Initial Devastation. A diagnosis of one of the Interstitial Lung Diseases usually called Pulmonary Fibrosis - IPF (= Idiopathic (no known cause) Pulmonary (lung) Fibrosis (scarring) will do that to you and your loved ones. Doctors usually have a tough time breaking the news and many rely on grim statistics and your ability to go online and discover more about it yourself. Remember they are human too. The internet will give you the cold information on Idiopathic Pulmonary Fibrosis, Usual Interstitial Pneumonia, Farmers Lung, Interstitial Pneumonitis, Stiff Lung Disease, Hammond-Rich, Cryptogenic Fibrosing Alveolitis, and about 150 other types. Sadly, much of that information is old.
What it doesn't give you is a reason for hope or the glimmer of light you need to go on and there are lots of arguments for you to think more positive about your future than people who were diagnosed ten years ago. There are also many things that you can do while others (doctors, pharmaceutical companies, research labs and the government) are trying hard to find a cure / medication to stop or at least slow the progress of this disease. Here's what can happen: Just a few years ago being diagnosed with a disease called Pulmonary Artery Hypertension gave the patients a bleak future and now there are several medications to treat it and slow the progression down.
How does Pulmonary Fibrosis or IPF work?
Just think of a when you fell and cut yourself. Fibroblasts or the little repair units that we have in our entire body to prevent us from suffering continuous damage by wounds are activated in the damaged area. They produce collagen to repair the skin, stop the bleeding and as a result you see a scar where the wound used to be. Now imagine that you have an inflammation in your lungs that activates the fibroblasts to lay down layers of collagen even though there isn't a reason for it. Where in the skin it stops the bleeding and heals, in your lungs it makes the exchange of gases (Oxygen in - carbon dioxide out) harder. The process doesn't stop and it becomes harder to breathe once the wall thickening gets to an evolved stage. Over time less and less of the lungs is not affected and the breathing gets harder until you have to get on Oxygen treatment.
Idiopathic pulmonary fibrosis (IPF) is one disease that has no known cause, meaning that even a thorough evaluation can't find a particular reason for the lungs to scar and the scarring process to continue without stopping.
IPF is the most frequent diagnosed of the interstitial lung diseases.
Survival time for IPF is very often projected to be around 3 to 5 years but that's based on statistics and we all know how correct they are. (just remember our elections)
The number 3 to 5 years is already in use for more than a decade and it's like all statistical numbers very general. That number includes heavy smokers who come in at the end of the disease because they believed it was related to the smoking and way too many people who were misdiagnosed at first or who came in too late because of other reasons. They all drag the numbers down considerably. Although progression has been made over the last ten years with the introduction of the drugs Esbriet and Ofev we should see new ones in the next few years. Don't forget:
More trials than ever are ongoing to see if new medication works and more than ever pharmaceutical companies seem to have a genuine interest in bringing specific medication to the market.
This is a great film to help introduce you and loved ones to Pulmonary Fibrosis - see it at www.everybreathcountsfim.com
On the links and resources page we'll show you a few websites where you can go for the really difficult description of the disease but here are three important ones.
Patient Information Sheet:
Pulmonary Fibrosis Foundation
"Life is not measured by the number of breaths we take, but by the number of moments that take our breath away."
Make a positive commitment... and you'll move mountains.
"You gain strength, courage and confidence by every experience in which you really stop to look fear in the face. You are able to say to yourself, "I lived through this horror. I can take the next thing that comes along." . . . You must do the thing you think you cannot do."