Impact of COVID-19: Nursing challenges to meeting the care needs of people with developmental disabilities

BackgroundPeople with developmental disabilities (DD) are a population at high-risk for poor outcomes related to COVID-19. COVID-19-specific risks, including greater comorbidities and congregate living situations in persons with DD compound existing health disparities. With their expertise in care of persons with DD and understanding of basic principles of infection control, DD nurses are well-prepared to advocate for the needs of people with DD during the COVID-19 pandemic.ObjectiveTo assess the challenges faced by nurses caring for persons with DD during the COVID-19 pandemic and how the challenges impact people with DD.MethodsWe surveyed 556 DD nurses, from April 6–20, 2020. The 35-item mixed-method survey asked nurses to rate the degree of challenges faced in meeting the care needs of people with DD. We analyzed responses based on presence of COVID-19 in the care setting and geographically. One open-ended question elicited challenges not included in the survey, which we analyzed using manifest content analysis.ResultsStartlingly, nurses reported being excluded from COVID-19 planning, and an absence of public health guidelines specific to persons with DD, despite their high-risk status. Obtaining PPE and sanitizers and meeting social-behavioral care needs were the most highly ranked challenges. COVID-19 impacted nurses’ ability to maintain adequate staffing and perform essential aspects of care. No significant geographic differences were noted.ConclusionsDD nurses must be involved in public health planning and policy development to ensure that basic care needs of persons with DD are met, and the disproportionate burden of COVID-19 in this vulnerable population is reduced.     

Rasch Analysis of the Pediatric Quality of Life Inventory 4.0 Generic Core Scales Administered to Patients With Duchenne Muscular Dystrophy

ObjectivesThe objective of this study was to examine the psychometric properties of the Pediatric Quality of Life Inventory 4.0 Generic Core Scales (PedsQL 4.0 GCS) in Duchenne muscular dystrophy (DMD), a rare, severely debilitating, and ultimately fatal neuromuscular disease.MethodsPatients with DMD were recruited from 20 centers across 9 countries as part of the Cooperative International Neuromuscular Research Group Duchenne Natural History Study (NCT00468832). The psychometric properties of the PedsQL 4.0 GCS were examined using Rasch analysis.ResultsIn total, 329 patients with DMD (mean age 9 years, range 3–18 years, 75% ambulatory) completed the PedsQL 4.0 GCS. The most difficult instrument items, expressing the greatest loss in health-related quality of life, were those associated with emotional well-being (eg, being teased by other children, feeling sad, and not making friends), as opposed to somatic disability (eg, lifting heavy objects, participating in sports, and running). The mean item and person fit residuals were estimated at 0.301 (SD: 1.385) and −0.255 (1.504), respectively. In total, 87% (20 of 23) of items displayed disordered thresholds, and many exhibited nontrivial dependency. The overall item-trait interaction χ² value was 178 (115 degrees of freedom, P<.001). Our analysis also revealed significant issues with differential item functioning, and by investigating residual principal component loadings, the PedsQL 4.0 GCS total score was found to be multidimensional.ConclusionsThe PedsQL 4.0 GCS records information clinically relevant to patients with DMD, but the total scale score may not be fit for purpose as a measure health-related quality of life in this disease population.     

“Together in a pressure cooker”: Parenting children with disabilities during the COVID-19 lockdown

BackgroundCOVID-19 has had a disproportionate and unprecedented impact on children with disabilities, their parents and families. This impact has been particularly evident during periods of lockdowns and severe restrictions.ObjectiveThis study employed the social model of disability to illuminate negative and positive experiences of Israeli parents of children with disabilities during the first COVID-19 lockdown, as well as the way social environments, particularly educational and welfare services, shape that experience.MethodsThe study draws upon thematic analysis of written responses of 80 Israeli parents to open-ended questions.ResultsThree main themes were found: (1) lack of responsiveness of both informal and formal supports to family needs, mainly in the educational and welfare systems; (2) juggling multiple roles and tasks along with the parents' routinely intensive caregiving role; and (3) the impact on the family's wellbeing and relationships.ConclusionThese results are discussed in light of the social model of disability, showing how social restrictions and barriers affect both negative and positive experiences of these families. Also discussed are practical implications for future crises.     

The effects of the COVID-19 pandemic on the psychosocial well-being of people with disabilities

BackgroundBefore the COVID-19 pandemic, people with mobility, vision, hearing, and cognitive disabilities were at a higher risk of lower psychosocial well-being than people without disabilities. It is, therefore, of great importance to investigate whether the pandemic has exacerbated this difference.ObjectiveThis study examines whether people with disabilities (categorized as mobility, vision, hearing, cognitive, and any disabilities) report more COVID-19-related negative effects on psychosocial well-being (loneliness, decreased social contact, decreased hope for the future, concerns about being infected) than people without disabilities.MethodsWe analyzed population-based data from the Finnish Health, Welfare, and Services (FinSote) survey carried out in 2020–2021 (N = 22 165, age 20+). Logistic regression models were applied, controlling for the effects of age, sex, partnership, living alone, and education.ResultsAll disability groups, except those with vision disabilities, reported significantly more often that the pandemic increased loneliness than people without disabilities. There were no significant differences between the disability groups and people without disabilities in decreased social contacts. People with only mobility and cognitive disabilities reported significantly more often that the pandemic decreased their hope for the future than those without disabilities. All disability groups were more often concerned about being infected than people without disabilities, but this effect was not significant among people 75 or older.ConclusionThe psychosocial well-being of people with specific types of disabilities should receive special attention during crises like the COVID-19 pandemic.     

Matching Special Olympics registration data with administrative health databases: Feasibility and health status differences in children and youth with IDD

BackgroundChildren and youth with intellectual and developmental disabilities (IDD) experience health disparities. What is unknown is if data collected from children and youth with IDD who participate in Special Olympics is representative of children and youth with IDD who do not.ObjectivesAim 1: determine the feasibility of matching a database of registrants from Special Olympics Ontario (SOO), with population-based health services databases in Ontario, Canada housed at ICES. Aim 2: evaluate the differences between the database sources with regards to demographic variables and clinical status.MethodsUsing deterministic and probabilistic matching, registration data from SOO were matched to administrative health databases. Established algorithms were used to determine the prevalence of asthma, diabetes, and mental disorder in addition to demographic variables.ResultsThe matching rate was over 90%; 8404 were attributed to children and youth between the ages of 0–19 years. When comparing SOO participants with IDD to non-SOO participants with IDD, children and youth who participate in SOO were, on average, older with no further differences between groups on clinical or demographic variables. When comparing those previously not identified in the health services databases (from SOO) to those with IDD identified by ICES, the SOO participants appear to use the health system less, possibly indicating a better health status.ConclusionsResearch conducted on child and youth who participate in Special Olympics Ontario can be generalized to the broader population of children and youth with IDD in Canada when adjusted for age; however, care should be taken when comparing levels of overall morbidity.     

COVID-19 vaccine perceptions in New York State's intellectual and developmental disabilities community

BackgroundPeople with intellectual and developmental disabilities (IDD) are at disproportionate risk for severe COVID-19 outcomes, particularly those living in congregate care settings. Yet, there is limited data on vaccine perceptions in the disability community.ObjectiveTo explore COVID-19 vaccine perceptions in individuals with IDD, their family members, and those who work with them, to inform a statewide vaccine information and messaging project.MethodsA national survey, adapted in five languages for the IDD community, was distributed to a convenience sample of IDD organizations throughout New York State. Constructs included vaccine intention, reasons for vaccine hesitancy, and trusted sources of vaccine information. Zip code data were used to map respondent location and vaccine preferences.ResultsOf n = 825 respondents, approximately 75% intended to or had received the vaccine across roles (i.e., people with developmental disabilities, family members, direct care workers) and racial/ethnic groups. Greater vaccine hesitancy was reported in younger individuals and those making decisions on behalf of a person with IDD. Concerns included side effects and the swiftness of vaccine development. Black and Hispanic participants had heightened concerns about being an “experiment” for the vaccine. Trusted sources of information included healthcare providers and family members. Respondents who intended to/received the vaccine were dispersed throughout the state.ConclusionsVaccine preferences in this New York State disability community sample align with national data. Identified concerns suggest the need for community education that addresses misperceptions. Age and race differences in perspectives highlight the need for tailored education, delivered by trusted messengers.     

Rare multiple congenital anomalies-hypotonia-seizures syndrome type 1 (MCAHS1) – the clinical and molecular summary

Multiple congenital anomalies-hypotonia-seizures syndrome type 1 (MCAHS1) is a rare autosomal recessive genetic disease belonging to glycosylphosphatidylinositols biosynthesis defects (GPIBD), a group of recessive disorders characterized by intellectual disability, hypotonia, and seizures. Glycosylphosphatidylinositols (GPIs) are glycolipids that anchor and remodel cell proteins. These processes are highly conserved and fundamental in the metabolism of all eukaryotes, including humans. Here, we have reported a male patient presenting with hypotonia, intellectual disability, and epilepsy, who underwent whole exome sequencing (WES). The analysis revealed the presence of two deleterious variants in PIGN that encodes GPI ethanolamine phosphate transferase-1 – one novel (c.1247_1251delAAGTG; p.Glu416Glyfs*22), and one that has been previously reported in the medical literature (c.1434+5G>A) resulting in MCAHS1. The detailed clinical assessment followed by the medical literature review also pointed out transient macrosomia and unreported in MCAHS1 advanced bone age and postnatal tall stature. These symptoms suggest that MCAHS1 shares a phenotypic overlap with disorders associated with overgrowth. To conclude, our case report and summary of the medical literature may be helpful for clinicians and geneticists who diagnose patients presenting with hypotonia accompanied by tall stature, advanced bone age, and transient macrosomia.     

The translation,cultural adaptation and psychometric evaluation of the Manchester Foot Pain and Disability Index in Persian-speaking Iranians with foot disorder

BackgroundFoot pain has a significantly detrimental effect on mobility, function and health status. Researchers have developed a number of measurement tools to assess foot health and its impact on the quality of life. This study was conducted to translate the original English Manchester Foot Pain and Disability Index (MFPDI) into Persian and to examine the psychometric properties of the Persian version.MethodsA total of 206 patients with a range of foot disorders filled out the Persian version of the MFPDI and the Short-Form 36 Health Survey (SF-36). The Persian version of the MFPDI was re-completed by the participants at an interval of five to seven days. The analyses performed included internal consistency, test-retest reliability, construct validity, and floor and ceiling effect analysis.ResultsThe Persian MFPDI had a good internal consistency (Cronbach’s α ≥ 0.70) and test-retest reliability (ICC = 0.84). The construct validity of the Persian MFPDI was demonstrated to be acceptable as a result of its significantly strong negative correlations with the SF-36.ConclusionThe results of this study showed that the Persian version of the MFPDI is a valid and reliable measure to assess foot-related disability.