Sensors in the care of persons with visual- or visual-and-intellectual disabilities:Use, needs, practical issues, and ethical concerns

BACKGROUND: Sensor technology may improve the quality of life of persons with visual and/or intellectual disabilities. However, there is no general consensus on its utility and implementation. OBJECTIVE: In this exploratory study the aim was to provide an overview of sensors for persons with disabilities to address priorities and ethical concerns for future research. METHODS: Using a qualitative (Delphi) method, 17 interviews were carried out with 20 representatives in the field of visual- or visual-and-intellectual disabilities (in general: six experts in sensor technology, domotics, and eHealth, specific for persons with a visual or visual-and-intellectual disability: three client representatives; three caregivers; four care team managers; two developmental psychologists; one physician; and one paramedic; age ranges 25-61 years). Atlas.ti software was used to code data and major themes were identified using qualitative analyses. RESULTS: The most used sensors were for surveillance and health and the most desired were for behavior. Different sensors were considered most important for future implementation by the groups of participants, such as sensors for lighting, posture, and entertainment by client experts. Furthermore, the majority of participants agreed that sensors should be easy to use and understand and ethical issues (e.g. privacy, informed consent) should be considered. CONCLUSION: The current applications of sensor technology in clinical practice and future research needs were determined by interviewing experts, caregivers, and client experts

Metabolic syndrome in 25% of older people with intellectual disability

Methods. Prevalence of the metabolic syndrome, according to National Cholesterol Education Program-Adult Treatment Panel III criteria, was assessed with standardized physical examinations in 470 Dutch adults with ID, aged >= 50 years, who receive residential care from three Dutch care providing organizations. Because of skewing towards an overrepresentation of females and more moderate to severe ID in the study population, reweighting was applied to obtain a representative population prevalence. Correlations with patient characteristics were analysed with logistic regression analyses. Results. Four hundred and twelve participants completed all assessments. The weighted prevalence of the metabolic syndrome was 25.1% [95% confidence interval (CI) 21.0-29.3%], with a significantly higher risk for people with mild ID. Conclusions. The prevalence of the metabolic syndrome in the population with ID is significantly higher than that in the general Dutch population aged >= 50 years (15.7%, 95% CI 13.5-17.9%)

Prevalence of Cardiovascular Risk Factors in Older People With Intellectual Disability

The prevalence and correlates of cardiovascular risk factors in older adults with intellectual disability was examined. We conducted a cross-sectional study with 50- to 90-year-old clients (N = 470) of three Dutch intellectual disability care providing organizations and found that healthy behavior was low, with 98.9% of the participants having an unhealthy diet and 68.3%, a lack of exercise. Smoking (13.6%) and alcohol abuse (0.3%) were relatively minor problems. Abdominal overweight (70.4% diabetes (8.7% hypertension (36.8% and hypercholesterolemia (31.8%) were highly prevalent. These profiles have important implications in determining the risk of cardiovascular disease in people with intellectual disability. Campaigns to promote health should be focused on education and the introduction of preventive screening programs

Timing of cognitive decline in CLN3 disease

Background: CLN3 disease is a major cause of childhood neurodegeneration. Onset of visual failure around 6 years of age is thought to precede cognitive deterioration by a few years, but casuistic reports question this paradigm. The aim of our study is to delineate timing of cognitive decline in CLN3 disease. Methods: Early neurocognitive functioning in CLN3 disease was analyzed using age at onset of visual and cognitive decline and IQ scores from literature-derived patient descriptions, supplemented with IQ scores and school history from a retrospective referral center cohort. We analyzed protracted and classical CLN3 separately and added a control group of patients diagnosed with juvenile onset macular degeneration (early onset Stargardt disease) to control for possible effects of rapid vision loss on neurocognitive functioning. Results: Onset of cognitive decline at a mean age of 6.8 years (range 2–13 years, n = 19) paralleled onset of visual deterioration at a mean age of 6.4 years (range 4–9 years, n = 81) as supported by an early decline in IQ scores in classical CLN3 disease. Onset and course of vision loss was similar in patients with protracted CLN3. The decreased IQ levels at diagnosis (mean 68.4, range 57–79, n = 9) in the referral cohort were consistently associated with an aberrant early school history contrasting normal school history and cognition in Stargardt disease patients. Conclusions: Cognitive dysfunction is universally present around diagnosis in classical CLN3 disease

Motor function impairment is an early sign of CLN3 disease

Objective To delineate timing of motor decline in CLN3 disease. Methods Motor function, assessed by the 6-Minute Walk Test (6MWT), was evaluated repeatedly in 15 patients with CLN3 disease, resulting in 65 test results and during one occasion in 2 control cohorts. One control cohort (n = 14) had isolated visual impairment; a second cohort (n = 12) exhibited visual impairment in combination with neurologic impairments. Based on 6MWT reference values in healthy sighted children, z scores of 6MWT results in patients with CLN3 disease and control cohort individuals were calculated. 6MWT results were correlated with age-including multilevel modeling analysis allowing assessment of imbalanced repeated measurements-and with Unified Batten Disease Rating Scale (UBDRS) scores. Results In CLN3 disease, 6MWT scores were already impaired from first testing near diagnosis (mean z scores of -3.6 and -4.7 at 7 and 8 years of age, respectively). Afterwards, 6MWT scores continuously declined with age (r = -0.64, p <0.0001) and with increasing UBDRS scores (r = -0.60, p = 0.0001), confirming correlation with disease progression. The decrease was more pronounced at a later age, as shown by the nonlinear multilevel model for 6MWT results in CLN3 disease (y = 409.18 - [0.52 x age(2)]). In contrast, an upward trend of 6MWT scores with age was observed in the control cohort with isolated visual impairment (r = 0.56; p = 0.04) similar to healthy, sighted children. The control cohort with additional neurologic impairments displayed a slightly decreased 6MWT walking distance independent of age. Conclusions The 6MWT unveils early onset of motor decline in CLN3 disease