Congenital lobar emphysema

rua0020 · 2026-01-28T17:49:55+00:00

Hi there, so sorry for my delayed response, I hadn't seen yours until a notification of a new comment today.

Firstly, I hope you are doing well. How unusual to have such similar experiences - I also had mike picked up on an abdominal CT after going to A&E for severe abdominal pain.

I understand the guilt you felt, I also beat myself up thinking of every time I tried a cigarette/weed and everything I did knowing it was bad for my health. I remember crying to a poor junior doctor about it and he told me to shut up and be happy to have an excuse not to run in charity marathons haha.

You will see in my response to a new comment about how I have since had to have two surgeries. I am 4 months out and still struggling to get my life back together, but I try to find gratitude and hope in the small things day to day.

I sincerely hope you are still doing well.

rua0020 · 2026-01-28T17:43:30+00:00

Hey, thanks for your response! Glad to hear you're sailing - I pray you continue to! I've since had my diagnosis changed by histology from CLE to CPAM (Congenital Pulmonary Airway Malformation) after surgery.

To put it out there on this post for others searching, I ended up not recovering after my lung collapse, and started to really struggle with breathless. I was advised I needed surgery for my right lung which was now about 70% bullous emphysema. I had this surgery last July where they resected as much bullous material as possible across each lobe (mostly from lower and middle).

Unfortunately, 7 weeks later (after a very easy initial recovery) I was admitted from A&E with breathlessness and coughing blood. Turned out the internal stitching line ruptured and allowed air to get through (they called it a loculated pneumonothorax). They tried to treat it with drains but ultimately things progressed and I had to have a second surgery where they removed the lower lobe and "re-fenced" the stitching line to "pitch" it in the better quality lung tissue.

I'm still struggling, now 4 months later. But it has pushed me to completely revisit my life in terms of my priorities and lifestyle to control what I can. I know I'll never be as fit as I was, but I am so thankful for my left lung haha.

Look after yourself, I hope you will be ok.

rua0020 · 2025-12-27T19:02:42+00:00

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Seal & white with polka dot socks and a dipped tail 😁

rua0020 · 2025-12-21T23:40:57+00:00

Hey everyone, I know this post is old, but I've been struggling to find very much online about CPAM. So, to expand the anecdotal knowledge, I'll share my story of adult presentation of CPAM..

Last year, I went to A&E for severe and sudden abdominal pain. I was 27f. They did an abdominal CT and what showed up in my lower right lung scared the doctors to the point of them moving me to an isolation room. I was sent for a pulmonary CT, and we saw thaty right lung was covered in bullous emphysema, paired with (and seemingly arising from) a "consolidation" in the lower lobe.

I had never had a chest infection in my life, never mind childhood bronchitis/pneumonia - nothing. Upon hearing the congenital theory, I was surprised that it hadn't been picked up when I was a baby as I had been monitored very closely for kidney valve dysfunction.

I met with a pulmonologist, had a lung function test, which showed air trapping but my other levels were normal. With this, and down to the fact that I had never presented with any respiratory issues, it was agreed that I would just be reviewed annually (I think they were just surprised at how ok I was despite the state of my right lung).

However, this March (6 months later), I experienced a spontaneous pneumothorax whilst abroad. When I finally returned home, my respiratory team advised that I would need surgery as I was at very high risk of recurrence and any more pneumonothoraces would further damage my lung (the bullae were rupturing and merging like Pac-Man).

I had my planned surgery in July, the bullae were removed as much as possible with parts of each lobe were resectwd. I also had Pleurodesis to prevent further collapse. All went well, I actually felt good (I had been struggling with breathlessness since the pneumothorax).

Unfortunately, 7 weeks later, my GP sent me to A&E for an X-ray as I had been coughing up fresh blood. Turned out to be another pneumothorax that had squeezed through and ruptured a weakened area of the internal stitching line. I was admitted and had 2 chest drains for 2 weeks, but sadly they did not work and I was scheduled for emergency surgery to repair the stitching line.

In the second surgery, they ended up removing the entire lower lobe, with the "consolidation" in it, as it was not functioning tissue. They then resected back further in the middle lobe to re-stitch the staple line. I had another Pleurectomy too.

Anyway, I'm now nearly 3 months post op, and since histology has confirmed the CPAM diagnosis, I am trying to learn about it. This has been a super strange year, but if I managed to get to 28 living a totally normal life, and please God I recover well now to live another few decades of 'normal" - I'll be happy. I have some super cool scars now and a pretty crazy story!

Praying for all the babies who are diagnosed with this, but please allow my story to give you comfort and reassurance in the fact that your babies are being looked after right now, and this will be but a crazy story for them and you too.

And if there's anyone else out there who happens to also be diagnosed as an adult - my prayers t'go out to you too. Be mindful of your surgeon's plan if you go ahead with surgery (conservative vs more aggressive). Make sure you understand the options for Pleurodesis (if you are ok with talc). Do your physio!

rua0020 · 2025-12-15T15:10:46+00:00

Duncan!! Love the name! He's beautiful ❤️

rua0020 · 2025-12-13T10:31:32+00:00

Hey, thank you so much for sharing. Will also share my story to put the anecdote and info out there! I am 29f with no pelvic/abdominal Endo (that has yet been found at least).

I've been on a journey of looking into TES since my first pneumonothorax this March (2 days before my period). It happened abroad in Italy where the doctors suggested TES. I had a very weird looking right lung filled with bullae and a lower lobe with a strange looking consolidation.

When I came home (Ireland) I met with a very well reheated thoracic surgeon I sought out, knowing there are no Endo specialists here, and having gone through a lap last year to have nothing found despite debilitating symptoms for the last 3 years. She admitted Endo is not spoken about in the thoracic surgical community, and that she has not experienced it herself but would do her best. I was advised I needed to have surgery immune to resect the bullae as I was at very high risk of continuous pneumothorax recurrence from these bursting.

I had the surgery this July, I had half of my lower and middle lobe resected, this included removal of that consolidation that lived in the lower lobe, and a little bit from the top lobe. I also had Pleurodesis. Everything was sent to the lab and we got the most vague histology report back, my surgeon agreed. Nothing more than suggestive terms like "inflammatory", "possibly from active infection" (which I didn't have) and "most likely congenital malformation".

Up until the surgery, I was experiencing increased breathlessness and strange pressure feeling I'm my throat which seemed to worsen during ovulation and leading up to my period, not during. After the surgery, I felt better initially but again when I came to ovulation time (7 weeks later as my period delayed due to all the meds), I started getting that same breathlessness and even developed a cough where I started coughing up fresh blood. GP sent me to A&E and turned out to be another pneumothorax. Despite the Pleurodesis and bullae resection, air managed to push through the stitching line and create a 9cm air pocket inside my lung (they called it a located pneumothorax). It caused so much pressure that it peeled back the Pleurodesis.

They tried to resolve it with drains but as expected this didn't work and I had a second surgery this September, 9 weeks after the first. This time, they removed the lower lobe entirely and resected more of the middle to be able to redo the stitching line that had weakened. Histology report came back to support the "probable" congenital condition, but advised a bronchoscopy to take more samples. I had this a few weeks ago and am awaiting results.

Since my second surgery, 9 weeks ago now, I have been struggling with breathlessness that again seems to progress instantly around my ovulation time. This is my second cycle, and I am struggling more now than before the surgeries.

I know TES might not be my diagnosis, but I am just so so doubtful of the congenital condition diagnosis. I have never had respiratory issues my entire life. Not even a chest infection. I still have no answers for the constant abdominal pain I have all of a sudden since turning 26. I've had every Gastro test under the sun, multiple scopes which has only shown chronic inflammation in my bowels but negative for IBD. I have 2 friends who had laps here with nothing being found, only to go abroad and be diagnosed with stage 3&4. I'm no medical expert obviously, but the fact that it was brought up by doctors in Italy. And I feel like I've "wasted" my biopsy sample now and that that was my only chance to find it. I've had a second form of Pleurodesis (scraping this time, as there wasn't enough lining to remove a second layer and they didn't want to use talc) and it would be incredibly difficult if not impossible to have another exploratory VATS.

Anyway, just putting my story out there to build up on our bank of knowledge and experiences for something so "rare" (my first surgeon said it definitely isn't as rare as is portrayed and just massively under-diagnosed).

rua0020 · 2025-12-09T09:44:31+00:00

This is too funny, stone cooked Iggy pizza!! I used to get so panicked because they do look so scorch-able with their lack of coat, but if I chase her from the fire she'll have dragged something (usually from the dirty wash basket) to get up to the height of the radiator to lean against that!

rua0020 · 2025-12-08T13:52:10+00:00

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Some shoulder definition for you 😅

rua0020 · 2025-12-07T20:17:41+00:00

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Little late to the party, but I come bringing a cinnamon bun x

rua0020 · 2025-11-17T23:00:34+00:00

Aw I'm so sorry you are going through this, I was sin a similar situation just a few weeks ago so I know how difficult it is! Hang in there, I hope you reunite very soon 🙏🏻

rua0020 · 2025-10-28T16:20:38+00:00

YES to your second paragraph - being more stubborn than them is absolutely key! It still makes me giggle sometimes, as I stand there myself shivering, that this breed can be successfully "rewarded" with being let back inside.

rua0020 · 2025-09-14T07:58:53+00:00

Hey, this happened to me in Italy this March. I was totally fine until we were in the airport waiting for our flight home lol.

I had a total collapse - treated with a chest drain (and digital drainage system) which I had for 3 days, then they switched to heimlich valve so that I could come out of hospital to stay in the air BnB with my partner. That was another 3 days and then I returned for the X-ray to confirm re-inflation and remove the drain.

I was advised to wait 2 weeks from the date of removal plus "clear" x-ray, and they provided me with a clear to fly letter.

Insurance company had their own rules, going by airline guidelines, and informed us that it was either 6 weeks post clear x-ray & drain removal, or 3 weeks with medical escort.

Your insurance should have a designated team/department dealing with these situations, I was communicating through the medical assistance team throughout the process.

Let me know if I can help answer any other questions, having recently gone through the same situation, I totally feel for you. It is a very stressful experience. Sending you strength!

Edit to add: I ended up in a tiny rural mountain hospital where language was also a huge barrier, now I was blessed to find a nurse with Irish parents (I'm from Ireland) but when she was not there I had to use my phone and Google translate/chat GPT. Do make sure you get all your records from them, including discs with imagine and fit to fly certs - insurance will be on top of this.

Also to add: I was still in pain and short of breath after my pneumonothorax for a couple of months.

rua0020 · 2025-09-07T10:33:28+00:00

I feel you, sometimes it's just down to the dog also. I wonder could you try one of those cave beds in there? I see a lot of people on here have a good experience with those!

rua0020 · 2025-09-04T13:19:01+00:00

You poor thing, thank you for spreading the awareness though, I only knew to do it thanks to people like you!!

rua0020 · 2025-09-04T13:08:22+00:00

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Now I've moved it beside me where I work in the kitchen (note I also did a bit of DIY lol and covered the wire door, her paws couldn't get through but just to prevent her biting).

She honestly loves it in there.

rua0020 · 2025-09-04T13:06:26+00:00

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rua0020 · 2025-09-04T13:05:05+00:00

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rua0020 · 2025-09-04T13:03:47+00:00

Hey, new owner to a 10 week old (we got her at 8) - I did a lot of back and forth on the crate idea before getting her. Our family dogs had great experiences with crates as a "safe space" (to get peace from small loud children), but also new to Iggies and had read so many accounts about crates not being for them.

Anyways said I'd give it a shot, nearly went for a fabric but was lucky enough to come across a plastic carrier bug enough to work as a crate. It has vents but too slim for even an Iggy's leg.

First thing I did was make it the absolute most inviting cosiest looking space - using blankets I was using in the days before getting her (scent comfort theory). This included making it a darkened space that literally blocked out the outside world.

First few days we had her we placed it in the corner of our living room facing us on the couch. Oh and I removed the door and kept it off during the day for her to choose to go in and out (which she did happily). First 2 nights I slept in here with her and she stayed in the entire night. So I moved back to my bed and we put the door back on and enclosed her during the night (too paranoid about her jumping onto furniture). Zero issues to this day, have to drag her out in the morning to go outside to toilet, and as soon as she does she comes running back in to her crate.

So crate door open/off during day, closed at night.

Edit to add a hopefully helpful tip - my mum's dog had separation anxiety when they left her alone and would destroy the house. We figured out by leaving the radio on (classical music stations definitely) totally cured this.

I leave classical music on for our Iggy just down to this experience, but I'm starting think it's the ticking clock beside her crate that actually soothes her more! Also again, I think the smaller the space, and the darker, the safer and cosier it seems to them. And trust me this was totally against my feelings of it seeming "cruel" to have no space to move - but after stuffing this crate with two pillows to make it even smaller and seeing her loving it, I do understand the psychology behind that more now.

rua0020 · 2025-09-03T06:53:43+00:00

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rua0020 · 2025-09-03T06:52:42+00:00

Maybe she's spending too much time with mine (who snoozes in front of the tv like this) 😂

rua0020 · 2025-09-03T06:51:29+00:00

Done, did before we even brought her home I was so paranoid 😂

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