Effects of positive behaviour support delivered by direct staff on challenging behaviours and quality of life of adults with intellectual disabilities: A multicentre cluster-controlled trial.

BACKGROUND: Effects of staff provided positive behaviour support (PBS) for individuals with intellectual disabilities are unclear. METHOD: Using a multicentre non-randomised cluster controlled design, 26 teams of residential group homes, including 245 staff members of 167 individuals with intellectual disabilities, were allocated to a PBS or control group. Conducting multilevel analyses (n = 123) we examined individuals' changes in irritability, other challenging behaviours and quality of life. RESULTS: Compared to controls, irritability did not significantly decrease more in the intervention group, but lethargic behaviours did. Personal development and self-determination significantly increased. Irritability of individuals in the PBS group with higher levels of irritability or lower levels of intellectual disability significantly reduced more compared to controls. CONCLUSIONS: PBS was effective in reducing irritability of individuals with severe levels of irritability or intellectual disabilities. Moreover, PBS decreased lethargic behaviours and improved several domains of quality of life.

Preferred outcome measures in treatments for challenging behaviour in individuals with intellectual disabilities: Results of an inclusive Delphi method.

BACKGROUND: Interventions for challenging behaviours in individuals with intellectual disabilities benefit from outcome monitoring that takes clients' preferences into account. We determined clients' and representatives' preferred outcome domains and measures to secure their involvement in treatment decisions for challenging behaviours. METHOD: We used an inclusive Delphi method. A focus group of individuals with mild intellectual disabilities and representatives of those with moderate and severe intellectual disabilities prepared the first round by assisting us in collecting possible outcomes. Panels of individuals with intellectual disabilities and representatives were composed to achieve consensus on instruments for preferred outcome domains. RESULTS: Preferred outcome domains were behaviour, side-effects of psychotropic drugs, quality of life, daily functioning, caregiver burden and family quality of life. Corresponding outcome measures included self-report, interview and proxy-scales, including spoken versions. CONCLUSION: Including the preferred domains on outcomes of interventions for challenging behaviours is recommended. Research on corresponding outcome measures is necessary.

Exploring the association of staff characteristics with staff perceptions of quality of life of individuals with intellectual disabilities and challenging behaviours.

BACKGROUND: This study aimed to examine the associations between individual staff and staff team characteristics and quality of life of individuals with intellectual disabilities and challenging behaviours. METHOD: With multilevel analyses, we examined educational level, experience, attitudes and behaviours of 240 staff members, in relation to their perception of quality of life of 152 individuals with intellectual disabilities and challenging behaviours they cared for. RESULTS: Two individual staff characteristics were related to better quality of life: higher educational and self-reflection levels. Of the team characteristics, higher educational level, higher self-efficacy and more friendly behaviour were associated with better quality of life. Unexpectedly, higher staff-individual ratio was related to lower quality of life. CONCLUSIONS: Both individual staff and staff team characteristics are associated with quality of life, indicating the need to take staff team characteristics into account when examining quality of life.

An open label discontinuation trial of long-term used off-label antipsychotic drugs in people with intellectual disability: The influence of staff-related factors.

BACKGROUND: Results of discontinuation of antipsychotics in people with intellectual disability are variable and may depend on staff factors. METHOD: We attempted to taper off antipsychotics in 14 weeks after which participants were free to restart. We investigated the influence of support professionals' feelings towards challenging behaviour, their knowledge of psychotropic drugs and clinicians' judgements of participants' behavioural functioning on whether or not antipsychotics were completely discontinued after 16, 28 and 40 weeks. RESULTS: Of the 129 participants, 61% achieved discontinuation at 16 weeks; at 28 and 40 weeks, 46% and 40% were completely discontinued. Staff's feelings of Depression/Anger towards their client's behaviour, less knowledge about psychotropic medication and clinicians' judgements of behavioural worsening were negatively associated with achievement of discontinuation. CONCLUSIONS: To enhance discontinuation off-label drug use, staff's feelings should be explored, their knowledge of psychotropic drugs improved and reasons for clinicians' judgements of participants' behavioural worsening investigated.

Exploring barriers and facilitators in the implementation and use of guideline recommendations on antipsychotic drug prescriptions for people with intellectual disability.

BACKGROUND: It is unknown why professionals' adherence to guideline recommendations on antipsychotic drug prescription in the intellectual disabilities care is insufficient. This study aimed to explore barriers and facilitators in the implementation and use of these recommendations. METHODS: In-depth interviews with four intellectual disability physicians, two psychiatrists and five behavioural scientists were used to explore the implementation and use of guideline recommendations. RESULTS: Barriers in adhering to recommendations were lack of collaboration of different disciplines involved in the treatment of psychiatric and behavioural disorders and lack of enforcement of an appropriate monitoring of side- and treatment effects. When guideline recommendations were translated into organizational policies, clinicians were able to divide responsibilities and tasks, needed to appropriately implement guideline recommendations in daily clinical practice. CONCLUSIONS: To facilitate the use of guideline recommendations, organizations should translate recommendations into organization-specific policies, involving physicians and behavioural scientists in this process, while simultaneously creating more practical and technological support.

Knowledge and expectations of direct support professionals towards effects of psychotropic drug use in people with intellectual disabilities.

BACKGROUND/INTRODUCTION: In this study, we investigated intellectual disability support professionals' knowledge and expectations towards effects of psychotropic drug use on behaviour and drug use in their clients, because shortcomings may lead to misinterpretations of behavioural symptoms and inappropriate drug use. METHODS: Two self-designed questionnaires were used to measure the knowledge and expectations of 194 support professionals in 14 residential facilities regarding psychotropic drug use and effects of antipsychotics on behavioural, cognitive and mental functioning of people with intellectual disability. The psychometric properties of both questionnaires were adequate. RESULTS: A majority of the professionals had unrealistic expectations regarding the positive effects of antipsychotics on cognitive and behavioural functioning, and 94% scored below the cut-off scores regarding knowledge; 60% indicated they needed education and training. CONCLUSIONS: To achieve sufficient collaboration of intellectual disability support professionals in reducing inappropriate psychotropic drug use of clients, vocational educational training is needed.

Assessment of Drug-Associated Extrapyramidal Symptoms in People With Intellectual Disability: A Comparison of an Informant-Based Scale With Clinical Rating Scales.

Drug-associated extrapyramidal symptoms (EPS) in people with intellectual disability (ID) may be difficult to recognize, and clinicians' assessments may be hampered by lack of patients' capacities to adequately cooperate and by lack of reliable instruments to measure EPS in this population. Therefore, we compared assessments based on professional caregivers' observations with the informant-based validated Matson Evaluation of Drug Side Effects (MEDS) scale with assessments by clinicians using a set of clinical rating scales, most of which have not been validated for use in this population. We also compared 2 dyskinesia scales by replacing the widely used but not validated Abnormal Involuntary Movement Scale with the validated Dyskinesia Identification System Condensed User Scale (DISCUS) in half of the set of scales. We used linear regression to analyze associations between EPS as measured with MEDS and EPS as measured with the sets of scales at item and at scale level.Of the 30 MEDS items, 6 were associated with items of the other scales. At scale level, we found no significant associations. Comparison of the Abnormal Involuntary Movement Scale with the DISCUS indicated that the DISCUS may be preferable for use in people with ID.Results may be explained by shortcomings in education and training of caregivers and by lack of reliable assessments and rating scales for EPS in people with ID.We conclude that there is an urgent need for education and training of care professionals and clinicians in this area and for studies investigating the psychometric properties of rating scales.

Integrative Care for Challenging Behaviors in People with Intellectual Disabilities to Reduce Challenging Behaviors and Inappropriate Psychotropic Drug Prescribing Compared with Care as Usual: A Cluster-Randomized Trial.

People with intellectual disabilities (IDs) often present with challenging behaviors (CBs) mostly due to inappropriate environments and mental and physical disorders. Integrative care is recommended to address CBs. However, in clinical practice, psychotropic drugs are often prescribed off-label for CBs, although the effectiveness is unclear, and side effects frequently occur. We conducted a cluster-randomized controlled study to investigate the effect of integrative care provided by a collaboration of an ID specialized mental healthcare team and participants' own ID service providers' care team on reducing CBs and inappropriate off-label psychotropic drug prescriptions compared with care as usual. Participants (N = 33, aged 19-81 years) had a moderate, severe, or profound intellectual disability and used off-label psychotropic drugs. The primary outcome measures were the Aberrant Behavior Checklist and the total dose of psychotropic drug prescriptions. At the study endpoint of 40 weeks, we found no effect of the intervention on the total ABC score and on the total dose of psychotropic drug prescriptions. In the intervention group, however, the psychotropic drug dose decreased significantly, while CBs did not change. The small sample size and not-completed interventions due to organizational problems may have affected our findings. This study illustrates the difficulties in the implementation of integrative care.

Effects of controlled discontinuation of long-term used antipsychotics on weight and metabolic parameters in individuals with intellectual disability.

Antipsychotics are frequently prescribed agents in individuals with intellectual disability, often for behavioral symptoms. Efficacy of antipsychotics for this is ambiguous, so discontinuation should be considered. Weight gain and metabolic dysregulation are well-known adverse effects of antipsychotics which increase the risk of the metabolic syndrome. We performed a discontinuation study in 99 adults with intellectual disability, living in residential facilities who used antipsychotics for behavioral symptoms for more than 1 year. The aim of the present study was to investigate the effects of discontinuation of long-term used antipsychotics on weight, body mass index (BMI), and parameters of the metabolic syndrome and to investigate the influence of genetic polymorphisms and medication factors on these outcomes. Discontinuation of antipsychotics led to a mean decrease of 4 cm waist circumference, of 3.5 kg weight, 1.4 kg/m2 BMI, and 7.1 mm Hg systolic blood pressure. In those participants who had not completely discontinued use of antipsychotics we found a decrease in weight and BMI and an increase in fasting glucose. The presence of the C-allele of serotonin 5-hydroxytryptamine receptor polymorphism rs141334 was associated with higher waist circumference and higher plasma levels of triglycerides and lower levels of high-density lipoprotein. Achievement of complete discontinuation predicted a larger decrease in waist circumference and BMI. In conclusion, results of the study show the beneficial effects of discontinuation of long-term used antipsychotics on metabolic outcomes.

Difficulties in Addressing Diagnostic, Treatment and Support Needs in Individuals with Intellectual Disability and Persistent Challenging Behaviours: A Descriptive File Study of Referrals to an Expertise Centre.

Service providers may experience difficulties in providing appropriate care to optimize the functioning of individuals with intellectual disability and challenging behaviour. External consultation to identify and address the unmet support needs underlying the behaviour may be beneficial. Applying the multidimensional American Association Intellectual and Developmental Disabilities (AAIDD) model may facilitate this approach. We aimed to describe the content and outcomes of consultation for individuals with intellectual disability and challenging behaviour referred to the Dutch Centre for Consultation and Expertise in relation to the AAIDD model. Interventions were based on the clients' diagnostic, treatment, and support needs and were categorized according to the five dimensions of the AAIDD model. Outcomes of the consultations were assessed based on reports in the file and rated as 'clear improvement', 'improvement' or 'no improvement or deterioration'. In two-thirds of the 104 studied files, consultees were satisfied with the improvement in functioning. Interventions targeted the difficulties of the service providers in supporting their clients and were most often applied within the Health and Context dimensions of the AAIDD model. We may conclude that consultation of an expert team may be valuable to support the care providers, and the use of the AAIDD model may be helpful to address the unmet needs to improve the functioning of individuals with challenging behaviour.

Withdrawing Antipsychotics for Challenging Behaviours in Adults with Intellectual Disabilities: Experiences and Views of Experts by Experience.

People with intellectual disabilities (PwID) are frequently prescribed long-term antipsychotics for behaviours that challenge (BtC) despite the lack of proven effectiveness and the increased risks for side effects of these medications in this population. National and international good clinical practice guidelines recommend deprescribing antipsychotics for BtC, which is often not successful due to environmental and other factors. The involvement of all stakeholders, including PwID, is crucial for deprescribing. However, studies showed that PwID and/or their families are often not involved in decision-making regarding the (de)prescribing of antipsychotics despite their desire to get involved. Moreover, studies on the views of PwID regarding their experiences of withdrawing from antipsychotics are lacking. The aim of this study was to gain insight into the views of PwID by investigating their experiences of discontinuation of long-term prescribed antipsychotics for BtC. A qualitative study was set up. Seven experts by experience with mild intellectual disabilities were interviewed. After six interviews, data saturation was achieved. Interviews were transcribed verbatim. Using phenomenological analysis, themes on lived experiences were extracted. Each consecutive interview was analysed. The four main themes extracted from the interviews were the quality of treatment, knowledge and information about psychotropics and the process of withdrawal, support from the participants' environment and the coping style of the interviewees themselves.

Withdrawing Antipsychotics for Challenging Behaviours in Adults with Intellectual Disabilities: Experiences and Views of Prescribers.

International current best practice recommends the discontinuation of antipsychotics for challenging behaviours in people with intellectual disabilities (ID), due to lack of evidence of efficacy and risks of harmful side-effects. In clinical practice, discontinuation may be difficult. The aim of this study was to gain insight into prescribers' practice by investigating their experiences with the discontinuation of long-term antipsychotics for challenging behaviour. From professionals' associations thirty-four registered ID physicians, psychiatrists and specialist mental healthcare nurses were recruited who completed an online questionnaire in this survey-study. Almost all participants had attempted to deprescribe antipsychotics for their patients with ID. Sixty-five percent of participants achieved complete discontinuation in 0-25% of their patients, but none in over 50%. Barriers were a lack of non-pharmaceutical treatments for challenging behaviours and caregivers' and/or family concern. Seventy percent of participants indicated that their institutions had encouraged implementing their discontinuation policies in line with the new Dutch Act on Involuntary care and a new Dutch multidisciplinary guideline on problem behaviour in adults with ID. Support and facilitation of clinicians from institutions' managers and political and professional bodies may be helpful in further implementation of best practice in the treatment of challenging behaviour in people with ID.

Use of antipsychotic drugs in individuals with intellectual disability (ID) in the Netherlands: prevalence and reasons for prescription.

BACKGROUND: We investigated antipsychotic drug prescription practice of Dutch ID physicians, studying prevalence of antipsychotic drug use, reasons for prescription and the relationship between these reasons and patient characteristics. METHODS: A cross-sectional study of medical and pharmaceutical records in a population living in residential settings of three care providers for persons with IDs in the Netherlands (n = 2373). RESULTS: Prevalence of antipsychotic drug use was 32.2% (95% CI 30.1-33.9). Behavioural problems were the reason for prescription of antipsychotic drugs in 58% of cases and psychotic disorder or psychotic symptoms in 22.5%. In 11.7% the diagnosis of psychotic disorder was specified according to DSM-IV criteria. In 18.5% the reason for prescription was not noted in the medical record. Behavioural problems as reason for prescription was associated with profound and severe ID, living in a central location and male sex. Psychotic disorder specified according to DSM-IV as indication for prescription was negatively associated with profound and severe ID and with presence of an additional mental disorder. Absence of a noted reason for prescription was associated with female sex and with the presence of an additional mental disorder. DISCUSSION: Current prevalence and reason for prescription of antipsychotic drugs are similar with outcomes of previous studies. Our results show the continuing lack of evidence-based psychopharmacological treatment in mental health care for persons with IDs.

Changes in Health-Related Quality of Life in People With Intellectual Disabilities Who Discontinue Long-Term Used Antipsychotic Drugs for Challenging Behaviors.

Health-related quality of life in people with intellectual disabilities can be affected by challenging behaviors and side effects of antipsychotics. The aim of this study was to evaluate the effect of discontinuation antipsychotic drugs on health-related quality of life, including data from 2 discontinuation trials: an open-label trial of various antipsychotic drugs and a double-blind trial of risperidone. In both studies, antipsychotics were discontinued in 14 weeks, with steps of 12.5% of the baseline dosage every 2 weeks. Health-related quality of life was measured at baseline and at 16 weeks, and 40 weeks after baseline, by means of the RAND-36 (domains on physical well-being, role limitations caused by physical or emotional problems, vitality, pain, mental well-being, social functioning, general health, and changes in health). Participants who had completely discontinued antipsychotics according to the scheduled discontinuation and were still free of use at 40 weeks were compared with those who had incompletely discontinued. Physical well-being showed an increase in the group that had achieved complete discontinuation. Social functioning showed a decrease in the group that incompletely discontinued, which recovered at follow-up. Mental well-being decreased at 16 weeks but recovered at follow-up, regardless of complete or incomplete discontinuation. To conclude, discontinuation of antipsychotics had a positive effect on physical well-being when complete discontinuation was possible. When complete discontinuation was not possible, there was a negative effect on health-related quality-of-life domains. However, none of the unfavorable effects were irreversible.

An Open-Label Discontinuation Trial of Long-Term, Off-Label Antipsychotic Medication in People With Intellectual Disability: Determinants of Success and Failure.

Although physicians are aware of the risks of prescribing long-term off-label antipsychotics in people with intellectual disability, attempts to discontinue often fail. This study aimed to identify potential determinants of successful and failed discontinuation. Long-term off-label antipsychotics were tapered in 14 weeks, with 12.5% of baseline dose every 2 weeks. Participants living in facilities offered by intellectual disability service providers, ≥6 years, with an IQ <70 were eligible to discontinue antipsychotic use, as judged by their physicians. The primary outcome was achievement of complete discontinuation at 16 weeks; changes in the Aberrant Behavior Checklist (ABC) and its 5 subscales were secondary outcomes. Potential determinants of the success or failure in discontinuing antipsychotics were psychotropic drug use and participants' living circumstances, medical health conditions, and severity of behavioral symptoms and neurologic side effects. Of 499 eligible clients, 129 were recruited. Reasons for client non-participation were clinician concerns that discontinuation might increase challenging behaviors and changes in clients' environment. Of the 129 participants, 61% had completely discontinued antipsychotics at 16 weeks, 46% at 28 weeks, and 40% at 40 weeks. ABC total scores increased in 49% of participants with unsuccessful discontinuation at 16 weeks. Autism, higher dose of antipsychotic drug, higher ABC and akathisia scores, and more-frequent worsening of health during discontinuation were associated with a lower incidence of complete discontinuation. Thus, in a selected sample of participants whom responsible clinicians had deemed discontinuation of antipsychotics could be attempted, 40% had achieved and maintained discontinuation at end of follow-up. Physicians should try to address patients' conditions that may hamper discontinuation.

Health-related quality of life in people with intellectual disability who use long-term antipsychotic drugs for challenging behaviour.

BACKGROUND: Many people with intellectual disabilities use long-term antipsychotics for challenging behaviour and experience side-effects from these drugs, which may affect Health-related Quality of Life (HQoL). AIMS: This study aimed to investigate HQoL in people with intellectual disabilities who use long-term antipsychotics and to investigate its associations with challenging behaviour and physical symptoms often associated with antipsychotics. MATERIALS AND METHODS: We used baseline data of two studies of long-term used antipsychotics. The RAND-36 and the emotional and physical wellbeing subscales of the Personal Outcome Scale (POS) were used to assess HQoL. Associations with challenging behaviour, measured with the Aberrant Behavior Checklist (ABC) and physical symptoms (extrapyramidal, autonomic, metabolic) with HQoL outcomes were analysed by univariate and multivariate linear regression. RESULTS: The mental subscales of the RAND-36 and emotional wellbeing of the POS were associated with the irritability and lethargy ABC-subscales. Physical wellbeing was negatively associated with parkinsonism urinary problems, dysphagia and temperature dysregulation possibly due to antipsychotics use. CONCLUSION: Both mental and physical wellbeing are related to challenging behaviour and physical symptoms associated with antipsychotics. Therefore HQoL could be a helpful measure when balancing benefits and disadvantages of antipsychotics prescribed for challenging behaviour.

[Challenging behaviour in patients with intellectual disability]. / Gedragsproblemen bij mensen met een verstandelijke beperking.

Challenging behaviour in patients with intellectual disability may be caused by physical, psychological and environmental factors. In 3 case histories, a multidisciplinary assessment in diagnosis and treatment is shown to be needed. A 48-year-old man with mild intellectual disability presented with cognitive deterioration, aggressive behaviour and physical symptoms. The diagnosis was mood disorder and dissociative symptoms. His functioning improved during and after treatment with psychotropic drugs and non-verbal psychotherapy. A 10-year-old girl with moderate intellectual disability who presented with temper tantrums was diagnosed as having no psychiatric disorder, but symptoms caused by environmental factors. After proper support and education for the parents, the patient's challenging behaviour disappeared. A 61-year-old woman with Down syndrome and Alzheimer's dementia presented with screaming, restlessness, struggling during daily care, and confusion. She was prescribed haloperidol and oxazepam and the screaming behaviour decreased. However, she kept on struggling while being cared for and was restless while sitting in her wheelchair. After a proper medical examination it appeared that the problem behaviour was caused by collapsed vertebrae and osteoporosis.

Functional analysis of novel genetic variation in the thyroid hormone activating type 2 deiodinase.

CONTEXT: Thyroid hormones (TH) are important for normal brain development and abnormal TH regulation in the brain results in neurocognitive impairments. The type 2 deiodinase (D2) is important for local TH control in the brain by generating the active hormone T3 from its precursor T4. Dysfunction of D2 likely results in a neurocognitive phenotype. No mutations in D2 have been reported yet. OBJECTIVE: The objective of the study was to identify D2 mutations in patients with intellectual disability and to test their functional consequences. DESIGN, SETTING, AND PATIENTS: The patients were selected from the multicenter Thyroid Origin of Psychomotor Retardation study, which is a cohort of 946 subjects with unexplained intellectual disability. Based on characteristic serum TH values, the coding region of the DIO2 gene was sequenced in 387 patients. Functional consequences were assessed by in vitro D2 assays or intact cell metabolism studies using cells transfected with wild-type or mutant D2. RESULTS: Sequence analysis revealed two heterozygous mutations: c.11T>A (p.L4H) in three subjects and c.305C>T (p.T102I) in one subject. Sequence analysis of family members revealed several carriers, but no segregation was observed with thyroid parameters or neurocognitive phenotype. Extensive tests with different in vitro D2 assays did not show differences between wild-type and mutant D2. CONCLUSION: This study describes the identification and functional consequences of novel genetic variation in TH activating enzyme D2. Family studies and functional tests suggest that these variants do not underlie the neurocognitive impairment. Altogether our data provide evidence of the existence of rare but apparently harmless genetic variants of D2.

Determinants of physical health parameters in individuals with intellectual disability who use long-term antipsychotics.

Individuals with intellectual disability frequently use antipsychotics for many years. This may have detrimental health effects, including neurological symptoms and metabolic and hormonal dysregulation, the latter possibly affecting bone metabolism. There is large variability in the degree in which antipsychotic agents lead to these health problems. In the current study we investigated potential determinants of physical symptoms and biological parameters known to be associated with use of antipsychotics in a convenience sample of 99 individuals with intellectual disability who had used antipsychotics for more than one year for behavioural symptoms. We focused on extrapyramidal symptoms; on overweight and presence of components of the metabolic syndrome; and on elevated plasma prolactin and bone turnover parameters. As predictor variables, we used patient (sex, age, genetic polymorphisms, and severity of intellectual disability) and medication use (type and dosage) characteristics. We found extrapyramidal symptoms to be present in 53%, overweight or obesity in 46%, and the metabolic syndrome in 11% of participants. Hyperprolactineaemia and one or more elevated bone turnover markers were present in 17% and 25%, respectively. Higher age and more severe intellectual disability were associated with dyskinesia and a higher dosage of the antipsychotic drug was associated with parkinsonism. Less severe intellectual disability was related to higher Body Mass Index. Use of atypical antipsychotics was associated with higher diastolic blood pressure and elevated fasting glucose. Clinicians who prescribe antipsychotics in individuals with intellectual disability should carefully balance the potential benefits of prolonged treatment against the risk of health hazards associated with the use of antipsychotics.