Ventilation Question

[PaulC]

I have bulbar onset ALS, diagnosed Oct 2022. I have full lower motor mobility, most of my symptoms are in the throat (swallowing, speech, and some breathing issues), but I do get out of breath easily (walking up stairs, etc.) After doing the respiratory tests at the quarterly ALS Clinic, they found on my "breathing out" test (not sure what it is called), I was at 41%. My "sucking in" test wasn't too bad, but pushing air out not as good. So they ordered and brought me a ventilator (Astral 100). They want me to use it at night saying, "It will give your diaphragm a rest at night." My question for the forum is, for those that began using a ventilator at night before needing it during the day, how did it help you or your loved one? And did it improve or seem to slow the negative progression of your respiratory test scores (assuming a quarterly clinic appointment)?

My question to the respiratory therapist was, "If I use my diaphragm and other breathing muscles significantly less for 8 hours a day, won't they be more likely to atrophy? Won't using the ventilator before I need it cause me to need it sooner?" With muscles, it's usually use-them-or-lose-them. His answer was no, but his explanation of why was a little vague.

In doing some research, I found several studies about Ventilator Induced Diaphragm Dysfunction, where patients on ventilators in fact lose diaphragm muscle mass and force when on a ventilator for as little as 12-18 hours. It's well documented for control triggered ventilators (where the machine decides when to offer help). It appears that with assist triggered ventilators (where the machine senses when the patient begins to inhale and then kicks on) the atrophy is much less. It still occurs but is slight.

Since I'm functioning fine without the ventilator and I'm sleeping through the night, other than my nightly bathroom trip, I'm wondering what advantages any of you have experienced from starting the ventilator before it was needed. Or would you recommend to wait?

 

[oldgeek]

Good questions. As long as you can, you can try to use non-invasive ventilation "NIV" which is a CPAP or BiPap machine that uses something like external nasal pillows or full face mask. I used a full face mask at first but after awhile, it sounded like I was wearing a Whoopie Cushion on my face. The big problem for me as that my swallowing got weaker and I kept getting aspirational pneumonias. So I finally got a trach and started by only using the vent at night. Love it. Lately, my breathing has gotten worse (they say my diaphragm is fully paralyzed and so I use my "intercostal muscles" and tummy to breathe until those muscles give out. You might ask them for a "sniff test". It's a quick/easy imaging test where you sniff in and they measure how well you diaphragm is working. With my muscle weakness, I now use a vent almost full time and my legs are weak now too so I carry it all around on my new power chair. One last tip, you might spend about $50 and get one of the all night pulse oximeters (a couple of brands on Amazon) so you can keep track of desaturation events at night. I check my sats about once per month and my team of RTs can work with my pulmonology team to make adjustments. Good luck.

 

[Nikki J]

Just a quick note. Op has an astral which is fine but clarifying for future readers PALS should never have cpap. Yes it is a form of niv but it is not safe for us We need a bipap mode

 

[MarkD]

My diaphragm was affected first basically When I was officially diagnosed about a year ago and I started using my trilogy ventilator to sleep at night, because I simply could no longer pull in the air automatically when sleeping. It's been a year now and I use the what I call the puffer when I sit at my computer desk and I take just a sip puff of air no and then. I can still walk albeit much more weekly now, and not use a ventilator for a little bit. I used the face mask for a long time, but it started to form a small break on the bridge of my nose, so I went to the nose pillow at night and I wear this little Velcro thing that helps hold my mouth closed at night when I use the nose pillow. It took a week or so to get used to sleeping with the nose pillow, but now I actually like it better as I feel there's a better seal for the air for one thing.

 

[lgelb]

For reference, the "puffer" is a 22mm mouthpiece attached to the hose or a straight connector, providing the ability to "sip 'n' puff" the air instead of using a mask.

The mouthpieces are a couple of bucks, come in straight or angled versions (which may matter depending on which muscles are weak), and you can mount the hose so the mouthpiece is easy to reach. Even if you don't ordinarily use it, it can come in handy at shower time, for example, if you need to use BiPAP then.

Paper tape/"gentle paper tape" or a cloth liner can help with the nose bridge, for those who like their masks otherwise.

Optimal settings and a well-fit mask are the best defense against "using up" muscles in the service of BiPAP. (Paul and I are discussing those). The idea is to support your own breathing, not to overwhelm (as many initial settings do) or challenge it.

So just a reminder that I am always happy to help evaluate where you are and suggest how you can better match what the machine does to what you can still do.

Best,
Laurie

 

[lisa g]

I'm having an issue with oxygen saturation levels. I haven't had any issues with my PFT 's which I have every 6 months. About a week ago I told my husband that I felt that I wasn't getting a deep breath. It seemed to be worse when I was out in the heat in the pool. Last Monday I had PT and mentioned to my therapist that I felt sluggish and was having an issue getting a deep breath. She put a pulse oximeter on my finger and the reading was 86% saturation with 125 heart rate. She called my husband over to tell him that she couldn't work with me with those levels and advised him to take me to the ER to get checked out. She also pointed out to him that my lips had a blue tint to them.

I argued with him to take me home and let me use my trilogy for awhile but he wasn't having it. So we went to the ER and after an EKG, massive bloodwork, chest x-ray, covid test (negative) and an ABG they told me that they were admitting me. We all know the obstacles we face when we're in the hospital, what got me worrying is that the triage nurse didn't know what ALS was. So I was monitored for a couple of days with my saturation levels going up and down. I was finally released when they felt that my numbers were high enough.

Yesterday I went back to PT and before my session they checked my numbers and once again I dropped to 88%. My pulmonologist received the hospital report and called he wants to see me tomorrow to figure this out. My neurologist also called and said use the trilogy for a while during the day. I'm curious what my pulmonologist will say tomorrow.

 

[PaulC]

Thanks for your advice and responses. I appreciate the insight. I feel I'm at the beginning of this journey and my breathing isn't that bad. My pulse-ox tests are in the mid 90's. I don't see a "need" for the ventilator support at night. I don't mind using it if it will provide a benefit, as long as I'm not accelerating my dependence on it. I appreciate Laurie's email support to help with getting the best settings for my machine.

Lisa G, I hope you can get those levels in a good range.

 

[Statius@]

I began using a trilogy at night and during naps 7 yrs ago and haven't any decline in lung function (my numbers we were 90-120% until last yr--my 8th since dx when they dropped to the 70's). I don't use the trilogy during the day and am rarely short of breath. So fm my sample of one using a bipap hasn't had any deleterious effects.

Ed

 

[PaulC]

Thanks Ed. That's encouraging. I appreciate the feedback. What was the reasoning for using it while sleeping if your lung function was good and no shortness of breath? Were you not breathing as well when sleeping?

 

[oldgeek]

One thing to keep in mind is that if the diaphragm is weak or paralyzed, as mine is, all the work of breathing is done by shoulders, stomach, and intercostal muscles. I can tell you from experience that they tire quickly. I've been on ventilators (one for sleep and other portable during the day) through my trach for over a year now. I can get off the vents for several minutes and do fine; but the rest of the time I "ride the vent' and let it do all the breathing. My docs tell me that in my case, 80% of my calorie burn is spent just trying to breathe (I frankly don't understand this). But, I do know that I have lost quite a bit of weight and if I'm off the vent for a long time, my entire body feels exhausted.

WRT the vents, they are super configurable. For example, you can have the patient trigger all the breaths and the vent will only step in if the volume or rate drops below a certain threshold. The Trilogy Evo measures this over a longer period of time and I hated it. I felt like the vent and I were constantly fighting each other. The Breas vents I have now have a much shorter period to track and so if you need more air, you don't have to wait for the vent to catch up. Also, you can have pressure control or volume control. During the day, I like pressure control so that I can trigger the vent with a sip (through my trach) and it will push in and help me breathe. At night when I don't breathe at all so I just let the vent do it all with volume control.

One thing to keep in mind in your Dr Google searches is that kids have different responses to vents than us older folks. So, be careful drawing conclusions for studies done with kids vs adults. Sounds like the stuff you cite is from pediatric studies...

Good luck