Mobility question- looking for information.

[Sophia51]

My husband was diagnosed with PBP in May and his speech is rapidly disappearing. In addition, he is experiencing muscular weakness in his chest, arms and legs. It seems very fast. Our consultant said that despite this, my husband may remain mobile until the end, as respiratory weakness will more likely be the cause of immobility.
Does any one with experience of PBP have any more information about the end of life stages - as it is very difficult to find much about it on the internet? We need to be prepared and the more we know, the better we can face it.
Many thanks in advance.

 

[lgelb]

I am very sorry to hear about your husband. We will support you however we can, Sophia.

It is now suggested that PBP is an ALS subtype, though as with other forms, a small minority of people live a decade or more. There is a "better" variant called isolated bulbar palsy (IBP), but it appears that is not what he has.

Therefore, it may be more realistic to think of this as bulbar-dominant ALS. Most often life expectancy falls on the <4y side, especially if there is FTD, which is more common than in limb onset, or of course inadequate respiratory support.

I would make sure BiPAP and a feeding tube are supplied as quickly as possible if those are interventions he wants, since working too hard to breathe would use energy he doesn't have, drastic weight loss is hard to come back from, and aspiration pneumonia is a concern with impaired swallowing. I would certainly have those discussions in addition to the standard advance directive planning, which is all the more important considering there may be cognitive issues ahead.

Mobility deficits are considered "less prominent" in PBP than limb onset, but that can be a very close difference, and again, people here have found it pays to stay ahead of the game in terms of anticipating mobility devices. Of course, preserving communication is a priority, too, so feel free to post any questions about mobile devices, laptops, apps, etc.

If you use the search link at the top right and enter terms like bulbar, swallowing, speech, feeding tube, etc., you can find threads that are helpful in thinking about the future.

Best,
Laurie

 

[Sophia51]

Thank you, Laurie.