My experience with Ellen, Catherine and Amyotrophic Lateral Sclerosis

ALS is also called Lou Gehrig’s Disease

Are there any three words in the English language that terrorize the imagination more than Amyotrophic Lateral Sclerosis or ALS also called Lou Gehrig’s Disease? Not for me. I recall the day that I got the call from my sister’s husband that “the doctor said it looks like ALS”. My stomach churned and I started to cry.

That was a pivotal moment of clarity after months of waiting and hoping for a different diagnosis. As an Occupational Therapist I knew what she was facing and how it was likely to progress. Pete Frates (petefrates.com) had just become a celebrity of sorts as a young, talented Massachusetts college baseball player newly diagnosed with ALS. He went on to become the face of ALS and the Ice Bucket Challenge phenomenon which generously funded much needed research into ALS. Pete Frates died at age 34 at home with his family in Massachusetts.

Ellen had left arm weakness

Ellen had been complaining about muscle weakness and dropping things from her left hand for a few months. As an OT I first thought it must be “carpal tunnel syndrome”, an overuse injury from her desk job.  Then later, when it got worse we thought it must be “some other overuse injury”. 

“What the hell happened to you?”

Ellen had a difficult mammogram a few months earlier which caused her a lot of pain on the left side at the time of the test. Could the mammogram have caused a nerve injury to her arm? She underwent EMGs and the technician performing the study remarked during the test “what the hell happened to you!?” 

“It must be ALS”

Ellen was finding it difficult to do her job at a large insurance company. She  didn’t want to risk overusing  her “good” right hand while compensating for the left side weakness. She took a leave of absence from work and saw two additional neurologists for their opinions. One suggested an infusion that was daily for one week suggesting that it could be “chronic Lyme disease”.  In the end the result was “we’ve ruled out everything else…it must be ALS”. As it turns out, that is typically how the diagnosis gets made. She was 56 at the time. The progression of her disease took less than two years before she died on June 21 which is Global ALS Awareness Day.

Catherine wasn’t progressing in therapy

Several years later Catherine was admitted to the suburban Boston rehab center where I worked. She had just undergone lumbar spinal decompression surgery. She had progressive lower extremity weakness that was attributed to nerve compression. But her legs kept getting weaker after the surgery in spite of therapy. That was unexpected. What was happening?  

“Make her work harder!”

Catherine went back to her Boston neurosurgeon who ultimately performed another spinal decompression surgery, this time in the neck region because her arms were starting to weaken. Again, rehabilitation did not progress in the expected fashion. As a trained therapist I was expecting a gradual improvement in her function, sensation, strength and movement as post op pain and inflammation subsided. When I called the surgeon to report her lack of progress he said “What do you mean she can’t hold the phone or walk…. Make her work harder!” 

“It must be ALS”

Catherine’s muscles were starting to tighten, stiffen and become painful. Her frustration was intense. She underwent muscle biopsy and EMG studies as well as numerous lab tests. In the end the result was “we’ve ruled out everything else…it must be ALS”. She was 78 at the time.

What is ALS?

In researching ALS after Ellen’s diagnosis, I went to a fundraising walkathon and open house at the ALS Therapy Development Institute (als.net) in Cambridge, MA. The tour guide showed us the research lab and stated that ALS is not one discrete disease. It’s more similar to what we understand about cancer. Their research shows various presentations of the disease, just as there are various types of cancer. It’s one of the reasons it is hard to study and treat ALS.

Currently in the USA there are very few medications for ALS and they only treat symptoms.

Nothing has  been discovered to cure the disease. The decline and disease progression generally last 1.5-3 years after diagnosis.

What’s hard about living with ALS?

What’s hard about working with an ALS patient is that the decline and progression are rapid. On Monday Ellen could hold an adapted fork to feed herself, but on Wednesday she couldn’t and had to be fed.

On Tuesday she could walk with a little family assist but by Thursday she needed much more help to walk.

Other diseases have a relapsing/remitting presentation, like MS, or a stable presentation once the initial insult does its damage, like a stroke. With ALS it’s a steady, progressive and profound loss of function

What does PALS stand for? What is CALS?

The rapidity of decline in a PALS  (short for “person with ALS”) is difficult on patients’ and caregivers alike. (CALS is a Caregiver for someone with ALS.)No sooner does the patient accept and work through a functional challenge than a new one shows up and a clever compensatory strategy no longer works.

Progression of ALS

Other aspects of function are also at the mercy of ALS, not just movement and strength. There’s severe pain, contractures, loss of speech, weakened swallow, emotional lability, bedsores and more. The muscles of breathing eventually weaken causing inability to manage secretions increasing the likelihood of pneumonia.

When she got to that point my sister opted to have a feeding tube inserted directly into her stomach. The tube was used for hydration and nutrition as well as for medications. In that regard it was an important part of her treatment plan. It kept her comfortable.

Locked in

Usually brain function in ALS patients is preserved but the patient becomes “locked in” or unable to speak while still aware of the environment. Bowel and bladder control is preserved but the patient is unable to move independently to relieve themselves. 

My sister Ellen sustained a broken toe that nobody knew about for a long time. It must have happened during a difficult transfer. While being moved by someone her toe somehow got hurt. She was unable to verbalize about the pain in her toe at that point. She tried hard to make her eyes convey meaning, which was impossible to interpret. Ultimately it was the redness and swelling of the toe that indicated a fracture.

Shortly thereafter she began using a communication board with a laser pointer attached to her glasses frame. With head movement she could point to a picture or spell out a word, letter by letter. 

Living with ALS

Fortunately our family home was available to accommodate Ellen’s needs since there was a newly built accessible bathroom on the first floor.

The first floor bathroom had been designed with others in mind, for Aging in Place. The bathroom had a space saving pocket door opening, and a large walk in shower stall with a 2 inch lip. Mom graciously moved to an assisted living nearby so the family could focus on Ellen’s needs and Mom could socialize and be taken care of.

Catherine was not as fortunate and resigned herself to living in a nursing home during COVID with no family allowed in to provide support for her.

While many home adaptations were tried for Ellen and Catherine, all were short lived. The ones that had the most profound impact for staying in a two story suburban colonial home were an outdoor entry ramp, an indoor stair lift, a power lift recliner chair and a sturdy bidet with remote controls.

Durable Medical Equipment

A free aluminum ramp was found on craigslist.com and set up to run from an entry door to a covered porch right into the garage. A garage is the best destination for a ramp so that weather is not a factor when moving in and out of a car.

A stair lift was also donated which an electrician  nephew was able to run the wiring for. That enabled Ellen to stay upstairs on the second floor at night and enjoy that privacy for many months.

Privacy was a major priority for her. For both women hand splints were important in preventing deformity, maintaining function, appearance, comfort and flexibility.

A used power lift recliner was found for Ellen which she used for napping, position changes, socializing  and transfers. A high end bidet was purchased that featured warm water, warm air and a wall mounted remote control function that Ellen could activate with pressure from her left elbow.

Her daughter bought an alerting device that Ellen could keep in her pocket, attached to a tether, to get someone’s attention if she was alone in a room, unable to speak. It had to be setup in just the right position to be activated by her. 

End of life

Ellen opted not to have mechanical breathing intervention when her respiration became affected. The thought of dying by suffocation terrified her, so it was a difficult decision to make. A home hospice MD reassured her that morphine could be used at the end to manage her breathing, for a peaceful transition to death.

A hospital bed was moved into the first floor dining room. A portable Hoyer lift was brought in by hospice as was a rolling commode/shower chair.

Read my mind

It was challenging to know how to communicate meaningfully with my newly non-verbal sister. Should I chatter away non stop to fill up the silence that is inherent in one way communication?

Maybe I could read the news or a book or magazine to her or we could watch TV together. It became impossible to read body language or facial expressions once she was unable to move and speak. Her caregivers became skilled at “reading her mind” when she opted not to use a high tech communication device. 

Saying goodbye to Ellie Pooh

I realized that it was important for me to tell Ellen in a thoughtful and loving way what she meant to me, her big sister. I did an internet search about “what to say to a loved one who is dying soon”. That was SO helpful! 

My thoughts flowed and I typed my sentiments on two single pages and glued them, facing each other, inside a blue file folder, like a large greeting card. With Love to ELLie-Pooh  was printed in fancy calligraphy on the outside front cover. The file folder could then be opened and propped up in front of her on a pillow so she could read it, when she wanted to as long as someone set it up for her. Doing that helped me tremendously. It served to acknowledge what we knew was inevitable rather than pretending to be cheerful and falsely hopeful.  I have no way of knowing what it meant to her.

Catherine has ALS

The experience of living through ALS with Ellen at home helped me to befriend Catherine a few years later as she lived through ALS in a nursing facility. No other staff person in that 160 bed facility had experienced ALS or understood the disease process. I became Catherine’s caregiver/advocate as her voice weakened and COVID restrictions precluded visitors. Her needs were sharply different from every other patient in the care facility and it became my mission to teach the staff to understand her and care about her wellbeing. 

Saying goodbye to Catherine

While writing this I thought that Catherine would like to read this essay. I had mentioned my sister’s ALS journey to her many times as we worked through her ALS challenges.  I found another blue folder that I adhered my typewritten pages to and presented it to Catherine. I wrote her name in calligraphy on the front cover and propped it up for her so she could read it when she wanted to. She learned that, for me, working with her was very meaningful.

Knowing and befriending her renewed the memory of my sweet, fragile and funny little sister Ellen. Working with Catherine gave me a way of putting my knowledge and experience to good use for the benefit of another person with ALS. 

“I’m fine, everything is OK”

Whenever I think about Catherine I’m reminded of her good cheer, her intelligence and interest in the world, her easy personality and the way she cared about others. Each time we met I started off by asking her “How’s everything?” and she proceeded to tell me whatever was on her mind. At the end of that, her final comment was always “Other than that I’m fine, everything’s OK”. She knew that would get a smile and laugh out of me, since we both knew how untrue the statement was. She was not OK, she was dying of a dread disease. 

Catherine passed away in late 2021.

Our family group continues involved with the ALS Association of Rhode Island and is committed to fund raising for Team Ellie Pooh

Check out alspathways.com for resources about living with ALS

This is a communication board. The user was unable to speak at all
A communication board that Ellen used with a laser pointer attached to her glasses
ALS Therapy Development Institute, Cambridge, Massachusetts
Representing Team Ellie Pooh of the ALS Association of Rhode Island