Breath, Wheel, Fight, Dance

Sudden prolonged weakness. Too weak to move, hard to breath. Linda and a care worker use the sheet to roll me in bed. I lose almost two hours. Linda tells me I had problems breathing, stopped breathing. She breathes for me, holds me.
I was going to go wheeling that day. I twitch my head to get the worker closer. "Tomorrow" I promise her, "I'll wheel tomorrow."

It might take 2-3 hours to dress and be ready but I will wheel. I am going to be in a 5K within a month, then a 10K, the women’s cancer 5K and Terry Fox 10K.

This week I found a new foundation: the seven people doctors in who study autonomic failure diseases have created this foundation together. They are just now starting on a drug trial, a drug and control group (group that gets no meds) for finding a drug that helps. I think about the Hospital which specialized in Parkinson’s and MSA-p, and how the director told me I needed an autonomic specialist

“Where? Is there one here?” I asked and she promised to look. But she never found one.

Three years later and there is the start of one. The doctors are looking to base themselves in one or two locations. I want to live long enough to know that there will be something other than pain relief and gatorade for the next person who gets this.
“It is a cruel, cruel disease.” Linda said to me today. It is, cruel on both of us. It will be studied at last, medicine at last, not while I live, but soon maybe.

They found three patches of Fentanynl on me, left from previous days. Oops! The patches burn into my skin, so I have to use aloe afterward. You can see the burn on my neck in the previous post. Linda added the adhesive cover patches to the wish list.

We use covers on the Fentanyl patchs because I don’t lie in bed like others, like they expect me to. I move, and wheel, and exercise. One day I’m going clubbing.

“I want to video my workout next time.” I say, “I don’t think they would believe it. I’ve haven’t had a care worker yet who can keep up with my exercise. Not even the first 20 minutes.”

“I don’t think the readers know what you do to survive.” Linda says. “I couldn’t do it.”

I use my hand to rub between my nose. “If you want it bad enough…”
I am rarely at the computer now, either far too ill, or if not, I am up and 'doing.' When I am here, I get my fix of good films and plan my next moves. Also I watch this, so that I remember to keep fighting, and dancing.

Here is my remedy! So while I go dance with the Enemy, you keep on moving, ‘kay? And turn up the volume! ("...everybody seems to have given up hope of trying. I haven't. It isn't easy and it isn't supposed to be” – Terry Fox at 3,113 km)

“Watch out Earl! It’s still moving!”

Using lines from horror shows seemed appropriate. I AM still alive. Though not awake that much, nor have I yet to be fever free for more than 16 hour for now: 50 straight days. I DO REST. I just don’t have the opportunity to totally rest often, as taking my dump of the every other day takes 3-6 hours (not a rest) and usually uses all resources creating a fever. We have found I can ‘safely’ talk about 60 minutes a DAY. More than that or speaking faster than ‘disability Beth time’ makes me use up energy, which often produces a fever, then seizures, etc later in the day. So I am still alive but yesterday overheated while stickering postcards, tried to make it to the door, did not, then dragged myself using an elbow and my abdominal muscles the 30 feet to the bathroom in 35 minutes. Turns out, that was REALLY exhausting as I slept for 13 hours. A couple days before that, I talked a lot, did a lot for seven hours and then….slept for 12 hours. This makes for short days. Still working on that ‘fever free for three days’ thing. Wish I had a counter I could html in here to put in for days like 1, 1, 1, 0, 0, 1, 2!!! – then at three there is fireworks.

To add to further problems, Beacon announced that they will no longer book overnighters for me because…..it takes to much time administratively. That due to my condition and to me a lot of the workers leaving (we were told sort of off the record that people who do overnighters want to sleep and kind of work for an hour, sleep a lot, and get $200, that’s why they do them – I am NOT that, so I have more workers refuse to come again, or quit than I say, “Don’t send”) it takes too much ‘administrative effort’ to try and book someone to take care of me overnight. So they aren’t doing it anymore. This combined with the ‘no GP’, ‘No one will administer IVIG, though approved’, is making me feel less than loved. Where are the hugs, caregiving and smiling doctors of shows and commercials? Is it a human rights case? Sure. Is that going to get me a judge in a week saying that they are required to provide caregivers for me while the case works through the system over the next couple years? NAW! But.....the Olympics are here!

When I say, ‘fever’, I mean, an inability to control temperature regulation or the effects until the blood in the brain is affected. So since my core is usually sent lots of heat, as I lose energy the external cooling becomes less effective, the energy for resistance is gone and I get a fever. If I am cooled, I will lose the fever (and then have like a 94 degree temp or go into shock). So, while it is ‘autonomic function control loss of the Central Nervous system vascular system for temperature regulation’ – ‘Fever’ is usually what happens, so lets go with that.

Obituary’ or The one left behind: The Booth Gardner visit.

In many movies, I think of Mad Max, or Platoon, where you see a main character lef behind while everyone is leaving, pulling out. They have no choice. There is literally a visual sea of the ‘other’, that destructive force we fear as it seems to know only pain, fear and death, closing in. In Platoon, the character, shot in shoulder, is running with over a hundred enemy soldiers after him. The Huey helicopters, having risen could look down and see him running, running, shot, falling, up and running again for the landing zone.

In the same way in Mad Max films, having used himself as a human decoy, the Mel Gibson character is shown, exhausted waiting as those who have been chasing close in. He looks out, seeing the plane, or convey getting away, and the whole stance says what has been said in so many books and movies: “they aren’t coming back....are they?”

Regarding Booth Gardner. In the reverberations which will continue for some time to come in my life I stand on the edge looking out and know that ‘they’, modern medicine, the advances in science, the doctors and specialist, they aren’t coming back for me.

They have people they can save, and they are going to them.

I never really believed, not REALLY that there wasn’t some cure or specialist out there who could change my condition, they just had to want to. Yet it is always different when you hear from the white coats.

I am sorry.

In going to Booth Gardner I carried your compassion, your best wishes, your donations, your kindness and the best hope of both of us.But before going to Booth Gardner, we made one last attempt to get Vancouver's head neurologist letter which Booth Gardner did NOT have and which neither my previous Victoria neurologist nor my ex GP (Jimmy) could find. It had the nerve conduction tests and other data so we asked the ex-GP's admin worker and suddenly it WAS found, in his file no less (he was away on vacation). We got it the day we left Victoria to go to Booth Gardner.

The letter had in it not only extensive diagnostic information, but an order to begin the IVIG treatment which I had been trying to get for two years, as anecdotally it was one of the few things to stop autonomic failure progression. After the nerve condition the IVIG had been approved.

The letter was a year old.

Last year: last May, the Victoria Neurologist and the GP were instructed to created an autonomic failure baseline and then administer three to six months of IVIG. As this treatment was the best chance for both autoimmune an non-autoimmune diseases of autonomic failure. If autoimmune was discovered, or the baseline showed a slowing, stopping or improvement, then I could continue to another medication or stay with IVIG, depending on results.

This letter was never shown to me. Instead the Victoria Neurologist and my now ex-GP (Jimmy), in daily contact, told me that taking a baseline would NOT lead to treatment (the neurologist said this to my face, it a session we recorded without her knowledge). She had previously told me that the province would NEVER authorize IVIG as my problems were ‘in my head’ and worked to have me put into a locked ward instead. After I made a formal complaint she was removed from the case and the opening for the locked ward disappeared.

My GP, Jimmy, however for the next 11 months, not only had the letter but twice pulled it up on his computer to read sections to me (but never had a copy for us). He also during this period refused to refer or authorize all diagnostic care, all specialist referrals, including the many, many requests for a neurologist. Nor the requests for endrocrinologist and refused to treat the anemia or thyroid, or even test the hormone levels (as the medication required every 6 months for liver function tests).

I now have a GP I have met once, and I need a neurologist referral, a baseline test, and then IVIG treatment. In the Canadian system that amount of referrals will take between 11 and 14 months. Would I already have received IVIG treatment having a neurologist a year ago? Yes, I would have had at least six months of treatment by now.

Even if somehow the treatment could begin tomorrow, I will NEVER regain the nerves that are lost, never be able to leave the house without an oxygen converter as my lungs are failing, I will not have the extensive brain damage reversed, never again the same in reserves nor will I be able to take a normal dump for the rest of my life (oddly rather important: don’t take one for two weeks and see if you agree?). There is a year of permanent nerve damage. Last year, I was one month from my Japan trip, a trip which Linda said a few months ago, simply could NEVER happen again, in my condition.

My GP, the person entrusted for my care, told me I would only receive treatment if a specialist told him what to do. A specialist told him to give me IVIG. He did not. Instead he told me he would only give me treatment if I went to the US for diagnosis. The province felt that I had a serious enough condition to spend $18,000 and perhaps more to TRY and save me. My GP ensured, by not allowing a neurologist referral that no one else would ever see this letter (as if I had a neurologist, the letter would have to be forwarded or they could contact Vancouver themselves).

That was what we read in the Van driving to Booth Gardner the day before. The pictures of me smiling at different races, races I don’t know if I am able to finish THIS year flashed on my screen saver. A year sometimes makes a great deal of difference.

At Booth Gardner, a complete history was taken as well as review of the tests and this ‘new’ letter. Booth Gardner is NOT a center for simply autonomic failure but rather a Parkinson’s center for MSA. What they could tell me is this: I did not have MSA (or a variant they were familiar with). I have central autonomic failure. I also have peripheral autonomic failure. It is not known how that is possible but it the facts. I also do NOT have POTS which is the non-lethal form of central autonomic failure. I also have peripheral neuropathy (progressive) of which there is no understandable connected cause. When asked what could cause such destruction the head Neurologist of the center said, “Heavy Metal poisoning?” (Could you PLEASE stop sending me liquid Mercury cookies!) which is like saying ‘Radiation poisoning’ as both don’t damage only the Axon, they simply warp the DNA, eat the nerves and wreck damage on a level beyond predicting. I have not, to my knowledge, been wearing my Cherynoble earrings.

The Neurologist in BC who saw thousands of people with neurological condition and a few with autonomic failure said it was not DNA related, and unknown if auto-immune related. It could be just ‘one of those diseases’ like Lou Gehrig disease/ALS was until it had a name. Why do I get it? Why me? Who knows? The head of the center which dealt with thousands of people coming with autonomic failure each year could tell me what it was: something which should be impossible – both central and peripheral autonomic failure, much less the peripheral neuropathy. I had the lethal kind of central autonomic failure. Indeed outside of anecdotal evidence that IVIG works on some people (best with those with AAN) the ONLY hope for autonomic failure are those with MSA-P, who have a 5% chance of extending life expectancy. She felt I needed a referral to a specialist to only rare autonomic failure cases and is giving one. I pointed out that at the progression and the types of autonomic failure left, that I was not only terminal, but rapidly terminal. She agreed that ‘those with autonomic failure (of my type) had a shortened life expectancy.” But again emphasized that I did NOT have MSA (or a variant she had seen before in her thousands of cases) and I needed to see an expert in autonomic failure (AAN, is often called MSA because no one has ever heard of it; this is what I have believed I have, however I often use MSA as it is easier to understand. She does NOT know if I have AAN). She also wrote that the anemia and thyroid needed to be dealt with. And this was my diagnosis. My Obituary.

All those words but what does it mean? It means I thought for a day and then cried a lot. It means I spent about $800+ to get the answer. The answer is no. I am terminal. I do not have a form of autonomic failure that has any way to survive or even extend life; except for the faint hope of IVIG, not currently studied in trial as not enough people can be collected or live to the end of trail period (usually 6 months to a year) to have a clinic trial. I am progressing at what appears to be 4-7 times the speed of other people with central autonomic failure. By pure stats, I should be dead. Plus I have peripherial autonomic failure, which is unheard of, but I still have both AND the neuropathy.

What can an autonomic specialist tell me? Maybe that someone else has what I have, or that it IS AAN or something new and they call it MSA-Q or they haven’t and they will want to autopsy me. Or maybe like this visit, they will end up with a list of what they know which still ends with “I just don’t know!”

Medicine isn’t coming back for me.

The question I need to answer is how much more time and energy will I spend, how much more money will I borrow, beg, put Linda into debt in order to find out a bit more about these diseases. I think Linda, Cheryl and others would like to at least a consult with an autonomic specialist. I would like what I fought for, and won, it turns out, a year ago: Treatment.

Was the IVIG withheld by the neurologist out of spite? The same neurologist who ordered another MRI because she said nothing would show up on an MRI but something did, so she ordered that section done AGAIN, and a year later it was determined to be ‘unknown.’? Why, why did my GP do it? Out of dislike? It is hard to believe that he would complain about needing guidance when he had it, that he would force us to spend money we didn’t have, money I had to beg publically for in order to get an opinion in the USA only from …confusion?

A year without treatment when I could have gotten better. It rings around in my head like pealing of the bells.

I asked Linda the point of chasing this specialist, then the Chicago program, then the Vanderbilt? She said, maybe for another woman, another generation. I said, with tears on my face, “It’s been over 100 years and they haven’t found anything YET.”

Yesterday was somewhat dodgy, hands black from lack of oxygen, arms and legs deep purple, much like this afternoon: TIA’s, stopped breathing, the usual. I have decided that if there is a visit to an autonomic specialist I will go, but that I want to try and set up the IVIG treatment as soon as possible, within six months if that is possible, even if I have to go to the newspapers. I want to gather reserves as much as I can and enjoy life while I have it. I know that every day, and every breath is borrowed time, that there isn’t any magic cure, or hope. And yet, I do have hope; hope that my life will be better. That I will be able to say “no” to the medicos, because they either accommodate me, or they just wait. Because either I live this life my way, or lose that chance forever.

Like the last 24 hours, there will be more depression, crying, and Linda and I holding each other. (3) But I am going on Friday to Sakura-con, if I am strong enough: I want to buy some art books for the blog. I want to buy some cat ears to send as presents. I bought some presents today in town to send out and when the total came up went there was a, “Wha?” moment. But it is okay, because this might not be what I originally sold my books for but this is who I am.

“Why,” asked the salesperson, “would you send presents to people you don’t even know, or have never met?”

I asked her if she remembered what it was like getting a surprise present as a child. She did. I said that many of the presents were FOR children and some were to help people remember that feeling of getting a present; the child-like wonder and anticipation.

This has been the worst couple of days as I can remember. And there is a lot more staring into space ahead. It is one thing to sort of know that you have a rather horrid death ahead and another thing to KNOW. To know that, no, they aren’t coming back to help or save you and from now on it will likely be more difficult, more painful, and yes, the road that leads to one end.
I AM trying to notice the flowers and the sunshine, and not just that ROAD, that destination. I got a bustier and thong underwear for $17 today! I have an outfit for sakura-con, and I want, I really, really want this to just be a fun time. To be a time I DO remember, one of the good memories of this summer. Since I am Not Dead Yet, and plan to stay that way (your not sending me heavy metal or radioactive cookies would HELP in that!). As long as I breath, I want to LIVE. Okay, I know what I have lost, I know what I am losing; now it is up to me to figure out how to make a life where I gain something, anything: from joy, from enjoyment, from just a smile.

My fucking ex-GP!!! Now there is a man who needs a postcard (and his license pulled, and a cell in the Hague for ‘Crimes Against Humanity’). Sorry, not quite all adjusted yet.

BP and brain problems: "Help, I've fallen and I can't get up!"

I have lay down and not been able to get up. It was light, now it is dark. They say tomorrow may be a storm, so they may not be chainsaws at 8:00 am. I don’t know. I am in the space “beyond.” I do not eat. I do not drink. I sleep. I wake confused. I sleep again.

I cannot remember things. I do not want to be one of these people who says they cannot blog because of the blahs. I do not have the blahs. My heart does not seem to be able to produce enough force to keep my blood pressure from dropping. This raises my heart rate. This used to be called Orthostatic Hypo-tension because it is a sign of autonomic failure when you stand up and your Blood Pressure drops and your heart rate raises to try and compensate. A change of 30 mlb is needed for diagnostic standard.

However, now, I am sitting, I am sitting with Linda and Cheryl, but I am dizzy, I cannot see from one eye. We take my blood pressure, the systolic is 120, then 108, then 95. It is dropping and my heart rated goes up and up 110, but with a heart that flutters instead of beats. The systolic and diastolic are close together – there is no pressure in my veins. There is no pressure to get the blood to my brain. This makes me dizzy and have other problems.

It seems that I now have autonomic failure of Orthostatic (change from a static position) Hypo-tension, except I seem to have STATIC Hypo-tension now as well. I don’t move and my heart cannot account for the change. What is the change? Unknown, some further failure in the vascular system, it appears. Would putting a pacemaker in make a difference. Without knowing the cause (are the veins just all opening simultaneously after I have been sitting still for 50 minutes?), how could a pacemaker compensate. Without getting the blood to the brain, I cannot function. Where do you go to buy a home centrifuge? That would force the blood to my brain.

I am having a time of it, I have pushed myself as hard and as far as I can, and when I figure out how to combat this invisible problem, I will be full steam ahead (maybe I need a steam pump for my head?). I do not know. Truth be told, is that I feel very u-h for the last long while. And I except I will be u-h for some time to come. I do not have Mono, as it becomes harder weekly to do the postcards, and less are done each week. When will I become rested enough to be ill?