CPAP for Tracheobronchomalacia

Hello,

I did a search to see if any topics had already been posted on this subject but didn't turn up any hits. So I'm afraid I might be alone here on this.

I've recently been diagnosed with congenital tracheobronchomalacia (which is a collapse of the trachea, bronchi, and other upper airways). In my case, the bronchoscopy shows 100% collapse upon exhale. So, I was prescribed a CPAP machine to keep my airways open. I have to use it as much as possible, certainly all night but as often as I can/need to during the day. The TBM causes a horrible cough and I get in fits of coughing where I can't breathe.

My difficulty has been insurance. Because it isn't for OSA of some type, they're having trouble covering it even though it is one of the treatments for TBM and certainly the least invasive. I've had FIVE doctors prescribe it including 4 pulmonary specialists one of whom is also a sleep specialist. They're trying to let the CPAP reduce some of the inflammation that the coughing has caused so that they can also look at more permanent longer term solutions, though they say I'll be on CPAP at night regardless forever. So, even though I didn't need it, they insurance wanted me to have a sleep study which didn't show any apneas but did show that the CPAP helped my breathing. They're trying to get a pre-auth now from my insurance so I'm just praying it goes through. I currently have a machine on loan from the hospital because they said I can't be without one but I can't use the humidifier because it leaks all out the bottom of the machine. Also the quattro mask that they are letting me use doesn't fit properly so I wake up with blisters on my nose.

I guess I was just curious if anyone else has used CPAP for this type of diagnosis and wanted to join in the community a bit. I'm looking forward to learning more about CPAP from all of you and am grateful that a community like this exists.

Take care,
Rae

Hi and welcome to the forum! Your situation sounds yucky (I think that's the technical term ) I hope it improves for you and you get the insurance.

Do you know what pressure they set the machine to? If you can please list your equipment. (look under User Control Panel top left on this page)

As for the nose blisters it may be that the mask is too tight. Try adjusting it while lying down. You need the least amount of strap tension that stops the mask from leaking. There are lots of posts on how to deal with the blister; bandaids, moleskin etc so do a bit of searching and you'll get all the info you need.

You may not have OSA but you are using a XPAP and mask so we may be able to help

Again, welcome!

Hi Rae --

I have to agreed with snnnark -- your situation sounds yucky! I hope they can get you your own machine quickly and get you headed down the right path.

I participate here under the "not so usual" circumstances as well. I have a neuromuscular disease that is causing me to have difficulty breathing when I lay down. The thinking is that my diaphragm muscles are too weak to keep me breathing properly at night, especially during REM sleep. I have gone from a CPAP to BIPAP, to BIPAP with oxygen and ultimately ended up on a Ventilator with oxygen (my current setup).

The people here have not only welcomed me to stay, but have gone out of their way to try to help me understand the equipment I'm using and the settings.

While I personally have never heard of the condition you suffer from, I'm sure there are some here that will be more than happy to help you through this process.

Welcome aboard. I look forward to following your story.

I just wanted to welcome you to the forum as well. I sure am sorry to hear you have TBM. That is the first I have heard of it so I appreciate your sharing. There are lots of good people here who can help w/the PAP part of your therapy.

I have only a little knowledge of this subject, and I know a little knowledge is a dangerous thing, but I want to share anything that might be helpful. I occasionally see medical malpractice cases involving laryngomalacia and tracheomalacia. In infants, these conditions can be present at birth because of excessively soft cartilage that allows the larynx and trachea to collapse during inspiration. I believe bronchomalacia is the same thing, just further down. In infants, at least, it can resolve over time as cartilage hardens. Standard treatment is a tracheostomy, but if the trach becomes infected or inflamed, and needs time to heal, the baby is put on CPAP. This splints the structures through which air passes open just as it does for OSA patients. Therefore I can say from actual knowledge that CPAP is sometimes used in the treatment of tracheomalacia, although I do not know if this is true in adults.

Welcome to the forum. Thanks for sharing your story, we can all learn from it.

Hello and welcome. You say you were recently diagnosed. Just wondering if you have been symptomatic all your life but not diagnosed? A friend's little granddaughter is as PST described. Many cpaps are made so that they can be used with or without the humidifier. It may be that you'll need to go without it during the day for portability, then use it at night. Before you allow them to give you just any machine (assuming it will be approved), I'm hoping you'll get some pointers on here regarding particular machines that have features most suitable for your needs. Wouldn't it be nice if one was super lightweight, could be strapped over your shoulder, and runs on battery power! Sure hope you find this treatment helpful to your condition.

Hi Everyone!

Thanks so much for making me feel so welcome! I really appreciate it. It feels good to have other people dealing with similar things even if they're not using the CPAP for the same reasons.

I have been symptomatic all my life but I had pneumonia two years running when I was a very small child and drs just dismissed the horrible cough as having come from that. The situation has deteriorated a lot over the past year+ starting with a bad lung infection last year (either bronchitis or pertussis). I now have a chronic cough that often will go on literally continuously for several hours at a time. It isn't enjoyable. I have a connective tissue disorder called Ehlers Danlos Syndrome which is what they are attributing the TBM to.

My pressure was originally set at 8. But when I was in the hospital a couple of weeks ago they orders were set to start at 8 and titrate up to 12, though RT never came back up to alter the settings. After getting home with the loaner, the pressure wasn't controlling the cough after a few days so I searched online and found out how to change the settings and upped the pressure to 9 (figuring that it was still within the orders). At my unnecessary sleep study, we ended up at 10 when I was awake and 8 when I was sleeping but the tech told me that she'd never done a sleep study for someone with my condition so I'm a little unsure about it. I came to the place of realizing that I can work with my family dr (who's awesome) and eventually the sleep dr when I see her to alter the pressure if needed. One potential question I have that so far none of my docs can answer is will pressure that's too high on the CPAP eventually cause the "stretchiness" and laxity in my airways to get worse??

I've started working with my local DME to try to get my own machine. So far, they have been fantastic and are bending over backwards to help me. The PA was sent in either late Wed or yesterday morning and they put a rush on it so I'm hoping it'll get approved soon without having to do a lot of appeals, etc. The DME is recommending the ResMed S9 with the humidifier and the heated hose. Because of the cough component, the dr and DME feel like the humidity will be really helpful in quieting the cough and hopefully getting my airways less inflamed. It sounded like a good unit from what I've read online. We discussed the Everest 3 but since I'm gonna be putting a lot more hours on this machine than the typical patient, my DME rep felt that ResMed will stand behind the machine and it was more important to get a workhorse for my primary machine and look at possibly getting the Everest 3 (if I can save enough pennies) as a secondary machine later on. The S9 is pretty small w/o the humidifier and it can run on a DC adapter so it sounded like a decent solution. They also told me we could look at alternative battery options for it down the road.

So, I hope I got to all of your questions. I'll try to add the hospital machine make and model. I know it's several years old.

Again, thanks for such a warm welcome and allowing me to join in the community.

Oh, about the Quattro mask. They seem to want me in a FF mask because of the cough which makes sense. I actually don't mind the feel of a FF mask I just wish I had one that fit me. The RT that brought me the mask didn't take any time to fit me and we never put it on with the CPAP on - just kinda held it up to my face and showed me how to tighten the headgear. During the sleep study, I tried a hybrid FF mask, a Fisher-Pykell (sp?) under chin FF mask with a firm gray foam, the Respironics gel FF mask and I think one other that I can't remember. I hated all of them.

They're recommending I try the FullLife by Respironics especially because of the improved line of sight for during the day. Eventually, my DME thought I might do well with a day mask and night mask so that if I can't find just one mask with a good line of sight AND fit throughout the night, it gives more options.

Wow, I have to damit that just reading th name of your illness was scary. I do hope someone here will be able to help. If nothing else we can offer a bt of comfort and friendship. Welcome to the board.

Laura

Hello, I am a little confused as to why your sleep study turned out normal. There should have been obstructive apneas and or hypopneas all over the place. Do you know what your AHI (apnea hypopnea index) was before they applied the CPAP for treatment?

Welcome to the forum! Sorry to hear about your condition. I hope the CPAP will help! We can definitely help with the 'technical' side of things

I would think that breathing out against the pressure would actually help strengthen the airways (like resistance training builds muscle ) . But I'm not a doctor!

I had the same thought as finchy. If your airways are collapsing wouldn't there be some sort of apnea-like effects?
Did they do a full night on CPAP without a baseline PSG (i.e. without the CPAP), or a split-study where you go half the night without CPAP and half the night with CPAP? Did they measure your blood oxygen saturation while awake and asleep (pre-cpap)?

We're glad you're here! Of course you're welcome, because this is cpaptalk.com, we're open to all hoseheads, not just those with OSA! Your situation sounds yucky, scary, and complicated to me, but it appears that you have some helpful doctors on your side, which can only help. I imagine (and HOPE!) that the insurance knuckleheads will come around and pay for what you need.
Try as many masks as possible. The hybrids or full face ones will probably work best because of your cough. If you cough with just a nasal mask all that great therapy air goes out of your mouth.
Let us know how things go!

I just noticed that you're not using a humidifier. That could be causing some damage, you should go back and knock down their door and raise heck until you get one that works (or check that you're connecting it properly). I remember reading a journal article a few years ago about how humidification should be mandatory with CPAP especially when dealing with pre-existing lung conditions. I'll never find that article back I'm sure, but you can probably find it via google. Basic idea is that the extra air pressure wipes out all the moisture in the lungs causing extra irritation, and that can't be helping you at all!

echo wrote:Welcome to the forum! Sorry to hear about your condition. I hope the CPAP will help! We can definitely help with the 'technical' side of things

I would think that breathing out against the pressure would actually help strengthen the airways (like resistance training builds muscle ) . But I'm not a doctor!

I had the same thought as finchy. If your airways are collapsing wouldn't there be some sort of apnea-like effects?
Did they do a full night on CPAP without a baseline PSG (i.e. without the CPAP), or a split-study where you go half the night without CPAP and half the night with CPAP? Did they measure your blood oxygen saturation while awake and asleep (pre-cpap)?

No, because of the condition, they didn't want me to be without CPAP so they did the entire night as a titration. I started out at 10 and then after I was asleep, they reduced me to 8. At one point, before I was asleep, after a terrible coughing spell that had lasted 2+ hours and finally was quieting down, it felt like someone sucked all the air out of my lungs. I immediately starting coughing again to the point of almost panic, I couldn't get my breath and ended up coughing so badly it trigger the vomit reflex and I ended up having to swallow it back down since I couldn't get the mask off fast enough. (Sorry, probably TMI). When I asked the tech about it the next day, she said she tried to turn down the machine at that point and she noticed it retriggered the cough and turned it back up. She said she'd never had anyone be able to tell about the difference in pressure like that before. But, what I have is so unusual that she'd never had another patient in the sleep lab with anything like it. So, I suppose that it's possible I did/do have apneas, etc but since I was on the CPAP the whole time, they didn't get recorded. So, it's an unusual situation all the way around.

In terms of the humidifier, I don't think I'm doing anything wrong, at least that I could figure. What's happening is that I fill it with water to the fill line that the tech showed me, and it slowly drains out the bottom of the machine via all the little cutouts. I was worried that since the heater is under it, it might cause a huge problem that I don't want to deal with (like electrocution!). So, I gave up and have been using it dry. A friend let me borrow her unit for a bit and it did have humidification but it would never last through the night (set on level 2 out of 5). Her machine was a Respironics as well, the Remstar Auto M Series A-Flex but it was already setup as CPAP only with out any fluctuations in airflow. I couldn't figure out why the water would run out on it.

So, I'm keeping fingers crossed and hoping and praying that my CPAP will get authorized soon. It would be great to have my own machine and actually get fitted for my own mask. *sigh* (Not that I'm not grateful for the loaner from the hospital...don't know what I would have done to control the cough and open my airways without it.

Thanks again for all the welcomes. I'm happy I found this place!