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To update the 2006 Canadian guidelines for primary care of adults with developmental disabilities (DD) and to make practical recommendations based on current knowledge to address the particular health issues of adults with DD.
Knowledgeable health care providers participating in a colloquium and a subsequent working group discussed and agreed on revisions to the 2006 guidelines based on a comprehensive review of publications, feedback gained from users of the guidelines, and personal clinical experiences. Most of the available evidence in this area of care is from expert opinion or published consensus statements (level III).
Adults with DD have complex health issues, many of them differing from those of the general population. Good primary care identifies the particular health issues faced by adults with DD to improve their quality of life, to improve their access to health care, and to prevent suffering, morbidity, and premature death. These guidelines synthesize general, physical, behavioural, and mental health issues of adults with DD that primary care providers should be aware of, and they present recommendations for screening and management based on current knowledge that practitioners can apply. Because of interacting biologic, psychoaffective, and social factors that contribute to the health and well-being of adults with DD, these guidelines emphasize involving caregivers, adapting procedures when appropriate, and seeking input from a range of health professionals when available. Ethical care is also emphasized. The guidelines are formulated within an ethical framework that pays attention to issues such as informed consent and the assessment of health benefits in relation to risks of harm.
Implementation of the guidelines proposed here would improve the health of adults with DD and would minimize disparities in health and health care between adults with DD and those in the general population.
Mettre à jour les lignes directrices canadiennes de 2006 sur les soins primaires aux adultes ayant une déficience développementale (DD) et présenter des recommandations pratiques fondées sur les connaissances actuelles pour traiter des problèmes de santé particuliers chez des adultes ayant une DD.
Des professionnels de la santé expérimentés participant à un colloque et un groupe de travail subséquent ont discuté et convenu des révisions aux lignes directrices de 2006 en se fondant sur une recherche documentaire exhaustive, la rétroaction obtenue des utilisateurs du guide de pratique et les expériences cliniques personnelles. La plupart des preuves disponibles dans ce domaine viennent de l’opinion d’experts ou de déclarations consensuelles publiées (niveau III).
Les adultes ayant une DD ont des problèmes de santé complexes, dont plusieurs diffèrent de ceux de la population en général. De bons soins primaires permettent d’identifier les problèmes de santé particuliers dont souffrent les adultes ayant une DD pour améliorer leur qualité de vie et leur accès aux soins de santé et prévenir la morbidité et le décès prématuré. Ces lignes directrices résument les problèmes de santé générale, physique, comportementale et mentale des adultes ayant une DD que devraient connaître les professionnels des soins primaires et présentent des recommandations pour le dépistage et la prise en charge en se basant sur les connaissances actuelles que les cliniciens peuvent mettre en pratique. En raison de l’interaction des facteurs biologiques, psychoaffectifs et sociaux qui contribuent à la santé et au bien-être des adultes ayant une DD, ces lignes directrices insistent sur la participation des aidants, l’adaptation des interventions, au besoin, et la consultation auprès de divers professionnels de la santé quand ils sont accessibles. Elles mettent aussi en évidence la nature éthique des soins. Les lignes directrices sont formulées dans le contexte d’un cadre éthique qui tient compte des questions comme le consentement éclairé et l’évaluation des bienfaits pour la santé par rapport aux risques de préjudice.
La mise en œuvre des lignes directrices proposées ici améliorerait la santé des adultes ayant une DD et minimiserait les disparités sur les plans de la santé et des soins de santé entre les adultes ayant une DD et la population en général.
The terms developmental disabilities (DD) or intellectual disabilities are used synonymously in Canada (equivalent to learning disabilities in the United Kingdom) to refer to a range of conditions in which lifelong limitations in intellectual functioning and conceptual, social, and practical skills are noticeable before age 18 years.1 Estimates of the prevalence of DD vary between 1% and 3% of Canadians. Most reside and receive health care in the community.2 A growing proportion of them are living longer than in the past.2,3 Their health needs and access to primary care vary individually with factors such as the etiology of their DD, coexisting physical and mental health characteristics, severity of functional limitations, quality of environment and social supports, and age.4
Health disorders in people with DD frequently differ from those encountered in the general population in terms of prevalence, age of onset, rate of progression, degree of severity, and presenting manifestations. These disorders are also more likely to be multiple and complex in those with DD.3 They therefore require the support of health professionals who are willing, knowledgeable, and skilled to address their particular challenges and vulnerabilities in maintaining health.
Various studies in Canada, the United States, the United Kingdom, and Australia have shown that people with DD, as a group, are poorly supported by health care systems.5 The United Nations’ Convention on the Rights of Persons with Disabilities,6 which has been ratified by Canada, includes the right to health care. Primary care providers are the lynchpin in efforts to ameliorate health care for people with DD. Primary care providers are often the most consistently available health professionals involved in caring for people with DD and in interacting with regular caregivers. Their contribution is vital for disease prevention, early detection, and appropriate management. They can help to assess the need for referral to specialized and interdisciplinary health services when these are available. They also provide continuity and coordination of care. Reliable guidelines, however, are required to inform primary care providers about the particular health needs of people with DD and the best approaches to management.
There is an especially pressing need for such guidelines concerning adults with DD. Adulthood, usually after 18 years of age, is when people with DD are no longer deemed eligible for pediatric and adolescent services, although this age varies provincially in Canada. In the Canadian health care system, service gaps resulting from transition to the adult care system, which generally has fewer resources and is less specialized and more fragmented than the pediatric and adolescent care systems, present enormous challenges to adults with DD and their caregivers. These challenges are complicated by recent increases in life expectancy and the aging of people with DD, and by their integration into the community. Thus, while more people with DD are moving into the adult care system than in the past, there are insufficient numbers of knowledgeable and experienced primary care providers to support them. To compound the situation, there have been fewer publications addressing the screening, assessment, and management of health disorders and challenges of adults with DD, relative to such publications for infants and children with such disabilities.
In 2005, a consensus colloquium involving knowledgeable and experienced clinicians and researchers in DD from across Canada and abroad formulated the “Consensus Guidelines for Primary Health Care of Adults with Developmental Disabilities” (hereafter referred to as the 2006 Guidelines).7 Subsequent consultations with users of the 2006 Guidelines, as well as developments in research and practice, led to expanding and updating those guidelines, which remain, to our knowledge, the only comprehensive guidelines for the primary care of adults with DD in Canada. References to various clinical tools that might help in applying the guidelines have also been added.
A consensus development method was used to update the 2006 Guidelines. This consisted of 2 steps: meticulous electronic and manual searches for relevant publications and discussion of recommended changes to the 2006 Guidelines by knowledgeable and experienced Canadian clinicians and researchers on primary health care of adults with DD who participated in a consensus colloquium in March 2009 and in a subsequent working group.
A librarian familiar with research on DD undertook ongoing, comprehensive electronic searches in PubMed and PsycINFO for publications in English from 1990 to 2010 that were indexed under, or contained in their title, abstract, or text, the terms mental retardation, intellectual disability (disabilities), or developmental disability (disabilities). Publications from Great Britain were also searched for the terms learning difficulties, learning disability (disabilities), or learning disorders. These were cross-referenced with a long list of physical and mental health key words relating to medical assessment, diagnosis, treatment, prognosis, health care access, need, planning, services, and delivery. The search was then expanded to include specific health issues highlighted in the 2006 Guidelines. In addition, the librarian undertook manual searches using cited references in Scopus and Internet searches for relevant publications that had not been indexed by any of the above-mentioned electronic databases.
Search results were downloaded to and organized in an electronic database management system known as RefWorks. Two family physicians, a psychologist, and a psychiatrist drew on this database and on comments regarding the 2006 Guidelines gained from various users and reviewers. They were each assigned a section of the 2006 Guidelines for which they were to propose revisions.
Participants who helped to formulate the 2006 Guidelines and others who had completed training courses on the guidelines between 2006 and 2009 were invited to a day-long colloquium in Toronto, Ont, in March 2009. Among the 39 participants were practitioners in family medicine, nursing, pediatrics, psychiatry, psychology, occupational therapy, and speech-language pathology. Before the colloquium, all had access to the librarian’s entries into the RefWorks database and received a summary of feedback from users and reviewers of the 2006 Guidelines. The prepared proposals for revisions were discussed in small groups and in plenary sessions, and a summary of accepted revisions was presented and discussed at the end of the colloquium in relation to the priority criteria adopted in the 2006 Guidelines (Table 1).8 A working group consisting of 7 participants, with a family physician in the leading role, met monthly between March 2009 and March 2010 to draft the first version of the updated guidelines. They incorporated into the 2006 Guidelines the changes discussed and accepted during the colloquium. This working group also reviewed published supporting evidence for all the guidelines, including those from supplementary electronic and manual searches for publications undertaken after the colloquium to address particular issues that were not foreseen in the original literature searches. The working group judged the level of evidence supplied for any modified or new guidelines, using the classification scheme adopted in the 2006 Guidelines (Table 2).
The first draft of the updated guidelines was circulated for review by participants in the colloquium as well as several invited consultants who were unable to attend the colloquium. Based on the feedback received, the working group prepared the second and final draft between March and October of 2010. This version was sent to participants in the colloquium and review process for their approval; it was then submitted for review for publication.
On the whole, there has been substantial elaboration and rewording of most of the 2006 Guidelines, with attention paid to rendering the considerations and recommendations easier to understand and apply.
Compared with the 2006 Guidelines, the focus of the updates is more clearly on health conditions and needs of adults with DD that diverge from those of the general population. Although primary care guidelines that have been found effective for preventing diseases in the general population should normally also be applied to people with DD, the guidelines in Table 31,2,9–180 provide additional recommendations and appropriate modifications to standard practice that are relevant for adults with DD. However, some recommendations that apply to the general population have been included in the updated guidelines either because they pertain to tests and other interventions from which adults with DD tend to be excluded or because there is inadequate evidence-based guidance specific to adults with DD.
Many disparities and challenges encountered by adults with DD in primary care stem not only from the paucity of information available to care providers but also from attitudes and practices that fall short of respecting the dignity of people with DD. The selection of updates was guided by the ethical framework adopted for the 2006 Guidelines, which emphasized respect for the dignity of adults with DD throughout their lives, the importance of their care-giving relationships and communities, and the need to take into account the health issues particular to them, individually and as a group. Thus, the 2006 Guidelines regarding informed and voluntary consent (guideline 7 in Table 3)1,2,9–180 and advanced care planning (guideline 8) were amplified. The value of consulting, educating, and enlisting the support of caregivers underlies many updated recommendations. In making recommendations for management of particular health conditions of adults with DD, consideration was given to what would most likely benefit the overall health and well-being of these adults while involving the least possible risk of restrictions, harmful side effects, distress, and other burdens. This framework was relevant, for example, when considering decreasing the recommended frequency of tests in the 2006 Guidelines, such as those for glaucoma and thyroid disorder (in guidelines 11b and 19a), when it was unlikely to result in any substantial difference in detection rates. In other cases, references to tools that improve communication, adapt standard test procedures, or minimize the distress from interventions experienced by adults with DD were added to specific recommendations.
A new guideline on detecting pain and distress (guideline 4) was added, as pain and distress can manifest in atypical ways in adults with DD (eg, different physical cues or changes in behaviour).
Furthermore, new guidelines were included for screening and prevention of infectious diseases (guideline 20), cancer (guideline 21), and alcohol or drug abuse (guideline 30), because it has been shown that adults with DD are less likely than those in the general population to be included in preventive screening programs, to do self-examinations, or to report abnormalities or difficulties in these areas.4
The behavioural and mental health guidelines contain substantially more detail than in the 2006 Guidelines, with new categories added to address nonpharmaceutical interventions (guideline 26) and psychotropic and antipsychotic medications (guidelines 27 and 28). An important new recommendation (guideline 28a) rejects the routine use of antipsychotic medications for problem behaviour, specifically aggressive challenging behaviour, without a confirmed robust diagnosis of schizophrenia or other psychotic disorder.131
The aim of these guidelines is to inform primary care providers of the most prevalent health issues of adults with DD as a group and of the best approaches to management. However, any such set of guidelines will always be limited in their application by the reality that adults with DD are not a homogeneous group nor do they experience health disorders in the same way. These guidelines are not meant to replace attentive observation and prudent clinical decisions. The most appropriate care for an adult with DD takes into account relevant factors in his or her particular circumstances. Furthermore, although these guidelines are generally applicable to adults with DD, primary care providers will need to address additional specific health issues when there is a known cause of DD.
The division of these guidelines into distinct physical, behavioural, and mental health categories was intended to facilitate their application. Several guidelines, however, address the interaction of physical factors with behavioural and mental health ones, and between these and environmental factors and other determinants of health. More research is needed into such interactions and their implications for the deployment of an interdisciplinary and holistic approach to primary care of adults with DD. A helpful advance for researchers has been the POMONA Project’s identification of 18 measurable health indicators and the development of a survey tool for gathering health data for adults with DD across 14 European countries.181,182
Some of these updated guidelines recommend the use of resources and specialized services that, while generally available in Canada, might be lacking or inaccessible in some regional health service systems. In such circumstances, it is necessary to adapt these guidelines to allow primary care providers to provide a reasonable standard of care and to develop practical resource-sharing strategies (eg, using clinical video-conferencing).
Further study of the effect of the guidelines on improving primary care of adults with DD and their health outcomes is essential. The extent to which they are applied must be assessed, and when they are not used the reasons need to be determined. It is likely that a comprehensive approach involving the training of primary care providers in the content and use of these guidelines, developing clinical tools to help apply them, and establishing clinical support networks could work in concert to increase the use of these guidelines. Since 2005, the Ontario Ministry of Community and Social Services, the Ministry of Health and Long-Term Care, and Surrey Place Centre have been co-sponsoring the Developmental Disabilities Primary Care Initiative, which aims to integrate these various components. Evaluation of this initiative is being undertaken and should shed light on whether this comprehensive approach promotes the application of these guidelines by primary care providers of adults with DD, changes their practices, and improves health outcomes.
People with disabling conditions, including those with DD, have been aptly described as being at risk of a double disadvantage. Having a debilitating health condition in many studies renders likely study participants ineligible, and research on DD is generally a low priority for researchers.183 Most of the recommendations specifically concerning adults with DD in the updated guidelines are supported by level III evidence based on expert opinion or published consensus statements. Three are based on randomized controlled trials, systematic reviews, or meta-analysis (level I evidence), and 7 are based on less methodologically rigorous studies (level II). Even when level I or II evidence for recommendations for the general population was found, but no level I or II evidence relating specifically to people with DD, it was thought prudent, in view of differences between these 2 groups, to reject, adapt, or formulate new guidelines based on expert opinion (level III evidence) for these guidelines.
Ethical and practical difficulties in conducting research on people with DD have been discussed in some recent studies.184,185 Because of the vulnerabilities of adults with DD, the ethical management of research involving their participation requires careful attention to the likelihood of benefit and of risks of substantial harm; issues surrounding consent, privacy, and confidentiality; and access to the benefits of the research findings.
In order to remain relevant and useful clinically, guidelines for the primary care of adults with DD in Canada should be updated regularly in light of new findings in practice and research. As knowledge and experience are gained from primary care providers caring for adults with DD who are living longer and residing in greater numbers in the community, expert opinion and consensus will continue to be helpful in updating the guidelines. Ethical and high-quality research on primary care of adults with DD, however, remains an urgent priority.
These updated guidelines are a product of the Colloquium on Guidelines for the Primary Health Care of Adults with Developmental Disabilities held on March 20, 2009, in Toronto, Ont, and attended by the following participants: R. Balogh, M. Bissell, E. Bradley, T. Broda, S. Campbell, D. Champ, T. Cheetham, K. Cowan, R. Denton, L. Dunn, A. Fewster, C. Forster-Gibson, E. Ghazal, G. Gillis, E. Grier, J. Heng, B. Hennen, B. Isaacs, J. Jones, D. Joyce, M. Kelly, M. Korossy, M. Limbos, D. Lougheed, J. Ludlow, Y. Lunsky, S. McMillan, S. Morris, J. Nachshen, T. O’Driscoll, H. Ouellette-Kuntz, S. Perreault, J. Rao, S. Stemp, L. Tao, V. Temple, A. Veltman, and M. Westerhof. The project was supported by the Ontario Ministry of Community and Social Services, the Ministry of Health and Long-Term Care, Surrey Place Centre and the Surrey Place Centre Foundation.
As a group, adults with developmental disabilities (DD) have poorer health and greater difficulty accessing primary care than does the general population. They have different patterns of illness and complex interactions among comorbidities. These guidelines update the general, physical, behavioural, and mental health recommendations for adults with DD, especially for those conditions not screened for by routine health assessments of the general population. Ethical issues, such as informed consent and assessment of benefits in relation to risks, are addressed. Among the most important updates are consideration of atypical manifestations of pain and distress in adults with DD and a strong recommendation to avoid inappropriate long-term use of antipsychotic medications to address behavioural issues.
Collectivement, les adultes ayant des déficiences développementales (DD) sont en moins bonne santé et ont plus de difficultés à avoir accès aux soins primaires en comparaison de l’ensemble de la population. Les maladies évoluent différemment et présentent entre elles des interactions complexes chez ces personnes. Les lignes directrices font la mise en jour des recommandations pour la santé générale, physique, comportementale et mentale des adultes ayant une DD, en particulier pour les problèmes qui ne sont pas dépistés dans les évaluations systématiques de la santé dans la population en général. Elles traitent des questions d’ordre éthique, comme le consentement éclairé et l’évaluation des bienfaits par rapport aux risques. Parmi les mises à jour les plus importantes, on peut mentionner les manifestations atypiques de la douleur et de la détresse chez les adultes ayant une DD et une très forte recommandation d’éviter l’utilisation à long terme inappropriée des antipsychotiques pour les problèmes comportementaux.
This article has been peer reviewed.
Cet article a fait l’objet d’une révision par des pairs.
Dr Sullivan was the project lead and was responsible for the development of the initial concept as well as all aspects of the guideline development and revision. Dr Berg provided expert clinical and scholarly input throughout the development of the guidelines. Dr Bradley led the revision process of the behavioural and mental health section. Dr Cheetham provided expert clinical and editorial input during the revision process, including extensive review of published evidence. Dr Denton led the revision discussions surrounding the general guidelines, contributed to the guideline revision process, and provided input on final editorial decisions. Mr Heng played a lead role in all aspects of the project, including the draft and final revision process. Dr Hennen played a lead role in all aspects of the project. Dr Joyce led the revision discussions surrounding the physical health guidelines, reviewed the full text of all cited references, and provided substantial editorial input throughout the revision process. Ms Kelly provided critical coordination of the project and contributed substantially to all aspects of the revision process. Ms Korossy searched and screened the published literature, set up and organized the RefWorks database housing published evidence, and participated in all stages of the revision and editorial process. Dr Lunsky was co-lead of the revision process on the behavioural and mental health section from a psychology perspective. Ms McMillan contributed expert input throughout the revision process from a nursing perspective. All authors approved the final version for publication.