The need to distinguish between Alzheimer’s and other dementias

This is a long article in a recent LA Times about whether it’s important to distinguish between Alzheimer’s and other forms of dementia. Understandably, the focus is still on Alzheimer’s Disease.

Here are some excerpts:

* “Alzheimer’s disease is the most feared and most common form of dementia, accounting for between 60% and 80% of all dementia cases diagnosed. But at least seven other forms of dementia, and dementia linked to the movement disorder Parkinson’s disease, can cause loss of memory, reasoning, judgment and the ability to speak, comprehend and care for oneself.”

* “Doctors and insurers, including the federal government, which administers Medicare, are asking some variants of the same questions: If an effective test, which costs between $3,000 and $5,000 a shot, can diagnose dementia early, and distinguish Alzheimer’s from other forms of dementia, should it be recommended to patients with cognitive concerns and routinely covered by their insurance? Would it make patients’ lives better, or lower the cost of their care?”

* “At the Alzheimer’s Assn. International Conference in London last week, researchers reported their preliminary findings from a trial that is testing the impact of diagnostic testing for Alzheimer’s disease on nearly 19,000 Medicare beneficiaries … with a diagnosis of either ‘mild cognitive impairment’ or atypical dementia. The study … set out to find out whether knowing — getting the costly test that would offer either confirmation or reprieve — would change the way that patients with cognitive troubles are treated, or the way that they plan their lives. The preliminary results suggested it did. After getting the results of a PET brain scan to detect and measure amyloid deposits, which are the key hallmark of Alzheimer’s disease, roughly two-thirds of the subjects saw their medication regimens changed or were counseled differently by their doctors about what to expect. That new information may have guided family caregivers in planning their own futures, or prodded patients to make financial decisions and power-of-attorney assignments sooner. Some who learned that they did not have Alzheimer’s discontinued medications that can have unpleasant side effects. Others learned they do have Alzheimer’s and decided to enroll in clinical trials that will test new drugs.”

* “A second study presented in London analyzed data from several studies, and found that in a large population of research participants with cognitive concerns, brain amyloid PET scans led to a change in diagnosis in approximately 20% of cases.”

* “To the estimated 16 million Americans living with some form of cognitive impairment, telling the difference could make a significant difference. Dementia forms with different origins progress differently (or sometimes not at all). They respond best to different medications, and will come to require different levels of care and treatment. Some (though not Alzheimer’s) can even be reversed with treatment. Being able to distinguish which form of dementia a patient has should help doctors and caregivers to make better choices.”

Here’s a link to the full article:

www.latimes.com/science/sciencenow/la-sci-sn-alzheimers-transcranial-magnetic-stimulation-20170726-story.html

Science Now
Is it Alzheimer’s or another dementia form? Why doctors need to distinguish and how they might do so
by Melissa Healy
LA Times
July 27, 2017

Robin

 

PSP and CBS excerpts from curriculum on dementia for healthcare professionals

Someone in our local support group recently sent me this link to US Dept. of Health and Human Services’s curriculum for physicians (especially primary care physicians) and healthcare professionals (social workers, psychologists, pharmacists, emergency department staffs, dentists, etc.) on dementia. Though the web address includes the term “Alzheimer’s,” frontotemporal dementia is also mentioned in this curriculum:

Training Curriculum: Alzheimer’s Disease and Related Dementias
Health Resources and Services Administration (part of Dept of HHS)
bhw.hrsa.gov/grants/geriatrics/alzheimers-curriculum

One of the types of frontotemporal dementia is the “motor type,” which include corticobasal syndrome and progressive supranuclear palsy.

Here are some excerpts on frontotemporal dementia.

Robin

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Overview of Mild Cognitive Impairment and Dementia for an Interprofessional Team (Module 1)

Frontotemporal Dementia Types
* There are at least 3 distinctive clinical syndromes, each with heterogeneous neuropathology.
– Progressive behavior/personality decline: behavioral variant FTD (bvFTD)
– Progressive language decline: Primary progressive aphasia (PPA)
– Progressive motor decline: corticobasal syndrome, amyotrophic lateral sclerosis, or [progressive] supranuclear palsy. FTD with progressive motor decline is rare. FTD with progressive motor decline can involve movement problems/slowed movement, muscle rigidity (Parkinsonian symptoms), body stiffness, and changes in behavior or language.
* Behavioral variant FTD (bvFTD) is the most common variant. It is characterized by marked personality changes and changes in social conduct.


Understanding Early-Stage Dementia for an Interprofessional Team (Module 5)

Early-Stage Frontotemporal Degeneration (FTD): Overview
* FTD is a heterogeneous group of diseases with overlapping clinical symptoms but different causative genes and differing underlying pathologies.
* FTD is caused by damage to frontal and/or temporal lobes. Impairments generally progress quickly but memory often remains intact.
* Persons with FTD demonstrate changes in behavior and personality, language problems, and motor problems.
( Memory impairment is minimal in early stages.


Palliative and End-of-Life Care for Persons Living with Dementia (Module 12)

When to Consider Hospice Care in Persons with End-Stage FTD
* Persons with end-stage FTD are generally younger and healthier than persons with other types of end-stage dementia.
* As with other dementias, FTD is often not recognized as a terminal diagnosis.
* End-stage FTD may “look different” than other advanced dementias.

 

“How to Choose a Dementia Care Facility. Avoid My Mistakes.”

This is a very helpful article about choosing a dementia care facility.  The author is North Carolina-based Donna Plunkett St. Clair, whose husband was diagnosed with dementia in 2010 at age 59 and then with Lewy Body Dementia (LBD) in 2015 at age 65.  Donna shares the mistakes she made in choosing the wrong care facility…twice.

Here are the ten lessons Donna learned:

1.  Start investigating potential facilities NOW.
2.  Learn how your loved one’s care will change as he/she declines.
3.  Learn how “problems behaviors” are defined and ask about examples of what might lead to a resident being forced to leave the facility.
4.  Know what you can afford.
5.  Assess if the facility is using innovative designs.
6.  Ask if the resident can safely go outside.
7.  Ask about safety.
8.  Inquire about staffing levels, activities, and supervision for holidays, evenings, and weekends.
9.  Ask if residents are encouraged to stay hydrated, and are offered second helpings and snacks.
10.  Check service levels and quality when the facility least expects you.

These lessons are detailed in the article here:

www.lewybodydementia.ca/dementia-care-facility-choice-avoid-mistakes/

How to Choose a Dementia Care Facility. Avoid My Mistakes.
By Donna Plunkett St. Clair
Posted to Lewy Body Dementia Canada
May 29, 2016

Robin

 

Lewy Body Dementia Info on Dementia Aide (website)

Dementia Aide, a relatively new website (dementiaaide.com), is focused on selling what it calls dementia-related products.  While a few things such as t-shirts are disorder-specific, most of the products are caregiving items.  They have pages on their website for Alzheimer’s Disease (AD), frontotemporal dementia, vascular dementia, and Lewy body dementia (LBD).

The LBD section, written in September 2016, won’t be added to our list of “Top Resources” but it’s worth checking out.  They seem to have pieced together information from lots of different resources (not always giving attribution every place they could.)  For example, the chart on the difference between LBD, Parkinson’s Disease (PD), and Alzheimer’s is straight from the Lewy Body Dementia Association but this is only pointed out in one place (not everywhere the chart is).

You might check out their infographic on what they say are the four stages of LBD (on the symptoms page).

The only obvious error I saw was that they don’t have an accurate description of “Lewy body dementia” within the Lewy body disease family.  They show Lewy body dementia is the same thing as Dementia with Lewy Body.  Actually, Lewy Body Dementia is an umbrella term that refers to both Dementia with Lewy Bodies and Parkinson’s Disease Dementia.

Here’s a link to the LBD section:

www.dementiaaide.com/pages/lewy-body-dementia

Robin

Six tips on coping with inappropriate dementia behavior; saying “I’m sorry”

The Capital Gazette newspaper has a column written by Mary Chaput of the Department of Aging and Disabilities of Annapolis, MD.  A recent column had a question about frontotemporal dementia.  Ms. Chaput’s answer applies to dealing with someone with any type of dementia.

In terms of dealing with inappropriate behavior, she offers six tips:

* Don’t take the behavior or comments personally.
* Be empathetic.
* Don’t argue.
* Look for the situation(s) and environmental factors that trigger the behaviors.
* Talk with your family member’s physician about the behavior.
* Keep in mind that this, too, shall pass.

Another question was about saying “I’m sorry” to placate someone with dementia.

Here’s a link to the full column:

www.capitalgazette.com/lifestyle/ph-ac-cc-caregivers-0402-20170401-story.html

Caregivers Corner: Be patient and empathetic when dealing with frontotemporal dementia
by Mary Chaput, Correspondent
Capital Gazette
April 1, 2017

Robin

 

Notes from webinar on three non-AD dementias, including Lewy Body Dementia

There was a 90-minute webinar on April 6th, hosted by Resources for Integrated Care, was packed with lots of info on dementia with Lewy bodies (DLB) and frontotemporal dementia (FTD) with less info on vascular dementia (VAD).  (Note that throughout the webinar, with few exceptions, the term “Lewy body dementia” was used.)  The webinar, for both healthcare professionals and family caregivers, was fast-paced.  Presentations were for the first hour, and then there was a 30-minute Q&A.

You can find a link to the webinar recording here:

www.resourcesforintegratedcare.com/GeriatricCompetentCare/2017_GCC_Webinar_Series/Beyond_Alzheimers

You can find a link to the webinar slides here:

www.resourcesforintegratedcare.com/sites/default/files/Beyond_Alzheimers_Disease_0.pdf

VAD, DLB, and FTD are the most common dementia types after Alzheimer’s Disease (AD).  Here are short definitions:

* Vascular dementia – cognitive deficits most often associated with vascular damage in the brain, either micro or macro in nature.

* Dementia with Lewy Bodies – a dementia that also includes one or more of these core findings: recurrent and detailed visual hallucinations, parkinsonian signs, and fluctuating cognition.

* Frontotemporal dementia – a disease often seen in individuals with onset of cognitive symptoms at a younger age; these individuals present most often with executive and language dysfunction and significant behavioral changes.

The management strategies that are effectively in AD are less effective in these other three dementia types.

Brain Support Network volunteer-extraordinaire Denise Dagan listened to the webinar a couple of times and took extensive notes on the LBD content and some notes on the other two dementias.  She has provided the time stamp at various points so that you can fast forward through the recording, if you’d like.

Robin

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Denise’s Notes from

Beyond Alzheimer’s Disease, Other Causes of Prerogative Dementia in the Older Adult
Webinar hosted by Resources for Integrated Care
April 6, 2017

Presenters:
* Melinda S. Lantz, MD, Chief of Geriatric Psychiatry, Mount Sinai Beth Israel Medical Center, New York, NY
* Geri Hall, PhD, ARNP, CNS, FAAN, Banner Health, Phoenix, AZ
* Rebekkah Wilson, MSW, Dementia Care Consultant and Trainer
* Sharon Hall, Family Caregiver (to someone with FTD)

After many housekeeping comments and credits, introduction of the webinar presenters begins at time stamp 3:15.

DR. LANTZ SPEAKS….

DEMENTIA

After some audience polling, Dr. Lantz begins at time stamp 9:15 with how dementia is identified and diagnosed by evaluation as there are no clinical tests yet available to diagnose any type of dementia.

Dementia is a major neurocognitive disorder.  According to the American Psychiatric Associations Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (APA DSM5), to be diagnosed with dementia a person must exhibit significant decline from a previous level in the five domains of cognitive functioning, which include:
* Complex attention
* Executive function
* Learning and memory, language
* Perceptual-motor
* Social cognition

Diagnosis is based on collateral information including self-reporting, standardized neuropsychological testing or quantified clinical assessment.

Cognitive deficits interfere with everyday activities, social or occupational functioning.

MCI

Mild Neurocognitive Disorder [Mild Cognitive Impairment (MCI)], according to the APA DSM5, does not meed criteria for dementia.  MCI is described as:
* Subjective and objective decline in cognitive domain: primarily memory, language or motor
* No significant impairment in:
— Other cognitive domains
— Activities of Daily living
— Social or occupational functioning
* 10-15% of patients with MCI progress to develop dementia each year
* “Precursor of dementia” versus spectrum of normal aging
* Patients with MCI should be identified and monitored for cognitive and functional decline due to their increased risk for dementia
* There are no currently FDA approved medications for MCI

VASCULAR DEMENTIA

Vascular Dementia = dementia due to cerebrovascular disease.
* Cognitive loss due to cerebrovascular disease
* 2nd most common cause in late-life after Alzheimer’s.
* Risk factors:
— Hypertension (high blood pressure)
— Diabetes
— Hyperlipidemia
— Smoking
— TIAs (transient ischemic attack) or mini-stroke is a neurological event with the signs and symptoms of a stroke, but which go away within a short period of time.
— Stroke.
* Decline may be abrupt due to a stroke or series of TIAs
* Cognitive loss may be focal, with more awareness of symptoms
* Disturbance of emotions and mood is common
* Caregiving needs are due to medical and physical conditions (paralysis of one side of the body due to stroke, and multiple medical problems and medications).
* Decline may be step-wise with plateaus in symptoms.  They may recover after 1st stroke, but decline years later due to more strokes.
* Mixed variants of dementia (Alzheimer’s with vascular dementia) are common.
* More men than women because men have more vascular disease than women.

She displayed a scan of vascular dementia at time stamp 13:45

She inserted a slide of two other types of vascular dementia at time stamp 14:00, but didn’t discuss them.

LEWY BODY DEMENTIA

Lewy Body Dementia
* Memory loss and other cognitive deficits that often have a fluctuating and variable course, and relatively rapid onset
* Motor rigidity, parkinsonism features
* Prominent hallucinations, usually visual
* Unsteady gait, syncope (fainting), unexplained falls
* More rapidly deteriorating course
* 3rd most common dementia after Alzheimer’s and Vascular dementias
* more common in men than women

She displayed a microscope image of a lewy body at time stamp 15:30

Sensitivity to all psychiatric meds, but definitely psychosis requiring meds, which makes it complex to treat.

FRONTOTEMPORAL DEMENTIA

Frontotemporal dementia at time stamp 16:12
* Significantly earlier onset between 40-60 yrs old because of slow and subtle onset
* Atrophy prominent in the frontal and temporal lobes of the brain (associated with personality, mood, behavior, impulse control)
* Slow onset with early changes in personality, impulse control and language
* Memory, arithmetic, copying figures often preserved until later in the course so difficult to diagnose early on
* Behavior often disinhibited, repetitive, socially inappropriate
* Prominent personality change very early
* Early onset with very slow, progressive decline
* Memory impairment later in the course
* Behavior changes: Disinhibition, impulsivity, apathy, depression, verbal outbursts
* Lack of recognition, agnosia (Inability to interpret sensations and hence to recognize things, people and/or sounds.  With agnosia they are particularly unable to recognize their own changes in behavior and capabilities, hampering diagnosis and caregiving.)
* Treatment is very symptom driven as there are no agents available for prevention or slowing progression

Two subtypes: Pick’s disease and frontotemporal dementia with parkinsonism at time stamp 17:52

She displayed a Pick body microscope image at time stamp 18:05

TREATMENT

Treatment of Cognitive Symptoms of Vascular and Lewy body Dementia:
* Cholinesterase Inhibitors slows the rate of decline:  Donepezil, Galantamine, Rivastigmine, for mild to moderate Alzheimer’s (AD), Vascular dementia (VAD), mixed the benefits relatively similar: +3 points ADAS-Cog, 4 to 6 Month delay in progression
* Individual response variable and difficult to measure:  20% of patients show “Greater than average” response, 20% “some response”, but 30-50% show no response
* Greatest effect appears to be delay in need for nursing home placement of 6 to 8 months
* NO ROLE for use in Frontotemporal Dementia (FTD), may worsen behavioral symptoms

Treatment of Cognitive Symptoms:
* Rivastigmine may be more helpful in LBD than the other available cholinesterase inhibitors
* Memantine is approved by the FDA for Alzheimer’s, but not enough evidence to show benefit for other types of dementia
* Cholinesterase inhibitors have no benefit in FTD and may worsen mood and behavior
* Cholinesterase inhibitors may cause nausea, vomiting, weight loss (due to GI upset) and bradycardia (slow heart rate)

Pharmacologic Treatment for targeted severe symptoms:
* The idea is to minimize symptoms without side effects, especially from poly-pharmacy.
Use of these is justified when patients are disturbed by hallucinations, especially if they try to act on them in dangerous ways.
* Not a substitute for good care and behavioral management.
* Psychosis (hallucinations, delusion) > Rx options: Antipsychotic Agents (risperidone, olanzapine, quetiapine, aripiprazole)
* Depression, Anxiety, & Irritability > Rx options: SSRI (sertraline, citalopram) or bupropion, trazodone, mirtazapine)
— These are particularly common early in these dementias, when mood treatment can be particularly helpful.

Pharmacologic Treatment for physical aggression:
* These are all off-label drug uses, but can be helpful.
* Severe Physical aggression (also helpful for severe impulsivitity and mood lability > Rx options: Mood Stabilizer (valproate, lithium, carbamazepine, gabapentin)
* Moderate Physical Aggression > Rx options: Mood Stabilizer

She put up three slides with Dosing Guidelines and Side-Effects charts for antidepressants and mood stabilizers at time stamp 23:37

DR. HALL SPEAKS….

Symptom presentation in dementia depends on these factors and directly affect care challenges:
* Location of the degeneration
* Function of the degenerated area of the brain
* Pathologic changes at the cellular level (e.g. presence of Lewy bodies)
* Comorbid conditions
* Environmental factors producing excess disability
* Premorbid personality

At time stamp 25:22 she put up PET scan imagines distinguishing dementias from each other (kind of amazing).

The location of damage is different, therefore so are care needs:
* Due to time constraints we will examine two more common non-Alzheimer’s dementias:
— Lewy Body Dementia (LBD)
— Frontotemporal Lobar Degeneration (FTD or FTLD)

LEWY BODY DEMENTIA

Lewy Body Dementia:  Three common presentations
* Regardless of the initial symptom, over time all three presentations of LBD will develop very similar cognitive, physical, sleep and behavioral features.
* Some individuals will start out with a movement disorder leading to the diagnosis of Parkinson’s disease and later develop dementia.  This is diagnosed at Parkinson’s disease dementia.
* Another group of individuals will start out with a cognitive/memory disorder that may be mistaken for Alzheimer’s disease, but over time two or more distinctive features of fluctuating cognition and psychosis (hallucinations and/or delusions) become apparent leading to the diagnosis of ‘dementia with Lewy Bodies’ (DLB).
* Lastly, a small group will first present with neuropsychiatric symptoms, which can include hallucinations (primarily visual, but also olfactory or auditory), behavioral problems, and difficulty with complex mental activities, also leading to an initial diagnosis of DLB.

Common Symptoms of Lewy Body Dementia:
* Sleep disorders (can be present up to two years prior to LBD diagnosis)
— Acting out dreams while asleep
— Excessive daytime sleepiness
— Restless leg movement
* Impaired thinking
— Executive function (planning, processing information.)
— Memory fluctuates
— Ability to understand visual information fluctuates
* Problems with movement
— Tremors, stiffness, slowness and difficulty walking
— Anti-Parkinson medications often don’t work well and may cause initial hallucinations
* Altered sensory perception (particularly visual-spacial perception)
* Hallucinations
— Often of animals or children
* Behavioral and Mood Symptoms
— Depression, apathy, anxiety, agitation, delusions or paranoia
* Changes in Autonomic Body Functions
— Blood pressure control, temperature regulation, postural control (tend to fall a lot), bladder and bowel function
* Exquisite Sensitivity to medications, particularly those that affect the central nervous system.
— Don’t use old generation antipsychotics.  With new antipsychotics, start with a low dose and ramp up to an effective dose.
— Don’t use general anesthesia.

Care of Lewy Body Dementia:
* Similar to Alzheimer’s – decrease stimuli, increase rest, promote exercise (Big & Loud Programs) and balance
* Safety due to REM sleep disorder, fall precautions due to autonomic dysfunction, swallowing issues
* Supportive (self-care) activities of daily living (ADLs)
* Control of misleading environmental stimuli (especially TV) and medications that trigger hallucinations and delusions
* Prepare family for response to potential aggression
* LBD Association and support groups

Use of Therapies:
* Physical therapy – Design a program and teach to patient/family focusing on postural stability and core strength
* Exercise Programs – Big and LOUD Programs!!!
* Recreational Therapy
* Occupational therapy – ADLs
* Speech pathology – swallowing, spoken volume
* Pharmacist consultation – OTCs can be problematic and interact, so ask before taking anything.

Lew Body Dementia Support and Resources slide includes a list of resources, including encouragement to attend local support group meetings.

Robin’s note:  See Brain Support Network’s list of top LBD resources here, which includes the two resources Dr. Hall mentioned:

www.brainsupportnetwork.org/education/lewy-body-dementia/

Denise’s note:  If you are in the San Francisco Bay Area, please attend Brain Support Network’s LBD caregiver-only support group meetings!  We just met last Sunday.  Our next meeting is in June.  Email Robin Riddle to get on the meeting reminder email list.

FRONTOTEMPORAL DEMENTIA

She spoke about Frontotemporal Dementia (FTD) from time stamp 31:29 until her summary at 46:43.  FTD is often misdiagnosed for 10-14 years and is the most difficult to care for, because of youth and psychiatric issues.

SUMMARY

Summary:
* As diagnostic specificity improves, non-Alzheimer’s dementias will be diagnosed more frequently.
* A “one-side-fits-all” care program will not meet the needs of people with non-Alzheimer dementias.  They differ in terms of symptom presentation, behavioral responses, and ability to tolerate medications.  [When searching for a day program or caregivers for your family member with a non-Alzheimer’s dementia diagnosis, interviewing for their understanding of they type of dementia your family has, and their preparedness in caring for that type of dementia, is critical in finding the right program or caregiver.]
* Families and care providers are desperate for answers, ongoing support, and to seek out others suffering from similar conditions.
* Interdisciplinary care and research is essential for humane approaches to these vexing conditions.

MS. WILSON SPEAKS…

Rebekkah Wilson, MSW spoke next at time stamp 46:29

Beyond Alzheimer’s Disease, Impact on the Individual/Family System:
* Challenges with diagnosis
* Symptoms generally less recognized/understood
* Caregiver burden
* Younger onset considerations
* Community resources and considerations
— Legal
— Financial
— Emotional support
— Care options

Psychosocial Impact – Diagnosis
* Misdiagnoses common
— in FTD as late onset mental health issues (bi-polar), or marital problems
— in LBD when initial presentation is non-motor symptoms
* Rearview mirror clearer; but by then there are consequences of actions taken prior to diagnosis
— unnecessary treatments, or surgery
— impulse control issues in FTD can result in overspending of college & retirement funds, or loss of jobs & stability
* Sense of relief to get diagnosis
— Negative experiences prior to diagnosis
— Empowerment through learning about diagnosis (now I know what I’m dealing with)
* Present and future support – Programs are set up on an Alzheimers disease model.  Some areas of the country are more progressive and have more support groups and day programs/caregivers for non-Alzheimer’s dementia.

Psychosocial Impact – Symptoms
* Less recognized because Alzheimer’s disease is everyone’s reference point
– Memory may not be impacted, so dementia not recognized and symptoms not addressed
* Safety considerations due to brain changes (driving, wandering, judgement)
* Fluctuations in mental status misinterpreted (esp. in young patients misdiagnosed as bi-polar, marital discord, OCD, etc.)
* hallucinations/REM
* Impact of FTD symptoms
– Legal
– Financial
– Employment
– Social

Psychosocial Impact – Caregiver Burden
* Nature of Symptoms
— FTD – especially behavior and language changes
— LBD – especially cognitive fluctuations (can be moment to moment, rather than good morning & bad afternoon)
* High caregiver stress
* Isolation due to being a ‘rare’ diagnosis (need to find a support group with the same non-Alzheimer’s diagnosis)
* Age of onset impacts normalization (especially FTD & young onset Alzheimer’s)
— Expect memory and cognitive changes in older adults.
Younger people with dementia are misdiagnosed with mental health issues or arrested for unruly public behavior.
— Parents providing care for person with dementia (PWD)
— Care for PWD while caring for kids in household (sandwich generation)
* Ambiguous loss / anticipatory grief

Psychosocial Impact – Young Onset
* Developmental stage of the family
— Young kids at home, frequently in young onset families.
— Aging parents caring for their children with young onset dementias.
* Concerns about genetics
* Career and employment considerations
* Financial implications – primary caregiver may now have to work (maybe more than one job, depending on their skill set.)
* Challenges finding services because they are not trained to care for non-Alzheimer’s dementia

Legal and Financial Considerations
* Legal planning documents (maybe guardianship or conservatorship)
— Power of Attorney for Healthcare
— Power of Attorney for Finances
— Living Will
* Employment Laws
* Early Onset diagnosis impacts income and insurance (pre-Medicare age)
* Medicare
* Social Security Disability
* Social Security Compassionate Allowance to expedite benefits
* Additional option for financing care

The Compassionate Allowances (CAL) initiative is a way to expedite the processing of SSDI and SSI disability claims for applicants whose medical conditions are so severe that their conditions obviously meet Social Security’s definition of disability.  It is not a separate program from SSA’s two disability programs, SSDI and SSI.

There is no special application or form that is unique to the CAL initiative.  Individuals with a CAL condition apply for benefits using the standard SSA process for filing claims for SSDI, SSI or both SSDI and SSI benefits.  SSA will expedite the applications of those with a CAL condition.  Applications for disability may be filed online, in the local field office, or by calling 1-800-772-1213.  To learn more about the CAL initiative, see:

www.ssa.gov/compassionateallowances/

Community Resources and Services
* Support Groups for both caregiver AND the person with a diagnosis
* Care models in programs, including day facilities and nursing facilities are based on Alzheimers, but expanding.
* Families are using online information & medical community to educate care models.
* Limited experience and understanding of non-Alzheimer’s dementia

Care Planning
* Establishing the care team (Neurologist, PT, OT, Pharmacist, speech therapist, and the primary caregiver.)
* Determining goals for care team, based on location of care (home or facility) (comfort & safety).  Focusing on the primary goal(s) helps to make decisions.  For example, when choosing a care facility for someone with LBD, focusing on fall prevention as the primary goal may eliminate several options.  Narrowing the number of choices makes the decision easier.
* Support for the person with the diagnosis
* Knowledge and support of the care partners
– Family/friends
– Professionals

Challenges with Home & Community Based Care in Non-Alzheimer’s dementias
* Facility care for FTD
* Cognitive fluctuations as a challenge for care in LBD
* Hospice / palliative care & denials of admission because guidelines are based on Alzheimer’s

Best Practices for Home & Community Based Care
* Recognize care for non-Alzheimer’s dementias is different than Alzheimer’s
* Structure very important
* “Failure free,” low demand engagement
* Group versus individual activities (For Alzheimer’s people, group activities work well, while for young onset individual and non-Alzheimer’s dementias, independent activities work better)
* Recognize preferences may differ from other residents if younger
* Risky or impulsive behavior may increase safety concerns.

MS. HALL SPEAKS…

Sharon Hall spoke last about being a family caregiver of two types of dementia simultaneously at time stamp 1:07:47

Cares for both her 90+-year-old mother with vascular dementia and her husband with bvFTD – yikes!

Feels most difficult aspects is changing role from daughter/wife to caregiver through ambiguous loss process.

Challenging to early onset patient in finding long-term care, especially day programs.  Her husband is physically robust so people don’t expect his behavior to be dementia, but think he’s joking or has mental illness.  Day programs don’t have activities he’s interested in.

Physical contact is ok for people with Alzheimer’s, but if you touch someone with FTD they WILL touch you back.  They also say inappropriate things.  She tried handing out cards excusing his behavior, but she finds talking with people about it, even including her husband in that conversation, educates people better.  Sometimes, they don’t believe her.

Meds are not the only way to manage behavior, especially in FTD, and can make things worse.  It is better to be their advocate and understand how to manage behavior without it.  You can’t expect someone with dementia to follow standard rules of behavior.  You have to go with whatever they’re doing, as long as it’s not dangerous or too disruptive.

Being their advocate, especially in understanding where difficult behaviors come from, is key.  Employing their assistance in as many activities of daily living as they can manage, throughout the day but including rest periods, is the best way to minimize behavior problems.  It occupies their attention, keeps them out of trouble and from being bored, and makes them feel they still are useful members of the family and society.

There’s a crazy financial burden with young onset dementia families, because they had to stop working early so they qualify for less SSI than people who retire later.  Especially people with FTD, because of spending compulsions, can spend through college and retirement savings so must be taken off all financial accounts as soon as diagnosis is made.

FTD has unusual eating habits, crave carbs, etc.  Hygiene can be an issue, but nobody ever dies from not taking a shower.  Forcing participation in conventional hygiene and other tasks on someone with non-Alzheimer’s dementias can bring on unwanted behaviors.  Understanding what you’re dealing with and working with them is the key.  If it’s not dangerous, let them do what they want to do.

QUESTION AND ANSWER

Q&A at time stamp 1:16

Sharon, what techniques work best to manage behaviors.
I apologize to him if she sees she’s done something to trigger a behavior.  Being on his side diminishes the behavior.

Rebekkah, can a healthcare surrogate with no family member present, place a dementia patient in assisted living or LTC if its in their best interest?
A medical decision maker can

Dr. Hall, do you have suggestions for alternatives when access to site services is a challenge?
Psychiatric services, but start with medical and neurological workup.

Dr. Lantz, FTD has been connected with concussion or head trauma?
No (listen for longer answer)

Dr. Hall, are sensory used to treat patients with challenging behaviors arising from dementia?
Sensory rooms are used to treat dementia.  (listen for longer answer)

Dr. Lantz, In early FTD what techniques do we use for early treatment and diagnosis?
monitor and evaluate symptoms and mitigate problems.  PET scan.  Team diagnosis approach, especially behavioral neurology training will better recognize FTD.

What do any of you know about a web based tool called WE Care Advisor geared to help family caregivers?
Nobody has heard of it.

You all stress the importance of interdisciplinary team supporting dementia and caregivers.  Non-medical support?
Area Agency on Aging, the Alzheimer’s Association, access to social services and medical help, as well.
FTD & LBD have national resources but those 2 have local resources.
Join a support group to share resources, moderators educate on resources in these groups.

Dementia friendly communities?  How do I find one?
They look at developing activities and resources places for people with dementia, including painting, coffee shops with dementia-firendly hours.  Locations are based on minimal stimulations, etc.  People with FTD have a harder time participating because their dementia peers with other diagnosis can’t communicate with them well.  Your local newspaper may have done an article on the opening of one in your area to help you find one near you.

“Dementia caregivers: Learning to live in your loved one’s reality” (Philadelphia Inquirer)

This Philadelphia Inquirer article is a report on an Alzheimer’s caregiver conference in Valley Forge.  The keynote speaker was Tam Cummings, a gerontologist from Texas.  Ms. Cummings and other speakers made several points:

* “Cummings urged family members to ask their doctors more questions — as many questions as they might ask if the diagnosis were cancer.  Knowing more, she said, may help them understand and cope with the memory lapses, confusion, delusions, falls, depression, and stubbornness that often accompany dementia.”

* “A recurring theme: People with dementia have brain damage that limits what they can do and how they can think.  It’s those around them who have to change. … If people with dementia are being obstinate or aggressive, it’s up to caregivers to try to figure out whether there is an explanation that their loved one can no longer communicate: Are they in pain? Are they afraid? Were the instructions too complicated? Have perceptual changes made the environment look dangerous to them?”

* “Cummings told the crowd that ‘your loved one’s reality is your reality.'”

This short article is worth reading.  Here’s a link to it:

www.philly.com/philly/health/Dementia-caregivers-Learning-to-live-in-your-loved-ones-reality-alzheimers-association.html

Dementia caregivers: Learning to live in your loved one’s reality
Updated: April 26, 2017 — 3:01 am EDT
by Stacey Burling, Staff Writer
Philadelphia Inquirer

Robin

“Next-Generation Tau PET Tracers Strut Their Stuff” – differentiating PSP from AD

This is a report by Alzforum from the Alzheimer’s/Parkinson’s 2017 conference in Vienna at the end of March.  The focus of the report is on next-generation tau PET tracers.  Tau is the protein involved in Alzheimer’s Disease (AD), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), Pick’s disease, and chronic traumatic encephalopathy.

There are five new PET tracers under development.  The report says:

“[The] new tracers…appear at first glance to be able to overcome the limitations of the earlier compounds. In general, the newcomers boast higher brain uptake and more specific binding, yielding cleaner-looking scans with sharper distinction between positive and negative findings. While the older tracers work only in AD, some of the new ones appear to light up other tauopathies, as well. Researchers at Piramal Imaging wowed the crowd with scans showing a distinct, specific pattern of binding of their tracer in progressive supranuclear palsy (PSP) compared to AD.”  (Check out the online version of the article for AD vs. PSP images.)

The first-generation tau PET tracers described in the report are:  Lilly/Avid’s AV-1451 (flortaucipir) and THK5351, discovered at Tohoku University in Sendai, Japan, and licensed by GE Healthcare for commercial distribution.  The report indicates that both tracers have lots of problems.

As a result, many researchers are “now eyeing Merck’s and Piramal’s [tracers]. … Merck reported on their tau PET tracer, MK-6240, at the Human Amyloid Imaging (HAI) meeting held January.”  Other companies working on tau ligands include Genentech, Roche, and Janssen.

“Piramal started a Phase 1 trial on four people with AD, three with PSP, and two healthy controls. … Notably, AD and PSP scans revealed distinct patterns. In PSP, only a few discrete regions, mainly the pallidum and substantia nigra, lit up. In contrast, AD patients took up tracer in broader areas known to accumulate tau tangles, such as the lateral temporal lobe, hippocampus, entorhinal cortex, and precuneus.  Curiously, one of the AD patients had a negative tau scan. Stephens noted this patient had mild AD, with an MMSE of 26, and may not have accumulated much pathological tau yet. Incidentally, other PET experts, too, noted that as more research groups image both amyloid and tau pathology in the same cognitively impaired people, they are finding a few whose scans are amyloid-positive but tau-negative.”

Here’s a link to the full report:

www.alzforum.org/news/conference-coverage/next-generation-tau-pet-tracers-strut-their-stuff

Next-Generation Tau PET Tracers Strut Their Stuff
Series – AD/PD 2107 Draws Record Number of Scientists To Vienna
14 Apr 2017
by Alzforum

Robin

 

FTD Disorders Registry is now live

The FTD Disorders Registry is an online database to collect information from those affected by all types of Frontotemporal Degeneration: behavioral variant FTD (bvFTD), any one of the primary progressive aphasias (PPA), progressive supranuclear palsy (PSP), corticobasal degeneration (CBD), or FTD with motor neuron disease (also called FTD-ALS). Persons diagnosed, caregivers (current/former), family, and friends can join and tell your story.

Brain Support Network encourages all those affected by PSP and CBD to join the registry.

www.ftdregistry.org

Even if your family member has passed away, you can still join the registry and tell your story.

Robin

Excerpts on PSP and CBD in “The Dementias” (NIH online-only booklet)

This email may be of interest to those dealing with the dementia forms of PSP and CBD.  (Not everyone with these diseases has dementia.  To read about the types of PSP and CBD, look under the “PSP Education” and “CBD Education” pages of the Brain Support Network website.)

The National Institutes of Health (nih.gov) has several publications on neurological diseases.  I recently came across their booklet on “The Dementias,” which includes a section on tauopathies as types of dementia.  Tauopathies are caused by the abnormal accumulation of the protein tau.  Both corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) are covered.  Other tauopathies addressed include frontotemporal disorders (such as Pick’s) and argyrophilic grain disease (AGD).  (In brain donations we’ve helped with, AGD co-occurs in about 20% of all PSP cases.)

“The Dementias” booklet links to the NIH pages on CBD and PSP.  I think the PSP detail page is quite good (and it’s listed as one of our “Top Resources for PSP”).  I don’t think the CBD detail page is nearly as good.

Here are excerpts from the tauopathies section.  Look at the booklet online for other chapters — risk factors, diagnosis and treatment, etc. — and other types of dementia.

Robin

————————–

nia.nih.gov/alzheimers/publication/dementias/types-dementia

Excerpts from

The Dementias
NIH Online Booklet
Published September 2013 (Last Updated July 2016)

Types of Dementia

Various disorders and factors contribute to the development of dementia. Neurodegenerative disorders such as AD, frontotemporal disorders, and Lewy body dementia result in a progressive and irreversible loss of neurons and brain functions. Currently, there are no cures for these progressive neurodegenerative disorders.

Some types of dementia disorders are described below.

Tauopathies

In some dementias, a protein called tau clumps together inside nerve cells in the brain, causing the cells to stop functioning properly and die. Disorders that are associated with an accumulation of tau are called tauopathies.

In AD, the tau protein becomes twisted and aggregates to form bundles, called neurofibrillary tangles, inside the neurons. Abnormal clumps (plaques) of another protein, called amyloid, are prominent in spaces between brain cells and are a hallmark of the disease. Both plaques and tangles are thought to contribute to reduced function and nerve-cell death in AD, but scientists do not fully understand this relationship. It is not clear, for example, if the plaques and tangles cause the disorder, or if their presence flags some other process that leads to neuronal death in AD.

Other types of tauopathies include the following disorders:

Corticobasal degeneration (CBD) is a progressive neurological disorder characterized by nerve-cell loss and atrophy (shrinkage) of specific areas of the brain, including the cerebral cortex and the basal ganglia. The disorder tends to progress gradually, with the onset of early symptoms around age 60. At first, one side of the body is affected more than the other side, but as the disease progresses both sides become impaired. An individual may have difficulty using one hand, or one’s hand may develop an abnormal position.

Other signs and symptoms may include memory loss; trouble making familiar, focused movements (apraxia) such as brushing one’s teeth; involuntary muscular jerks (myoclonus) and involuntary muscle contractions (dystonia); alien limb, in which the person feels as though a limb is being controlled by a force other than oneself; muscle rigidity (resistance to imposed movement); postural instability; and difficulty swallowing (dysphagia). People with CBD also may have visual-spatial problems that make it difficult to interpret visual information, such as the distance between objects.

There is no cure for CBD. Supportive therapies are available to reduce the burden of certain symptoms. For example, botulinum toxin can help control muscle contractions. Speech therapy and physical therapy may help one learn how to cope with daily activities.

Frontotemporal disorders (FTD) are caused by a family of brain diseases that primarily affect the frontal and temporal lobes of the brain; they account for up to 10 percent of all dementia cases. Some, but not all, forms of FTD are considered tauopathies. In some cases, FTD is associated with mutations in the gene for tau (MAPT), and tau aggregates are present. However, other forms of FTD are associated with aggregates of the protein TDP-43, a mutated protein found among people with a type of ALS that is inherited. Mutations in a protein called progranulin may also play a role in some TDP43-opathies.

In FTD, changes to nerve cells in the brain’s frontal lobes affect the ability to reason and make decisions, prioritize and multitask, act appropriately, and control movement. Some people decline rapidly over 2 to 3 years, while others show only minimal changes for many years. People can live with frontotemporal disorders for 2 to 10 years, sometimes longer, but it is difficult to predict the time course for an affected individual. In some cases, FTD is associated with progressive neuromuscular weakness otherwise known as amyotrophic lateral sclerosis (ALS, or Lou Gehrig’s disease). The signs and symptoms may vary greatly among individuals as different parts of the brain are affected. No treatment that can cure or reverse FTD is currently available.

Clinically, FTD is classified into two main types of syndromes:

* Behavioral variant frontotemporal dementia causes a person to undergo behavior and personality changes. People with this disorder may do impulsive things that are out of character, such as steal or be rude to others. They may engage in repetitive behavior (such as singing, clapping, or echoing another person’s speech). They may overeat compulsively; lose inhibitions, causing them to say or do inappropriate things (sometimes sexual in nature); or become apathetic and experience excessive sleepiness. While they may be cognitively impaired, their memory may stay relatively intact.

* Primary progressive aphasia (PPA) causes a person to have trouble with expressive and receptive speaking—finding and/or expressing thoughts and/or words. Sometimes a person with PPA cannot name common objects. Problems with memory, reasoning, and judgment are not apparent at first but can develop and progress over time. PPA is a language disorder not to be confused with the aphasia that can result from a stroke. Many people with PPA, though not all, develop symptoms of dementia. In one form of PPA, called semantic PPA or semantic dementia, a person slowly loses the ability to understand single words and sometimes to recognize the faces of familiar people and common objects.

Other types of FTDs include:

* Frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17), a rare form of dementia that is believed to be inherited from one parent and is linked to a defect in the gene that makes the tau protein. The three core features are behavioral and personality changes, cognitive impairment, and motor symptoms. People with this type of FTD often have delusions, hallucinations, and slowness of movement and tremor as seen in Parkinson’s disease. Typical behavioral/personality characteristics include apathy, defective judgment, and compulsive and abusive behavior. Diagnosis of the disorder requires the confirmed presence of clinical features and genetic analysis. Palliative and symptomatic treatments such as physical therapy are the mainstays of management.

* Pick’s disease, a tauopathy subtype of FTD characterized by hallmark Pick bodies—masses comprised of tau protein that accumulate inside nerve cells, causing them to appear enlarged or balloon-like. Some of the symptoms of this rare neurodegenerative disorder are similar to those of AD, including loss of speech, inappropriate behavior, and trouble with thinking. However, while inappropriate behavior characterizes the early stages of Pick’s disease, memory loss is often the first symptom of AD. Antidepressants and antipsychotics can control some of the behavioral symptoms of Pick’s disease, but no treatment is available to stop the disease from progressing.

Progressive supranuclear palsy (PSP) is a rare brain disorder that damages the upper brain stem, including the substantia nigra (a movement control center in the midbrain). This region also is affected in Parkinson’s disease, which may explain an overlap in motor symptoms shared by these disorders. Eye movements are especially affected, causing slow and then limited mobility of the eye. The most common early signs and symptoms include loss of balance, unexplained falls, general body stiffness, apathy, and depression. A person with this type of dementia may suddenly laugh or cry very easily (known as pseudobulbar affect). As the disorder progresses, people develop blurred vision and a characteristic vacant stare that involves loss of facial expression. Speech usually becomes slurred, and swallowing solid foods or liquids becomes difficult. PSP gets progressively worse, but people can live a decade or more after the onset of symptoms. Dextromethorphan, a common ingredient in cough medicine, has been approved for the treatment of pseudobulbar affect.

Argyrophilic grain disease is a common, late-onset degenerative disease characterized by tau deposits called argyrophilic grains in brain regions involved in memory and emotion. The disease’s signs and symptoms are indistinguishable from late-onset AD. Confirmation of the diagnosis can be made only at autopsy.