Bronchoscopy Question

[emason]

My 17 month old son has a CRMS dx (df508 + a "varying consequence" mutation and negative sweat). We switched clinics about 4 months ago. Our new clinic felt that my son's health history and culture history warranted a bronchoscopy. Last Wednesday he had the bronch as well as an endoscopy and an Infant PFT. Normally they have you return 2 weeks post procedure but because of the holiday it looks like we may not be getting in until sometime next month. I've asked the nurse to talk to our pulm about getting us results over the phone at least - we'll see if it happens - but in the meantime I have a couple questions about the limited information I do already know about his results and I was hoping someone could help give me some ideas of what this could indicate.

So the first thing they did was an infant PFT under sedation. His pulmonologist told me that he was "confused" by my son's scores (indicating they were lower than he thought they would be BUT he did not tell me any of the numbers). He said that following the PFT they put him under general anesthesia for the bronch - and it appears he had some issues with the anesthesia which I guess is not that uncommon with these kids? But he needed albuterol at that point to help his airway stop constricting. Anyhow, the pulm said that there was a significant amount of inflammation in his left small airway. He seemed to be surprised by that. My son is already on twice daily nebulized pulmicort but we also got sent with a prescription for oral prednisone for 3 days and he said we would be switching to a stronger steroid at our next visit with an inhaler and a spacer. He said he did not scope the upper airway because "that would have been too much" for my son.

Apparently there were no mucus plugs. He said as far as mucus, everything looked really good. He kind of tossed around phrases like "reactive airway" and "sensitive lung" but wouldn't go as far as to say that was a certainty - or even really explain what that meant. And the GI mentioned that the only issue the pulm mentioned to him was an "edema"? Which confused me further. (yes, i should have asked more questions but I was overwhelmed just trying to keep it all straight! and jotting down key words) And as of 5 days out there was nothing growing on the lung culture other than normal flora.

So can anyone help get me on the right train of thought here? What causes small airway inflammation? Since his mucus looks good would this maybe be more indicative of something like asthma? Or is small airway inflammation still on the CF radar? I honestly don't have a clue! I know he doesn't have any common allergies - those have already been tested. And we have no family history of asthma either. And with CRMS being so grey - I guess I am very confused that he had this much inflammation and what that even could possibly indicate.

Any help processing would be appreciated! I know I will eventually have results but I am anxious to start understanding and I like to try to prepare myself with possibly scenarios BEFORE talking to doctors so I can follow along better and not get thrown off by something unexpected. I'm a little wee bit Type A

 

[Aboveallislove]

The reactive airways, reached airways, use of steroids, for our DS, is related to an asthma component which also can cause inflammation in my understanding. With the pft did they do albuterol in the middle and retest? If so when you ask for the results make sure to find the pre and post numbers and improvement. For DS the improvement after for infant pft was very significant and they to
d us it was indicative of an asthmatic component in addition to his cf.

 

[emason]

They did repeat after albuterol. I'm super anxious to know the results because the pulm made it seem like he didn't expect it to go the way it went.

The hardest part for me is that he is CRMS - so I'm trying to get a handle on what this means as far as is this indicative of early disease and he's possibly leaning towards cf? Is small airway involvement with no mucus plugs commonin children with CF? Or whether it's possibly unrelated OR simply a "related" issue but not exactly a tic in the CF column.

​

 

[Aboveallislove]

Hey Mom,
I totally get the wanting to know if the signs point to CF. DS definitely has CF, but I vividly recall trying to get the doctor to say "mild CF" based on xrays, lung sounds, no excerbations...there was just something I needed to cope to hear "mild CF," so my mind could then extrapolate that that was his future. Of course, the answer was mild lung involvement now, but that there is no way to predict if it will continue, etc. Obviously for us, it didn’t change anything re treatments, etc., if we heard "mild CF" or not, but I mentally needed that. So I can imagine it is similar for you...not wanting them to say this means it is CF because mentally that means something different than if CFRM. What I’m wondering if it would help is to just think of it as your little ones CFTR doesn’t work right, no matter what we call it. (I totally get though that the calling it CF has a different dimension for a mom.) And that we’ll treat what is not working aggressively. From what you wrote it does sound like an asthamtic component but the thing is that might mean that the CFTR defect is of more significance. (Small airway issues arise with CF as does inflamation). Also, keep in mind that Kalydeco could well work for your little one and the combo too (661/Kalydeco) so the "varying consequences" mutation on meds could well mean that the CFTR gene functions at 80-90% which is what the combo is showing. (Do you know if the other gene is on the label or part of the Kalydeco filing to expand the label?) Not sure if any of this helps. Hugs and prayers,
Love

 

[The Dot]

I am an RT and worked in 2006 at Oakland Children's where I would assist with the Pediatric PFTs and Bronchoscopies. However, none of that really makes any difference, just throwing it out there. What you are stating here that the doctor said indicates that your child's airways are really angry. In other words, inflamed (edematous), perhaps because of extreme sensitivity (to what?). Your child could have asthma, most likely has allergies. Now days, all that wheezes is not labeled asthma, though it is treated much the same. If a person has airways which react the way asthmatic lungs react, but only under extreme circumstances and not on a regular basis, it is labeled reactive airways. Often, one doctor will say reactive airways, and the next will say asthma. Sometimes they will use whatever label will get insurance to pay (sad but true). So, all of this could be in addition to or a part of CF, but it is very treatable. Once you get the inflammation under control, you will be able to back off on the steroids. Then, if you can figure out what the trigger is, you will, of course, avoid it. You may end up needing to see an allergist. (BTW - my son's pediatrician strongly advised against taking him to an allergist and it was the best money I ever spent - even though I was self employed as a baby sitter and had no insurance - obviously, before my RT days.) God Bless you and your child on your journey through the medical maze.

 

[emason]

That's exactly it. I know that many people will say that it's not important what it's called as long as issues are being treated but it kind of is important to me. I would like to know if it appears that my child has a progressive disorder VS related but non progressive health issues. It's a big difference I feel. And it feels like the "definition" of CRMS keeps shifting which also has me unsure what to expect from his health. When he was diagnosed earlier this year the CFF website basically said CRMS kids are expected to remain healthy. Now it says people with CRMS "may experience problems in the parts of the body often affected by CF." Which to me is so confusing! So they may have health issues related to airways, sinuses, reproduction, pancreas etc. but then what is the distinction? Just the sweat score? I am so confused by it all. And also, when you have a child with CRMS vs CF, there is only so much that the CFF will help you with. So there is a distinction in assistance too.


As far as Kalydeco - Right now his mutation (G1069R) is not listed nor is it part of the next group. I saw it pop up on a patent request from a couple years back but it hasn't made any cuts I guess. He is one of only 10 listed in the CFTR with it so I assume that might have something to do with it. I imagine we could still get it off label if he ends up needing it? But again, I'm sure that process isn't clear cut and I'm thinking he'd need to have a CF diagnosis to qualify.

It's one of those things where I just want an answer and I know it's not that easy. I know the good news is that he's so far on the mild side which is why it's hard to diagnose but living in diagnostic limbo has it's own stresses as well. Constant wondering if I'm doing too much or too little. In fact, the strangest emotion after his bronch that I had was relief that there was something going on in his airway. How terrible does that sound!? But his last clinic kept telling me that all of his sicknesses and the infections he kept getting were just normal kid stuff since his sweat scores were so low. I kept thinking something was going on with his breathing and they kept poo pooing me. Even when he had x-rays that showed issues - I was told they were "poor quality" films. Then I switched clinics and they started him on daily nebs and CPT based on his health history and culture history and I wondered if I was putting him through too much unnecessary treatments. It was weighing on me, the idea that he might be perfectly healthy and I'm forcing him through daily nebs and CPT and blood draws and then the bronch etc etc. So when the pulm said his inflammation was pretty significant, that's not good news by any means, but it just meant that my mommy radar was right. And the first clinic was wrong. And he has something going on - even if it's not enough to diagnose CF. He's having something in his airways. But that's where I hit a wall because I really don't understand the significance of his inflammation either. And It's KILLING me that with the holidays we are probably not going to get much clarity until sometime after the New Year.

 

[emason]

I am an RT and worked in 2006 at Oakland Children's where I would assist with the Pediatric PFTs and Bronchoscopies. However, none of that really makes any difference, just throwing it out there. What you are stating here that the doctor said indicates that your child's airways are really angry. In other words, inflamed (edematous), perhaps because of extreme sensitivity (to what?). Your child could have asthma, most likely has allergies. Now days, all that wheezes is not labeled asthma, though it is treated much the same. If a person has airways which react the way asthmatic lungs react, but only under extreme circumstances and not on a regular basis, it is labeled reactive airways. Often, one doctor will say reactive airways, and the next will say asthma. Sometimes they will use whatever label will get insurance to pay (sad but true). So, all of this could be in addition to or a part of CF, but it is very treatable. Once you get the inflammation under control, you will be able to back off on the steroids. Then, if you can figure out what the trigger is, you will, of course, avoid it. You may end up needing to see an allergist. (BTW - my son's pediatrician strongly advised against taking him to an allergist and it was the best money I ever spent - even though I was self employed as a baby sitter and had no insurance - obviously, before my RT days.) God Bless you and your child on your journey through the medical maze.

Thank you for your insight! I am very new to this all and before my son's dx I can say that I really knew nothing about airways/cf/asthma - any of it! So I am behind the curve here.

He did have a blood test for allergies including our regional ones (triggered after his 3rd croup - our pediatrician said that's her normal process is to rule out allergens) which showed no allergies to anything. But I imagine there are many other things that could be triggers - it's never that simple right? lol

He has been on an inhaled steroid for the last 4 months - .5mg of pulmicort 2x daily. As I mentioned we also did a 3 day oral prednisone for him right after the bronch and the pulm said we'd be moving to a stronger steroid with an inhaler/spacer starting at our next clinic appt (whenever that ends up being). Would they need to do another bronch to check his inflammation? It was also only one lung - his left one. Right was wide open. Which I thought was strange. Wouldn't asthma or allergies affect both lungs? I guess I don't know how it works so maybe that's a silly assumption. I just was really confused how one lung could be so sensitive while the other is just normal as can be. I know you may not be able to answer these questions. This is actually my train of thought just getting dumped out on the screen. Helpful though because all of these replies are helping me forumlate questions that I do have and want to ask.

 

[The Dot]

Interestingly enough, my son had the blood test which showed he was not allergic to anything (even though he went into anaphylactic shock when he had his first peanut butter cookie)! However, when the allergist did the pin prick tests, they showed that he was HIGHLY allergic to just about everything. I do not know how to explain this as it was never explained to us, but it happens.

 

[The Dot]

And, no, another bronch should not be necessary just to see if the inflammation has gone down, unless they are trying to pinpoint something in that single lung. They should be able to tell by his symptoms. ALSO - if it is only his left lung - he may have acid reflux that he is aspirating into that lung when he sleeps. My grandson who lives with me had something similar to this and was thought to have CF (though a negative sweat test) until we took him off asthma meds and put him on reflux control meds. Now, he is one of those kids who has reactive airways --- just occasionally has a really bad episode of asthma-like symptoms.

 

[emason]

And, no, another bronch should not be necessary just to see if the inflammation has gone down, unless they are trying to pinpoint something in that single lung. They should be able to tell by his symptoms. ALSO - if it is only his left lung - he may have acid reflux that he is aspirating into that lung when he sleeps. My grandson who lives with me had something similar to this and was thought to have CF (though a negative sweat test) until we took him off asthma meds and put him on reflux control meds. Now, he is one of those kids who has reactive airways --- just occasionally has a really bad episode of asthma-like symptoms.

That's actually what the doctor had thought it was going to be initially - related to reflux. So they had the GI do an endoscopy after the bronch and now I'm thinking it's unlikely that is the issue. He said my son has very very mild reflux. he didn't see much of anything on his scope that indicated any significant reflux issues. The GI said on a scale of 1-5 my son was a 1 *maybe* 2. He took a biopsy of one small area just to be certain. He said maybe he could benefit from a reflux med but he seemed to think it wasn't really that necessary. Not sure if they are going to try it just in case. I'm guessing it wouldn't hurt.

I went into this thinking if anything the endoscopy was going to be more telling. But now I feel more confused by the bronch part and the endoscopy didn't really provide the answers I thought it would! I am SO incredibly curious to see what our pulm's take on this is because he's been saying from the start that he felt that the issues likely were resulting from the GI side of it but I just feel like none of this adds up to what we thought it was going to.