By Lori Ruhlman

When my husband Tom was diagnosed with ALS, we were embraced by the ALS community. But as his health declined, I didn’t really feel ALS was what was stealing my husband from me. I felt more connected to people losing loved ones to frontotemporal dementia, also known as FTD. The recent article by the Association about the connection between ALS and FTD has helped bring me full circle.  I DID lose my husband to ALS… a particularly cruel and nasty form of ALS that includes FTD.

Here is MY ALS story.

When hundreds of people streamed into our local park six years ago to do a community ALS Ice Bucket Challenge (with my husband Tom as one of the central local ALS honorees), I was astonished and touched. I also knew it would take me years to fully digest the magnitude of the event.

I was right. Today, the kindnesses shown that day make me cry with gratitude. I feel the icy water of the challenge and the incredible warmth of that giant community hug. And I remember the internal conflict I felt.

I had been hesitant when friends wanted to organize the day in honor of Tom. I was a reluctant participant and, honestly, a very weak advocate for ALS. When it came time to thank the crowd, I handed the megaphone to our son.

The reason: Tom wasn’t a typical sufferer of ALS. I struggled with even calling his disease ALS, because the harder and scarier truth was that something was stealing his personality, thoughts, and intellect much faster than ALS seemed to be weakening his muscles. 

I suspected this even at the very beginning of his ALS diagnosis. The symptoms were not just physical. He was subtly different. And even as this became increasingly pronounced and obvious to me, it seemed I was alone and shouting into the dark. At the ALS Clinic in Syracuse, with a renowned ALS doctor, the focus was only on the slight weaking of his hands and the decline of his lung function. Tom was on the “slow track” of ALS, and might have ten full years to live, his doctor said. Tom clung to this, announcing it proudly: “I have ALS, but I am on the slow track.” He couldn’t sense the other changes in himself that were keeping me awake at night.

Each visit to the clinic, I tried to express worry over subtle changes I was seeing: the repetitive coined comments he made to everyone we saw on our nightly walks; his changing work ethic; his growing lack of empathy. He was missing appointments and making embarrassing jokes. But it was so subtle and hard to define. I didn’t want to say all of this in front of him at a doctor’s appointment. I didn’t want to hurt his feelings, make him defensive, or make him self-conscious, so I handed the health care providers my written observations.

He was friendly and jovial with the people in the clinic. He loved going there for his monthly appointments. He would walk down the hallway as though he was a celebrity, stopping inappropriately inside other exam rooms to say hello to the nurses and doctors. He wasn’t like any of the other patients. When his doctor suggested Tom needn’t rush to sell his business because he would likely have several if not many years to be productive, I cringed. I knew Tom’s sharp business acumen was rapidly fading.

Researching at night when he was sleeping, I eventually found the answer myself. Frontotemporal degeneration or frontotemporal dementia (FTD).

I read the symptoms (the exact same list recently published in an article by the Association):

• 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

Tom fit almost every one. My heart sunk, and yet I was also relieved. Finally, an answer. I knew what was happening to the man I loved. It wasn’t “only” ALS. Was it ALS at all?

Back then (2007-08), doctors and scientists were learning of the link between FTD and ALS, but it was not often discussed. A neuropsychologist finally said the words that those late-night Google searches had already told me. 

Research is increasingly showing the link between ALS and FTD, and the recent article by the Association has welcomed me “home.” The Association’s article says this:

“An ALS diagnosis is a devastating diagnosis in and of itself, but to learn your loved one has FTD as well can make it even more challenging to comprehend.”

Now we know that 10 to 15 percent of people with FTD also experience motor neuron degeneration, meaning they have FTD-MND; and that some people with ALS will experience cognitive decline and even frontotemporal degeneration, called ALS-FTD. 

Although my husband was first diagnosed with ALS, I now believe he experienced symptoms of FTD long before our journey took us to neurology departments seeking the reasons behind his whole-body fasciculations.  

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In the years he declined, and in the years since his death, I have come to know the journeys of many others affected by “just” FTD or by combined ALS-FTD. And I know that the “unknowing” is often the most painful period, leading to the biggest tragedies and the greatest angst. FTD often leads to destructive behaviors, job loss, financial ruin, divorce, and heartache. (We were “luckier” than some others in that we didn’t face most of those things). What we did face was the unknowing.

The extended period of unknowing faced by most who experience this disease is agony.  And it wastes time during a critical period as the affected person slips away. By the time you know what it is, by the time you recognize what is happening, it is too late. It is too late to have meaningful conversations like you might have with “only” ALS. It is too late to discuss end of life options. It is too late to say goodbye to the whole person. 

Now, thanks to ongoing work by scientists, the Association can stress “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.” 

By the time our community gathered in September of 2014, Tom’s FTD prevented him from taking in all that kindness or expressing his gratitude. And the FTD prevented me from being an eloquent and grateful spokesperson for The ALS Association. We didn’t “fit in” with ALS and shifted focus to the AFTD, which addresses the biggest losses our family endured.

Now, thanks to ongoing research and communications, I could (and can and will) stand up with a megaphone to discuss the horrors of ALS, which can steal a person’s ability to think, communicate and feel, as surely as it steals their ability to walk, talk and breathe.

Special thanks to Lori Ruhlman for sharing her family’s story and their personal journey with ALS. To learn more about ALS and FTD, read our blog here. To learn more about what The ALS Association is doing to better understand cognitive changes and ALS, visit our website here.