rocmg, I'm sorry about your mum's fall, hopefully it was just one of those things that can happen, and won't become more of a problem.
I"m just now reading this thread for the first time, so I will attempt to answer your original question.
I received a diagnosis of PBP July 08. At first I didn't really understand t the significance of it, I even posted on this forum that my neurologist had stopped short of actually diagnosing me .... then.... her report from my visit came in the mail, and after reading it I realized that what she'd actually stopped short of was saying to me was that it would progress beyond the bulbar region. When she was talking to us in person, she was so gentle about it, kept saying how it "probably" was ALS, but it was too early to tell, she had tears in her eyes at one point, and that empathetic quality is, I think, pretty rare to run across in a neurologist! .... So, then this report comes in the mail, and its all just typed out in black and white ( because it obviously was written to inform my other doctors of what had been found) I just stared at the paper for the longest time. She said in the report how she'd spent over 40 minutes counseling us about it. Which she did, and I heard her, but apparently I excel at Denial!
Here, as far as I can tell, is the history.
The winter of 05/06 I started getting severe muscle spasms in my legs, & occasionally my abdomen. The occurred most frequently when I would go to bed after working a trip. I assumed it had something to do with flying, although, I'd been flying for many years, and had not had problems with them before. They were definitely worse after working though, so it just seemed to follow that there was a cause and effect. I "googled" it, and what popped up said that charlie horses usually were nothing to worry about, so, simply put, I didn't.
By spring of 06 I was battling increasing fatigue, I chalked it up to jet lag, and submitted a transfer request to come back to domestic flying. It took me the longest time to realize after I was back to keeping normal hours that it had done nothing to help with the fatigue; that it wasn't jet lag causing me to be so tired. I'd go to work in the afternoon, fly to somewhere on the west coast, fall into bed, get up still exhausted, fly back to the east coast, and pretty much lay on the couch until it was bedtime again. I wasn't sleepy, just fatigued. I was working progressively less and less. It would literally take me 4 days to recover from a 2 day trip.
I was still getting the cramps. I'd had dry eye for a while, but, again, I had a very dry workplace environment. By late May or early June I also had dry mouth, I would have these awful white stringy things in my mouth when I'd wake up in the morning. So, I eventually went to my GP, he tested me for diabetes, and Sjögren’s syndrome. Everything came back normal.
Somewhere around July or August 06 I started noticing something weird going on with my fingers of my right hand, when I would twist open the cap of one of those little mini liquor bottles on the plane, my fingers would kind of get stuck.
It was not until the fall of 06 that I started going to the doctor in earnest about any of this. But the leg cramps always got dismissed (until I saw my neurologist at Hopkins for the first time)
Here's a point of interest that I missed for the longest time: People ~ even doctors ~ hear what they are expecting to hear. When I started going to the doctor, trying to sort out the fatigue, etc, I would tell them about my fingers. What they actually heard was a middle aged woman with a history of osteoarthritis in her spine, saying her joints in her fingers were stiff. In actuality, what I was feeling was the spasticity, like I was encountering resistance such as an elastic band around my fingers. So, the doctors are hearing one thing, and I'm describing another, but don't I don't grasp that they're not getting it.
Anyway, back to the chronology: In the late autumn of 06 I started having voice problems. Especially on the phone, people would either ask if they woke me, or if I was getting sick. They noticed it way before I was aware of it. I also had some breathing sensation strangeness going on, which was originally treated as asthma, and then the thinking was the asthma meds were maybe causing the voice problems. As the meds weren't helping, I went off of it (Advair) and it made no difference one way or the other.
By January of 07 I was being seen by a rheumatologist, and he thought maybe it was Sjögren’s, even though by then all antibody tests for everything had been repeated and repeated and always were negative. ( I think all in all I maybe had the various antibody tests done no less than 6 times, perhaps more).
So, one of the most common medications for Sjögren’s is Plaquenil, I was started on this, and what got better were my leg cramps (it is a quinine drug) I was also put on prednisone, and eventually methotrexate. Nothing made a difference with my voice or with the fatigue, but I didn't get cramps (and no one but me seemed to care about them anyhow)
By autumn of 07 I finally saw a specialist who'd primary concern was the voice and throat. She took one look at my vocal cords, said there was atrophy (first time ever this was mentioned) and wanted to do an EMG (which I'd never heard of) The EMG came back with the pattern of active and chronic denervation, reinnervation, but no mention of PBP or other forms of MND were made. Instead, I got to have the MG panels and lyme tests run again ~ why not, right?
By winter 07/08 I started having some problems connected with eating. I would get spasms when chewing, things like that. It was then, in December of 07, that I remember being on the phone with my laryngologist, and her first bringing up cranial nerves, the 9th and 10th in specific. I'd never even thought one way or the other about which nerves controlled the muscles in my throat before. I'd never heard the terminology "bulbar" anything! She didn't allude to it either, but did say she thought specific cranial nerves were what were causing my problems.
In January of 08, my local doctors decided I needed to go to Hopkins or Mayo.
I think it was May I first saw my neuro there. I also had my first (mild) case of aspiration pneumonia around then. I'd had a modified swallow study done, and it revealed I had weakness in every part of the eating/swallowing process. I was so surprised. I just was not that aware.
In June of 08 I had trouble opening and closing an airplane door while doing my annual qualification training for the FAA. This training is done in a large facility, and there are actual aircraft doors and exit windows for every plane we would work, in there. ~ pilots get really cool simulators, plus the doors windows and emergency equipment, but flight attendants just get doors, windows, and emergency equipment (oh well) This door that I almost failed opening, was the same exact door I'd opened and closed for at least 6 previous years. I even asked one of the instructors if perhaps changes had been made to them from the previous year, and she said it was the same door since when we first started flying that style aircraft, which I think was 2001 or 2002. Other doors gave me some trouble, but this one was my wake-up call. I knew my arms were weak. The entire rest of training I could hardly even concentrate, because I kept thinking that if I had the PBP (at this point my diagnosis was only "possible" PBP ) it was advancing beyond the bubar region.
So, a couple of weeks later, I received the PBP diagnosis, then about a month after that, I started noticing some leg weakness.
Right now, from my perspective, most of the progression is going on in my legs, and to a lesser extent my arms, but its really so hard to tell. You would think it wouldn't be. You'd think a person would just "know", but I don't. I think that with my leg weakness, and my arms, I can tell easier, because the bulbar stuff is just sneakier. How is a person going to know if their epiglottis is closing at the right moment, or tight enough? But that same person (me) has no trouble noticing when she can't raise up on her toes to reach something off of a shelf. Does this make sense?
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