Early breathing difficulties

[Jimmyccorn]

Hi. I 67M here diagnosed last August. Still mobile, but left arm and hand most impacted where my onset was. March 18th I woke up with breathing issues. Got appointment with RT and my sitting FVC remained 66% but supine had falled to 55%. Now I am on a ventilator 9 hours a night with an hour or two in the afternoon so I can talk better. No bulbar issues. Is it common for breathing to take such a hit before other limb disability? I fear I may exit this world with most function except breathing!

 

[Nikki J]

There aren’t any rules for this. It is good you have respiratory support now. Some people find using it stabilizes breathing for a while.

 

[Jimmyccorn]

Thanks Nikki. I am aware of the “no rules” rule and my brand new $40k Swiss made Nocsn ventilator ( free rental via Medicare/AARP Plan G) is a marvelous machine! I just spent $15k for a new roll in shower on thefirst floor and I want to live long enough to need it…..ALS is more unpredictable than the tariffs!
Thank youor allyur contributions to this forum!

 

[JAM]

You say no bulbar issues, but then say you use vent to speak better. I thiyght that as well as the breathing are bulbar issues, no?

 

[lgelb]

We use many muscles below the bulbar region to breathe and talk.

 

[JAM]

I did not know that. My PAL’s was dx with limb onset but bc he is so difficult to understand I thought maybe he was actually bulbar.

 

[lgelb]

In some limb onset, bulbar impairment doesn't show up for a while. In others, it comes along pretty quickly. Speech, swallowing, and breathing, may be affected at different points in time because different muscles can compensate for a while, for example, using abdominals more in breathing instead of chest muscles.

It's still accurate to say "limb onset," if that's what he started with.

 

[JAM]

That makes sense. He doesn’t have any breathing issues yet, but his mouth muscles must be super weak bc he doesn’t enunciate much so understanding him is hard.

 

[blh8287]

My FVC now is 77%, MIP 18%, MEP 27%. Uvula and velum very weak now, tongue fasciculations and weak. Primary striping wave and proximal escape and tertiary esophagus contractions.

 

[KAD]

Hi all - bulbar refers to the cranial nerves, which control speech muscles (tongue, lips, velum/soft palate, etc. which are also used in swallowing) voice production, and facial expression. The respiratory muscles are mainly controlled by cervical and thoracic (neck and middle back/chest) branches off the spinal cord. This is why speech and swallowing difficulties might occur at different times than breathing problems. Although problems with adequate respiratory support can also impact a person’s speech clarity as noted by Jimmy.