“Seeing is believing,” an old saying advises. Those words no longer apply for Bob Yetz.
Yetz, 71, suffers from a rare form of Alzheimer’s disease called posterior cortical atrophy, or PCA. As with other Alzheimer’s patients, Yetz’s brain tissue is deteriorating. But the damage is progressing primarily in the folds of gray tissue – the cortex – at the back of his brain. This area is responsible for processing visual information sent from the eyes to the brain.
The degeneration of the posterior cortex means that people with PCA have generally healthy eyes, but see a very different world than most of us, said Dr. Victoria Pelak, a neuro-ophthalmologist and specialist in PCA who sees Yetz and other PCA patients at the Sue Anschutz-Rodgers Eye Center on the Anschutz Medical Campus. For example, they may be unable to see objects or people directly in front of them or struggle to read because lines of text run together.
“The posterior cortex takes in all the information coming in through the eyes and then parses it out to different areas of the brain that help us understand spatial relationships, how fast something is moving and what we’re looking at, like a scene, a face or a color,” Pelak said. “The problem with PCA is that the brain can’t use the good information coming to it from the eyes.”
Difficult to diagnose
It is also difficult to get precise information about PCA, Pelak said. She noted that of the estimated 5.7 million people in the United States diagnosed with Alzheimer’s disease, roughly one-quarter don’t present initially with memory loss. Of these “atypical” forms of the disease, language loss is the most common symptom, she added, primarily because it is easier to recognize than the vision degeneration caused by PCA.
“We have no tests for a sensitive, early diagnosis,” Pelak said.
Yetz, who lives in Lone Tree with his care partner, Barbara Hubbard, relied on his eyes for 27 years at Lockheed Martin in Sunnyvale, California, first as a machinist and toolmaker and later as a cost scheduler. It was during his work in the latter job that Yetz said he noticed his visual acuity slipping.
“I started to struggle with figures,” he said. At home, simple chores like balancing the checkbook posed problems. “The numbers weren’t clear; the small print was fuzzy. I wasn’t able to add the numbers, even with a calculator. I had no idea what was going on.”
Early danger signs
Yetz retired in 2010 and moved to Reno, Nevada. But the warning signs he’d noticed earlier became a blaring alarm when he got behind the wheel of his car. Distinguishing the colors on stoplights became a challenge and his then-wife warned him he wasn’t seeing signs and other landmarks he should have. After a frightening narrow miss with a bicyclist who escaped his
attention, Yetz voluntarily gave up his driver’s license.
These signs of cognitive deterioration, combined with neurologic and neuro-psychological testing, pointed to Alzheimer’s disease. But it would not be until 2014 that PET and CT scans in Reno confirmed the diagnosis. The presence of PCA remained in the shadows until 2018 after extensive testing by Thomas Wodushek, a PhD neuropsychologist with the Department of Neurosurgery at the University of Colorado School of Medicine, and a subsequent examination by CU neurologist Dr. Brice McConnell. McConnell, in turn, referred Yetz to Pelak, who identified the PCA.
On the surface, it was hardly a cheering diagnosis. There is no cure for PCA, and Pelak noted that it can also progress to “more typical” Alzheimer’s disease, with its attendant memory loss and progressive dementia. A recent visual examination showed that Yetz had lost perception in the left half of both eyes. Hubbard notices his problems seeing things that to others are in plain sight, and he struggles to find objects placed on a background with a similar color.
Finding meaning
But Yetz, an affable and thoughtful man, prefers to concentrate on the positives in his life. After his 2014 Alzheimer’s diagnosis, he became a staunch advocate for research and education with the Reno chapter of the Alzheimer’s Association. The work included trips to Washington D.C. for talks to members of Congress in support of funding for research into the disease. He’s offered to lend similar support to Pelak and has provided an endowment for PCA research. He will also donate his brain for research after his death.
“You can sit back and feel sorry for yourself, but if you dwell in that mode, it’s worse,” Yetz said. “Never once did I have a thought of, ‘Oh, poor me.’ I knew I could do something to be helpful.”
Helping hand
He does so with the full support of Hubbard. They met about 15 years ago at Lockheed Martin, where she worked in software configuration management. They shared a house as roommates a couple of times – “laughing a lot, playing darts,” as Hubbard recalled – before losing touch for a time after Yetz’s health travails set in.
They eventually reconnected and Yetz stopped in Denver to see Hubbard on his way to North Carolina, where he planned to live in the home of a nephew. When that didn’t work out, he asked Hubbard about renewing the roommate arrangement, but under far different circumstances than before.
She gave him a straightforward answer: “I’ll be your care partner.”
It might have been fate, Hubbard added, but she also makes the decision sound less like a life change than a continuation of good works.
“I’m from a family of public servants,” she said. “It runs in our blood.” She said she learned a powerful lesson about the value of giving back while caring for her late mother. Hubbard suffered at the time from a severe lung infection, and her mother’s retirement home friends pitched in to help her.
“They adopted me,” said Hubbard who was, in turn, inspired to reach out to and visit her mother’s friends. “Her friends became my friends,” she said.
Hubbard readily admits that she didn’t fully grasp what was in store for her when Yetz joined her a year ago. But she said she concentrates on shortening his frustration when he can’t find things and taking time out when he repeats things several times. “I try to find a solution,” Hubbard said. She was also instrumental in getting Yetz care at UCHealth, a role that is apparent as she scrolls through her phone to confirm the dates of the appointments that led to his PCA diagnosis.
Just as importantly, Hubbard is a friend and companion for Yetz. In late September they took a 15-day tour of Italy that Hubbard commemorated with several hundred photos of landmarks and artifacts in places like Pompei. Yetz used a walking cane to help him get around while Hubbard kept a watchful eye on him in unfamiliar surroundings – the only mishap being his trip over a raised light dome in the sidewalk that split his lip without dampening his spirits.
“I was able to go to Italy and enjoy it,” Yetz said. “You shouldn’t change your life [because of your condition]. You should do as much as you can. Barbara’s help has been super important. Being alone would be a struggle.”
Caregiver support
A PCA diagnosis can be isolating for both patients and caregivers. Pelak leads the bimonthly Colorado Posterior Cortical Atrophy Support Group, the only one of its kind in Colorado, and to her knowledge, in the country, that is devoted solely to in-person meetings with PCA patients and caregivers. The group underscores the point that as difficult as the disease is for those who suffer it, their families and loved ones also need support to deal with the stress of providing care, Pelak said. She noted that many PCA patients lose their independence at a relatively early age and therefore require long hours of attention to keep them safe.
“The role of caregivers is crucial,” Pelak said. “The better we support caregivers, the better the patient does in terms of quality of life and living longer.” She added that technology, such as audiobooks and voice-controlled smartphone apps, can help keep people with PCA engaged without taxing their vision and cognition.
In addition to her core mission of assisting individuals like Yetz, Pelak said she has a twofold mission in drawing attention to PCA: to raise awareness of the symptoms of the disease among patients and providers, and to spur additional research. She led a 2017 study that provided online surveys to PCA patients and caregivers and to physicians caring for them. The goal was to gather information about the clinical backgrounds of patients, the burdens faced by caregivers, and insights from physicians about the kinds of care they provide.
“By using social media to explore patient and caregiver experiences and physician experiences with PCA, we can gather information from people all over the country and the world,” Pelak said. “This allows us to hear themes that lead to important research questions that we might not have thought to explore. That is what happened with our study. Thus, we heard themes that will, and did, lead to research questions.”
Pelak also was co-author of a paper that established, through a consensus of providers, criteria for classifying PCA – another key to standardizing research findings with “common principles and language,” thereby potentially increasing the number of people diagnosed with the disease. As the study concludes, a standard “framework and terminology” for classifying PCA could help specialists interpret research data conducted in many settings, from behavioral studies to drug trials, and “provide a foundation for future collaborative work.”
That work will have the best chance of success with the backing of people like Bob Yetz, Pelak added. “If patients are aware of PCA, they will always be creating and teaching others about our goals,” she said.
For Yetz, the disease that has robbed him of so much also has given him something of great value. He points to a picture of a sign he saw during his time in Reno. It reads, “You will recognize your own path when you come upon it. Because you will suddenly have all the energy and imagination you need.”
The words blazed the trail for what is now the direction of Yetz’s life. “It told me I can help people with and without the disease. I found my purpose in late life.”