PPFE from the land down under

Hello my first post - please be gentle 😊 I am also an Aussie, so greetings to all of you. I have PPFE which is declining. Would love to hear from people who have the same condition. I need to learn! Thanks

[CDPO16]

Hi and welcome. What is PPFE?

[Hidden]

Welcome. And wow, that’s a rare diagnosis, indeed. The numbers are going up now as it’s increasingly being recognised as a separate entity to PF/IPF, so there are people out there that just haven’t been given the correct, PPFE label, but up to 2017, there were only 100 diagnosed cases worldwide. It was only described in a medical paper for the first time in 1992, and the first paper in English to mention it wasn’t published until 2004.

I could be wrong, but I’m not aware of anyone else on the board at the moment with the same diagnosis, and would definitely remember if I’d seen it mentioned because of how rare it is. However, there are a number of people here with other ILD diagnoses, so it’s probably still going to be a useful place for you to hang around and have a chat. If nothing else, we’re a friendly and caring bunch that get what it’s like to live with - or care for people with - lung conditions.

[SORRELHIPPO]

I am not the slightest help with regard to your diagnosis, but, am feeling a little raw around the edges when It comes to Aussies at present. You can have a smile at an England cricket fan, after the men's Ashes this winter, with me now following the Women's one day World Cup, when, who do we meet in the final, the Australians. Hope you get some help on here.

[Hidden in reply to SORRELHIPPO]

I do believe you’re a bit of a cricket obsessive, SH! I love it too but I don’t stay awake all night like you 😊 This year, England were a disaster, an embarrassment in fact, so I hope the women do better! X

[SORRELHIPPO in reply to Hidden]

Well we have made the final.

[Hidden]

Sadly, we’re all in shock from the death of one of our loveliest members, so bear with us, mija. You are very welcome here, we have members from across the world and it’s a very friendly & supportive forum. Does treatment for PPFE vary much from ILD/PF? Because there are quite a few members with those ailments who might pick up on your post. I do hope you’re under the care of a good hospital, in my experience (of a different lung condition) that makes a massive difference x

[cofdrop-UK]

A very warm welcome to you mija1234. Cx

[Alberta56]

Welcome to the forum mija. We are a friendly and sometimes funny group, though at the moment we are all sad and rather subdued. We can't give you medical advice, but perhaps hopefully can pass on hints about the techniques/ gadgets we find helpful to cope with lung conditions. Anyway we're always here for a laugh or a moan. Best wishes. xxx

[watergazer]

Hi and welcome mija1234. I am unaware of PPFE Please explain what is is whenever you have time. X

[Izb1]

Hi mija1234 and welcome to the site. I have read through Charlie G reply and it really is a rare disease so cant offer any advice, but please keep us updated on your progress it may help other members. Its also good to compare treatment to other countries. I hope you have managed to dodge the dreaded covid over there and are keeping well x

[Kcn3]

Hi mija1234.Dodgylungrunner on this site has recently been diagnosed with PPFE. I believe he is in the uk.

I know what PPFE is.

How is your lung function at present? Are you on supplementary oxygen ?

Has a lung transplant been discussed?

Like another reply PPFE has very much previously been diagnosed as IPF.

I was informed by the Brompton in London that most of the research for this horrible disease is being done in Japan.

I am in contact with a radiologist at UCL in London and fundraising to help research at UCL. The radiologist says drs and consultants are not recognising how PPFE looks on scans and xrays and his aim is to educate these people to spot it and the hope is in 5yrs drug trials will be possible.

More cases are being diagnosed as knowledge increases.

Have you been told what has caused your PPFE? Have you had a bone marrow transplant?

[Hidden in reply to Kcn3]

I’ll just tag Andy dodgylungrunner in hopes he might see mija’s post…

[Hidden in reply to Hidden]

Thank you!

[dodgylungrunner British Lung Foundation in reply to Hidden]

Thanks for the tag and apologies for only just replying! I've been out of the loop for a while but I'd be happy to chat to you Hidden at any time and tell you what I know and how I've been managing it as best I can over the last 8 years.

[Walkwalkwalk]

Hi Mina. I do have the diagnosis of PPFE. it was not attributed to me until 2015 when ( I partly joke about this) my consultant was trying to make sense seeing that I was still functioning after an original IPF diagnosis in 2006.I was new to him in 2015 having moved within the U.K. At first he questioned the original diagnosis

( I think perhaps as I was still alive?) but that was reaffirmed. Then came the exploration to label PPFE.

Charlie is right in saying that there are probably more cases than thought. I suspect that mine goes back to 2006 diagnostically.

Research is beginning and the PPFE You Tube Group does have some information.

I am avoiding this becoming too long as a reply but I am very keen to make contact with others with this diagnosis so please be in touch.

Grace

[CDPO16 in reply to Walkwalkwalk]

I'm sorry that you find yourself with this diagnosis too Grace but at least one or two of you can now support each other as well as the general support available from the forum. I hope that you continue to 'function' for a long time yet. Carole xx

[Hidden]

Wow that is exactly what happened to me! Will be in touch 😊