Introducing myself

[snowrail]

After several kind invitations I figured I should introduce myself.

I'm Shannon Larratt, somewhat well-known and somewhat notorious artist and internet entrepreneur that retired a few years ago at the age of 35 after starting various adventures. Fortunately and unfortunately this retirement synchronized with my tubular aggregate myopathy's symptoms (primarily weakness and extreme pain) making it difficult to work. The diagnostic process was unbearably slow, as I'm sure it was for many people, make more complicated in my case because it was preceded with the removal of a large benign bone tumor/growth from my leg (which was caused by over-calcification of a tiny muscle tear, and may or may not be related), a surgery that caused nasty nerve damage in that leg resulting in cascading neurological pain. This sent us down some false corridors when the pain and weakness from the tubular aggregate myopathy started to accelerate since we assumed they were neurological in nature.

I have significant weakness in my legs and more recently arms. CT scans show that my back has a great deal of damage as well but I was in exceptional shape before it really kicked in, so I am not yet aware of the back issues. However, my main "complaint" is the pain, which is all-consuming at this point. There is not a moment of my life that I do not experience a horrible dull throbbing pain akin to what it feels like to really abuse yourself beyond the point of failure at the gym. I try and maintain an active and positive life but it is not easy and gets harder every day. I don't feel like I have a whole lot left to fight this with.

The pain is currently being treated with opiates, methadone specifically. I've had good results with opiates, and prior to going on methadone was on prescribed oxycodone as well as self-medicating with any additional opiates including heroin. This decision had the unfortunate political side-effect of slowing down my treatment because many doctors wrote me off as an addict with drug-seeking behavior, which was absolutely false. Not that it matters, but the reason that I like opiates is that I experience no high whatsoever -- no euphoria, no drowsiness, no mental blurriness, nothing... Just pain reduction. I've tried other things -- various off-label anti-depressants, lyrica/gabapentin, even marijuana, and they've either not worked at all or had horrible side-effects. Opiates, including the methadone, have worked well for me.

Unfortunately, my treatment is currently at a stand-still because after going in for a routine sleep-study (a normal part of increasing opiates to high levels) they discovered that I had significant central apnea with my breathing stopping for as much as five minutes at a time. This was (is) also happening to a lesser extent while I was awake. At first the assumption was that this was being caused by the opiates -- and they may well be aggravating it, which has made them cautiously freeze my opiate dose where it has been for the past six months -- but the "profile" of the central apnea makes it appear to be caused by brain damage rather than the methadone. But that's all we know and it's where I'm at now.

Additionally, I am experiencing a number of troubling neurological side-effects (and I know that this is not unheard of with TAM but I have had difficulty finding anything specific) and am awaiting further testing to get to the bottom of it (this is complicated by the fact that I have metal in my body that rules out MRI scans). We don't currently know whether the neurological effects are TAM-related, drug-related, psychiatric (this has been rather stressful!), or something else altogether.

And there's my introduction. Thanks for maintaining this forum. With how little information is out there about this condition, every little byte counts.

[Craig Lane]

Hi Shannon,
Your story will have many of us nodding as we all have found the path to diagnosis a slow and difficult path to tread. Littered as it is with misdiagnosis and bureaucracy. I was told by my consultant Professor that the only way to diagnose TAM was by muscle biopsy, the sample must not be treated for transport as this destroys the evidence. Therefore the biopsy must be taken at the same place as the test is carried out. (Details of this test available through Georgers' pages)
I am interested in what damage the CT scans found and the relevance to your TAM. I am aware as we all are of the differences in medical practice across the globe, it maybe that Canada is one of the places that steps up to a challenge rather than the usual "You're unfixable, on the scrapheap with you" attitude.
If CT is a useful tool it could save long waits and non-invasive exploration.

On a different tack, I have a little of your background and can empathize with your stress and frustration, especially with regards to pain management. I am very "lucky" in as much as I have a very high pain threshold and for most of the time can grin and bare the constant hung over and beaten up on the way home feelings. Poking fun at my own inadequacies whilst accepting some of my limitations has helped. Those folk who seem to cope best are those with a somewhat strange sense of humor. I find a balance of planning the things I am going to do against what I actually do (with heavy leaning toward the planning side) works well. Not trying to do any one task for any longer than bearable. Half an hour to watch an interesting program seems like a great idea in the planning stage , lets face it we can all sit about doing jack for half an hour. Then ten minutes later a move to make a coffee or sandwich slips into the plan as sitting for any longer is crippling. Halfway through making the coffee the cup decides it would be happier in pieces on the floor and makes it's bid for freedom. Maybe the original plan of watching the program was best after all, so back to the sofa or chair. So it goes on constantly planning and modifying the plan. I have redesigned my garden about twenty times once I even found the way to the garden shed but, I still have the same rubble and weeds left over from when I was able to implement a plan. Does it get me down......... Of course it does if I let it but I make a new plan and thank the stars I didn't waste all that time and effort doing all that work I didn't need to.
This post is prime example and may explain its' disjointedness. Four hours and many changes in plan. This short reply becomes an epic.
Really interested in the sleep apnea as well my wife insists on waking me up to "breathe" so I can assume it affects me also. I did do a sleep study perched on an ironing board in what may have well been Piccadilly station for all the noise, comings and goings. Surprise the results were inconclusive.
That's it as I have a change of plan.

[snowrail]

Alright, this board moves slowly so forgive me in taking a month to reply.

I'm not sure what the specifics of how the CT scan looked, all I know is that they showed muscle abnormalities that correlated exactly with both my pain and the electrical tests they did. So I don't know what they saw, but I can confirm that TAM is visible on the CT scan. That said, at the time they had no idea that it was TAM, just that there was something wrong with the muscles. The biopsy wasn't figured out until some time later.

My apnea is quite extreme, and we're not exactly sure what's causing it. I am having approximately 60 central apnea events per hour while I sleep, and slightly less while I'm awake -- even while awake I regularly notice that I'm not breathing and have no "desire" to do so, or I notice myself suddenly gasping for air as the impulse kicks in. My O2 saturation is usually in the 80s, and drops as low as 70% at times. Breathing usually stops for no more than 30 seconds, but occasionally stops for several minutes. In addition, my heart has an equivalent event once or twice an hour... My heart rate is normally about 50-55bpm, quite steady, but about once an hour it stops for five or six seconds... Drops to 10-15BPM temporarily, and then just turns back on. Also about once an hour it goes into rapid fibrillation for a few seconds. No one knows why, and besides some headaches it doesn't have any obvious health effects, as disturbing as it sounds. Interestingly, this problem stops almost completely when I am in REM sleep... Other than that it's pretty constant, whether I am awake or asleep.

Like I said, I don't know how related to the TAM this is. When I was younger I had an interest in apnea and freediving and regularly held my breath for over five minutes. I also have an interest in meditation and am able to temporarily stop my heart consciously... So I don't know if I messed myself up playing games one shouldn't play, or if this is TAM related, or if it's medication related (opiates can cause such things), or if it's something else entirely.

Even on heavy opiates, my pain is essentially untreated. When I was taking heroin, I was able to control the pain, but that was two years ago, and my pain was much lower then I think. So I don't know if it would even work any more. But what I'm doing now isn't working. We're changing up the medications a bit to try new things, but I don't expect miracles given what I've seen so far. So I'm in significant and constant pain and I don't see that changing any time soon. In addition, I've hit a point where I'm starting to feel significantly limited as well. Earlier this year I could still handle a 10 mile hike -- albeit it would come with a few days of severe pain and inactivity afterwards -- but now it's a struggle every time I stand up and walk across the room, and even a short walk or an hour of light activity means I'm going to have half a week of hellish recovery...

[Craig Lane]

Thanks for the info Shannon. Seems the CT could be used but only if the operator was specifically looking for the aggregates. As Pete has pointed out to me even the biopsy needs the right prep for diagnosis. I know from my own experience that the chemicals they use to preserve sample for transport destroy the evidence.
As far as pain management it is different for each of us but I think universally, if it hurts stop. Unlike the old days when we would just puss through that just amplifies the issue here. As frustrating as it can be not to be able to do all the things we used to do with ease if we want any quality of life we have to dial back on the action. Driving on and spending days or weeks recovering is just progressing a chronically degenerative disorder.
I am sure one of the guys on here is an anaesthetist I will research that in a minute maybe he can steer you in the direction you need with pain management. As the doctors are unable or unwilling to help we have to help ourselves.
Thanx again for you contributions.