A Journey Without a Map: Facing Frontotemporal Dementia

Popular ideas about the symptoms of dementia or Alzheimer’s conjure up images of forgetfulness, a person lost in a familiar place or unable to recognize loved ones.

This is not what it looked like when Mary VanNortwick’s husband of 20 years, Chris, started acting strangely. “If he was going to make a tuna salad, he would go buy the tuna, come home, then go to the store for the mayo; shortly after, I would see him grabbing his keys to go get the bread.”

She didn’t necessarily conclude that anything was wrong. “It was a very elusive situation. You question yourself, the other person, wonder if you are just wound too tight.”

In fact, when she took him for testing, she simply believed Chris needed some basic training in life skills like learning how to take care of his money or why he shouldn’t take phone calls from solicitors — and why he shouldn’t urinate in the neighbor’s planter. His filters on appropriate behaviors seemed to be waning.

But her husband did not need life skills. In November 2013, he was diagnosed with frontotemporal dementia (FTD). If that wasn’t enough, another evaluation five months later revealed that he also had amyotrophic lateral sclerosis (ALS). Both FTD and ALS are neurodegenerative disorders with symptoms that tend to overlap and even accelerate one another.

Mary knew something about ALS, nothing about FTD. She started reading. The disease damages the tissue in the part of the brain that regulates impulse control, planning and judgment, emotions, speech and movement. The early symptoms of FTD show up as unusual behavior. These changes start slowly, and usually show up when people are in their 50s or early 60s. No cures or treatments exist for either condition.

One Day At a Time: Dementia, Decline

Needless to say, the prognosis stunned Mary, Chris and their two daughters. “It’s just not the course you thought you were going to be on,” she says.

Yet knowing what was going on helped explain what Mary was witnessing. She saw Chris losing motor skills and depth perception. He was “walking into doorways, not picking his foot up all the way, dropping things. And then there was the perseveration, when he latches onto something and won’t let it go. Not changing a pattern or getting off a thought.”

In a moment of lucidity, Chris told his wife, “I’m going to die.”

Mary says, “What can I say? Both conditions have 2-5 year lifespans, 1-3 years with both, our care providers say. So we’re just journeying through it now, taking one day at a time.”

For Mary, this means watching the two neurological diseases wreaking havoc on her husband, whose symptoms seem to leapfrog between two conditions. “We’re starting to see decline in the cells that carry language ability. In there behind his eyes, we know there is a thought to convey, but there isn’t the vocabulary to say it, the physical ability to speak. For instance, if talking about something in the holiday category, he’ll say Easter and mean Christmas. And now with ALS it’s hard for him to speak at all.”

As the diseases progress, Chris is often not in reality, but still aware. “It’s so weird,” Mary says, trying to explain. “One day, he looked at his tongue and his hands, said his nerves felt like fireflies under his skin in his cells where ALS was taking hold.”

Since Chris can no longer be alone, Mary relies on companion care to help.

Hunger for a Roadmap

The biggest struggle for Mary has been the unpredictability of how the disease will manifest. She explains:

You’re just watching this slow progression. You try to accommodate and stay one step ahead. And then it’s some other decline. There’s nothing that can tell me what will happen. And I understand; this is just life. It’s unfolding. It comes to us this way. But I hunger for a roadmap. You can prepare for anything. But not knowing what will happen? That’s the biggest strain.

Finding Support for FTD

While support groups exist for Alzheimer’s and ALS, none exist for FTD in Central Virginia, as it is a much rarer disease.

So Mary attends a support group for dementia and Alzheimer’s. “People hear dementia and think Alzheimer’s, but it’s very different. My husband’s brain is dying, much like circuitry — flickering neurons in his brain. Circuitry is firing, firing dimly, then not firing at all. It goes on and off all day long.”

Even though the conditions don’t present the same way, Mary says enough similarities exist to make the group worth her while. “The underpinnings are common: we’re all making adjustments. Helping them still live meaningful lives in whatever capacity they can. We have to pare things down and streamline communication to be better understood.”

It was at one of these meetings that Mary got advice she finds most valuable:

A new friend said to me, ‘Your job is to keep him safe. If he’s making you crazy because he has to do something one way and only that way, it doesn’t matter if it doesn’t matter.’ This advice has saved me. Makes me able to adjust to the decline. This is maddening, frustrating, but it doesn’t matter. It’s okay. That would have robbed me of all my wellness.

UVA Neurology

And at UVA, Mary found a team she calls “amazing” and “fantastic” in their efforts to help her prepare for the challenges ahead. “Of course, they provide excellent care. But they provide equally great personal care.” She gives examples of the warm nurses and front-desk receptionist who make their visits seamless.

“And at the beginning, Dr. Ted Burns said, ‘Tell me how your husband does life.’ I said, ‘The cup runneth over. Never just half full.’ And Burns said, ‘Then let him do ALS that way. Now, how do you do it.?’ I said, ‘Batter up.’ ‘So if you need information, get it,’ Burns said. ‘Talk to people. Do this the same way you do everything.’

Self-Care

A wellness professional, Mary knows the value of taking care of herself so that she can take care of Chris. “I go to bed, I get my sleep, I walk, I eat super well. I do things I enjoy, so when I’m with him I’m with him, fully present. If you’re not well, you’re not there, and nobody wins. So I try to make it first things first.”

A go-with-the-flow approach has helped Mary manage the constantly shifting landscape of her husband’s illnesses:

It’s a challenge and source of joy: Leaving as much in place as possible until it has to be taken away. The emphasis is on the afflicted as much as the caregiver. We can’t make their world prematurely small so that we can handle it better. I believe the best adjustment is one made from both ends at the same time giving consideration to all involved, preparing for the end while savoring the journey.

The Gift of Humor

Mary, Chris and their daughters have found using humor to sometimes be the best medicine when dealing with symptoms like delayed hearing, word-processing and reaction time.

“This delay can be as much as 10-20 seconds, and it’s definitely increasing. We simply added, “Wait for it. Wait for it…”. Everyone laughs, including Chris. And there’s the misinterpretation of instruction. I’ll ask him to fill the dog’s bowl with dry food and he’ll get out the milk. Again, humor is invoked. “Really, Chris? Really?” He laughs and we start over. It’s impossible to respond with humor every time, but when we can pull it off, it never fails to make it better for everyone.”

When Mary talks about Chris, her voice lilts with respect and pride. “He lives to serve other human beings,” she says. Even now, he volunteers, and she is grateful for the people he works with. “They’re trying to keep him engaged. He’s very valued. “

On top of that, Mary tells us that when he dies, Chris wants to donate his brain to FTD research at UVA.

As for herself, Mary faces the future with an unflinching bravery that also includes helping others. “I want to start a support group for FTD,” she says. “And bring people together to talk, especially since what’s really hard is how this disease manifests differently and at a different pace for everyone.”

Mary encourages others to reach out, and continues to do so in the lives of others around her. She speaks reassuringly: “Don’t face FTD alone.”

Get Help

Read a book: Mary recommends “The 36-Hour Day” by Mace and Rabins and “Dementia” by Susan Kiser.

Find online support from the national Association for Frontotemporal Degeneration.

Learn more about UVA’s expertise with dementia and ALS.