Rates, causes and predictors of all-cause and avoidable mortality in 163 686 children and young people with and without intellectual disabilities: a record linkage national cohort study.

OBJECTIVES: To investigate mortality rates and associated factors, and avoidable mortality in children/young people with intellectual disabilities. DESIGN: Retrospective cohort; individual record-linked data between Scotland's 2011 Census and 9.5 years of National Records for Scotland death certification data. SETTING: General community. PARTICIPANTS: Children and young people with intellectual disabilities living in Scotland aged 5-24 years, and an age-matched comparison group. MAIN OUTCOME MEASURES: Deaths up to 2020: age of death, age-standardised mortality ratios (age-SMRs); causes of death including cause-specific age-SMRs/sex-SMRs; and avoidable deaths. RESULTS: Death occurred in 260/7247 (3.6%) children/young people with intellectual disabilities (crude mortality rate=388/100 000 person-years) and 528/156 439 (0.3%) children/young people without intellectual disabilities (crude mortality rate=36/100 000 person-years). SMRs for children/young people with versus those without intellectual disabilities were 10.7 for all causes (95% CI 9.47 to 12.1), 5.17 for avoidable death (95% CI 4.19 to 6.37), 2.3 for preventable death (95% CI 1.6 to 3.2) and 16.1 for treatable death (95% CI 12.5 to 20.8). SMRs were highest for children (27.4, 95% CI 20.6 to 36.3) aged 5-9 years, and lowest for young people (6.6, 95% CI 5.1 to 8.6) aged 20-24 years. SMRs were higher in more affluent neighbourhoods. Crude mortality incidences were higher for the children/young people with intellectual disabilities for most International Statistical Classification of Diseases and Related Health Problems, 10th Revision chapters. The most common underlying avoidable causes of mortality for children/young people with intellectual disabilities were epilepsy, aspiration/reflux/choking and respiratory infection, and for children/young people without intellectual disabilities were suicide, accidental drug-related deaths and car accidents. CONCLUSION: Children with intellectual disabilities had significantly higher rates of all-cause, avoidable, treatable and preventable mortality than their peers. The largest differences were for treatable mortality, particularly at ages 5-9 years. Interventions to improve healthcare to reduce treatable mortality should be a priority for children/young people with intellectual disabilities. Examples include improved epilepsy management and risk assessments, and coordinated multidisciplinary actions to reduce aspiration/reflux/choking and respiratory infection. This is necessary across all neighbourhoods.

Cohort profile: Scotland's record-linkage e-cohorts of people with intellectual disabilities, and autistic people (SCIDA).

PURPOSE: To investigate health, mortality and healthcare inequalities experienced by people with intellectual disabilities, and autistic people, and their determinants; an important step towards identifying and implementing solutions to reduce inequalities. This paper describes the cohorts, record-linkages and variables that will be used. PARTICIPANTS: Scotland's Census, 2011 was used to identify Scotland's citizens with intellectual disabilities, and autistic citizens, and representative general population samples with neither. Using Scotland's community health index, the Census data (demography, household, employment, long-term conditions) were linked with routinely collected health, death and healthcare data: Scotland's register of deaths, Scottish morbidity data 06 (SMR06: cancer incidence, mortality, treatments), Prescribing Information System (identifying asthma/chronic obstructive pulmonary disease; angina/congestive heart failure/hypertension; peptic ulcer/reflux; constipation; diabetes; thyroid disorder; depression; bipolar disorders; anxiety/sleep; psychosis; attention deficit hyperactivity disorder; epilepsy; glaucoma), SMR01 (general/acute hospital admissions and causes, ambulatory care sensitive admissions), SMR04 (mental health admissions and causes), Scottish Care Information-Diabetes Collaboration (diabetic care quality, diabetic outcomes), national bowel screening programme and cervical screening. FINDINGS TO DATE: Of the whole population, 0.5% had intellectual disabilities, and 0.6% were autistic. Linkage was successful for >92%. The resultant e-cohorts include: (1) 22 538 people with intellectual disabilities (12 837 men and 9701 women), 4509 of whom are children <16 years, (2) 27 741 autistic people (21 390 men and 6351 women), 15 387 of whom are children <16 years and (3) representative general population samples with neither condition. Very good general health was reported for only 3389 (15.0%) people with intellectual disabilities, 10 510 (38.0%) autistic people, compared with 52.4% general population. Mental health conditions were reported for 4755 (21.1%) people with intellectual disabilities, 3998 (14.4%) autistic people, compared with 4.2% general population. FUTURE PLANS: Analyses will determine the extent of premature mortality, causes of death, and avoidable deaths, profile of health conditions and cancers, healthcare quality and screening and determinants of mortality and healthcare.

Respiratory-associated deaths in people with intellectual disabilities: a systematic review and meta-analysis.

OBJECTIVE: To review and synthesise evidence on rates of respiratory-associated deaths and associated risk factors in the intellectual disability population. DESIGN: Systematic review and meta-analysis. DATA SOURCES: Embase, CINAHL, ISI Web of Science (all databases including Medline) and PsychINFO were searched for studies published between 1st January 1985 and 27th April 2020 and examined study and outcome quality. Reference lists and Google Scholar were also hand searched. RESULTS: We identified 2295 studies, 17 were included in the narrative synthesis and 10 studies (11 cohorts) in the meta-analysis. Data from 90 302 people with intellectual disabilities and 13 808 deaths from all causes in people with intellectual disabilities were extracted. Significantly higher rates of respiratory-associated deaths were found among people with intellectual disabilities (standardised mortality ratio(SMR): 10.86 (95% CI: 5.32 to 22.18, p<0.001) compared with those in the general population, lesser rates for adults with ID (SMR: 6.53 (95% CI: 4.29 to 9.96, p<0.001); and relatively high rates from pneumonia 26.65 (95% CI: 5.63 to 126.24, p<0.001). The overall statistical heterogeneity was I2=99.0%. CONCLUSION: Premature deaths due to respiratory disorders are potentially avoidable with improved public health initiatives and equitable access to quality healthcare. Further research should focus on developing prognostic guidance and validated tools for clinical practice to mitigate risks of respiratory-associated deaths. PROSPERO REGISTRATION NUMBER: CRD42020180479.

Birth incidence, deaths and hospitalisations of children and young people with Down syndrome, 1990-2015: birth cohort study.

OBJECTIVE: To investigate current Down syndrome live birth and death rates, and childhood hospitalisations, compared with peers. SETTING: General community. PARTICIPANTS: All live births with Down syndrome, 1990-2015, identified via Scottish regional cytogenetic laboratories, each age-sex-neighbourhood deprivation matched with five non-Down syndrome controls. Record linkage to Scotland's hospital admissions and death data. PRIMARY OUTCOME: HRs comparing risk of first hospitalisation (any and emergency), readmission for children with Down syndrome and matched controls were calculated using stratified Cox proportional hazards (PH) model, and length of hospital stay was calculated using a conditional log-linear regression model. RESULTS: 689/1479 (46.6%) female and 769/1479 (51.9%) male children/young people with Down syndrome were identified (1.0/1000 births, with no reduction over time); 1235 were matched. 92/1235 (7.4%) died during the period, 18.5 times more than controls. More of the Down syndrome group had at least one admission (incidence rate ratio(IRR) 72.89 (68.72-77.32) vs 40.51 (39.15-41.92); adjusted HR=1.84 (1.68, 2.01)) and readmissions (IRR 54.85 (51.46-58.46) vs 15.06 (14.36-15.80); adjusted HR=2.56 (2.08, 3.14)). More of their admissions were emergencies (IRR 56.78 (53.13-60.72) vs 28.88 (27.73-30.07); first emergency admission adjusted HR=2.87 (2.61, 3.15)). Children with Down syndrome had 28% longer first admission after birth. Admission rate increased from 1990-2003 to 2004-2014 for the Down syndrome group (from 90.7% to 92.2%) and decreased for controls (from 63.3% to 44.8%). CONCLUSIONS: We provide contemporaneous statistics on the live birth rate of babies with Down syndrome, and their childhood death rate. They require more hospital admissions, readmissions emergency admissions and longer lengths of stays than their peers, which has received scant research attention in the past. This demonstrates the importance of statutory planning as well as informal support to families to avoid added problems in child development and family bonding over and above that brought by the intellectual disabilities associated with Down syndrome.

Relative influence of intellectual disabilities and autism on mental and general health in Scotland: a cross-sectional study of a whole country of 5.3 million children and adults.

OBJECTIVES: To determine the relative extent that autism and intellectual disabilities are independently associated with poor mental and general health, in children and adults. DESIGN: Cross-sectional study. For Scotland's population, logistic regressions investigated odds of intellectual disabilities and autism predicting mental health conditions, and poor general health, adjusted for age and gender. PARTICIPANTS: 1 548 819 children/youth aged 0-24 years, and 3 746 584 adults aged more than 25 years, of whom 9396/1 548 819 children/youth had intellectual disabilities (0.6%), 25 063/1 548 819 children/youth had autism (1.6%); and 16 953/3 746 584 adults had intellectual disabilities (0.5%), 6649/3 746 584 adults had autism (0.2%). These figures are based on self-report. MAIN OUTCOME MEASURES: Self-reported general health status and mental health. RESULTS: In children/youth, intellectual disabilities (OR 7.04, 95% CI 6.30 to 7.87) and autism (OR 25.08, 95% CI 23.08 to 27.32) both independently predicted mental health conditions. In adults, intellectual disabilities (OR 3.50, 95% CI 3.20 to 3.84) and autism (OR 5.30, 95% CI 4.80 to 5.85) both independently predicted mental health conditions. In children/youth, intellectual disabilities (OR 18.34, 95% CI 17.17 to 19.58) and autism (OR 8.40, 95% CI 8.02 to 8.80) both independently predicted poor general health. In adults, intellectual disabilities (OR 7.54, 95% CI 7.02 to 8.10) and autism (OR 4.46, 95% CI 4.06 to 4.89) both independently predicted poor general health. CONCLUSIONS: Both intellectual disabilities and autism independently predict poor health, intellectual disabilities more so for general health and autism more so for mental health. Intellectual disabilities and autism are not uncommon, and due to their associated poor health, sufficient services/supports are needed. This is not just due to coexistence of these conditions or just to having intellectual disabilities, as the population with autism is independently associated with substantial health inequalities compared with the general population, across the entire life course.

Age at identification, prevalence and general health of children with autism: observational study of a whole country population.

OBJECTIVES: Reported childhood prevalence of autism varies considerably between studies and over time, and general health status has been little investigated. We aimed to investigate contemporary prevalence of reported autism by age, and general health status of children/young people with and without autism. DESIGN: Secondary analysis of Scotland's Census, 2011 data. Cross-sectional study. SETTING: General population of Scotland. PARTICIPANTS: All children (n=916 331) and young people (n=632 488) in Scotland. MAIN OUTCOME MEASURES: Number (%) of children/young people reported to have autism and their general health status; prevalence of autism; prevalence of poor health (fair, bad and very bad health); odds ratios (95% confidence intervals) of autism predicting poor health, adjusted for age and gender and OR for age and gender in predicting poor health within the population with reported autism. RESULTS: Autism was reported for 17 348/916 331 (1.9%) children aged 0-15, and 7715/632 488 (1.2%) young people aged 16-24. The rate increased to age 11 in boys and age 10 in girls, reflecting age at diagnosis. Prevalence was 2.8% at age 10 (4.4% for boys; 1.1% for girls), and 2.9% at age 11 (4.5% for boys; 1.1% for girls). 22.0% of children and 25.5% of young people with autism reported poor health, compared with 2.0% and 4.4% without autism. Autism had OR=11.3 (11.0 to 11.7) in predicting poor health. Autistic females had poorer health than autistic males, OR=1.6 (1.5 to 1.8). CONCLUSION: Accurate information on the proportion of autistic children and their health status is essential plan appropriate prevention and intervention measures and provide resources for those who may put demand on services designed for autistic people.

Prevalence of long-term health conditions in adults with autism: observational study of a whole country population.

OBJECTIVES: To investigate the prevalence of comorbid mental health conditions and physical disabilities in a whole country population of adults aged 25+ with and without reported autism. DESIGN: Secondary analysis of Scotland's Census, 2011 data. Cross-sectional study. SETTING: General population. PARTICIPANTS: 94% of Scotland's population, including 6649/3 746 584 adults aged 25+ reported to have autism. MAIN OUTCOME MEASURES: Prevalence of six comorbidities: deafness or partial hearing loss, blindness or partial sight loss, intellectual disabilities, mental health conditions, physical disability and other condition; ORs (95% CI) of autism predicting these comorbidities, adjusted for age and gender; and OR for age and gender in predicting comorbidities within the population with reported autism. RESULTS: Comorbidities were common: deafness/hearing loss-14.1%; blindness/sight loss-12.1%; intellectual disabilities-29.4%; mental health conditions-33.0%; physical disability-24.0%; other condition-34.1%. Autism statistically predicted all of the conditions: OR 3.3 (95% CI 3.1 to 3.6) for deafness or partial hearing loss, OR 8.5 (95% CI 7.9 to 9.2) for blindness or partial sight loss, OR 94.6 (95% CI 89.4 to 100.0) for intellectual disabilities, OR 8.6 (95% CI 8.2 to 9.1) for mental health conditions, OR 6.2 (95% CI 5.8 to 6.6) for physical disability and OR 2.6 (95% CI 2.5 to 2.8) for other condition. Contrary to findings within the general population, female gender predicted all conditions within the population with reported autism, including intellectual disabilities (OR=1.4). CONCLUSIONS: Clinicians need heightened awareness of comorbidities in adults with autism to improve detection and suitable care, especially given the added complexity of assessment in this population and the fact that hearing and visual impairments may cause additional difficulties with reciprocal communication which are also a feature of autism; hence posing further challenges in assessment.

Prevalence and general health status of people with intellectual disabilities in Scotland: a total population study.

BACKGROUND: Prevalence of intellectual disabilities varies considerably between studies. People with intellectual disabilities experience health inequalities, but most studies comprise small or incomplete populations. We investigated in a whole country population the (1) prevalence of intellectual disabilities and (2) general health status compared with the general population. METHOD: Data were from Scotland's Census, 2011. We calculated the prevalence of intellectual disabilities, reported general health status of people with and without intellectual disabilities and the extent of health-related limitations to daily activities. We conducted logistic regressions to determine the ORs of intellectual disabilities predicting poor health and associations with age and gender. RESULTS: Of Scotland's 5 295 403 population, 26 349 (0.5%) had intellectual disabilities; 15 149 (57.5%) were males and 11 200 (42.5%) were females; 5234 (0.6%) were children/youth (0-15) and 21 115 (0.5%) were adults (16-75+ years). Identification of intellectual disabilities rises until age 5 years, with a further small rise by age 9 years. Children and adults with intellectual disabilities reported more poor health (47.9% and 40.3%) than the general population (2.1% and 13.8%) and were more limited in activities by their health. Intellectual disabilities had an OR of 43.2 (95% CI 40.8 to 45.7) in predicting poor health; the influence of increasing age on poor health was markedly interacted by presence of intellectual disabilities, likely to be due to a 'healthy survivor' effect within the intellectual disabilities population. CONCLUSION: People with intellectual disabilities have poorer general health than other people, especially children and young people. Accurate information on population prevalence and health status is essential to plan appropriate resources.