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Objectives: In families with a parent diagnosed with amyotrophic lateral sclerosis (ALS), children's adaptation depends among others on how their parents communicate with them about the disease and its trajectory. The aim of this study was to explore parents' and children's perceptions of ALS-related family communication. Methods: A qualitative analysis using a conventional content analysis approach was applied to interview data previously collected from 21 parents (8 with ALS) and 15 children (age 13–23 years) about their experiences living with ALS. Results: Three themes emerged from the interviews: communication topics, styles and timing. Communication topics include facts about disease and prognosis, feelings, care and equipment, and the end. Although most parents perceived the familial communication style concerning ALS as open, the interviews revealed that both parents and children sometimes avoid interactions about ALS, because they do not know what to say or how to open the dialogue, are afraid to burden other family members, or are unwilling to discuss. Communication timing is directed by changes in the disease trajectory and/or questions of children. A family-level analysis showed that ALS-related family communication is sometimes perceived differently by parents and children. Conclusions: The study provides a better understanding of what, how and when parents and children in families living with ALS communicate about the disease. Most families opened the dialogue about ALS yet encountered challenges which may hamper good familial communication. Through addressing those challenges, healthcare professionals may facilitate better communication and adaptation in families with a parent with ALS. [ABSTRACT FROM AUTHOR]

To determine the feasibility, reliability, and sensitivity of remotely monitoring muscle strength loss of knee extensors using a novel portable fixed dynamometer (PFD) in patients with amyotrophic lateral sclerosis (ALS). We conducted a pilot study with a newly developed device to measure knee extension strength. Patients performed unsupervised PFD measurements, biweekly, for 6 months at home. We evaluated feasibility using adherence and a device-specific questionnaire. Reliability was assessed by (1) comparing unsupervised and supervised measurements to identify systematic bias, and (2) comparing consecutive unsupervised measurements to determine test-retest reliability expressed as intraclass correlation coefficient (ICC) and standard error of measurement (SEM). Sensitivity to detect longitudinal change was described using linear mixed-effects models. We enrolled 18 patients with ALS. Adherence was 86%, where all patients found that the device suitable to measure muscle strength at home; 4 patients (24%) found the measurements burdensome. The correlation between (un)supervised measurements was excellent (Pearson's r 0.97, 95%CI; 0.94 − 0.99) and no systematic bias was present (mean difference 0.13, 95%CI; −2.22 − 2.48, p = 0.91). Unsupervised measurements had excellent test-retest reliability with an average ICC of 0.97 (95%CI: 0.94 − 0.99) and SEM of 5.8% (95%CI: 4.8 − 7.0). Muscle strength declined monthly by 1.9 %predicted points (95%CI; −3.0 to −0.9, p = 0.001). Using the PFD, it proved feasible to perform knee extension strength measurements at home which were reliable and sensitive for detecting muscle strength loss. Larger studies are warranted to compare the device with conventional outcomes. [ABSTRACT FROM AUTHOR]

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. Results: In 14 cases, 13 people with amyotrophic lateral sclerosis and 12 caregivers were interviewed; and in 10 of these cases, 5 healthcare professionals. Participants described decision-making on gastrostomy as a continuous process of weighing (future) clinical need against their values and beliefs in coming to a decision to accept or reject gastrostomy, or to postpone decision-making, while being supported by loved ones and healthcare professionals. Participants described gastrostomy as inevitable, but retained agency through control over the timing of decision-making. They said physical necessity, experiences of loss and identity, and expectations about gastrostomy placement were important factors in decision-making. Decision-making was described as a family affair, with caregivers supporting patient choice. healthcare professionals supported people with amyotrophic lateral sclerosis during the decision-making process and respected their autonomy and values. People with amyotrophic lateral sclerosis stressed the importance of adequate information on the procedure and the benefits. Conclusion: People with amyotrophic lateral sclerosis feel in control of decision-making on gastrostomy if they are able to make their own choice at their own pace, supported by loved ones and healthcare professionals. Person-centered decision-making on gastrostomy requires early information exchange and repeated discussions with people with amyotrophic lateral sclerosis and their caregivers, incorporating their values and respecting patient choice. [ABSTRACT FROM AUTHOR]

Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) are inflammatory neuropathies that can lead to considerable limitations in daily activities and in social participation. However, systematic evaluation of these self‐reported limitations is lacking in the currently available studies. Understanding the impact of these diagnoses on patients' life is important to optimize management strategies. Aim: To systematically assess the self‐reported limitations in activities and participation and determine associated factors. Methods: A survey study was conducted in 2021 in a cohort of patients with CIDP (n = 257) and MMN (n = 148) from a university hospital. The survey included the Rasch‐built Overall Disability Scale and the Utrecht Scale for Evaluation of Rehabilitation‐Participation, questions addressing personal and disease‐related factors and treatment. Multivariate linear regression analysis was used to determine associations with disease‐related and personal factors. Results: A total of 147 CIDP and 103 MMN patients responded. Limitations in activities were reported by 70.7% CIDP and 52.2% MMN patients with moderate to severe limitations in 22.4% and 5.9% patients, respectively. Participation restrictions were reported by 50% of CIDP and 40% of MMN patients, nevertheless satisfaction with participation was high. Fatigue, pain and resilience were independently associated with limitations in activities and satisfaction with participation in CIDP patients. Conclusions: Activity limitations and restrictions in participation are common in CIDP patients and to a lesser extent in MMN patients. Fatigue, pain and resilience independently contributed to perceived limitations in CIDP patients. Referral to a rehabilitation physician is warranted to address these limitations appropriately. [ABSTRACT FROM AUTHOR]

The aim of this study was to determine the interrater reliability and stability over time of the Capacity Profile (CAP). The CAP is a standardized method for classifying additional care needs indicated by current impairments in five domains of body functions: physical health, neuromusculoskeletal and movement-related, sensory, mental, and voice and speech, in children from 3 to 18 years of age. The intensity of care in each domain is defined from 0 (no need for additional care) to 5 (needs help with every activity). The intensity of additional care in each of the five separate domains indicates the CAP for the individual child. We developed the CAP to inform the parents and other caregivers of children with non-progressive, permanent neurodevelopmental disabilities, such as cerebral palsy and myelomeningocele, about the consequences of these conditions. To determine interrater agreement and stability over time, the CAPs of 67 children (39 males, 28 females) with a neurodevelopmental disability (mean age 18y [SD 1.2y]; range 14-22y) were assessed based on a semi-structured interview. In addition, the CAPs of the same individuals at the age of 3 years were determined based on a chart review. Interrater agreement of the CAP at the age of 3 was good to very good (weighted kappa 0.64-0.92). Agreement between the CAP at the age of 18 and the CAP at the age of 3 (providing evidence for stability over time) was also good (weighted kappa 0.68-0.77), except for the domain "physical health functions", about which agreement was relatively poor (0.47). We conclude that the CAP is a reliable instrument for classifying the additional needs of a child with a non-progressive, permanent neurodevelopmental disability. The preliminary evidence for the stability over time of such needs according to the CAP should be validated in a prospective study. (Contains 3 tables and 1 footnote.)

Objective: Uniform data collection is fundamental for multicentre clinical trials. We aim to determine the variability, between ALS trial centers, in the prevalence of unexpected or implausible improvements in the revised ALS functional rating scale (ALSFRS-R) score, and its associations with individual patient and item characteristics. Methods: We used data from two multicentre studies to estimate the prevalence of an unexpected increase or implausible improvement in the ALSFRS-R score, defined as an increase of 5 points or more between two consecutive, monthly visits. For each patient with a 5-point or more increase, we evaluated the individual contribution of each ALSFRS-R item. Results: Longitudinal ALSFRS-R scores, originating from 114 trial centers enrolling a total of 1,240 patients, were analyzed. A 5-point or more increase in ALSFRS-R total score was found in 151 (12.2%) patients, with prevalence per study center ranging from 0% to 83%. Bulbar onset, faster disease progression at enrollment, and a lower ALSFRS-R score at baseline were associated with a sudden 5-point or more increase in the ALSFRS-R total score. ALSFRS-R items 2 (saliva), 9 (stairs), 10 (dyspnea), and 11 (orthopnea) were the primary drivers when a 5-point or more increase occurred. Conclusions: Sudden 5-point or more increases in ALSFRS-R total scores between two consecutive visits are relatively common. These sudden increases were not found to occur with equal frequency in trial centers; which underscores the need for amending existing standard operating procedures toward a universal version and monitoring of data quality during the study, in multicentre research. [ABSTRACT FROM AUTHOR]