About Randy’s Diagnoses

Bulbar-onset amyotrophic lateral sclerosis (ALS)

  • A progressive, incurable, and fatal disease

  • The average life expectancy of people with ALS is 1.5 - 2 years

  • It expresses itself differently in each person

  • It is more commonly known for starting out in the limbs; however, Randy’s type has started out by affecting his speech, swallowing, and cognitive functions. 

Frontotemporal dementia (FTD), commonly associated with ALS

  • A type of dementia that affects Randy's decision-making, emotional control, memory, and social behaviors. 

  • More common with bulbar-onset ALS

Health Management

If you are feeling unwell, particularly with cold or flu symptoms, it is crucial that you inform us and reschedule your visit until you are fully recovered. Due to Randy’s ALS, he has an increased risk of respiratory complications, so even minor infections like colds can pose a significant risk.

If you notice that his nose is dripping, it’s not a big deal… just let him know and maybe offer him a Kleenex. Because of his FTD, he doesn’t always realize when his nose is running.

When he coughs or even chokes when eating or drinking, do not be alarmed. This is what he has to do to clear foods and liquids from his windpipe. 

  • Smile and compliment strong coughs. If he sounds kind of wet and rattly, you can help by encouraging him to give another stronger cough

  • You can even demonstrate by coughing yourself or clearing your throat loudly. Because he is losing sensation, he sometimes doesn’t realize when he has stuff going down his windpipe, so we’ve been asked to help him remember to clear his throat.

Safety

Because the right frontotemporal lobe controls your ability to make safe judgments, Randy may have trouble catching errors or recognizing dangerous situations. 

For activities with potential risks, like cooking, Randy benefits from a "buddy system." Act as his cognitive buddy, providing an extra set of eyes and ears to look out for things (like flammable objects near an open flame, or needing to open the door to the deck if the smoke alarm goes off, etc.) 

If you are doing physical activities together, please keep an eye out for signs of fatigue such as slowing down or heavy breathing, and suggest you take breaks as needed. ALS often leads to rapid fatigue and reduced stamina, so one of his primary goals is to conserve energy. 

Randy’s doctors have advised that he should not drive. For his safety and the safety of others, please offer to drive or arrange for someone else to drive when outings are planned.

Behavioral Changes

Apathy: A lack of emotional reaction/response is just part of his FTD. Randy is still the sweetheart that we all know and love. If he seems lacking in responding to other people’s emotions, know that he still cares very much about his friends and family, even if he isn’t as expressive as he once was.

Agitation and Impatience: Randy can become more easily agitated or impatient, especially when he gets confused or doesn’t understand why he’s not allowed to do something. 

Language and Memory: FTD with Bulbar ALS affects Randy’s ability to process language. Please be extra patient, as Randy may not remember previous instructions or conversations. Break down information into manageable parts, and repeat them if necessary. If he repeats a previous question, try to answer as though it is the first time (because for him, it is!). 

Stubbornness and Impulsivity: This is common in FTD associated with ALS. Randy may get fixated on something that is not helpful or even harmful to him. Here are some approaches that can help:

  • Validation and Reassurance: Recognize Randy’s concerns and his feelings—a simple acknowledgement like "I see this is really important to you" can make him feel heard. Following up with reassurance about how you or someone else are going to handle these concerns (even if it’s not right now) might help alleviate some of his anxieties.

  • Distraction and Redirection: Redirect Randy's attention towards activities he enjoys or topics that interest him. This could be discussing a favorite memory, engaging in hobbies like puzzles, or looking at photo albums.

  • Limit Choices: Too many options can overwhelm him and exacerbate fixative behavior. Simple, clear choices or structured decisions are helpful, such as deciding between two activities he enjoys.

  • Remove Triggers: If certain objects or images are triggering this behavior, they can be temporarily removed from his environment or new items can be introduced that capture his interest.

Communication

Randy is learning to use a text-to-speech app called “Speech Assistant AAC” to help when people can’t understand him or when he gets tired while speaking. 

Please be patient and allow him extra time to express himself, especially during group conversations.

If you cannot understand him, feel free to ask him to use his Speech app to communicate. We are trying to encourage him to practice using it and normalize its use, so that he is very comfortable using it by the time he completely loses his ability to speak.

Daily Routines

We’ve been told that routines and consistency will help Randy feel at ease and reduce anxiety, especially as his FTD progresses. 

Randy is aiming to be awake each day by 8AM and in bed by 11PM. We do not expect you to keep those hours, but we would appreciate your help in encouraging him as he tries to keep to that schedule.

We appreciate your help in maintaining a calming environment for Randy. We’ve been told that having familiar objects around, reducing clutter, and keeping important objects in the same place will help avoid confusion. That’s already a challenge for us to maintain on our own, so we would appreciate any assistance you can give us with that.

Randy’s favorite cognitive exercise is putting together jigsaw puzzles. He likes doing the small wooden puzzles on his own, but he loves when people join in on the big puzzles with him. 

  • He finds it helpful when people help him collect all the edge pieces for him to put together, or sort the puzzle pieces by color/pattern.

  • He gets really intimidated at the beginning of a large puzzle and will even have a tendency to give up if he “doesn’t feel like he’s making progress,” but that’s because his FTD affects his executive functioning skills and he gets easily overwhelmed. Once you get things started and break things down into smaller, more manageable sections (like by sorting things into colors for him), he can actually put together really difficult patterns.  

  • If he gets stuck, offer to switch sections with him, or offer him pieces that you know go in the section he’s trying to solve.

  • With the larger puzzles, he likes to solve side-by-side, but he often likes to put in the last 30-ish pieces on his own. However, he loves having witnesses nearby when he puts in the very last piece. :)

 

Thank you for helping us care for Randy—your visits truly bring him so much joy! 💖