The relationship between clinical presentation and the nature of care in adults with intellectual disability and epilepsy - national comparative cohort study.

BACKGROUND: A quarter of People with Intellectual Disabilities (PwID) have epilepsy compared with 1% of the general population. Epilepsy in PwID is a bellwether for premature mortality, multimorbidity and polypharmacy. This group depends on their care provider to give relevant information for management, especially epilepsy. There is no research on care status relationship and clinical characteristics of PwID and epilepsy. AIM: Explore and compare the clinical characteristics of PwID with epilepsy across different care settings. METHOD: A retrospective multicentre cohort study across England and Wales collected information on seizure characteristics, intellectual disability severity, neurodevelopmental/biological/psychiatric comorbidities, medication including psychotropics/anti-seizure medication, and care status. Clinical characteristics were compared across different care settings, and those aged over and younger than 40 years. RESULTS: Of 618 adult PwID across six centres (male:female = 61%:39%), 338 (55%) received professional care whereas 258 (42%) lived with family. Significant differences between the care groups existed in intellectual disability severity (P = 0.01), autism presence (P < 0.001), challenging behaviour (P < 0.001) and comorbid physical conditions (P = 0.008). The two groups did not vary in intellectual disability severity/genetic conditions/seizure type and frequency/psychiatric disorders. The professional care cohort experienced increased polypharmacy (P < 0.001) and antipsychotic/psychotropic use (P < 0.001/P = 0.008).The over-40s cohort had lower autism spectrum disorder (ASD) and attention-deficit hyperactivity disorder (ADHD) comorbidity (P < 0.001/P = 0.007), increased psychiatric comorbidity and challenging behaviour (P < 0.05), physical multimorbidity (P < 0.001), polypharmacy (P < 0.001) and antipsychotic use (P < 0.001) but reduced numbers of seizures (P = 0.007). CONCLUSION: PwID and epilepsy over 40 years in professional care have more complex clinical characteristics, increased polypharmacy and antipsychotic prescribing but fewer seizures.

People with Intellectual Disabilities, Dysphagia and Post-Covid Syndrome.

People with Intellectual Disability (ID) were more likely to contract COVID-19 infection and more likely to die from the consequences. However, there is no evidence on the long-term impact of COVID-19 infection in people with ID. Post-Covid Syndrome (PCS) is an established diagnosis that requires specialist clinical support. To date there is no data on how common PCS is in people with ID, or how symptoms present. Dysphagia is identified as a clinical marker because of the known association with PCS, and the clear objective diagnostic criteria applicable through specialist assessment. This investigation presents a cohort of people with ID, who developed dysphagia/worsening of dysphagia post diagnosis with COVID-19. Cases were identified through support from the Royal College of Speech and Language Therapists. Data was collected by electronic survey, including application of the COVID-19 Yorkshire Rehabilitation Scale-modified (C19-YRSm). The C19-YRSm is a validated assessment tool for PCS and it's impact upon functional disability. This case series identifies that symptoms consistent with PCS are present in people with ID, post-COVID-19 infection. The risk of diagnostic overshadowing or misdiagnosis is high due to the subjective nature and the quality of PCS symptoms. People with ID who develop PCS may not be readily identified by clinical services and therefore not be accessing the specialist medical support required. Furthermore, changes in behaviour secondary to PCS may lead to unnecessary increased prescribing of psychotropic medication which in itself risks worsening dysphagia. Dysphagia could be an important bellwether to identify PCS in people with ID.

Chronic constipation in people with intellectual disabilities in the community: cross-sectional study.

BACKGROUND: One-third to half of people with intellectual disabilities suffer from chronic constipation (defined as two or fewer bowel movements weekly or taking regular laxatives three or more times weekly), a cause of significant morbidity and premature mortality. Research on risk factors associated with constipation is limited. AIMS: To enumerate risk factors associated with constipation in this population. METHOD: A questionnaire was developed on possible risk factors for constipation. The questionnaire was sent to carers of people with intellectual disabilities on the case-loads of four specialist intellectual disability services in England. Data analysis focused on descriptively summarising responses and comparing those reported with and without constipation. RESULTS: Of the 181 people with intellectual disabilities whose carers returned the questionnaire, 42% reported chronic constipation. Constipation was significantly associated with more severe intellectual disability, dysphagia, cerebral palsy, poor mobility, polypharmacy including antipsychotics and antiseizure medication, and the need for greater toileting support. There were no associations with age or gender. CONCLUSIONS: People with intellectual disabilities may be more vulnerable to chronic constipation if they are more severely intellectually disabled. The associations of constipation with dysphagia, cerebral palsy, poor mobility and the need for greater toileting support suggests people with intellectual disabilities with significant physical disabilities are more at risk. People with the above disabilities need closer monitoring of their bowel health. Reducing medication to the minimum necessary may reduce the risk of constipation and is a modifiable risk factor that it is important to monitor. By screening patients using the constipation questionnaire, individualised bowel care plans could be implemented.

The views of psychiatrists on proposed changes to the England and Wales Mental Health Act 1983 legislation for people with intellectual disability: A national study.

BACKGROUND: The Draft Mental Health Bill proposes removal of both intellectual disability and autism from Section 3 of the Mental Health Act for England and Wales (MHA). This would lead to people with intellectual disability (PwID) and/or autism could not be detained beyond 28 days, in the absence of diagnosed co-occurring mental illness. AIM: To obtain views of psychiatrists working with PwID in England and Wales regarding the proposed MHA changes. This study focusses specifically on the impact on PwID. METHODS: A cross-sectional online mixed methodology survey of Likert and free-text response questions was developed, to ascertain perceptions of proposed legislative changes to the MHA. A non-discriminatory exponential snowballing technique leading to non-probability sampling was used to disseminate the survey. Quantitative data was analysed using descriptive statistics, Mann-Whitney and Fisher's exact tests. Thematic analysis was conducted on free text responses. RESULTS: A total of 82 psychiatrists (33%) from approximately 250 eligible completed the survey. Nearly two-thirds (64%) reported good awareness of the proposed changes, with over half (55%) reporting disagreement with the changes. Psychiatrists working in inpatient settings for PwID reported increased awareness of the changes, less agreement with the reforms, and increased expectations of the reforms having negative unintended consequences, compared to their peers working exclusively in the community. Consultants reported greater disagreement with the changes compared to their non-consultant peers. Qualitative analysis identified five main themes: impact on diagnosis and treatment, seeking alternative options, introducing inequities, resources, and meeting holistic care goals through the Care, Education and Treatment Reviews (CETR) process. CONCLUSION: Psychiatrists working with PwID report widespread disagreement with the proposed changes to the MHA for PwID, with greater levels of disagreement among those working in inpatient services. Caution with respect to the proposed changes, and monitoring of the impact of the changes if implemented, is advised.

The long-term psycho-social impact of the pandemic on people with intellectual disability and their carers.

BACKGROUND: People with intellectual disabilities (PWID) are at six times higher risk of death due to COVID-19. To mitigate harm, as a high-risk group, significant social changes were imposed on PWID in the UK. Alongside these changes, the uncertainty of the pandemic influence, caused PWID and their carers to encounter significant stress. The evidence of the pandemic's psycho-social impact on PWID originates mainly from cross-sectional surveys conducted with professionals and carers. There is little research on the longitudinal psycho-social impact of the pandemic from PWID themselves. AIMS: To examine the long-term psycho-social impact of the pandemic on PWID. METHODS: A cross-sectional survey, following STROBE guidance, of 17 Likert scale statements (12 to PWID and 5 to their carers) to ascertain the pandemic's psychosocial impact was conducted. Every other PWID open to a specialist Intellectual Disability service serving half a UK County (pop:500,000) was selected. The same survey was re-run with the same cohort a year later. Descriptive statistics, Mann-Whitney, Chi-square and unpaired-t tests were used to compare responses. Significance is taken at p < .05. Comments were analysed using Clarke and Braun's approach. RESULTS: Of 250 PWID contacted, 100 (40%) responded in 2020 and 127 (51%) in 2021. 69% (2020) and 58% (2021) reported seeking medical support. Carers, (88%, 2020 and 90%, 2021) noticed emotional changes in PWID they cared for. 13% (2020) and 20% (2021) of PWID had their regular psychotropics increased. 21% (2020) and 24% (2021) had their pro re nata (PRN) medication adjusted. PWID or carers demonstrated no statistically significant variation in responses between themselves from 2020 to 2021. PWID were more likely to report being upset/distressed compared to their carers' perceptions of them in both years (p < .001). Four themes were identified. CONCLUSION: This longitudinal study highlights the diverse psycho-social impact of the pandemic on PWID in the UK. The Pandemic's psycho-social impact has been significantly underestimated.

Mortality risk in adults with intellectual disabilities and epilepsy: an England and Wales case-control study.

BACKGROUND: People with epilepsy (PWE) and people with intellectual disabilities (ID) both live shorter lives than the general population and both conditions increase the risk of death further. We aimed to measure associations between certain risk factors for death in PWE and ID. METHODS: A retrospective case-control study was conducted in ten regions in England and Wales. Data were collected on PWE registered with secondary care ID and neurology services between 2017 and 2021. Prevalence rates of neurodevelopmental, psychiatric and medical diagnoses, seizure frequency, psychotropic and antiseizure medications (ASM) prescribed, and health activity (epilepsy reviews/risk assessments/care plans/compliance etc.) recorded were compared between the two groups. RESULTS: 190 PWE and ID who died were compared with 910 living controls. People who died were less likely to have had an epilepsy risk assessment but had a greater prevalence of genetic conditions, older age, poor physical health, generalized tonic-clonic seizures, polypharmacy (not ASMs) and antipsychotic use. The multivariable logistic regression for risk of epilepsy-related death identified that age over 50, medical condition prevalence, antipsychotic medication use and the lack of an epilepsy review in the last 12 months as associated with increased risk of death. Reviews by psychiatrists in ID services was associated with a 72% reduction in the odds of death compared neurology services. CONCLUSIONS: Polypharmacy and use of antipsychotics may be associated with death but not ASMs. Greater and closer monitoring by creating capable health communities may reduce the risk of death. ID services maybe more likely to provide this holistic approach.

The potential unintended consequences of Mental Health Act reforms in England and Wales on people with intellectual disability and/or autism.

The draft Mental Health Bill, which amends the Mental Health Act 1983 for England and Wales, proposes protections for people with intellectual disability and/or autism (ID/A) to prevent detention in hospital in the absence of mental illness. This editorial critically appraises the positive impact and unintended consequences of the proposed reforms for people with ID/A.

Clinical letters to patients with intellectual disabilities after psychiatric review: A quality improvement project.

AIM: This Quality Improvement Project sought to improve communication between patients with intellectual disabilities and their psychiatrists by sharing medical information using an easy read letter format following psychiatric review. BACKGROUND: Writing directly to patients is in keeping with good medical practice. Previous studies have shown patients with intellectual disabilities prefer letters tailored to meet their needs. METHOD: An easy read letter was used by nine psychiatrists who handed them to 100 consecutive patients after review. Feedback of acceptability to patients was obtained using a three-item facial rating scale and the use of free text. Feedback of acceptability was obtained from participating psychiatrists. RESULTS: Patients found the easy read letter helpful and felt it should be used routinely. Psychiatrists felt this approach was beneficial as well as aiding patient understanding of review. CONCLUSIONS: The easy read letter was reported to improve communication following psychiatric review. Limitations are acknowledged but it is concluded that an easy read letter should be adopted as routine practice following psychiatric review, for people with intellectual disabilities.

Implementing proposed reforms of the Mental Health Act for people with intellectual disability and autism: the perspective of multidisciplinary professionals in intellectual disability teams.

BACKGROUND: A recent government white paper sets out proposals for reforms to the Mental Health Act 1983 (MHA). Some of these proposals affect people with intellectual disabilities and/or autism. AIMS: To explore both positive and unintended negative effects of the proposed reforms by gathering the perspectives of healthcare workers from multiple disciplines, working with intellectual disability and/or autism in community and in-patient settings. METHOD: A 14-question electronic questionnaire, comprising free-text, multiple choice and five-point Likert scale responses, was sent out via email between April and July 2021, to all multidisciplinary team professionals working in specialist intellectual disability community and in-patient teams in Hertfordshire Partnership University NHS Foundation Trust. RESULTS: There were 45 responders, of whom 53% worked in in-patient settings and 47% in out-patient teams. Respondents comprised healthcare professionals from multiple disciplines, 80% of which were non-medical. Most responders agreed with the general principles of the proposed reforms. However, 80% felt there would be potentially unintended consequences, and 76% thought that substantial investment in community services was required in advance of the proposed reforms. CONCLUSIONS: The proposed MHA reforms may have unintended consequences for people with intellectual disabilities and/or autism. The findings of this study raised key concerns that need to be explored further and addressed before the MHA reforms are implemented. These include community provision, safeguards and use of the Mental Capacity Act, the potential for under or overdiagnosis of mental illness, and effects associated with the criminal justice system.

Bone health, intellectual disability and epilepsy: An observational community-based study.

OBJECTIVES: Intellectual disability (ID) and epilepsy are independent risk factors for osteoporosis. Diverse predisposing factors influence this, for example in ID, genetics and poor nutrition and in epilepsy, anti-seizure medication (ASM). Around 25% people with ID have epilepsy, majority treatment resistant. ASMs polypharmacy is common. However, little is known about the bone-related characteristics of this vulnerable group. A prospective observational cohort study of bone profile across a community ID Epilepsy service was undertaken to understand this. MATERIALS & METHODS: Participants were on minimum 2 years of ASMs. Baseline demographics, epilepsy data, bone metabolism biomarkers, bone mineral density (BMD) and vitamin D levels were collected. Doses needed to correct vitamin D insufficiency/deficiency were calculated. RESULTS: At baseline, of 104 participants, 92 (90.2%) were vitamin D insufficient/deficient. Seventy-six (73.1%) had a DEXA scan, 50 of whom-in the osteopaenic/osteoporotic range. DEXA scores between ambulant and non-ambulant patients were significantly different (p = .05) but not for ID severity. A high alkaline phosphatase (ALP) predicted lower vitamin D levels. Borderline significance (p = .06) in calcium levels between normal and high ALP was identified. There were no significant associations between parathyroid hormone, inorganic phosphate and magnesium levels, with vitamin D status or DEXA hip T-scores. Normalizing vitamin D levels (mean 101.4 nmol/L) required an average of 1951IU cholecalciferol daily. CONCLUSIONS: Vitamin D deficiency is highly prevalent in people with ID and epilepsy treated with ASMs impacting likely on their bone health. Screening with vitamin D levels, ALP and DEXA in this group should be pro-actively and routinely considered.

Epilepsy related multimorbidity, polypharmacy and risks in adults with intellectual disabilities: a national study.

BACKGROUND: A quarter of people with Intellectual Disability (ID) in the UK have epilepsy compared to 0.6% in the general population and die much younger. Epilepsy is associated with two-fifths of all deaths with related polypharmacy and multi-morbidity. Epilepsy research on this population has been poor. This study describes real-world clinical and risk characteristics of a large cohort across England and Wales. METHODS: A retrospective multi-centre cohort study was conducted. Information on seizure characteristics, ID severity, relevant co-morbidities, psychotropic and antiseizure drugs (ASDs), SUDEP and other risk factors was collected across a year. RESULTS: Of 904 adults across 10 centres (male:female, 1.5:1), 320 (35%) had mild ID and 584 (65%) moderate-profound (M/P) ID. The mean age was 39.9 years (SD 15.0). Seizures were more frequent in M/P ID (p < 0.001). Over 50% had physical health co-morbidities, more in mild ID (p < 0.01). A third had psychiatric co-morbidity and a fifth had an underlying genetic disorder. Autism Spectrum Disorder was seen in over a third (37%). Participants were on median two ASDs and overall, five medications. Over quarter were on anti-psychotics. Over 90% had an epilepsy review in the past year but 25% did not have an epilepsy care plan, particularly those with mild ID (p < 0.001). Only 61% had a documented discussion of SUDEP, again less likely with mild ID or their care stakeholders (p < 0.001). CONCLUSIONS: Significant levels of multi-morbidity, polypharmacy and a lack of systemised approach to treatment and risk exist. Addressing these concerns is essential to reduce premature mortality.

Attention-deficit hyperactivity disorder in people with intellectual disability: statistical approach to developing a bespoke screening tool.

BACKGROUND: Attention-deficit hyperactivity disorder (ADHD) is common among people with intellectual disability. Diagnosing ADHD in this clinically and cognitively complex and diverse group is difficult, given the overlapping psychiatric and behavioural presentations. Underdiagnoses and misdiagnoses leading to irrational polypharmacy and worse health and social outcomes are common. Diagnostic interviews exist, but are cumbersome and not in regular clinical use. AIMS: We aimed to develop a screening tool to help identify people with intellectual disability and ADHD. METHOD: A prospective cross-sectional study, using STROBE guidance, invited all carers of people with intellectual disability aged 18-50 years open to the review of the psychiatric team in a single UK intellectual disability service (catchment population: 150 000). A ten-item questionnaire based on the DSM-V ADHD criteria was circulated. All respondents' baseline clinical characteristics were recorded, and the DIVA-5-ID was administered blinded to the individual questionnaire result. Fisher exact and multiple logistic regressions were conducted to identify relevant questionnaire items and the combinations that afforded best sensitivity and specificity for predicting ADHD. RESULTS: Of 78 people invited, 39 responded (26 men, 13 women), of whom 30 had moderate-to-profound intellectual disability and 38 had associated comorbidities and on were medication, including 22 on psychotropics. Thirty-six screened positive for ADHD, and 24 were diagnosed (16 men, eight women). Analysis showed two positive responses on three specific questions to have 88% sensitivity and 87% specificity, and be the best predictor of ADHD. CONCLUSIONS: The three-question screening is an important development for identifying ADHD in people with intellectual disability. It needs larger-scale replication to generate generalisable results.

COVID-19 deaths in people with intellectual disability in the UK and Ireland: descriptive study.

BACKGROUND: Rapid spread of coronavirus disease 2019 (COVID-19) has affected people with intellectual disability disproportionately. Existing data does not provide enough information to understand factors associated with increased deaths in those with intellectual disability. Establishing who is at high risk is important in developing prevention strategies, given risk factors or comorbidities in people with intellectual disability may be different to those in the general population. AIMS: To identify comorbidities, demographic and clinical factors of those individuals with intellectual disability who have died from COVID-19. METHOD: An observational descriptive case series looking at deaths because of COVID-19 in people with intellectual disability was conducted. Along with established risk factors observed in the general population, possible specific risk factors and comorbidities in people with intellectual disability for deaths related to COVID-19 were examined. Comparisons between mild and moderate-to-profound intellectual disability subcohorts were undertaken. RESULTS: Data on 66 deaths in individuals with intellectual disability were analysed. This group was younger (mean age 64 years) compared with the age of death in the general population because of COVID-19. High rates of moderate-to-profound intellectual disability (n = 43), epilepsy (n = 29), mental illness (n = 29), dysphagia (n = 23), Down syndrome (n = 20) and dementia (n = 15) were observed. CONCLUSIONS: This is the first study exploring associations between possible risk factors and comorbidities found in COVID-19 deaths in people with intellectual disability. Our data provides insight into possible factors for deaths in people with intellectual disability. Some of the factors varied between the mild and moderate-to-profound intellectual disability groups. This highlights an urgent need for further systemic inquiry and study of the possible cumulative impact of these factors and comorbidities given the possibility of COVID-19 resurgence.

Guidance for the Treatment and Management of COVID-19 Among People with Intellectual Disabilities.

The current COVID-19 pandemic is a pressing world crisis and people with intellectual disabilities (IDs) are vulnerable due to disparity in healthcare provision and physical and mental health multimorbidity. While most people will develop mild symptoms upon contracting severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), some will develop serious complications. The aim of this study is to present guidelines for the care and treatment of people with IDs during the COVID-19 pandemic for both community teams providing care to people with IDs and inpatient psychiatric settings. The guidelines cover specific issues associated with hospital passports, individual COVID-19 care plans, the important role of families and carers, capacity to make decisions, issues associated with social distancing, ceiling of care/treatment escalation plans, mental health and challenging behavior, and caring for someone suspected of contracting or who has contracted SARS-CoV-2 within community or inpatient psychiatric settings. We have proposed that the included conditions recommended by Public Health England to categorize someone as high risk of severe illness due to COVID-19 should also include mental health and challenging behavior. There are specific issues associated with providing care to people with IDs and appropriate action must be taken by care providers to ensure that disparity of healthcare is addressed during the COVID-19 pandemic. We recognize that our guidance is focused upon healthcare delivery in England and invite others to augment our guidance for use in other jurisdictions.

Deprescribing of psychotropic medication in a 30-year-old man with learning disability.

A 30-year-old man with learning disability, living in a residential home with no documented mental health diagnosis, on the autistic spectrum was prescribed carbamazepine, olanzapine and propranolol to manage behaviour on a long-term basis. He was able to successfully discontinue both of his psychotropic medicines. The reduction was carried out in the community under the supervision of the learning disability psychiatrist. He is less tired, more alert and better able to express himself. He has expanded his activities and increased his access to the community. He is able to cope better with changes to his routine. His behaviours are well managed by the behavioural strategies in place and he has now been discharged by the psychiatrist to the general practitioner.