Pulmonary Fibrosis – Symptoms and Care

Shortness of Breath
Chest Tightness
Cough
Fatigue

If you have all these symptoms you may have Pulmonary Fibrosis, a disease that causes lungs to scar up so that eventually you cannot breathe (pulmonary means lung and fibrosis means scarring). Every breath is an effort. You may also notice clubbing of your fingers and loss of appetite.

If you do notice these symptoms you should go to your doctor as soon as possible and get a referral to a respirologist/pulmonologist. They will be able to organize all the tests to help make an accurate diagnosis of whatever ails you. It is a difficult disease to diagnose so a referral to an Interstitial Lung Disease (ILD) specialist is the best thing to have. There are over 200 ILDs. Your doctor should be able to organize this for you.

It is a very serious disease with no cure yet and the prognosis is death within 3 to 5 years. But there is some hope which will be discussed later in this article.

Some cases of pulmonary fibrosis (PF) have known causes, asbestos, bird and animal proteins, molds and fungus, coal dust (black lung disease), some prescription drugs, radiation and exposure to the environment and others. There is also some evidence that PF may disproportionately affect people with rheumatoid arthritis, scleroderma and other connective tissue diseases. There is also some risk of IPF if you suffer from Gastro Esophageal Reflux Disease (GERD).

Where there is no known cause then it is called Idiopathic Pulmonary Fibrosis (IPF). Idiopathic just means “no known cause”.

There does appear to be a genetic factor in play as some people exposed to these risk factors get the disease and some do not. Also, continuing with the genetic issue, some families have more than one member get the disease and some do not.

As previously stated, there is no cure yet but there is hope and some help with the symptoms.

At the moment the only intervention known to meaningfully extend life is a lung transplant and thanks, to the efforts of researchers around the world, including at Toronto General Hospital (TGH), these are becoming an improved option. They have even increased the available supply of lungs through ex vivo lung treatments developed at TGH.

Lung transplants are still difficult to get but there are treatments that slow down the disease in most patients. Right now in Canada these are only available through your respirologist and should be started as soon as possible. So, don’t delay that appointment. He or she may also prescribe medicines to alleviate the worst symptoms. If you have GERD they will probably prescribe medicine to control that. Don’t be alarmed if they mention palliative care. That doesn’t necessarily mean you are dying right away. Palliative care is also used for controlling symptoms.

There are thousands of brilliant minds around the world researching this disease and they are collaborating with each other. CPFF Founder Robert Davidson and others have met many of them and it is their belief that a cure will be found.

You may be tempted with people telling you about “miracle” cures. Before taking in any substances please check with your doctor. Some of these treatments can be really harmful.
There are things you can do to help yourself with this awful disease.

Exercise. It is difficult to exercise but it does help. In addition, if you do become eligible for a lung transplant they will look at your health to determine whether you can take a large operation like a transplant. A free exercise video is available at https://cpff.ca/living-with-pf/rehab-and-exercises/one-breath-at-a-time-exercise-video

Eat well. A good diet is good for you and, although you may have lost your appetite, you need to stay as healthy as possible by eating well.

Find support. Check the web and see if there is a Support group in your area. If you join one then make sure your Caregiver is involved as well. He or she will be so important in you dealing with all the issues that will arise.

Have a Caregiver. Your Caregiver, often a spouse, is the most important person to help you. She or he will be able to accompany you to doctor’s appointments and make notes so nothing is forgotten, look after things at home that you may no longer be able to do, encourage you and join you in exercising and a host of other little things that you may forget or just not feel like doing.
You must make sure your Caregiver stays well. Being a Caregiver is a very hard job. They know there is nothing they can do to make the disease go away but they want to. Imagine how hard that is for them. Don’t get mad with them. It’s the disease not them or you. Let them know how much you know they care.

Stay positive. CPFF Founder Robert Davidson and many others who did found a positive attitude helped.

Internet research. Patients should be cautious about what they read online (based on Dr. Jolene Fisher’s et al article which you can find at:
https://www.atsjournals.org/doi/full/10.1164/rccm.201512-2393OC). Patients should be skeptical of anything they see online that disagrees with what they hear from their doctor given that recent research has shown that much of the information that is available online is inaccurate and out of date.

For more information visit the Canadian Pulmonary Fibrosis Foundation at www.cpff.ca

This article is not intended as medical advice and should not be read as such. See your doctor for medical advice regarding his or any other health issue.