LEGAL ASPECTS OF CARE FOR THE MENTALLY RETARDED, MENTALLY DEFICIENT, AND MENTALLY ILL.
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In subjects with Fragile X Syndrome (FXS), the mutation of Fragile X Mental Retardation Type 1 ( FMR-1) gene at Xq27.3 predisposes to Mental Retardation (MR), autistic-like behaviour and to a variety of psychiatric syndromes. However, the longitudinal course of autistic-like behaviour profile and psychiatric morbidity is untested. In this study, we followed up people with FXS for 10 years to establish the stability of their autistic- like behaviour profile and psychiatric morbidity. The autisticlike behaviour profile was assessed using Brief Disability Assessment Schedule (B-DAS) and relevant items from Handicaps, Behaviour and Skills (HBS) Schedule. The psychiatric morbidity was assessed using data from the case notes, Mini Psychiatric Assessment Schedule for Adults with Developmental Disability (Mini PAS-ADD) and clinical interview. Our findings suggest that the autistic-like behaviour pattern is a stable phenotypic feature of FXS, but for increase in resistance to change over time. There is a tenfold increase in the prevalence of psychiatric morbidity in FXS compared to the general population, which does not increase significantly over time.
BACKGROUND: The prevalence of reflux oesophagitis (RO) in the normally intellectual population is about 2%, while this condition in the intellectually disabled has an estimated prevalence of 10%. METHODS: We investigated the presence of RO among 1687 intellectually disabled, with an IQ < 50, from 5 different institutes in the Netherlands. All were scored for possible associated factors and reflux symptoms, and compared with the overall population (n = 1580) from the same institutes (controls). Also, the effect of treatment on symptoms was evaluated after at least one year of therapy. RESULTS: Gastro-oesophageal reflux disease (GORD) was suspected clinically in 169 patients based on the following symptoms: vomiting, haematemesis, anaemia, rumination or behaviour problems. At endoscopy RO was diagnosed in 107 of 1687 patients (6.4%): 17 (15.9%) grade I, 34 (31.8%) gr. II, 42 (39.3%) gr. III and 14 (13.1%) gr. IV RO (Savary-Miller classification). Cerebral palsy, constipation, anticonvulsant drugs, an IQ < 35, underweight and gastrostomy feeding appeared to be possible associated factors, while as reflux symptoms persistent vomiting, haematemesis, iron deficiency anaemia, rumination, and behaviour problems were found. Concerning therapy, surgery was found to be effective in 38%, H2 receptor antagonists in 60% and the proton-pump inhibitor omeprazole in 96%. CONCLUSIONS: In this group of Dutch intellectually disabled patients with IQ < 50 RO was diagnosed in about 6% (107 of 1687), mostly severe grades of oesophagitis. Several possible associated factors were significantly present. From non-specific reflux symptoms persistent vomiting was the most indicative factor. In this population the most effective treatment of RO was long-term omeprazole therapy.
BACKGROUND: Based on the developing clinical and legal literature, and using the framework adopted in draft legislation, capacity to make a valid decision about a clinically required blood test was investigated in three groups of people with a 'mental disability' (i.e. mental illness (chronic schizophrenia), 'learning disability' ('mental retardation', or intellectual or developmental disability), or, dementia) and a fourth, comparison group. METHODS: The three 'mental disability' groups (N = 20 in the 'learning disability' group, N = 21 in each of the other two groups) were recruited through the relevant local clinical services; and through a phlebotomy clinic for the 'general population' comparison group (N = 20). The decision-making task was progressively simplified by presenting the relevant information as separate elements and modifying the assessment of capacity so that responding became gradually less dependent on expressive verbal ability. RESULTS: Compared with the 'general population' group, capacity to make the particular decision was significantly more impaired in the 'learning disability' and 'dementia' groups. Importantly, however, it was not more impaired among the 'mental illness' group. All the groups benefited as the decision-making task was simplified, but at different stages. In each of the 'mental disability' groups, one participant benefited only when responding did not require any expensive verbal ability. CONCLUSIONS: Consistent with current views, capacity reflected an interaction between the decision-maker and the demands of the decision-making task. The findings have implications for the way in which decisions about health care interventions are sought from people with a 'mental disability'. The methodology may be extended to assess capacity to make other legally-significant decisions.
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A screening of hearing and visual function was performed using clinical assessment methods in a Dutch institutionalized population of 672 people with mild to profound intellectual disability (ID). Because the studied population was not comparable to the total Dutch population with ID, subgroups were distinguished according to level of ID, age younger and older than 50 years, and the presence or absence of Down's syndrome (DS). The prevalences of both hearing and visual impairment were considerably increased in all subgroups, as compared with the general population. In the least affected group, i.e. those < 50 years with a mild or moderate ID by other causes than DS, the prevalences of hearing and visual impairment were 21% and 4%, respectively (as opposed to 2-7% and 0.2-1.9% in the general Dutch population <50 years, respectively). The prevalence of hearing impairment showed a sharp and highly significant increase in individuals with DS and subjects > or = 50 years. To a lesser extent, young adults with severe or profound ID had an increased risk of hearing impairment. Visual impairment and blindness were specifically highly prevalent in people with severe or profound ID (51% < 50 years of age). Down's syndrome and an age > or = 50 years were also significant risk factors for visual impairment. There was an alarmingly high prevalence of combined sensory impairment, especially in those with severe or profound ID (20%). Although hearing impairment had been diagnosed prior to this screen in 138 people and visual impairment in 65 individuals, a first diagnosis of hearing impairment was made in 128 subjects and of visual impairment in 90 cases. This highlights the tendency for sensory impairments to go unnoticed in people with ID, which is not restricted to those with severe or profound ID. Therefore, the present authors stress the importance of regular screening as outlined in the existing IASSID international consensus statement.
People with intellectual disabilities make up about 1% of the population of Europe. As trends toward community life advance, they have become more visible and more likely to access generic health systems. Yet evidence suggests that there are striking disparities between the health of this group and that of the general population. Increased longevity means that adults in this group expect to live longer lives and thus to encounter age-related risks for various health conditions. The 'Pomona' project, funded by the EU Health Monitoring Unit, aims to develop a set of health indicators for people with intellectual disabilities. It will build on the work accomplished by the team developing ECHI--European Community Health Indicators. This article outlines the rationale for the project, key elements in its implementation and expected outcomes.
Smoking policies and smoking practices of residents and employees in a state-supported residential system were investigated. Smoking policies existed in almost every agency, although state- and vendor-operated programs had vastly different policies. State-operated programs prohibited employees from smoking in residences and restricted residents who smoked to designated areas. One third of vendor-operated agencies allowed residents and staff members to smoke in designated areas, whereas one quarter stipulated that residences be smoke-free. Smoking among employees was higher and among residents lower than in the general population. The feasibility of specific approaches to reduce passive smoking and attitudes and beliefs about human rights issues related to smoking are presented.
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We evaluated an enriched teaching program for reducing resistance and indices of unhappiness displayed by 3 individuals with profound multiple disabilities during teaching sessions. The program involved presentation of preferred activities before, during, and after each teaching session, discontinuation of identified nonpreferred activities, and a brief break and preferred activity following occurrence of resistance. Implementation of the enriched teaching program was accompanied by reductions in resistance and indices of unhappiness for each participant. Results also indicated no negative impact of the program on participant performance of the teaching steps or staff teaching proficiency. Staff questionnaire responses provided a degree of social validation for the observed changes in that staff reported the participants liked the enriched teaching program more than the traditional teaching process. Directions for future research discussed include identifying critical aspects of the overall program and the potential relation between teaching proficiency and the program's enrichment effectiveness.
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