A Caregiver’s Guide to Frontotemporal Degeneration

Authors Gary and Lisa Radin have a very personal, in-home care experience with dementia, and want to share their journey to help other caregivers.

A Caregiver's Guide to Frontotemporal Degeneration

Learn more about their book, “What If It’s Not Alzheimer’s? A Caregiver’s Guide to Dementia,” and their story.

Caring for Someone at Home

It has been many years since our bodies have recovered from the incredibly challenging task of caring for a loved one, yet our minds still have vivid memories of the overwhelming experience. We are the wife and son of an intelligent, loving, and generous husband and father. We are the caregivers of a beautiful man who died at age fifty-eight after suffering from a neurodegenerative brain disease.

Our four years of providing in-home care unraveled a series of events that we discovered no one could ever be prepared for. Every day included the challenges of what doctors to consult, where to go for financial assistance, who could provide us with support, how to get information, and when we would ever deal with the loss. We were driven to find answers to questions that would help us understand, cope, and manage and put us on a path to learn everything we could from every source we could find. Finding almost nothing, the only answer we did see to make it through was to pave our own road.

“What If It’s Not Alzheimer’s? A Caregiver’s Guide to Dementia” is a map of the road we traveled. It is a collection of information addressing everything we had to confront and conquer while caring for our loved one.

Learning More About Non-Alzheimer’s Dementia

The medical profession has been distinguishing dementia illnesses in greater depth in recent years. As a result, diagnosis has led to other dementias as clinical observations rule out Alzheimer’s. For this reason, the medical community and our ever-growing caregiving society must educate itself and disseminate the distinctions that will provide better treatment and care to those afflicted with frontotemporal degeneration (FTD) and related disorders.

Non-Alzheimer’s disorders are often considered rare; however, they are not actually that uncommon. It could be said that FTD is often misdiagnosed and under-recognized.

Too many people struggle with unanswered questions, little direction, and no diagnosis, sometimes for as long as years. For this reason, we created this guidebook that will be useful to caregivers who are moving down the road and to professionals who are directing them.

For you, the caregiver, we understand what lies ahead. It is a difficult time and certainly an emotional one. Your commitment to provide the best quality of life for your loved one is recognized. And we know that the time and energy it takes is unparalleled. Be strong, be fearless, and, most of all, keep on loving the one you care for as well as yourself.

Understanding FTD and Caregiver Challenges

FTD is a disease process that results in progressive damage to the temporal and/or frontal lobes of the brain, the parts of the brain that control “executive functions” such as decision-making, personality, social behavior and language. It causes a group of brain disorders that share many clinical features. FTD is also commonly referred to as frontotemporal dementia, frontotemporal lobar degeneration (FTLD), or Picks disease.

FTD is distinct from other forms of dementia in two important ways:

  1. The hallmark of FTD is a gradual, progressive decline in behavior and/or language (with memory usually relatively preserved). As the disease progresses, it becomes increasingly difficult for people to plan or organize activities, behave appropriately in social or work settings, interact with others, and care for oneself, resulting in increasing dependency on caregivers.
  2. Onset of FTD often occurs in a person’s 50s and 60s, but has been seen as early as 21 and as late as 80 years. Roughly 60% of cases occur in people 45-64 years old (Knopman, 2011), thus FTD can affect work and family in a way dementia in older patients does not.

Because of the nature of these symptoms (and the fact that a patient is often “too young” for dementia to be considered), FTD is often initially misdiagnosed as a psychiatric problem or movement disorder, such as Parkinson’s disease. Alzheimer’s disease is another possible misdiagnosis.

It is important for caregivers and families to think about long-term management issues and identify a team of experts who can help with difficult medical, financial and emotional challenges. It is imperative to have a physician who is knowledgeable about FTD and approaches to treatment. Other medical specialists who may be helpful include: speech and language pathologists, occupational and physical therapists, neuropsychologists, nurses (especially home-care nursing), social workers and genetic counselors. Non-medical professionals may include an elder lawyer and clergy.

Currently, there are no treatments to slow or stop the progression of FTD. However, research is advancing and initial clinical trials are underway. Although specific symptoms may vary from patient to patient, FTD is marked by an inevitable deterioration in functioning. The length of progression varies, from 2 to over 20 years with an average of 7-13 years from the onset of symptoms (Onyike and Diehl-Schmid, 2013). FTD itself is not life-threatening. It does, however, predispose patients to serious complications such as pneumonia, infection, or injury from a fall. The most common cause of death is pneumonia.

Caregivers have so many challenges. It is critical to build a support network that expands beyond professionals to include family, friends and other community relationships and resources.  Some important things to help manage being a caregiver:

  • Educate yourself about the disease and it progression
  • Understand what is happening to your family member – this is the key to learning how to cope with the disease
  • Tell your family and friends about the diagnosis and what it means
  • Ask for help
  • Join a support group
  • Make use of respite resources
  • Take care of yourself

FTD vs. Alzheimer’s Disease

Both frontotemporal degeneration FTD and Alzheimer’s disease (AD) are characterized by atrophy of the brain, and a gradual, progressive loss of brain function. However, several important distinctions can help to differentiate between the two:

  1. FTD is primarily a disease of behavior and language dysfunction, while the hallmark of Alzheimer’s disease is loss of memory.
  2. FTD often begins earlier than AD with an average age of onset in the 50s and 60s, a full 10 years before the average Alzheimer’s patient is diagnosed.
  3. FTD patients exhibit behavioral and personality changes (lack of concern for social norms or other people, lack of insight into their own behaviors), but retain cardinal features of memory (keeping track of day-to-day events, orientation to space and time).
  4. AD patients display increasing memory deficits, but typically retain socially appropriate behavior.
  5. Some FTD patients may have only language dysfunction (this is seen in the two types of progressive aphasia: semantic dementia and progressive non-fluent aphasia). And the pattern of language loss may be specific, such as an inability to name a familiar, everyday object.
  6. The language decline seen in AD patients involves a milder problem with recalling names and words.
  7. FTD patients are more likely to display early motor abnormalities, such as difficulty walking, rigidity or tremor (similar to Parkinson disease), or muscle atrophy and weakness.

Facts about FTD:

  1. FTD affects an estimated 50,000-60,000 Americans (Knopman, 2011; CurePSP, www.psp.org).
  2. FTD represents an estimated 10-20% of all dementia cases. It is recognized as one of the most common presenile dementias (meaning it occurs in a younger population).
  3. The prevalence worldwide is uncertain with estimates of FTD amongst people ages 45 to 64 between 15 – 22 per 100,000 (Knopman, 2011).

About “A Caregiver’s Guide to Dementia”

What If It's Not Alzheimer's Book Cover

Although the public most often associates dementia with Alzheimer’s disease, the medical profession now distinguishes various types of “other” dementias. “What If It’s Not Alzheimer’s? A Caregiver’s Guide to Dementia” is the first and only comprehensive guide dealing with frontotemporal degeneration (FTD), one of the largest groups of non-Alzheimer’s dementias. This thoroughly updated edition offers new daily-care ideas for caregivers to implement, reveals new discoveries of how genes and proteins are linked to the causes of dementia, explains the changes in terminology that have developed over the past several years, explores nonpharmacological approaches to managing care, and provides more guidance and resources to aid caregivers along this challenging journey. All contributors to this volume either are specialists in their fields or have exceptional hands-on experience with FTD sufferers. The wealth of information offered in these pages will help both healthcare professionals and caregivers of someone suffering from non-Alzheimer’s dementia.

“What If It’s Not Alzheimer’s?,” 3rd Edition, is available on October 7, 2014. It can be found online, in print and e-book formats from major sellers such as Amazon and Barnes & Noble.

Excerpt from “What If It’s Not Alzheimer’s?”

(2014 Prometheus Books)

Part 2 INTRODUCTION: A Framework for Quality of Life with FTD

Sharon S. Denny, MA

A Framework for Daily Life

“I didn’t cause it. I can’t change it, and I can’t control it. But I do have choices about how to live each moment. . . . I will make life as enjoyable, dignified, and meaningful as possible for as long as I can.”

—Eleanor, caregiver for her husband

Not too long ago, people receiving a diagnosis of dementia were advised to get their affairs in order and prepare for the end. Public awareness of Alzheimer’s disease and other dementias and advocacy by people who are themselves diagnosed is starting to reduce past stigma and put more focus on living with the disease. Someone with FTD did nothing to cause the disease, and in the absence of much-needed treatment, cannot control its eventual outcome. But countless large and small choices will help you live as well as possible for as long as possible.

Consider How You Want to Live

While there are many things about FTD and changing care needs that cannot be predicted or controlled, you can control your approach to the disease. Discuss your goals with as much input as possible from the person diagnosed. Are there ways to position yourself to be near supports and resources before you need to access them? What can you do to lower the stress and stimulation around you? Are there things you want to do together or trips you want to take? You will need to do some things differently, but you can still pursue shared goals and interests and create memories together.

Adjust Expectations

Someone with FTD will have more trouble focusing and interacting when they are tired or in an overly stimulating environment. Look for new ways to do things you enjoy and value. Adjusting expectations and planning activities and commitments accordingly will make it possible to stay engaged more effectively with fewer difficulties.

Develop a Positive Daily Routine

No lifestyle change will stop the progression or reverse the disease. However, items that promote overall physical health and mental and emotional well-being are positive practices for all, including those with FTD. Predictable routines provide an important outline for the day for both the person with FTD and the caregiver. Create an engaging and balanced routine of daily-living activities such as meals, household tasks, and errands with physical activity; mental stimulation; hobbies; social interaction and spiritual development. Be consistent yet flexible to accommodate your day-to-day reality.

Clinicians are beginning to study the effectiveness of pharmacological and nonpharmacological interventions in FTD, but evidence-based practices are lacking. The principles of general dementia care must be adapted to fit the particular needs of people with behavioral variant FTD and primary progressive aphasia. Each intervention must be tailored creatively to the individual, based on knowledge of the person and his or her situation. Nothing will work optimally from the start. When you routinely observe and adjust each intervention, it becomes an experiment that will provide ideas to build on in the next situation.

Be Prepared to Advocate and Educate

Awareness is improving, but there are far too few services that are experienced in serving people with FTD. Do not be surprised that friends, family, and service providers are not familiar with FTD. The main caregiver knows best the affected person’s background, preferences, abilities, and needs. Your advocacy is the most effective way to access care. Develop a folder of relevant articles, booklets, and resources for more information that you can take to new providers. Being prepared to educate can help to temper the frustrations you encounter.

Do Not Go It Alone

The emotional, physical, and logistical challenges of managing daily care are impossible for anyone to handle alone. Those who choose to accept assistance will be more effective and satisfied caregivers. People grow in confidence gradually as they begin to speak with closest family or friends about the disease. Identify specific tasks that are needed and people who can help. Most people want to help and appreciate suggestions for how they can.

Remember, the goal is to make each day as good as it can be.

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About The Author

Profile photo of Gary Radin