Control in the absence of choice: A qualitative study on decision-making about gastrostomy in people with amyotrophic lateral sclerosis, caregivers, and healthcare professionals.

Background: Gastrostomy is recommended in amyotrophic lateral sclerosis for long-term nutritional support, however, people with amyotrophic lateral sclerosis and healthcare professionals perceive decision-making as complex.

Method: To explore their perspectives on decision-making regarding gastrostomy, we used semi-structured interviews with people with amyotrophic lateral sclerosis, who had made a decision, and their caregivers; healthcare professionals were interviewed separately. Interviews were transcribed and analyzed thematically.

Discussing Personalized Prognosis Empowers Patients with Amyotrophic Lateral Sclerosis to Regain Control over Their Future: A Qualitative Study.

The ENCALS survival prediction model offers patients with amyotrophic lateral sclerosis (ALS) the opportunity to receive a personalized prognosis of survival at the time of diagnosis. We explored experiences of patients with ALS, caregivers, and physicians with discussing personalized prognosis through interviews with patients and their caregivers, and in a focus group of physicians. Thematic analysis revealed four themes with seven subthemes; these were recognized by the focus group.

Current practices and barriers in gastrostomy indication in amyotrophic lateral sclerosis: a survey of ALS care teams in The Netherlands.

Objective: To describe current practices and barriers and support needs in gastrostomy indication and decision-making amongst rehabilitation physicians of ALS care teams in the Netherlands.

Methods: Cross-sectional online survey of rehabilitation physicians of ALS care teams in the Netherlands. Survey items covered current practices in (i.

Differences in Societal Participation Across Diagnostic Groups: Secondary Analyses of 8 Studies Using the Utrecht Scale for Evaluation of Rehabilitation-Participation.

Objective: To determine differences in participation problems between diagnostic groups and to examine diagnosis as a determinant of participation with and without statistically accounting for confounders.

Design: Secondary analyses of data from 8 studies.

Setting: Community, the Netherlands.

Participation and autonomy in the first 10 months after diagnosis of ALS: a longitudinal study.

More insight is needed into participation in daily activities and autonomy among patients with amyotrophic lateral sclerosis (ALS). Aims of this study were (1) to describe the course of participation restrictions and autonomy in participation during the first 10 months after diagnosis; (2) to study the influence of the rate of ALS progression on the course of participation. : Secondary analysis of data from the longitudinal multicenter FACTS-2-ALS study.

Discussing personalized prognosis in amyotrophic lateral sclerosis: development of a communication guide.

Background: Personalized ENCALS survival prediction model reliably estimates the personalized prognosis of patients with amyotrophic lateral sclerosis. Concerns were raised on discussing personalized prognosis without causing anxiety and destroying hope. Tailoring communication to patient readiness and patient needs mediates the impact of prognostic disclosure.

Using patient-reported symptoms of dyspnea for screening reduced respiratory function in patients with motor neuron diseases.

Background: Poor monitoring of respiratory function may lead to late initiation of non-invasive ventilation (NIV) in patients with motor neuron diseases (MND). Monitoring could be improved by remotely assessing hypoventilation symptoms between clinic visits. We aimed to determine which patient-reported hypoventilation symptoms are best for screening reduced respiratory function in patients with MND, and compared them to the respiratory domain of the amyotrophic lateral sclerosis functional rating scale (ALSFRS-R).

Aerobic Exercise Therapy in Ambulatory Patients With ALS: A Randomized Controlled Trial.

Background: Weakness caused by motor neuron degeneration in amyotrophic lateral sclerosis (ALS) may result in avoidance of physical activity, resulting in deconditioning and reduced health-related quality of life (HRQoL).

Objective: To study the effectiveness of aerobic exercise therapy (AET) on disease-specific and generic HRQoL in ambulatory patients with ALS.

Methods: We conducted a multicenter, assessor-blinded, randomized controlled trial.

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

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. In this cross-sectional study, adults with SMA were invited to complete a questionnaire.

Social participation of adult patients with spinal muscular atrophy: Frequency, restrictions, satisfaction, and correlates.

Introduction: In this study we assessed social participation in 62 adults with spinal muscular atrophy (SMA) types 1c-4.

Methods: The outcome measure used was the Utrecht Scale of Evaluation Rehabilitation-Participation (USER-P) with Frequency, Restrictions, and Satisfaction scores, and a hierarchical regression analysis.

Results: Early-onset (types 1, 2, and 3a) and late-onset (types 3b and 4) SMA patients reported similar frequency and satisfaction scores.

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.

Correlates of health related quality of life in adult patients with spinal muscular atrophy.

Introduction: To improve care for patients with spinal muscular atrophy (SMA), we assessed the physical and mental quality of life (QoL) in 62 adult patients with SMA.

Methods: Physical component scores (PCS) and mental component scores (MCS) of the Short Form-36 Health Survey (SF-36) were obtained. Correlations with demographics, disease severity, and emotional distress were assessed.

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.

[Amyotrophic lateral sclerosis, a heterogeneous disorder].

ALS is a disease characterized by the progressive loss of upper and lower motor neurons leading to weakness and spasticity. Diagnosis of ALS is based on exclusion. ALS and frontotemporal dementia (FTD) constitute the extremes of the spectrum of one disease.

Cognitive behavioural therapy and quality of life in psychologically distressed patients with amyotrophic lateral sclerosis and their caregivers: Results of a prematurely stopped randomized controlled trial.

Our objective was to compare the effects of cognitive behavioural therapy (CBT) and usual care (UC) on quality of life (QoL) in psychologically distressed patients with ALS and their caregivers. We conducted a multicentre randomized controlled trial (RCT). In 16 weeks, patient-carer pairs received five to 10 CBT sessions plus usual care (UC) or UC alone.