What Is FTD and How Is It Connected to ALS?

FTD (frontotemporal degeneration or frontotemporal dementia) refers to a group of disorders that causes progressive damage to the temporal and frontal lobes of the brain associated with personality, behavior and language.  Loss of function in this area of the brain can lead to impulsive behavior and speech difficulties.  Usually FTD does not affect the parts of the nervous system that control muscle movement, but about 10-15% of people with FTD also experience motor neuron degeneration called FTD with motor neuron disease (FTD/MND) or FTD with ALS.  Over the past 15 years, doctors and scientists’ knowledge of the connection of these diseases has rapidly grown through genetic discovery, brain imaging studies and biomarker studies.  Specifically, researchers were able to confirm the connection between FTD and ALS when the TAR DNA-binding protein 43 (TDP-43) was identified as the central protein in both ALS and the most common type of FTD.  Additionally, up to 40% of FTD cases have been found to carry a C9orf72 gene mutation, which is most common in genetic causes of ALS.

Lobes of Brain
In many cases, FTD symptoms are noted prior to the diagnosis of ALS.  FTD symptoms include:

  • increase in inappropriate actions
  • loss of empathy and other interpersonal skills
  • lack of judgment and inhibition
  • apathy
  • repetitive compulsive behavior
  • decline in personal hygiene
  • changes in eating habits
  • lack of awareness of thinking or behavioral problems
  • difficulty in using and understanding written and spoken language
  • loss of knowledge of word meaning

Paula Richard describes what it is like to experience a loved one living with the symptoms of FTD/ALS:

In 2015 my husband was finally given a diagnosis of ALS/FTD . ALS alone is a terrible disease, but to have FTD with it is awful. Seeing my loved one go through the everyday struggles is heartbreaking. The cognitive issues outweigh the ALS part sometimes. He has bad comprehension and doesn’t know right from wrong. His behavior is like taking care of a little kid. I’m always getting onto him to not do something or to do something. I know none of this is his fault, but dealing with it is very stressful and overwhelming. I get angry with him and then I feel guilty. In addition to having FTD , he has also has PBA ( excessive laughing). When he laughs it’s like a kid laughing at you. He can embarrass you. My loved one is slowly having his life taken away from him. Not being able to do the things he loved to do and just everyday things. Having a support system is very important, having patience and understanding is a must, but very challenging. Life is so very different now, but having God in our lives helps. We just try and take one day at a time.

As Paula mentions, FTD with ALS creates additional challenges for caregivers and it is important to have a strong support system and for caregivers to be able to acknowledge the changes in the behavior and thinking of their loved one.

Some helpful recommendations for caregivers include:

  • Simplify communication with the affected person. Break up sentences into short phrases.  Ask yes/no questions. Slow down when speaking.
  • Provide supervision and accompany the person to all appointments to make sure information is accurately relayed and retained.
  • Set realistic expectations for the person with ALS and set realistic expectations for yourself. If your requests introduce frustration, irritability or withdrawal for either you or the person with ALS, your expectations need to be modified and most likely simplifed to meet the needs of your loved one’s current thinking abilities.  Also, if you are feeling overwhelmed do not wait until you are feeling underwater.  Think ahead to what options you have for help or time away, if needed.
  • Educate providers and caregivers about where to set expectations for your loved one.  In an ideal world, all healthcare providers would be on the same page about what to do and how to accomplish it.  But many healthcare provides outside of dedicated ALS clinics may not have much experience with ALS/FTD and how it affects people.
  • Continue to enjoy activities that bring joy and can be conducted safely, while refraining from activities the result in stress or risk of safety or liability.

While FTD with ALS is rare, current research suggests that up to 50% of people with ALS might experience some degree in change in thinking and behavior.  It is important for all who are impacted by ALS to know that cognitive impairment is possible and how to help those affected adjust to the changes.  For more information on FTD visit The Association for Frontotemporal Degeneration.

For more information on ALS, visit The ALS Association St. Louis Regional Chapter’s website.

Thank You for Sharing Your Journey with Me

By Heather Burns, MSW, LMSW, ALS Association Care Services Coordinator

Today, I received two phone calls. I had that familiar gut wrenching feeling when the names of the patient’s loved one’s flashed across the giant iPhone screen. I hesitated when answering, as if maybe that could change what the caller was about to say…

“My loved one has passed away.” I knew it was coming before today. I knew it was coming before they picked up the phone to call me. I knew it the moment I walked in to meet the family for the initial home visit, but with ALS, I never truly know when I may actually get that call. Everyone’s progression, while always devastating, is always different.

During both of these calls, the loved one on the other end thanked me for the support I provided them and their family member. After almost four years, I still find myself a bit tongue tied when someone thanks me for what I did for their family. Yes, it is my “job”, but it is a job that I have chosen. I take pride in my work and in helping others. But honestly, I think I struggle with what to say back because how do I appropriately and genuinely express my gratitude towards THEM? My appreciation for having just had the privilege of MEETING them and their beloved? The gratefulness of being welcomed into their home during one of the most vulnerable times of their lives? Learning some of the struggles they faced as a family before this disease even became a factor within an hour of meeting? How do I tell the patients and families that I meet how thankful I am that they have given me perspective on what it means to live? Or what it means to die? Is it even appropriate for me to do so? Would they be taken aback when I say it?

How do I adequately and appropriately express to the men and women that I am meeting in my work, that they are the ones providing me with a service? That they are giving me a gift? It’s not wrapped neatly in Tiffany Blue. I cannot hold it or exchange it for a different size. It is a rare gift with no price tag that money could never buy. It is perspective, it is life lessons, it is sorrow, and it is beautiful.

So, from this point forward, I am making a commitment to those I serve to do better at expressing my thankfulness. To the family’s I have worked with in the past, present, and future I want you to know this: While you are facing one of the most difficult times of your life, a time when you may feel like you have lost a sense of who you are, lost the connection to who you were, or a time when you may have no idea who you will be…thank you. Thank you for giving me the privilege of meeting you and for walking alongside you during this journey.

Lighting the memorial candle at the Springfield Walk to Defeat ALS
Heather Burns lighting the memorial candle at the Walk to Defeat ALS.

Heather Burns began working with the St. Louis Regional Chapter as a care services coordinator in 2013. She assists families by educating them on ALS and symptom management, helping to reduce their financial burden by presenting community resources, and offering emotional support for both patients and caregivers.  She received her Masters of Social Work in 2017 from the University of Missouri, St. Louis.

5 Myths about ALS

In these times we live in — the information age — we have access to more facts and data than ever before, but not everything we read or watch is correct.  The Ice Bucket Challenge brought unprecedented awareness to the general public about ALS, but with more exposure also came more misinformation.  Below we break down 5 of the most common misunderstood “facts” about ALS.

  1. ALS is a hereditary disease only.

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There has been a lot of media coverage recently about the amazing breakthroughs that have happened identifying mutations in genes that cause familial ALS.  Familial ALS accounts for about 5-10% of people diagnosed with ALS.  In those families, there is a 50% chance that each offspring will inherit the gene mutation and may develop the disease. The majority of people diagnosed with ALS have no family history of the disease.

  1. Only older people are affected by ALS.

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Most people who develop ALS are between the ages of 40 and 70, with an average of 55 years old.  But there are many cases of people in their twenties and thirties diagnosed with ALS.  This “Young-onset” ALS typically refers to patients younger than 45 years old and accounts for about 10% of all cases.  The disease has even struck people in their teens.

  1. Brain function in not affected in people with ALS

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While it is common for brain function to NOT be affected in people with ALS, approximately 20% of individuals with ALS also develop frontotemporal degeneration (FTD) which is characterized by changes in personality, behavior and speech.  Another rare form of ALS, known as ALS-parkinsonism-dementia complex (ALS-PDC) is characterized by signs and symptoms of ALS in addition to movement abnormalities (slow movements, stiffness and tremors) and loss of intellectual function.

  1. If you’re paralyzed with ALS you can’t feel your body.

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The latter stage of ALS is commonly described as someone being completely paralyzed.  The definition of paralysis is a loss or impairment of voluntary movement, but many people also associate paralysis with a loss of feeling as well as function.  This belief may be because many people with many people with brain or spinal cord injuries that are paralyzed also have lost feeling in the part of their body that is affected.  People with ALS can still feel their body, even though they can’t control its movement.

  1. ALS affects everyone the same way.

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From the initial symptoms to how the disease progresses, ALS can affect people very differently.  The first symptoms of ALS can vary widely depending on the type of onset, bulbar or limb.  People with limb onset usually notice a weakness in their hands or feet and people with bulbar onset might notice a change in their voice first.  The rate at which ALS progresses is also quite different person to person.  While, most people with ALS live with the disease for 2 to 5 years, some people may live 10 or more years with the disease.

You can find out more about ALS by visiting our website: www.alsa-stl.org.

Caregiver Confidential: “Laughter Is the Best Medicine”

A few weeks ago, I asked my friend Jessica if she had any ideas for future posts. Jessica replied, “What about the role of laughter in illness?” Initially, I rejected that suggestion. After all, what part of ALS was funny? I reflected back to my husband Brian’s courageous struggle with the disease, and I didn’t recall us sharing any light or humorous moments. However, the more I ruminated on the topic, the more fascinated I became. I was familiar with the old adage, “laughter is the best medicine,” but could this expression apply to patients with terminal illness?

My investigation started with a phone call to my daughter Leah. “Did you and your Dad share any funny moments when he was sick,” I asked? Leah recalled that Brian and his sister Joyce had a good laugh creating silly and outrageous epitaphs for Brian’s headstone during a 2008 visit. My friend Jessica also concurred that Brian made some humorous remarks to her. With my new-found knowledge, or memory jogged, that Brian occasionally expressed humor about his illness and mortality, my interest was piqued. But what did the experts have to say?

In fact, what does “Laughter is the best medicine” mean? According to various sources, this saying refers to the potential health benefits of laughter, including a reduction in pain, anxiety, and fear. Another important health benefit may be the sense of well-being and improvement in mood. According to Cancer.Net, “Laughter can provide a sense of perspective when you are faced with challenging circumstances.”

While researching the role of humor specifically in ALS, I came across an inspiring article from the Washington Times, “Couple Uses Laughter, Love in Fight With ALS.” The following captures the importance of humor and positive outlook for ALS patients and their families: “The amazing thing about Kip? He can’t move a thing on his body. He can’t speak. He can’t breathe on his own. But he’s still so funny—so funny and charming. ALS isn’t an immediate death sentence. Your quality of life can still be fulfilling with this disease.”

In another example, ALS Worldwide published a post, “Humor Is a Great Medicine”, Ken, an ALS patient, stated, “As I continue to fight this ugly disease, joking helps me get through the most challenging times. It is critically important to keep a sense of humor when you have ALS. There are funny and absurd moments to be found every day if you are aware. Humor is what helps me stay positive and it makes this disease more bearable.”

In retrospect, I realize that Brian was being himself despite his disease—a person with a good sense of humor who could laugh at himself or the absurdity of a situation (unfortunately, ALS provides plenty of material). I, on the other hand, never found anything remotely funny about caregiving or ALS. In reference to my earlier anecdote about the epitaphs, I remember feeling appalled and disapproving that Brian and Joyce were engaging in what I perceived as morbid behavior. In reality, I think everyone finds their own way to cope with challenging, life-altering situations; some use humor like Brian, whereas others use escapism and denial, like me. Reflecting back, I wish I had seen the bigger picture, so we could have shared some laughs together, as we did when Brian was healthy. Only now do I acknowledge that humor can be a positive attribute and coping mechanism when facing terminal illness and mortality. It makes us human and allows us to live life to the fullest.

Have you and your loved one shared any funny moments? How do you feel about humor in the context of terminal illness? Please share your experiences and thoughts in the comments section. Let’s continue the conversation.

Idelle thumbnail

Idelle Winer was primary caregiver to her husband Brian, who passed away in 2010 after a 5-year battle with ALS. A retired medical publishing professional, Idelle offers her unique perspective on a variety of caregiving topics in our feature Caregiver Confidential.

Behind the Ice Bucket Challenge: How My Own Fight Against ALS Helped Fuel a Phenomenon

Powerful blog post from our friend Pat Quinn, courtesy of WebMD.

By Pat Quinn

When you’re diagnosed with a disease that has a life expectancy of 2-5 years, you will do anything to change that. Almost 5 years ago, I was stunned as I heard my doctor say, “It’s conclusive, we can confidently diagnose you with ALS.” It was the most surreal moment of my life. Sure, I had had some crazy twitching in my arms. Yes, my hands had become weak. But, 2-5 years to live? No, that was unacceptable to me. I was only 30 years old! So, after the initial shock wore off, I decided that I was going to fight.

The disease that was supposed to take my strength started turning me into a pit bull. I became obsessed with bringing attention to ALS – local events, community outreach, speaking, networking – I did everything I could think of to make people aware. I can’t lie – I had selfish motives. I wanted to live. I was entering what should have been the prime of my life – I should have been focusing on the joys of finding love and starting a family, not fighting a terminal disease! My fight to add years to my life grew into a passion to fight not just for myself, but for everyone with ALS. That passion got stronger every time I met a new ALS patient. One of those patients was Pete Frates in Boston, whose website I happened to come across in my online searches. Pete was young, a stellar athlete, and diagnosed with ALS a year before me. I emailed, he answered, and soon we formed a special bond that would eventually play a major role in the biggest movement in the history of philanthropy: the ALS Ice Bucket Challenge.

You may not know it, but the Ice Bucket Challenge wasn’t originally created specifically for ALS. It was going around for a number of different causes until one golfer in Florida took the challenge for his cousin’s husband who had ALS. It caught on in that patient’s town, and the power of social media brought it to my attention. (Thank you Facebook!) I will never forget the day I saw a friend from grade school take the challenge and said he was doing it in my honor. I was blown away, but more importantly, it clicked: If this can connect so easily from one individual to another, we might have something here. Excited about the possibilities, I got my closest friends to dump buckets of ice water over their heads and it spread throughout my network – the one I had so worked so hard to build after diagnosis. Pete took the challenge, too, and shared it to his huge network. No one could have imagined what would unfold next. Soon we were right in the middle of a social media phenomenon! Hundreds of likes on every Facebook post. New videos were being uploaded every couple seconds – literally, seconds! It’s one thing to see your family and friends take the challenge. But somehow, every day it got bigger and bigger! The support for ALS I was hoping to create was taking place right before my eyes. As it happened, my head was spinning. It was surreal. Within a week, while you were probably filming your Ice Bucket Challenge in the backyard, so were the biggest athletes, famous celebrities, and even world leaders.

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Taking the ALS Ice Bucket Challenge in 2015

A worldwide movement like the Ice Bucket Challenge doesn’t just happen. It’s not like any of us were sitting in a dark room drawing up blueprints to orchestrate a massive social media movement. I believe that certain things in your life happen for a reason. Not everything, of course (I’m not crazy), but I truly believe parts of your time here on earth happen for a reason. Life presents us with opportunities, and then it’s up to us to make something happen.

Do I believe I was diagnosed with ALS for a reason? YES. Without getting too spiritual, I know God chose me to take on ALS. I have to believe this. It keeps me going. I was young. I was strong. The people I’ve met throughout my life would do anything for me. That kind of network would have the ability to create necessary awareness.

Do I think the Ice Bucket Challenge started in a town 10 to 15 minutes away from where I lived for a reason? YES.

Do I think I reached out to Pete Frates after diagnosis, became friends, eventually leading us to catapult the Ice Bucket Challenge for a reason? YES. There is no doubt in my mind that we met each other to change the course of ALS. It was our job to recognize its potential and make something happen…that’s exactly what we did!

These days, there’s no awkward pause after I tell someone I have ALS. I no longer have to follow up with “you know, Lou Gehrig’s disease,” because people are aware – and that was the reason for it all. The Ice Bucket Challenge allowed me to accomplish what I set out to do: create unparalleled awareness leading to research that will one day make ALS treatable and, God-willing, cured.

I would do anything to go back to being my normal, pre-ALS self. Anything. But I can’t dwell on that. I am facing an undefeated opponent, and I’m going to do everything I can to beat it. I know that ALS may one day take my physical life, but my fight will last forever.

PQPat Quinn was diagnosed with ALS (Amyotrophic Lateral Sclerosis) in 2013, a month after his 30th birthday. Since then, Pat has become a strong advocate, explaining to others that having no effective treatments or a cure is not acceptable Pat was at the forefront of the ALS Ice Bucket Challenge, which raised over $ 220,000,000 globally. He has spoken before Congress, at companies like Google and Facebook, and at universities across the country. Pat has received many accolades for his advocacy including a nomination for TIME Magazine Person of the Year. To learn more, please visit www.q4tw.com.

Remembering and Reflecting: Creating a Keepsake for your Family

As one faces a terminal illness, such as ALS, it can be rewarding and fulfilling to review one’s life journey and reminisce about favorite people, experiences, and events, for remembering and reflecting on your life, in order to help you celebrate your successes, cherish your loved ones, and honor your journey.  It is also important to reconcile or accommodate difficult or painful memories or events, providing an opportunity to forgive yourself and others if appropriate.  Especially during this time of year when we are celebrating holidays and are with family and friends, projects such as the ones below can make very meaningful gifts, not only to those you love, but also as a gift to yourself.

Life Review Timeline:  Place the major events of your life on a timeline. You may need several pieces of paper taped together or a large poster board to accommodate the length of your timeline. Include your successes, special dates, important events, and favorite memories. If you are willing, allow other family members and friends to contribute their memories of you, too. This activity allows you to acknowledge your accomplishments, reflect on troublesome or painful events, and recognize special people or times throughout your life. This timeline, created by you, will also serve as a wonderful keepsake for your family.

Count Your Blessings:  Make a list of all the things you are thankful for. such as loved ones, friends, possessions, special events, honors, successes, and abilities. Whenever you need a “pick me up,” read through your list and reflect on all the things that you are thankful for. Another option is to keep a “Blessings Journal” by writing five things you are thankful for at the end of each day. Some days your lists may look the same; other days you may find new treasures to be thankful for. By focusing and considering all these positive influences, you are celebrating the life you have created and honoring the blessings that have been given to you.

Messages to Loved Ones:  This activity can take several forms, all of which provide your loved ones with tangible messages from you for the future.

  • Video taped messages: Have someone video tape you as you record messages to your loved ones, to be viewed at a later date. These messages could be your thoughts and wishes on special occasions, such as birthdays, graduations, or weddings, or they could be general communications that you would like your loved ones to be able to replay and share in the future.
  • Voice recorded messages: You can also record a verbal message, without the visuals of the video, for your loved ones under the same guidelines as above.
  • Written letters: Write or type letters to loved ones expressing your thoughts and wishes. You can also dictate your letter to a trusted friend or family member, if you are unable to write. These letters can be sealed and dated to be opened at a time of your designation.

All About Me:  Record answers to questions. That can be used as queries or for a starting point for discussing your life, your dreams, your ambitions, and your reflections with your loved ones. Have someone record or write down your responses to create a treasured collection of your responses.

  • Who was your best friend in childhood?
  • Where did you live when you first moved out of your parents’ house on your own?
  • Of all the cars you have owned, which was your favorite and why?
  • What is your favorite book?
  • What is your favorite song?
  • What is your favorite movie?
  • What is your favorite food?
  • How did you meet your spouse/partner
    • Talk about your dating/courtship.
    • Who proposed to whom and how?
  • What was your first job
  • If you could have been in any occupation, what would it have been and why?
  • When you were a child, what did you want to be when you grew up?
  • What quality do you think is most important in a friend?
  • What is your favorite attribute or ability in yourself?
  • What was the scariest moment in your life?
  • What was the funniest thing to ever happen to you?
  • What was the saddest moment or event in your life?
  • What was the most unusual thing that ever happened to you?
  • If you were stranded on a desert island, which three things or people would you want to have with you?
  • If you could change one thing that you did in your life, what would it be?
  • What are you proudest of?
  • What is your favorite hobby and why?
  • What was your favorite subject in school and why?
  • Name three people who influenced your life the most
  • Which teacher had the biggest impact on you and why?
  • If your life story was made into a movie, who would portray you?
  • If a genie could grant you three wishes, what would they be?
  • What is your lucky number?
  • Describe your greatest adventure
  • Talk about a time when you were awestruck
  • What was your favorite thing about your mother?
  • What was your favorite thing about your father?
  • What was the nicest thing someone ever did for you?
  • What makes you laugh?
  • Name three people who you admire and why

Journaling:  Keep a journal of your thoughts, concerns, wishes, and feelings. Journaling can be a cathartic way of expressing emotions and releasing pent-up feelings. It can also serve as a process of self-reflection, soul-searching, and life celebration. Don’t over think what you are writing; allow your thoughts to flow onto the paper without editing or critiquing yourself. This process will allow you to address issues that are concerning you and work through difficult or unknown problems. It will also allow you to document what is important and meaningful to you.

We want to know what has helped you.  Please let us know in the comments if you have tried any of these ideas for reflecting or if you have found something else that helps you.

Caregiver Confidential Goes Monthly

In my first 3 posts, I related my family’s journey with ALS, hoping that other caregivers would connect with some aspects of my experience. Going forward, Caregiver Confidential will be a monthly forum for caregivers to share ideas and the unique experiences and perspectives of caring for a loved one with ALS.

But first, let me describe how my daughter Leah and I coped after my husband Brian passed away in 2010. Although the first year as a widow was a huge life adjustment, I still had a semblance of my pre-ALS life. Leah was living at home, working, and applying to medical school. I enjoyed being part of her journey into medicine, and it kept me mentally occupied. Then the bottom fell out—Leah left to attend medical school in Philadelphia in July 2011, which was a painfully difficult adjustment and the point at which I was forced to confront that I was now alone, as a single person, after a 36-year marriage. Slowly, I have regained my footing, but I also have had considerable time to ruminate about my experience as a caregiver.

So, what did I learn from being a caregiver to a loved one with ALS? Here’s a brief partial list (I will go into greater length on some of these topics in future posts):

  1. Adopt the sentiment, “Today is the best day of your life.” While there is always hope even in terminal illness, try to live with your loved one in the moment, because you do not know what tomorrow will bring. (This mindset runs counter-intuitive to the saying, “Tomorrow will be a better day.”) Maybe go on that once-in-a-lifetime vacation, for example, to create memories.
  2. Be flexible in your approach to caregiving and do not have any expectations. So much about ALS is out of your control.
  3. As the primary caregiver, take care of your own health and needs. Do not be afraid to ask for help or think it reflects poorly on you if you cannot manage all of the caregiving duties. A break from caregiving will make you a better, more patient caregiver.
  4. Forgive yourself for having bad moments with your loved one. It is okay to feel angry, overwhelmed, and resentful at times. Being a caregiver to a loved one with ALS may be the most challenging job you will ever have.
  5. Seek out professional help, be it a counselor, clergy, and/or social worker. Getting another perspective or talking to a professional can be very therapeutic.
  6. Remember that the ALS Association is a wonderful resource and partner for dealing with many aspects of ALS. I can attest from personal experience that Beth Barrett of the St. Louis office was key to many of our family’s caregiving decisions.

What lessons have you learned as a caregiver? What suggestions would you pass along to other caregivers? Please share your experiences in the comments section. Let’s continue the conversation.

Idelle Winer

Idelle Winer was primary caregiver to her husband Brian, who passed away in 2010 after a 5-year battle with ALS. A retired medical publishing professional, Idelle offers her unique perspective on a variety of caregiving topics in our feature Caregiver Confidential.

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