Good morning. Around 13 years ago, following my very first chest x-ray, it was discovered that I have a hypoplastic right lung with pulmonary atresia. This very rare condition means that I basically only breathe with my left lung, which has grown to become sufficient enough for my needs. I now have bronchiectasis and recurrent chest infections.
When it was first discovered I had numerous tests and images, and both my respiratory and cardiac consultant were satisfied that I needed no surgery, just a watching brief as such. Since then, I have lived with the knowledge that I am very unusual and have felt that my condition just confuses many health professionals, who continue to tell me that I'm "okay". I don't feel okay, I feel a bit of a freak, I've tried to find others with my condition to compare notes, but to no avail. I have no idea of my prognosis, not sure the consultants do really.
I have recently moved into a different health trust area and am yet to see a consultant who has encountered my condition before. I've also taken a turn for the worse and am getting increasingly concerned about a change in my mucus, extremely noisy breathing and general malaise. I'd love to know if there's anybody else out there who has this condition, please message me! Thanks for reading 😁
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Deb_Dragon
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What a difficult position you are in. And I do understand your feelings of alienation. I'm not sure where you live, but would it be possible to be assessed by a specialist at a leading pulmonary hospital? I think the Royal Brompton in London, is a famous one. For all I know you may have already done this? It does sound as if you need more specialised care.
My condition is rare but not as rare as yours. I have more or less exhausted all pharmaceuticals and now am on the lung transplant list. Has this ever been mentioned as a possibility for you?
I think it is important to talk. Even though all of us here have different conditions, I think being able to share and support each other helps us so very much. Please do come here often. I'd really like to know more about you and your condition.
If you feel like you are worse, then please do keep pointing this out to your medical team. My lung function test came back with no change from last year which made me very happy. However, I know for sure it has become much harder for me to function. It's really not all in the numbers. Have you had a lung function test recently? You ARE NOT alone. 😊
Cas, thanks so much for your kind reply. I believe my condition is known as Scimitar Syndrome, although that's not been mentioned by any of my consultants. I found some information online about it but it appears that it is mainly linked to infant mortality, so I suppose I'm lucky to be here at all!
There is no pulmony artery between my right lung and my heart so the lung is cystic and redundant in the lower parts. I have had a full MRI of my heart at John Radcliffe and it appears to be operating normally, although there is some question about pulmonary hypertension in the right chamber.
I have just had another ECG and had plenty of lung function tests. It was initially thought that I might benefit from removal of the right lung completely, but once they had monitored me for a while and established that I have a full lung function with my left lung then they decided it was unnecessary. I do however now feel that my bronchiectasis has worsened significantly because if recurrent chest infections.
It doesn't help that I moved and the consultant who has been dealing with me in my new health trust is, by her own admission, a generalist. She has asked for me to be seen by a more experienced colleague. It's only taken a year of me raising my concerns and being told it's what I should expect. I KNOW there has been a change in my breathing and my mucus, and it is making me very anxious! It doesn't help that I had a few episodes of coughing blood around 9 months ago and my breathing is worse since then.
I'm sorry to hear of your challenges...would you mind explaining your condition to me (in private if you prefer?). I wil certainly mention the Royal Brompton when I'm eventually seen next (3 months wait apparently 🙄). I might well be useful for their research team if nothing else 😂 Here's hoping someone listens to me soon...
Please don't give up being an advocate for yourself. I know it seems like no-one is listening or understands, but I think if you keep at it, it will get through eventually.
Thank you for wanting to know about my condition. I have Bronchiolitis Obliterans, a very rare disease of the small airways. Basically, the bronchioles seal off or "obliterate". This disease is a manifestation of Rheumatoid Arthritis, a disease I have had for ten years now. I have had my lung disease for almost four years I think.
I'm sorry about the horrible episode you had nine months ago. Just know, you can come here anytime, there will always be people who care.
Thanks Cas, I too am awaiting diagnosis of either RA or Lupus. My rheumatologist thinks my bronchiectasis is a bit of a red herring as it's caused by my congenital condition, but I'm beginning to wonder if my latest problems might be a complication of RA. I had an Anti-CCP result of 900+ which indicates a high probability of RA. I also have the other physical symptoms but was told two years ago that I had Fibromyalgia because I had no markers in my blood at that point. I was never given the anti-CCP result back then as it was still outstanding at the time of my consultation 🙄 Could have been an overlooked positive? I will ask some questions!
You sound even more complicated than I am, and I do feel for you. Every time I see any new doctor virtually the first thing I hear is 'you're very complicated aren't you?". I'm sure you get the same. It doesn't help your confidence does it? I do think that you should insist on seeing someone who knows what they are doing, and the Royal Brompton is the place to go. Since I moved I have been seen at the adult congenital heart disease clinic at the Bristol Royal Infirmary, who have found other things wrong with my heart, as well as pulmonary hypertension. I also have bronchiectasis and obliterative bronchiolitis, which causes pulmonary fibrosis. The first cardiologist I saw and my respiratory physician are getting together to see if there is anything they can do to improve my quality of life, which would be nice, but it has taken two years to get this far. The NHS is definitely more tortoise than hare I'm afraid. I do hope things improve for you soon, but don't put up with second best. Good luck xx
Thanks Carnival, yes I do get the same 🙄 My initial diagnosis was from one of the most senior respiratory consultants in Wales, and he told me I was his first case!! I then moved to Gloucester and was happy with the care I received there, but I think things have fallen by the wayside somewhat since my return to a different health trust in Wales.
I am getting extremely anxious about the changes, especially since I was diagnosed with stage 1 melanoma in January. It's all been removed now but I spent a particularly difficult 5 weeks assuming my lung issues were linked to that. As you do 😒
I am going to ask a lot more questions at my next appointment. I think I've just been fobbed off so far because nobody has come across a case quite like mine. Good luck to you in your daily struggles 🍀😁 xx
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