Effectiveness of an outpatient rehabilitation programme in patients with neuralgic amyotrophy and scapular dyskinesia: a randomised controlled trial.

BACKGROUND: Neuralgic amyotrophy (NA) is an acute inflammation of nerves within the brachial plexus territory leading to severe pain and multifocal paresis resulting in >60% of patients having residual complaints and functional limitations correlated with scapular dyskinesia. Our primary aim was to compare the effects of multidisciplinary rehabilitation (MR), focused on motor relearning to improve scapular dyskinesia and self-management strategies for reducing pain and fatigue, with usual care (UC) on shoulder, arm and hand functional capability in patients with NA. METHODS: In a non-blinded randomised controlled trial (RCT), patients with NA (aged≥18 years, scapular dyskinesia, >8 weeks after onset) were randomised to either an MR or an UC group. MR consisted of a diagnostic multidisciplinary consultation and eight sessions of physical and occupational therapy. Primary outcome was functional capability of the shoulder, arm and hand assessed with the Shoulder Rating Questionnaire-Dutch Language Version (SRQ-DLV). RESULTS: We included 47 patients with NA; due to drop-out, there were 22 participants in MR and 15 in UC for primary analysis. The mean group difference adjusted for sex, age and SRQ-DLV baseline score was 8.60 (95%CI: 0.26 to 16.94, p=0.044). The proportion attaining a minimal clinically relevant SRQ-DLV improvement (≥12) was larger for the MR group (59%) than the UC group (33%) with a number needed to treat of 4. CONCLUSION: This RCT shows that an MR programme focused on motor relearning to improve scapular dyskinesia, combined with self-management strategies for reducing pain and fatigue, shows more beneficial effects on shoulder, arm and hand functional capability than UC in patients with NA. TRIAL REGISTRATION NUMBER: NCT03441347.

Long-term outcomes for females with early-onset dystrophinopathy.

AIM: To study long-term disease course for females with early-onset dystrophinopathy, including common (female) symptoms, challenges in social participation, the need for care, and current healthcare management to support guideline development. METHOD: Twelve females with early-onset dystrophinopathy were followed for a median period of more than 17 years (range 1-36). RESULTS: One patient died owing to end-stage cardiac failure. Cardiac abnormalities were observed in three of the remaining 11 participants. Respiratory function was reduced in seven of 10 participants. Fatigue, myalgia, lower back pain, and arthralgia were reported in more than six of the participants. Functional status varied from exercise intolerance to wheelchair dependency. Most or all of the 10 participants reported restrictions in participation in work (n = 10), household duties (n = 10), sports (n = 9), and education (n = 8). Only a few participants received followed-up pulmonary (n = 2) or rehabilitation (n = 3) care. INTERPRETATION: Females with early-onset dystrophinopathy experience a wide range of impairments, comorbidities, limitations in activities, and restrictions in social participation. The whole spectrum should be acknowledged in the healthcare setting. Neuromuscular and cardiac follow-up are indispensable. Additional respiratory assessment and rehabilitation care are expected to improve health status and support daily activities and participation. WHAT THIS PAPER ADDS: No standard diagnostic procedures seem to exist for female patients suspected for dystrophinopathy. Female participants with early-onset dystrophinopathy experienced a broad scope of burdening symptoms, such as fatigue, myalgia, lower back pain, and arthralgia. None of participants worked full time, all felt restricted in paid work, and most felt restricted in education. Most participants showed decreased lung function, while only one was symptomatic. Availability of rehabilitation care may improve support for daily activities and participation for females with early-onset dystrophinopathy.

Occurrence of symptoms in different stages of Duchenne muscular dystrophy and their impact on social participation.

INTRODUCTION/AIMS: As life expectancy improves for patients with Duchenne muscular dystrophy (DMD), new symptoms are likely to arise. This aims of this study are: (1) to explore the prevalence of a broad variety of symptoms in the various stages of DMD (with and without steroid use); (2) to explore the prevalence of common secondary diagnoses; and (3) to evaluate the social participation level of patients with DMD older than 16 y of age; and to explore correlations between social participation and symptoms. METHODS: A cross-sectional self-report questionnaire, including questions on functional level and health status, as well as a standardized participation scale was distributed among Dutch patients with DMD. RESULTS: Eighty-four male patients with a mean age of 22.0 (SD = 10.0) y were enrolled. The most prevalent and limiting symptoms were difficulty coughing (58%), coldness of hands (57%), contractures (51%), stiffness (49%), fatigue (40%), myalgia (38%), and low speech volume (33%). Prevalent secondary diagnoses included cardiac disease (14%), neurobehavioral diagnosis (13%), low blood pressure (13%), and arthrosis (5%). Social participation correlated negatively with coldness of hands (r = - .29; P < .03), decreased intelligibility (r = - .40; P < .003), and chewing problems (r = - .33; P < .02). DISCUSSION: The prevalence of a broad spectrum of symptoms and secondary diagnoses is high in patients with DMD, and some of these symptoms are correlated with social participation. Growing awareness of new symptoms and secondary diagnoses among patients, caregivers, and professionals can enhance their recognition, possibly facilitating prevention and early treatment.

Self-management develops through doing of everyday activities-a longitudinal qualitative study of stroke survivors during two years post-stroke.

BACKGROUND: A description of the complexity of the process of self-management and the way stroke survivors give meaning to their process of self-management post-stroke is lacking. This study explores how stroke survivors managed their lives, gave meaning to their self-management post-stroke and how this evolved over time. METHODS: Data was generated through participant observations and interviews of 10 stroke survivors at their homes at 3, 6, 9, 15 and 21 months post-discharge. A constant comparative method was chosen to analyse the data. RESULTS: 'Situated doing' was central in stroke survivors' simultaneous development of self-management and their sense of being in charge of everyday life post-stroke. Doing everyday activities provided the stroke survivors with an arena to explore, experience, evaluate, develop and adapt self-management and being in charge of everyday activities and daily life. The influence of stroke survivors' partners on this development was sometimes experienced as empowering and at other times as constraining. Over time, the meaning of self-management and being in charge changed from the opinion that self-management was doing everything yourself towards self-managing and being in charge, if necessary, with the help of others. Moreover, the sense of self-management and being in charge differed among participants: it ranged from managing only at the level of everyday activities to full role management and experiencing a meaningful and valuable life post-stroke. CONCLUSIONS: The findings of this study indicate the doing of activities as an important arena in which to develop self-management and being in charge post-stroke. Stroke self-management programs could best be delivered in stroke survivors' own environment and focus on not only stroke survivors but also their relatives. Furthermore, the focus of such interventions should be on not only the level of activities but also the existential level of self-management post-stroke.

Effectiveness and cost-effectiveness of a self-management group program to improve social participation in patients with neuromuscular disease and chronic fatigue: protocol of the Energetic study.

BACKGROUND: Chronic fatigue is present in more than 60% of the patients with a neuromuscular disease and can be their most disabling symptom. In combination with other impairments, fatigue often results in low levels of physical activity and decreased social participation, leading to high societal costs. 'Energetic' is a self-management group program aimed at improving social participation, physical endurance and alleviating fatigue in these patients. The primary aim of this study is to evaluate the effectiveness and cost-effectiveness of the Energetic program. METHODS/DESIGN: A multicentered, assessor-blinded, two-armed randomized controlled trial is conducted with evaluations at inclusion and four, seven and fifteen months later. The study includes patients with a neuromuscular disease and chronic fatigue and, when present, their caregivers. The participants are randomized (ratio 1:1) to either an intervention group, receiving the Energetic program, or a control group, receiving usual care (i.e., no specific intervention). The Energetic program covers four months and includes four modules: 1) individually tailored aerobic exercise training; 2) education about aerobic exercise; 3) self-management training in applying energy conservation strategies; and 4) implementation and relapse prevention in daily life. Two months after cessation of the program a booster session is provided. The primary outcome is the perceived performance score of the Canadian Occupational Performance Measure (COPM). Secondary outcomes include the COPM-satisfaction score, and measures of fatigue, physical endurance, activity engagement, mood, and self-efficacy. Caregiver burden is also evaluated as a secondary outcome. Health-related quality of life and medical and societal costs are assessed to estimate cost-effectiveness of the program. DISCUSSION: The Energetic study is the first randomized controlled trial to evaluate the effectiveness and cost-effectiveness of a combined physical and self-management group training program for improving social participation, physical endurance and alleviating fatigue in patients with neuromuscular diseases. It will generate new insights in (cost-)effective rehabilitation strategies for these incurable conditions. TRIAL REGISTRATION: Clinicaltrials.gov NCT02208687 .

Development of the writing readiness inventory tool in context (WRITIC).

This article describes the development of the Writing Readiness Inventory Tool in Context (WRITIC), a measurement evaluating writing readiness in Dutch kindergarten children (5 and 6 years old). Content validity was established through 10 expert evaluations in three rounds. Construct validity was established with 251 children following regular education. To identify scale constructs, factor analysis was performed. Discriminative validity was established by examining contrast groups with good (n = 142) and poor (n = 109) performers in paper-and-pencil tasks. Content validity was high with 94.4% agreement among the experts. Two reliable factors were found in the performance of paper-and-pencil tasks with Cronbach's alphas of 0.82 and 0.69 respectively. The contrast groups differed significantly in two WRITIC subdomains: "Sustained attention" and "Task performance". Our findings indicated that the WRITIC is feasible for use in the classroom.

Writing Readiness Inventory Tool in Context (WRITIC): reliability and convergent validity.

BACKGROUND/AIM: This study examined the reliability and convergent validity of the Writing Readiness Inventory Tool in Context, a measurement evaluating writing readiness in kindergarten children (aged from five to six years). METHODS: Test-retest reliability was established with 59 children, inter-rater reliability with 72 children and convergent validity with 119 children. All participants were typically developing kindergarten children. Convergent validity was examined with the Beery-Buktenica Developmental Test of Visual-Motor Integration and the Nine-Hole Peg Test. RESULTS: We found excellent test-retest and inter-rater reliability on the future norm-referenced subdomain 'Task performance' of Writing Readiness Inventory Tool in Context with intra-class correlation coefficient ranging from 0.92 to 0.95. On the other criterion-referenced subdomains, we found fair to good reliability with intra-class correlation coefficient ranging from 0.70 to 1.0 and weighted Kappa ranging from 0.30 to 0.89. Correlations with the Beery-Buktenica Developmental Test of Visual-Motor Integration and the Nine-Hole Peg Test were moderate with rs ranging from 0.34 to 0.40 and these are comparable with correlations in other handwriting studies. CONCLUSION: Writing Readiness Inventory Tool in Context is an assessment of writing readiness that is stable over time and between raters. The expected moderate correlations with the Beery-Buktenica Developmental Test of Visual-Motor Integration and the Nine-Hole Peg Test support the construct of writing readiness.

The capability approach in rehabilitation: developing capability care.

PURPOSE: To develop a multidisciplinary outpatient rehabilitation intervention for people with neuromuscular diseases (NMD) based on the capability approach: capability care for persons with NMD. MATERIALS AND METHODS: The development process is described using a framework of actions for intervention development. It has been an iterative process consisting of a design phase based on theoretical insights and project group discussions, and a refine phase involving input from relevant stakeholders. RESULTS: Multidisciplinary efforts have resulted in the development of capability care for rehabilitation of persons with NMD. It can focus both on facilitating and achieving functionings (beings and doings), as well as looking for alternative functionings that fulfil the same underlying value, thereby contributing to the persons' well-being. To facilitate a conversation on broader aspects that impact on well-being, persons with NMD receive a preparation letter and healthcare professionals are provided with guiding questions and practical tools to use. CONCLUSIONS: We have shown that it is possible to develop a healthcare intervention based on the capability approach. We hope that rehabilitation professionals will be encouraged to use capability care and that other medical professionals will be inspired to develop capability care in their respective fields. REGISTRATION: Registered at trialregister.nl NL8946.

The capability approach can be used for development of healthcare interventions.Capability care in rehabilitation focuses on realising what is of real value to the person.The capability approach and the ICF are complementary and can both be used in rehabilitation.

Longitudinal Course of Long Finger Flexor Shortening in Males with Duchenne Muscular Dystrophy: A Retrospective Review1.

BACKGROUND: Shortening of the long finger flexors (Flexor Digitorum Profundus, FDPs) in Duchenne Muscular Dystrophy (DMD) causes reduced hand function. Until now, longitudinal studies on the natural course of the shortening of the FDPs are lacking, which impedes recommendations on timing and evaluation of preventive measures. OBJECTIVE: To investigate the longitudinal course of the FDP length during different disease stages focusing on symmetry, timing, and decline of the FDP length. METHODS: A retrospective, longitudinal multicenter study was conducted in the Radboud university medical center and the Leiden university medical center. The FDP outcome was measured using goniometry and gross motor function was assessed using the Brooke score. Longitudinal mixed model analyses were used to describe the course of the FDP outcome, and to investigate symmetry in both hands. RESULTS: Data on 534 visits of 197 males (age ranged 4-48 years) showed that in the ambulatory stages the FDP outcome was within a normal range. The mean decline in FDP outcome is 3.5 degrees per year, the biggest decline was seen in Brooke 5 (>15 degrees per year). In Brooke 4, 41% of the FDP outcome was < 40 degrees. No significant differences were found between right and left. CONCLUSIONS: This study supports the consideration of preventive measures to delay shortening of the FDPs in DMD patients transitioning to a Brooke scale of 4 or higher. Besides, natural history of FDP outcome has been established, which provides a base to evaluate (preventive) interventions.

Quality of life and support needs in children, adolescents, and young adults with facioscapulohumeral dystrophy, a mixed-method study.

BACKGROUND AND OBJECTIVES: Quality of life (QoL) in children with facioscapulohumeral dystrophy (FSHD) seems plausible decreased. Little is known about factors influencing QoL in children with FSHD. Our objective is to explore factors contributing to the QoL of children, adolescents, and young adults with FSHD, to describe how they experience life with FSHD, and to report their support needs. METHODS: We performed a mixed-method study with individual age-appropriate semi-structured interviews assessing QoL in children, adolescents, and young adults with FSHD and their parents. To characterize the sample, quantitative data on QoL, pain, fatigue, and participation were collected. Interview data was analyzed using a thematic analysis. RESULTS: Fourteen patients participated (age between 9 and 26 years old, eight males and six females). The degree of FSHD severity, as indicated by the FSHD-score, did not correlate with QoL. Older children had a lower QoL than younger children. Children and adolescents strived for normality regardless of physical discomfort. Phenotypical features of FSHD led to insecurity aggravated by hurtful comments of others. The unpredictability of disease progression and its implications for career and parenthood choices led to a generalized feeling of uncertainty about the future. Support was found within family and friends. Participants expressed a need for peer support and psychological support as well as recommending it to others. DISCUSSION: Quality of life in childhood FSHD is diminished caused by their physical limitations, altered appearance, fear of social rejection, and uncertainty of the disease progression in the future. A fear of social rejection most likely contributes to striving for normality regardless of physical discomfort. Support should be focused on acceptance and coping with hurtful comments. It should preferably be individualized, easily accessible and not offered as therapy but rather as tutoring for children.

Ready for handwriting? A reference data study on handwriting readiness assessments.

INTRODUCTION: Early evaluation of writing readiness is essential to predict and prevent handwriting difficulties and its negative influences on school occupations. An occupation-based measurement for kindergarten children has been previously developed: Writing Readiness Inventory Tool In Context (WRITIC). In addition, to assess fine motor coordination two tests are frequently used in children with handwriting difficulties: the modified Timed Test of In-Hand Manipulation (Timed TIHM) and the Nine-Hole Peg Test (9-HPT). However, no Dutch reference data are available. AIM: To provide reference data for (1) WRITIC, (2) Timed-TIHM and (3) 9-HPT for handwriting readiness assessment in kindergarten children. METHODS: Three hundred and seventy-four children from Dutch kindergartens in the age of 5 to 6.5 years (5.6±0.4 years, 190 boys/184 girls) participated in the study. Children were recruited at Dutch kindergartens. Full classes of the last year were tested, children were excluded if there was a medical diagnosis such as a visual, auditory, motor or intellectual impairment that hinder handwriting performance. Descriptive statistics and percentiles scores were calculated. The score of the WRITIC (possible score 0-48 points) and the performance time on the Timed-TIHM and 9-HPT are classified as percentile scores lower than the 15th percentile to distinguish low performance from adequate performance. The percentile scores can be used to identify children that are possibly at risk developing handwriting difficulties in first grade. RESULTS: WRITIC scores ranged from 23 to 48 (41±4.4), Timed-TIHM ranged from 17.9 to 64.5 seconds (31.4± 7.4 seconds) and 9-HPT ranged from 18.2 to 48.3 seconds (28.4± 5.4). A WRITIC score between 0-36, a performance time of more than 39.6 seconds on the Timed-TIHM and more than 33.8 seconds on the 9-HPT were classified as low performance. CONCLUSION: The reference data of the WRITIC allow to assess which children are possibly at risk developing handwriting difficulties.

Evaluation of Primary Allied Health Care in Patients Recovering From COVID-19 at 6-Month Follow-up: Dutch Nationwide Prospective Cohort Study.

BACKGROUND: Patients recovering from COVID-19 often experience persistent problems in their daily activities related to limitations in physical, nutritional, cognitive, and mental functioning. To date, it is unknown what treatment is needed to support patients in their recovery from COVID-19. OBJECTIVE: This study aimed to evaluate the primary allied health care of patients recovering from COVID-19 at 6-month follow-up and to explore which baseline characteristics are associated with changes in the scores of outcomes between baseline and 6-month follow-up. METHODS: This Dutch nationwide prospective cohort study evaluated the recovery of patients receiving primary allied health care (ie, dietitians, exercise therapists, occupational therapists, physical therapists, and speech and language therapists) after COVID-19. All treatments offered by primary allied health professionals in daily practice were part of usual care. Patient-reported outcome measures on participation, health-related quality of life, fatigue, physical functioning, and psychological well-being were assessed at baseline and at 3- and 6-month follow-up. Linear mixed model analyses were used to evaluate recovery over time, and uni- and multivariable linear regression analyses were used to examine the association between baseline characteristics and recovery. RESULTS: A total of 1451 adult patients recovering from COVID-19 and receiving treatment from 1 or more primary allied health professionals were included. For participation (Utrecht Scale for Evaluation of Rehabilitation-Participation range 0-100), estimated mean differences of at least 2.3 points were observed at all time points. For the health-related quality of life (EuroQol Visual Analog Scale, range 0-100), the mean increase was 12.3 (95% CI 11.1-13.6) points at 6 months. Significant improvements were found for fatigue (Fatigue Severity Scale, range 1-7): the mean decrease was -0.7 (95% CI -0.8 to -0.6) points at 6 months. However, severe fatigue was reported by 742/929 (79.9%) patients after 6 months. For physical functioning (Patient-Reported Outcomes Measurement Information System-Physical Function Short Form 10b, range 13.8-61.3), the mean increase was 5.9 (95% CI 5.9-6.4) points at 6 months. Mean differences of -0.8 (95% CI -1.0 to -0.5) points for anxiety (Hospital Anxiety and Depression Scale range 0-21) and -1.6 (95% CI -1.8 to -1.3) points for depression were found after 6 months. A worse baseline score, hospital admission, and male sex were associated with greater improvement between baseline and 6-month follow-up, whereas age, the BMI, comorbidities, and smoking status were not associated with mean changes in any outcome measures. CONCLUSIONS: Patients recovering from COVID-19 who receive primary allied health care make progress in recovery but still experience many limitations in their daily activities after 6 months. Our findings provide reference values to health care providers and health care policy makers regarding what to expect from the recovery of patients who receive health care from 1 or more primary allied health professionals. TRIAL REGISTRATION: ClinicalTrials.gov NCT04735744; https://tinyurl.com/3vf337pn. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): RR2-10.2340/jrm.v54.2506.

Experiences of patients with facioscapulohumeral dystrophy with facial weakness: a qualitative study.

PURPOSE: This study focuses on the functional and psychosocial consequences of facial weakness of patients with facioscapulohumeral muscular dystrophy (FSHD) and how they manage their daily lives. MATERIALS AND METHODS: We conducted a qualitative study. Sixteen FSHD patients with varying degrees of facial weakness were interviewed using a semi-structured interview guide. Data were analyzed using the constant comparison approach based on the Straussian Grounded Theory. RESULTS: Reduced facial expression affected different aspects of a participant's life, which is reinforced by fatigue. Particularly the younger participants described the confrontation with reduced facial expression as upsetting. The unpredictability of the progression of facial weakness makes many participants insecure and concerned. They generally tend to avoid discussing facial weakness with loved ones as well as with strangers. CONCLUSIONS: Patients would like the expert teams to shed more light on effective skill training and psychosocial support, especially for the younger patient group. A multidisciplinary approach is needed in addition to programs focusing on the individual aspects of facial weakness. As the experienced psychosocial effect is not commonly equal to the objective degree of facial weakness, we recommend a tailored approach. Finally, these programs should point out the importance of the patient's own ingenuity.Implications for RehabilitationFacial weakness affects both activities and social participation in patients with facioscapulohumeral muscular dystrophy (FSHD), which is reinforced by fatigue.Many participants try to stay down to earth and focus on their ability to self-manage their obstacles regarding facial weakness.Thus, future treatment programs should have a multidisciplinary approach and should point out the importance of the patient's own ingenuity.

Evaluation of Allied Healthcare in Patients Recovering from Covid-19: Study Protocol and Baseline Data of s National Prospective Cohort Study.

OBJECTIVE: To report the study protocol and baseline characteristics of a prospective cohort study to evaluate longitudinal recovery trajectories of patients recovering from COVID-19 who have visited a primary care allied health professional. DESIGN: Report of the protocol and baseline characteristics for a prospective cohort study with a mixed-methods approach. PATIENTS: Patients recovering from COVID-19 treated by primary care dietitians, exercise therapists, occupational therapists, physical therapists and/or speech and language therapists in the Netherlands. METHODS: The prospective study will measure primary outcome domains: participation, health-related quality of life, fatigue, physical functioning, and costs, at baseline, 3, 6, 9 and 12 months. Interviews, on the patients' experiences with allied healthcare, will be held with a subsample of patients and allied health professionals. RESULTS: The cohort comprises 1,451 patients (57% female, mean age 49 (standard deviation 13) years). Preliminary results for the study cohort show that 974 (67%) of the participants reported mild/moderate severity symptoms during the infection period and patients reported severe restrictions in activities of daily living compared with previous research in other patient populations. Both quantitative and qualitative, will provide insight into the recovery of patients who are treated by allied health professionals. CONCLUSION: In conclusion, this will be the first comprehensive study to longitudinally evaluate the recovery trajectories and related costs of patients recovering from COVID-19 who are treated by allied health professionals in the Netherlands. This study will provide evidence for the optimal strategy to treat patients recovering from COVID-19 infection, including which patients benefit, and to what extent, from treatment, and which factors might impact their recovery course over time. The preliminary results of this study demonstrated the severity of restrictions and complaints at the start of therapy are substantial.

Living with myotonic dystrophy; what can be learned from couples? A qualitative study.

BACKGROUND: Myotonic dystrophy type 1 (MD1) is one of the most prevalent neuromuscular diseases, yet very little is known about how MD1 affects the lives of couples and how they themselves manage individually and together. To better match health care to their problems, concerns and needs, it is important to understand their perspective of living with this hereditary, systemic disease. METHODS: A qualitative study was carried out with a purposive sample of five middle-aged couples, including three men and two women with MD1 and their partners. Fifteen in-depth interviews with persons with MD1, with their partners and with both of them as a couple took place in the homes of the couples in two cities and three villages in the Netherlands in 2009. RESULTS: People with MD1 associate this progressive, neuromuscular condition with decreasing abilities, describing physical, cognitive and psychosocial barriers to everyday activities and social participation. Partners highlighted the increasing care giving burden, giving directions and using reminders to compensate for the lack of initiative and avoidant behaviour due to MD1. Couples portrayed the dilemmas and frustrations of renegotiating roles and responsibilities; stressing the importance of achieving a balance between individual and shared activities. All participants experienced a lack of understanding from relatives, friends, and society, including health care, leading to withdrawal and isolation. Health care was perceived as fragmentary, with specialists focusing on specific aspects of the disease rather than seeking to understand the implications of the systemic disorder on daily life. CONCLUSIONS: Learning from these couples has resulted in recommendations that challenge the tendency to treat MD1 as a condition with primarily physical impairments. It is vital to listen to couples, to elicit the impact of MD1, as a multisystem disorder that influences every aspect of their life together. Couple management, supporting the self-management skills of both partners is proposed as a way of reducing the mismatch between health services and health needs.

Meet and eat, an interdisciplinary group intervention for patients with myotonic dystrophy about healthy nutrition, meal preparation, and consumption: a feasibility study.

Purpose: To develop and evaluate an interdisciplinary group intervention for patients with myotonic dystrophy regarding healthy nutrition, meal preparation, and consumption, called Meet and Eat.Materials and methods: A design-based approach was used, exploring experiences and needs of patients with myotonic dystrophy and their next of kin. This resulted in a 4-week interdisciplinary outpatient group intervention to increase awareness and change behavior. In a mixed-method feasibility study this intervention was evaluated with participants and facilitators.Results: Participants reported on social impact of difficulties with eating and drinking (speaking, swallowing, and social embarrassment) and a variety of complaints affecting meal preparation and consumption. Patients and family members expressed the wish to learn from others. After delivery of the intervention, self-reported outcome measures demonstrated improvement on eating and drinking issues in daily life. The qualitative evaluation showed increased awareness and appreciation of the following design principles: peer support, multidisciplinary approach, active involvement of patient with myotonic dystrophy and their next of kin by using personal goals and motivational interviewing.Conclusion: Participation in Meet and Eat seemed to increase insight in patients' condition and ways of management. However, a longer self-management life style program is recommended to change behavior.Implications for rehabilitationPatients with myotonic dystrophy experience physical, cognitive and psychosocial problems to manage healthy nutrition, meal preparation, and consumption;This multidisciplinary group intervention Meet and Eat in patients with myotonic dystrophy appears to be feasible and seems to result in increased insight and management strategies;Peer support, multidisciplinary approach, personal goals, and involvement of family were key factors;To change eating and drinking behavior, a 4-week intervention is too short.

Reflections of patients and therapists on a multidisciplinary rehabilitation programme for persons with brachial plexus injuries.

PURPOSE: There is lack of knowledge, evidence, and guidelines for rehabilitation interventions for persons with neuralgic amyotrophy (NA) or brachial plexus pathology. A first pilot study, evaluating the effect of an integrated rehabilitation programme, showed improvements in activity and participation levels. AIM: To gain insight, from the perspective of patients and therapists, into the critical ingredients of the programme, that contributed to improvements in activity and participation. MATERIALS AND METHODS: A qualitative study using semi-structured interviews with eight patients and five therapists (three occupational therapists and two physical therapists). Participants were asked to identify and describe factors regarding the rehabilitation that they perceived as positive and aspects of the programme that could be improved. Data were analysed using a constant comparative approach. RESULTS: Patients reported (1) Time to diagnose: "Finally I'm in the right place;" (2) Awareness: "They gave me a mirror;" (3) Partnership: "There was real contact with the therapists; we made decisions together;" (4) Close collaboration: "Overlapping scopes of practice; doing the same from a different perspective;" and finally (5) Self-management: "Now I can do it myself." Therapists reported (1) "Patients knowledge and understanding is critical to success;" (2) "Activate problem solving and decision making;" (3) "Personalize your therapy; it's more than just giving exercises and information;" (4) "Constant consultation within the team; consistency in messages and approach;" and (5)" Ultimately the patient is in charge." CONCLUSIONS: The critical ingredients, correspond well with each other and include a person-centred approach, education, support in problem solving and decision making and an integrated team approach. These ingredients provided the patients with confidence to take responsibility to manage their everyday lives, the ultimate goal of the programme. Implications for rehabilitation Both patients and therapists believe that the ability to self-manage and take control should be the outcome of high quality integrated rehabilitation programmes for patients with neuralgic amyotrophy and/or other brachial plexus injuries. A person-centred, collaborative, and integrated team approach, among all members of the team, are critical components of care delivery in personalised interventions. Critical programme ingredients are knowledge and education of both the patient and therapists; partnership between patient-therapist and within the team; patient activation and self-reflection; and personalised care. Patients recommend more options for personalisation of the intensity and duration of rehabilitation, the possibility to consult a psychologist and peer support within a group setting.

Self-management program improves participation in patients with neuromuscular disease: A randomized controlled trial.

OBJECTIVE: To investigate the effectiveness of Energetic, a self-management group program combining aerobic training, energy conservation management, and relapse prevention to improve social participation in patients with neuromuscular disease (NMD) and chronic fatigue. METHODS: In this multicenter, assessor-blinded, 2-armed randomized controlled trial with repeated measurements, 53 patients with various types of NMD and chronic fatigue were randomly allocated to Energetic, a 4-month group intervention, or to usual care. The primary endpoint was social participation assessed with the Canadian Occupational Performance Measure (COPM) performance scale immediately postintervention. Secondary outcomes included COPM satisfaction scale, 6-Minute Walk Test (6MWT), and Checklist Individual Strength-subscale fatigue. Participants were followed for 11 months postintervention. Data were analyzed with linear models that account for repeated measurements. RESULTS: Directly after intervention, the mean group difference for COPM-performance was 1.7 (95% confidence interval [CI] 1.0-2.4; p < 0.0001) in favor of the intervention group (n = 29), adjusted for baseline, sex, diagnosis, and work status. This effect was retained at 11 months follow-up (0.9; 95% CI 0.0-1.7; p = 0.049). The COPM satisfaction scale and 6MWT improved more in the intervention group compared to usual care. After 3 and 11 months follow-up, most beneficial effects on social participation and functional endurance were retained. CONCLUSION: Energetic led to sustainable improvements in social participation and functional endurance compared to usual care in patients with NMD and chronic fatigue.Clinicaltrials.gov IDENTIFIER: NCT02208687. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that a combination of aerobic training, energy conservation management, and relapse prevention improves social participation in patients with NMD and chronic fatigue.

Development of a tool to guide referral of patients with neuromuscular disorders to allied health services. Part one.

PURPOSE: Reasons for referral of patients with chronic, slowly progressive neuromuscular disorders (NMD) to occupational therapy (OT), physical therapy (PT) and speech therapy (ST) are often unclear. One-off consultations by OT, PT and ST can help patients and physicians to decide if therapy is needed. We present a construct for a questionnaire, the Perceived Limitations in Activities and Needs Questionnaire (PLAN-Q). Its aim is to signal problems and needs of patients with chronic, slowly progressive NMD with a view to referral for one-off consultations by OT, PT and ST. We report on the construct, item pool, response options and the item reduction procedure as the first development stages for the PLAN-Q. METHODS: The International Classification of Functioning, Disability and Health (ICF) served as source for items and response options. Five clinical health professionals of the Neuromuscular Center Nijmegen (NMCN) formed an expert panel. They were asked to reach consensus on construct, item pool and response options. Subsequently a sample of patients with NMD (n=21) that attended the NMCN was asked for their approval of the concept questionnaire. Data of a large cohort (n=208) of patients with a broad variety of chronic NMD were used to carry out item reduction. RESULTS: The expert panel reached consensus on the selection of 56 items covering eight activity categories relevant to OT, PT, and ST. They also reached consensus on response options regarding 'capacity' and 'need for therapy or advice'. The patient sample approved the concept PLAN-Q. The item reduction procedure resulted in a reduction to 25 items covering eight activity categories. CONCLUSION: The PLAN-Q has proceeded through the first stages of development and is now ready for further assessment of reliability and validity.

Development of a tool to guide referral of patients with neuromuscular disorders to allied health services. Part two.

PURPOSE: The Perceived Limitations and Needs Questionnaire (PLAN-Q) was developed to guide referral for a one-off consultation by occupational therapy (OT), physical therapy (PT), and speech therapy (ST) consultation, to provide a tailor-made advice on allied health interventions. This article reports on the testing of validity and reliability of the PLAN-Q. METHODS: In the validation study, 208 patients with a broad spectrum of neuromuscular disorders completed the PLAN-Q, Medical Outcome Study short-form 36-item version (SF-36), and the Impact of Participation on Autonomy (IPA) questionnaires. A subsection of 51 patients, whose physical condition was stable, participated in the evaluation of the intra-rater reliability of the questionnaire. The theoretical construct was tested with factorial analysis, subscales were constructed and reliability and validity of the PLAN-Q subscales were assessed. RESULTS: Factorial analysis resulted in an 18-item self-report questionnaire. Items were grouped into four subscales ('physical capacity', 'transferring', 'oropharyngeal capacity', and 'hand-use'), each with two dimensions ('capacity' and 'need for help'). The internal consistency of all subscales was good (Cronbach's alpha: 0.77-0.94) as well as the intra-rater reliability of the subscales 'physical capacity' and 'transferring' in the 'capacity' dimension (Kappa: 0.70-0.75). The 'need' dimension showed poor intra-rater reliability suggesting that 'need for help' is a variable phenomenon that changes between two points of measurement. The construct validity of the subscales against the SF-36 and the IPA was satisfactory. CONCLUSION: The PLAN-Q is a valid self-report instrument that measures patients' perception of capacity and needs in domains relevant to referral for a one-off OT, PT, or ST consultation.