behavioral changes and management strategies with the aging person with down syndrome lilian thorpe...

Behavioral Changes And Management Strategies with the

Aging Person with Down Syndrome

Lilian Thorpe MD PhD FRCP

Geriatric Psychiatrist

Professor,

Departments of:

Community Health and Epidemiology

and

Psychiatry

University of Saskatchewan

Overview

• Down Syndrome: The “Cook’s Tour”• Life-story: Gayle• Management strategies

– Assessment– The individual– The environment– Collaborations

Down Syndrome: A Brief Summary

History of DS• First complete description of DS by Seguin, 1846• Report by Down on “Observation on the ethnic

classification of idiots” 1866 • 62 cases published by Fraser and Mitchell 1876

mentioned maternal age as a risk factor, described phenotypic description, neuropathology and noted dementia risk.

• LeJeune & Jacobs determined DS was caused by trisomy 21, 1959.

Korenberg JR et al. Advances in the understanding of Chromosome 21 and DS. IN: Down Syndrome: Advances in Medical Care. Ed Lott & McCoy Wiley-Liss 1992

DS: Genetic Spectrum

• Most have complete trisomy of chromosome 21

• Approximately 5% have only partial trisomy 21 (from translocations)

• About 2% have mosaicism (some cells with trisomy 21 and some without).

Nora & Fraser (Eds). Medical Genetics. Principles and Practice, 4th edition. Lea and Febiger Phuiladelphia1993

Korenberg JR et al. Advances in the understanding of Chromosome 21 and DS. IN: Down Syndrome: Advances in Medical Care. Ed Lott & McCoy Wiley-Liss 1992

Global Trend in Life Expectancies in DS

Year publication

Mean Life Expectancy

Country

1929, 1949 9, 12 England

1963 18 Australia

1973 30 USA

1982 35 Transnational

1991 56 USA

2002 60 Australia

Bittle & Glasson. Clinical, social, and ethical implications of changing life expectancy in

Down syndrome. Developmental Medicine and Child Neurology. 2004;16:282-286

Percentage Survival Estimates (1 and 10 year) for Cohorts with DS in Australia

0

10

20

30

40

50

60

70

80

90

100

1948-57

1966-76

1976-85

1980-85

1986-90

1991-1996

1 year

10 years

Glasson et al. The changing survival profile of people with Down's syndrome: implications for genetic counselling. Clin Genet. 2002 Nov;62(5):390-3

Survival Curve for DS in Western Australia, N=1332, 1953-2000

Glasson et al. The changing survival profile of people with Down's syndrome: implications for genetic counselling. Clin Genet. 2002 Nov;62(5):390-3

Typical Neuropsychological Profiles Of Adults With Down Syndrome (DS)

Vicari S. Motor development and neuropsychological patterns in persons with Down Syndrome. Behavior Genetics 2006;36(3):355-364.

Motor development

• Motor milestones slightly delayed on average

• Later milestones are more delayed• Wide variation in milestone acquisition• Hypotonia and lack of control of muscle

stiffness are both associated with abnormal gait and postures


Global Cognitive Development: the IQ

• Average IQ for people with DS is usually in the moderate-severe range (IQ=25-55).

• IQ in DS progressively deteriorates with age (unlike in general population)

• IQ deterioration may be associated with early onset Alzheimer's disease (? Prodromal symptoms)


Linguistic Abilities

• Poor in adolescents and young adults with DS– Worse than typically developing peers of the same

mental age– Worse than those with other causes of ID with similar

overall IQ– Telegraphic type of speech

• Production more affected than comprehension• Not definitively shown to be due to hearing

impairment: research either shows no relationship between hearing loss and linguistic abilities, or only only a small contribution (<10%)


Visuospatial Abilities

• Consistent with mental age

• Visual processing more impaired than spatial processing


Memory

• Compared to mental-age typically developing (TD) children, those with DS have poor verbal STM. – This is independent of articulatory

difficulties– Much of this is due to deficits of the

central executive system• DS participants have relatively better visuo-

spatial STM than verbal STM.


Health Issues In Down Syndrome

• Increased mortality rates before age 1 and after 35 YO

• Hearing loss• Vision loss (Cataracts, keratoconus, strabismus)• Cardiac abnormalities (50% in trisomy 21)• Hypothyroidism• Immune functioning (pneumonia, sinus, ear

infections and chronic rhinitis)• Cancer- leukemia

Dalton et al. Association between AD and DS : Clinical observations.IN: AD, DS and their relationship. Ed Berg et al. Oxford University Press1993

Health Issues In Down Syndrome

• Small maxilla and mandible, relatively large, furrowed tongue, abnormal dentition

• Sleep apnea• Increased rates of seizures: 33% in some

studies. 40% of seizures begin before age 1, and 40% after age 30s

• Atresia of portions of the GI tract• Polypharmacy

Dalton et al. Association between AD and DS : Clinical observations.IN: AD, DS and their relationship. Ed Berg et al. Oxford University Press1993

Behavioural and functional changes with aging in DS

• Plateau in most abilities by early 20s• High prevalence of mood and anxiety

symptoms in late adolescence and early 20s, especially at transitions

• Increasing rigidity and obsessional symptoms in mid to late 40s

• Typical symptoms of cognitive decline in late 40s and 50s

• Formal dementia diagnosis in mid to late 50s.

Changed to sertralineless sedated

Completed school, declined workshop

Born in 1957 with DS

Diagnosed with depression age 18, started on amitriptyline

Weight loss begins

Parents decided against institution

Started special school for ID in 1963

Traumatic surgery and dental work: agarophobia

Functional decline, teeth rotting

Stops speaking

Weight 63 lbs & failing

1950 1960 1970 1980 1990 2000 2007

Gayle: DS

Contributors to behavioral changes in older adults with Down Syndrome

• Baseline cognitive and psychological functioning; learned behaviors

• Health challenges with aging– Physical problems (arthritis, pain)– Mental health challenges (depression, anxiety,

psychosis)– Cognitive decline

• Family transitions• Environmental issues

Management: Individual issues Assessment

• Potential contributors to behaviours– Social/environmental factors– Biological factors– Psychological factors

• Pattern and frequency of behaviours (ABC charting)–Antecedents–Behaviours–Consequences

Management

• Social/environmental– Routines Individualize, simplify– Staff functioning Education, support– Mix of co-residents Modify to situation– Physical space, noise Dementia friendly– Light Windows, light boxes– Social involvement and support Involve family– Activities including exercise Increase

Management

• Psychological– Personality style adapt interventions to suit– Past experiences explore and adapt care– Mental disorders

• Depression bio-psycho-social• Anxiety bio-psycho-social• Psychosis antipsychotics (?)

– Comprehension simplify demands– Disinhibition external controls, ?Meds– Poor impulse control external controls, ?Meds

Management

• Biological– Discomfort/pain Treat- regular dosing– Sensory deficits Minimize– Physical illness Optimize– Medication effects “De-prescribing”

Management

The environment

Adapting group homes and clustering people with similar needs

Provide enhanced seniors’ care in larger facilities designed for ID

Adapting workshops to the needs of older people

The nursing home

Thorpe LU, unpublished data, 2008

Percentage of 1429 Residents in 13 Saskatoon Nursing Homes With Intellectual Disabilities

(Data as of August 2007)100.0

34.1 33.3

11.4 12.2 10.13.7 3.6 0.6

7.1

0.0

10.0

20.030.0

40.0

50.0

60.0

70.080.0

90.0

100.0

0 - 19 20 - 39 40 - 59 60 - 64 65 - 69 70 - 74 75 - 79 80 - 84 85 + Total

Age of Admission of Person with Intellectual Disability to Saskatoon Nursing Home:

Percentage in Each Age category. (Data as of August 2007)

0.0

5.0

10.0

15.0

20.0

25.0

30.0

35.0

40.0

0-19 years 20-39years

40-59years

60-64years

65-69years

70-74years

75-79years

80-84years

Thorpe LU, unpublished data, 2008

Nursing Homes

• 13% of older people with intellectual disabilities in New Zealand live in rest homes, designed for the generic older adult population (Hand and Reid,1996)

• 5.9% of people with ID or developmental disabilities (DD) receiving services are in nursing home facilities in the USA, which has decreased by 13.4% since 1970. (Prouty, Smith and Lakin, 2005)

Age of residents with ID in 112 residential aged care facilities in Victoria compared with all permanent

residents in Victorian residential aged care facilities

0

5

10

15

20

25

30

35

40

45

50

<65 65-74 75-84 85-94 95+

ID

All

Bigby et al. A survey of people with ID living in residential aged care facilities in Victoria. J Intellect Disabil Res. 2008 May;52(Pt 5):404-14.

Age of entry of residents with ID in 112 residential aged care facilities in Victoria compared with all permanent

residents in Victorian residential aged care facilities


0

10

20

30

40

50

60

70

<65 65-74 75-84 85-94 95+

ID

All

Difficulties Associated With Residents With ID 'Fitting In'

• The most common issue identified by aged care providers (48%) were the difficulties associated with residents with ID 'fitting in' to the type of activities and support provided by the facility to other residents.

• This was seen as primarily owing to:– Younger age, different interests, difficult behaviours, different care

needs. • Other issues identified were:

– Need for more individualized attention (24%)– Social isolation (11%)– Negative attitudes of other residents (6%)– Lack of appropriate staff training (6%) – Shortage of resources to adapt to their particular needs (10%).


Opportunity For Meaningful Relationships

• Over a quarter of residents were reported as having no positive relationships with other residents (28.0%).

• In the 'exceptional' facilities (those with large numbers of ID residents), only 5% of residents were reported as not having any positive relationships with other residents, compared with 28.7% in the smaller facilities.

• Residents in the two exceptional facilities were twice as likely to have a close or 'special' friendship with another resident than those in facilities with a smaller number (65.0% compared with 33.0%).


Providing developmentally appropriate programming in nursing homes

Management strategies: challenges and barriers

• Related to the individual person (and family) needing services (previous slides)– Medical comorbidities, behavioral/ mental health issues,

level of intellectual functioning

• Related to the providers of services– Education/knowledge, attitudes, reimbursement, support

• Related to the institutions within which people receive and provide services– Fragmentation vs intersectoral collaboration, resources,

philosophy of care

Breaking down barriers: Collaborations

• Maintaining the relationship– Discuss, define and change roles and responsibilities

as needed– Nurture relationships at the personal support worker,

manage and administrator levels through both partner organizations

– Engage in ongoing communication and problem-solving at all levels.

– Be open to different plans and strategies that are jointly developed-consider what works best for the individual.

Stemp & Stephens, Long Term Care, 2011

Medical issues: Empowerment and knowledge

• Improve knowledge of front-line staff and families by educational interventions

• Improve access to online information• Provide practical, brief, and clear

information to the physician– Consider written summary prior to

appointments

Quality of life• Maintain friendships and contacts

– Family– Staff– Other residents– Organizations like churches

• Facilitate community integrations and connections

• Allow for “retirement” or reduced hours• ?Other?

Questions for discussion

• Specialized ID aging or generic aging services?

• Nursing led group home system versus normalized “home” environment?

• Aging in place at all costs or move to nursing home when care gets heavy?

Late Life Treatment Issues

• Cholinesterase inhibitors (ie donepezil or Aricept)

• Other cognitive drugs (memantine or Ebixa)

• Institutionalization• Feeding tubes• Aggressive treatment of aspiration

pneumonias

Questions?

behavioral changes and management strategies with the aging person with down syndrome lilian thorpe...

Documents

downs syndrome

aging person

international year

years of age

life expectancies

complete trisomy of

intellectual disabilities

survival of individuals