Ppfe

[Kcn3]

Last week my daughter was confirmed as having ppfe. She had a bone marrow transplant 11 yrs ago as she had acute lymphoblastic leukaemia.

Is there anyone out there in the same situation?

[Janzo54]

I dont know anything about this but just sending love and hugs 💓

[Caspiana]

Hello Kcn3 ,

Sorry to interrupt your post but I think many people don't know what PPFE is so I shall just add a little explanation. Maybe you will get more responses that way.

"Pleuroparenchymal fibroelastosis (PPFE) is a rare pulmonary fibrosis that is clinically characterized by upper-lobe predominant fibrosis. PPFE is a slowly progressive disorder and its first symptom is dyspnea or dry cough".

I'm sorry I don't know anything about this disease but I hope someone with a similar experience can help you. Best wishes to you and your daughter.

Cas xx 💐

[jmsutt73 in reply to Caspiana]

Thank you, Caspiana! I asked the lady before I saw your explanation. Hope all is going well with you. I finally go a firm diagnosis last week. Will go on Ofev tomorrow. Hopeful! Much love, J

[Caspiana in reply to jmsutt73]

What did they diagnose you with jmsutt73 ? xx

[Kcn3 in reply to Caspiana]

My daughter was diagnosed with Ppfe, that you posted earlier .

[Caspiana in reply to Kcn3]

Hello again. My comment was to Jmsutt. 😊

[jmsutt73 in reply to Caspiana]

IPF was first mentioned as a possibility in November 2017. Minimal volume loss was noted on ct-scan. Then it was maybe, maybe not; radiologist's note after an HRCT in January 2019 said "possible fibrosis." In May 2019, my pulmo said to have PET in Nov 2019, did and he thought it looked as if the 'minimal volume loss' had progressed, so another HRCT. That one left him with no doubt, plus the radiologist who read it is highly regarded in this area. I begin Ofev tomorrow. Sure praying that it slows it down; pulmo said "too old for transplant." I am eternally grateful that this did not show up before my daughter passed, and that I was able to give her the care that she needed. Thx for caring, Caspiana. J

[Caspiana in reply to jmsutt73]

I am glad you got a diagnosis jmsutt73 and yes, I am glad you could see your daughter through her illness. I often feel the same about my children. I fortunately did not lose them, but I feel grateful my diagnosis came after they were grown up. I don't know how I would have managed with RA and BO if they were still little. xx ☘️

[Hidden in reply to Caspiana]

You would have though. I have BO and RA Plus a couple of others and I still look after my granddaughters mainly the 2 year old while my daughter works! It’s amazing what you can do if you have to. By the way I am having Rituximab regularly really helping with the BO

[jmsutt73]

So sorry to hear about your daughter. Sounds like she has had to contend with a lot of illness. God's blessings be with her. If you don't mind...what is Ppfe?

[sassy59]

I’m also sending lots of love and good wishes to your daughter. Xxx

[andersBE]

I just found your post my searching thru Google.

Yes, I have EXACTLY the same diagnosis as your daughter.

In 2009 I had Acute lymphoblastic

leukemia.

I had Total body irradiation and then an Allogeneic Bone Marrow transplant from my sister.

I live in Brussels, Belgium, but my donor (my sister) lives in London.

My disease first started "eating" (if we can improperly say so) my lung, but then became stable.

I have a full life, a job and PPFE has not limited my life in any way, apart not being able to do sports anymore.

Of course everyone is different, but there are other documented patients that with the same clinical history as me and your dauther developed a "stable disease" condition.

Follow-ups are NECESSARY and testing regularly the lung capacity too.

If I can help you in any way, please let me know...

[andersBE in reply to andersBE]

I would also add...

If you have PPFE, like me, catching the coronavirus can be deadly.

After consulting with my pneumologist, I am currently at home, I am homeworking and limiting to the maximum any contact with external people...