Anyone in this group with constrictive bronchiolitis?

[daniellegagne22]

Hi i am writing on behalf of my sister who was diagnosed with with CB 9 months ago after having a biopsy a year ago today..we are identical twins by the way😊...im writing to get more information and see who has it as well to help us with a little insite of this disease...Shes getting worse and there is no cure for this lung disease..and she never smoked a day in her life!I don't want to looose my sis😭she has 4 kids that need her along with hee family..please if anyone knows anything and can help I would rallt2 appreciate it!

[Katinka46]

Constrictive Bronchiolitis is another name for Obliterative Bronchiolitis and Bronchiolitis Obliterans (not to be confused with Bronchiolitis Obliterans Organsing Pneumonia). And yes, I have it. It is rare but there are a few here on the forum with it. There is usually a cause: some autoimmune diseases can cause it, RA or Lupus, and also toxic inhalations or some infections.

I am sorry that your sister has been diagnosed with it. Does she know what caused it? Where are you? UK? America?

Are you/she happy with the doctors she is seeing? I am on Azithromycin which can help to slow down or even stabilise the condition. I will find out from. PFT tomorrow if, after 6 months, there has been any changes. There are some trials for OB patients using Perfenidone which is used for Pulmonary Fibrosis. The reasoning is that OB is also a fibrotic condition.

Please keep in touch as us OBers need to stick together. Rare diseases are known as Zebras, hence my Avatar. The Zebra club is not one anyone would wish to join but we can support each other.

All the best

Kate xxx

[daniellegagne22 in reply to Katinka46]

The doctors don't know how she got it! No autoimmune disease they already tested her for that..but she had a tumor removed 4 years ago..called castlemans disease...that they only found after she started feeling out of breathe..she googled it recently and it is known to cause ob!crazy how the doctors didnt put 2 and 2 together...we are from USA..but now she is feeling worse and has pulmonary hypertension can't even walk up a flight of stairs without feeling out of breathe.The pulmonologist is nice and listens to her..but we feel that he doesnt know that much about this disease...he still can't somehow believe it...she recently went to Boston mass...to see if she had possibly blood clots in her lungs..because her lungs dont look too bad to how she is feeling..but that was a negative..no blood clots at all...so it is all the lungs doing...kind of feels like were being the doctors as well as her being the patient...and worried that there just wasting time to where she is going to need a transplant soon..they say it looks like her lungs are 10%scarred at this point..but her pulmonologist said she shouldn't be feeling the way she.does...she is on prednisone and also oxygen at night...how long have u had this disease? Are you on oxygen?? And do u do daily with regular house work ans stairs??sorry for all thw questions..but We dont know where to turn..I am looking to try to find a specialist that handles ob near CT but can't seem to find one..I just told her about this group so hopefully she joins this convo soon 😀😊

[Katinka46 in reply to daniellegagne22]

Hello again. One of the frustrations of OB is that the symptoms are worse than the numbers from the tests suggest. Which makes it hard to diagnose. And, for me, too easy for doctors to dismiss my concerns and think that I was exaggerating my symptoms. The small airways are sometimes known as the “silent zone” for these reasons. I am 71. It seems that the damage to the bronchioles was caused by PEs (blood clots in the lungs) that I got after a hip operation in January 2010. It was an unusual spread and extent of PEs. No-one has ever come across anyone developing OB from PEs so my consultant (pulmonologist to you) is going to do some research. It took 8 years to get a diagnosis.

I struggle to get around, even in the house, but I’m not on oxygen. I have to stop all the time to recover.

K x

[Hidden]

Just a little thing that I hope will cheer you up. I have a colleague in her 60s whose Mother has lifelong OB and is in her 80s keeping everyone on their toes.

I hope that you and your sister get lots of support from the lovely folks on here.

[daniellegagne22 in reply to Hidden]

Are sure its the same one?

[Hidden in reply to daniellegagne22]

On readin wheezyofs post it seems so but I am no expert in it.

[wheezyof]

Hello,

I'm sorry to hear about your sister. I too have OB. I'm in a better position than her because my family have all grown up.

I found it difficult to get information about OB.

Some sites on the internet do read as doom and gloom, so I don't bother with them.

Healthy eating, decaff tea and coffee (to save drying my lungs), gentle exercise, pacing myself, resting when I need to and taking my medication all help.

I have azithromycin antibiotic 3 days a week and use 3 inhalers, ventolin, fostair and spiriva respimat.

I've 'met' some lovely helpful people here.

If you are in the UK you and your sister could join a Breathe Easy group. The British Lung Foundation will tell you of one near you. The groups are for people with lung problems and their family, carers and friends.

Wishing you and your sister well xc

[daniellegagne22 in reply to wheezyof]

How long have u had it for? And Are you on oxygen? Everyone keeps telling us stay away from google lol...it has hwlped us learn alot..but also scares the heck out of us

[wheezyof in reply to daniellegagne22]

No I'm not on oxygen yet. I first got ill four and a half years ago. Some of the search results from google are terrible. According to some of them I've only got six months left!

That's rubbish. I shall keep going for a long while yet. I've had to make some changes, I live in the slow lane now but as I'm 66 I would possibly have started to slow down soon anyway.

I've found new easier ways to do a lot of daily tasks and I accept that I can't do as much as I used to, however, google's wrong, I'm not dead yet. xx

[Caspiana]

Hello daniellegagne22 .

I have it too. Entering my fourth year and awaiting a lung transplant. 😊 I got mine from having Rheumatoid Arthritis for many years. xx 🌿

[daniellegagne22 in reply to Caspiana]

Oh wow...i hope you get ur new lungs soon!!😊

[daniellegagne22 in reply to daniellegagne22]

Are you on oxygen hun? They say her lungs are inflamed mostly..and 10% scarring ..but can they be wrong? Is there scarring they cant see?how do they know for sure...based on her breathing test...(forgot what its called)they said she should be 24/7 on oxygen or dead..her pulmonologist doesnt believe it's all her lungs doing her Symptoms..I know it affects the small airways...is that why they can't see how bad her lungs really are?

[Caspiana in reply to daniellegagne22]

Hi there. 👋

They can't see much scaring on my scans either. But without a doubt my lungs are very damaged. I am on oxygen when mobile. My lung capacity (FEV1) is at 35% and my oxygen only drops when I am moving about. How about your sister? What is her FEV1? How much oxygen is she on? Have they mentioned a possible lung transplant? Have they said they want to do a lung biopsy? I did not have a lung biopsy. They made the diagnosis based on my spirometry test, scans and six minute walk tests. I did so many of these initially. I can't remember how many. I saw three specialists who have me they same diagnosis (I think it's important to get more than one opinion).

I think it's great you are being an advocate for your sister. Would she be able to join the forum as well? I can only imagine your level of worry. Take a deep breath. Take it a day at a time. And take care of yourself too. 😊🌸

Warm wishes,

Cas xx 🌸🌻

[daniellegagne22 in reply to Caspiana]

Yes she did a lung biopsy it is confirmed she had it about 9 months ago...her readings are coming up to about 13% which makes no sense to her pulmonologist! Shes only on oxygen at night 2L ..because when she lays down her oxygen drops ..it drops at times also with movement but not all the time..her pulmonologist said if she were to go now they'll laugh. Cause she's not there yet...the high resolutions scans are really showing nothing..and he said the small airways you can only see microscopic...her oxygen remains stable 90-93 usually unless she gets sick and coughs up mucus..yes I am soo scared for her thanks huh..I spend my days trying to figure this thing out! She's my ❤you know.

[Caspiana in reply to daniellegagne22]

13%?? Oh wow. Are they thinking of lung transplant? How long ago did she notice there was something amiss?

[ledge]

I have it too.... I've had it for what I can gather for I've had the disease for the last 3.5 years. When basically my fitness level dropped off a cliff and I found I couldn't even walk the normal half mile to work without almost collapsing. It took ages to be diagnosed, but eventually, a diagnosis came. I am I know on a downhill slope, but so far even with chest infections, I have managed to stay at about the same level. I do yoga once a week, pilates once a week, and a sort of aerobic body fitness class once a week too. I get whacked out quickly, but I have learnt to pace myself. No one knows how I got it, only I think I had a chest infection in the January, and by April I was on my knees as it were, which is how I went to the doctors to find out why I was so easily becoming breathless. I don't get a lot of support from my hospital, and my GP kindly as they are, are really not aux fait with the condition either. Anything I have learnt has been from this site.

[daniellegagne22 in reply to ledge]

How old are u if you dont mind me asking?and do you know how much lung function u have and scarring??

[Katinka46 in reply to ledge]

Hello Ledge

I’m sorry but I seem to have missed your posts or replies when you mentioned your diagnosis. I like to keep tabs on the OBers, because it’s rare and we need to share our experiences. Sorry to be bossy but I think you could do with more expert help from the medical profession. Where are you? UK?

All the best and keep us posted

K xx

[ledge in reply to Katinka46]

Hi Katinka46... thanks for your reply. Yes, I could do with more expert help - I am afraid, that my respiratory doctor, is weak in that department. To cut a long story short, I am being referred back to primary care as there is little he can do for me other than manage my symptoms. I was a bit downcast when I heard that. But I do have the support of the COPD respiratory nurse at my GP surgery. I am in Essex - I am due to have a fundoplication shortly, even though my lung function is poor, as it is hoped that my OB may have been primarily caused by my severe reflux and aspiration of acid into the lungs. It cant be ruled out either way if that is exactly the cause, but I do have both and reflux showed up about three years before the OB. But however, here I am with both OB and severe reflux.

I do read your posts, and its nice not being "alone" - but on the whole, I have remained relatively stable since my fall off the cliff lung wise . Even with a lung capacity of well under a litre.. 0.81 to be precise. But I manage to exercise as best I can, and well every day is a bonus for us all..xxx