Discussion Paper for Aging SIRG Round Table 2009
Ageing in Adults with Down Syndrome
Age Related Morbidity and Mortality
The emergent population of people with Down syndrome (DS) living into older age
(>40 years) in developed countries is a phenomenon of the last decade and is due to
the alignment of a number of factors:

DS is the single most common cause of intellectual disability.

Life expectancy of people with DS has increased from 10 years in the 1930s to
the mid to late 50s today. One year survival rates for DS in Western Australia
have increased from 46% in the 1940s to 80% in the late 1960s and early 1970s
(Mikkelsen 1981) to 90% in the 1990s (Leonard, Bower et al. 2000). Median life
expectancy for people with DS in the US has increased from 2 years in 1968, to
25 years in 1983 and 49 years in 1997 (Friedman 2001; Yang, Rasmussen et al.
2002). By 2000 75% of people with Down syndrome in Western Australia had
survived to age 50 years, 50% to 58.6 years and 25% to 62.9 years (Glasson,
Sullivan et al. 2002)

The baby boom population with DS is now in 6th decade and early 7th decade.
The population prevalence of Down syndrome in developed countries is in the order of
6-8 per 10 000 (Steele and Stratford 1995; Besser, Shin et al. 2007; Mantry, Cooper et
al. 2008). People with DS now account for 10-18% of the identified population with
intellectual disabilities (Singer and Strauss 1997; Glasson, Sullivan et al. 2002; Mantry,
Cooper et al. 2008). There has been a rapid increase in the number of people with DS
aged 40 years Indeed the average age of adults with DS in Scotland is 40 years
(Mantry, Cooper et al. 2008).
The flip side of increased life expectancy is the increase in mortality from the age of 3540 years. Life expectancy for people with DS appears to have plateaued between 55
and 60 years over the last 1-2 decades. There is a change in mortality patterns from
age of 40 years. Respiratory infection becomes the leading cause of death cf congenital
heart disease in those 19 - 40 years. (Bittles, Petterson et al. 2002)
What factors lead to increased mortality after age of 40 years? Factors identified in
various studies include

Alzheimer’s disease

Skill decline

Behaviour change

Apo E 4

Seizures

Mobility impairment

Vision impairment
Alzheimer’s disease is associated with premature mortality, secondary to complications
such as respiratory infections and seizures. It is well established that adults with Down
syndrome have very high rates of dementia of Alzheimer’s disease with average age of
diagnosis in the early to mid 50s. A prospective, longitudinal study in the Netherlands of
506 adults with Down syndrome aged 45 years and older found the prevalence of
dementia by age group to be: up to 49 years – 16.8%; 50-54 years – 17.7%; 55-59
years – 32.1%; 60 years plus – 25.6%. The prevalence of dementia doubled every 5
years up to 60 years. After the age of 60 years there was no actual decrease in the
incidence of dementia and the fall in prevalence was attributed to the increased
mortality associated with dementia. (Coppus, H. Evenhuis et al. 2006). Average life
expectancies of people with Down syndrome may not increase much beyond 60 years
until preventive or disease modifying treatments for Alzheimer’s disease become
available.
In addition to premature mortality the dementia of Alzheimer’s disease is
associated with high rates of morbidity including functional decline, increased
dependency as well as increasing morbidity including behavioural and psychological
symptoms of dementia, seizures, mobility impairments, feeding and swallowing
difficulties and aspiration pneumonia, often recurrent.
Older adults with DS also face considerable levels of other age related morbidity
and disability, especially sensory impairments and musculoskeletal disorders, which are
earlier in onset and of greater prevalence than people with other intellectual disabilities
and the general population. These are important disorders because of the associated
functional disability, pain, and distress, as well as the increased risk of mortality
associated with vision and mobility impairments. Functional decline in older adults with
Down syndrome cannot be assumed to be due to dementia of Alzheimer type which is
not inevitable in all adults with Down syndrome during current life spans. Careful
assessment is critical to appropriate treatment, management and the provision of care.
Sensory Impairments
Vision impairment including blindness
Hearing impairment including deafness
Musculoskeletal
Osteopororis
Osteoarthritis
Muscle strength, balance, falls
Mental Health
Lower overall mental ill health
Conflicting evidence regarding behaviour disorder and age
Behavioural and psychological symptoms of dementia
Life Events
Life events in older persons with Down Syndrome
Paul J. Patti, MA
George A. Jervis Clinic
New York State Institute for Basic Research in Developmental Disabilities
Staten Island, NY 10314 USA
When planning for the needs of people with intellectual disabilities (ID) as they
age through the life span, the impact of life events exposure on their behavior must be
taken into account. Life events exposure can have a direct as well as an indirect
influence on physical and mental health. People with ID respond to life events in a
similar way as people in the general population. A growing number of studies have
reported a significant relationship between life events exposure and psychiatric
problems in adults with ID [1-5]. Personal loss, moving to a new residence and
changing jobs have been implicated as risk factors for mental and physical ill health.
Adults with ID who experienced more negative life events also exhibit more behavior
problems and depressive symptoms [4].
It is likely that in different situations people with ID will experience one or more
life events as they age through the lifespan. When and where they occur can have a
bearing on their health status and placement. Thus far, few studies have investigated
the life events exposure in adults with Down syndrome (DS). Hamilton et al. included
129 DS adults in a sample of 624 ID adults (mean age 34.2 years) [6]. A strong
relationship between life events frequency and emotional/behavioral problems was
found in DS adults who were functioning in the mild range but not for those in the
moderate to severe range of ID. Patti et al. analyzed the life events experienced over a
5 year period by 211 adults ages 50 and over with ID who were living in community
group homes [6]. The findings indicated that adults in the sixth decade of life with DS
experience a greater number of life events than their non-DS counterparts of similar or
older ages. Relocations and medical events were significantly greater in those with DS,
whereas a significantly lower number of occurrences in all life events categories were
found in the non-DS group. In a follow-up study, there were significantly more
relocations over a 5 and 10 year period (i.e. changing group homes, nursing home
placement) due to functional decline associated with dementia for those with DS. Those
without DS tended to remain in their group home setting. The cumulative exposure of
life events with the additional onset of medical frailties associated with aging can
combine to create a stressful period for people with DS and often result in relocation to
another residential setting [7, 8].
For persons with and without DS living at home, the death of a parent can lead to
a change in location (e.g. moving in with a relative, moving to a group home). In a
recent report, placement out of the home and parental death were predictors of change
in health, functional abilities and behavior problems [23]. For older adults living in group
homes, the death of a parent or family member, changing day programs, and
loss/separation of a friend or roommate were more frequent occurrences in the lives of
adults with DS [7]. Changes in group home placement or other relocations regardless of
the underlying reasons can also have a negative effect on behavior and ability to
interact with others.
The general consensus is that adults with DS tend to experience more medical
changes after age 50 than those without DS. This coincides with the increased
incidence of dementia in DS adults [10, 11]. Often a person displaying age-related
decline needs to be moved to an alternative setting for increased care or for safety
management issues. Separation from familiar staff and peers can cause behavioral and
adjustment problems especially for those adults with DS who may also be experiencing
early signs of dementia. Medical events such as late-onset seizures, pneumonia,
fractures, and new medical conditions were found to occur more often after age 50 in
older adults with DS than those without DS. The increased number of relocations and
placements encountered by older DS adults may even contribute to their accelerated
rate of decline even when dementia is taken into account.
Care provider agencies need to monitor older adults with ID who have
experienced one or more life events because they are at risk for consequential change
in their behavior or functioning. Indications are that life events exposure can have a
cumulative effect over time which can impact behavior, health and stability. Recent
reports indicated that relocations and medical life events appear to occur more often in
older DS adults, and their effects may not be immediately recognized by family
members or group home staff [7, 8]. Further studies investigating life events and
changes in older adults with DS are necessary to identify and evaluate their short and
long term effects on health, placement outcome and mortality. Discussion Topics
Guidelines/ Guidance for the health care of older adults with DS
Guidelines/ Guidance for the health care and care of adults with DS and AD
Given the high rates of early onset age related disorders programmatic screening,
monitoring and preventive interventions are required to limit disability and premature
mortality. In the absence of specialist disability physicians, geriatric medicine has a role
to play. More research is required on preventive interventions, in particular in disorders
of swallowing and mobility.
Some researchers have provided guidance on annual screening in older people
with Down syndrome. (Evenhuis, Theunissen et al. 2001) recommend annual hearing
and vision checks. (van Allen, Fung et al. 1999) provide guidelines on annual health
assessment of older people with Down syndrome. However research is required on
preventive measures in particular with regards to disorders of eating drinking and
swallowing and musculoskeletal functioning.
What guidance should be given to clinicians and carers caring for someone with
DS and AD?
How should health systems meet responsibilities under the UN Convention on
the Rights of People with Disabilities?
The model of care in developed countries has split intellectual disability from
health services with the general expectation that generic health services will meet the
specific health needs of people with intellectual disabilities. Furthermore aged social
care and geriatric health generally have a service age of 60-65 years and this precludes
people with Down syndrome with early onset age related disorders. The potential
complex of multiple morbidities in ageing adults with Down syndrome as well as other
intellectual disabilities is an argument for specialist physicians and multidisciplinary
teams in intellectual disabilities. In the absence of specialist adult physicians in
intellectual disability, geriatricians are well placed to assess and manage the complex
mix of disorders presenting in older people with Down syndrome.
Who should manage complex health needs?
Training of geriatricians needs to address the health care needs of older adults
with Down syndrome and other intellectual disabilities. Policy needs to change to
provide a range of aged care services to people with Down syndrome from the age of
40 - 45 years. In addition programmatic proactive screening, monitoring and preventive
interventions are required to limit disability and premature death in older people with
Down syndrome. When preventive and disease modifying treatments become available
for Alzheimer’s disease the average life expectancy of people with Down syndrome
could increase well beyond 60 years and the numbers of elderly people with Down
syndrome will increase substantially beyond current expectations posing new
challenges to social and health care services.
Supporting Ageing in Place, Minimising Transitions
References
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