POST 5: The Beginning—(Part III of III) Google and the Doctor

(If you are reading this for the first time—this is a continuation of Post 3 and 4 – explaining the discovery of my mom’s FTD and Post 1 has an overview on what they heck we're talking about in this blog).

(Voice of daughter, Christina)
I don’t remember exact memories of the first signs of Mary’s disease. I remember her calling my husband “Toby,” pointing to a knife and asking for a fork, and skipping articles in her sentences.  As a Speech Language Pathologist, I worried about TIAs (Transient Ischemic Attack)-or Mini-strokes. Something was not right; it was a scary feeling but TIAs we could handle, watch for larger strokes and react quickly. I remember encouraging mom to get to the doctor late that summer 2007.


Slowly the rest of the family began to see it too. Lots of “forgetting”—which really wasn't forgetting, but misfires in her brain.  Depending on the exact area of injury, when a person with deficits or injury in his/her temporal lobe of her brain tries to speak or understand, the neurons trying to send the message to the damaged area, “reroute,” send the message to related areas. When a person with expressive language deficits cannot name, but instead names something related it’s called semantic aphasia. Thus “knife” is closely related to “butter”; Roth and Toby are both male names. So a related words may come out, but not the correct word. 

Aphasia is often misunderstood as confusion; it’s not confusion, just a detour that didn’t work out so well.   When a caregiver understands that Aphasia needs help with the detour, he can adjust how he interacts with her. If she is trying to tell him something, asking yes/no questions may help (to a point), or backing up and thinking about the whole picture is very useful. If a caregiver can see both sides of the situation, he can often help to redirect the person, which will make her feel more confident and successful.

Back to Mary’s story, being far from home, I was able to see and hear the changes in Mom, in different ways. She started calling less often, she didn't ask me the goings on in my life, she stopped sharing stories. When I asked questions, she often had a hard time relaying specific events, again not because of her memory, but because she couldn't attach meaning to the words I used. When I arrived home for Christmas, it was even clearer that things were going goofy. I could sense that she could tell something was awry; deep down I feared that she felt she was beginning to suffer what her uncle had suffered from, what our family believed at one time to be Alzheimer’s disease.

(Voice of Betsy) Between Jan 08 and April 08, Mom was poked and prodded…. EEG, SPECT Scan, PET Scan, MRI’s, blood tests…you name it, they did.  No answers.  At this time we hid the news from my mom’s parents and the rest of her family, deciding to not let them worry until we found out what was actually wrong. 

And mind you – even though mom was getting worse, it was still early stages.  I can remember calling home in late spring and mom being able to tell me what tests that did that day, what the heck a PET Scan was (using medical language-she was a nurse after all), and what the doctor was looking for.  Some days she would talk for an hour and not skip a beat, and I would be so hopeful we are making a big deal out of nothing.  I would hang up elated.  And then the next day she would stand in front of the kitchen sink, staring at it; confused and saddened; knowing she needed to do something but draw a total blank on why she was standing there.  (And then you could hand her the sponge and it would trigger something and she’d go at it).

With each test, the less fearful causes (menopause, mini-strokes, brain tumors, etc) were ruled out one by one.  Alzheimer’s was still a possibility but it didn't seem to fit. 
       1. Mom was 56

       2. Mom was not having memory problems.  She never repeated actions or stories.  She did not eat a Popsicle and ask for another one 30 seconds later or talk as if she was still in high school.
       3. Finally, Mom was very aware of her speech and executive “issues.”

And so – in the Spring of 2008, I became “one of those people.”  We all know the type – and we all have fallen guilty ourselves; “Those people” that spend hours upon hours on the internet playing doctor.  I began diagnosing my mom, constantly on the lookout for a simple answer- an answer with a solution.  Certain I would get to it before the doctors did.

Tina mentioned some aphasia thing, a condition of some dementia thing, neither of which I had ever heard of.

So, first, I called Tina back.

“What did you say Mom might have again?  PP what?”

“She’s showing signs of aphasia.  It may be, but hopefully not, Primary Progressive Aphasia --  PPA, it’s a form of FTD.”

So I ask Google: “What is Aphasia?”
Answer: Aphasia is a condition that robs you of the ability to communicate. Aphasia can affect your ability to express and understand language, both verbal and written.
Aphasia typically occurs suddenly after a stroke or a head injury. But it can also come on gradually from a slowly growing brain tumor or a degenerative disease…
Once the underlying cause has been treated, the primary treatment for aphasia is speech therapy that focuses on relearning and practicing language skills and using alternative or supplementary communication methods…”

Ok, that’s doesn’t sound great but not the end of the world.

A conversation I had with Adam echo’s in my head – “If Christina is right, that’s probably the worst diagnosis Mom could get.  It’s terminal and sometimes only takes a few years.”

I close my eyes and take a deep breath.  I reopen them and begin typing again.

“Primary Progressive Aphasia”

I search Mayo Clinic, Wikipedia, UPMC, UPenn,….I reword my search over and over, searching for hopeful words and phrases and push everything else to the back of my mind.  I’m looking for something to tell me things will be ok.

“…tend to worsen over time…”      --tend to – but  it didn’t say will worsen…
“…People with primary progressive aphasia are fighting against a condition in which they will continue to lose their ability to speak, read, write, and/or understand what they hear… In the early stages, memory, reasoning and visual perception are not affected by the disease and so individuals with PPA are able to function normally in many routine daily living activities despite the aphasia...”

Does that mean they can comprehend what is happening to them??
NY Times… “While symptoms of Alzheimer’s are readily recognized by friends and relatives but not those affected, people with P.P.A. are painfully aware of their struggle to communicate, often long before it is apparent to others.”
…People with primary progressive aphasia can become mute and may eventually lose the ability to understand written or spoken language.

But Mom is only 56….
Most people with FTD are diagnosed in their 50s and early 60s. Only about 10 percent are diagnosed after age 70. Alzheimer's, on the other hand, grows more common with increasing age. People with PPA can have a variety of different language symptoms and no two cases are exactly the same.

What is the end result?
FTD inevitably gets worse, cognitively and then physically…in advanced FTD, people typically become mute and bedbound…Studies suggest that most people with FTD survive an average of six to eight years, but survival can range from two to 20 years.

And there it was.

I can’t peel my eyes away from the last sentence, and more specifically, 2 to 20 years.  Which is worse?   

I read over more symptoms/treatments as the disease progresses but I am only half absorbing at this point.
“….Some patients with FTD develop Lou Gehrig's disease (also known as ALS)….incontinence….shrinking brain…Parkinson-like symptoms….inability to swallow….no treatment….no cure….” 

Oh Mom…

All that was left to consider was Alzheimer’s or FrontoTemporal Dementia/PPA.  April 16th, 2008 – Primary Progressive Aphasia is the preliminary diagnosis but not confirmed.  A spinal tap needed to be taken to rule out Alzheimer’s.  But they did not screen for the protein that was required to rule out Alzheimer’s, so my mom had to get another one in June 2008.  The doctor said we would have the results in two weeks.  Meanwhile, my mom’s emotional and cognitive state continued to diminish. 

My company had allowed me to temporarily transfer to the Pittsburgh office for a few months, so I was back living with Ma and Pa.  Mom and I would spend evenings putting together lists of “chores” for her to work on the next day – to keep her busy and focused.  Each day, it seemed to be a little bit harder.  I wondered how long it would be until she could not read the list anymore.  Not because she couldn't see it, but because the words stopped carrying meaning.  The start of the school year was just around the corner and she should be returning to work (school nurse).  I think by now we all knew she was not going to be allowed to return but no one was talking about it.

When we did not hear back after a few weeks, we called the doctor’s office.  Of course, it went straight to voice mail.  Finally, on 11 August 2008, a letter arrived at the house.

That’s it?!? What happens next? Was I reading this correctly?  Was my mom just given a terminal diagnosis through a six sentence letter?  Ending, by the way, with “Thanks so much for visiting, please share the results with family. Best regards.”  She doesn’t have pink eye for Christ sake! 

The diagnosis was life altering but delivered with the same level of regard my mom would have toward one of her frequent kids, “go take your math test, come back after if you still have stomachache.”  Oh wait, but the Doc didn’t even offer to see my mom again, so my mom showed more regard to her 5th graders faking a stomachache.   

I picked up the envelop to see if there is a second page of instructions, follow up request, information on what FTD is…treatment options…anything.  Of course there is nothing. 
I would give the letter to my dad next, but honestly, I don’t think we ever showed my mom.  Why hurt her more than she already was? For the first time in my life, our family felt truly and completely helpless and without any direction or support. 

The only other thing I remember from that day is asking Mom to make her chicken stuffing casserole for dinner.  God I loved that dish!  And cooking made her feel useful—who knew how long it would be until she couldn’t make her renowned casserole again?  – we didn’t even have a doctor anymore to wager a guess.

We were back to square one and Mom was alone…

Mom/Mary Margaret  - Summer 2006 (showing signs but hiding it from the world)

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