Penn FTD Center Roadmap

Roadmap Introduction

Here at the Penn FTD Center we understand that a diagnosis is challenging and complicated. We have put together a webpage of information to start your care journey. This road is not just for those newly diagnosed! We encourage you to use this information at any point in your FTD journey to better understand what support is available to you. Though the stops are numbered, there is no “one way” to move along the path. Please use whatever resources you need.

The Penn FTD Center is honored to provide this roadmap as a resource for all those living with and caring for an individual with FTD. While some of the resources here may be widely applicable, our roadmap focuses primarily on resources local to the Pennsylvania area.

Stop #1: Learn About FTD

Frontotemporal dementia (FTD) is a group of neurological conditions affecting the frontal and temporal lobes of the brain. These clinical syndromes are characterized by progressive deficits in behavior, executive function, and language caused by loss of neurons (brain cells) also called neurodegeneration. Neurodegeneration refers to any disease of the brain that results in parts of the brain shrinking in size.

FTD is often misdiagnosed as a psychiatric condition or depression. FTD is most commonly characterized by it's earlier age of onset, frequently found in those ages 40-65 years old.

FTD is an umbrella term for multiple subtypes, which are also referred to as clinical syndromes. Each syndrome has its own unique key characteristics, although there may be overlap between syndromes as someone progresses through their disease.

Click through the tabs above to learn more about the primary characteristics and symptoms of each subtype of FTD.

Symptoms of bvFTD can include:

Early impairments in social behavior.
- Progressive and not episodic.
- Represent a departure from their baseline personality.
- Behavioral features of disease can manifest differently from person to person.

Behavioral disinhibition
- Approaching strangers, rude comments, impulsivity, responding to solicitors, and falling for scams.

Apathy
- Loss of interest/motivation in previous hobbies, reduced hygiene requiring reminders.
Loss of Empathy
- Loss of emotional warmth, less social interest.
Strict rituals/compulsions
- Counting steps, preoccupied with an activity that is difficult to interrupt.
Hyperorality
- Gorging, weight gain, carbohydrate craving.
Cognitive impairment and evidence in imaging may be subtle in early stages of disease.

It is important that there is a reliable informant available who has consistent time spent with the patient to provide additional insight and context for clinical care.

The AFTD provides a diagnostic checklist for bvFTD which can be found here.

Primary Progressive Aphasia (PPA) is is characterized by noting the earliest symptom is language impairment that progress throughout the disease. Symptoms of PPA can include:

Non-fluent Variant (naPPA)
- Slow hesitant speech - difficulty getting words out
- Grammar/spelling mistakes
- Can have additional motor speech difficulties
- Slow halting speech
- Sound distortions in speech
- Slurred speech, unclear articulation, and changes in voice quality
- Impaired comprehension of syntactically complex phrases
- Can be associated with Progressive Supranuclear Palsy (PSP) /Corticobasal Degeneration (CBD)
Semantic Variant (svPPA)
- Difficulty with comprehension of words/objects.
- Trouble naming items (“thing”, “it”)
- Stereotypic phrases with limited meaning
- Fluent, empty speech production
- Loss of object knowledge/single word comprehension (semantics, difficulty describing what a word means)
- Impaired naming of objects
- Difficulty reading/recognizing irregular words like Choir, Yacht, Pint, etc.
- Difficulties with fine motor skills like handwriting, letter spacing, writing in a straight line, and making letters the correct size.
Logopenic Variant (lvPPA)
- Variable fluency - (rate of speech) from circumlocutions (“talking around the word”)
- Difficulty with repetition
- Speech with word-finding delays and difficulty with repetition
- Talking around the word
- Substituting sounds/syllables in a word, with the result being a word sounds similar to the intended word, but may have different meaning (i.e., saying “apple” as ‘able’).

The AFTD provides a diagnostic checklist for PPA which can be found here.

Symptoms of FTD ALS can include:

Cognitive Impairment (present in ~50% of ALS, ~15-20% FTD ALS)
- Executive impairment is typically more prominent than deficits in memory
- Evidence of a social disorder (bvFTD) is typically more prominent than language impairment
Upper Motor Neuron Symptoms
- Spasticity - involuntary movements from tight reflexes/muscles
- Increased reflexes
- Spasticity - Muscle stiffness, tightness, and resistance to movement
- Hyperreflexia - Overactive reflexes
Lower Motor Neuron Symptoms
- Weakness
- Trouble swallowing
- Muscle wasting/weight loss
- Fasciculations – Visible, involuntary twitching of an individual muscle
- Breathing difficulty, especially when laying flat

The AFTD provides a diagnostic checklist for FTD ALS which can be found here.

Symptoms of CBS can include:

Asymmetric Parkinsonism
- One side of the body slow, rigid and/or tremor
Cognitive impairment
- Executive impairments - planning/organizing
- Visuospatial impairment - judging distances, finding items in pantry
- Apraxia - Impairs a person's ability to plan and perform movements, even though they understand the task and have the physical ability to perform it (such as using a utensil)
- Alien limb phenomenon - Sensation that an arm or leg is not part of the body, accompanied by inability to control movement of the limb
- Can develop PPA/ bvFTD

The AFTD provides a diagnostic checklist for CBS which can be found here.

Symptoms of PSP can include:

Postural instability
- Increased risk of falls
- Difficulty maintaining balance and posture
Occulomotor dysfunction
- Double-vision for reading
- Inability to make voluntary eye movements like looking up or down or left to right
- Slowing of the ability to shift eyes up and down or left to right
- Photo sensitivity
- Reduce blink (eyelid apraxia)
Axial Rigidity
- Stiffness, particularly for neck, can be extended throughout the spine
Cognitive Impairment
- Executive planning and organizing
- Can develop PPA and/or bvFTD

The AFTD provides a diagnostic checklist for PSP which can be found here.

Stop #1
Learn about FTD

Whether you have a formal diagnosis or are just beginning your diagnostic journey, we know the rarity of this disease can make it hard to find comprehensive information. Getting a basic understanding of FTD will assist you or your loved one in feeling empowered to speak about FTD with clinical providers and others in your support network.

FTD can often be confused with Alzheimer's Disease. For information on the similarities and differences between FTD and Alzheimer's Disease, click here.

The National Institute on Aging has provided a free resource on Frontotemporal Disorders that is available to download here.

To better understand the terminology used when speaking about FTD, you can download and print the FTD Terminology resource here.

Stop #2: Build A Care Team

Stop #2
Build a Care Team

What is a Care Team?

A care team is a group of health care professionals who each support a patient through their specialized training and expertise.

Why is building a Care Team important?

We don’t want you to navigate this journey alone! Establishing this team early in a diagnosis is important so you have support and guidance as needs evolve.

Who may be on my Care Team?

Clinical Team Members

Primary care provider
- Your primary care provider is your primary doctor who oversees your whole health, not just health related to your brain. It is important to notify your primary care provider of changes in memory and thinking because they can help you navigate testing and diagnostics. They will continue to be your primary physician even after you meet with a neurologist.
Neurologist
- A licensed doctor that specializes in disorders of the brain. They can provide testing, determine a diagnosis, walk through treatment options, discuss medication management and explain prognosis.
- A neurologist provides care related to the brain; they are unable to replace care provided by a primary care physician. It is important to maintain connection with your PCP while seeking care from a neurologist.
Nurse practitioner
- A registered nurse with a graduate degree that works together with a medical doctor to diagnose and treat disorders of the brain. They can provide diagnoses, develop treatment plans, and discuss medication management. They are particularly skilled in addressing complex symptom management and supporting caregivers with a person-centered approach.
- For more information on the role of nurse practitioners, view our blog post here.
Neuropsychologist
- A specialized psychologist (therapist) who can administer specific assessments to more precisely understand changes that are happening in the brain.

Psychiatrist
- A licensed doctor that can provide talk therapy and/or pharmaceutical options to help manage psychiatric symptoms in a person living with dementia.

Social worker
- A social worker is a licensed professional who specializes in social, emotional, and legal support. A social worker can assist you with finding community resources, navigating behavioral symptoms in the person living with dementia, and planning for the future, emotionally and legally.
- For more information on how a social worker can help, view our blog post here.

Genetic counselor
- A genetic counselor is a licensed professional who specializes in guiding families through understanding familial risk as well as genetic testing. They provide support before, during, and after genetic testing to ensure you feel educated and equipped to make the decision of whether or not to have the testing done.
- For more information on genetic counseling, view our blog post here.

Registered Nurse
- A nurse is a licensed professional who can assist with questions about day to day care, behavioral issues, or general questions about diagnosis and prognosis.

Physical, occupational and speech therapists
- Each of these therapists offer specialized care related to physical movement (physical therapist), fine motor skills and day to day tasks (occupational therapist), and speech and language changes (speech therapists).

Psychotherapist or counselor
- You can find licensed and unlicensed individuals who may be able to provide talk therapy. Social workers, therapists, counselors, and peer mentors are just some of the titles of people that can provide support.

Home health care
- When considering adding more care assistance in the home, a home healthcare agency will set up a team for you. It is likely that your primary point of contact will be a nurse or specialized therapist (e.g., PT, OT, SLP). Financial coverage of home health care varies by insurance and providers. Check with your insurance company to determine what home health care options you may have.
  - Home health care can assist with bathing, dressing, mealtime, medication management, and care of complex medical conditions.
Assisted living care team
- When moving into assisted living, ask the facility what care teams they have in place. It is helpful to consider what support you may request when you move into assisted living.
Palliative care team
- Palliative care is a complementary medical service that can help to alleviate stress and pain associated with chronic health conditions. Ask your neurologist or nurse practitioner about whether adding palliative care to your care plan would be beneficial.

Non-Clinical Team Members

Primary caregiver: often spouse, child, sibling, friend
Neighbors, friends, religious groups
Community groups: adult day centers, senior living groups
FTD Support Groups
The Association for FTD, CurePSP, Lewy Body Dementia Association, Alzheimer’s Association
Elder care attorney or financial advisor

We’ve included a non-exhaustive list above of members that might be included in an FTD Care Team. For more information on Care Teams, click here for the Penn FTD Center blog post or here for the AFTD resources page.

To begin building your Care Team at the Penn FTD Center, you can contact the Department of Neurology Clinical Care line at 215-662-3606 for a new patient appointment.

To find clinical care in other areas, please use the AFTD Support Group and Diagnostic Center Locator linked here.

Stop #3: Legal & Financial Planning

Stop #3
Legal & Financial Planning

At this stop, you will begin asking yourself questions regarding logistical decisions that need to be made for any legal or financial situations. An FTD diagnosis may impact previously established legal and financial planning for both the person living with FTD and their care partner.

We list the questions as a starting point to be considered from both the person living with FTD and caregiver perspective. We encourage you to seek further consultation from appropriate professionals.

1. Do we have an emergency contact established?

Is an emergency contact established for both the personal living with FTD and the care partner? Is that emergency contact aware of FTD and their potential responsibility? Is the phone number, email, etc. for my emergency contact updated as necessary? This may include medical documents. work documents, neighbors, etc.

2. Do I need to establish POA?

By designating a power of attorney (POA), a person living with dementia can ensure their financial and other important decisions are in the hands of someone they trust. The person living with dementia maintains the right to make their own decisions until they no longer have legal capacity. The earlier that a POA can be established, the more likely the needs and wishes for the person living with FTD can be honored.

4. Are all appropriate documents updated with the correct beneficiary information?

FTD can cause a person to make irrational and risky decisions. Therefore, it is advised to consider updating documents related to finances to protect the person living with FTD. This may include bank accounts, retirement accounts, investment accounts, etc.

3. Are the appropriate legal documents updated?

There are many documents to consider updating after a person is diagnosed with FTD. This may include wills, property deeds, bank accounts, disability paperwork, Family and Medical Leave Act (FMLA) assistance, long-term care insurance, etc. For additional information, find resources available on the Alzheimer's Association website here.

5. Are you aware of federal and local disability support and services?

- Pennsylvania Disability Services

- Federal Disability Services

- Alzheimer's Association for Social Security Disability

This is not an exhaustive list of questions to consider. It is always best if you and your loved one consult with a lawyer.

For a lawyer in the Pennsylvania area, please consult the Pennsylvania Bar Association.

For additional lawyers, please consult the National Academy of Elder Law Attorneys.

For additional resources, please review the AFTD Planning for Legal, Financial and Health Insurance Issues.

Stop #4: Find Support & Resources

Stop #4
Find Support & Resources

Whether its support groups, advocacy organizations, educational conferences, or online resources, we want you to find the assistance necessary.

Support can mean a lot of different things to different people. To begin, we've listed organizations, blog posts, podcasts, and more to find local and regional aid.

Below is a list of Penn FTD Center blog posts that may be helpful in providing information, support, and practical tips for care.

Effective Communication Strategies

Driving & FTD

Travel Tips for a Loved One with FTD

Navigating the Holiday Season

GUIDE Program

What about the Kids?

How to Select a Home Care Agency

Adult Day Centers

Below is a list of FTD related patient advocacy groups.

Below is a list of organizations dedicated to provide support and resources for caregivers and those living with FTD and other related dementias.

AFTD

Alzheimer's Association

Cure PSP

Dementia Society of America

Home Instead

Lewy Body Dementia Association

Lorenzo's House

Additional podcast, blogs, and other media related to FTD can be found at the links below.

Remember Me Podcast

Book by Susan Koen, PhD

Book by Emma Heming Willis

Stop #5: Stay Active

Stop #5
Stay Active

Life doesn't stop for caregivers or those impacted by FTD. Here, we want to provide resources that can help you adjust to a new lifestyle. Staying active, mentally and physically, is important for both the caregiver and those living with FTD. There are organizations in the Philadelphia area dedicated to helping caregivers and those living with dementia to stay connected to their community.

Artz Philly

Philly Neuro Arts Network

The University of Pennsylvania Memory Center (PMC) has several programs and services to help those adjusting to a lifestyle of caregiving. Use the links below to explore their in-person and virtual programming.

The Caring Collective

Memory Cafe

Creative Expression Through Music

Comedic Connection

Programs & Services

Penn FTD Center Events

For information on healthy diets and lifestyle choices, use the resources below.

Healthy Hearts & Healthy Brains

Alzheimer's Association Eat Right

American Heart Association Food

For tips for caregivers, use the resources below.

Taking Care of Yourself

Stop #6
Consider Research

Stop #6: Consider Research

For those interested, we would encourage a deeper look into participation of research. Here you can learn what participation in clinical trials and observation research might include. Click the car icons below to download a handout on the language of clinical trials and obersavational research.

What to know about Clinical Trials

What to know about Observational Research

Participation in research, for those who chose to participate and are able to, has an enormous impact on advancing the clinical care for FTD and related disorders.

Data collected in observational research programs help the FTD research community to:

Understand natural history and progression of disease
Develop new diagnostic tools and markers of prognosis
Discover therapeutic targets that enhance and further the development of treatment trials focused on treating the underlying biology of FTD and related disorders
Understand and work to reduce barriers to health equity
Create and enhance support programs for people living with FTD

Do you know which trials or research you are interested in and want to get involved with at the Penn FTD Center? Click here for active clinical trials and click here for active observational research along with the appropriate contact information!

You can find additional clinical trials sites by clicking here or visiting clinicaltrials.gov.

The Penn FTD Center is incredibly thankful to all patients and families who make altruistic contributions through research and advocacy programs, and selflessly dedicate their time, energy, and trust to the FTD research community each and every day.

Roadmap Conclusion

Thank you for reviewing Penn FTD Center Roadmap! We hope this information will continue to be useful throughout the diagnostic journey. Throughout the "rest stops" we hope you understand that you are not alone.

For information from the Penn FTD Center, please contact 215-349-5863 or PennFTDC@pennmedicine.upenn.edu.