PPFE

Hi Redsheep,

There are some folks on this forum who live in Australia; I'm sure they will be able to tell you about PPFE specialists and hospitals.  I live in the USA, so I can't help much.  Regarding lung transplants:  'Joe' has posted really nice, thorough information on this site; he had PPFE and has had lung transplantation.  I'm sure you'll find his posts very interesting.  Best wishes to you.

Cheryl

 

[Respiratory function and alveolar biological changes under the effect of CDP-choline in pulmonary interstitial pathology: pulmonary fibrosis and sarcoidosis].[Article in French]

Pacheco Y, Douss T, Pujol B, Revol A, Vergnon JM, Biot N, Brune J, Perrin-Fayolle M.Abstract

Various anomalies of pulmonary surfactant have been described in relation to acute respiratory distress syndromes, hypersensitivity lung disease and pulmonary sarcoidosis. Phosphatidylcholine (PC) is the essential phospholipid component of pulmonary surfactant. Cytidine diphosphocholine (CDP-choline) is an essential intermediary in the biosynthesis of PC. The authors studied two groups of patients: one group consisted of diffuse interstitial pulmonary fibrosis and the other consisted of pulmonary sarcoidosis with parenchymal involvement. They observed quantitative and qualitative abnormalities of the phospholipid fractions of surfactant and more particularly of PC. The finding of a marked decrease in this phospholipid, especially in the cases of pulmonary fibrosis, justified the study of the therapeutic effects of CDP-choline. After one month of treatment with this substance, at a dose of 1 g I.M. per day, the PC fraction had returned to normal and, at the same time, there was an improvement in the PaO2 at rest and after exercise. Long term administration of CDP-choline appears to be valuable in the maintenance of the phospholipid equilibrium of pulmonary surfactant and in the improvement of the quality of alveolar gas exchange.

Hi Redsheep.

We live in Brisbane. My husband Brian (70) was diagnosed with PPFE 12 months ago after telling doctors for 4-6 years his breathing was getting worse.His FVC is now 67% and gas exchange 49%. His FVC was 114% when he first complained and perhaps 140-150% originally. We have just come back from 3 months overseas where we did some good walks but Brian's ability to go uphill is decreasing and he needs to do this quite slowly now. He sees specialist in Brisbane. We go to Melbourne frequently. His specialist is asking for permission to use one of the antifibrotic drugs but there is no proven benefit yet for PPFE. We think transplant is out at his age and it is also fairly rare for PPFE but Jo is a great living example of a success. They haven't done any transplants in the UK for PPFE and I don't think any in Australia.

I was about to write in Brian's profile the progress of his diagnosisi and treatment rather than repeating it all the time.

There is now a good Facebook site called Pleuraparenchymal Fibroelastosis Support group. There is a Private Group and a Community Group.

I will post more later if there is anything specific you would like to know.

Best wishes

Julie

Hi Redsheep,

I am hoping you are ok and have perhaps found a good specialist in Australia. I did reply but didn't mention our specialist professor Dan Chambers in Brisbane -- northside, he has a clinic at Prince Charles Hosp and private rooms nearby but you may not be in Brisbane or Queensland. It would be good to be in contact anf share information. I fyou care to reply to this and I can send youa private message,.

Very best wsiehs

Julie 

Hello Redsheep,

I also have a recent diagnosis of PPFE. I'm trying to find others who have it, I am hoping to find and connect with others who have it. There is a facebook group for PPFE that could use more people there, I just found it myself.

I'm also learning and have many questions about PPFE and how it affects our future. I did also hear about lung transplantation and there seems to be many here in the USA who have done very well getting new lungs after being diagnosed with a lung disease.

My doctor did lots of blood tests for autoimmune diseases, also a blood test for "short telomere" disease that was sent to John Hopkins, I'm awaiting results.

I am still not sure how bad it is, or how fast it is progressing, I guess in some time we will know those answers too. Meanwhile, I'm learning what I can. I have also found a couple other facebook groups for lung diseases that have been helpful. And forums like this one are helpful too. Have you checked out the Inspire dot com website? There is a forum for interstitial lung diseases and pulmonary fibrosis. I'm hoping I can find many helpful people here too on this forum.