[AnnieB3]

Hi. I just saw your post in the "sticky" thread above. Wow, you've been through a lot!

Hi folks, I'm new! I am a grandma who hasn't felt well for well over 40 years. I had to quit work as a nurse in 2003 due to my health. A couple of weeks later I was so ill I could hardly get out of my recliner.

I have doctored for years and had gotten to the point that I was darned if I was going to die without a diagnosis. The doctors always said it was in my head. Finally got diagnosed with sjogrens in 2003 by lip biopsy---negative blood work though.

I was very ill much of the time and had lots of bad infections--MRSA and I also had Mycobacterium kansasii (a form of noncontagious TB) and had to be treated for one year with 8 antibiotics pills (3 different kinds) a day.
Eventually I was sent to a very good immunologist who changed my life. He believed me!!! His FNP did my work up and read through the 11 pages on one side, that I had written for them plus all the paperwork they had for me to fill out. The immunologist introduced himself the first day for a few minutes and in those minutes he told me he thought I had myasthenia gravis.
I had the nasal twang which I have had for more years than I can remember. I also had a little drooping of the eyelids. I could not believe it as I had been questioning docs for years and they would all tell me I didn't have MG or sjogrens.

Well, the immunologist also did a whole bunch of blood work and it turns out that I have extremely low t-cells. Like as low as an AIDS patient. This low cell count, however, is related to the autoimmune disease. I also have Bullous Pemphigoid and asthma.

This low t-cells count really explained all the infections I have had since the 1970's. He also diagnosed my Hashimotos and my titers were off the charts. I am entering my 6th year of IVIG as I can't have Imuran, etc due to the chance of getting bad infection. I just started Plaquenil this past summer and it has helped my energy level and decreased some of the bad swelling in my sinuses, nose and throat.

I will be visiting on occasion and am interesting in hearing about new treaments and other MG issues. I have never had a crisis for which I am extremely thankful. I have told doctors for years that I often felt like I would quit breathing. Now I know what that was all about. Be back later. gto

I read this in Violet4941's post and wanted to put it here.

He was very worried about me as my antistriated muscle antibodies were and are 61,000 and he just thought I was headed for trouble.

Have you had a chest CT done? Do you know the levels of your ACh Receptor Antibody test? Did you have that done? With such a high antistriated muscle antibody count, I was wondering if you might have a thymoma. Have they ruled that out?

Did they do a biopsy to confirm the bullous pemphigus? I was wondering because discoid lupus can present like that.

With the low T-cells, have they come up with any immuno therapy for you other than IVIG?

There are a lot of well-informed, supportive people here. It's a great place to share experiences!

Annie

[69gto73]

Originally Posted by AnnieB3

Hi. I just saw your post in the "sticky" thread above. Wow, you've been through a lot!



I read this in Violet4941's post and wanted to put it here.



Have you had a chest CT done? Do you know the levels of your ACh Receptor Antibody test? Did you have that done? With such a high antistriated muscle antibody count, I was wondering if you might have a thymoma. Have they ruled that out?

Did they do a biopsy to confirm the bullous pemphigus? I was wondering because discoid lupus can present like that.

With the low T-cells, have they come up with any immuno therapy for you other than IVIG?

There are a lot of well-informed, supportive people here. It's a great place to share experiences!

Annie

Annie, I am not sure how this "reply" works on this board. Hope this is going to work!!!!

Anyway, my Ach receptor antibody is negative and has been everytime they check it. I have had thyoma ruled out also. O have had many chest x-rays done and numerous chest Cat scans. I go to an immunologist who is a pediatric oncologist pathologist. He is also involved in international immunological studies and has worked on bone marrow transplant teams. He is very knowledgeable.

Anyway, I had the Bullous Pemphigoid biopsied in 2002 by my Dermatopathologist. I had an upper arm that was about 4+ inches in diameter and red hot when I saw him. I really suffered with that bugger. He also cultured the 12 cm bullae on the back of my arm and I had bacterial and fungal infection.

I have done quite well on the IVIG and my MG is so much better. I had a terrible summer this year and when it cooled off I was much improved in 3 days. My neuro had upped my prednisone for a few months and I am in the midst of tapering off some. I have been on Prednisone 10 mgm every other day for about 6 years. My neuro likes to keep me on it so that I have a little protection for these bad times. I think she is right!!!

I saw where someone asked how you would know when you were having a crisis. Now, you need to realize that I have dealt with my condition for many years and researched a whole lot. Also, my neuro and I have a great relationship and she is a woman. She told me that when I woke up in ER on a table looking at the lights on the ceiling with a tube in my mouth I would know I had a crisis.

I took that with a grain of salt and a laugh as I knew what she meant. Crisis can happen fast and unexpectantly and we better be on our toes.

Anyway, I am looking forward to visiting here and taking opportunity of all the information.

I should add that all of my autoimmune blood studies are pretty high and my doc is amazed that I am doing as well as I am. Some people have relatively low titers and can be very, very sick. Thanks 69gto73

[alice md]

She told me that when I woke up in ER on a table looking at the lights on the ceiling with a tube in my mouth I would know I had a crisis.

I really hate it when neurologists give those kind of answers.
This doesn't mean that your neurologist is not a wonderful physician and person, it just means that this is the way neurologists look at this, which is (in my opinion) wrong.

It's true that we can't always predict serious and life-threatening complications, but many times early recognition and awareness of physicians and patients for ominous signs can prevent them.

Myasthenic crisis requiring respiratory support rarely just happens with out previous warning signs. There are steps that occur with respiratory muscle weakness. At first there is a feeling of breathing difficulty, compensation with rapid breathing and only after this is exhausted is there a gradual decompensation. At first leading to CO2 retention and only at later stages also hypoxemia, which untreated can lead to irreversible brain damage or even death.

Recognizing those signs on time and resting/cautiously increasing meds. may help avoid further worsening. If not, non-invasive respiratory support can be used in a significant number of patients who have respiratory compromise but a patent airway. Intubation should be kept as a last resort in patients that do not respond to any of those measures.

In the past, patients with significant respiratory muscle involvement would have a trach. so that they could easily receive respiratory support when required in their own home. Nowadays, the same can be done with a face mask as long as the patient is conscious and cooperative and and there is no obstruction of the airway.

Even patients with ALS, can be managed like that continuously for prolonged periods of time. So, obviously someone who has a transient weakness of his/her respiratory muscles and requires respiratory support for a few hours or even days.

[69gto73]

[QUOTE]

Originally Posted by alice md

I really hate it when neurologists give those kind of answers.
This doesn't mean that your neurologist is not a wonderful physician and person, it just means that this is the way neurologists look at this, which is (in my opinion) wrong.

It's true that we can't always predict serious and life-threatening complications, but many times early recognition and awareness of physicians and patients for ominous signs can prevent them.

Myasthenic crisis requiring respiratory support rarely just happens with out previous warning signs. There are steps that occur with respiratory muscle weakness. At first there is a feeling of breathing difficulty, compensation with rapid breathing and only after this is exhausted is there a gradual decompensation. At first leading to CO2 retention and only at later stages also hypoxemia, which untreated can lead to irreversible brain damage or even death.


I can understand your opinion regarding this subject, however, it didn't bother me at all. She has explained things to me over the years and I just asked the question to see if there was something new I didn't know.

I am informed about my medical condition and the minute I get weakness I pop a mestinon and sit down and rest. I have learned over the years how my body reacts and how fast it recovers. I take 15 mgm of mestinon at a time cause a whole tab makes me feel a little weird. If I don't start to recover from the weakness within a certain amount of time I will take the other half of the pill and that usually does the trick.

Some days I will have enough weakness that I just sit on the couch and rest. Other days I may just have a few tabs and that is the end of it. Some days I don't need any at all.

I live in a smaller community and have made sure that my hospital/clinic records are up to date with my myasthenia diagnosis so they know if I come in with severe weakness and respiratory distress that I am in crisis.

I also have a very good sense of humor and have found that it is the one thing necessary to live this life with chronic disease. Besides having about 5 autoimmune disease diagnosis and low t-cells I also have a hubby with celiac disease, microscopic colitis, COPD, Lung cancer 2009, CVA 2010 (atrial fib), who has also survived 2 back surgeries with resultant 4 severe infections (meningitis, cellulitis, discitis, septicemia all at once) which resulted in 75 days hospitalization. He was much later diagnosed with hypogammaglobulinemia. He has some other serious surgeries that resulted in me caring for him for many months and I am still hie caregiver even though he is up and about.

We have had a pretty sick household for many years and have learned to endure. I don't get too bent out of shape these days about most things. There is no way one can control all their health issues and sometimes to laugh is the only way to exist and cope. I am very thankful for my nursing background which has prepared me a lot for all that has transpired.

I have been a moderator on another forum at one time prior to being inundated with all the health issues of hubby and I. I am not trying to be a smarty pants, but just want you to know that I probably operate differently than some people. I don't know it all by a long shot, but I have learned a whole lot in the past few years just by virture of personal and family experience. The possibility of death has been present several times in our marriage and though not desired it is accepted as the possible/probable result of some of the illnesses. Thank you for taking time to respond to my post. gto

[alice md]

I can understand your opinion regarding this subject, however, it didn't bother me at all. She has explained things to me over the years and I just asked the question to see if there was something new I didn't know.

I am glad. Probably in the context of your discussion it sounded different.

I realize from what you say that you have a very good relationship which allows a bit of joking.

I believe that some of the jokes I have with my patients would sound quite horrible out of context.

I agree that sometimes to laugh is the only way to exist and cope.