My wife has PPFE with failed lungs. What is the next step?

Question

Hello doctor,

My wife first developed upper lobe scarring several years ago, and it has progressively worsened on each follow-up scan.

Recently, the imaging showed biapical pleural parenchymal disease along with scarring in the right middle lobe. The disease has clearly advanced over time.

Last month, she experienced a bilateral pneumothorax. The scan at that time revealed severe bilateral parenchymal scarring, atelectasis, architectural distortion, and marked lung volume loss.

Pulmonary function testing has since confirmed that her lung function has failed. The report states that it is consistent with pleural parenchymal fibroelastosis.

There is also a significant family history, since her father died at a young age from pulmonary fibrosis. The doctors we have been seeing have done nothing.

Despite these serious findings, she has not been offered oxygen therapy, medication, or supportive care. The medical team advised only a repeat CT scan in one year.

Kindly help.

Answer 1

Hello,

Welcome to icliniq.com.

I understand your concern.

I am really sorry you and your wife are going through this; it must feel very stressful and overwhelming.

From what you have described, the CT (computed tomography) findings and family history are highly concerning for progressive pulmonary fibrosis, most likely pleuroparenchymal fibroelastosis (PPFE), which can cause scarring, lung shrinkage, and worsening breathing issues.

Since there has been clear progression, failed lung function tests, and a recent episode of bilateral pneumothorax, this is not something to wait on for a year.

I strongly suggest you seek urgent care at a specialized interstitial lung disease (ILD) or pulmonary fibrosis center.

There, doctors can consider advanced options such as antifibrotic medications (Pirfenidone, Nintedanib), oxygen therapy, pulmonary rehabilitation, and even lung transplant evaluation.

It is very important to request a referral to a transplant-capable pulmonary team rather than relying only on routine follow-up.

So I request, please do not delay, as progressive fibrosis requires active management and supportive care.

And the most important thing to consider is that if her breathing suddenly worsens or if pneumothorax symptoms return, go to the emergency room immediately, since it can become life-threatening.

I hope this information helps you.

Thank you.