1

Healthy Ageing ~ Adults with

Intellectual Disability

Physical Health Issues

Core Authors

Heleen Evenhuis

C. Michael Henderson

Helen Beange

Nicholas Lennox

Brian Chicoine

A Joint Report of the Health and Aging Special Interest Research Groups of the

International Association for the Scientific Study of Intellectual Disabilities

January 2000

Submitted to the World Health Organization

Geneva, Switzerland

Acknowledgements

This report was initially developed at the 3rd International Roundtable on Health Issues in Manchester England. It was then circulated to both Health Issues and Aging SIRG working group members and selected others for commentary and amendments. The amended document became part of the working drafts circulated to delegates at the 10^th International Roundtable on Aging and Intellectual Disabilities in Geneva in 1999, and was discussed and amended further at this meeting. A set of summative broad goals was developed by the group and appears in this paper, which itself became part of the comprehensive WHO document on aging and intellectual disability (WHO, 2000). The primary goal of this paper is to organize information on physical health issues in older people with intellectual disabilities, and to present broad summative goals to direct further work in this area.

Partial support for the preparation of this report and the 1999 10^th International Roundtable on Aging and Intellectual Disabilities was provided by grant 1R13 AG15754-01 from the National Institute on Aging (Bethesda, Maryland, USA) to M. Janicki (PI).

Suggested Citation

Evenhuis, H., Henderson, C.M., Beange, H., Lennox, N., & Chicoine, B. (2000). Healthy Aging - Adults with Intellectual Disabilities: Physical Health Issues. Geneva, Switzerland: World Health Organization.

Report Series

1 #1 Evenhuis, H., Henderson, C.M., Beange, H., Lennox, N., Chicoine, B., & Working Group. (2000). Healthy Aging - Adults with Intellectual Disabilities: Physical Health Issues. Geneva, Switzerland: World Health Organization.

#2 Walsh, P.N., Heller, T., Schupf, N., van Schrojenstein Lantman-de Valk, H., & Working Group. (2000). Healthy Aging - Adults with Intellectual Disabilities: Women's Health Issues. Geneva, Switzerland: World Health Organization.

#3 Thorpe, L., Davidson, P., Janicki, M.P., & Working Group. (2000). Healthy Aging - Adults with Intellectual Disabilities: Biobehavioral Issues. Geneva, Switzerland: World Health Organization.

#4 Hogg, J., Lucchino, R., Wang, K., Janicki, M.P., & Working Group (2000). Healthy Aging - Adults with Intellectual Disabilities: Aging & Social Policy. Geneva: Switzerland: World Health Organization.

#5 Janicki, M.P., & Breitenbach, N. (2000). Healthy Aging - Adults with Intellectual Disabilities: Summative Report. Geneva: Switzerland: World Health Organization.

1. Introduction: A lifespan, developmental perspective on healthy ageing and intellectual disability

The majority of people, including people with intellectual disability, live in the world's less developed countries. Because of the paucity of information regarding the health status and needs of persons with intellectual disabilities in less developed countries, it is hard to make universal statements regarding "healthy ageing" for people with an intellectual disability. The highest priorities for the majority of people with intellectual disabilities in all countries likely include basic health care, adequate nutrition and housing, education, civil rights, and political, social and economic stability. An international perspective on healthy ageing for persons with intellectual disabilities must acknowledge that the available literature largely reflects the experiences of clinicians and researchers in industrialized countries. Nelson and Crocker in 1978 called for affiliations between academic developmental physicians and physicians serving persons with intellectual disabilities in large institutions. A current high priority should be the development of alliances between policy makers, advocacy groups, physicians, educators and other professionals serving people with intellectual disabilities in less developed and industrialized countries (for an example, see Helm, Crocker & Rubin, 1999).

Recommendation 1

To develop a worldwide perspective on healthy ageing and intellectual disabilities through affiliations between interested parties in industrialized and developing countries that promote advocacy, trans-cultural and cost-effective clinical practices, research, and the exchange of information and expertise.

Although there is more information regarding the health status of people with intellectual disabilities in industrialized countries, it remains difficult to make general statements regarding strategies for healthy ageing. Large, industrialized countries- such as the USA- may exhibit profound regional differences in the prevalence rates for intellectual disabilities (MMWR, 1996). These differences reflect socioeconomic factors, differences in the definition of intellectual disabilities, and case-finding techniques (Schrojenstein Lantman-de Valk, 1997). People with intellectual disabilities constitute a heterogenous population. The "two group" model is an attempt to point out that people with mild cognitive impairment may have different etiologies and clinical issues than people with more severe cognitive impairment (who may be more likely to have associated syndromic conditions and other developmental disabilities) (Capute & Accardo, 1990). Furthermore, industrialized countries exhibit variations in the way that health care and other services are organized and delivered to people with (and without) an intellectual disability, and these pre-existing differences in service delivery have an impact on the relevance of specific strategies to promote healthy ageing.

Industrialized countries are witnessing an increase in the longevity of adults with an intellectual disability (Janicki et al, 1999). As more people with intellectual disabilities attain older age, it is important to note that excess functional impairment, morbidity, and even mortality can result from the consequences of early age-onset conditions, through their long-term progression or their interactions with older age-onset conditions. An example of the potential consequences of long-term progression is the high incidence of esophageal reflux in children with cerebral palsy and severe motoric compromise. If childhood-onset esophagitis is not identified and treated, it can lead to high rates of esophageal stricture or cancer in adulthood (Roberts et al, 1986; Bohmer et al 1996, 1997a,b; Cook, 1997). An example of the interaction of early-age onset and later-age onset conditions is, in persons with Down syndrome, the superimposition of adult-onset sensorineural hearing loss on childhood-acquired conductive hearing loss resulting from inadequately treated middle ear infections (Evenhuis, 1995a,b). The long-term consequences of therapeutic interventions also need to be considered- examples are movement disorders that may result from the prolonged use of neuroleptic medications (Haag, Ruther & Hippius, 1992; Wojcieszek, 1998), and bone mineralization disease that may occur secondary to the chronic use of certain anticonvulsants (Bikle, 1996; Phillips, 1998). Although more research needs to be done, it is apparent that healthy ageing for people with an intellectual disability requires a dynamic, lifespan clinical approach.

Recommendation 2

Health care providers caring for people with intellectual disabilities of all ages should adopt a lifespan approach that recognizes the progression or consequences of specific diseases and therapeutic interventions

2. Special issues in health care, healthy ageing, and intellectual disability

Research indicates that specific populations of people with intellectual disabilities have particular health risks. These populations may be defined by the presence of specific syndromes (hence termed syndrome-specific), or by the extent of the central nervous system compromise that has caused the intellectual disability (leading to associated developmental disabilities such as epilepsy, cerebral palsy, and some forms of visual impairment). In addition, populations may be defined by their placement within specific habilitative and residential programs and access to basic health care services. The resulting lifestyle and environmental issues and health promotion/disease prevention practices may directly cause, or interact with, hereditary factors, to protect against or confer specific health risks. Finally, the increased longevity of persons with intellectual disabilities in industrialized countries leads to the definition of populations by chronological older age- and a subsequent increased risk of acquiring adult and older-age associated conditions.

3. Syndrome-specific conditions

Persons with specific syndromes constitute a clinically and numerically important portion of the population with an intellectual disability. These syndromes can be caused by toxins, injuries, infections, and genetic/metabolic disorders which affect the central nervous system and, in some cases, other organ systems, during the developmental period. Moreover, these effects can become manifested, and clinically anticipated, at different stages of the lifespan. Down syndrome is a relatively common chromosomal disorder that, in addition to causing an intellectual disability, results in a relatively high risk for a number of conditions. In the neonatal period, Down syndrome can be associated with congenital defects of the heart, gastrointestinal tract, eyes, and other organs (Pueschel & Pueschel, 1992). Throughout the lifespan, persons with Down syndrome manifest higher risks for specific endocrinological (especially hypothyroidism), infectious, dermatologic, oral health, cardiac, musculoskeletal and other organ system disorders (Murdoch et al, 1977; Sare et al, 1978;Dinani & carpenter, 1990; Pueschel & Pueschel, 1992; Song, Freemantle & Selicowitz, 1993; Marino & Pueschel, 1996). In addition, they exhibit high rates of disorders of the special senses of vision (Pires da Cunha & Belmiro de Castro Moreira, 1996) and hearing (Strome & Strome, 1992; Roizen et al, 1993). Older adults with Down syndrome have an increased risk of the early development of age-related visual and hearing disorders (Buchanan, 1990; Evenhuis et al, 1992), epilepsy (McVicker, Shanks & McCleeland, 1994) and dementia (Wisniewski et al, 1985; Lai & Williams, 1989; Evenhuis, 1990; Burt et al, 1995; Zigman et al, 1995; Devenny et al, 1996). Adults with Down syndrome have decreased longevity compared to the general population of people with intellectual disabilities (Janicki et al., 1999). Fragile X syndrome is the most common inherited disorder associated with an intellectual disability. People with Fragile X syndrome exhibit relatively high rates of mitral valve prolapse (Loehr et al, 1986; Sreeram et al, 1989), musculoskeletal disorders (Davids, Hagerman & Eilert, 1990), early female menopause (Conway et al, 1998; Murray et al, 1998), epilepsy (Ribacoba et al, 1995) and visual impairments (Maino et al, 1991). Adults with Prader-Willi syndrome are prone to high rates of cardiovascular disease and diabetes arising from morbid obesity (Greenswag, 1987; Lamb & Johnson, 1987). Other syndromes may not be as common or easily identifiable as Down syndrome, Fragile X syndrome, or Prader-Willi syndrome; however, the same principle of knowledge of syndrome-specific issues may lead to the enhanced functional and health status of persons who have them. Examples are the deafness and eye abnormalities that occur in people with intrauterine toxoplasma, cytomegalovirus infections or foetal alcohol syndrome (Evenhuis & Nagtzaam, 1998).

Knowledge of the specific age-related health risk factors associated with Down syndrome and other syndromes can lead to enhanced prevention or early diagnosis of potentially impairing conditions and, possibly, increased life expectancy. Other relatively common syndromes associated with an intellectual disability that can have an impact on health status across the lifespan include Williams syndrome, Angelman syndrome, and tuberous sclerosis.

In addition, prenatal medical practices (such as the prevention of premature delivery) and the early identification of metabolic syndromes through neonatal screening (such as those that detect phenylketonuria or congenital hypothyroidism) have already led to treatments that can prevent or mitigate intellectual disabilities. Genetic counseling also helps to prevent inherited disorders that are associated with intellectual disabilities. In the future, the field of biomolecular genetics may provide further advances in the prevention or treatment of intellectual disabilities and other impairments that are caused by genetic/metabolic syndromes.

Recommendation 3

Children presenting with intellectual disabilities should have thorough diagnostic searches for etiologies and syndromes to optimize their current and future health care.

4. Associated developmental disabilities arising from central nervous system compromise

A significant number of persons with intellectual disabilities do not have specific syndromes, but exhibit associated developmental disabilities that reflect central nervous system compromise. These associated developmental disabilities may result in both primary and secondary diseases or impairments; they constitute a large component of mortality during childhood (Boyle, Decoufle & Holmgreen, 1994). An important example is cerebral palsy (Rosen & Dickinson, 1992). Children and adults with intellectual disabilities and cerebral palsy with severe motoric and functional impairments have decreased life expectancies compared to the general population (Evans, Evans & Alberman, 1990; Crichton, Mackinnon & White, 1995; Strauss & Shavelle, 1998; Strauss, Shavelle & Anderson, 1998). In addition to these motoric impairments that can adversely affect speech, mobility, and survival, children with intellectual disabilities and cerebral palsy present with high rates of strabismus and cerebral visual impairment (Schenk-Rootlieb et al, 1992; Erkkila, Lindberg & Kallio, 1996) and bladder dysfunction (Boone, 1998). Spasticity may require medical or neurosurgical treatment to alleviate pain, prevent deformities, and enhance function (Russman & Romness, 1998): orthopedic surgery can also be required (Renshaw et al, 1996). Children and adults with intellectual disabilities and cerebral palsy also exhibit a high risk for a number of secondary disorders. Upper gastrointestinal dysmotility, resulting in dysphagia, esophageal reflux and gastric emptying disorders, may lead to dental erosion, esophagitis, anemia, feeding, problems, aspiration and pneumonia (indeed, respiratory disease is the leading cause of death in people with cerebral palsy and severe motoric impairments) (Reilly & Skuse, 1992; Arvedson et al, 1994; Mirrett et al, 1994; Rogers et al, 1994; Bvhmer et al, 1997b, Shaw, Wetherill & Smith, 1998). People with intellectual disabilities and cerebral palsy are also prone to lower gastrointestinal dysmotility; this may cause constipation and fecal impaction (Cathels & Reddihough, 1993), and death due to bowel obstruction and intestinal perforation (Jancar & Speller, 1994). Bone demineralization with consequent fractures and decubitus ulcers may occur secondary to long-standing immobility and nutritional deficiencies (Brunner & Doderlein, 1996; Wagemans et al, 1998). Children and adults with cerebral palsy and severe or multiple impairing conditions require multidisciplinary care (Lowes & Gries, 1998). In later life, the chronic abnormalities of muscle tone may lead to chronic myofascial pain, hip and back deformities (including degenerative vertebral spine disease that may cause myelopathy); worsening bowel and bladder function is also seen (Harada et al, 1996; Mikawa Y, Watanabe R & Shikata J, 1997;Turk et al, 1997; Saito et al, 1998). The optimization of function and survival for people with cerebral palsy throughout life depends on the anticipation and identification, and prevention or treatment, of both primary and secondary disorders.

People with intellectual disabilities and epilepsy have other health risks. Children with intellectual disabilities and intractable epilepsy present with higher rates of cerebral palsy, visual impairment, and severe cognitive impairments (Steffenberg et al, 1995). In addition to the risk of status epilepticus (which is more common in children with co-existing neuro-impairments such as cerebral palsy), epilepsy is associated with injuries such as fractures (Desai, Ribbans & Taylor, 1996; Jancar & Jancar, 1998). People with intellectual disabilities and epilepsy have an increased mortality due to sudden death, aspiration episodes, and pneumonia (Forsgren et al, 1996). Unrecognized or inadequately treated seizures can impair cognitive function (Aldenkamp, 1997). Epilepsy syndromes associated with an intellectual disability (Dulac & N'Guyen, 1993: Ohtsuka, 1998) may prove difficult to treat and lead to a worsening of seizure control (Udani et al, 1993; Branford, 1998) and progressive cognitive impairment (Oka et al, 1997). However, some people with an intellectual disability and epilepsy exhibit a remission of the epilepsy in later life- the need for anticonvulsant medication needs to be regularly reappraised (Goulden et al, 1991; Brodtkorb, 1994). A coordinated and comprehensive approach to the management of epilepsy in people with intellectual disabilities may result in optimal management (Coulter, 1997)- health care service models do not always foster this type of approach.

Other examples of associated developmental disabilities that can result from central nervous system compromise, with obvious health status and functional repercussions, include autism, mental health issues, and some disorders of vision.

Recommendation 4

Persons presenting with an intellectual disability should have expert care to identify and treat associated developmental disabilities such as cerebral palsy, epilepsy, autism, and disorders of vision.

5. Conditions related to lifestyle and environment and health promotion/disease prevention practices

Industrialized countries have varying habilitative and residential philosophies and practices for persons with intellectual disabilities. In the North America, Australia, and in many European countries, many governments have implemented measures to close large publically -operated institutions and move residents into a variety of small community-based settings. Other countries have opted to modify the institutional model. In addition, countries exhibit wide variation in expenditures for supports and services for people with intellectual disabilities (for USA, see Braddock et al, 1998). It is important to note that, throughout the industrialized world, many people with intellectual disabilities have experienced or continue to experience placement in large institutions. Previous or current residence in large institutions place many people with intellectual disabilities at risk for past or present exposure to a number of infectious diseases, including tuberculosis (Lemaitre et al, 1996), hepatitis B (Hayashi et al, 1989; Stehr-Green et al, 1992; Cramp et al, 1996), and Helicobacter pylori (Bohmer et al, 1997).

Recommendation 5

People with intellectual disabilities with current or previous histories of life in large institutions should be evaluated for evidence of infectious diseases such as tuberculosis, hepatitis B, and Helicobacter pylori.

As people with intellectual disabilities, particularly those with milder cognitive impairments, are offered more lifestyle choices, there is the potential that some of these choices may result in a higher potential for risky behaviors and conditions that result from the lifestyle choices, or the interaction of lifestyle and hereditary factors. People with intellectual disabilities living in the community may engage in tobacco use (Burtner et al, 1995; Hymowitz et al, 1997; Tracey and Hoskin, 1997), other substance abuse (Westermeyer, Phaobtong & Neider, 1988; Moore & Posgrove, 1991; Christian & Poling, 1997), violent behavior (Pack, Wallander & Brown, 1998), and high-risk sexual activity (Cambridge, 1996). Behavioral factors of people with intellectual disabilities and their carers contribute to the high rates of peridontal disease noted in people with intellectual disabilities (Beange, McElduff & Baker, 1995; Lucchese & Checchi, 1998; Scott, Marsh & Stokes, 1998). A sedentary lifestyle, with consequent risks of deconditioning, obesity, (and diseases related to obesity including coronary artery disease, hypertension and diabetes) has been noted in people with intellectual disabilities in a variety of residential settings (Rimmer, Braddock & Marks, 1995; Beange, McElduff & Baker, 1995; Fujiura, Fitzsimmons, Marks & Chicoine, 1997). For people with intellectual disabilities, targeting lifestyle issues (Turner & Moss, 1996) may result in substantial gains in longevity and older-age quality of life and functional capability. Special programs that target healthy behaviors such as safe sex practices (Ager & Littler, 1998), avoidance of tobacco and other harmful substances (Tracy & Hosken, 1997), good oral hygeine (Nicolaci & Tesini, 1992), optimal exercise and dietary habits (Pitetti, Rimmer & Fernhall, 1993, Golden & Hatcher, 1997), and fire safety education (Janicki & Jacobson, 1985; MacEachron & Krauss, 1985), need continued development.

Recommendation 6

People with intellectual disabilities, and their carers, need to receive appropriate and ongoing education regarding healthy living practices in areas such as nutrition, exercise, oral hygeine, safety practices, and the avoidance of risky behaviors such as substance abuse and unprotected or multiple partner sexual activity.

Presently, however, there is no research to suggest that preventative health practices that are recommended for the general population, throughout the lifespan, should be withheld from people with intellectual disabilities. Standard immunization schedules and age-appropriate screening protocols for conditions such as dental disease, sensory impairments, various forms of cancer (with the possible exception of PAP smears in women who have no history of sexual activity), glaucoma, hyperlipidemia, and hypertension, should be offered to people with intellectual disabilities.

Recommendation 7

People with intellectual disabilities should receive the same array of lifespan preventative health practices as those offered to the general population.

6. Older age-related conditions

A number of recent studies have addressed the health status of middle-age and older adults with intellectual disabilities. These studies vary in methodology, and include longitudinal residence carers surveys (Anderson, 1993), interviews with subjects with intellectual disabilities and their carers (Cooper, 1998), carers interviews combined with medical chart reviews (Kapell et al, 1998), health status questionnaires of physicians providing care to subjects (Hand, 1994), questionnaires of direct care staff and physicians (Schrojenstein Lantman-de Valk et al, 1997), comprehensive medical assessment of subjects by a developmental physician (Beange, McElduff & Baker, 1995), and comprehensive and longitudinal assessment of subjects by a developmental physician (Evenhuis, 1995a,b; Evenhuis, 1997a). Only one of these studies attempted to identify subjects who were not previously registered or residing within the intellectual disabilities service system, resulting in a 15% segment of the older population with an intellectual disability (Hand, 1994). It is significant that the study that utilized comprehensive medical assessment by a developmental physician (of subjects who were being managed by community-based primary care physicians) uncovered a high number of previously undiagnosed conditions (Beange, McElduff & Baker, 1995). The cumulative research suggests that older adults with intellectual disabilities have rates of common adult and older age-related conditions that are comparable to or even higher than that of the general population (Minihan & Dean, 1990; Anderson, 1993; Hand, 1994; Beange, McElduff & Baker, 1995; Evenhuis, 1997: Schrojenstein Lantman-de Valk et al 1997; Kapell et al, 1998; Cooper, 1998). For many people with intellectual disabilities, the risk of a variety of chronic diseases that are acquired during adulthood, and that are associated with older-age morbidity or functional impairment, reflects the same interplay between hereditary predisposition and environment that is present in other older persons. However, as discussed above, factors related to syndromes, associated developmental disabilities, and lifestye and environmental issues, may account for higher rates, compared to the population without intellectual disabilities, for a number of conditions. Previously noted examples include obesity, dental disease, gastroesophageal reflux and esophagitis, constipation, and deaths due to bowel obstruction and intestinal perforation and gastrointestinal cancer. Other examples include non-atherosclerotic heart disease (Kapell et al, 1998; Cooper, 1998), mobility impairment (Kearny, Krishnan & Londhe, 1993; Evenhuis, 1997), thyroid disease (Kapell et al, 1998), osteoporosis (Center, Beange & McElduff, 1998) psychotropic drug polypharmacy (Tu, 1979; Gowdy, Zarfas & Phipps, 1987; Schrojenstein Lantman-de Valk et al, 1997), and deaths due to pneumonia (O'Brien, Tate & Zaharia, 1991; Janicki et al, 1999).

Recommendation 8

Health care providers serving older adults with intellectual disabilities should recognize that adult and older-age onset medical conditions are common in this population, and may require a high index of suspicion for clinical diagnosis.

Sensory impairments appear to constitute an area of special vulnerability for older adults with intellectual disabilities (Warberg M & Rattleff J, 1992;Wilson & Haire, 1992; Schrojenstein Lantman-de Valk et al, 1997). Although causes of visual and hearing loss may be present in rates similar to those in the general population (presbyacusis, cataract, presbyopia, macular degeneration, glaucoma, diabetic retinopathy), the resulting impairment may be more severe because of pre-existing, childhood onset visual and auditory pathology (Schrojenstein Lantman de-Valk et al, 1994; Evenhuis, 1995a,b).

Functional decline in older adults with intellectual disabilities warrants careful evaluation; a decline in functional status should not be peremptorily attributed to behavioral issues or dementia (Prasher & Chung, 1996; Burt et al, 1998). Comprehensive evaluations of older adults presenting with changes in state or functional decline and intellectual disabilities have yielded high rates of (often-concurrent) treatable conditions. Examples include affective disorders, sensory impairments, delirium, and undiagnosed medical conditions (Evenhuis, 1997b; Evenhuis, 1999; Thorpe, 1999; Chicoine, McGuire & Rubin, 1999; Henderson et al, in press). It is important to note that, because of communication difficulties, medical and mental health disorders may present atypically. Even people with an intellectual disability and dementia may have a relatively high burden of treatable medical conditions that may have an additive effect on disability (Cooper, 1999). The reversal of functional decline should be sought for people with intellectual disabilities of all ages, and not solely for functional or quality of life issues- severe functional impairment is related to decreased life expectancy in people with intellectual disabilities of all ages (Eyman et al, 1990).

Recommendation 9

Functional decline in older adults with intellectual disabilities warrants careful medical evaluation; undiagnosed mental health and medical conditions can have atypical presentations in people with limited language capabilities. Regular screening for visual and hearing impairments should be implemented for people with intellectual disabilities during the childhood and late-adulthood years.

7. Barriers to health care services in healthy ageing and intellectual disabilities

In theory, people with intellectual disabilities living in industrialized countries have equal access to essential health care services. As mentioned previously, countries (and regions within countries) vary in their models of health care delivery for people with intellectual disabilities. However, it is worth noting the general barriers that exist in providing care to people with intellectual disabilities (see Seltzer & Luchterhand, 1994), although the significance of these barriers may vary by region and type of health care system. It is important that health care providers and policy makers acknowledge that many people with intellectual disabilities have special needs that may require modification of standard health care practices and service models.

Communication difficulties arising from intellectual disabilities or associated motor impairments can serve as barriers to accurate medical evaluation. The medical history, in many cases, is derived from carers observations. In these cases, the health care provider is dependent on the verbal or written reports of carers that know the patient. People with intellectual disabilities can benefit from the training of carers in health-related issues- particularly basic assessment skills (Crocker & Yankauer, 1987). There is evidence that, in places where deinstitutionalization has led to placement of people with intellectual disabilities in the community, health care has deteriorated because carers were not familiar with the individuals (Linaker & Nottestadd, 1998). Carers need to be able to recognize signs of distress in persons with severe cognitive impairment (LaChapelle, Hadjistavvropoulos & Craig, in press); at the same time, individuals who have potential communication skills need to be educated in the effective communication of pain or distress (Bromley, Emerson & Caine, 1998). In addition, unresolved concerns about informed consent for or refusal of health services may, at times, prove to be a barrier for some people with intellectual disabilities (O'Donnell, 1994). Even in optimal circumstances- when the ill person with an intellectual disability is accompanied by knowledgeable carers- informant-based medical history taking takes time. Concepts of health care productivity need to be altered when considering the population of people with intellectual disabilities and significant communication difficulties.

Physical barriers may constitute a problem for many persons with intellectual disabilities and other disabling conditions. Older women with cerebral palsy, with and without an intellectual disability, have reported difficulties obtaining dental and gynecologic care because of accessibility problems (Turk et al, 1997). Health care facilities should be easily accessible to persons with an intellectual disability who may have a variety of physical and sensory impairments.

Behavioral issues constitute another potential barrier. Persons with intellectual disabilities may have difficulty cooperating with examinations and procedures. Health care providers need to be educated regarding the confusion, fear, and frustration that many people with intellectual disabilities may experience when they access health care services. Again, more time may be necessary to reassure someone with an intellectual disability. Habilitative programs or health care providers should address the issue of health care- not just in terms of healthy living, but also by increasing understanding and confidence in using health services (McRae, 1997; Lunsky, 1999). Protocols for safe conscious sedation may be helpful for some people with an intellectual disability. In other cases, general anesthesia may be necessary to enable safe and thorough health maintenance exams and procedures.

Behavioral issues can also play an important role in successful acute rehabilitation after disease, insults or injury. Also, teaching persons with an intellectual disability how to use assistive or prosthetic devices, such as canes, walkers, wheelchairs, braces, dentures, eyeglasses and hearing aids, may require more time and special techniques.

For many people with intellectual disabilities, the most important barrier to effective medical care is case complexity. People with intellectual disabilities may access a variety of medical subspecialists, dentists, audiologists, mental health providers, and other health care professionals. Case management is crucial for the optimal utilization of health care services for people with intellectual disabilities who have complex needs requiring multidisciplinary expertise (Walsh, Kastner & Criscione, 1997).

It is worth noting that, in some countries or states, health care rationing or reimbursement schedules may constitute barriers to basic health services. In addition, administrators and policy makers need to understand that, in some cases, clinically indicated and relatively expensive techniques and expertise may prove cost-effective in the long-term.

Recommendation 10

Health care providers and policy makers need to eliminate attitudinal, architectural and health care reimbursement barriers that interfere with the provision of high quality health services for people with intellectual disabilities.

Recommendation 11

Carers need training in assessing and communicating the basic health status of the adults with intellectual disabilities.

Recommendation 12

Health care case management needs to be provided to adults with intellectual disabilities who have complex needs.

8. The role of the physician in healthy ageing and intellectual disabilities: Primary care and developmental physicians

Physicians can play a pivotal role in the functional attainments and quality of life of many persons with intellectual disabilities. However, successful habilitation and community placement may depend on the prevention or identification of a variety of health issues. Accordingly, the physician is one member of a health care team. Other important team members include nurses, audiologists, nutritionists, dentists, mental health specialists, and rehabilitation specialists. An interdisciplinary approach may be required for a number of health issues, including visual and hearing impairment (Evenhuis, 1995a,b), swallowing disorders (Kennedy et al, 1997), urinary incontinence (Bradley, Ferris & Barr, 1995), dental care (Editorial, 1998), and geriatric assessment (Carlsen et al, 1994).

Many adults with intellectual disabilities do not need special medical attention. It is important for primary care physicians to recognize that, in general, adults and older persons with an intellectual disability have the same needs for disease prevention, diagnosis, and treatment as other members of the population. For routine care, health status can improve by ensuring regular encounters with primary care physicians (Martin, Roy & Wells, 1997), and through "opportunistic" health assessment at the time of encounters (Jones & Kerr, 1997). However, some persons with intellectual disabilities and specific health risks (because of syndrome-specific issues, associated developmental disabilities, and complex neuropsychiatric conditions) may require regularly scheduled, easily administered screening protocols (Cohen, 1997; Piachaud, Rohde & Pasupathy, 1998).

It is noted that, in many countries, the relatively frequent contact between adults and older persons with an intellectual disability and primary care physicians based in the community is a new and largely unplanned phenomenon arising from the deinstitutionalization and increased longevity of persons with intellectual disabilities. Evidence suggests that community-based primary care physicians in some regions may not provide access or have the expertise or professional back-up to care for people with intellectual disabilities who have severe or complex impairments (Strauss & Kastner, 1996; O'Brien & Zahari, 1998; Strauss et al, 1998). Primary care physicians need to be able to get access to information through a variety of means: formal consultations, telephone consultation systems, internet communication, clinical guidelines, training seminars, and written materials such as texts (see Lennox, 1999). In complex cases, established referral paths to developmental physicians and other specialists with intellectual disabilities expertise can be crucial.

Developmental physicians, trained with a lifespan approach to developmental disabilities, can provide valuable expertise to primary care physicians and other health care providers serving people with intellectual disabilities. The influence of this specialty can range from preparing written guidelines and training programs for primary care physicians and other health care providers, to providing formal and informal consultation services for complex patients. In addition, they can provide leadership in the area of clinical research.

Health care providers need evidence-based practice standards (Lennox & Kerr, 1997), similar to the international guidelines for the screening and diagnosis of visual and hearing impairments in persons with intellectual disabilities, recently developed by the IASSID Special Interest Research Group on Health Issues (Evenhuis & Nagtzaam, 1998). Comparable standards need to be developed for other specific interventions, conditions, diseases, and syndromes. Most important is a need for leadership to more fully introduce people with an intellectual disability of all ages- who comprise a substantial portion of the human population- into basic and postgraduate medical education.

Lastly, there is a need for medical specialists with interest and expertise in intellectual disabilities. Psychiatrists, neurologists, physiatrists, otolaryngologists, ophthalmologists and other specialists with intellectual disabilities knowledge can be enormously helpful to colleagues in their own disciplines, as well as to primary care specialists and developmental physicians.

Recommendation 13

An interdisciplinary approach is required for a variety of clinical issues involving people with intellectual disabilities.

Recommendation 14

Health care systems need to provide educational and clinical practice supports for primary care physicians caring for people with intellectual disabilities.

Recommendation 15

The development of the discipline of lifespan developmental medicine is necessary to provide medical education, practice standards, clinical expertise, research, and professional leadership regarding the special needs of people with intellectual disabilities of all ages.

9. Conclusion: Areas for future research

The development of research to enable healthy ageing in persons with intellectual disabilities represents a new and complex area. Previously mentioned is the need to provide evidence-based practice standards to enhance health status, longevity, functional capability, and quality of life. Other high priority research areas include:

The acquisition of additional clinical and epidemiological knowledge regarding specific syndromes, with linkages to basic science research in biomolecular genetics and metabolism.

The development of adapted diagnostic and therapeutic methods for people who have difficulties with cooperation or communication.

The development and evaluation of interdisciplinary interventions for complicated conditions (e.g. sensory impairment, dysphagia, communication, and functional decline).

The development of clinimetric measures in a number of areas -functional capability, quality of life, mental health, pain assessment, and clinical diagnosis- that are sensitive and specific, easy to administer, and applicable to persons with a wide range of mental and physical capabilities.

The evaluation of clinical guidelines- including referral protocols- to support community-based primary care physicians, within specific health care systems, to care for people with intellectual disabilities.

The evaluation of the applicability of a new discipline of lifespan developmental medicine to lead in interdisciplinary care, health care education, service delivery, and research for people with intellectual disabilities.

The development of the knowledge base regarding the health status and needs of people with intellectual disabilities living in less developed countries.

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Healthy Aging - Adults with Intellectual Disabilities

Biobehavioral Issues

Senior Authors

L. Thorpe (Canada), P. Davidson (USA), M. Janicki (USA)

A Report of the Aging Special Interest Research Group of the International

Association for the Scientific Study of Intellectual Disabilities

January 2000

Submitted to the World Health Organization

Genhve, Switzerland

Acknowledgements

This report was developed primarily with input from: N. Bouras (UK), K. Drummond (UK), S. Moss (UK), K. Bishop (USA), V. Prasher (UK), D. Burt (UK), N. Schupf (USA), G. Weber (Austria), S. Vicari (Italy), A. Dalton (USA), J. Jacobson (USA), K. Wang (Taiwan), P. Ladrigan (USA), C. M. Henderson (USA), H. San Nicolas (Guam), K. Hauser (USA) and secondarily from delegates present at the 10^th International Roundtable on Aging and Intellectual Disabilities, World Health Organization, Geneva, Switzerland, April 20-23, 1999. This document was developed initially in draft form in 1998 by L.Thorpe and P. Davidson after the 9^th International Roundtable on Aging and Intellectual Disabilities in Cambridge, England. It was then circulated to Aging SIRG working group members and selected others for commentary and amendments. The amended document became part of the working drafts circulated to delegates at the 10^th International Roundtable on Aging and Intellectual Disabilities in Geneva in 1999, and was discussed and amended further at this meeting. A set of summative broad goals was developed by the group and appears in this paper, which itself became part of the comprehensive WHO document on aging and intellectual disability (WHO, 2000). The primary goal of this paper is to organize information on biobehavioral issues in older people with intellectual disabilities, and to present broad summative goals to direct further work in this area. These are included within the text and at the end of this document.

Suggested Citation

Thorpe, L., Davidson, P., & Janicki, M.P. (2000). Healthy Aging - Adults with Intellectual

Disabilities: Biobehavioral Issues. Geneva, Switzerland: World Health Organization.

Report Series

#3 Thorpe, L., Davidson, P., Janicki, M.P., & Working Group. (2000). Healthy Aging - Adults with Intellectual Disabilities: Biobehavioral Issues. Geneva, Switzerland: World Health Organization.

#5 Janicki, M.P., & Breitenbach, N. (2000). Healthy Aging - Adults with Intellectual Disabilities: Summative Report. Geneva: Switzerland: World Health Organization.

1. Background

In nations with established market economies, most adults with intellectual disabilities who live past their third decade are likely to survive into old age, and experience the normal aging process. As in the general elderly population, in spite of gradual declines in a variety of domains, they can still have active and varied lifestyles with an excellent quality of life. Age associated, functional declines must be separated from specific losses due to physical illness, dementia, depression, sensory loss, and social and environmental factors. The interaction between biological, psychological and social aspects of aging remains the most important factor in the functional outcome of a person with intellectual disabilities.

Very little empirical data exists about normal psychological functioning developmental processes throughout the life-span in people with intellectual disabilities. Seltzer (1993) presents the best model, linking behavioral, cognitive and affective outcomes to the negotiation of developmental tasks of aging in the context of a variety of interacting individual, social and environmental antecedent conditions, such as intellectual ability, social competence, personality, physical condition, environment and learning history. Every person has his/her own individual set of antecedent conditions, and has different opportunities to successfully negotiate the developmental tasks of aging.

Goal 1 To improve the understanding of normal psychological functioning throughout the life-span of people with intellectual disabilities

People with intellectual disabilities in general have restricted social roles and more limited social networks, and thus fewer opportunities to experience and learn from some of the tasks commonly experienced by those without intellectual disabilities, particularly those who have spent considerable time in more restricted institutional environments. Mid to older life changes such as bereavement may thus have a greater impact, and with a greater likelihood of adverse functional outcome. The acceptance of mortality for example, which is an integral part of aging in people without intellectual, is often hindered by a lack of exposure to rituals such as funerals in an attempt to shield the person from unpleasant events.

Furthermore, the magnitude of individual adverse reactions to stressors may be accelerated because of cognitive impairment (pre-existing and/or degenerative, as in the dementias), poor self-esteem and poor perception of self-competence due to repeated adverse life experiences over the life-span, and poor social support.

Goal 2 To improve knowledge and awareness of age-related stressors and their impact on older people with intellectual disabilities

2. Psychiatric and behavioral disorders

For the purpose of this paper we have defined mental disorders as disorders that can be classified into diagnostic systems such as the ICD10. Biological, psychological and social factors disorders may all contribute to their expression. Behavioral disorders on the other hand are patterns of maladaptive behaviors (usually as perceived by an informant) that interfere with typical life functioning. They may be related to another mental disorder in the individual, biological vulnerability, longstanding learned behaviors, or a mismatch between environmental expectations and resources with the individual's capabilities and wishes: for example, a behavioral problem such as wandering in a demented person may be maladaptive if the individual lives in an open facility close to a busy highway, but contribute to the maintenance of physical abilities in a well-designed dementia unit due to regular exercise.

Major mental disorders, although less common than behavioral disorders, are still fairly frequent in elderly people with intellectual disabilities. Day and Jancar (1994) reviewed this topic and found an overall prevalence of about 10%. Some disorders such as dementia increase with age, which is particularly noticeable in those with Down Syndrome (DS). As in the general elderly population, psychotic disorders also increase with age, but are less frequent than mood and anxiety disorders. Interestingly, due to "differential mortality" or the tendency for healthier people to live longer, older cohorts may actually be healthier in many domains than younger cohorts (Janicki, Dalton, Davidson & Henderson, in press), and show greater functional abilities than the young until the oldest ages.

Most studies find that, compared to the general population, behavioral disorders are more common in people with intellectual disabilities at all stages of the life span. There seems to be an association with age mostly in those individuals that have dementing disorders (Moss & Patel, 1995).

3. Etiology

Social, cultural, environmental and developmental factors and stressors have significant impact on the expression of both psychiatric and behavioral disorders in older people with intellectual disabilities (Day & Jancar, 1994). Stressors may be multiple, and include separation from or death of a parent, loneliness and sudden relocation. Unfortunately, little is known about quantifying these influences on age-related changes in persons with intellectual disabilities. However, the general consensus of clinicians in the filed is that all perceived symptoms need to be evaluated in a broad context, and not necessarily attributed to one individualized factor but explored as part of a complex interaction of the individual with the environment.

Goal 3 To understand and appreciate the social, cultural environmental and developmental context of behaviors and their functions in older people with intellectual disabilities

Biological contributions to mental and behavioral disorders are also important, and often increase with age. Examples include sensory loss and dementia in DS, feeding abnormalities in those with cerebral palsy due to reflux, and a variety of other behavioral changes related to chronic medical illnesses (Lantman de Valk et al., 1998; Davidson et al., 1995). Of course, genetic risk factors for the major mental illnesses such as schizophrenia or bipolar disorders continue to be present in old age as in the general population, and specific behavioral clusters associated with developmental syndromes may persist from younger years into old age.

4. Detection and assessment of mental disorders

Major mental disorders in older people with intellectual disabilities may have considerable negative impact on cognitive, affective and general functioning as well as on the quality of life of the person. It is important therefore to detect and optimally treat these, especially treatable disorders such as depression. However, diagnosis is already more difficult in older people in general due to higher rates of comorbidity, polypharmacy and a reduced tendency to voice psychological compared to physical complaints, and this is magnified in the intellectual disabilities group, particularly in the most disabled segment. The presence of seizure disorders and their treatments additionally complicates the assessment of mental functioning, although this may be more pronounced in younger age groups that tend to be more multiply disabled. Other challenges in the intellectual disabilities group include communication barriers, baseline behavioral abnormalities (secondary to brain abnormalities, learned maladaptive behaviors, and environmental deprivation) overlapping with core mental illness symptomatology, and more florid stress related decompensation.

Health care providers that are not familiar with intellectual disabilities have difficulty making accurate mental health assessments, yet carers that are most able to report changes in the usual functioning generally do not have the necessary knowledge of mental disorders. Unfortunately, in most parts of the world there are few specialists with both intellectual disabilities and psychogeriatric expertise that would be able to bridge that gap. Cultural perspectives on normative behavior may further color how seemingly "deviant" behavior, which may be attributable to intellectual disabilities, may be perceived. Tests and assessment instruments are often not available in local languages.

In many cases the combination of the above individual, environmental and care system difficulties leads to a lack of differentiation between mental illness and intellectual disability, with both over and under diagnosis of mental illness, each of which can lead to adverse consequences. Although florid and disruptive behaviors are likely to come to the attention of mental health services, milder symptoms such as early depression and cognitive impairment may be missed, whereas there may be an overdiagnosis of disorders like schizophrenia due to the diagnosticians' unfamiliarity with the presentation of older people with intellectual disabilities and stress decompensation, for example.

Ideally, assessment of biobehavioral issues involves interviewing the person as well as their carers, and exploring the environment as a potential contributor to the symptoms. Interactions between the older person's cognitive, affective and general functional abilities with the environment and care system must be explored. Frequencies of symptoms and possible correlation to other environmental events can be analyzed by charting identified behaviors and symptoms. A thorough medical evaluation, including visual and auditory assessments should precede a final mental health diagnosis.

Screening instruments exist for various mental disorders in intellectual disabilities, but must be developmentally and culturally appropriate. General instruments include the Psychopathology Instrument for Mentally Retarded Adults (PIMRA; Matson), and the Reiss screen (Reiss, 1987). The Mini-PAS-ADD (Prosser et al., 1997) and the PASS-ADD Checklist (Moss et al., 1998) have been developed specifically to improve case recognition in this population. These instruments are not sufficiently specific or sensitive to make a diagnosis, but are useful to indicate the need to obtain further mental health assessment.

Instruments designed for specific disorders, such as the Beck Depression Inventory (Beck, Ward, Mendelson, 1961) and the Zung Self-Rating Depression Scale (Zung, 1965) have been adapted and simplified for use in intellectual disabilities by Kazdin and associates (Kazdin, Matson, Senatore, 1983). These, as well as others such as the Hamilton Rating Scale for Depression (Hamilton, 1960) have been used successfully to assess depression in people with intellectual disabilities and mental disorders.

The diagnosis of dementia in intellectual disabilities has been discussed at length, as people with DS are at very high risk of developing this. The instruments used in the general population are difficult to use due to floor effects, and furthermore, baseline abilities in intellectual disabilities are so varied that only repeated measures over time are likely to result in an accurate assessment of dementia. It is suggested that behavioral measures should be repeated at set intervals after age 40 in DS, and after age 50 in others with intellectual disabilities to detect functional changes, which can then be further evaluated clinically. The IASSID/AAMR practice guidelines give more detail on assessment and care management in dementia (Janicki et al, 1996).

Auxiliary diagnostic tools such as computerized tomography (CT), positron emission tomography (PET), single photon emission computerized tomography (SPECT) and magnetic resonance imaging (MRI) may be helpful diagnostically, and might eventually become more routinely used, at least in developed nations.

Goal 4 To improve the detection and holistic assessment of mental disorders such as depression, anxiety and dementia in older people with intellectual disabilities.

Goal 5 To increase mental health knowledge and skills in professionals, carers and families of older people with intellectual disabilities.

5. Interventions

Interventions in general must embody the best information from two separate bodies of evidence; the mental health-intellectual disability (dual-diagnosis) literature, and the psychogeriatric literature. Data from the psychogeriatric literature is important as it considers physical and mental changes developing longitudinally with the aging process. Data from the mental health-intellectual disability literature is important because it identifies issues specific to or more prevalent in people with intellectual disabilities, and focuses on interventions that have particular use in this area. Both fields are now starting to address the role of autonomy and choice-making by adults in the development and treatment of mental health symptoms.

Ideally, interventions for behavioral and mental disorders should first consider prevention: primary, i.e., strategies implemented to prevent all occurrence of the problem; secondary, i.e., early treatment of a problem to prevent its full expression; and tertiary, i.e., strategies to minimize functional impairment due to the problem once firmly established. (It should be remembered that the "problem" referred to is not necessarily only directly related to the older person with an intellectual disability, but is really the interaction of multiple variables as described earlier, culminating in the perception of their being a "problem" by some person, usually in the care system or the community.)

Primary prevention strategies for behavioral and mental disorders are not comprehensively understood, but some issues are known to be associated with a reduced prevalence. Decreased use of large congregate care such as institutions reduces the frequency of a variety of maladaptive behaviors, infectious diseases as well as polypharmacy, which is responsible for many other secondary adverse effects. Increased work on communication skills and identification of sensory deficits often reduces the development of maladaptive behaviors such as aggression, and increases adaptive behavior. Increased availability of rewarding activities, and increased provision for autonomous choice making in various domains is also associated with positive behavioral outcomes, although systematic studies are difficult to perform. Humane, non-abusive living environments sensitive to the needs of their older residents with intellectual disabilities likely also foster reduced development of maladaptive behaviors. Finally, staff that are trained to understand and deal with the emotional needs and stresses of their residents will better provide an emotionally supportive environment that will minimize the occurrence of challenging behaviors or the perception of the person as "a problem."

Primary prevention of the major mental disorders such as schizophrenia is less likely, as there is a large biological and genetic component to most of these. However, the recurrence of individual episodes of illness can be minimized by reducing stressors if possible, providing sensitive support for those that do occur, and ensuring appropriate medication use.

Goal 6 To develop living environments that are responsive to the mental health needs of older people with intellectual disabilities.

Secondary prevention of mental and behavioral disorders involves appropriate early detection, assessment and treatment of the designated problem, by careful involvement of biological, psychological and social interventions. It is crucial to involve the person themselves, staff, family and community in the holistic treatment planning process, and provide sufficient training to allow carers to continue therapeutic interventions after any professional involvement has ended. Modifications may need to be made to the home and work environment and/or staff approaches to the person. Needs that may be expressed in a maladaptive behavioral way must be met more productively, and alternate expressions taught. Supportive therapy, individual or group behavioral therapy, family therapy and social skills training might all be of help, as might be the involvement of spiritual elders or healers, depending on the cultural milieu. Unfortunately, there are too few clinicians, even in the developed world, who have the skills to undertake psychotherapy for individuals with intellectual disabilities. There are fewer still who are aware of the psychological issues related to functional decline, grief secondary to loss of family or friends, and other life changes that take place as people age.

Pharmacotherapy is most often used in the most severe, potentially harmful behavioral syndromes or in the more biologically driven mental disorders, and must be tailored to age related vulnerability. Medication pharmacokinetics, including drug volume of distribution, protein-binding, hepatic metabolism and renal clearance need to be considered in formulating psychotropic regimens. Treatment response time often lengthens with old age, and strange environments such as inpatient settings may result in significant stress that makes the assessment of change difficult. In addition, some older adults with intellectual disabilities may be receiving medications for chronic medical conditions, and the potential for drug interactions should be carefully considered. Thorough knowledge of the biomedical state of each older adult, as well as close coordination with primary health care providers, is necessary for the safe prescription of psychotropic medications. Adverse effects such as sedation, increased confusion, constipation, postural instability, falls, incontinence, weight gain, sex steroid dysregulation and other endocrinologic or metabolic effects, impairments of epilepsy management, and movement disorders must be minimized.

There must always be the awareness of risk and benefit calculations that require detailed knowledge of the specific adverse effects and drug interactions of each particular agent. The potential for acute and long term adverse effects should be determined and discussed with adults and carers at the time of initial prescription and during regularly scheduled psychotropic medication reviews.

Tertiary prevention, or the treatment of established disorders with the goal of minimizing further functional disabilities, becomes more important with the increasing age of the person. Although older people, as do young people, have the right to safe, effective treatment, at times the aging process has brought about so many changes that a realistic goal becomes modified from cure to maximization of overall psychosocial outcomes. The maintenance of mobility, the preservation of meaningful social interaction, and the maximizing of cognitive and affective functioning becomes paramount. Possible hazards and unpleasant side effects of treatments must balance the reasonable likelihood of positive response, resulting in difficult end-of-life decision making for the person and significant caring others.

Goal 7 To promote mental health and minimize negative outcome of mental health problems in older people with intellectual disabilities

6. Service provision

Formal services that specifically provide mental health care to older people with intellectual disabilities are minimally to nonexistent throughout the world. Service provision needs to be adapted to best deal with the local cultural and health care environment, and this is very variable. In some areas basic life necessities, let alone mental health delivery to the general population are not yet available, and the disabled population is often last to benefit when this does come about. The primary need may be basic supports in these areas, whereas in other more privileged areas sophisticated education about the assessment and treatment of behavioral and mental disorders to care providers may be a reasonable goal. An overriding goal, however, in the development of any of these diverse services is to include the acceptance of basic principles. These include maintenance of respect for the individual and their families, involvement of the person's own needs and wishes in any treatment plan, and finally development of treatment plans that are minimally restrictive, culturally sensitive, and that foster the growth and autonomy of the person. All treatment programs should be broadly based with biological, psychological and social components.

Goal 8 To increase mental health services and supports in their own communities for older people with intellectual disabilities.

Goal 9 To collaborate with older people with intellectual disabilities and their support system in developing culturally sensitive, humane, and least restrictive mental health interventions with an integrated bio-psycho-social orientation.

7. Quality of life issues

During the past decade there has been increasing concern regarding the outcomes of treatment and involvement in intellectual disability services in the assessment of the social value of services. A similar shift has also occurred in other sectors, such as child and adult social services, public health, youth corrective activities, senior services and mental health. This type of reorientation in most sectors represents a substantial change in how the benefits of human services and other public or humane enterprises are gauged. The intended end result is tailoring of the services and supports to each individual in ways that encourage and promote the participation of that particular person with an intellectual disability in valued social roles. This is achieved by focusing the benchmarks for effective services upon outcomes with evident lifestyle impacts.

These desirable lifestyle impacts are usually embodied by the expression"quality of life," but are informed by philosophical implications of human and disability rights developments in many nations. From this standpoint, the value of professional services delivered in a high quality manner, the effects of those services, and the efforts of social groups, service groups, and advocates are ascertained with regard to impacts on lifestyle and related personal and social opportunity.

Valued outcomes that serve as a basis for demonstrating the social value of intellectual disability services, but which may vary in their particulars within different cultures, may include: (1) Increased practical, leisure, or life enhancing skills, such as those involved in making choices between alternative activities, and those which allow a person to access community opportunities (e.g., work or retirement activities), including enduring benefits; (2) Improved or maintained dietary and general health status that prevents physical health factors from becoming an untoward hindrance on typical activity; (3) A varied rhythm of life involving preferred activities and recognition that challenge and productivity must continue throughout old age; (4) Participation on a regular and full basis in the general life of their community and with friends and acquaintances of one's preference; and (5) An increased and well-established social network of acquaintances, friends and valued social amenities.

With increasing age, gerontological research has validated the expected belief that engagement and minimization of life stressors have health preventive value and can lead to prolonged life and stable health status. Life factors that provide for sound nutrition, access to valued activities, safe and pleasant domicile, and intellectual challenge can minimize stress, organic or environmentally derived psychopathology and reactive behaviors. A quality old age among persons with intellectual disabilities will be based on the same factors that provides for a quality old age among other persons.

Goal 10 To improve the quality of life in older people with intellectual disabilities and mental health problems

8. Research

Most research in the area of mental or behavioral disorders or problems has had treatment as its focus. Much less has been done about the causes and risk factors of such disorders and their prevention. Almost all of the data available comes from populations of persons with intellectual disabilities from nations with established market economies, where research funding has been most available and there has been a critical mass of workers who specialized in this field. For instance, prevalence data for psychiatric and behavioral disorders may differ between nations with established market economies and developing nations and treatment outcomes may vary where the cultural ethos may inhibit referrals and special resources or services are limited. Improved health status and prevention in developing nations, the principal goal of WHO, must depend on identification of special issues pertaining to developing nations and application of techniques that permit information to be gathered free of cultural or other restraints.

Well-controlled research in mental and behavioral disorders as they occur in persons with intellectual disabilities is limited. Most of the work over the past 30 years addresses treatment issues; fewer focused on diagnosis or etiologic factors, or prevention. Only a small number address basic mechanisms. These disappointing data probably reflect several things, including a well-known lack of a research focus or funding. As a consequence, there are limited numbers of scientists in the field and a lack of programmatic efforts in research centers addressing any relevant issue related to intellectual disabilities. Without specific attention from health planners and ministerial level policy makers, as well as a critical mass of investigators working on a common problem in programmatic ways, little converging data can emerge and, quite likely, few if any major discoveries will appear quickly.

Promising lines of inquiry relate to both treatment strategies and biological determination and regulation of behavior. Rigorous methodologies are available to undertake controlled or randomized clinical trials for behavioral and pharmacologic interventions. Recent advances in molecular genetics and neuropharmacology provide new opportunities for linking severe behavioral and psychiatric disorders to brain neurochemistry. The field must move toward a research focus that includes a better balance of studies of basic mechanisms, translational and clinical outcome studies.

Goal 11 To develop a research agenda that will provide evidence concerning each goal for all nations.

9. Conclusions

Aging issues in older persons with intellectual disabilities still remain to be appropriately identified, assessed and resolved. The complex interaction between biological, psychological and social aspects is arguably the most important area of need at the start of the next millennium. Psychiatric and behavioral disorder prevalent among adults with intellectual disabilities may be both transnational and culture bound. The prevalent literature is based in the nations with established market economies where the longevity of adults with intellectual disability is more pronounced and has become a normative phenomenon. To what extent this same longevity and prevalence of psychiatric and behavioral disorders is shared among nations, other than those with established market economies is unknown.

The analyses in this paper rely heavily on research results from nations with esta