What a year.
For the last few months of 2017 and the first five months of 2018 I was annoyed by a chronic dry cough and shortness of breath. The cough was not constant. A flight of stairs would result in one or two hard, dry, coughs. Talking for more than a minute or two would as well. But, I’m a bit of a talker. I just figured I wasn’t pausing to breathe enough. Which made sense because I was also getting out of breath more often than usual.
I think that may be what confused me the most about my symptoms. I’ve suffered from mild asthma symptoms for most of my life. What I was experiencing was similar. Sort of. There was a difference and the fact that there was a difference was bothering me. As the winter drew to a close and warmer weather returned I was a bit concerned that my breathlessness was not improving. Cold weather was always a catalyst in the past and I’d assumed it was this time as well. I realized I was wrong and made an appointment with my primary care doctor.
I visited my doctor in early May of 2018. She listened as I explained my symptoms and felt that it was possible that I needed to take a more aggressive approach to controlling my asthma with medication. She prescribed a couple of things which should have worked.
I returned to my doctor in late May to follow up and explain that my symptoms were unchanged. I also emphasised that I knew what asthma felt like and that what I was experiencing was different. I just wasn’t the same. She listened closely to my breathing and suggested a chest x-ray and pulmonary function test to establish a baseline and see if there was anything else to be concerned with.
In mid-June I had results back that indicated that I was presenting symptoms of a restrictive lung disease. I was advised to see a Pulmonologist for further refinement of the diagnosis. My doctor went ahead and ordered a high resolution CT scan of my chest since she felt that it would be the first step that any Pulmonologist would want to take in refining the diagnosis. While she was careful to remind me that the diagnosis would need to be confirmed by a specialist, she felt that there was a strong possibility that I had Pulmonary Fibrosis.
My family and I began searching the Internet for an understanding of what was happening to me and quickly realized that it would be wise to seek out the best medical care possible. We found that there was only one nationally ranked facility in Illinois that had a practice specializing in Interstitial Lung Disease (ILD), which includes Pulmonary Fibrosis (PF) and that it was ranked well down the national list. The Mayo Clinic in Rochester MN was ranked 2nd in the nation and about a 5 hour drive so we figured it would be a good place to start. Surprisingly, I was able to get accepted as a patient fairly quickly and had my first visit in early August of 2018.
This is where my journey gets increasingly confusing. I had no idea it would be so difficult to nail down a specific diagnosis. I was learning quickly though.
Saying that I have Pulmonary Fibrosis meant that I had scarring in my lungs. At first that was all we knew. I was told that 50% of the time it’s possible to determine what is causing the scarring. If it’s not possible to identify the cause it would be classified as Idiopathic Pulmonary Fibrosis (IPF). IPF is the condition we kept running into in our Internet searches that caused us such concern. Everywhere we turned we were confronted with a prognosis that indicated a 3-5 year life expectancy once diagnosed. What we began to learn was that this prognosis was for patients with unexplained (Idiopathic) scarring of the lungs (Pulmonary Fibrosis).
My first visit to Mayo clinic resulted in a number of tests being performed that identified a source of my PF. That was good news! It meant that I did not have IPF and was therefore not on that rather horrible 3-5 year life expectancy prognosis track. Unfortunately, test results were pointing toward a reason for my PF, the reason was still not specific and would require more testing. My diagnosis was refined and now being called Hypersensitivity Pneumonitis (HP or HSP). HP is similar to an allergy in which the body is reacting to something in my environment. However, it’s different in that the reaction is inflammatory. In short, the opinion was that I was unusually sensitive to something in my environment which was causing inflammation in my lungs which was resulting in scarring. Ultimately it was determined that a lung biopsy would be the most definitive test possible so I agreed to undergo the procedure.
In mid-August of 2018 I returned to Mayo for the biopsy. Several wedge shaped sections of lung tissue were surgically removed and processed for a variety of tests. The specimens were tested for various molds and other airborne toxins that are common in the environments in which I’ve lived and worked. All the tests were negative.
The best treatment for HP is to avoid the antigen – whatever it is that is causing the inflammatory response in my lungs needs to be avoided. Unfortunately, we were unable to determine what it is in my environment that is responsible. Again, about 50% of cases are never identified. In all likelihood my body is simply over reacting to something in my environment that doesn’t bother most people, AND I’ve probably been exposed to this most of my life. The level of inflammation has simply gotten gradually worse over time and the constant inflammation has caused permanent damage (scarring) in my lungs. Since I don’t know what environmental toxin to avoid, the only treatment option is to try and manage the inflamation.
In September of 2018 I was put on oral steroids (Prednisone) intended to reduce the inflammation in my lungs. It worked! My symptoms were reduced significantly. Unfortunately, there are significant side effects which I was not tolerating well. As it turns out, there is only one other medication option available and it’s not on my insurance companies formulary (list of approved medications). My Pulmonologist prescribed the medication by it was rejected by insurance. We’ve appealed the rejection based on medical necessity and as the year turns to 2019 are still waiting for a decision from the insurance company.
My biggest surprise throughout this entire experience has been that there wasn’t a definitive diagnosis. No one has been able to say exactly what I’ve got and what can or cannot be done to deal with it. So… I’ve been reading as much as I can and educating myself to find a way to understand where I’m at.
What I know is that I have Chronic (it’s been with me a long time) Fibrosing (it’s causing scarring) Hypersensitivity (I’m unusually sensitive) Pneumonitis (inflammation involving my lungs). The scarring will never improve. If I can manage the inflammation I can prevent additional damage. I now understand that my focus needs to be on inflammation management.
2019 will be the year I learn about chronic inflammation. What I’ve learned thus far is that chronic inflammation may be the root of may diseases so I’m certainly not alone.
I’ll post more updates as things progress. I intend to post more regularly so I don’t have as much to catch up on. We’ll see how that goes!