Hi - I have PPFE

[RaspberryRed]

Hello, I was diagnosed with PPFE last year. I just found out about this forum and would like to get in touch with others with the same ILD.

[Patk1]

Welcome to the forum x

[Alberta56]

Welcome to the forum. Hope someone with your condition will be along soon.

[Izb1]

Hi and welcome to the site RaspberryRed. I have never heard of your condition but am sure others have and will, be along soon x

[watergazer]

hi and welcome. I’ve not heard of your condition before x

[BionicLady]

Hello,

Welcome 😊

I have Bronchiectasis and year before last I was diagnosed with PPFE which is some sort of scarring.

What are your symptoms? I'm not sure I can tell you much about it as I'm not sure myself how different I am meant to be feeling and was surprised I had it.

💕

[RaspberryRed in reply to BionicLady]

hi BionicLady,

thanks for your reply. I’ve never joined a forum like this before.

PPFE is a sub group of IPF I believe. It stands for Pleuralparachymal Fibroelastosis. I started with an irritating cough about 18 months ago, gradually became short of breath when out and about, walking up hills, hiking etc. Went to the GP about a year ago - chest X-ray showed a lot of pulmonary fibrosis. It’s progressed so I now use oxygen for all activity (but not at rest yet). Following many tests with my local ILD team I have been referred on for lung transplant assessment ( 4 days next month).

There is no other treatment. I’m also fighting weight loss which is a characteristic, and have bouts of largely dry cough. I’ve had a small pneumothorax so far which is also a feature of PPFE.

Does any of that seem familiar to you?

[BionicLady in reply to RaspberryRed]

This forum is can be very supportive, especially when feeling so low and vulnerable which most of us feel like either some or most of the time.

Oh yes, the breathlessness did really come to light now I think of it, tight chest before I was diagnosed.

Because I've had Bronch for so long, I usually put every symptom down to that, which is probably not a good idea. I had pneumothorax years ago which managed to sort itself out and was due to a very bad infection.

How do you feel about the transplant? I wish you all positivity that you can move forward with that 💫

[RaspberryRed in reply to BionicLady]

I’m sorry that you’ve had your condition for such a long time; I know very little about it. Are you doing OK?

I guess I’m finding it so hard because I was very fit and well until just over a year ago and it’s all developed very fast.

Tbh I’m pretty scared about just going in for the assessment tests, let alone the prospect of an actual transplant. Still, first I have to pass the tests, then, who knows, I could ‘age out’ before the opportunity for a transplant comes up or not make it.

So one step at a time.

[Kcn3]

Ppfe is not the same as IPF. IPF is within the lungs and PPFE is on the edge ,the pleura.Is your PPFE idiopathic, cause not known? Or is the possible cause known?

A lung transplant is the only way out of PPFE. But that too has life long consequences which will be explained when you go for the transplant assessment. A transplant is not a cure, it will extend your life. It is the hardest of all solid organ transplants to go through.

Have you been out on Ofev? Or is your weight loss given you a low BMI?

What is your lung capacity at the moment?

Please keep as active as possible, as PPFE does pull the lungs in as the fibrosis increases, shrinking the lungs and making them hard. This cannot be reversed.

By keeping active so having to breathe deeply you will be fighting against the PPFE . You breathe deeply keeping the lungs expanded fighting against the fibrosis pulling them in.

Dodgylungrunner seems to be doing well from his last post. So maybe you could message him.

Sorry for the blunt information but no point in lying. It is very hard when there is so little to help with PPFE.

My daughter and I have been and will continue to fundraise for Joe Jacob at UCLH, specifically to help research into PPFE caused by a bone marrow transplant.

[RaspberryRed in reply to Kcn3]

Thank you for your reply. I should have said I have Pleural Parachymal Fibroelastosis which is a pulmonary fibrosis rather than say IPF. Unfortunately I’m only too aware of my options, which if I don’t clear the transplant assessment are 0.

They have identified I have a tert gene mutation which may be responsible - I’m seeing a geneticist next week to find out more. You probably know PPFE can follow 2 pathways - slow or rapid decline. Genetic mutations are usually linked with rapid decline ☹️

I had 2 lung function tests - unfortunately I can’t get good results (also quite common with PPFE I think) but I had FVC 1.79, FEV 48% , last summer, then 1.67, 46% in November. My recent appointment was cancelled as I had a pneumothorax about 6 weeks ago. A big decline set in late February- ironically while I was nearly through a pulmonary rehabilitation course! Just started using ambulatory oxygen in March but it’s already been stepped up from using an Inogen out and about to hi-flow concentrators at home and cylinders out and about (just last week).

I don’t take antifibrotics as I lost about a stone last year and am having to work really hard at my diet to keep above the 20bmi required for transplant. I’ve also read some Japanese studies that found antifibrotics do not work with PPFE.

Getting the motivation to exercise is hard - I was due to start another set of pulmonary rehab with oxygen but it was postponed- again for the pneumothorax.

I’m very sorry to hear about Joe Jacob. I have read that transplant can trigger.

[Kcn3 in reply to RaspberryRed]

Yes reports in Japan do state the Ofev can make it worse. Dodgylungrunner on this group has been doing well with it.Ppfe is an ILD and is in a different place within the lung than PF.

It's good you're going to do another course of rehabilitation.

At Papworth the criteria 3yrs ago was a lung function of less than 40% to get on the transplant list. So good that you've been listed for assessment .

By the time my daughter was given her Inogen she wasnt able to breathe deeply enough together it to work, so she too had too cart back backs of oxygen which also got heavy and ran out quickly. So we had to always take a few extras tanks when we went out.

Joe Jacob is the consultant radiographer who is doing the research into PPFE, that we are fundraising for to contribute towards his research.

Justgiving.com/Nathaliesoffe