Some advice with unexpected progression

[noChomei]

Hello forum,

I’m hoping for some insight into my Dad’s ALS because he seems to be declining much quicker than anticipated. Dad was diagnosed in September after about 12+ months of diagnostic investigations. At the time of this diagnosis his respiratory function was at 79%, but only two months later he dropped to 49%. He is now struggling to breath normally, and eating food is sometimes accompanied by chocking scares. The clinic that’s overseeing his care commented that this decline in respiratory function now excludes him from the installation of a feeding tube, and his appointment for a non-invasive ventilation machine has been prioritised and moved forward to three weeks from now.

This afternoon Mum called the ambulance because she was concerned he wasn’t getting enough oxygen, after his words would trail off mid-sentence with sort of micro-unconsciousness moments. His struggling to eat a whole meal now, and his sleep is described as quite disturbed with lots of rapid, short, almost hyperventilating, but weaker breathes. I now feel like anything the specialists and nurses explained would be months away need to be organised ASAP, like a hospital bed and a wheelchair. Thankfully, we have voice banked just last week.

He’s presently at hospital being monitored, but I’m wondering if waiting three weeks for a non-invasive ventilation machine is too far away? Also, his speech is really ‘tonguey’ (for want of a better word), as though his tongue isn’t able to keep up with his thoughts.

When he was diagnosed, we was told two years maybe — but it’s moving really quickly. Is this common?
Thanks in advance

 

[lgelb]

I'm sorry you are facing this rapid progression.

If he can't eat and a feeding tube is withheld, two years is certainly not possible, nor is anything like that achievable with untreated respiratory insufficiency. I am puzzled as to why a tube would be ruled out, when there are case series showing successful RIGs (placement of the tube in Radiology) at FVCs as low as 17% (while using BiPAP during the procedure). Surely they could organize a machine for him and get it done. In the US, that would be a no-brainer in most areas. I would ask why not.

From your description, yes, he needs BiPAP now, and while sometimes machines that are portable (with rechargeable batteries, so not requiring mains power) take longer to get, a mains powered machine that he could use at home and during a RIG should be in stock somewhere locally. So again, I am puzzled why this would not be done, especially since he is in hospital. Is he on a ventilator in hospital? How is he receiving nutrition there?

To be frank, what you are describing could take him rather quickly if he does not have adequate respiratory and nutritional support. So I would say and do whatever is at the top of your list for him. I would also take your concerns up the chain if you are not being heard on this.

I agree, at home, a hospital bed is a necessity, and a wheelchair as well since he is a fall risk given varying levels of consciousness, apart from what his walking abilities might be otherwise.

What hospital/clinic does he attend? There may be Aussies with thoughts. You can also use the search function for past experiences.

You might also be able to have his team consult with another centre, so at the risk of the obvious, MND Australia has a list on line (I cannot link to it because we do not permit donation sublinks). They may also be able to help expedite things in some way.

 

[noChomei]

I will read and reply in a little more detail when everything is over. Dad is now in palliative care, unconscious from elevated CO levels and is now in the final stages of dying. hard to wrap my head around seeing him taking the bins out yesterday morning. shocked.

 

[Nikki J]

I am very sorry.

 

[lgelb]

Let us know if you have any questions. Wishing him a peaceful passage.