Posted , 32 users are following.
Has anyone been diagnosed with pleuroparnchymal fibroelastosis (PPFE). Pulmonologist and radiologist think I might have it. Not much written on it - fairly new to the medical community, I believe. Prognosis doesn't sound good. Thank you.
Sherry (USA)
9 likes, 335 replies
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victoria48297 CherylH
Posted
Hi Cheryl.
I have recently been diagnosed with PPFE. I am only 29 so quite young so I'm told.
As previously stated by Paula PPFE is very rare there are only 120 recorded cases world wide.
There are also only a hand ful of Drs who understand the condition and are recognised as such.
Luckily for me I live in the UK and am been treated at the Royal Brompton hospital London ( where Paula's scans were sent) my consultant is one of the few who is researching PPFE as a condition.
I am having lots of tests so they can identify progression speeds.
In July I will have a CT/ MRI to monitor my venous structure in the lung. Also a nuclear lyphangiogram another lung function test.
I have had bloods to check my T cells.
Hopefully will get a better idea for the future but the condition PPFE does not have a great prognosis.
Nice to read all ur comments.
Victoria x
CherylH victoria48297
Posted
Hi Victoria,
Yes, you are very young to have the diagnosis. I'm 66 (not so young!). Fortunately, you are in a place that is up to date on the condition. I work at a big University in the college of medicine with a hospital connected, so my pulmonologist here probably knows more about it than the community pulmonologists in the area, but that isn't saying a whole lot. I check our National Institutes of Health publication Website for new research articles on PPFE, and there haven't been many new articles for awhile. However, it does sound like they have a pretty sound way of diagnosing the condition now. Yes, you are fortunate to be close to a medical facility and doctors who know a lot about PPFE.
Kind regards,
Cheryl
Spud22 victoria48297
Posted
Hi Victoria I'm Paula and I hope you don't mind me writing to you. I thought I was young being diagnosed with PPFE 4 yrs ago at the age of 46 but you beat me on that one. I would just like to say 4 yrs on and I am still walking, yoga, doing everyday normal activities I even ski'd last year although it was all down hill. So although the disease is progressing in my case it is at a slow rate. How are you feeling generally? What are your symptoms like? If you would like to keep in touch no worries I feel great just to be in contact with people with the same condition. Regards Paula
victoria48297 Spud22
Posted
Hi Paula.
I only got a diagnosis last year but have had symptoms for 3 years now.
I have a heart condition too so that is why it too a while to diagnose as they needed to rule my heart out first.
I had sudden onset of breathlessness, blue hands and lips. Extreme tiredness and chest pains.
I still get all these symptoms. I also get a build up of what feels like fluid in my upper chest.
I know the symptoms now so I know when I'm going to have a bad day/ week. I still work. I have 2 children one who has SEN so takes a lot of time.
Just got to get on with it. I do what I can and when I need to and am able to I rest.
Take care.
Kind regards
Victoria x
CherylH victoria48297
Posted
Hi Victoria and Paula,
I hope you don't mind my jumping in here -- I am curious about something. After reading a fair amount of research journal articles on PPFE, it appears that people diagnose with PPFE have certain physical characteristics: thinner stature, narrower (flatter) thoracic area. Personally, I do fit these traits. I'm wondering if you would classify yourself as having the same?
Regarding age: it seems that there is no age barrier in regards to PPFE, and I think the three of us prove that theory! For what it's worth though, I am much older, and I still work by choice, I go to the gym three times a week, and have five little grandkids that I run around with. I think I've had mild symptoms for probably 5 years. I also think that my condition has gotten slightly worse, but not by much.
I am very happy that we have all met each other via this blog. It gives me comfort being able to discuss this openly with others who are familiar with the condition (I never know if I should call it an illness or a disease, so I call it a condition). For each of us, I hope for the best and pray that we do well.
Best regads,
Cheryl
Trinitee CherylH
Posted
Trinitee victoria48297
Posted
CherylH Spud22
Posted
Hi Paula, This is Cheryl from the USA. I just wanted to touch base with you to see how everything is going in Australia
How have you been doing? So far, I think my PPFE has stayed pretty much the same over the last 6 months. Anyhow, I hope all is going well. I've been wondering how you and Victoria are doing. Regards.
CherylH victoria48297
Posted
Hi Victoria, This is Cheryl from the USA. I just wanted to touch base with you and Paula to see how you are doing. How is everything going healthwise? So far, I think my PPFE has stayed pretty much the same over the last 6 months. Anyhow, I hope all is going well. I've been wondering how you and Paula are doing. Regards.
victoria48297 CherylH
Posted
I am doing well at the moment. I have got a week of tests in July to work out the speed of progression of my PPFE.
Hope u are doing ok.
Regards
Vic
CherylH victoria48297
Posted
Hi Vic,
i will be keeping you in my thoughts in July. Hopefully, it hasn't progressed much at all. Hopes and prayers coming your way! I'm doing well.
Cheryl
Trinitee victoria48297
Posted
Hi Victoria,
Glad to hear you doing well. i was recently diagnosed with PPFE as well. Hopefully you've had minimal progression.
Take care,
Trinitee
Trinitee Spud22
Posted
i was recently diagnosed with PPFE and I am glad to have the opportunity to connect with others. Hope you are doing well.
Take care,
Trinitee
Spud22 CherylH
Posted
Hi Cheryl
?Im good thank you Cheryl its great to read yours and Trinitees comments like you the prognosis is always on my mind but I try and keep it to the back of my mind and carry on with day to day living... as you know I am being well looked after here in Sydney by both the Lung Disease Clinic and Lung Transplant Clinic (i am not on the list) they are both keeping and eye on me... but as said in previous comments there is no drug specifically for this disease and I don't think the prednisone or doxyline helps (only my personal thoughts)... with this in mind I approached the Lung Disease clinic and asked if there was any trial drug out there for a similar Disease and that I was willing to try anyting to prolong my life expectancy, I have now been taking the drug Pirfenidone for 6 weeks (this is a drug for Pulmonary Fibrosis and might help slow down the rate of fibrosis progression, it is not availalbe in Australia yet, hopefully in two years time but I have been allowed it on compasionate grounds) the down side is that it is 9 x tabs a day but I have dropped the other two drugs.... I can only keep my fingers crossed that it keeps my progression stable to which I won't find out for another year.... thankfully my progression has been slow but everytime I have a lung function test 3 monthly it is in decline... I am still very mobile, walk 3 km each day with Spud my dog and clean up after my two 17 year old boys!! I have only recently been asked to attend the Respiritory Rehabilitation, which I am booked in to start next week, but like you said Cheryl I think I am too fit but I will still go.....
?Trinitee you said you have read that only 3 IPPFE patients have had lung transplants do you know what the outcome of these are??? (3 isn't a lot is it considering its the only option we have......)
?Cheryl you also asked whether I have the slim trait and yes i am I never budge above above 59kilos and im 5ft 8.....but i have always been slim.
?Thanks Cheryl for keeping this feed open and your kind thoughts and that goes for you to Trinitee, it is good to chat and get our thoughts and experiences out there, hopefully we can help each other.
Regards
Paula
CherylH Spud22
Posted
HI Paula,
It was good to read your post. Sounds like you are doing well (maybe it's all of that exercise you're getting trying to keep up with two 17 year old boys -- whew! - and walking Spud.). It seems like the prescription drugs could be keeping your progression stable. One thing my primary care physician did tell me is that we all lose a certain amount of lung function after the age of 30 -- everyone does. So, we're all going to progress a little bit! I did read an interesting research article yesterday -- seems like Rosemary was useful in treating lung fibrosis in mice. Of course, they did all kinds of stuff to the Rosemary to extract the antioxidants, but it gives me hope that sooner rather than later our researchers will find something to help us other than lung transplantation.
Take care,
Cheryl
virginia29538 victoria48297
Posted
Hi victoria48297,
Wishing you well on your tests. Since my husband is in the middle of being diagnosed, I would love to know the type tests you are having in July to define progression! We weren't aware of any means of doing this! Wishing you well and keeping you in our prayers! Va.
lucretia54913 victoria48297
Posted
CherylH Spud22
Posted
Hi Paula!! We haven't communicated in a long time. How is everything? I manged to get by this winter (so far) without catching the flu (knock on wood for good luck); however, I had my fair share of colds; in fact, I have one now. I hope you're doing well. When you have an opportunity, please write. Best wishes, Cheryl