Respiration question

[sunandsea]

Hi, I hope my question isn't foolish. Do all PALS have respiration difficulties? My husband's respiration is good and as everything else has progressed, his breathing has not become worse. I'm wondering if this means we have more time - but it will be an issue at some point - or do some lose the battle with the disease while respiratory function is still quite good? I sometimes just want to know how this is going to play out and what is around the corner for us. (Or maybe I don't want to know.)
Thanks, everyone.

 

[GregK]

Dr. Hawking did not need ventilation until long after he was in his PWC.

 

[lgelb]

No foolish questions here, Sun. Most people die for reasons related to respiratory impairment, so unless he dies of something else, it is likely to come into play at some point. I am assuming he had limb onset, in which typically respiratory difficulties come later. How much later, we cannot say.

 

[sunandsea]

Thank you for not making me feel foolish. I could spend hours reading on the forum and am trying to learn as much as I can.
You're right - he had limb onset and is now unable to walk. But he has incredible upper body strength. His ability to walk seemed to go so quickly I assumed his upper body skills would as well. I'm thrilled that they haven't. He also has some cognitive decline and that is presenting the most challenging.
Thank you again.

 

[tripete]

The perceived rate of decline of a particular muscle group can depend upon several factors. Of course the type of onset is one and the muscle condition prior to ALS is another. I was a runner and biker and had very strong legs. Also my onset type was respiratory, so it has taken much longer for my legs to completely go, they have been steadily declining but are not gone. My doctors relate this to the condition they where in prior to the disease. On the other hand my lungs and ability to breathe where extremely high also but do to the respiratory onset have been the first to decline.

So if your husband always had a strong upper body the perceived rate of decline will be appear to be slower do the condition he was in. He will probably notice that he fatigues quickly before noticing that his strength is gone. For him though he will feel weak and be frustrated by the difficulties. I am still stronger than my wife, but for me the noticeable difference from what I once could do is frustrating at best.

 

[Ells]

It's good that he doesn't struggle w/ respiratory function currently - long may it continue!! Weak breathing muscles ends most of PALS' lives.

As he's not too mobile, he may not be exerting himself to a level where he'd find himself out of breath. Does he get Pulmonary Function Tests?

 

[chally]

Hey sun, mine is limb onset,first hands,arms then feet,legs
In pwc 95% now breathing is starting to weaken. This nightmare is rough! We all go at different rates but to be sure we all go. Comfort is all we can ask for as it happens.
Frustration,& anger excelerate the progress for me. Feeling a bit down right now as I just dropped off another plateau and when that happens nothing is right or good. Stay strong! Love ya chally

 

[sunandsea]

Thanks everyone -
he does have pulmonary function tests and they have been good. It's surprising because he had asthma in the past and yet he seems to be doing alright. He is using a trilogy - so I guess not that alright - for the beginnings of sleep apnea and after having pneumonia this winter. He is so tired this week - so perhaps there is more impact than I realize. I hope not.
We can't get the bipap / trilogy to work tonight so I'm nervous. It keeps setting off the alarm as if there is a leak. He doesn't keep it on all night but he does initially and then through the night if I can convince him to put it back on. Tonight he won't have it if I can't figure out where the leak is.
Ells- you're right - he isn't active so isn't exerting himself. I'm sure it would be different if he were still mobile.
Chally - sorry you're down and coming to a new point. You're right, this disease is a nightmare. My husband doesn't say much these days and it helps to hear from others what helps. thank you.
Tripete - thanks for the insight. H wasn't much of an athlete but was actually trying to improve his physical state by working out more when all of this started. As I mentioned, his lungs weren't the healthiest either. Guess it is just a mystery sometimes how it progresses. Sorry - I know it is very frustrating. He is becoming depressed again. Understandable but so hard to see.

 

[Angeljo2]

My husband was diagnosed with ALS in February they say he is in the last stages his own vent with oxygen feeding to but doing well otherwise we just had to go back into the hospital with high respiration from alert had a slight pneumonia coming home soon doing better this is all new to us onset was so quick but I believe he'd been having symptoms for a few years with the tremors all over his body and slowly lossing over 100 pounds within the last year I am his nurse 24 seven seems to be working out good I like reading some of the things that yall have to say sometimes it helps me, thank you so much and God bless

 

[lgelb]

Sun, you can turn off the leak alarm. Let me know if you need help doing that.

 

[affected]

So sorry it's all so hard Sun, let us know how you go and hopefully you can turn that alarm off.

 

[sunandsea]

Laurie - Can you help with the alarm? That would be wonderful. Thank you. I called the respiratory therapist who tried to help over the phone. I can try her again today but would love to get him on it as soon as I can. He uses it during the day because he won't keep it on at night.

Tillie - thanks. It was a rough night here.

My husband woke around 4 am, confused, unable to communicate what he needed, sitting on the side of the bed. (He needed to use the urinal and couldn't tell me and couldn't seem to remember how so went all over the bed and floor.) He has been more confused the last couple of days but this was even more so last night/this morning. The aphasia we have been seeing was much more pronounced. Yesterday, he kept repeating the same phrases over and over but now he can't even utter a phrase. I'm getting head nods and shakes to answer yes and no questions. I feel like we are entering a new stage of the ftd and this is much more concerning than the loss of physical abilities. One of his doctors (we are being seen by two centers) doesn't think he has ftd because he doesn't present with the symptoms of aggression or anger or inappropriate comments. But the cognitive decline and now oral expression is so marked, it has to be dementia. Right? Do patients always have aggression? He is the opposite - childlike and loving. I hope the aggression never comes.

Any words of advice? Thanks.

 

[lgelb]

Sun,
Sent you a PM about the alarms. Being off the Trilogy could add to the confusion, so hopefully you can get him back on it and make sure nutrition/hydration is what it should be. Is he on any new drugs or supplements?

Also, it is not true that FTD presents with negative emotion necessarily, and FTD is not the only form of cog impairment seen with ALS.

 

[sunandsea]

Thank you - got the message just as the respiratory therapist was arriving. She found the problem - some water at the connection of hose and machine. It is working now and he's sleeping with it on.

No new drugs. He hasn't eaten as much as usual the past 24 hours but getting ensure and liquids down. I'm wondering if we have reached a new normal in terms of speech and behavior. Glad to hear (and I thought it was the case) that aggression isn't always present in ftd. Or perhaps this is another cognitive impact. I used to think the problem was that the doctor didn't know my husband before so couldn't see the change/impact. Now there is such an impact, one doesn't need to know what he was like before. This isn't normal behavior.

Many thanks again for your help.

 

[affected]

sun you can research FTD (and don't confuse it with AD).

there are 3 variants and your husband may have the language variant.

Mostly here we have talked about the behavioural variant as that is the one most of us here have dealt with. Dave hasn't had all that anger etc with Julie.

another member here a few years back (sadly I forget her user name) had a PALS with FTD that was very child-like, no anger.

The confusion between saying yes or no and giving the right head nod or shake is a really common stage PALS with FTD get to.

If there is any CO2 retention of course it just gets worse.