My name is Linda and I work with the MGABC
I first noticed that I had a problem with my left eye lid drooping. This was the spring of '99 and I put it off to
being a bit overweight and didn't think anything more of it. I did notice that I had some double vision during that summer.
Not double vision like seeing 2 separate images, but more of a shadow image. This I put off as a scratch on my contact lenses.
One day the end of oct. '99 I was putting in my contacts, and realised that I was looking at a double image of my finger holding
the lense, before it was in my eye. Therefore it couldn't be a scratch on my contact lense as I thought. I had also noticed
that I was being more of a 'slug'. I would fatigue when I did most things, especially when I washed my hair; I'd have to use
only one arm at a time while the other one rested. I'd have to stop and rest my shoulder 3-4 times when I was brushing my
teeth because my shoulder would get so weak.
I called my primary
physician who saw me right away when she heard of my double vision. She did a brief exam, said it was neurological not optical
and which neurologist did I wish to see? (I am an RN and worked in the ICU, so knew most of the specialists) She spoke on
the phone with neurologist, he suggested I try Mestinon. I went home, took 30 mg of Mestinon and waited 40 minutes. Then I
went to brush my teeth to see if there was any difference. Not only was my droopy eye better, but I could brush my teeth with
out stopping. I didn't feel like a 'slug' anymore, I felt like my old 'normal' self.
I saw the first neuologist with in a week. He did a repetitive nerve stimuli test on my wrist, then on my face. This is non-invasive
and has 2 sticky patches stuck about 2" - 10cm apart and electrical stimulation in series of 10 or so are applied. A
normal test shows the same amount of muscle response to repeated stimuli. A test in MG patients should show a lessening muscle
response in repeated stimuli. My response was normal. (This normal response I realise now was something that would follow
me through all tests.)
I had an MRI of my head early on new
years eve '99, it too thankfully was normal. In Jan. '00 I was seen by another neuro (neuro #2) at Vancouver General Hospital's
Neuormuscular diseases unit. There I had another physical exam, which so impressed the neuro that he called in his residents
to have a look and told them this is what MG looks like. I was stsrted on prednisone at this time to try and supress my immune
system,I had another repetitive nerve stimuli test, again normal, then I had single fibre electromyography. (SFEMG also known
as a jitter test) I was normal for this test. A very narrow needle is inserted under the skin in to a muscle and the muscle
is tested while being used. Again, I tested normal. I also had blood work to test for the Acetlycholinesterase Receptor antibody
(AChR) I was negative for this as well. It is known that approximately 20% of myasthenics are AChR antibody negative. I was
told that I have Myasthenia Gravis as my symptoms are classic. I was referred to a neurologist at the University of British
Columbia, who was at the time the recognised MG specialist in BC if not western Canada. He also was and is continuing to do
research into MG.
At UBC I saw neurologist #3, who also
did his exam, concluded that I have MG, solely based on my symptoms and response to Mestinon. I had a CT scan of my chest
at this time to look at my Thymus for tumours. The thymus lies under your breat bone, is used to get your immune system up
and running as a child, then should shrivel up and is not used past puberty. Most people think that the AChR anti body is
made in the thymus. My thymus was clear of tumours, but large. The plans were made to remove my thymus in hopes that I would
have less MG symptoms.
By mid April '00 I was weaned
off of the prednisone, and I was was given 3 runs of plasmapheresis, or plasma exchange as it is also called. One treatment
each for 3 days on the 3 days immediately before having the Thymectomy. The plasma exchange takes about 3-4 hours, and your
blood is removed, separating the plasma from all the cells. The cells are returned with new IV fluid and plasma protiens,
while the old plasma, supposedly carrying the AChR anitibodies and other antibodies that are not yet found that may cause
MG, is discarded. After I had these treatments, I felt like a new woman. I could go for a long walk and not have my legs fatigue.
I could hold my head up straight with out my neck getting sore form holding it up. My eye lids were up, and no double vision.
Hooray!!!
I had my Thymus removed by a sternotomy approach. That's cutting the chest and breast
bone open. I spent an uneventful 24 hours in the critical care recovery room, then was returned to my room. I was in hospital
a total of 2 weeks. Most stay for a shorter time but I had a couple of complications which I won't get into here. At the end
of the 2 weeks in hospital before I was released, I had 4 days of IVIG.
I am unable to say
what made me feel much better: the plasma exchange, the thymectomy or the IVIG, but I felt great for 9 months.
By the Spring of 2001, I was having a hard time holding my head up, my ptosis and diplopia were back, and it was
a challenge to walk any distance more than 15m.
I was now starting Immuran, another immunosuppressant
to try to suppress my immune system. It was suggested that I also restart prednisone but I was very resistant to this. Unfortunately
I was not able to take Immuran for more than a month as my liver enzymes, that tell how well the liver is functioning, rose
10 times the normal in that first month. If I stayed on Immuran, I would have severely damaged my liver. I tried another couple
of days of IVIG, but the benefits only last for a few days to a week at most. I wasn't getting any better, just weaker.
During the fall of 2001, I started Cellcept. It's another immunosuppressant used in transplant that has shown promise
when used for as treatment in other diseases where immunsuppression is needed. I started with a dose of 1000mg twice a day.
Over the next 2 years, my dose went from there to 1500mg in the morning and 1000mg in the evening to 1500mg twice a day, for
a total of 3000mg or 3 grams a day. I was also started on prednisone again sometime early 2002 I think.
I continued to work, albeit it was getting harder and harder to work. one time while I was on the Cellcelpt and prednisone
I had a stress fracture in my foot and needed a cast for 6 weeks. It is a good reminder that even when taking extra Calcium
and Vit. D. when on prednisone, that these things can still happen.
In 2003 I was able to
reduce my work hours to 3/4 time while keeping a full time position by taking either holidays, or unpaid time. This enabled
me to work a little bit longer. I was encouraged by my physician that I should be off work and resting, but I'm a wee bit
stubborn. I really enojyed my job and I think part of me thought that if I 'give up' then I may never return to my job. By
the end of 2003 and early 2004, my local neurologist had tried as best he could to get me into some kind of state where I
had some strength and stamina.
The end of June of 2004, is when I worked my last shift. I
was at work, but so weak the it was hard to stand, hard to hold my bottom jaw shut, I couldn't see well because ofhte double
vision and both eyes were dropping. That wasn't out of the ordinary by this pint, but I wasn't strong enough to remove the
screw on lid of a water bottle. In my little brain, I thought if I'm too weak to do that, I shouldn't be here. I haven't mention
yet, that I am a registered nurse, and I was working at the time in an open heart intensive care unit. I was expected to and
was providing life support to these patients, and yet I could barely stand and swallow. It was at this point that I left my
job to take care of myself.
My last IVIG was in August 2004 and I ended up with aseptic meningitis
from it and no benefits from the IVIG at all. It's at this point that he referred me back to the neuromuscular diseases unit
in Vancouver. The neurologist that I saw there, in my opinion, was very narrow minded and didn't like to think outside the
box. I was told that I didn't have MG. In fact this neuro didn't know what I had, other than MG but it couldn't be MGbeause
I was negative for AChR antibodies, and anti MuSK antibodies. My SFEMG was 'normal' but my clinical signs said MG. I was told
to come back when I had something that I could be treated for. I made a request and was granted to be seen by the neurologist
at the University of BC, that confirmed me having MG back in early 2000. He reviewed what has gone on over the last 6 years,
did another physical exam, and reiterated that "of course I have MG." He worked ordered a 3 day treatment plan of
plasmapheresis, over a one week period. This was done in early march 2005. To try to convince the neurologist at VGH that
I did in fact have MG I was to be seen before and after the treatment so that the changes in my condition could be documented.
This was done, after much hassle and a return trip tothe city, but it finally did convince the neurologist at VGH that I did
in fact have MG and that I just don't present like anyone else. I have chosen to have the neuro at UBC be my primary neuro
for the present time being.
I have not been having a good time of it recently. After the plasma
exchange in early March 2006, I had almost 2 weeks of great stregnth and stamina. It was wonderful to be alive, go for walks,
do chores around the house and yard, and just be! Mid March I was started on 125mg of Prednisone a day. It's a very big dose!
I has like a maniacal tasmanian devil. I had days were I could do anything for acouple ofh ours, then I'd have to rest. I'd
rush out and weed the garden, then plant mroe plants, then vacuum the hosue then wonder if I could paint the inside. Well
it wasn't quite like that, but it gives you an idea of what it was like. That lasted for about 10 days. Then, I've continued
on that dose of prednisone,and Cyclosporine was added. 75mg twice a day. Cyclosporine is another drug used in transplant,
to try and suppress the immune system. It's older,and has more side effects than Cellcept, so is used less often by most physicians.
I was one this for 2 weeks, and had to increase the dosage up to 125mg twice a day, keeping the prednisone also at 125mg a
day. I have to admit that by mid April 2006 I am feeling very unwell. The prednisone has made me very irritable, and gives
me great trouble sleeping even with prescription sleeping pills. I don't know if the tremor I feel is from the prednisone
or the cyclosporine. My hands, feet and tongue were getting numb, (from the cyclosporine) and I was having urinary urgency
with at times incontinence (from the cyclosporine), geez, I'm only 46, much too young to think about diapers! The worse I
htink was the bloating and gas pains. By The end of the day I was so bloated I would look like I was having twins at full
term. I was so bloated that I'd be short of breath. I still had droppy eye lids, double vision, weak arms and legs, to the
point where washing my hair and brushing my teeth are chores that I ofter have to rest at while I do them. I often have my
lower jaw hanging open because it takes too much effort to close it. I have to be careful of the foods I eat so that I don't
mix consistencies and choke. Rice often, if it's dry, makes me choke. I will often have to cough at the end of a meal, as
the food just sits in my throat if there's no more to push it down. This was documented on a swallowing exam with barium.
My dog now gets drooled on by me when I bend over him to say hi, as the drool comes out of my mouth onto him. I think it's
only fair... On a good day I can walk slowly on even ground for about 45 minutes before I've reached the point
where I don't know if my legs will hold me up.
The neuro at UBC thinks maybe I was on too
much prednisone and has reduced me down to 100mg /day. The cyclosporine has been stopped since the 26th of April. For a week
now my BP has been elevated, more in the evenings that in the day time. My usual before it rose was 100/60 - 110/70 Now it
averages in the 155-85 during the day and frequently 160-98 by evening. I will see my GP next week and speak with the UBC
neuro the same day. It's a never ending story! All I want is to feel better. I also received in the mail this week my papers
for reassessment with the long term disability insurance people. Only time will tell when will happen with that.
I hope I haven't bored you with my tale, and I'd be more than happy to have other's stories up here as well. Just
send them to me and I'll post them here.
Sept. 6/2007 Another update! I wish others would
send me their stories... Anyway, in May I was so weak that I was needing a cane to get around. I fell a couple of times, mostly
because my legs just gave out from under me. My arms were weak, activities of daily living were really a challenge. eating
was taking a long time, approx. an hour to eat a supper meal because I had to go slow so I wouldn't choke. I did manage to
convince my GP to contact my neuro in Vancouver, and after speaking with him, arranged a course of plasma exchange of 3 treatments
over a 5 day period.
These treatments were difficult only because I have such poor veins,
I was black and blue by the end of the week. I saw the neuro at the start of the week, and at the end, for comparison. Unfortunately
I didn't have any noticeable improvement following the plasma exchanges. At this point, my neuro has UNdiagnosed me again
but remains doubtful of whatelse I could have. He tried to send me to another MG specialist at UC Davis in Sacramento California,
but the trip was not approved by my insurance. I now have an appointment with another MG specialist at the University of Western
Ontario in London Ont. for later in Oct. 07. Until then I continue with prednisone, methotrexate and mestinon which funnily
enough does slightly improve my muscle strength.
It's now a month since I've seen Dr. Nicolle
at UWO in London Ont. I admit that he did a very thorough history and exam. I also had a total of 4.5 hours of nerve testing,
electromyography and Single fibre electromyography. The end result was he's 90% sure that I do not have MG. He did admit that
I may be in the 10% of MG patients that test negative for all antibodies and electromyography, with little responce to medical
treatment. I was supposed to have a muscle biopsy, but he could only do a needle biopsy and he says that because of the amount
of tests needed on the muscle biopsy, an open biopsy is needed to retrieve a larger sample. Since I've returned from UWO,
I'm still waiting to hear from the Geneticsist and or my neuro in Vancouver.
Another update
will come once I've had the results of the muscle biopsy which will probably take place after the new year.
So now it's the end of March 2008 and I am still waiting for the myscle biopsy.
It
has been rescheduled to April 14th. I imagine it'll take over month to get results.
Unrelated
to the 'MG' that I've not got, I have a genetic increase in my factor VIII on the hemophelia panel, which means that
I'm at risk for clots. I've already had clots in my leg and one travelled to my lung in '90. Now I'm being investigated to
see if I've had a Transient Ischemic Attack (TIA) I'll keep you posted.
April 14th 2008. I've
had the muscle biopsy, taken from my left bicep. Thankfully it's not been as sore as I was lead to believe. Now the wait for
the results begin. Since coming home from Vancouver, the fatigue has hit and it's all I can do to summon the will and strength
to walk the dog.
May 9th 2008, no word yet on the results of the muscle biopsy. I've been
off prednisone now for close to a month. I notice now that prednisone did have some benefits; I wasn't quite so weak and it
sure lessened the amount of muscle, joint and headaches that I now feel.
May 15th/2008
THE MUSCLE BIOPSY RESULTS are back. The good news is that everything is normal. Cell shape, size, function including
the mitochondria are all normal. So now I'm back where I was 9 years ago. Same symptoms and no diagnosis. My GP is wondering
about Fibromyalgia, so I am starting physio and accupuncture next week. I'll keep you posted.
Sept
11/2008
I've stopped going to physio because it was causing much more pain. My Dr. is treating
the symptoms while not knowing what the cause is. I have a referral to a pain clinic and I hope to get a referal to a Rheumatologist.
It's not a rough summer with lots of pain, constant fatigue (I still have the MG symptoms) and frequent headaches.
