Matthew P. Janicki, Ph.D.mjanicki@uic.edu

Nordic Conference on Intellectual Disability and Dementia

Uppsala, Sweden ● 22 November 2010

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Anticipating Onset and ChangeDifferential Effects of Types of DementiaFactors and ModelsAssessing Quality

Anticipating Onset and ChangeDifferential Effects of Types of Dementia

Factors and Models

Assessing Quality

Providing adequate and appropriate care and services to people with intellectual disabilities (ID) affected by dementia

Knowing what are the presenting problems

Looking at what the natural environment offers

Adapting the environment to accommodate changes

Knowing how decline will progress and what behaviors may be evident and when

Continuing to adapt environment, including supports

Planning forward

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when “onset” occurs having readily available assessment and

diagnostic services special needs in early stage – mostly early

memory loss and frustration due to noticed changes

training for creating “dementia-capable” environments, whether at home or in programs

preparing for eventual loss of capabilities (e.g., advance directives, housing, financial instruments, etc)

accommodating late stage care

Onset is speculative . . .

– the best we can do is identify that

point when significant change or

impairment has become noticeable

- in some people noticeable changes

have been proceeded by MCI –

“mild cognitive impairment”

- early 50s for DS – late 60s for ID

Janicki 8

Note difference between DS (yellow)and other ID (green)

Source: janicki, M.P. & Dalton, A.J. (2000). Prevalence of dementiaand impact on intellectual disability services. Mental Retardation,38, 277-289

0

2

4

6

1 2 3 4 5

Hypothetical test scores on series of performance measures over 5 years

Although all generally begin at about same level, different rates of decline occur over time

Lack of training among clinicians

Lack of knowledge of assessment protocols to assess dementia

Lack of assessment services

Lack of familiarity with identifying dementia among medical practitioners

Confusion by symptoms and misdiagnosis

Assessment challenges

Memory loss

Difficulty performing familiar tasks

Problems with language

Disorientation to time and place

Poor or decreased judgment

Problems with keeping track of things

Misplacing things

Changes in mood or behavior

Changes in personality

Loss of initiative

Alzheimer’s Disease International

Janicki 13

► Family brings in adult with ID/DS or raises concern Acute or chronic duration changes

► Problems identified during office visit memory/thinking problems symptoms (weight loss, appearance,

functional difficulties) trigger suspicions

self-report

► Problems identified in health setting nursing or other staff note problems dementia identified in emergency

room, hospital, etc

► Problems identified in community friends, neighbors, church members,

or others report

accidents or falls at home

police referral

confusion and disorientation (getting lost)

► Problems identified within residential setting Noticeable disorientation

Strange behaviors, such as hoarding, wandering constantly, new shadowing, etc.

Loss of interest and notable change in personality

[Source: adapted from Boise et al., 1999 - The Gerontologist]

Applying screening and assessment measures

Controversy as to whether this is intrusive

Balance privacy vs. risk

Benefits

Pick up on early warning signs

Helpful with early differential dx

Associate with data from biomarkers

Easy ways to collect quick impressionistic data

Protocol for recording a set of signal performance tasks and behaviors linked to potential early expression of dementia

Can be used as part of the individual’s permanent record or program plan to periodically track changes in behaviors that might indicate cognitive and motor changes associated with dementia

Available at http://www.uic.edu/orgs/rrtcamr/recordingbehav.htm

Difficult behavior

Fragility

Dementia

Terminal illness

Potential of dying

Age

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Compromising behaviors Agitation

Combativeness and aggression

Resistance

Catastrophic reactions or extremes in emotional response

Suspiciousness

Delusions, and hallucinations

Sundowning

Wandering

Nuisance behaviors

Rummaging

Hiding

Hoarding

Shadowing

Repetitive activities

Pica (eating non-food

things)

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Compromising behaviors Agitation

Combativeness and aggression

Resistance

Catastrophic reactions or extremes in emotional response

Suspiciousness

Delusions, and hallucinations

Sundowning

Wandering

Nuisance behaviors

Rummaging

Hiding

Hoarding

Shadowing

Repetitive activities

Pica (eating non-food

things)

Source: Alzheimer’s Disease: Unraveling the Mystery. (n.d.). US Dept of Health & Human Services

Source: Figure 1 from Wilkosz et al., (2009). Trajectories of cognitive decline in Alzheimer’s disease. International Psychogeriatrics, 28,1-10.

Varying trajectories may be a function of the etiology and nature of the dementia, the health of the person, and/or measurement artifacts

Varying trajectories have implications for continual assessment and adaptations to care management

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• Retrogenesis is the process by which degenerative mechanisms reverse the order of acquisition in normal development• The functional stages of AD precisely and remarkably mirror the acquisition of the same functional landmarks in normal human development • The stages of AD can be translated into corresponding developmental ages and the age-associated needs can be used to account for the overall management and care needs of adults

with AD

Development Decline

years years

[Reisberg et al. (2002). Evidence and mechanisms of retrogenesis in Alzheimer's and other dementias: management and treatment import. Am J Alzheimers Dis Other Demen, 17(3):169-74]

Measures of risk of death due to dementia include: Age

Shortness of breath

Bowel incontinence

Weight loss

Using the 12-item Advanced Dementia Prognostic Tool (ADEPT) –the higher the score the higher the risk of death

Difficult to predict mortality in persons with dementia

High-quality palliative care is prescribed toward end-of-life

Hospice care is recommended

Source: Prediction of 6-Month Survival of Nursing Home Residents With Advanced Dementia Using ADEPT vs Hospice Eligibility Guidelines. Susan L. Mitchell; Susan C. Miller; Joan M. Teno; Dan K. Kiely; Roger B. Davis; Michele L. Shaffer. JAMA. 2010;304(17):1929-1935.

The limitations of defining onset are understood

There is familiarity with symptoms

Assessments are sensitive and targeted toward picking up functional change

Assessments discriminate rationale for decline or change

Assessments help identify trajectories

Assessments help define nature of care adaptations

Anticipating Onset and Change

Differential Effects of Types of DementiaFactors and Models

Assessing Quality

Dementia is a term delineating a set of behavioral presentations… generally associated with memory loss or impairment, behavioral decline, and changes in overall personal functioning

However, changes due to dementia may have different ‘faces’ and progression depending of the etiology and nature of the neuropathology

Knowing the underlying cause of the dementia can help with planning care and estimating future needs

Diagnostic precision

Potential medication treatment variations

Developing expectations of residual life years

Setting up care management plans on expected behavioral presentations and progression

Communication and interaction variations

Projecting expectations for change in care needs

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AD w/

Parkinson's

16%

Other

5%

VAD or

Parkinson's

19%

Alzheimer's

(AD)

60%

Lewy body, fronto-temporal, etc.

VAD: Vascular Dementia

Vascular dementia►Abrupt onset►Underlying vascular

disorder present (e.g., hypertension, heart disease)

►Early impairment in motor skills

►Brain scan shows evidence of strokes or related changes

Alzheimer’s disease►Gradual onset,

relentless progression►Underlying vascular

disorder not always present

►Deterioration in a broad range of intellectual disabilities

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Dementia with Levy Bodies

FrontotemporalDementia

Alzheimer’s Disease

Dx of dementia plus two of the following features:

(1) prominent fluctuation in cognition and alertness(2) recurrent visual hallucinations(3) spontaneous motor features of parkinsonism (not drug-induced)

Early personality change and abnormal behavior; loss of social graces; marked change in character and disordered interpersonal conduct; emotional blunting with loss of empathy and sympathy; “hyperorality” (overeating, food fads, smoking and oral exploration of objects)

Early memory loss, spatial disorientation and language deficits; late personality change (interpersonal skills, social graces, manners are preserved until later in the disease)

Visual hallucinations include seeing people, animals or objects

Depression, delusions, repeated falls and syncope (fainting)

Working memory tends to be worse, episodic memory tends to be better, and sematicmemory tends to be equal to patients with AD

Speech output reduced, adynamic – later mutism

Memory variable

Visuospatial function preserved

SPECT: anterior

hypoperfusion

EEG: normal

Fluent aphasia with poor comprehension

Severe amnesia

Spatial disorientation

SPECT: posterior

hypoperfusion

EEG: slow

Source: Alzheimer's Association. (2010). Changing the trajectory of alzheimer’s disease: A national imperative. Accessed on July 11, 2010, from http://www.alz.org/documents_custom/trajectory.pdf

Proportion of Americans Age 65 and Older with Alzheimer’s Disease by Stage of Disease, 2010–2050

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EARLY or MILD

STAGE

2 to 4 years or longer

MID- or MODERATE

STAGE

2 to 10 years

Compressed staging for adults

with Down syndrome

LATE or SEVERE

STAGE

1 to 3 years of longer

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Early Stage Middle Stage Late Stage

Confusion and

memory loss

Disorientation in

space

Problems with

routine tasks

Changes in

personality and

judgment

Difficulties with ADLs [“activities of daily

living”]

Anxiety, paranoia, agitation and other compromising behaviors

Sleep difficulties

Difficulty recognizing familiar people

Loss of speech

Loss of appetite, weight loss

Loss of bladder and bowel control

Loss of mobility

Total dependence on others

Generally most people with ID will have Alzheimer’s dementia … some will have other types

Knowledge of the nature and course of the type of dementia will help with program design and planning

Anticipating Onset and Change

Differential Effects of Types of Dementia

Factors and ModelsAssessing Quality

Environmental supports

Primary care practices

Program applications

• Compensate for cognitive losses and disorientation

• Support safety and mobility

• Offer protection against injury and disease

• Minimize behaviors that disrupt the equilibrium of the home or pose a danger to self or others

• Help maintain dignity and skills

Windows or doors that are hard to open

Poor lighting, particularly in kitchen or bathroom

Rugs, irregular floors and other tripping hazards

Tubs and showers that are hard to use

Stair widths or heights that are difficult to negotiate

Appliances and utensils that are challenging to use

Poor layout of rooms, such as bathroom distant from bedroom

Source: Brody, J. , Lifestyle tweaks to make aging easier. International Herald Tribune, October 27, 2010, p. 14.

Cognitive symptoms Impairment in memory, language, attention,

visuospatial and executive functions

Behavioral symptoms Depression, agitation, delusions, hallucinations, sleep

disorders

Impairment in activities of daily living Instrumental, social and basic functions

Source: Emre, M. (2005). Managing treatment expectations. Presentation at 21st Conf of Alzheimer’s Disease International, Istanbul, Turkey

Cognitive symptoms

Impairment in memory, language,

attention, visuospatial and

executive functions

Behavioral symptoms

Depression, agitation, delusions,

hallucinations, sleep disorders

Impairment in activities of daily

living

Instrumental, social and basic

functions

Source: Emre, M. (2005). Managing treatment expectations. Presentation at 21st Conf of Alzheimer’s Disease International, Istanbul, Turkey

Use of clinicians experienced with dementia

Continue assessment and behavioral observations

In-service training and consultation with staff/carers

Personal supports to adults affected

Specialty teams to help with program planning

ΨRN

SW

Rethinking how daily life and

regular activities are approached

Lessening stimulation,

while promoting

involvement and challenge

Modifications in communication

methods

Supporting residual skills

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Differential rates of decline

Different effects by type of dementia(Alzheimer’s vs. vascular vs. other)

Stage-specific needs (mid- vs. late)

If Down syndrome, differences in rate of

decline, onset, duration

If at home, supports provided to carers education on dementia, respite, adaptations to home

Being affected by dementia generally will mean that independent living (without any co-sharing) will be impossible

Small group home care is becoming the most viable alternative to institutional care

Mid 1

Early Mid 1 Mid 2 Late

Aging-

in-place• single

care home

and stable

stay

EarlyMid 1

Mid 2

Late

In-place-

progression• multiple

care homes &

movement

with progression

Mid = mid-level

Linear adaptations and care

Sequential adaptations and care

Option A Option B

Mid 2

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Small group homes seem like practical solutions to the desire of health officials and families for affordable neighborhood care

Staff-to-resident ratios need to be stage dependent e.g., more staff assigned with mid-stage ambulatory residents

Staff expertise needs to be associated with stage of care

With ambulatory residents – staff capable of engaging residual memories and capabilities, providing socialization and diversionary activities

With non-ambulatory, late-stage residents – staff capable of providing basic nursing care and careful attention to personal needs

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Dementia onset is variable but comes predictably earlier among adults with DS

Individual duration is an unknown and can only be predicted in aggressive forms

Oversight will be needed beginning with the latter early stage

Personal care and behavioral oversight are the two main staff time drains

Eventual outcome will be nursing care

Do you tell an adult showing signs of dementia what ishappening?

Under what circumstances? What supports do you offer? When do you bring it up? What about advance directives, wills,

and beneficiary planning?

Pamphlet was created by Scottish Down’s Syndrome Association to use with adults with Down syndrome and intellectual disabilities

Consensus meeting held February 5-7, 2001 in Edinburgh, Scotland. Attendees drawn from research, practice, advocacy and family caring, developed a set of principles.

The principles support continued community care, a focus on the individual, designing dementia capable environments and services, and careful planning to include adults with ID and AD in generic AD services and resource allocations.

1

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The Edinburgh Principles mirror the guiding principles for community care of people with dementia in the general population; and

Reinforce the notion that people with ID affected by dementia experience the same type of challenges and can benefit from the same types of progressive services

ADI’s Global Perspective(April 2002: Vol. 12, no. 1; p.6)

Promote quality of life using a person-centeredapproach

The driving force should be individual strengths, capabilities, skills and wishes

Involve the individual, her/his family and other closest supports in all phases of assessment and services planning and provision

Plan and provide supports and services that help the person remain in his or her chosen home and community

www.uic.edu/orgs/rrtcamr/dementia

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1Adopt an operational philosophy that promotes quality of life

2 Affirm that individual strengths guide decision-making

3 Involve the individual and family in all planning & services

4 Ensure availability of appropriate DX and service resources

5 Plan & provide supports to optimize remaining in community

6 Ensure that people with ID have access to same dementia services provided to others in population

7 Ensure that community planning involves focus on adults with ID

Edinburgh Principles

Factor

Age The risk of developing Alzheimer's disease increases with age

Gender Alzheimer's disease affects women more frequently than men

Family history Fewer than 1% of Alzheimer's disease cases are inherited, mostly those with onset before age 65

Head injury Studies have shown a link between Alzheimer's disease and a significant head injury

Environmental toxins There is some suspicion that increased exposure to certain substances, such as aluminum, may make a person more susceptible to Alzheimer's disease

Low educational level Although the reason is not clearly understood, some studies have shown that low education levels can be related to an increased risk for Alzheimer's disease

Other factors Research also suggests that high cholesterol levels and high blood pressure -- factors linked to heart disease and stroke -- may also increase the risk for developing Alzheimer's

Source: http://www.webmd.com/alzheimers/guide/causes-risk-factors

Some 50% of institutionalized adults with AD lose their ability to eat 8 years after onset

In moderately demented adults, food is often neglected and caloric intake falls

In severely demented adults, hindrance or prevention of oral nutrition occurs by aversive feeding behaviors (AFBs), such as 'refusal to eat', 'resistive‘, and 'dysphagia'

Source: [Riviere, S. (2002), Int J Geriatr Psychiatry, 17(10):950-955]

Eating (feeding) difficulties are a function of the age of the carer, the severity of dementia, and the adult’s autonomy and psychological functioning (i.e., mood and behavior disorders)

Both the adult’s level of cognitive impairment and family stress often predict which adults with AD living at home will develop eating problems

Nutritional information and support to families are effective strategies to prevent Aversive Feeding Behaviors (AFBs)

Source: [Riviere, S. (2002), Int J Geriatr Psychiatry, 17(10):950-955]

Minimizing risk factors

Overweight and obesity is found in excess

among adults with intellectual disabilities3

- which can lead to a variety of ailments

and heighten risk for dementia…

- Type 2 diabetes, high blood pressure,

raised fat levels in blood, coronary heart

disease, heart failure, stroke2

- Childhood obesity is particularly

insidious as it is highly related to higher

rates of extreme obesity when those

children reach adulthood1

- In the general population, 34% of adult

Americans (about 73m adults) are obese

(e.g., ~30 pounds or more over a healthy

weight)1

- the role models for ideal weight may be

people who themselves have excess weightSource: 1Hellmich, N., U.S. “Obesity rate appears to be leveling off…”. USA Today, 14 January 2010, p.8D; 2Smith, R. “Half of adults ‘will be obese by 2050’”, The Daily Telegraph (London), 17 October 2007, p. 6; 3Rimmer,J.H., & Yamaki, K. (2006). Obesity and intellectual disability. Ment Retard Dev Disabil Res Rev. 2006;12(1):22-7.

Source: USA Today, 2 July 2009, p. 8B

Aging is a lifelong process

Exercise, nutrition, physical and mental health, and social conditions all affect how healthy you may be in older age

“Successful Aging” means being free of disease, being active and involved in older age, and contributing to society

Anticipating Onset and Change

Differential Effects of Types of Dementia

Factors and Models

Assessing Quality

International dementia care standards/guidelines call for Provision of care with ‘dignity’ Recommended schedule of assessments and diagnostics ‘Dementia capable’ design of environments Provision and allocation of sufficiently trained staff

Quality influences for small group care include Clinical features Compensating environments Quality of life Caregiving

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Clinical features

• those aspects of a disease or condition as they interact or affect the person

Compensating environments

• characteristics of the living space - such as the number of residents per living area, the layout of the circulation system, the characteristics of the public spaces, and the programmatic use of the common and dining rooms

Qualitative aspects

• how an adult experiences the environment or perceives comfort in a situation

Caregiving

• how care is provided (that is, the mechanisms of care organization)

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The quality of care literature with respect to small group living and dementia care is still in the formative stage

Findings… Size is a factor with respect to quality

Optimally, a modest and manageable group size permits a sense of being at home, opportunities for involvement in small group activities, and engagement with familiar staff

Nature of the physical environment is a factor Design features provide for privacy and safety, facilitate activities and movement within the dwelling, and

offer gathering places for interaction that together foster functional facility

Staff and their capabilities are a factor When trained in aspects of dementia and dementia care, staff can provide a personally rewarding and

nurturing atmosphere

Quality is also affected by the characteristics of the residents and the nature of the operating principles that govern the program’s functions

Overall, blending of these features may serve as a foundational framework for general defining quality of care and quality of life in dementia care settings

Dementia Progression

Continued Assessment

Resident Behavior

Group Home Design

Care Provision

Capabilities

Staff Capabilities

Administrative Investment

Group Home Quality FactorMeasurement

Structural features Functional features

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Quality factors with applications to dementia care

• aspects related to early and appropriate assessment

• environmental modifications to a living setting

• staff education and functionality for stage-adapted care

• flexible long-term services provision that recognizes progression of decline and loss of function

A quality care model

should encompass

these general areas:

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Identification of common

quality prescriptions by dementia

organizations

Identification of factors and

features contributing

to and affecting quality

Synthesis of various

contributions to common

quality indications

Agreement on key

features that need to be present to

define acceptable

quality

Development of

measurement tools for assessing

components of quality

care

Assessment of level of

quality consistent

with prescribed presence of

key indicators

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Development of a quality of care measurement model

Analysis

Synthesis

Identification of specifics

Construction of a QC scale

Quality Outcome (scores)

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Analysis

Synthesis

Identification of specifics

Construction of a QC scale

Quality Outcome (scores)

72

Analysis

Synthesis

Identification of specifics

Construction of a QC scale

Quality Outcome (scores)

73

Analysis

Synthesis

Identification of specifics

Construction of a QC scale

Quality Outcome (scores)

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An operational working model could be applicable internationally for adapting residential environmentsthat is made up of four critical components:

Early and appropriate periodic assessment

Environmental modifications to a living setting and dementia-friendly architecture

Staff education and functionality for stage-adapted care

Flexible long-term services provision that recognizes progression of decline and loss of function

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Usage… Consistency check with established standards

Defining components necessary for new program development

Development of compliance standards

Development and application of measurement instruments

Links to payment and reimbursement criteria

Links for remediation activities

Sources of research and evaluation data

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Matthew P. JanickiUniversity of Illinois at ChicagoChicago, Illinois USAmjanicki@uic.edu

For information on ID and dementia:

http://www.rrtcadd.org/TA/Dementia_Care/ Resources/Info.html

For access to our University website:

http://www.rrtcadd.org/index.html