Don and Claire Bratcher: Imagining a World without ALS

ALS Association volunteers are a special breed of superhero — they are the heart and soul of this Chapter. They give up countless hours of their personal time to provide us with all manner of help – in the office, in the homes of people living with ALS, on our board of directors, as public policy advocates, on our committees, at our special events – without asking for anything in return.  

So many of our volunteers have been personally impacted by ALS, and have seen up close and personal the devastation the disease brings upon a family.  These wonderful people become volunteers to fight for a cure and to ensure that other families are supported physically and emotionally on their disease journey.

Two of those special volunteers are Don and Claire Bratcher, who have been ALS warriors and volunteers for close to 15 years.  Claire recently agreed to share their story and we’re delighted to post it here in her own words. 


And so my story begins. In 1990, at age 80, my dad, Larry Boberschmidt, Sr., was diagnosed with spinal muscular atrophy and died at age 82. In 1994, at age 55, my sister, Marie Serena was diagnosed with ALS and died at age 57. In 2002, at age 55 my sister, Eleanor Ecuyer, also heard the words, “You have amyotrophic lateral sclerosis (ALS)” – aka Lou Gehrig’s disease – and died at age 57.  I then realized my dad probably was misdiagnosed and also had ALS.

The most common form of ALS in the United States is “sporadic” ALS, which means that the disease can affect anyone, anywhere. Our family falls in to the other ten percent of cases – the inherited form of the disease, called “familial” ALS.  Families members who fall into this category have a fifty percent chance that they will inherit the gene mutation and may develop the disease.

 

Familoy members
Family members we celebrate.

Lou Gehrig’s wife name was Eleanor. On July 4, 1939 at the young age of 36, Lou Gehrig announced to the crowd in attendance at Yankee Stadium who came to watch him play, that instead he was retiring from the game he loved because he had been diagnosed with ALS. My sister Eleanor died on July 4th. Coincidence? I don’t think so. Eleanor was probably the most independent person I have ever known, but when you live with ALS, this horrific disease robs you of all of your independence which was, for Eleanor, the most difficult thing about having ALS. She didn’t want to have to depend on everyone for everything. I believe by her death occurring on July 4th, it was a way she showed that she was regaining her independence.  Despite all of that, she considered this disease a gift, as it brought people together in prayer who ordinarily would not even speak to each other.

Memory Wall
Our grandson, Shane Hill, looking at our family’s photos on the memory wall at the Walk to Defeat ALS.

In 2003, I decided that I needed to do something more. I knew that I couldn’t waste any more time with negative feelings about what ALS had done to our family, but decided instead to channel those feelings into positive actions. I reached out to the St. Louis Regional Chapter and asked what I could do to help. That is when the Eleanor’s Hope team was formed and we became a part of the Walk to Defeat ALS. I still felt I needed to do more, so I became a part of the St. Louis Regional Chapter’s Walk committee. I didn’t know it at the time, but not only was I becoming a member of this committee, I was joining a fabulous group of people that I now consider my ALS family.

Eleanor's hope 1
The Eleanor’s Hope Team

The Eleanor’s Hope team continues to walk for those who can’t and will continue to be part of the Walk to Defeat ALS as long as it takes. As the song goes, “I Can Only Imagine.” I would add to that and say that my prayer is “I can only imagine a world without ALS.” I believe we are very close to realizing that dream.

Claire and Don Bratcher
Eleanor’s Hope Team Captain

If you would like to become a volunteer and join a team of warriors dedicated to fighting ALS, please visit our website to learn more about available opportunities.  

And, join us at the Springfield Walk to Defeat ALS June 16th at Southwind Park, and at the St. Louis Walk to Defeat ALS on June 23rd at Forest Park.

Caregiver Confidential: Memories

We didn’t realize we were making memories, we just knew we were having fun.
—Unknown

A few weeks ago, I was sorting through boxes of family photos in the hope of organizing and creating some photo albums. During my search, I came across photos from our trips to Mexico and Hawaii in 2006 through 2008. Although my husband Brian had been diagnosed with ALS when the photos were taken, I had fond memories of our vacations. I recalled how much Brian, our daughter Leah, and I enjoyed ourselves despite his illness. We had never been to Playa del Carmen, Puerto Vallarta, and the Hawaiian Islands, so each trip was magical and a new adventure. Although somewhat challenging to travel with Brian as his disease progressed, in retrospect I am so thankful that we created happy memories during what otherwise was a very sad period.

I can attest to the importance of spending meaningful family time that does not dwell on your loved one’s illness. When we took our 2006 trip to Mexico, our daughter Leah was unaware that Brian had ALS. Brian’s symptoms were barely noticeable to anyone but me. On our next excursion in November 2007, Brian had just retired from his law practice and had told colleagues, friends, and family about his diagnosis. Brian enjoyed our trip to Puerto Vallarta, whether the cuisine, music, or walks on the beach. Fortunately, he had only minor physical limitations.

By the time of our vacation to Hawaii in January 2008, however, we needed to be creative in our approach to vacation activities. We could not do hikes or big excursions because Brian’s balance and mobility were limited. Instead we stuck to walks on paved sidewalks along the beach, driving tours with stops, and lots of wonderful eating. We enjoyed a Luau and took an amazing whale watching tour. Simply put: We had a great time.

Hawaii vacation
Idelle, Leah, and Brian enjoying dinner in Hawaii, January 2008.

We also created some wonderful memories by hosting a family reunion for all the Winer cousins in May 2008. Initially when Brian’s sister Joyce proposed St. Louis as the reunion site, I was unsure whether it was a good idea. It turned out to be a great idea and a much-needed distraction from wheelchair shopping and home modification. Brian and I enjoyed making the hotel and restaurant reservations, hosting a welcome cocktail party at our home, and engaging in good conversation with supportive family. It was the best medicine that could have been prescribed.

My message here is simple: Create a legacy of happy memories for your entire family while you can. It does not matter where you go and what you do; it is the people you are with that matters. With ALS, there is such a small window of opportunity to make memories and share meaningful time together, aspects of life that healthy people all too often take for granted. For an ALS patient, today—not tomorrow—is the best day of one’s life, and it is important to make every moment count.

How has your family created lasting memories? What activities have you enjoyed together? Please share your experiences and thoughts on this or any aspect of caregiving, current or past, 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.

The National ALS Registry

The National ALS Registry is the single largest ALS research project ever created and the only population-based registry in the U.S.  The registry collects information and demographics on people with ALS, connects patients to clinical trials, and funds ALS research.  The purpose of the registry is to learn more about who gets ALS and what causes ALS.  This will hopefully give scientists a more complete understanding of the disease and enable them to find a cure.

Get the Facts- ALS registry

The National ALS Registry also includes a Biorepository that stores samples for future research.

Biorepository

Recently, the Registry was used for the first time to recruit patients and caregivers into a landmark patient-focused drug development survey that captured important information on the burden of ALS across multiple dimensions, including: symptom experience, quality of life, health care resource utilization, and emotional well-being.  Other important advances made possible by the ALS Registry include attaining a 96 percent enrollment rate for registrants to be notified about ALS research opportunities (more than 100,000 such notifications being sent for over 30 clinical trials) and research funding for 13 institutions to examine what factors may contribute to ALS.  ATSDR recently released the third report on the results of the data gathered by the National ALS Registry.  You can read the report here.

If you are interested in joining the National ALS Registry or learning more about the latest news from the Registry, please visit https://www.cdc.gov/als/.

It is vital that the National ALS Registry continues to be fully funded.  Congress has started work on the Fiscal Year (FY) 2019 appropriations process and Representatives Eliot Engel (D-NY) and Peter King (R-NY) are circulating a bipartisan Dear Colleague letter in the House of Representatives (see it here), urging their colleagues to support $10 million in funding for the Registry.  The deadline for this letter has passed, but there is still more you can do to help make sure your Representatives are aware of the importance of issues affecting people with ALS.

Visit the ALS Association’s Advocacy Action Center to learn about the latest updates and news about legislative and regulatory issues being addressed by The ALS Association and to sign up to receive alerts when your action is needed to support people living with ALS.

Living with ALS: Keep Your World Large

By Julia Henderson-Kalb, OTD OTR/L and Elissa Held Bradford, PT, PhD, NCS

What do you want to be able to do in a day? What activities are important to you? Everyone craves activity. It is part of our human experience (1). However, oftentimes when people are diagnosed with a disease like ALS, their world tends to shrink. They might isolate themselves, stay at home the majority of the time, and stop doing the things they love to do in exchange for activities that aren’t very meaningful to them, like watching TV for hours on end. As part of the therapy team at Saint Louis University’s ALS Certified Center of Excellence, one of our priorities is to help people diagnosed with ALS understand how to keep their world LARGE so that they can enjoy meaningful activity for as long as possible.

What causes the world to shrink for someone living with ALS? It might be physical. The loss of mobility due to muscle weakness, balance issues, or low endurance can make it more difficult to get from point A to point B. Getting into and out of a car or using public transportation can be a problem. Once you arrive at your destination, maneuvering around might be challenging for many reasons-if there is a great distance to cover, the terrain isn’t smooth, or there isn’t enough space to move comfortably, to name a few. Loss of fine motor coordination and gross motor coordination can make completing daily activities more complicated.

Other causes of isolation from the world might not be as physically obvious but are no less influential. People living with ALS often experience feelings of depression, loss of control, and loss of “sense of self” when they are having difficulty or no longer able to complete daily activities in the way they once did (2). A common example is no longer going out to eat with family and friends, despite enjoying and participating in this activity frequently previously. When searching out the reason for this change, it is often embarrassment related to challenges with eating such as dropping utensils or coughing a lot. Furthermore, the loss of ability to verbally communicate with others has been found to be linked to a decrease in quality of life (3).

So what can be done to overcome these barriers and keep your world LARGE for as long as possible? There are several strategies to be considered to stay engaged in meaningful activities.

1.    Exercise/Physical Activity

While it might not be right for everyone with ALS, studies have shown that moderate-intensity exercise helps to maintain the strength you already have. If a person with ALS is not active, greater weakness can occur on top of the weakness caused by the disease itself (4). Prior to starting an exercise program, it’s important to talk with your doctor. Referral to a physical therapist or occupational therapist (either at an outpatient clinic or through home health) will ensure that the exercise program is appropriate for your needs and will avoid further damage to your muscles.

2.    Activity/Environmental Analysis

Sometimes the difference between being able to complete a beloved activity or not is simply a matter of changing the approach to how you do it. Sitting rather than standing while cooking can reduce the amount of energy it takes. Moving gardening items to easily accessible locations and creating a raised garden bed can allow you to garden with decreased risk of falling or using too much energy on the task.  An environmental assessment by an occupational or physical therapist can make your home or office safer and more manageable. An occupational therapist can observe you completing a specific task and give you recommendations on how to make the task completion easier.

3.    Equipment

There is a piece of medical equipment for just about every issue you can think of, and that includes helping you complete your desired activities despite most physical limitations. Adaptive equipment can be used to help you do anything from brush your teeth to drive a car. Insurance covers a few items, but many have to be paid for out-of-pocket. It is recommended that you discuss equipment options with an occupational or physical therapist so that you get the items that best fit your needs. The therapist might have an idea of how to contain cost by equipment by using something you already have or can put together at home. If you do need to purchase equipment, it is a good idea to search the internet for best pricing options. The ALS Association also has a loan closet that might have the piece of equipment you want, which can reduce cost.

When walking becomes difficult due to decreased strength, balance, or endurance, mobility devices offer options for moving around the home or in the community in a safe and effective manner. Devices from straight canes to power wheelchairs can make it possible for you to get to where you need to go with a reduced risk of falls and decreased expenditure of energy. It is recommended that you see a physical therapist or occupational therapist in order to determine what type of device is best for your particular need.

4.    Delegation

When in doubt, delegate it out! ALS is a disease that allows you to only have so much energy in your energy bank every day, and once it is gone, it’s gone. Choose the activities that are most important to you and then ask other people to help you with the other tasks. If you really want to go to your grandson’s baseball game but know that you only have so much energy in the day, someone else might need to fold the laundry. If you want to go to lunch with friends, you might need help getting dressed and ready for the day. Consider your body and your energy level. In most cases, your friends and family want to help in any way they can. Use that help as it is offered so that you can do the things that matter most to you. Remember it takes a village. We all have different roles to play at different times in our lives.

5.    Focus on What Matters Most

ALS is a chronic, progressive disease. It changes the way a person is able to move, talk, eat, and even breathe. But it cannot take away who you are as a person and what you mean to those who love you. What matters most is different for every individual. But knowing what that is and really focusing on it can free person from worrying about other things. For example, if time spent with friends and family is what matters most to you, slurring your speech in front of them might seem less important than not seeing them at all. If being out in nature is most important to you, then does it really matter if you walk or ride in a power chair to get there? Letting go of what you can’t do and allowing yourself to focus on doing what you love regardless of your limitations can create a greater sense of control, identity, and appreciation of life (5).

Elissa Held Bradford, and Julia Henderson-Kalb are part of the multidisciplinary care team at the ALS Certified Center of Excellence at Saint Louis University Hospital.


References:

(1) Boyt Schell, B.A., Gillen, G., & Scaffa, M.E. (2014). Willard and Spackman’s occupational therapy (12th ed) Lippincott, Williams, & Wilkins: Philadelphia, PA.

(2) Radomski, M.V. & Trombly Latham, C.A. (2014). Occupational therapy for physical dysfunction (7th ed). Lippincott, Williams, & Wilkins: Philadelphia, PA.

(3) Felgoise, S.H., Zaccheo, V., Duff, J., & Simmons, Z. (2016). Verbal communication impacts quality of life in patients with amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 17, 179–183.

(4) Bello-Haas, V.D. & Florence, J.M. (2013). Therapeutic exercise for people with amyotrophic lateral sclerosis or motor neuron disease (review). The Cochrane Collaboration: Johne Wiley & Sons, Ltd. doi: 10.1002/14651858

(5) Foley, G., O’Mahony, P., & Hardiman, O. (2007). Perceptions of quality of life in people with ALS: Effects of coping and health care. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 8(3), 164-169.

Technologies That Make Life Easier for Caregivers

By June Duncan

Assistive technologies for the disabled have come a long way since the advent of automated wheelchairs and hearing aids. Smart technology has opened a new world of possibilities for people with disabilities and those who care for them. Smartphones, tablets, and an ever-growing list of apps are helping the disabled improve mobility, communication capabilities, speech, and vision. One of the greatest benefits is the ability to foster better communication between the disabled and their caregivers. Today, technology enables disabled persons to care for themselves more effectively, which makes things easier on caregivers, who often suffer from fatigue and burnout. Technology also helps give disabled persons more independence and a greater sense of confidence and control over their own environment.

Speech

Tablets have become a valuable tool, helping people with speech disabilities communicate through apps that provide access to an extensive list of words. Augie AAC and Speak for Yourself give people access to more than 13,000 words with the tap of a finger. Both apps transform your tablet into a device for people who have autism, cerebral palsy, and other conditions that constitute a complex communications need. It even aids the development of verbal speech.

Smartphone Navigation

Smartphones today are dynamic tools for communicating and accessing information. Speech recognition technology allows people with visual impairments or learning disabilities to use smartphones. It’s an especially useful tool for people with dyslexia. It reads texts and emails out loud and even allows users to use social media sites like Instagram. Apple has been a pioneer for decades in different forms of smart technology aimed at helping disabled people live more independent lives. Its assistive touch technology helps people with motor-control problems use an iPhone or iPad without having to use their fingers in complex ways to size the screen and adjust touch-based controls (like volume control) more easily. Voice-activated systems can also help visually impaired people control their home entertainment systems.

Notification Light System

One especially useful notification system can be customized to communicate different messages to hearing-impaired individuals through a system of multicolored lights that can alert them, for example, when someone comes to the door, when it’s time to leave for school or work, or when dinner is ready. The system can be integrated with smart home-control technologies like Amazon’s Echo. There is also a two-way communication technology that can translate sign language into text for people who are not hearing impaired, a useful tool for helping caregivers and care subjects communicate.

Cognitive Challenges

Individuals with cognitive challenges and memory impairment sometimes have trouble taking care of themselves, forgetting to take medications or prepare meals. A program called Lively uses sensors to track the daily movements and behavior patterns of cognitively disabled individuals. Caregivers can log into a web page to track their care subject’s movements and activities, making sure he or she is sleeping and eating properly and performing other normal daily activities. Still more sophisticated Internet-based smart technologies allow wheelchair-bound individuals to interact with their surroundings in ways that wouldn’t be possible otherwise. Lights, blinds, thermostats, appliances, sprinklers, and even pet feeders can be controlled with a smartphone, as can door locks and home security systems.

Technology is helping improve the lives of people with many different disabilities. It’s also beneficial to caregivers who live on-site or provide care remotely, protecting loved ones who are highly vulnerable to accidents or mishaps caused by their disabilities. Communication is the key, and there are many apps and systems that can make it easier for individuals with visual and hearing impairments to communicate with their caregivers.


June Duncan is the primary caregiver to her 85-year-old mom and the co-creator of Rise Up for Caregivers, which offers support for family members and friends who have taken on the responsibility of caring for their loved ones.  She is passionate about helping and supporting other caregivers and is currently writing a book titled, The Complete Guide to Caregiving: A Daily Companion for New Senior Caregivers, due out in Winter 2018.

For more information and resources for caregivers, please visit www.alsa-stl.org.

 

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.