Disability Register Research Articles

Mortality from sudden unexpected death in epilepsy (SUDEP) in a cohort of adults with intellectual disability

People with intellectual disability (ID) and epilepsy are more likely to die prematurely than the general population. A significant number of deaths in people with epilepsy may be potentially preventable through better seizure control, regular monitoring and raising awareness among patients and carers. The aim of this project was to study mortality from sudden unexpected death in epilepsy (SUDEP) in adults with ID. All adults (≥20 years old) living in Leicester city, Leicestershire and Rutland, UK, with ID between 1993 and 2010 were identified using the Leicestershire Intellectual Disability Register database. People with and without ID who died during the same period were identified using death certificate data from the Office for National Statistics (ONS). Deaths from probable and definite SUDEP were identified. Additional information on adults with ID who had died from probable or definite SUDEP was obtained from case notes and post-mortem reports, where available. Cases of probable and definite SUDEP in adults with ID were compared with the general population using standardised mortality ratios (SMRs). A total of 898 adults with ID had died over the 18-year study period. Of these, 244 deaths (27%) occurred in people with ID who had a diagnosis of epilepsy. Twenty-six people with ID died from probable or definite SUDEP, which was the second most common cause of death among adults with ID and epilepsy. All-cause specific SMRs were 2.2 [95% confidence interval (CI): 2.0-2.4] and 2.8 (95% CI: 2.5-3.1) for men and women with ID respectively. SMRs were 3.2 (95% CI: 2.7-3.8) and 5.6 (95% CI: 4.6-6.7) for men and women with epilepsy and ID respectively. During the same study period, 83 adults without ID had died of probable or definite SUDEP. The SMRs for SUDEP in patients with ID were 37.6 for men (95% CI: 21.9-60.2) and 52.0 for women (95% CI: 23.8-98.8). We found that in the majority of ID cases there was little detailed documentation on the circumstances surrounding deaths, no communication with patients/carers about risk of SUDEP and an absence of post-mortem reports or carers' referral for bereavement counselling. The authors believe that a comprehensive risk management under a multiagency/multidisciplinary framework should be undertaken for all adults with ID and epilepsy in day-to-day clinical practice to reduce mortality in people with ID.

Intellectual disability: population‐based estimates of the proportion attributable to maternal alcohol use disorder during pregnancy

The aim of this study was to examine the association between maternal alcohol use disorder and intellectual disability in children. All mothers with an International Classification of Diseases (ICD) 9 and/or 10 alcohol-related diagnosis, a proxy for alcohol use disorder, recorded on the Western Australian health, mental health, and drug and alcohol data sets were identified through the Western Australian Data Linkage Unit (n=5614 non-Aboriginal; n=2912 Aboriginal). A comparison cohort of mothers without an alcohol-related diagnosis was frequency matched on maternal age within maternal Aboriginal status and year of birth of their children. Linkage with the Western Australian Midwives Notification System (1983-2001) identified all births to these mothers (n=10 664 and 7907 respectively). Linkage to the Western Australian Intellectual Disability Database and Register of Developmental Anomalies identified cases of intellectual disability with no identified genetic origin (intellectual disability) (n=1487) and fetal alcohol syndrome (n=66). Odds ratios (ORs) and 95% confidence intervals (CIs) for intellectual disability were calculated using logistic regression incorporating generalized estimating equations and used to estimate population-attributable fractions. At least 3.8% (95% CI 2.84-4.89%) of cases of intellectual disability could be avoided by preventing maternal alcohol use disorder: 1.3% (95% CI 0.81-1.86%) in non-Aboriginal and 15.6% (95% CI 10.85-20.94%) in Aboriginal children. We observed a three-fold increase in the adjusted odds of intellectual disability in children of mothers with an alcohol-related diagnosis recorded during pregnancy (non-Aboriginal OR 2.89, 95% CI 1.62-5.18; Aboriginal OR 3.12, 95% CI 2.13-4.56), with a net excess proportion of 3.7% and 5.5% respectively. One-third (32%) of children diagnosed with fetal alcohol syndrome had intellectual disability. Maternal alcohol use disorder is the leading known risk factor for intellectual disability with no identified genetic origin.

Reported numbers of patients with rare diseases based on ten-year longitudinal national disability registries in Taiwan

This paper aims to describe a general demographic picture of patients with rare diseases in Taiwan and particularly focuses on the prevalence of rare diseases over time, age and gender distributions. We analyzed data mainly from the national disability registry from 2002 to 2011 in Taiwan, Republic of China. The results showed that the number of rare diseases increased from 93 to 193 between 2002 and 2011 and that the prevalence of rare diseases increased from 0.02 to 0.74 per 10,000 people in this time period. The gender ratio (male/female) was between 1.02 and 1.13 during this time period, with male cases representing a higher percentage than female cases in the rare disease population. The occurrence of rare diseases was significantly increased in children 3-5 years of age and elementary school children 6-14 years of age. The data also revealed that the occurrence of rare diseases in Taiwan was attributed primarily to pathogenic diseases and secondarily to genetic diseases. To obtain precise epidemiological data on rare diseases for future healthcare planning, this study highlights the importance of the cooperation of healthcare authorities with the social welfare department to strengthen the ability of the public healthcare system to regularly monitor and measure the occurrence of rare diseases in the community.

Gender and geographic differences in the prevalence of intellectual disability in children: Analysis of data from the national disability registry of Taiwan

Intellectual disability (ID) is not uncommon in children, but data at the national level are limited, especially those on geographic differences. On the basis of the Disabled Welfare Law, Taiwan began to certify disabled residents and provide various services in 1980. All the cases are registered, and the registry provides a rare opportunity for studying ID at the national level. Using the data from 2004 to 2010, we calculated the prevalence of ID in children by age, gender, and geographic area and assessed the changes over time. We limited analyses to children at least 3 years of age, because certification before 3 years old is discouraged by the government. We found that from 2004 to 2010, the registered cases between 3 and 17 years old ranged from 20,531 to 23,547, and the prevalence of ID increased constantly from 4.40/1000 to 5.79/1000 (p<0.01), which generally increased every year in all age groups (p<0.01). In each year there were more boy cases than girl cases, and the boy-to-girl ratio generally decreased with age (p<0.01 for chi-square test for trend in all years). The prevalence rate ratio ranged from 1.33 to 1.37 (p<0.01 in all years), and the changes in the rate ratio were small over the years. We observed a higher prevalence in the rural areas over the years, and the prevalence rate ratio ranged from 1.34 to 1.43 (p<0.01 in all years), with an increasing trend over time (p<0.01).

Differences in Obesity Rates Between People With and Without Disabilities and the Association of Disability and Obesity: A Nationwide Population Study in South Korea

ObjectivesThe objective of this study was to identify the differences in obesity rates among people with and without disabilities, and evaluate the relationship between obesity rates and the existence of disabilities or characteristics of disabilities.MethodsMass screening data from 2008 from the National Disability Registry and National Health Insurance (NHI) are used. For analysis, we classified physical disability into three subtypes: upper limb disability, lower limb disability, and spinal cord injury. For a control group, we extracted people without disabilities by each subtype. To adjust for the participation rate in the NHI mass screening, we calculated and adopted the weight stratified by sex, age, and grade of disability. Differences in obesity rates between people with and without disabilities were examined by a chi-squared test. In addition, the effect of the existence of disabilities and grade of disabilities on obesity was examined by multiple logistic regression analysis.ResultsPeople with disabilities were found to have a higher obesity rate than those without disabilities. The obesity rates were 35.2% and 35.0% (people with disabilities vs. without disabilities) in the upper limb disability, 44.5% and 34.8% in the lower limb disability, 43.4% and 34.6% in the spinal cord injury. The odds for existence of physical disability and grade of disability are higher than the non-disabilities.ConclusionsThese results show that people with physical disability have a higher vulnerability to obesity.

Gender and geographic differences in the prevalence of autism spectrum disorders in children: Analysis of data from the national disability registry of Taiwan

The prevalence of autism spectrum disorders (ASD) in the world has increased dramatically in the recent decades. However, data at the national level are limited, and geographic differences are seldom evaluated. According to the law, the local governments in Taiwan began to certify disabled residents and provide various services in 1980, and the central government maintains a registry of certified cases. The registry started to enroll cases of ASD in 1990, providing a unique opportunity for studying ASD at the national level. Because the government discourages the certification under 3 years of age, we limited our analyses to those who were at least 3 years old. Using the registry data from 2004 to 2010, we calculated the prevalence of ASD by age, gender, and geographic area and assessed the changes over time. From 2004 to 2010, the registered cases between 3 and 17 years old increased from 3995 to 8072 annually, and the prevalence generally increased every year in all age groups (p<0.01). In each year there were more boy cases than girl cases, and the prevalence rate ratio ranged from 5.64:1 to 6.06:1 (p<0.01 in all years), with an increasing trend over time (p<0.01). A higher prevalence was observed in the urban areas over the years, and the prevalence rate ratio ranged from 2.24:1 to 2.72:1 (p<0.01 in all years), with a decreasing trend over time (p<0.01).

ICF disability measured by WHO-DAS II in three community diagnostic groups in Madrid, Spain

ObjectiveThe planning, provision and monitoring of medical and support services for patient groups with chronic ailments may require disability assessment and registration. The purpose of this study was to assess disability in three groups of patients with chronic obstructive pulmonary disease (COPD), chronic heart failure (CHF) or stroke. MethodsConvenience samples of consecutive patients diagnosed with COPD (102), CHF (99), and stroke (99) were taken from 1,053 primary care users in the southern area of the autonomous region of Madrid. The patients were informed of the study and were assessed in their homes by trained field workers using the World Health Organization Disability Assessment Schedule II (WHO-DAS II). ResultsNone of the groups had patients with extreme disability on their global WHO-DAS II scores. The prevalence of severe disability differed among the groups and was highest for stroke and CHF (33.33% and 29.29%, respectively) and lowest for COPD (14.71%). The three groups shared two similar traits, namely, a higher prevalence of disability among women than men, and a specific pattern by domain, with the highest prevalence of severe/extreme limitations being found in household life activities and mobility. Severe restrictions in Social Participation were more frequent in patients with stroke and CHF. The group with moderate disability according to the global WHODAS II score (n=94) showed a high prevalence of severe limitations in mobility, life activities and self-care. ConclusionsDisability among non-institutionalized persons with COPD, CHF and stroke is frequent and shows gender- and domain-related patterns similar to those described in a population-based study performed using the WHO-DAS II in elderly persons in Spain. ICF-validated disability categories could be useful in epidemiological surveys, individual assessments and primary care data monitoring systems.

The Highs and Lows of Counting Autism

The Highs and Lows of Counting Autism

Prevalence of Autism Spectrum Disorders in a Total Population Sample

Experts disagree about the causes and significance of the recent increases in the prevalence of autism spectrum disorders (ASDs). Limited data on population base rates contribute to this uncertainty. Using a population-based sample, the authors sought to estimate the prevalence and describe the clinical characteristics of ASDs in school-age children. The target population was all 7- to 12-year-old children (N=55,266) in a South Korean community; the study used a high-probability group from special education schools and a disability registry and a low-probability, general-population sample from regular schools. To identify cases, the authors used the Autism Spectrum Screening Questionnaire for systematic, multi-informant screening. Parents of children who screened positive were offered comprehensive assessments using standardized diagnostic procedures. The prevalence of ASDs was estimated to be 2.64% (95% CI=1.91-3.37), with 1.89% (95% CI=1.43-2.36) in the general-population sample and 0.75% (95% CI=0.58-0.93) in the high-probability group. ASD characteristics differed between the two groups: the male-to-female ratios were 2.5:1 and 5.1:1 in the general population sample and high-probability group, respectively, and the ratios of autistic disorders to other ASD subtypes were 1:2.6 and 2.6:1, respectively; 12% in the general-population sample had superior IQs, compared with 7% in the high-probability group; and 16% in the general-population sample had intellectual disability, compared with 59% in the high-probability group. Two-thirds of ASD cases in the overall sample were in the mainstream school population, undiagnosed and untreated. These findings suggest that rigorous screening and comprehensive population coverage are necessary to produce more accurate ASD prevalence estimates and underscore the need for better detection, assessment, and services.

ADHD management and disability register: an electronic aid for the nice guideline implementation

AimWe designed an electronic software that will assist a wide range of healthcare professionals to implement the major aspects of the National Institute of Clinical Excellence (NICE) guidelines both within...

In-Depth Injury Analysis using the National Integrated Accident Data Linked to Permanent Disability and Trauma Registration Data in Japan

In-Depth Injury Analysis using the National Integrated Accident Data Linked to Permanent Disability and Trauma Registration Data in Japan

Increased incidence of disability due to mental and behavioural disorders in Iceland 1990–2007

Aims: To explore longitudinal changes in the importance of mental and behavioural disorders and their subgroups among people receiving disability pension in Iceland in the period 1990 to 2007.Method: Estimation of the incidence of disability pension was based on national demographic data and information from the national disability register which includes the main diagnoses causing disability.Results: The share of mental and behavioural disorders among new recipients of disability pension increased from 14 to 30% for women and 20 to 35% men. There was a marked increase in the incidence of disability due to disorders related to the use of alcohol and other psychoactive substances, mood disorders, disorders of psychological development and behavioural and emotional disorders with onset usually occurring in childhood and adolescence. The proportion of mental and behavioural disorders as the primary diagnosis among new recipients of disability pension increased.Conclusion: The most likely explanation for the changes we observe is alteration in social conditions leading to a lower threshold for seeking disability pension for mental disorders.

Marriage and family life in people with developmental disability

It has been reported that people with developmental disability are less likely to marry and have a family life compared to the general population. However, it has yet to be fully ascertained if this is true. This study aimed to establish the number of married people with developmental disability on the Leicestershire Learning Disability Register, whether or not any differences exist with regard to marriage and family life between ethnic groups in adults with developmental disability. The study sample was obtained from the Leicestershire Learning Disability Register and classified by ethnic origin as Asian, White or other. Case ascertainment of marital status showed that 4.6% of the study sample had ever married, the rate in the white ethnic group was 4.4% while in the Asian group 9.4%, the difference not being statistically significant. Individuals from the Asian group who had ever married, were significantly younger, significantly more likely to have children living within the household and had a signifi...

Emergency department utilization and determinants of use by 0- to 6-year-old children with disabilities in Taipei

Although many studies have explored emergency services for children, there are few published reports of the utilization of emergency services by children with disabilities. The present study attempts to provide data regarding the utilization of, and factors affecting, emergency department visits by disabled children in Taipei. A general census of 1006 children with disabilities, identified from the Taiwan National Disability Registry System in Taipei, was conducted. The overall response rate was 38%, yielding a sample of 340 disabled children. The results showed that 30.1% of children with disabilities had utilized emergency department services over the past 4 months with an average of 1.4 visits per child. The most common reasons for emergency visits were fever (34.7%), respiratory symptoms (24.2%), abdominal pain (15.8%), injury (7.4%), and epilepsy seizures (7.4%). This study also found, using a logistic regression model, that emergency department utilization may be associated with household economic status and the reported physical health of children with disabilities. The ‘deficit’ and ‘balance’ household economic status groups gave odds ratios of 3.902 (95% CI = 1.469–10.364) and 3.311 (95% CI = 1.249–8.779), relative to the ‘surplus’ group. The model also indicated that those children with disabilities who were reported as being in poor physical health had 11.359 times (95% CI = 2.968–43.469) the likelihood of using emergency care than those whose physical health was in excellent condition. The study suggests that in order to maximize the health of children with disabilities, medical care stakeholders should consider who are the most likely groups to use emergency department services and develop anticipatory guidance or preventive services for this vulnerable population.

A comparative study of stress and unmet needs in carers of South Asian and white adults with learning disabilities

People with learning disabilities have high dependency needs and high prevalence of physical, psychological and social morbidities. Some studies have shown that South Asian and white populations have a similar prevalence of learning disabilities and related psychological morbidity (McGrother et al, 2002), although other studies have shown an increased prevalence of severe levels of learning disabilities in the South Asian population (Emerson et al, 1997). The aim of this study was to compare stress levels and unmet service needs in informal carers of South Asian and white adults with learning disabilities.A sample of 742 informal carers was selected from the Leicestershire Learning Disability Register. Data on carers' and subjects' demographic details, stress levels and unmet service needs were analysed and compared using chi‐square tests and logistic regression analyses. Substantial differences were observed between the two groups. Carers of South Asian adults with learning disabilities reported significantly higher levels of care provision and unmet needs. Major stress was reported in 23% of carers. This was more common in carers with poor health, in those caring for younger adults, carers of adults with psychological symptoms, and in those with an expressed need for moral support or respite care.Stress is common among informal carers of adults with learning disabilities and inequalities, in reported care given and unmet needs, exist between carers of South Asian and white adults. Practitioners need to be aware of factors associated with stress when assessing carers in this population.

Intellectual disability co-occurring with schizophrenia and other psychiatric illness: population-based study

The epidemiology of intellectual disability co-occurring with schizophrenia and other psychiatric illness is poorly understood. The separation of mental health from intellectual disability services has led to a serious underestimation of the prevalence of dual diagnosis, with clinicians ill-equipped to treat affected individuals. To estimate the prevalence of dual diagnosis and describe its clinical profile. The Western Australian population-based psychiatric and intellectual disability registers were cross-linked (total n=245,749). Overall, 31.7% of people with an intellectual disability had a psychiatric disorder; 1.8% of people with a psychiatric illness had an intellectual disability. Schizophrenia, but not bipolar disorder and unipolar depression, was greatly over-represented among individuals with a dual diagnosis: depending on birth cohort, 3.7-5.2% of those with intellectual disability had co-occurring schizophrenia. Pervasive developmental disorder was identified through the Intellectual Disability Register and is therefore limited to individuals with intellectual impairment. None the less, pervasive developmental disorder was more common among people with a dual diagnosis than among individuals with intellectual disability alone. Down syndrome was much less prevalent among individuals with a dual diagnosis despite being the most predominant cause of intellectual disability. Individuals with a dual diagnosis had higher mortality rates and were more disabled than those with psychiatric illness alone. The facility to combine records across administrative jurisdictions has enhanced our understanding of the epidemiology of dual diagnosis, its clinical manifestations and aetiological implications. In particular, our results are suggestive of a common pathogenesis in intellectual disability co-occurring with schizophrenia.

Disparities between persons with and without disabilities in their participation rates in mass screening

The purpose of this study was to determine the number of persons with and without disabilities who participated in the National Health Insurance (NHI) chronic disease mass screening programs in South Korea. The data were obtained from mass screening claims submitted to the NHI and National Disability Registry. Factors affecting the participation rate included demographic variables, socioeconomic status, residential region, and disability type and severity. A multiple logistic regression analysis was used to evaluate the relationship between participation rates and disability type and severity adjusted for confounding factors. The analysis revealed that persons with a disability were less likely to participate in mass screening programs than those without a disability (35.8% vs. 40.2%). Multiple logistic regression analysis indicated that persons with severe disabilities had lower participation rates than those without disabilities [adjusted odds ratio (aOR): 0.64, 95% confidence interval (CI): 0.63-0.64]. In particular, persons with severe disabilities such as limb, brain, visual and internal organ impairment, were less likely to participate in the mass screening programs. However, persons with mild disability had higher participation rates than those without disabilities (1.03, 1.02-1.03). Although the prevalence rates of chronic diseases are higher among persons with disabilities, various types of impairments such as limb, brain, visual and internal organ impairment, hinder participation in mass screening programs for chronic diseases. The reasons for this disparity must be investigated and health policies must be altered to make preventative treatments more accessible to persons with disabilities.

Administrative Prevalence of autism spectrum disorders based on national disability registers in Taiwan

The aim of this paper was to describe the over time prevalence of autism from 2000 to 2007 in Taiwan, with particular focus on the age, gender, prevalence and causes. We analyzed data from the public web-access information collected by the Department of Statistics, Ministry of the Interiors, Taipei, Taiwan. The data included: (1) the physically and mentally disabled population by age and severity level; (2) the physically and mentally disabled population by cause; and (3) the Taiwan general population by age. The registered number of people with autism drastically increased from 2062 to 7207 (increase rate = 249.5%), and the male/female ratio of autism was 6.0–6.6 from 2000 to 2007. The autistic cases increased significantly, particularly in children between 6 and 11 years of age, occupying about 43.9–49.5% of the autistic population. The reported prevalence of each age group, aged 0–5 years was 2.4–7.8/10,000, aged 6–11 was 5.0–17.3/10,000, and aged 12–17 years was 2.1–10.4/10,000 from year 2000 to 2007. The main cause of autism was congenital (48.5–58.0%), followed by disease (9.5–31.1%) or other (20.0–32.7%). An increase in prevalence would suggest the need for more attention and funding of services for children with autism.

National disability registers report on causes of intellectual disability in Taiwan: 2000–2007

The main purposes of the present analysis were to describe the causes of intellectual disability (ID) and examine its overtime change from 2000 to 2007 in Taiwan. Data of the present study mainly come from the public web-access information which collected by the Department of Statistics, Ministry of the Interiors, Taipei, Taiwan. Data were obtained from two ways of 2000–2007 national data: (1) The physically and mentally disabled population by cause; (2) Taiwan general population by age. The present results found that the congenital disability and the disease were the main causes among the ID population in Taiwan. The overtime trend (2000–2007) of causes among the ID population illustrated that the percentage of congenital-caused ID was decreasing and the disease-caused ID was increasing slightly. However, both of above two causes – congenital- and disease-caused ID prevalence – were increasing slightly in the Taiwan general population in the year 2000–2007. The present analysis suggests that the current disability registers should re-examine the ID-caused categories according to the evidence-based literatures regarding attributive risks for this group of people in Taiwan.

Population with intellectual disability based on 2000–2007 national registers in Taiwan: Age and gender

Primary prevention of intellectual disability (ID) has long been a goal of public health professionals, without a clear picture of the ID population, efforts to understand its nature and improve the lives for this group of people will be impossible. The purpose of this paper was to describe the over time prevalence of ID from 2000 to 2007 in Taiwan, particular focused on the age and gender distributions. We analyzed data for the present study from the 2000 to 2007 Taiwan national disability registers, mainly were obtained from two ways: (1) the Physically and Mentally Disabled Population by Aged and Level; and (2) Taiwan General Population by Age. The results revealed that the registered number of people with ID drastically increased from 71,012 to 91,004 and the general population was slightly increasing from 22,276,672 to 22,958,360 in the year of 2000 and 2007 in Taiwan. The increase rate of population in ID cases is 13.67 times of the general population (ID = 28.15%, general population = 2.06%) and the ID prevalence was increasing from 0.318% to 0.396% in this period of time. Males occupied more percentage than female in ID population (roughly the male/female ratio was 1.37). The ID population was particularly significant increased during the age periods of the school and young adult. However, in the elderly age groups (≧60 years) showed the percentage of Taiwan general population was 2.9 times of ID population percentage to the age structure in 2007 in the analysis. The present analysis suggests that the prevalence is varied in different time. Therefore, it is needed to conduct the incidence as a choice in the public health system to monitoring and measuring the occurrence of people with ID in the community.