National Alzheimer's Project Act: Public Comments on Advisory Council Meeting, January 2020

Good morning. Thanks to Helen for taking time to read my brief comments. I had hoped to be in attendance for this first meeting in 2020, a new decade, to support my dear friend Jane as she spoke to you today. I had hope, too, to be present to thank Dr. Levey for reminding me of the importance of a worldview that would include a “glass half full” and for helping me connect with those involved in the development of the Savvy Caregiver Training and subsequent recent research on the Savvy Tele-health model that was presented to the Council in January 2019.

Jane’s story of her sister Ellen is a very poignant journey and reinforces the challenges experienced by many individuals with intellectual disabilities and their families. Last year Jane presented at the NDSS Adult Summit along with a Medical Ethicist. Collectively they conveyed the importance of maintaining quality of life until the end of life for one and all and of the importance of the medical pledge to do no harm as people face end of life challenges.

2020 is a number that my brother Bill would have embraced wholeheartedly. He was a man who loved dates and calendars and kept daily a fastidious health record on his calendar for years.

2020 would have been the year of Bill’s 60th birthday. I often wonder how he would have aged and what wisdom he would have imparted as they years went by. I wonder too, if he would have continued to love Julia Robert and maintained his alias, Harrison Ford, as he traveled by plane independently and introduced himself to the flight crew using his alias!

Bill died on February 25th, 2010 of complications of Alzheimer’s disease. He was 49 years old. I will forever remain haunted by his end of life experience. Nonetheless, as I reflect on these 10 years since his departure I am reminded that the “glass is, indeed, half full”.

In this time span I have been gifted with the opportunity to meet and work with many incredible people that I would not have met, had it not been for my brother Bill. This includes so many families from across the globe, each with their own compelling stories of love, commitment and life long challenges. Some of you who have been at this table for many years will recall such heart wrenching stories laden with challenges that I related to you...like Betty, Frank and Richard.

For the past several years I have been given the opportunity to come to this table and give voice to the special issues that people with Down syndrome and other intellectual disabilities and their families’ experience. I am grateful that some of you have been able to relate to these unique issues encountered by a unique population, a population that can enable each of us to reflect on life’s meaning on compassion, kindness, equality and inclusiveness.

I believe that Bill left behind what I consider divine energy. He helped me step out of my comfort zone and speak, often and adamantly about people that are often forgotten and voiceless. He helped me impart his loving healthy perspective as I attempted to convey what is needed to by so many with intellectual disabilities as they age and face the challenges of AD or other dementia. He challenged me to think about who is really disabled, is it those with the label or is it those of us with limited vision who cannot see what each of us has the capacity to contribute in life.

In 10 years time many things have been accomplished...added research dollars, inclusion in the National Plan, dollars spent on training, increased dialogue about this special population along with new voices and faces who can continue as engaged advocates for those who often cannot voice their own needs. In the next 10 years may we have the wisdom, capacity and the ability to recognize the necessity to continue our efforts on behalf of this very important group of people.

For these things I am grateful.

I am from Sea Girt, NJ.

I was sister, legal guardian, primary caregiver, housemate, and sidekick for my sister Ellen Boyle, a woman with Down syndrome. We lived together. She died at home on hospice from end stage Alzheimer’s Disease at age 52 in 2018.

When she was born in 1965, our family was told her life expectancy would be into her 20s. She lived at home her entire life graduating from high school at age 21, worked for 20 years and then attended day programs. She was extremely active in special olympics, social, civic and church activities. She was well known in the community and was beloved. She exceeded so many expectations, but at age 50 was diagnosed with Alzheimer’s, something we never imagined. This marked start of a steady and fast decline that resulted in her death just two years later.

We now know that those with trisomy 21--Down syndrome--are at very high risk for Alzheimer’s/Dementia and for many signs become evident in their 40s and 50s.

Alzheimer’s caused changes in Ellen that were baffling to understand, painful to watch, and extremely difficult to adapt to. But it was equally challenging to access the information and the resources needed to navigate the disease process, to preserve a quality of life, ensure comfort, and ultimately support a peaceful death. Her accurate diagnosis was as devastating as it was difficult to obtain. We encountered the reality that at the time there was less understanding of Alzheimer’s in persons with Down Syndrome.

At the urging of the National Task Group on Dementia on I/DD, in New Jersey we have begun a Family Support Group for Down syndrome and Alzheimers/Dementia. In just one year, 40 families have joined us seeking support, information and resources. As word spreads, we hear from more families each month. Sadly we had 4 deaths this past year.

On behalf of those aging with Down syndrome and their families and caregivers, please be aware of the significant impact of Alzheimer’s Disease on those with Down syndrome and consider their special needs in your work.

I am the Chief Executive Officer for Jewish Foundation for Group Homes, a Montgomery County, MD-based provider of community living supports to adults with developmental disabilities. I began my career in this particular field in 1987, and have watched both the systems of services and supports, as well as the philosophies that inform them, change in overlapping ways over the ensuing 32 years.

At the turn of the 20th Century, the average lifespan of a person born with a developmental disability was 19 years. By the turn of the 21st Century, the average lifespan had grown to 66 years. Advancing science and medical technology has had a profound and lasting impact on lifespan, and people with developmental disabilities are living longer than at any point in human history. We rightly celebrate longer, fuller lives for people with developmental disabilities--my organization, JFGH supports a woman who is 92. She is, by virtually any standard, a miracle.

At the same time, people with developmental disabilities living longer brings challenges to systems of long term supports and services and healthcare systems, to name but two, for which we are inadequately prepared and resourced. More specifically, Medicaid-financed systems of supports for Americans with developmental disabilities are ill-prepared to support people who are experiencing forms of early-onset dementia and Alzheimer’s disease; and, while Home and Community Based Service (i.e., Medicaid waiver) programs have proliferated across the US since the 1980s to support people in community settings, very little attention has been paid to how these programs can shift to support people with developmental disabilities as they age. Concepts that are bandied about as colloquialisms, such as ‘aging in place’, for people who develop neurotypically, and models of care and support are being developed. For people with intellectual and developmental disabilities (IDD), these models of care are much slower in their development, and are broadly inaccessible.

Among the Council’s membership, Dr Matthew Janicki, a recognized expert in the relationship of IDD to dementia and dementia care, and a small band of his colleagues, have informed the research literature as to approaches to supporting people with IDD and dementia in community settings. And, there is enough in the literature to create a body of best practice for community-based organizations like JFGH. However, the public funding that pays for community-based supports is not aligned with those best practices at best, and at worst, is simply insufficient to provide any meaningful care that is tailored to the needs of people with IDD receiving Medicaid waiver supports who are experiencing dementia.

As an example, JFGH supports a gentleman--“John”--with Down syndrome who is 61 years old. As you all perhaps know, people with Down syndrome experience early onset dementia and Alzheimer’s disease at a disproportionally higher rate than their neurotypically developing peers. To some extent, John was a bit atypical to the extent that he lived well into his 50s without experiencing any signs of dementia. Three years ago, John’s life changed radically and seemingly overnight. Signs of advancing dementia, subtle at first, because substantial. In an exceptionally short period of time, John lost basic skills in activities of daily living, including his abilities to feed himself, use the bathroom independently, and even ambulate independently about his environment. More substantial than these losses was the radical change to his personality. A once gregarious, fun loving friend and colleague to many stopped speaking, interacting socially, making eye contact, and the host of other personality traits that had once earned him the nickname “Disco John.”

As the direct support and other staff at JFGH watched this process, we were unprepared as an organization to support the staff’s process of adjusting to John’s fast-evolving support needs, as well as how to support staff emotionally as they experienced John’s loss of connection to them. While staff aren’t--and arguably should not be--family, they experienced John’s loss of capabilities every bit like a family member might.

As JFGH grapples with fitting John into a service system that is not equipped and resourced to support his needs, we are left to grapple with the potential of simply discharging his from our care as “medically complex,” knowing that he would live out his days in a nursing home, a strange and unpredictable environment at which John will never be “home.”

In JFGH’s community living supports, 40% of the people we currently serve are aged 40 years or more. These are people for whom supports must evolve to both acknowledge the aging process, the onset of dementia that is likely, and how best to support them where and how they age. Dr Janicki and others have provided a support framework designed to deliver quality outcomes for people with IDD and dementia--we have a good idea of what to do.

What we don’t have is the means with which to do it.

My call to action to you today is several fold.

To whatever extent the Council can, I urge you to consider taking a specific position(s) that:

1. Advances a wider, intentional dialogue on practical solutions that are culturally appropriate and accessible to people with intellectual and developmental disabilities who are experiencing dementia;
2. Advocates for resources being explicitly dedicated to the support needs of people with intellectual and developmental disabilities who are experiencing dementia--from research funding to specialized services and supports (through, for example, demonstration waivers) that are fully funded; and,
3. Informs the development of a set of universal support standards to address the care needs of people with IDD and dementia that can be used to assess system ‘readiness’ to support the aging population of people with IDD.

In full disclosure, those of us in the field of long term supports and services for people with IDD have made an art form of “admiring the problem.”

In 2002, then-16th United States Surgeon General, Dr David Satcher, issued a report in which he lamented the lack of access to quality healthcare for people with IDD that could deliver an improved health status and better health outcomes. Eighteen years later, progress has been glacial.

In 2013, Dr Janicki and colleagues published Guidelines for Structuring Community Care and Supports for People with Intellectual Disabilities Affected by Dementia.

And, in 2020, organizations like JFGH, largely funded through Medicaid waivers, continue to grapple with how best to support people like John with funding that is inflexible and simply doesn’t contemplate shifts in supports needed to allow John to age in place, to be supported through his journey, to be surrounded by the people most committed to the quality of his life.

The Guidelines are developed, JFGH and countless other agencies like ours are ready to implement and test them, to amass data that inform the evolution of best--and actually evidence-based--practices, and to support people with IDD as they age in place in their homes and their communities. To any extent the Council can push this agenda, you have a willing partner in JFGH, and I stand at your beckon call.

I am the Program Manager for The Association for Frontotemporal Degeneration. I appreciate this opportunity to offer comments from AFTD.

This morning’s presentations on the epidemiology of dementia were excellent. As you may know, the epidemiology of FTD disorders remains largely unknown. Even basic facts such as the prevalence and incidence of FTD are uncertain. AFTD’s estimate of 60 thousands cases in the US is based on a combination of sources and covers all the clinical diagnoses that fall under the umbrella of frontotemporal degeneration. This includes Progressive supranuclear palsy, corticobasal degeneration, all types of primary progressive aphasia, as well as behavioral variant FTD.

Sixty thousand cases is probably an underestimate, considering how often FTD is misdiagnosed. Adding to the challenge is that FTD is probably not a single disease and is associated with several different pathologies, most notably the proteins tau and TDP-43, and a handful of genes including C9orf72 which overlaps with ALS. Until this complex puzzle of pathologies and overlapping clinical syndromes is sorted out, large-scale epidemiological studies will be untenable.

The key to this puzzle are biomarkers. This is reflected in the research recommendations generated by the 2019 ADRD research summits, where the term biomarker is used 48 times. In FTD, developing biomarkers for diagnosis, prediction and disease monitoring in the next 2-7 years is a top priority. In response to the critical importance of biomarkers, AFTD launched the FTD biomarkers initiative. Since 2016 AFTD has awarded 13 grants to develop a variety of FTD imaging tests and fluid biomarkers for clinical trials and diagnosis. There are more details about current and past biomarkers project on AFTD’s website and most studies are included in the NIA’s IADRP database.

If you are interested in more information AFTD Biomarker Initiative or any of the other research funded by AFTD please do not hesitate to reach to me or any of AFTD’s research staff.

Good afternoon and thank you for your time today. I am Vice President of Professional Affairs for The Gerontological Society of America (GSA). GSA honors aging across the lifespan and is the oldest and largest interdisciplinary organization devoted to research, education, and practice in the field of aging. GSA’s principal mission--and that of our 5,500 members--is to promote the study of aging and disseminate information to scientists, practitioners, decision makers, and the public. A large segment of our membership is devoted to clinical practice and research on Alzheimer’s Disease and Related Dementias and some of our members are current or past members of the Council.

As we wait for a cure to emerge, how can we better support persons with dementia and their care partners?

On behalf of GSA, I’d like to highlight one promising area: improved recognition and management of neuropsychiatric symptoms associated with dementia.

Evidence suggests that persons with neuropsychiatric symptoms - or NPS - experience worse outcomes than those without NPS. These outcomes include greater impairment in activities of daily living, earlier institutionalization, and accelerated mortality.

Among the various NPS, dementia-related psychosis includes delusions (false beliefs) and hallucinations (seeing or hearing things that others do not see or hear). These symptoms can be frequent, severe, persistent, and distressing to persons with dementia and their care partners.

Dementia-related psychosis is distinct from psychosis experienced by individuals with schizophrenia and other psychiatric illness, and it can be highly stigmatizing for persons with dementia. Dementia-related psychosis is also one of the factors that may lead families and care providers to seek long-term care placement for their loved ones with dementia.

In August 2019, GSA published a report “Dementia Related Psychosis: Gaps and Opportunities for Improving Quality of Care.” The report was developed in collaboration with experts in geriatrics, psychiatry, neurology, pharmacy and nursing. In it, we summarize best practices and propose several improvements to advancing quality of care that we hope will be widely read and implemented.

Three key recommendations are:

1. Providers need better ways to document a diagnosis of dementia-related psychosis so they can develop appropriate care plans.
2. Care teams need more training and resources, so they may proactively communicate with persons with dementia and their care partners about symptom progression and how to cope.
3. More research is needed to support evidence-based strategies for treatment.

We invite the Council Members to review our full report on the GSA website (https://www.geron.org/programs-services/alliances-and-multi-stakeholder-collaborations/dementia-related-psychosis).

Thank you for your dedication and service on the Council and to people living with dementia and their care partners.

Thank you for another opportunity to provide public comment to the Advisory Council.

In November, I had the pleasure of attending the Neurological Conditions Surveillance Summit, which was an all-day event for national leaders and stakeholder organizations in neurological disorders. The event was organized by Association of State and Territorial Health Officials (ASTHO) and hosted at the Centers for Disease Control and Prevention (CDC) Headquarters in Atlanta, GA.

The following introduction was included in the event materials:

“As a part of the 21st Century Cures Act, Congress authorized the Centers for Disease Control and Prevention (CDC) to develop a National Neurological Conditions Surveillance System (NNCSS) that provides useful estimates of neurological conditions in the United States. CDC will use existing data and emerging tools to create efficient, reusable processes and models that can be applied to multiple conditions to provide disease-specific surveillance information.”

In the first half of the day, attendees learned about the NNCSS, the advocacy efforts behind its origins, funding details, and the progress made in developing and pilot testing in two initial disorders--multiple sclerosis and Parkinson’s disease. Presentations included descriptions of 3 stages of the initial project plan starting in FY2019 and continuing beyond FY2022, pending further funding.

During the afternoon, attendees participated in small group discussions to brainstorm potential criteria that might be considered for the selection of the next several diseases to be added into the NCSS as early as FY2022. Representatives of each small group then presented a summary of their group’s suggestions. Based on the composition of each small group, the recommendations varied widely from the perspectives of rare disorders to very common disorders.

I bring this initiative to the attention of the Advisory Council for several reasons.

First, this is a project of direct relevance to AD/ADRD and as such may be a topic of interest for a future council meeting. While quite a bit is known about the incidence and prevalence of Alzheimer’s disease, there is insufficient data on Alzheimer’s disease-related dementias. The NNCSS appears to be an important resource for narrowing that informational gap.

Next, during the event attendees learned that initial funding for the NNCSS presents budgetary limitations for dedicated project management and neurology staff assigned to the project. As such, the NNCSS presents an opportunity for the sub-committees to consider funding recommendations.

Lastly, the addition of future disorders to the NNCSS database will also require the involvement of patient advocacy organizations and disease experts. The Advisory Council on Alzheimer’s Research, Care and Services serves as an important intersection between federal agencies and a variety of dementia stakeholder types. I see an advocacy opportunity to collaborate on messaging to the CDC about including dementia in its next round of diseases to be added to the database.

I hope these comments will be useful to the Advisory Council and the dementia stakeholders both in the room and watching remotely.

With continued respect and admiration for the work of the Advisory Council on Alzheimer’s’ Research, Care and Services.

I am a geriatric psychiatrist at Butler Hospital and Associate Professor at Brown University. I prepared these public comments with the Professor of Medicine and Chief of the Division of Geriatrics at UCLA. These comments are our own.

The Assistant Secretary for Planning and Evaluation (ASPE) and this Advisory Council must address payment reform for dementia care and treatments. There are at least six working models in the US for dementia care which improve quality, achieve better clinical outcomes for persons living with dementia and caregivers, and lower costs; but, care from these models is not widely available.

ASPE should be aware that on Nov 7, 2019 a one-day meeting called “The Payment Models for Comprehensive Dementia Care” conference convened over fifty clinicians, researchers, advocates, payers and other leaders with expertise in dementia care and healthcare payment. Conference goals were to review short-term solutions for payment reform and describe next steps to accelerate use of current and new payment models. The conference was held in Washington D.C. with support from The John A. Hartford Foundation and Hebrew SeniorLife. Conference outcomes will be published this year.

Meanwhile, HHS should act now to advance payment reform efforts through three activities:

1. Continue work previously conducted by ASPE regarding Examining Models of Dementia Care;
2. Convene at least one work group to address payment reform for comprehensive dementia care; and,
3. Begin immediately to monitor how the inclusion of dementia as a risk adjustment modifier in the CMS Heirarchical Condition Category (HCC) coding affects the definitions of populations of people living with dementia, the quality of care, the types of care received, and the health outcomes of those individuals.

These recommendations are in alignment with the National Plan to Address Alzheimer’s Disease’s Strategy 1.E (Facilitate translation of findings into medical practice and public health programs) and all Strategies under Goal 2: Enhance Care Quality and Efficiency.

I am 46 years old and I have Down Syndrome. I live in New Jersey with my parents. I graduated from high school and I have worked at Banana Republic for 15 years. I take watercolor and dancing lessons. I love to do things with my friends.

I grew up with a very close group of friends and we all have Down Syndrome. We have been together since the 1980’s. There were eight of us and we all enjoyed Special Olympics, bowling, dances and parties. Four of my eight friends have died from Alzheimer’s Disease in their early 50’s. They are Tricia, Judith, Danny and Craig.

None of us knew this would happen to us and our families. People who have Down Syndrome get Alzheimer’s Disease very early.

I am afraid for what the future holds for me and my family. Please include us in the programs you develop.

Thank you for your attention.

My daughter is also providing written testimony today. When she was born with Down Syndrome in 1973, my husband and I were told that she would live to be 40. None of the medical experts knew much more than that at the time. No one anticipated the ravages of Alzheimer’s Disease that were coming our way.

As parents we kept our children home. We started Early Intervention and Pre-School programs for them. We fought to have them integrated into school systems and to be provided transportation. We advocated for job training so they could be productive citizens. We created Transitional Services from the school system to the work force.

The “Good News’ is people with Down Syndrome are living longer than ever. The “Bad News” is there are no services for them as they age prematurely and develop Alzheimer’s Disease. As parents we have been advocating for them their whole lives.

I helped start a Caregiver Support Group in New Jersey for families who are caring for a loved one who has Down Syndrome and Alzheimer’s Disease. We started the group in January 2019 and we have been overwhelmed by families who have reached out to us. At this time we have 40 members. We meet monthly in person or participants can also join by phone or computer. These families kept their loved ones home and are now facing a very cruel end to the efforts, love and support they have already given.

Please keep those who have Down Syndrome who are aging in your future plans and services.

On behalf of patients and families who have been affected by the always-fatal diagnosis of Prion Disease and who are desperately hoping for new discoveries and a cure, we ask for assistance in gaining recognition of Prion Disease as a “Related Dementia” wherever ADRDs are defined.

CJD (Creutzfeldt-Jakob Disease) is a rapidly progressive dementia caused by prions, normal proteins that misfold in the brain and cause disease, much like a-beta and tau in Alzheimer’s Disease and Frontotemporal Dementia, and alpha-synuclein in Lewy Body Dementia. Advances in the field of Prion Disease directly led to the discovery of protein mechanisms, which is currently being exploited to improve diagnosis and develop treatment targets through protein amplification assays like real-time quaking induced conversion (RT-QuIC). Further discoveries about Prion Diseases would be equally applicable to other ADRDs.

Prion Disease/CJD parallels AD in many ways, including the disease mechanism, treatment targets, symptoms, patient experience, and caregiving experience.

Diagnostics and breakthroughs discovered for one protein misfolding disease will continue to assist the others. Prion Diseases naturally occur in animals and are transmissible, which makes Prion Disease the protein misfolding disease with the most valid animal models for research.

To exclude CJD/Prion Disease from “Related Dementias” does not serve patients, caregivers, doctors and scientists. Excluding Prion Disease from the ADRD label prevents valuable research that would improve the ADRD field. We respectfully ask for your assistance in recognizing Prion Disease/CJD as part of the Alzheimer’s Disease and Related Dementias group.