AGING IN DEVELOPMENTAL DISABILITIES (R)

DR.JAY RAOM.B.B.,S. ,D.P.M. ,M.R.C.PSYCH(U.K.)., F.R.C.P.(C).

ASSOCIATE PROFESSORUNIVERSITY OF WESTERN

Approximately 80,500 individuals age five and over in Ontario have a developmental disability (2009)

16,000 individuals above 45 years of age.

“the number and proportion of older people with intellectual disability is increasing and will continue to do so until after the baby boom generation moves into later life in 2012.”

Age Range

Year Projected

Population

Prevalence 0.4%

Prevalence

0.5%

Difference

65+ 2007 63,922 255 319 64

2018 89,240 357 446 89

45-65 2007 226, 838

907 1,134 227

2018 241,267

965 1,206 241

Uncertain Prevalence of Developmental Disabilities Among Older Adults in the CRD,

2007 and 2018

The aging and disability service systems have historically developed in parallel but separate tracks ...

… despite often overlapping concerns about issues such as affordable housing, public transportation, access to healthcare, long-term care needs, and economic stability.

As a result, the geriatric care system lacks the disabilities-specific skills and experience needed to care for older adults with developmental disabilities.

A recurrent theme in the literature is that while the disabilities care system and the geriatric care system share many common concerns, neither is well equipped to provide services to older adults with developmental disabilities.

The former lacks skills and experience with geriatric care; the latter lacks skills and experience with disabilities.

LIFE EXPECTANCY AND AGING IN DEVELOPMENTAL DISABILITIES

1. LIFE EXPECTANCY WAS LOW IN THE 1920s.

2. For Down’s, it was in the early 20s.

3. A large number were in institutions.

4. Cause of death was usually Bronchopneumonia.

5. TODAY, LIFE EXPECTANCY IS AROUND

67 YEARS OF AGE.

U.S. data suggest that the mean age at which people with developmental disabilities die was

66 years in 1993

59 years in the 1970s

19 years in the 1930s

Among people with less severe disabilities who do not have Down’s syndrome, life expectancy is now approaching that of the general population.

On the basis of changes in life expectancy and the aging of the baby boom generation, U.S. studies

suggest that …

…the total population of people with developmental disabilities aged 55 and over will double by 2030.

Some conditions occur in people with developmental disabilities more often than in the general population, for example,

thyroid disorders, heart disorders, sensory impairments.

the rate of psychiatric problems among the older people with developmental disabilities is two to four times the rate in other older people.

Other age-related health issues are more frequent in people with particular syndromes.

The best known are :

the increased risk of precocious aging, dementia, and increased sensory loss in people with Down’s syndrome.

adults with Down syndrome have a higher prevalence (15% to 40%) of early-onset Alzheimer’s disease occurring 15-20 years earlier compared to the general population,

may experience hypothyroidism and sleep apnea more frequently (McCarron, Gill, McCallion, & Begley, 2005).

osteoporosis in women with cerebral palsy due to long-term inactivity

osteoporosis in people with epilepsy due to their use of medications

bladder and swallowing problems in people with cerebral palsy

mitral valve prolapse and musculoskeletal disorders in people with Fragile X syndrome

Adults with Fragile X may have more issues with heart problems (mitral valve prolapse), musculoskeletal disorder, earlier menopause, epilepsy, and visual problems.

diabetes, cardiovascular disease, and obesity in people with Prader-Willis syndrome

As persons with cerebral palsy age, they have >>> an increased likelihood of having reduced mobility, bone demineralization, fractures, decreased muscle tone and increased pain, difficulty eating or swallowing, and bowel and bladder concerns (White-Scott, 2007).

People aging with cerebral palsy and epilepsy who use psychotropic and anti-seizure medications on a long-term basis also have a higher risk of developing osteoporosis (brittle bone disease) and tardive dyskinesia (repetitive, involuntary, purposeless movements caused by the long-term use of certain drugs).

This risk is often compounded by limited physical activity and diets low in calcium and vitamin D.

Anti-epileptic medications are also frequently given long-term to individuals with developmental disabilities.

Studies suggest that osteoporosis and osteomalacia (softening of the bones) are potential side effects of certain antiepileptic medication, and vitamin D may be reduced, leading to possible loss of bone mass.

AGING in Developmental Disability

As in the general population, aging brings the following problems:

PHYSICAL PROBLEMS

1. Cardiovascular disease

2. Musculo-skeletal disease

3. Gastro-intestinal problems

4. Sensory problems

Caution:

the Incidence and Prevalence of Dementia may be higher in Down’s.

But we have no population based data on Incidence and prevalence in other Developmentally disabled for specific comparison.

Alzheimer’s-like brain pathology alone does not indicate Alzheimer’s in Down’s.

Down’s, even in their 20s may have such brain configuration without actually manifesting any clinical decline.

Psychiatric problems

( HIGHER INCIDENCE AS ONE GETS OLDER)

1. Depression

2. Anxiety disorders

3. Mood disorders

4. Psychosis

COGNITIVE PROBLEMS

Slower ability to process informationMemory problemsAttention DifficultiesExecutive function deficits (impulsivity, poor problem

solving ability, difficulty in shifting, mood dysregulation)

Communicational difficulties

What is the BASE LINE? Developmentally disabled may already

have:

1. Epilepsy

2. Brain tumors (Tuberous sclerosis)

3. Immature, miswired cortex.

4. Eye (cataracts) and hearing problems

5. Poor articulation, expressive and Receptive language problems

What is the base line?

7. Thyroid problems (ex: Down’s)

8. Cardiac defects (ex: Down’s, VCF, Tuberous Sclerosis)

9. GI malformations/ Swallowing difficulties

10. Kidney problems (tuberous sclerosis)

11. Skeletal Deformities

12. Lung/Immune deficiencies

WHAT IS THE BASE LINE?

13 Anxiety disorders.

14 Mood instability

15 Executive function deficits

16 Memory and Attention difficulties

Contd:

Given such pre-existing conditions, the developmentally disabled are more likely to decline faster, with aging.

Often, these are not known because of inadequate health evaluation.

Psycho-Social Aspects: :

Older developmentally disabled experienceMORE LOSSES AND INCONSISTENCIES WHILE IN

CAREPOORER ACCESS TO MEDICAL FACILITIESFINANCIAL HARDSHIPSPOORER NUTRITIONLESS ACCESS TO RECREATION AND

APPROPRIATE JOB/ OCCUPATIONAL INVOLVEMENT

Context of aging

General population

there are declines in speed of processing, working memory, inhibitory functions, long term memory, decreases in brain structure and white matter

integrity (Parks, Reuter-Lorenz)

Medical morbidity, health and nutritional risks increase

Psycho-social

problems gather force

Developmentally Disabled

There may be pre-existing cognitive problems

Pre-existing Health and nutrition problems

Pre-existing psycho-social problems

Three Factors to be considered in agingNeuro-medical

vulnerabilities

Neuro-developmental issues

Ex: Scaffolding

Neuro-Executive Issues

Developmentally Disabled at higher risk for these

DD at disadvantage due to developmental immaturity of brain architecture

Pre-existing executive brain dysfunction

Neuro-developmental issues--- Scaffolding

In the younger brain: specialization of circuitry Ex: Remembering, working memory tasks, Novel

tasks In response to challenges, initially, a wider set of

neural circuits are recruited.

These are Scaffolds

As the task is over-learned, a specific, honed circuit is developed.

This provides the ability for efficient cognitive operations

In the older brain

Scaffolds are invoked To perform even familiar tasks and basic cognitive processes

In the olderScaffolds (wider net works) are recruited

even for low levels of task demand

(Ex: remembering where one put the car keys)

In the older

Generating scaffolds and recruiting them is even more inefficient

because of aging pathology

In the older Developmentally disabled we propose

Scaffolding, even in younger ages is inefficient

There is impaired ability to recruit Pre-frontal networks, especially

bilaterally

In older ages neurobiological decline is rapid or more profound in its impact resulting in poor scaffolding capacity

Whatever scaffolding there is , is penetrated by neural pathology leading to collapse of the scaffolds

(Parks, Reuter-Lorenz; Burke and Barnes;)

Executive Functions

Inhibit

Shift

Emotional Control

Monitor

Working Memory

Plan/ organize

Organization of Materials

Task Completion

EXECUTIVE FUNCTIONS

BRIEF

(1) the brain shrinks in volume and the ventricular system expands in healthy aging. However, the pattern of changes is highly heterogeneous, with the largest changes seen in the frontal and temporal cortex, and in the putamen, thalamus, and accumbens.

(2) The volumetric brain reductions in healthy aging are likely only to a minor extent related to neuronal loss. Rather, shrinkage of neurons, reductions of synaptic spines, and lower numbers of synapses probably account for the reductions in grey matter. In addition, the length of myelinated axons is greatly reduced, up to almost 50%.

(3) Reductions in specific cognitive abilities--for instance processing speed, executive functions, and episodic memory--are seen in healthy aging. Such reductions are to a substantial degree mediated by neuroanatomical changes, meaning that between 25% and 100% of the differences between young and old participants in selected cognitive functions can be explained by group differences in structural brain characteristics.

Several studies have suggested that cognitive deficits in normal aging arise from alterations in the functional integration of these coordinated brain systems

Altered fronto-occipital connectivity has also been observed with increasing

age (Moeller et al., 1996),

Fronto-parietal connectivity has been identified as important in memory function

alteration in patterns of functional connectivity

during memory tasks in older adults. Notably, many long-range connections between fronto-temporal, fronto-occipital, fronto-parietal temporal–parietal

regions were found to be impaired.

Aging & Neural Connections in AutismFrontal and Temporal development are stunted

at an early stage leading to lack of differentiation

This lack of differentiation leads to hyper-connectivity

Blocks coherence development with other critical brain regions

Lobes of the Brain

Connectivity problemsHYPO-connectivity

Orbito-frontalMixed sensory-motorOccipital/Parietal-

TemporalFrontal-posteriorLeft Intra-

hemisphere

HYPER-connectivity

Frontal-temporal

Left Hemisphere intra-hemispheric

Neural Changes with agingOrbitofrontal:

DisinhibitionLabilityIrritabilityImpulsivitySexual preoccupationDistractability

May go unrecognized

Lobes of the Brain

Neural Changes with aging Ventromedial PC:

Decreased verbal outputDiminished motor initiationWithdrawalapathy

Lobes of the Brain

Neural Changes with Aging Dorsomedial PC:

ApathyAkineticmutismincontinence

Lobes of the Brain

Neural Changes with Aging Dorsolateral PC:

Working memory Spatial Object-faces Verbal

Executive functionsLanguage sequencing

Neural Changes with Aging Frontal lobe:Dysfunction results in:

DisinhibitionEmotional labilityIrritabilityLack of drive, motivationDeficits in memoryAttentional deficitsApathy – akinesia – AbuliaAphasia

Neural Changes with Aging Temporal lobe:Dominant:

Euphoria Auditory hallucinations, illusions Thought disorder Anterograde amnesia Receptive language deficits Memory impairment

Non-dominant: Dysphoria Disinhibition of sexual and aggressive behaviours Cognitive difficulties

Neural Changes with Aging Parietal:Dominant:

Alexia, agraphia, acalculia Agnosis, left-right disorientation

Non-dominant: Impaired spatial ability Anosognosia Autopagnosia Apraxia, etc.

Neural Changes with Aging Occipital:

Disturbed spatial orientation (metamorphopsia)

Visual illusions

Visual hallucinations, etc.

CASE HISTORY - I

38 yr. old female, admitted with two months history of poor memory, disinhibition, emotional dyscontrol, incontinence of urine and bowels.

Worked as a cashier in a store for 12 years previously ( job shadowing)

All investigations normal.Mental status exam unproductiveDEPRESSION AS DEMENTIA

CASE HISTORY - II

67 year old man in a group home, previously well functioning, gradually became more withdrawn, irritable, forgetful, paranoid, impulsive.Did not enjoy activities, became very quiet.Treated with anti-psychotics, anti-depressants.Became more irritable, rages, ParkinsonianNeuro psychological assessment revealed

serious deficits.MRI indicated degenerative changesDEMENTIA AS DEPRESSION

EVALUATIONMULTIFACTOR EVALUATION is essentialCareful researching of past medical history and family history.Multidisciplinary involvementUse of structured inventories/rating scales

BUT REMEMBER:

THESE SCALES ARE NOT DIAGNOSTIC INSTRUMENTS but tools to enable management

MULTIFACTORCHALLENGES

I) Bio-Medical Conditions

II) Psychiatric Conditions

III) Developmental Disorders

IV) Emotional Issues

V) Sensory Processing Issues

VI) Environmental Factors

VII ) Communication Difficulties

VII) Learned Behaviour

. Bio-Medical Conditions Check Specify

Epilepsy A) Tonic/Clonic, generalized

B) Tonic/Clonic, C) Focal generalizedD) FocalE) AbsenceF) Complex partialG) Lennaux-GestautH) Other

Medical Disorders A) Heart disease

B) Lung disorders (asthma etc.)C) GI Disorders (GERD, hernias)D) AllergiesE) Endocrinal disordersF) Food sensitivitiesG) ConstipationH) ArthritisI) Chronic/acute painJ) Ear infectionsK) Diabetes

Neurological problems A) Head injury

B) MeningitisC) EncephalitisD) MigraineE) StrokeF) Movement disordersG) other

Vision problems A) cataracts

B) retinal damageC) GlaucomaD) BlindnessE) Poor visionF) Depth vision problem

Hearing Problems A) Loss of hearing

B) TinnitusC) HyperacusisD) deafness

Sleep Disorders A) initiating and maintaining

B) hypersomniasC) Sleep-WakeD) ApneaE) NarcolepsyF) Other

Medication related A) Akathesia

B) Abnormal involuntary movement disordersC) ConstipationD) DroolingE) DystoniF) Dry mouthG) other

V. SENSORY PROCESSING ISSUES Check Specify Rating*

28

Tactile function problems A) Oversensitive to touch

B) Under sensitive to touchC) Difficulty in tactile discrimination

1 2 3never some significant

29

Proprioceptive Dysfunction A) Sensory seeking

B) Difficulties in grading movements 1 2 3

30

Auditory function problems A) Oversensitive to sounds

B) Under sensitive to sounds 1 2 3

31

Vestibular Dysfunction A) Poor co-ordination

B) Poor muscle toneC) Oversensitive to movementD) Under sensitive to movement

1 2 3

32

Visual Function problems A) Oversensitive to visual input

B) Under sensitive to visual input 1 2 3

33

Oral Function Problems A) Oversensitive to Oral input

B) Under sensitive to Oral input 1 2 3

34

Olfactory function problems. A) Oversensitivity to smells

B) Under sensitivity to smells 1 2 3

Auditory-Language Processing Difficulties

Internal Regulation Difficulties.

Self-Regulation Difficulties

Impaired Social interactions

Emotional Regulation difficulties

VII COMMUNICATION DIFFICULTIES

Specify Rating*

47Speech problems

a) Dysfluencyb) Articulation problemsc) Voice disorders

1 2 3never some significant

48Language disorders

a) Aphasia (expressive, receptive, anomic, global)

b) Receptive language deficit

c) Expressive language deficit

d) Improper use of words, meaning

e) Inappropriate grammatical use

f) Reduced vocabulary

1 2 3

Auditory Processing Disorders

a) Difficulty with multi step instructions

b) Poor listening skillsc) Difficulty attending to

auditory informationd) Difficulty reading,

comprehension

Changes in environment

Stressful work/living environment

Care provider skill/competency deficit

Conflictual Home environment

Legal, Financial difficulties

ENVIRONMENTAL FACTORS

INVESTIGTIONS

CT, EEG,MRI,ULTRA SOUND,X-RAYBLOOD WORK – THE USUAL Neuro-cognitive assessmentsSkills assessments (OT)

TreatmentAssessment is the cornerstone

Treat physical as well as psychiatric issues

Dementia forms a small proportion of the problems in this population

Physical decline, cognitive difficulties, isolation, loneliness, losses, poor nutrition, neglected health issues, mood instability are more pressing problems in this population

Aging is a more challenging problem than dementia

This is true in the developmentally disabled because of the neuro-bio-psycho-social decline.

As more of the developmentally disabled get older, we may need to develop strategies for support ,and anticipate the resource implications

End of slide show.

Extra material follows leading to neurobiology of aging presentation (70 + slides)

0 3m 2y ADULT 30 + yrs

Specifically, more differences were observed in dorsal frontal and parietal regions with relatively few differences observed in cortices of the temporal and occipital lobes.