Possible PBP/ALS, need help!

[BernardBlack]

Hi,

My mother is being investigated for ALS. Our next appointment is in two weeks and her condition is deteriorating rapidly. I need some help.

Preface
Problems swallowing food since about a year. Has gotten much worse in the past 1-2 months. Full body EMG showed nothing. MRI shows no tumors. We haven't had bloodwork done to rule out infection, but she's been self-medicating with antibiotics/antivirals without any improvement. Camera exam of throat has shown double paralysis on the inside (don't know what the muscle is called but it affects swallowing). No bodily symptoms apart from the swallowing problems.

For the past two weeks she's having difficulty breathing and sleeping. Sleeping works best sitting up. The snoring sounds are not normal.

First and foremost, I am really scared that she will just die one night. Is this a possibility?

Second, if anyone has had experience with similar progression, please share your thought. If this turns out to be ALS, what kind of life expectance are we looking at with these serious bulbar symptoms?

I appreciate any input. Thanks beforehand.

PS.
Due to how the medical system works in our country, it is not possible to get an earlier meeting with a doctor unless you go into intensive care.

 

[Barbie]

Hi Bernard,

First, sorry you have to be here looking for answers. I just want you to know that while they are looking at ALS, it is still possible that it can be many things and you need to hold on to that hope.

You don't need to worry about mom dying one night out of the blue ==at least from ALS. If she has ALS, it doesn't work like that and doesn't happen that fast. And, if she does have ALS, no one can give you a life expectancy. the docs will say 3-5 years, but it could be quicker or slower. Try to just be there for her now and give her support. try to make her more comfortable, and keep on the doctors. One thing I noticed is that you said that an EMG showed nothing. in ALS, the EMG is a good test for ALS. A clean EMG means no ALS.

 

[Mediasmart]

There are EMG's and then there are the real deal EMGs. I went though two negative EMGs by a well meaning examiner; neither showing much of anything. It was hidden in the neuropathy. When the general symptons worsened and I was sent to an ALS clinic with very good equipment and personnel is when things were sorted out.

Just suggesting if affordable and geographically possible, go to the best clinic you can find.

 

[BernardBlack]

Hi Bernard,
...

Thanks for your answers Barbie. That makes me feel a little bit more reassured, at least for now.

There are EMG's and then there are the real deal EMGs. I went though two negative EMGs by a well meaning examiner; neither showing much of anything. It was hidden in the neuropathy. When the general symptons worsened and I was sent to an ALS clinic with very good equipment and personnel is when things were sorted out.

Just suggesting if affordable and geographically possible, go to the best clinic you can find.

Mediasmart,
We live in a country with socialized medicine, which makes it difficult to get a second opinion, especially for EMG tests, which is available in only a few hospitals. So we have to trust the doctors giving us the "real deal" EMG, as you call it, even though I don't know what you mean by that. But she has only done a full body EMG, which I have seen around the forums is not always conclusive.

 

[zoohouse]

If you know of a really good hospital that has excellent neurologists, maybe one associated with an ALS clinic, you could just present to the emergency room, stating that she is having severe difficulty breathing, especially while laying down. You can mention that she has had a gastroscopy and its findings, and even the negative EMG, but that the symptoms are worsening severely.

Generally ERs do not get into a lot of testing of chronic illnesses, but will most definitely deal with a dangerous situation like difficulty breathing. They may admit her so that the neurologist can see her, and once in hospital tests are expedited to try and clear the beds. I hope this helps you.

Canada has socialized medicine of a sorts, and when I didn't have confidence in a specialist that I had seen, I spoke with my general practitioner and asked to be sent to different one. She has been a very good advocate for me, but admittedly being a medical professional does make a difference.