Following the Alzheimer’s diagnosis,
I typed “brain disease” into the computer, rejecting all the Alzheimer’s answers because they did not fit with Wheels’ symptoms. Finally, Frontal Temporal Degeneration popped up. The bit of information fit Roger to a T.
I took that little bit to the next doctor appointment. Doctor looked at it, asked Wheels some questions. Then said, I know someone who can give us some definite answers. And, just like that, Roger was referred to a neuropsychologist at the state university in the Big City.
The neuropsychologist called a few days later
She interviewed me by phone, asking questions about his medical history, daily activities, diet, sleep and observations of changes in ability and interests.
Wheels couldn’t handle a round trip to the Big City, not quite four hours away. We always stayed at least one overnight and tried for morning appointments so we could drive back home in the afternoons. Decisions wore him out – he no longer cared about what to have for lunch, where to stay, what route to take. His autonomy was circling the drain.
Neuropsychological testing was an all day affair
sitting with all our winter coats and day’s supply of necessities in a waiting room. We became experts in such waiting rooms by looking for the corner seat furthest from the traffic at the admittance desk. A table in between two chairs was a bonus!! A nearby vending machine – double bonus!!
The neuropsychologist was very was patient when Wheels became grumpy at the length of time. The test was multi-factored and took six hours to administer. She spent time analyzing the results, and then another shorter session in the afternoon to explain her diagnosis. She said very little was known about the disease. The brain was degenerating in the left frontal area. As it progressed Wheels would lose his ability to speak, feed himself or swallow and eventually to walk. There was no way to predict progression, but she assured us there were ways to cope at every step of the way.
She gave us a name for the mystery that had been skulking in the background: Frontal Temporal Degeneration/ Primary Progressive Aphasia. I couldn’t even begin to wrap my mind around what I was hearing. I didn’t know what questions to ask. We had no idea what that meant for our life in the future. Apparently no one else did either.
The neuropsychologist referred us to a Speech pathologist. And a neurologist for PET scans. Only 12% of people get the right diagnosis from the first doctor they see, according to research. Our family doctor got it on the second try – enough to send us to the right specialist. 40% of those diagnosed saw four or more doctors before being correctly diagnosed. The neuropsychologist identified the disease and it was confirmed by the two other specialists.
The neurologist we saw had cared for Wheel’s sister with Parkinson’s. Her family spoke highly of him. The PET scan was needed to confirm PPA/FTD,. He said the disease was confirmed and rattled off something about tau tangles. I had never heard the term before – no explanation. Then he said “no known cause, no known treatment, no reason to come back.” And opened the door for us. It was a blow to the midsection – I literally could not breathe.
His PA said, “I will see you”. From then on, this hero made sure Roger had physical and occupational therapy in addition to speech language therapy. She prescribed a low dose anti-depressant. At the time, this was not the norm, but now this is much more common. PT and OT kept him moving, and facilitated adaptations to his changing abilities. The anti-depressant helped calm anxiety over what was happening to him. A hero who went out of her way and made a long term difference.
The Tau proteins the neurologist mentioned is currently explained at theaftd.org as “the majority of nfvPPA patients (60%) have abnormal accumulations of tau protein in the brain that are distinct from the type of tau protein that accumulates in Alzheimer’s disease”. In other words, the testing eliminated the possibility of Alzheimers.
The Speech Language Pathologist at the University Medical Center sold Roger on the benefits of speech therapy by letting him explore technology she presented. This made him beam. She treated him as an adult and his own person. He had used technology in the past and she honored that part of him. She also confirmed the Primary Progressive Aphasia due to Frontal Temporal Degeneration diagnosis. And directed us to the University Speech Language department located in our small city.
That became the brightest light in our journey with Primary Progressive Aphasia due to Frontal Temporal Degeneration.