We had to learn the ABCs of IPF after my late husband’s diagnosis
Understanding the terminology allowed us to become better advocates
I remember how exciting it was as kids when Mom served us alphabet soup, seeing all those little letters we were trying to learn. My late husband, Steve, and I felt like we were looking at a bowl of that soup when we started researching idiopathic pulmonary fibrosis (IPF) after his diagnosis.
Pulmonary fibrosis (PF), interstitial lung disease (ILD), and pulmonary hypertension (PH) all had acronyms that were new to both of us. There were other confusing terms and abbreviations, too, such as oxygen (O2), forced vital capacity (FVC), oxygen saturation of peripheral blood (SpO2), and diffusing capacity of the lungs for carbon monoxide (DLCO).
The only one I was familiar with was O2, but I only knew what it was, and not how Steve would eventually use it. There was a huge learning curve for both of us. A glossary and abbreviations list on the Pulmonary Fibrosis Foundation’s website became a starting point for our research.
Testing and diagnostics
Testing for PF also involves many acronyms. Generally, the diagnostic process starts with a chest X-ray (CXR) and a high-resolution CT scan (HRCT), both of which can be performed in the imaging department at most medical centers. The CXR may show signs of PF, but the HRCT is the gold standard of imaging, as it allows us to view thin slices of the lungs (like slices of a loaf of bread). Most people have heard of these two tests.
Pulmonary function tests (PFTs) are conducted in the pulmonary function lab within the respiratory department. These tests measure lung function, including FVC, but they can seem a bit odd — requiring patients to sit in a small, airtight chamber with a clip on their nose as they breathe through a mouthpiece. As they inhale and then exhale (until they can’t exhale anymore), a PFT also measures the transfer of gases from the lungs to the bloodstream.
A six-minute walk test, or 6MWT, may be performed in a pulmonologist’s office or in a hospital’s respiratory department. This test can help determine if a person needs supplemental oxygen. Although there is a general protocol, it may vary slightly from operator to operator.
Right heart catheterization (RHC), left heart catheterization (LHC), ventriculogram, and coronary angiogram procedures typically take place in a cardiac catheterization laboratory. A physician can measure the pressure, blood flow, and function of the heart and coronary arteries, as well as look for evidence of blockages.
Learn as much as you can
I urge people to learn as much as they can about PF so that they can be their own advocate. Steve and I met many medical professionals who knew little, if anything, about this interstitial lung disease back in 2009 — especially in emergency rooms. “Your oxygen can’t be that high,” they would say when we told them to set it to 6 liters/minute (L/m). The best offense in this “game” is to gain as much knowledge as you can.
Steve was first studied in the catheterization lab because the symptoms he experienced while hiking pointed to congestive heart failure (CHF) or coronary artery disease (CAD). Thankfully, when those tests came back normal, an astute cardiologist immediately ordered a chest X-ray.
Due to my career in healthcare, specifically as a radiographer and cardiology services manager, I was familiar with many of these acronyms; however, I was not aware of all of them. This gave us a slight advantage, but we still had to conduct extensive research and seek out relevant resources. I wish there had been somewhere to turn for help in determining the next steps and understanding what the journey would be like.
After retiring early, Steve learned a great deal from browsing the internet and joining Facebook groups. Today, I offer assistance to others through the virtual support group that I lead. I don’t provide medical advice because I’m not a physician, but I offer advice based on my own experience and always recommend that patients consult with their healthcare providers before making any changes or trying new treatments.
After completing all the medical tests, it’s time to go home and learn as much as possible about the journey ahead. Perhaps you may even find some comfort in a bowl of alphabet soup.
Note: Pulmonary Fibrosis News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Pulmonary Fibrosis News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to pulmonary fibrosis.
About the Author
John
Hi Debbie
Thank you for sharing your and your husband's IPF journey, your husband was wise to have you commit to sharing. I also have IPF, I am a Vet, and was diagnosed 2 years ago, now I am on the lung transplant waitlist for a double lung transplant. I turned 70 years old this month. I am on level 10 O2 at rest to keep my blood O2 over 90 (resting watching TV I can get it up to 95-97. I expect to get my transplant in the next 18 months.
I whole heartedly agree with you - do your own research online, when you see a word you don't understand Google it and explore the connections until you feel you understand it. I've read everything about everything having to do with IPF, PF, drugs to slow scarring progression, worldwide research for a cure for PF including drug trials and results, lung transplants, etc. So, most times I know more, and know more current information, then the Drs. After not understanding this, I realized each Dr is an expert in helping their patients with PF or COPD or CF but not on current drug trials or transplants - which is appropriate as until these drugs get to market they are not helpful to their patients, and they don't want to get their hopes up with things that may or may not be available in the future.
Right now there are drug trials in the USA that are testing cure drugs for PF scarring, but remember it takes an average of 14 years to bring a drug to market.
After the medical tests you described being the initial diagnostic and ongoing tests for people with PF, if you get evaluated for a transplant, you will be tested from the top of your head to the bottom of your toes inside and outside to see if there are any medical conditions that need treated before you qualify to be on the transplant waitlist. This takes over 4 months.
I was given the standard 3-5 years go live (I discovered that time frame came from a Mayo Clinic study). the Mayo Clinic is the gold standard as far as IPF and PF is concerned. But, after 1 year I was told I had only 2 years to live due to my scarring progression.
When you are down to 2 years to live you are eligible to start the transplant process. Most transplant programs cut off at age 65_ but there are many that go up to 70 and some have no age limit. The insurance options are: if you are a Veteran, and medicare if you are 65 or older, and your private insurance. Google transplant programs then look at their websites and look in Q's and see what is said about if they take medicare or your insurance plan. For Vets there is only 3 transplant centers in the USA who perform lung transplants. You may need to email or call.the transplant coordinator to find out at the transplant programs you are interested in. Under SRTR (Google it) you can find all kinds of good Info about every transplant program, all transplant programs are required to report their stats once a year to SRTR. UNOS also has some very good info about the transplant waitlist and transplants.
Before I knew I eligible to start the transplant process, all I knew was I was going go die in a couple of years so I did a lot to get my affairs in order, even to donating the majority of my clothes away to the extent my wife asked me - do you have anything left to wear? I was thinking of all the things she'd be left to do after I was gone to Heaven and wanted to ease her burden as long as I was able to do so. I planned my memorial service and planned to video a message to be played at if. As an Eagle Scout I lived by the Scout Motto - be prepared.
So do your own research - adopt "be prepared" as one of your mottos too.
Thanks
John
Carol Berry
I am a PF caregiver and would like to know if Debbie Klein has written anything to help.
Julia Hager
I was diagnosed with PF last year cause they found that one of my lungs has developed honeycombing on the bottom
Well now they have found a 1mm lung nodule on my right lung
So I go for a pet scan on Tuesday
I’m not going to make a mound out a ant hill
Just worried because I have had thoracic lung collapse 3 times but still have my lungs