CALS Roll Call Continued

[affected]

oh V, what a weekend. If you can find a way to afford some help you really need to do that. Sustainable is more than important.

rmt that is just so great. I agree, if he is putting on weight and tolerating the formula well now, don't rush, give yourselves a break and change him over to blended food as it feels right. Savour every moment of this great week!

 

[vltsra]

Robin, I'm so glad your husband is doing well on the feeding tube. It makes it easier for us although he still wants to eat by mouth. My PALS really hasn't seemed to lose much weight aside from muscle wasting. Glad you had a good week.

V

 

[Mary2]

My husband had a choking episode last night...on his own sinus drainage. Do you think it is time for a cough assist? Respiratory gave us a suction machine but I am not sure this is the answer.

 

[JimInVA]

Yes, Mary. You will want to have a CoughAssist at the ready. It is a VERY useful machine.

My best...

Jim

 

[JimInVA]

Adding onto my previous post...

We didn't use the suction machine until very late in Darcey's progression. We did, however, use the CoughAssist many times over the course of several years. We never knew when we'd need it. But when we did need it, it was an invaluable tool. If we traveled, the CoughAssist always got packed up and went with us. If Darcey had friends over, who wanted me to use that time to leave and do whatever I wanted (some break time), I made sure that they understood how to use the CoughAssist by having them actually use it on Darcey. There were a few times when I'd return and that friend had an episode that required the CoughAssist. They were grateful for the actual hands on training so that they weren't afraid to use it when an actual need arose.

Yes. Please get a CoughAssist.

My best...

Jim

 

[vltsra]

I will echo Jim's post. We started out with a twice daily regimen of cough assist. Now I find it is necessary more often. It keeps my PALS lungs clear.

 

[Mary2]

Thank you Jim and Vitsra....if it wasn't for this forum I wouldn't know there was such a machine as a cough assist.

 

[Bestfriends14]

Where would one be in their progression when they would need a suction or cough assist machine? My husband is at 15% according to his last SNIF test. I'm confused if we should get one soon. We met with the pulmonologist and they listened to him breath and they weren't too concerned about any additional machines.

Also, for those who remember my previous posts, he still has no feeding tube. I've never heard of a PALS, with his lack of respiratory abilities, not have a Gtube. Has anyone heard of this? The pulmonologist said it's because his bulbar region is still unaffected, his voice is strong, and he has a good appetite. Still...

This disease is confusing.

 

[JimInVA]

Darcey's ALS began in her extremities. But we were given a CoughAssist relatively early in her progression. As the lungs weaken in their ability to breath, it becomes even more difficult to cough up anything. The nerves just can't tell the muscles to do what they need to do... and the muscles, without exercise, begin to waste away. Early on, we'd go months without ever using the CoughAssist... but if Darcey got congested and it settled in the top of her lungs, the CoughAssist was the only thing that helped bring up the phlegm. As time went on, we used it more and more. I believe it saved her life on multiple occasions. Definitely a necessary machine.

My best...

Jim

 

[Bestfriends14]

Thanks Jim,

Wayne has been officially diagnosed for just over 4 years, but with quite aggressive progression of breathing issues since April of 2020. For now, he uses breath stacking to get a good, strong cough going, but I can't imagine that working forever. For about 5 minutes I though he was also slurring his words, but the SLP said no and that Wayne was just a mumbler. Phew! At least that's not an issue yet.

 

[lgelb]

As to your question about someone with breathing issues not having a feeding tube, Joanna, Larry was on 24/7 BiPAP for his last year, without a feeding tube. Tube placement was advised a year and a half before his death but since his connective tissue disorder made the procedure more risky, we opted to wait until he really needed it...which didn't happen. He ate the day he died.

He never had a CoughAssist either, because it was contraindicated in his case. It is also worth noting that for some people here, the CoughAssist made coughing worse and so its use was stopped. We also stopped using the suction machine after it chipped a tooth.

Everyone is different.

 

[Mary2]

PALS and I are sitting here wondering how he can call me if he needs the cough assist or help with choking. Is there a device on the market that is useful? I think a horn would be hard for Paul to use and a bell might not be loud enough. Any suggestions. Also we are Googling SNIF and trying to understand this metric. We have not been given a SNIF percent. As of yet we have not been referred to a pulmonologist.

 

[JimInVA]

Mary,

When Darcey began to sleep downstairs and I was upstairs, I used baby monitors. At that point in time, Darcey was able to make enough noise that I could hear her and I would wake up. Because it had video and two way communications, I'd look to see if she was moving her head... a sign that she had called me and it wasn't a dream.

Later, as her voice began to lose strength, she was unable to make enough noise to wake me. I asked our ALS Org folks and their technology specialist reached out with a device that sensed minimal movements. For our use, I'd aim it at her left eyebrow... which she could move quite well... even though anything below her neck she had no movement control over. The sensor had an infrared light that the connected device would lock onto our chosen spot. If the underlying spot (in our case, her eyebrow) moved enough, it set of a quite loud buzzer/bell that I could hear through the baby monitor. I'd get up and come investigate to see what the problem might be.

Sure, we had some occasional false alarms when she'd wake and open her eyes wide after a wild dream. But for the most part, it worked exactly as we needed it to. I was able to sleep more soundly and she was comfortable knowing that she could set off the alarm if she needed me for any reason. It was another ALS Org saving loaner closet item. Let me know if you need more particulars and I can point you to the folks that make it... or you can contact your ALS Org folks to see if they happen to have one.

My best...

Jim

 

[Bestfriends14]

Thank you @lgelb ! I always feel better hearing feed back from you, as well as @JimInVA .

 

[affected]

bestfriends we also often say here it isn't about the numbers so much about how you feel. So I would say, regardless of his numbers, if he seems to feel mucous on his chest and has a weak cough, a cough assist might be beneficial indeed.
When my Chris was a PALS I couldn't get any health professionals anywhere to listen to anything on the benefit of cough assist or suction for PALS. Now I see them becoming commonly prescribed (I run an Australian closed CALS group), so over the last 7 years there has been a shift in thinking. Now if we could just have Neudexta available here ...