included problematic pain (10), cardiomyopathy (5), and ventilatory failure (4). Features related to disability were (kypho)scoliosis (20) and edema/cellulitis (3) whilst comorbidities included back pain (5). Comparison of younger with older users revealed younger users had more features of muscular dystrophy affecting electric powered chair provision (56%) whilst older users had more comorbidity (37%). Tilt-in-space was prescribed for 81% of users, specialized seating for 55% and complex controls for 16%. CONCLUSIONS: Muscular dystrophy users were prescribed electric powered indoor/outdoor chairs with many additional features reflecting the consequences of profound muscle weakness. In addition to facilitating independence and participation, electric powered indoor/outdoor chairs have major therapeutic benefits. Implications for rehabilitation Powered wheelchairs have therapeutic benefits in managing muscular dystrophy pain and weakness. The use of specialized seating needs careful consideration in supporting progressive muscle weakness and the management of scoliosis. Pain, discomfort, pressure risk, and muscle fatigue may be reduced by use of tilt-in-space.

Integrated care of muscular dystrophies in Italy. Part 2. Psychological treatments, social and welfare support, and financial costs.

This paper describes the psycho-social treatments received by 502 patients with MDs and their relatives, and the costs for care sustained by the families in the previous six month period. Data were collected by the MD-Care Schedule (MD-CS) and the Family Problems Questionnaire (FPQ). Psycho-educational interventions were provided to 72 patients (14.3%), and social/welfare support to 331 patients (65.9%). Social/welfare support was higher in patients with DMD or LGMD, in those showing more severe disability, and in patients who were in contact with centres located in Northern Italy. Psycho-educational interventions were received by 156 (31%) relatives, and social/welfare support by 55 (10.9%) and mainly provided by Family/Patients Associations (83.6%). Relatives with higher educational levels, who spent more daily hours in the assistance of patients with DMD, and in contact with centres in Central Italy more frequently benefited from psycho-educational interventions. In the previous year, costs for care were sustained by 314 (63.9%) relatives. Financial difficulties related to patient's condition, were higher in families of patients who needed more intensive rehabilitation and daily hours of caregiving, and in families who lived further away from the reference's centre. These results showed that psycho-social aspects of MDs care are only partially met in Italy, and that ad hoc supportive interventions for these patients and their families should be potentiated.

Comparison of brain MRI findings with language and motor function in the dystroglycanopathies.

OBJECTIVE: To describe the spectrum of brain MRI findings in a cohort of individuals with dystroglycanopathies (DGs) and relate MRI results to function. METHODS: All available brain MRIs done for clinical indications on individuals enrolled in a DG natural history study (NCT00313677) were reviewed. Reports were reviewed when MRI was not available. MRIs were categorized as follows: (1) cortical, brainstem, and cerebellar malformations; (2) cortical and cerebellar malformations; or (3) normal. Language development was assigned to 1 of 3 categories by a speech pathologist. Maximal motor function and presence of epilepsy were determined by history or examination. RESULTS: Twenty-five MRIs and 9 reports were reviewed. The most common MRI abnormalities were cobblestone cortex or dysgyria with an anterior-posterior gradient and cerebellar hypoplasia. Seven individuals had MRIs in group 1, 8 in group 2, and 19 in group 3. Language was impaired in 100% of those in MRI groups 1 and 2, and degree of language impairment correlated with severity of imaging. Eighty-five percent of the whole group achieved independent walking, but only 33% did in group 1. Epilepsy was present in 8% of the cohort and rose to 37% of those with an abnormal MRI. CONCLUSIONS: Developmental abnormalities of the brain such as cobblestone lissencephaly, cerebellar cysts, pontine hypoplasia, and brainstem bowing are hallmarks of DG and should prompt consideration of these diagnoses. Brain imaging in individuals with DG helps to predict outcomes, especially language development, aiding clinicians in prognostic counseling.

Association of Transition Readiness to Intentional Self-Regulation and Hopeful Future Expectations in Youth With Illness.

BACKGROUND: Little is known about how transition readiness relates to other developmental skills of adolescence in youth with chronic illness. Better understanding of how transition readiness relates to these other developmental skills could lead to a broader array of tools to improve transition readiness. Intentional self-regulation (ISR) and hopeful future expectations (HFE) are 2 developmental skills of adolescence that improve with participation in developmental programming and thus are modifiable. METHODS: We explored associations between transition readiness, as measured by the Transition Readiness Assessment Questionnaire 29 (TRAQ-29) and ISR and HFE in youth with chronic illness recruited from a variety of subspecialty clinics from a major southeast medical center. RESULTS: A total of 71 adolescents with chronic illness were included in the analysis. The TRAQ-29 Self-Advocacy domain showed positive associations to both ISR (P = .03) and HFE (P = .009). In addition, the TRAQ-29 overall had positive associations to HFE (P = .04). CONCLUSIONS: The significant associations between TRAQ-29 Self-Advocacy domain scores and ISR and HFE suggest that transition readiness is developing within the context of other developmental areas in adolescence. More work is needed to see if the programming that improves these other developmental skills might also improve transition readiness.

Resilience and Function in Adults With Physical Disabilities: An Observational Study.

OBJECTIVES: To determine if resilience is uniquely associated with functional outcomes (satisfaction with social roles, physical functioning, and quality of life) in individuals with physical disabilities, after controlling for measures of psychological health (depression and anxiety) and symptom severity (pain, fatigue, and sleep disturbance); and to examine the potential moderating effect of sex, age, and diagnosis on the hypothesized associations between resilience and function. DESIGN: Cross-sectional survey study. SETTING: Surveys were mailed (81% response rate) to a community sample of 1949 individuals with multiple sclerosis, muscular dystrophy, postpoliomyelitis syndrome, or spinal cord injury. Participants were recruited through the Internet or print advertisement (28%), a registry of previous research participants who indicated interest in future studies (21%), a departmental registry of individuals interested in research (19%), disability-specific registries (18%), word of mouth (10%), or other sources (3%). PARTICIPANTS: Convenience sample of community-dwelling adults aging with physical disabilities (N=1574), with a mean Connor-Davidson Resilience Scale (10 items) score of 29. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Patient-Reported Outcomes Measurement Information System measures of Satisfaction with Social Roles and Activities and Physical Functioning, the World Health Organization's brief Older People's Quality of Life Questionnaire, and the Connor-Davidson Resilience Scale (10 items). RESULTS: After controlling for age, age squared, sex, diagnosis, psychological health, and symptom severity, resilience was significantly and positively associated with satisfaction with social roles (ß=.17, P<.001) and quality of life (ß=.39, P<.001), but not physical function (ß=.04, P>.05). For every 1-point increase in scores of resilience, there was an increase of .50 in the quality of life score and .20 in the satisfaction with social roles score. Sex also moderated the association between resilience and satisfaction with social roles (F1,1453=4.09, P=.043). CONCLUSIONS: The findings extend past research, providing further evidence indicating that resilience plays a unique role in nonphysical functional outcomes among individuals with physical disabilities.