Hi there, if you are treated with chronic ventilation (mostly not fulltime, but you always have it in your reach and use it when you need it), can you help me?

How does it work for you? (1)
Does it give you more strength / energy in general? (2)

For example:
I often have periods where I'm "near crisis" (though if docs didn't know I have this so very often, they would say it's a MG crisis). It means I have general weakness, mostly eyes to toes. Of course not all muscles are affected evenly, but still the only thing I can do is lay there, wait till it's subduces and not so very much else.
At those points my breathing problems get worse; not so much it will kill me or won't be managable with those nifty body systems, but still I do not get enough O2. I HATE that feeling the most: the feeling I can't breath. My diaphragm feels tight and like it's hard rubber; no way to expand (breath in) as much as I need.
Do you recognise this? (3)

At this point, if I use it: will it only help my breathing, or will it also give me more strenght in general? (4)

And what will it do if I use it at not so bad moments, but when I'm "just" out of breath? (5)

Does it give you more strength in your diaphragm? Does that feeling where your body tells you: "do not move or you will pass out" go away? (6)


Anyway, loads of questions...any information is very welcome.

FYI: my lungfunction is not good because of the MG. My saturation is on good times between 87 - 94 % on bad times it's worse.

[gr8ful]

Ravenclaw,
I'm not a doctor and surely don't want to offer 'real' medical advise but I think the following is true:

The body feels the urge to take a breath because it needs to exhale the CO2 that has built up in your body since the last breath. That feeling to take a deep breath is from too much CO2 in your blood rather than not enough O2.

I don't know how this might help you, but it might be something you could speak to your doctor about.

I hope you feel better asap. You deserve that, we all do.

[alice md]

Hi there, if you are treated with chronic ventilation (mostly not fulltime, but you always have it in your reach and use it when you need it), can you help me?

How does it work for you? (1)
Does it give you more strength / energy in general? (2)

I have been on intermittent respiratory support for nearly 5 years.
It led to significant improvement in my functional ability and quality of life.
It did take quite a while to find the optimal respirator and settings.


For example:

I often have periods where I'm "near crisis" (though if docs didn't know I have this so very often, they would say it's a MG crisis). It means I have general weakness, mostly eyes to toes. Of course not all muscles are affected evenly, but still the only thing I can do is lay there, wait till it's subduces and not so very much else.
At those points my breathing problems get worse; not so much it will kill me or won't be managable with those nifty body systems, but still I do not get enough O2. I HATE that feeling the most: the feeling I can't breath. My diaphragm feels tight and like it's hard rubber; no way to expand (breath in) as much as I need.
Do you recognise this? (3)

Yes.

At this point, if I use it: will it only help my breathing, or will it also give me more strenght in general? (4)

With the proper settings and use you will have both support in your breathing and less feeling of suffocation and overall improved sleep and strength. ( Don't expect to run the marathon, but you will do better).

And what will it do if I use it at not so bad moments, but when I'm "just" out of breath? (5)

With time you will learn when and how to use it, just like you know when you should eat or drink.

Does it give you more strength in your diaphragm? Does that feeling where your body tells you: "do not move or you will pass out" go away? (6)

Yes, yes and yes.

Thanks so much!

I'm just going to ask my doc, see what she says. Here, there is no one who gets intermittent resp support, it is never used as a treatment for MG.
So, it is very different and it is expensive.

But,for me it just seems like a healthier next step.

Alice, do you have any advice I can use to convince my doc (she's saying; it's going to be rituximab and/or prednisonecocktail)?

[alice md]

Originally Posted by Ravenclaw

Thanks so much!

I'm just going to ask my doc, see what she says. Here, there is no one who gets intermittent resp support, it is never used as a treatment for MG.
So, it is very different and it is expensive.

But,for me it just seems like a healthier next step.

Alice, do you have any advice I can use to convince my doc (she's saying; it's going to be rituximab and/or prednisonecocktail)?

First, it is eventually less expensive than other treatment options.
It is (unfortunately in my opinion) not commonly used as treatment in MG because there is not enough awareness of the importance of adequate respiratory support in this illness. Most neurologists see it as black and white-crisis or not crisis.

I too did not receive respiratory support for MG, but for an "unexplained neuromuscular disease" causing recurrent respiratory failure for which there didn't seem to be any effective treatment. There are still neurologists who would argue that the diagnosis of MG is my case is questionable, although many do accept it now. ( it is based on direct measurement of respiratory muscle force instead of indirect measurement by SFEMG and on anti-MuSK antibodies that were not found in the usual assays, but in a cell-based assay which is still considered experimental)

Regarding NIV, one thing you have to understand is that (as my respiratory physician recently put it nicely) even the most sophisticated respirator can't replace the normal natural pattern of breathing. So, possibly it is not such a bad idea to try and attain remission with Rituximab (if possible).

Those are obviously not easy decisions, and regardless I think you can benefit for now from proper NIV support.
BTW, respiratory support is being used in certain forms of congenital MG in which there is resp. muscle involvement, so I don't see why not in autoimmune MG. Also, NIV has been used successfuly in autoimmune MG to prevent need for intubation in patients with respiratory crisis.

Good luck and hope you feel better soon,

Alice

[StormE1]

I realize this is a little off topic, but I have had such good results with my CPAP (Constant Positive Air Pressure), that I wondered it if might not be a good support for your diaphram?

I know when i'm short on oxygen, i get "stupid", it definitely affects my cognition; as opposed to when I am high in lactic acid in the muscles, which is much more a feeling of panic. In my case both are the result of the arterial system's inability to uptake an enzyme that keeps it flexible and permeable, so gasses, nutrients and wastes can pass the capillary walls.

This means i have trouble getting oxygen in as well as getting CO2 out, and nutrients in, and wastes (such as lactic acid - a byproduct of muscle exertion) out. I always favor a treatment that does not involve drugs, if at all possible. I hope you find something that lets you breathe, i know that terrible feeling of gasping.

Best Wishes
StormE1