Parental and child adjustment to amyotrophic lateral sclerosis: transformations, struggles and needs

Background: Amyotrophic lateral sclerosis (ALS), progressive muscular atrophy (PMA) and primary lateral sclerosis (PLS), together referred to as ALS, are life-limiting diagnoses affecting not only patients but also the families surrounding them, especially when dependent children are involved. Despite previous research highlighting the vulnerability of children in these families, they are, as yet, often overlooked in healthcare. Efforts are needed to better support children in families living with ALS, both directly and through strengthening parents in their parental role. This study sought to gain a better understanding of parental and children’s experiences, struggles and support needs in families living with ALS. Methods: Semi-structured interviews were conducted with 8 parents with ALS, 13 well parents and 15 children, together representing 17 families. Interview data were analyzed using qualitative content analysis. Results: Three major themes were identified relating to (1) ALS-related transformations in families’ homes, activities, roles and relationships, that trigger (2) distress among families, which, in turn, evokes (3) emotional, psychological, educational and practical support needs. For emotional and practical support, parents and children mainly rely on their own family and social network, whereas they seek educational and psychological support from healthcare professionals. Conclusions: Our findings imply that ALS care professionals may foster family adjustment to living with ALS, most notably through encouraging parents to engage in a dialogue with their children about the many transformations, struggles and needs imposed by ALS and teaching them how to start the dialogue

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. 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

Decreased antibody response after severe acute respiratory syndrome coronavirus 2 vaccination in patients with Down syndrom

The risk of a severe course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in adults with Down syndrome is increased, resulting in an up to 10-fold increase in mortality, in particular in those >40 years of age. After primary SARS-CoV-2 vaccination, the higher risks remain. In this prospective observational cohort study, SARS-CoV-2 spike S1-specific antibody responses after routine SARS-CoV-2 vaccination (BNT162b2, messenger RNA [mRNA]-1273, or ChAdOx1) in adults with Down syndrome and healthy controls were compared. Adults with Down syndrome showed lower antibody concentrations after 2 mRNA vaccinations or after 2 ChAdOx1 vaccinations. After 2 mRNA vaccinations, lower antibody concentrations were seen with increasing age. In this prospective cohort study that included 222 adults with Down syndrome, a significantly lower antibody response was found after SARS-CoV-2 mRNA or vector vaccination compared to healthy controls. After mRNA vaccination, lower antibodies were found with increasing age

Decreased Antibody Response After Severe Acute Respiratory Syndrome Coronavirus 2 Vaccination in Patients With Down Syndrome

UNLABELLED: The risk of a severe course of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in adults with Down syndrome is increased, resulting in an up to 10-fold increase in mortality, in particular in those >40 years of age. After primary SARS-CoV-2 vaccination, the higher risks remain. In this prospective observational cohort study, SARS-CoV-2 spike S1-specific antibody responses after routine SARS-CoV-2 vaccination (BNT162b2, messenger RNA [mRNA]-1273, or ChAdOx1) in adults with Down syndrome and healthy controls were compared. Adults with Down syndrome showed lower antibody concentrations after 2 mRNA vaccinations or after 2 ChAdOx1 vaccinations. After 2 mRNA vaccinations, lower antibody concentrations were seen with increasing age. CLINICAL TRIALS REGISTRATION: NCT05145348

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

BackgroundGastrostomy 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.MethodTo 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.ResultsIn 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.ConclusionPeople 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

Sleep health practices and sleep knowledge among healthcare professionals in Dutch paediatric rehabilitation

Background: Sleep disorders are highly prevalent in children with neurodisabilities, yet they seem under-recognized in paediatric rehabilitation settings. The aim of this study was to assess among two groups of healthcare professionals (HCPs) working in paediatric rehabilitation: (1) sleep health practices and (2) knowledge about sleep physiology, sleep disorders and sleep hygiene. Methods: We performed a cross-sectional sleep survey among medical and non-medical HCPs and the general population. Participants (30 rehabilitation physicians [RPs], 54 allied health professionals [AHPs] and 63 controls) received an anonymous 30-item survey consisting of three domains: (1) general information, (2) application of sleep health practices and (3) sleep knowledge. Results: RPs address sleep issues more frequently in clinical practice than AHPs. Sleep interventions mostly consist of giving advice about healthy sleep practices and are given by the majority of HCPs. While RPs demonstrated the highest scores on all knowledge domains, total sleep knowledge scores did not exceed 50% correct across groups, with AHPs and controls showing equal scores. Sleep hygiene rules closest to bedtime and related to the sleep environment were best known, whereas those related to daytime practices were rarely mentioned across all groups. A small minority of HCPs (RPs 20%; AHPs 15%) believed to possess sufficient sleep knowledge to address sleep in clinical practice. No association was found between self-perceived knowledge and sleep knowledge scores among HCPs. Conclusions: Sleep should become a standard item for review during routine health assessments in paediatric rehabilitation settings. HCPs' limited exposure to sleep education may result in feelings of incompetence and inadequate sleep knowledge levels, affecting their sleep health practices. Appropriate sleep training programs should be implemented to empower HCPs with knowledge, skills and confidence, needed to recognize and treat sleep disorders in children with neurodisabilities, as well as to be able to guide parents

Year-round RSV Transmission in the Netherlands Following the COVID-19 Pandemic - A Prospective Nationwide Observational and Modeling Study

We initiated a nationwide prospective study to monitor RSV-related pediatric hospitalizations in 46 hospitals throughout the Netherlands between May 2021 - August 2022. We showed year-round RSV transmission in the Netherlands after an initial 2021 summer outbreak. The pattern was unprecedented and distinct from neighboring countries. We extended a dynamic simulation model to evaluate the impact of waning immunity on pediatric RSV hospitalizations in the Netherlands using four different scenarios. Our results suggest that the observed continuous RSV transmission pattern could be associated with waning immunity due to the period of very low RSV circulation during the COVID-19 pandemic

Daily intranasal palivizumab to prevent respiratory syncytial virus infection in healthy preterm infants: a phase 1/2b randomized placebo-controlled trialResearch in context

Summary: Background: Mucosal administration of monoclonal antibodies (mAbs) against respiratory pathogens is a promising alternative for systemic administration because lower doses are required for protection. Clinical development of mucosal mAbs is a highly active field yet clinical proof-of-concept is lacking. Methods: In this investigator-initiated, double-blind, randomized placebo-controlled trial, we evaluated intranasal palivizumab for the prevention of RSV infection in preterm infants (Dutch Trial Register NTR7378 and NTR7403). We randomized infants 1:1 to receive intranasal palivizumab (1 mg/mL) or placebo once daily during the RSV season. Any RSV infection was the primary outcome and RSV hospitalization was the key secondary outcome. The primary outcome was analyzed with a mixed effect logistic regression on the modified intention-to-treat population. Findings: We recruited 268 infants between Jan 14, 2019 and Jan 28, 2021, after which the trial was stopped for futility following the planned interim analysis. Adverse events were similar in both groups (22/134 (16.4%) palivizumab arm versus 26/134 (19.4%) placebo arm). There were 6 dropouts and 168 infants were excluded from the efficacy analyses due to absent RSV circulation during the SARS-CoV-2 pandemic. Any RSV infection was similar in infants in both groups (18/47 (38.3%) palivizumab arm versus 11/47 (23.4%) placebo arm; aOR 2.2, 95% CI 0.7–6.5). Interpretation: Daily intranasal palivizumab did not prevent RSV infection in late preterm infants. Our findings have important implications for the clinical development of mucosal mAbs, namely the necessity of timely interim analyses and further research to understand mucosal antibody half-life. Funding: Funded by the Department of Pediatrics, University Medical Centre Utrecht, the Netherlands