Respiratory Weakness Early- Typical Path

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ersmzd

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I read in another thread that there are a few on here that had respiratory issues early on and they all seemed to follow the same path, but I haven't found a post that talks about how the disease progressed. My Mom had a FVC of 34% when she was diagnosed last month. Her symptoms started with her voice about 10 months ago and breathing followed around 6 months ago. Can anyone share the path theirs has taken when breathing shows early signs of weakness? Thank you!

~Elizabeth
 

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I'm not quite sure what you are asking.

I think the first thing I have to say is that every person with ALS is different. There is not really a 'typical path'.

There are however some things we can try and help with.

If respiration is progressing rapidly then bipap is probably the most important thing to offer your mum as early as possible. If she is in favour of bipap, then using it as often as possible should be encouraged. Often a couple of different masks can be used between day and night to help cut down the pressure in the one place.

Resting as much as needed should be encouraged as fatigue is a part of ALS, but when breathing is affected it is huge.

Is your mum considering a trach and vent?

Some PALS elect for no interventions, some for a mixture of different interventions and getting it clear what you mum wants is really important so that you can help her in the way she wants.

My husband was also bulbar onset, he had a peg, but still lost weight rapidly and he did not have a bipap. He passed away 11 months after diagnosis due to respiratory failure, very peacefully at home.
 

lgelb

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Hi, Elizabeth, that pattern is called bulbar onset because of the muscles that are affected first. A BiPAP is certainly called for when breathing is compromised like your mom's. When she has difficulty eating and drinking, she will need to decide about a feeding tube as well. She will probably have problems with her arms, legs and/or trunk soon if she hasn't already. Does she have another way to communicate besides her voice, like a tablet?

Bulbar onset does tend to progress faster than limb onset, so you will want to work with her and your other family(?) to stay ahead of equipment she needs and planning for the future. Please feel free to ask any questions here.

Best,
Laurie
 

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Bulbar and Respiratory onset are two different things. I know "early" on in ALS they where considered the same. Most docs however now see them as separate. It does sound like your mom is more on the Bulbar side. I was Respiratory as was Gooseberry's husband, Steve. We seemed to follow very similar tracks but that really does not mean anything. He was diagnosed 6 months or so before me and it has been longer than that since he passed. My breathing is currently considered critical and I am on the edge of completely loosing use of my legs and left hand. I can still talk but have begun slurring and mumbling with what feels like "fat" tongue. So my path has been Respiratory, to Limb, to Bulbar.
 

ersmzd

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She has a bipap and uses it at night only. Peter, what was your FVC when you were diagnosed vs now? My mom's first als clinic is Monday so we'll get to hear all our options as well as see how the numbers have changed since she was diagnosed a month ago. Mom has lost one muscle in her left hand but otherwise her abilities remain mostly the same, just a tad slower. I've tried to get my dad to have the conversation with her about a feeding tube and venting, but he hasn't. Hopefully after the clinic they will be more open to discussing things.
 

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Elizabeth,

As we frequently note, FVC is not a good marker for life left. Ask about testing SNIP for a better, though still not very solid, picture. The best guide is how comfortable the PALS is with BiPAP and the extent of perceived respiratory distress/discomfort. Frequently, BiPAP settings need to be adjusted over time (which you, the caregiver can do), but which doesn't always happen.

Venting (most commonly used as shorthand for a tracheostomy + 24/7 invasive ventilator use) is a major decision and I wouldn't spring it on anyone in the early months of diagnosis. FYI, most PALS opt out of that. I'd also suggest further research before discussing.

Best,
Laurie
 

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I want to strongly reinforce the message provided by others who are far more knowledgeable than me. Each MND case is different. Looking for patterns may be useful for researches, but I don't think it offers much value to PALS and CALS, especially when considering FVC.

With that caveat, I will share my experience.

My first Pulmonary Function Test (PFT) was over 5 years ago and showed an FVC of about 60%. This was a huge surprise, as I had not yet been diagnosed and did not have any complaints about breathing at that time.

I was diagnosed about 2.5 years ago. By that time my breathing had degraded to where they were no longer doing full up PFTs (FVC was about 31%). Those tests were just too hard for me and I really appreciated the pulmonary doctor switching to just a screening spirometry, which is done at every appointment.

Breathing is the most rapidly progressing thing for me. My FVC is now at 23%. I do not struggle to breath, I just can't take a deep breath.

My view is that the FVC is an interesting number but is not very valuable in predicting how much time I have left or the quality of that time.

I choose to live my life the way I live it without regard to the FVC score. I am slowing down, but remain quite active. I plan to continue doing all that I can do every day.
 

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As Pete said, respiratory onset is a different animal. Steve presented with a paralyzed left diaphragm, and shortness of breath,.

Like others have said, there is no predictive course. My husband had periods of rapid progression and stability.

He had mostly lost the use of his legs but didnt want to be pwc or bed bound so he tried to walk with varying success. By this time he was 24/7 on his trilogy unless he was walking.

Everyone follows their own unique course.
 

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Thank you for sharing.
It was very encouraging. :)
 

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I would also add that at least per our Doctor, true Respiratory onset is very rare in ALS. The majority of cases that are not limb onset are Bulbar. I have met one person with respiratory onset in our local group, and two years post Dx he has very little limb or speech issues but his Dr. believes he has at most six months at this point. It is different for everyone.
 

tripete

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I would also add that at least per our Doctor, true Respiratory onset is very rare in ALS. The majority of cases that are not limb onset are Bulbar. I have met one person with respiratory onset in our local group, and two years post Dx he has very little limb or speech issues but his Dr. believes he has at most six months at this point. It is different for everyone.

This is true. I do fall into this category, as did Steve. The BiPap has kept me going despite a negative number MIP for 9+ months.
 

Lkaibel

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It's always good to hear from you Pete. I am glad you are still kicking.
 

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I don't know if this will be of any help to you Elizabeth . My husband presented with complete respiritory failure before there was any suspicion of a neuro muscular problem. He was trached and vented almost immediately, then ALS was diagnosed. It's path was respiritory, limb, and then bulbar.
 

ersmzd

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I know it's been said FVC is not a good indicator, but It seems hers has rapidly declined. Looking back at her first testing for shortness of breath, it was 61%. This was in early October. Late January was 34%. This week the guy said 16%, but honestly he didn't seem to know what he was doing. He said her FEV was twice what the FVC was so maybe her FVC should be higher. Because her respiratory numbers are so low they said it's now or never for a PEG. She is getting it Thursday morning.

Thank you all for your replies.

~Elizabeth
 

lgelb

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Elizabeth, because she has trouble breathing and does not seem to have a BiPAP as yet, she would likely do best with the least sedation required, which would mean doing the procedure in Interventional Radiology instead of a surgical suite, as is most often recommended in ALS anyway. Is that the plan?
 
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