Parents' perspectives on nusinersen treatment for children with spinal muscular atrophy.

AIM: To gain insight into parents' perspectives about their decision-making process concerning nusinersen treatment for their child, including perceived needs and concerns, and to explore factors that influence this process. METHOD: This was an exploratory qualitative interview study among parents of children with spinal muscular atrophy types 1 to 3. Data were analysed using inductive thematic analysis. RESULTS: Nineteen parents of 16 children representing 13 families participated. A wide variety of perspectives was reported ranging from a biomedical approach, which focused on battling the disease, to a holistic approach, which aimed for a good quality of life for their child. The most important factors that helped parents to decide were honest and neutral communication with their physician and access to available information. INTERPRETATION: It is important physicians understand that there are different perspectives influencing the decision-making process. Physicians should create an environment that allows parents to accept or reject treatment by communicating honestly and openly with them and by discussing both options extensively. Clear information about pros and cons, recent developments in research, and the experiences of other parents should be made available to enable parents to make an informed decision. What this paper adds Parents perceived different needs and concerns about nusinersen treatment, which emphasized individual differences. Parents' perspectives varied from battling the disease to preserving quality of life. Life expectancy, stopping deterioration, and improving quality of life were the perceived benefits of nusinersen treatment. Open communication about the pros and cons of treatment with clinicians facilitated decision-making. Clear and honest information facilitated the alignment of values and goals.

Development and assessment of the inter-rater and intra-rater reproducibility of a self-administration version of the ALSFRS-R.

OBJECTIVE: The Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) is widely applied to assess disease severity and progression in patients with motor neuron disease (MND). The objective of the study is to assess the inter-rater and intra-rater reproducibility, i.e., the inter-rater and intra-rater reliability and agreement, of a self-administration version of the ALSFRS-R for use in apps, online platforms, clinical care and trials. METHODS: The self-administration version of the ALSFRS-R was developed based on both patient and expert feedback. To assess the inter-rater reproducibility, 59 patients with MND filled out the ALSFRS-R online and were subsequently assessed on the ALSFRS-R by three raters. To assess the intra-rater reproducibility, patients were invited on two occasions to complete the ALSFRS-R online. Reliability was assessed with intraclass correlation coefficients, agreement was assessed with Bland-Altman plots and paired samples t-tests, and internal consistency was examined with Cronbach's coefficient alpha. RESULTS: The self-administration version of the ALSFRS-R demonstrated excellent inter-rater and intra-rater reliability. The assessment of inter-rater agreement demonstrated small systematic differences between patients and raters and acceptable limits of agreement. The assessment of intra-rater agreement demonstrated no systematic changes between time points; limits of agreement were 4.3 points for the total score and ranged from 1.6 to 2.4 points for the domain scores. Coefficient alpha values were acceptable. DISCUSSION: The self-administration version of the ALSFRS-R demonstrates high reproducibility and can be used in apps and online portals for both individual comparisons, facilitating the management of clinical care and group comparisons in clinical trials.

Performance and Self-reported Functioning of People With Chronic Idiopathic Axonal Polyneuropathy: A 4-Year Follow-up Study.

OBJECTIVE: To describe the course of performance of activities (observed and self-reported) of people with chronic idiopathic axonal polyneuropathy (CIAP) over 4 years and to assess the associations with muscle strength, sensory function, and psychological personal factors (intention, perceived behavior control [PBC], and feelings of depression or anxiety). DESIGN: Prospective observational study with measurement at baseline, 6 months, 1 year, and 4 years. SETTING: Outpatient neurology clinic. PARTICIPANTS: People with CIAP (N=92). MAIN OUTCOME MEASURES: Walking was measured using the shuttle-walk test (SWT), a pedometer (mean step count/d), and the "physical functioning" subscale of the Short Form-36 questionnaire. Muscle strength and sensory function were measured using a MicroFET handheld dynamometer and the Sensory Modality Sum score. Personal factors were assessed with the Hospital Anxiety and Depression Scale, and intention and PBC were assessed with a protocolized questionnaire. RESULTS: Multilevel model analysis showed a significant decrease over time in mean scores in performance of activities (SWT, step count), which was associated with older age and loss of muscle strength (SWT: ß=73.392, step count: ß=676.279, P<.001). Limitations in self-reported functioning (physical functioning) significantly increased and were associated with older age (ß=-0.916, P=.001), increased comorbidity (ß=-6.978, P=.024), loss of muscle strength (ß=7.074, P<.001), low PBC (ß=0.744, P<.001), and increased feelings of depression (ß=1.481, P<.001). CONCLUSIONS: Performance of activities of people with CIAP decreased over time (SWT, step count, physical functioning). Older age, loss of muscle strength, comorbidity, feelings of depression, and low perceived behavior control were associated with this decrease. However, there were considerable individual differences.

Psychological distress in partners of patients with amyotrophic lateral sclerosis and progressive muscular atrophy: what's the role of care demands and perceived control?

Caregivers of Amyotrophic Lateral Sclerosis (ALS) and Progressive Muscular Atrophy (PMA) patients often experience psychological distress. Yet, it is unclear which factors explain the variance in psychological distress. This study seeks to evaluate how care demands and perceived control over caregiving influence psychological distress using moderation and mediation analysis. Data were collected as part of a RCT and 148 partners of patients with ALS or PMA were included. Psychological distress was assessed using the Hospital Anxiety and Depression Scale. Care demands were operationalized as physical functioning (Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised) and behavioural changes of the patient (Amyotrophic Lateral Sclerosis-Frontotemporal Dementia-Questionnaire). Perceived control over caregiving was assessed using items adapted from the Job Content Questionnaire. Results showed that more behavioural changes and lower perceived control over caregiving were associated with higher levels of psychological distress in caregivers. Patients' physical functioning was not significantly related to caregivers' psychological distress. No moderation or mediation effects were found of perceived control over caregiving on the relationship between demand and psychological distress. Monitoring, psychoeducation and caregiver support with regard to behavioural changes in patients, seem to be important for the wellbeing of caregivers. Caregivers' perceived control might be a target for future interventions.

Cognitive and behavioural changes in PLS and PMA:challenging the concept of restricted phenotypes.

OBJECTIVES: Cognitive and behavioural changes within the spectrum of frontotemporal dementia (FTD) are observed frequently in patients with amyotrophic lateral sclerosis (ALS). Whether these changes also occur in other forms of motor neuron disease (MND) is not well studied. We therefore systemically screened a large cohort of patients with primary lateral sclerosis (PLS) and progressive muscular atrophy (PMA) for cognitive and behavioural changes, and subsequently compared our findings with a cohort of patients with ALS. METHODS: Using a set of screening instruments (Edinburgh Cognitive and Behavioural ALS Screen, ALS and Frontotemporal Dementia Questionnaire, Frontal Assessment Battery, and Hospital Anxiety and Depression Scale), the presence of cognitive and behavioural changes as well as anxiety and depression in 277 patients with ALS, 75 patients with PLS and 143 patients with PMA was evaluated retrospectively. RESULTS: We found a high frequency of cognitive and behavioural abnormalities with similar profiles in all three groups. Subjects with behavioural variant FTD were identified in all groups. CONCLUSIONS: The percentage of patients with PLS and PMA with cognitive dysfunction was similar to patients with ALS, emphasising the importance for cognitive screening as part of routine clinical care in all three patient groups. With a similar cognitive profile, in line with genetic and clinical overlap between the MNDs, the view of PLS as an MND exclusively affecting upper motor neurons and PMA exclusively affecting lower motor neurons cannot be held. Therefore, our findings are in contrast to the recently revised El Escorial criteria of 2015, where PLS and PMA are described as restricted phenotypes. Our study favours a view of PLS and PMA as multidomain diseases similar to ALS.

Support needs of caregivers of patients with amyotrophic lateral sclerosis: A qualitative study.

OBJECTIVE: The aim of this study was to explore the support needs of Dutch informal caregivers of patients with amyotrophic lateral sclerosis (ALS). METHOD: Individual semi-structured interviews were conducted with 21 caregivers of ALS patients. Audio-taped interviews were transcribed and data were analyzed thematically. RESULT: A total of four global support needs emerged: "more personal time", "assistance in applying for resources", "counseling", and "peer contact". Despite their needs, caregivers are reluctant to apply for and accept support. They saw their own needs as secondary to the needs of the patients. SIGNIFICANCE OF RESULTS: ALS seems to lead to an intensive caregiving situation with multiple needs emerging in a short period. This study offers targets for the development of supportive interventions. A proactive approach seems essential, acknowledging the importance of the role of the caregivers in the care process at an early stage, informing them about the risk of burden, monitoring their wellbeing, and repeatedly offering support opportunities. Using e-health may help tailor interventions to the caregivers' support needs.

Caregiver burden in amyotrophic lateral sclerosis: A systematic review.

BACKGROUND: Informal caregivers of patients with amyotrophic lateral sclerosis experience increased levels of caregiver burden as the disease progresses. Insight in the factors related to caregiver burden is needed in order to develop supportive interventions. AIM: To evaluate the evidence on patient and caregiver factors associated with caregiver burden in amyotrophic lateral sclerosis informal caregivers. DESIGN: A systematic review. DATA SOURCES: Four electronic databases were searched up to 2017. Studies that investigated quantitative relations between patient or caregiver factors and caregiver burden were included. The overall quality of evidence for factors was assessed using the Grading of Recommendations Assessment, Development and Evaluation approach. RESULTS: A total of 25 articles were included. High quality of evidence was found for the relation between caregiver burden and the factor "behavioral impairments." Moderate quality of evidence was found for the relations between caregiver burden and the factors "feelings of depression" of the caregiver and "physical functioning" of the patient. The remaining rated caregiver factors-"feelings of anxiety," "distress," "social support," "family functioning," and "age"-and patient factors-"bulbar function," "motor function," "respiratory function," "disease duration," "disinhibition," "executive functioning," "cognitive functioning," "feelings of depression," and "age"-showed low to very low quality of evidence for their association with caregiver burden. CONCLUSION: Higher caregiver burden is associated with greater behavioral and physical impairment of the patient and with more depressive feelings of the caregiver. This knowledge enables the identification of caregivers at risk for caregiver burden and guides the development of interventions to diminish caregiver burden.

Nurses' perceptions towards the delivery and feasibility of a behaviour change intervention to enhance physical activity in patients at risk for cardiovascular disease in primary care: a qualitative study.

BACKGROUND: Self-management support is widely accepted for the management of chronic conditions. Self-management often requires behaviour change in patients, in which primary care nurses play a pivotal role. To support patients in changing their behaviour, the structured behaviour change Activate intervention was developed. This intervention aims to enhance physical activity in patients at risk for cardiovascular disease in primary care as well as to enhance nurses' role in supporting these patients. This study aimed to evaluate nurses' perceptions towards the delivery and feasibility of the Activate intervention. METHODS: A qualitative study nested within a cluster-randomised controlled trial using semistructured interviews was conducted and thematically analysed. Fourteen nurses who delivered the Activate intervention participated. RESULTS: Three key themes emerged concerning nurses' perceptions of delivering the intervention: nurses' engagement towards delivering the intervention; acquiring knowledge and skills; and dealing with adherence to the consultation structure. Three key themes were identified concerning the feasibility of the intervention: expectations towards the use of the intervention in routine practice; perceptions towards the feasibility of the training programme; and enabling personal development. CONCLUSIONS: Delivering a behaviour change intervention is challenged by the complexity of changing nurses' consultation style, including acquiring corresponding knowledge and skills. The findings have increased the understanding of the effectiveness of the Activate trial and will guide the development and evaluation of future behaviour change interventions delivered by nurses in primary care. TRIAL REGISTRATION: ClinicalTrials.gov NCT02725203 .

Participation restrictions in ambulatory amyotrophic lateral sclerosis patients: Physical and psychological factors.

INTRODUCTION: The aim of this study was to assess the prevalence of participation restrictions in ambulatory patients with amyotrophic lateral sclerosis (ALS) and to identify physical and psychological contributory factors. METHODS: In this cross-sectional study, self-reported participation restrictions of 72 ambulatory ALS patients were assessed using the social health status dimension (SIPSOC) of the Sickness Impact Profile (SIP-68). Associations between SIPSOC and physical functioning, psychological factors, and demographic factors were analyzed using hierarchical regression analyses. RESULTS: Ninety-two percent of the patients reported participation restrictions; 54.9% could be explained by physical functioning; psychological factors accounted for 8.1% of the variance. Lung capacity, functional mobility, fatigue, and helplessness were independently associated with participation restrictions. CONCLUSIONS: Ambulatory ALS patients have participation restrictions, which may be influenced if early ALS care is directed toward lung capacity, functional mobility, fatigue, and feelings of helplessness. Muscle Nerve 56: 912-918, 2017.

Associations between psychological factors and health-related quality of life and global quality of life in patients with ALS: a systematic review.

OBJECTIVE: To systematically identify and appraise evidence on associations between psychological factors (moods, beliefs, personality) and Health-related QoL (HRQoL) and/or global QoL in patients with Amyotrophic Lateral Sclerosis (ALS). METHODS: A systematic review was conducted in several online databases (PsycINFO, EMBASE, PubMed and CINAHL) up to October 2015. Articles were included if they reported associations between psychological factors (moods, beliefs and personality) and HRQoL and/or global QoL in an ALS population. The search was limited to empirical studies, published in English, which provided quantitative data. The methodological quality of the included articles was assessed. RESULTS: In total, 22 studies were included. Mood was investigated in 14 studies, beliefs in 11 studies and personality in one study. Fifteen different psychological factors were extracted and assessed using 24 different measures. Twelve different QoL measures were used in the selected studies, subdivided into seven different HRQoL measures and five different global QoL measures. Higher levels of anxiety and depression appeared to be related to a poorer HRQoL, whereas a higher level of religiosity seemed to be associated with better global QoL. No conclusive associations were found for confusion-bewilderment (mood), spirituality, mindfulness, coping styles, hopelessness, perception of burden, cognitive appraisal (beliefs), neuroticism, extraversion, openness, agreeableness and conscientiousness (personality), due to insufficient or inconsistent evidence. Religiosity and spirituality appeared to become more positively associated over time. CONCLUSIONS: Our results suggest that higher levels of anxiety and depression are related to a poorer HRQoL, whereas higher levels of religiosity appeared to be related to better global QoL. Associations might change during the disease course. This review supports the importance of psychological factors with regard to ALS care. Further research is needed to supplement the available evidence and to investigate how psychological factors can be modified to improve QoL. REVIEW REGISTRATION NUMBER: PROSPERO 2015:CRD42015027303.

Measurement of clinically relevant functional health perceptions in patients with whiplash-associated disorders: the development of the whiplash specific activity and participation list (WAL).

PURPOSE: The International Classification of Human Functioning Disability and Health (ICF) provides insight into functional health status in patients with whiplash-associated disorders (WAD). In the assessment of functional limitations in patients with WAD, there are several condition-specific questionnaires available. Estimation of the true relationship between the separate constructs of the ICF is only possible if the items of the salient questionnaires measure exactly the constructs of interest, while not simultaneously measuring other constructs of the model. This study aimed to develop a condition specific and clinically relevant and usable instrument for patients with WAD that measures activity limitations and participation restrictions, as defined by the ICF framework. METHODS: Item generation consisted of (1) a semi-structured interview which was conducted among 69 WAD patients; (2) a Delphi study involving 13 health professionals experienced in the assessment of patients with WAD; (3) a literature search for items from self-assessment questionnaires for neck pain. RESULTS: A 35-item condition-specific self-assessment questionnaire for patients with WAD was developed. This new questionnaire measures purely activity limitations and participation restrictions according to the ICF and is based on patients' opinions and expert opinions. CONCLUSION: The whiplash activity and participation list tends to measure clinically relevant activity limitations and participation restrictions in WAD patients.

Health literacy and pain neuroscience education in an interdisciplinary pain management programme: a qualitative study of patient perspectives.

Introduction: Pain neuroscience education is part of interdisciplinary pain management programs (IPMPs). To date, the role of health literacy on patients' understanding of pain neuroscience education has not sufficiently been examined. Objectives: Drawing on interviews with patients with diverse levels of health literacy, this article explores patient perspectives on pain neuroscience education. Methods: Purposively sampled patients from an IPMP were interviewed twice (waiting list and after 4 weeks). A directed qualitative content analysis was performed with the Integrated Conceptual Model of Health Literacy as an analytic framework. Results: Thirteen patients with chronic musculoskeletal pain were interviewed: 4 men and 9 women aged from 21 to 77 years with diverse educational and mostly low health literacy. One participant dropped out after baseline. Some participants gained access to health information actively; others relied on the expertise of their healthcare providers. Most participants did not seem to receive the information in the pain neuroscience education as intended, experienced difficulties with understanding the message, negatively appraised the information, and were not able to apply this in their daily lives. Health literacy levels likely played a role in this. Conclusions: Pain neuroscience education tailored to patients' health literacy levels, information needs, and learning strategies is needed.

The clinical decision-making process of healthcare professionals within a personalized home-based rehabilitation program during sequential chemoradiotherapy for stage III non-small lung cancer: A case study.

INTRODUCTION: The purpose of this case study was to demonstrate the clinical decision-making process of healthcare professionals within a rehabilitation program during chemoradiotherapy (CHRT) for a high-risk patient diagnosed with stage III non-small cell lung cancer (NSCLC). The course of CHRT and patient's preferences, facilitators, and barriers were considered. CASE-DESCRIPTION: The patient was a 69-year-old man with a history of rheumatoid arthritis diagnosed with stage III NSCLC. INTERVENTION: A home-based, personalized, and partly supervised rehabilitation program during CHRT, including aerobic, resistance, and breathing exercises, as well as nutritional counseling. OUTCOMES: The patient suffered from side effects of CHRT, which required adjustments in the context and intensity of the exercises. An important facilitator for the patient was encouraged by his wife in following the home-based rehabilitation program. During home visits, the patient and physiotherapists performed the exercises together to help him to overcome the burden and motivate the patient to adhere to the rehabilitation program. CONCLUSION: This case study demonstrates that physical exercise training could be performed by adjusting training intensity and the way in which the physical exercise training was delivered, while the patient experienced side effects from CHRT. In addition, the involvement and support of (in)formal caregivers seems essential for adherence to rehabilitation.

Effects of aerobic exercise therapy and cognitive behavioural therapy on functioning and quality of life in amyotrophic lateral sclerosis: protocol of the FACTS-2-ALS trial.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder affecting motor neurons in the spinal cord, brainstem and motor cortex, leading to muscle weakness. Muscle weakness may result in the avoidance of physical activity, which exacerbates disuse weakness and cardiovascular deconditioning. The impact of the grave prognosis may result in depressive symptoms and hopelessness. Since there is no cure for ALS, optimal treatment is based on symptom management and preservation of quality of life (QoL), provided in a multidisciplinary setting. Two distinctly different therapeutic interventions may be effective to improve or preserve daily functioning and QoL at the highest achievable level: aerobic exercise therapy (AET) to maintain or enhance functional capacity and cognitive behavioural therapy (CBT) to improve coping style and cognitions in patients with ALS. However, evidence to support either approach is still insufficient, and the underlying mechanisms of the approaches remain poorly understood. The primary aim of the FACTS-2-ALS trial is to study the effects of AET and CBT, in addition to usual care, compared to usual care alone, on functioning and QoL in patients with ALS. METHODS/DESIGN: A multicentre, single-blinded, randomized controlled trial with a postponed information model will be conducted. A sample of 120 patients with ALS (1 month post diagnosis) will be recruited from 3 university hospitals and 1 rehabilitation centre. Patients will be randomized to one of three groups i.e. (1) AET + usual care, (2) CBT + usual care, (3) Usual care. AET consists of a 16-week aerobic exercise programme, on 3 days a week. CBT consists of individual psychological support of patients in 5 to 10 sessions over a 16-week period. QoL, functioning and secondary outcome measures will be assessed at baseline, immediately post intervention and at 3- and 6-months follow-up. DISCUSSION: The FACTS-2-ALS study is the first theory-based randomized controlled trial to evaluate the effects, and the maintenance of effects, of AET and CBT on functioning and QoL in patients with ALS. The results of this study are expected to generate new evidence for the effect of multidisciplinary care of persons with ALS. TRIAL REGISTRATION: Dutch Trial Register NTR1616.

Fidelity of primary care nurses' delivery of a behavioural change intervention enhancing physical activity in patients at risk of cardiovascular disease: an observational study.

OBJECTIVE: To evaluate the fidelity of delivery of a nurse-led intervention to enhance physical activity in patients at risk for cardiovascular diseases, the Activate intervention, by assessing: (1) self-reported fidelity of delivery; (2) observed fidelity of delivery; (3) quality of delivery of the Activate intervention and (4) nurses' beliefs about their capability, motivation, confidence and effectiveness towards delivering the Activate intervention, including behavioural change techniques. DESIGN: An observational study. SETTING: General practices in the Netherlands. PARTICIPANTS: Primary care nurses (n=20) from 16 general practices. PRIMARY AND SECONDARY OUTCOME MEASURES: Nurses' self-reported fidelity was evaluated using checklists (n=282), and the observed fidelity and quality of delivery were examined using audiorecordings of consultations of the delivery of the Activate intervention (n=42). Nurses' beliefs towards delivering the intervention were assessed using questionnaires (n=72). RESULTS: The self-reported fidelity was 88.1% and observed fidelity was 85.4%, representing high fidelity. The observed fidelity of applied behavioural change techniques was moderate (75.0%). The observed quality of delivery was sufficient and varied among nurses (mean 2.9; SD 4.4; range 0-4). Nurses' beliefs about their capability, motivation, confidence and effectiveness towards delivering the intervention increased over time. CONCLUSIONS: Nurses delivered most intervention components as intended with sufficient quality. Nurses believed they were capable, motivated and confident to deliver the intervention. They believed the intervention was effective to increase patients' physical activity level. Despite the high fidelity and moderate fidelity of applied behavioural change techniques, the varying quality of delivery within and across nurses might have diluted the effectiveness of the Activate intervention. TRIAL REGISTRATION NUMBER: NCT02725203.

Physical Activity during Cancer Treatment (PACT) Study: design of a randomised clinical trial.

BACKGROUND: Fatigue is a major problem of cancer patients. Thirty percent of cancer survivors report serious fatigue three years after finishing treatment. There is evidence that physical exercise during cancer treatment reduces fatigue. This may also lead to an improvement of quality of life. Such findings may result in a decrease of healthcare related expenditures and societal costs due to sick leave. However, no studies are known that investigated these hypotheses. Therefore, the primary aim of our study is to assess the effect of exercise during cancer treatment on reducing complaints of fatigue and on reducing health service utilisation and sick leave. METHODS/DESIGN: The Physical Activity during Cancer Treatment study is a multicentre randomised controlled trial in 150 breast and 150 colon cancer patients undergoing cancer treatment. Participants will be randomised to an exercise or a control group. In addition to the usual care, the exercise group will participate in an 18-week supervised group exercise programme. The control group will be asked to maintain their habitual physical activity pattern. Study endpoints will be assessed after 18 weeks (short term) and after 9 months (long term). Validated questionnaires will be used. PRIMARY OUTCOME: fatigue (Multidimensional Fatigue Inventory and Fatigue Quality List) and cost-effectiveness, health service utilisation and sick leave. Secondary outcome: health related quality of life (European Organisation Research and Treatment of Cancer-Quality of Life questionnaire-C30, Short Form 36 healthy survey), impact on functioning and autonomy (Impact on functioning and autonomy questionnaire), anxiety and depression (Hospital Anxiety and Depression Scale), physical fitness (aerobic peak capacity, muscle strength), body composition and cognitive-behavioural aspects. To register health service utilisation and sick leave, participants will keep diaries including the EuroQuol-5D. Physical activity level will be measured using the Short Questionnaire to Assess Health-Enhancing Physical Activity and will be monitored with an exercise log and a pedometer. DISCUSSION: This study investigates the (cost)-effectiveness of exercise during adjuvant treatment of patients with breast or colon cancer. If early physical exercise proves to be (cost) effective, establishing standardised physical exercise programmes during cancer treatment will be planned. TRIAL REGISTRATION: Current Controlled trials ISRCTN43801571, Dutch Trial Register NTR2138.

Blended psychosocial support for partners of patients with ALS and PMA: results of a randomized controlled trial.

Objective: To evaluate whether a blended (face-to-face and online) psychosocial support program for caregivers of patients with Amyotrophic Lateral Sclerosis (ALS) and Progressive Muscular Atrophy (PMA), aimed at enhancing feeling of control over caregiving, reduces psychological distress. Methods: A randomized controlled trial using a wait-list control design was conducted. Caregiver-patient dyads were randomly assigned to either the support program (n = 74) or to a wait-list control group (n = 74). The support program, based on Acceptance and Commitment Therapy, consists of 1 face-to-face contact, 6 online guided modules and 1 telephone contact. Participants filled in questionnaires at baseline, 3 and 6 months. Caregivers' feeling of control over caregiving was assessed using two self-efficacy measures. Primary outcome was caregivers' psychological distress. Secondary outcomes included caregiver burden, caregiver quality of life and patients' quality of life and psychological distress. Intention-to-treat and per-protocol analyses were performed using linear mixed models. Results: The support program had no effect on the primary or secondary outcomes, despite a significant positive effect on the intervening variable self-efficacy with regard to control over thoughts. Almost half of the caregivers did not complete the intervention with the most frequently reported reason being lack of time. Caregivers who completed the intervention evaluated the support program positively. Conclusions: The support program did not reduce distress of partners of patients with ALS/PMA but may be beneficial by increasing feeling of control over the caregiving situation. The high level of intervention drop outs may have limited the ability to detect an intervention effect.Trial registration: Netherlands Trial Registry NTR5734, registered 28 March 2016.

Effectiveness of the nurse-led Activate intervention in patients at risk of cardiovascular disease in primary care: a cluster-randomised controlled trial.

BACKGROUND: To understand better the success of self-management interventions and to enable tailoring of such interventions at specific subgroups of patients, the nurse-led Activate intervention is developed targeting one component of self-management (physical activity) in a heterogeneous subgroup (patients at risk of cardiovascular disease) in Dutch primary care. AIM: The aim of this study was to evaluate the effectiveness of the Activate intervention and identifying which patient-related characteristics modify the effect. METHODS: A two-armed cluster-randomised controlled trial was conducted comparing the intervention with care as usual. The intervention consisted of four nurse-led behaviour change consultations within a 3-month period. Data were collected at baseline, 3 months and 6 months. Primary outcome was the daily amount of moderate to vigorous physical activity at 6 months. Secondary outcomes included sedentary behaviour, self-efficacy for physical activity, patient activation for self-management and health status. Prespecified effect modifiers were age, body mass index, level of education, social support, depression, patient provider relationship and baseline physical activity. RESULTS: Thirty-one general practices (n = 195 patients) were included (intervention group n = 93; control group n = 102). No significant between-group difference was found for physical activity (mean difference 2.49 minutes; 95% confidence interval -2.1; 7.1; P = 0.28) and secondary outcomes. Patients with low perceived social support (P = 0.01) and patients with a low baseline activity level (P = 0.02) benefitted more from the intervention. CONCLUSION: The Activate intervention did not improve patients' physical activity and secondary outcomes in primary care patients at risk of cardiovascular disease. To understand the results, the intervention fidelity and active components for effective self-management require further investigation.Trial registration: ClinicalTrials.gov NCT02725203.

Psychological well-being in adults with spinal muscular atrophy: the contribution of participation and psychological needs.

Purpose: Patients with spinal muscular atrophy (SMA) suffer from slowly progressive weakness of axial, respiratory and proximal muscles, leading to restrictions in activity and participation. This study aims to investigate patients' level of psychological well-being, using the International Classification of Functioning model and self-determination theory as theoretical frameworks.Materials and methods: In this cross-sectional study, adults with SMA were invited to complete a questionnaire. Instruments to assess psychological well-being included the Satisfaction with Life Scale, the Rosenberg Self-Esteem Scale and the Positive and Negative Affect Scale. Hierarchical lineal regression analyses were performed to investigate the contribution of participation (International Classification of Functioning model) and satisfaction of the need for autonomy, competence and relatedness (self-determination theory) to well-being.Results: Ninety-two respondents (67%) returned the questionnaire. Levels of psychological well-being were comparable to that of healthy reference samples. Well-being was unrelated to sociodemographic variables or illness characteristics. By contrast, well-being was closely related to respondents' satisfaction with participation, and their sense of autonomy, competence and relatedness.Conclusions: This study illustrates the relevance of psychological needs for understanding well-being of individuals with SMA. Supporting patients in meeting their psychological needs should become an objective of person-centred care for this population.Implications for rehabilitationSpinal muscular atrophy is a rare inherited disease, characterized by slowly progressive muscle weakness.Psychological well-being, including satisfaction with life, self-esteem and emotional functioning of adults with spinal muscular atrophy appears very comparable with that of healthy reference samples.In line with the International Classification of Functioning framework, well-being in adults with spinal muscular atrophy may be improved by increasing their (satisfaction with) participation.Moreover, clinical assessment and management should focus on optimizing patients' satisfaction with their basic psychological needs (autonomy, competence, relatedness), as this is strongly related to indices of psychological well-being.

Patients' experiences with a behaviour change intervention to enhance physical activity in primary care: A mixed methods study.

OBJECTIVE: To explore the experiences of patients at risk for cardiovascular disease in primary care with the Activate intervention in relation to their success in increasing their physical activity. METHODS: A convergent mixed methods study was conducted, parallel to a cluster-randomised controlled trial in primary care, using a questionnaire and semi-structured interviews. Questionnaires from 67 patients were analysed, and semi-structured interviews of 22 patients were thematically analysed. Experiences of patients who had objectively increased their physical activity (responders) were compared to those who had not (non-responders). Objective success was analysed in relation to self-perceived success. RESULTS: The questionnaire and interview data corresponded, and no substantial differences among responders and non-responders emerged. Participating in the intervention increased patients' awareness of their physical activity and their physical activity level. Key components of the intervention were the subsequent support of nurses with whom patients' have a trustful relationship and the use of self-monitoring tools. Patients highly valued jointly setting goals, planning actions, receiving feedback and review on their goal attainment and jointly solving problems. Nurses' support, the use of self-monitoring tools, and involving others incentivised patients to increase their physical activity. Internal circumstances and external circumstances challenged patients' engagement in increasing and maintaining their physical activity. CONCLUSION: Patients experienced the Activate intervention as valuable to increase and maintain their physical activity, irrespective of their objective change in physical activity. The findings enable the understanding of the effectiveness of the intervention and implementation in primary care. TRIAL REGISTRATION: ClinicalTrials.gov NCT02725203.