It was another cold winter in Wisconsin, and Larry was outside with his wife, Rachel, in the deep February snow that typically coats the region at that time of year. But he noticed something unusual. The distinct footprints that trailed behind them seemed different this time. His wife’s tracks were connected on one side, a sign that her foot was dragging behind her.
Around the same time, they went to watch their son play basketball. Larry bound up the bleachers to reach the top, but when he turned, Rachel wasn’t there. Puzzled, he looked around and noticed that she’d fallen. She’d been falling a lot, she admitted to him later.
Slowly, Rachel became less engaged in conversations and more apathetic to what was going on around her. Initially, they’d thought she may be depressed, but it soon seemed to be a sign of something larger affecting her cognitive function, as she also began losing her ability to do more than one thing at a time.
“She used to be able to cook multiple meals at the same time,” Larry remembers. But suddenly, “cooking a meal with multiple things at once, there was no way that that was going to happen.”
An initial diagnosis came back as Parkinson’s, but they were suspicious that it wasn’t quite right based on her symptoms. They then tried the Mayo Clinic, which put her through a large variety of tests. Based on those results, Rachel received a new diagnosis: frontotemporal dementia.
Still, Larry wasn’t convinced they had the full picture of what was going on. He kept digging and found a study at UCSF for people with symptoms similar to Rachel’s. After flying out to San Francisco, they finally received a diagnosis that told them more specifically what was causing Rachel’s changes: progressive supranuclear palsy (PSP).
PSP is characterized by a variety of symptoms, not all of which everyone has. Balance, memory issues, apathy, losing executive function and losing the ability to multitask can all be symptoms of the rare disease.
Larry was initially relieved to hear the doctors tell his wife, “If you eat well and exercise, you’ll do well,” but he realized upon going home that night that, “what the doctor really meant is that she’ll still get worse.” After doing his own research and asking the doctor for a more realistic prognosis, he finally came to terms with what was to come: Rachel would soon lose the ability to walk, her apathy would get worse and she would lose her ability to swallow. With no cure for PSP, only treatments to help some of the symptoms, it was a difficult pill to swallow.
However, receiving a diagnosis “helped to know that something was going on.” Before the diagnosis, he and his kids had been frustrated with some of her behaviors. While she’d become more apathetic in her conversations, she’d also become more controlling in her actions. With teenage children, it had been a struggle and caused a rift.
But after receiving the diagnosis, Larry gathered with his kids, and they decided they must forgive. There was something bigger at play.
“The pandemic actually helped because we were all home,” Larry shares. “It’s taken some time, but there is now some acceptance.”
One thing that he wishes more people realized about rare disease diagnoses is that “it’s more than just the person with the disease.”
“It affects the family. It affects friends. It’s not just a disease, it’s a conglomeration of decisions and never ending surprises ... It’s just a tangled web of emotions.”
Less than two years after receiving her diagnosis, Rachel passed away from PSP in January 2021. The months leading up had been difficult. Rachel had to be moved to a memory care facility and was unable to leave because of COVID-19 precautions. Her Amazon Echo Show was a big blessing to the family during that time. The device, which allows for video calls, followed her in the room, so she didn’t have to find it. It allowed the family to connect with her in those final months, even as her cognitive functions faded.
“I miss her so much but know she is in a better place eating cookies, talking walks, playing piano, and playing Cribbage and Yahtzee with her dad,” Larry said.
To posthumously honor his wife, Larry has donated Rachel’s brain to science and passed over her de-identified medical records to researchers through AllStripes. “If we can help someone down the road, it’s worth doing,” Larry says proudly.
Thanks to Rachel, Larry and their entire family, Rachel’s memory will be carried on and help many more people in the PSP community to come.