“Gee, doc, ya think?” – Barbara speaks about her diagnosis and life with IPF

Posted May 19th, 2014 by

PatientsLikeMe member Barbara (CatLady51) recently shared about her journey with idiopathic pulmonary fibrosis (IPF) in an interview with us, and she spoke about everything from the importance of taking ownership of managing her condition to how she hopes to “turn on the light bulb” by donating her personal health data. Read her full interview about living with IPF below.

Some PF members report having difficulty finding a diagnosis – was this the case with you? What was your experience like? 

My journey started back in 2005, when after my first chest cold that winter, I was left with severe coughing spells and shortness of breath. An earlier chest x-ray didn’t indicate any issues, so I was referred to a local community-based respirologist (what we call a pulmonologist here in Canada) who wasn’t concerned with my PFT results. I also had a complete cardiovascular workup, again with no alarming results.

Then, in 2008, I had another chest cold. Growing up in a family of smokers and being the only non-smoker, I seemed to have managed to miss having chest colds, but 2005 and 2008 were definite exceptions. Again, a normal x-ray, another visit to the respirologist and another PFT that didn’t send up any alarms [although looking back at both 2005 and 2008, I can see where there was a definite indication that I was heading towards restrictive breathing problems]. Inhalers only made the coughing worse. The respirologist said I had “sensitive lungs” – gee, doc you think?

Then, in November 2010, I was laid out with another chest cold, coughing my lungs inside out, barely able to walk 10 feet. So the new family doctor calls me. This time, the x-ray report came back that I was showing signs of interstitial lung disease (ILD). What? So onto the computer and in to see the family doctor. When the doctor suggested sending me back to the local community-based respirologist I had previously seen, I said NO BLOODY WAY!

Instead, through a friend who is a thoracic surgeon at the University Health Network (UHN) in Toronto, I got a quick appointment at the ILD Clinic at Toronto General Hospital (TGH) in January 2011. Since I hadn’t yet had a HRCT, I was sent for one and returned to the clinic in June. The initial diagnosis was probably IPF, but maybe NSIP since my HRCT didn’t show the UIP-pattern. The decision was made to treat as IPF so no harmful treatment was undertaken. A biopsy was discussed, but was considered too early for that invasive test and that instead my disease would be monitored via non-invasive tests.

My ILD/PF specialist continued to monitor me and after another exacerbation early in 2012 and the PFT showing a progression of my lung disease, we decided to send me for a VATS biopsy. The September 2012 biopsy clearly indicated the UIP-pattern of lung damage and the IPF diagnosis was confirmed.

Over the last few years, I’ve learned a great deal. I know that the road to diagnosis is often long and complex with not all the pieces of information presenting at the same time — seldom with one test or series of tests taken at one point in time. I feel I’m fortunate that first I had that very unsatisfactory experience with the local community-based respirologist and that through my husband’s work we had met and become friends with a thoracic surgeon who is on the lung transplant team at TGH.

So even though I “naturally” followed the recommended course of action to get myself to an ILD/PF expert, my path to diagnosis wasn’t instantaneous. My biopsy could have just as easily shown that I had a treatable form of PF — still not good news but a different path.

Now with a confirmed IPF diagnosis, I’ve been assessed for transplant (June 2013) and found suitable but too early. But another winter of exacerbations and my ILD/PF specialist is now talking about going on the waiting list.

Another PF member, Lori, spoke about her “new normal” – how did your diagnosis change daily life?

Yes, life with PF has certainly been a series of adjusting to the “new normal” but up until February 2013 when I started oxygen therapy, the changes were small. I had to explain to people why I broke into coughing fits while talking on the phone or in person. I had to explain to people who offered water that thank you but it didn’t help since it was just my lungs telling me to talk slower or shut up. I had to explain to people that I wasn’t contagious when coughing. I had to explain to people that the huffing and puffing were just the “new me” and that they didn’t need to feel they had to jump in — that I would ask when I needed help.

But since going on oxygen therapy with my new facial jewelry and my constant buddy, I don’t have to explain that I have a disease but some people still like to ask questions and I enjoy answering them.

Life with PF and supplemental oxygen is definitely more complicated. I started with high-flow for exertion (6 lpm) and liquid oxygen (LOX). So I can’t spontaneously take off overnight (I would have to make arrangements about a week ahead to have  equipment and supplies delivered at my destination) and I probably can’t fly. But I’m a homebody so that has affected me very little. But I can’t leave the house without considering how long I will be and how many of my LOX portables to take with me.

I still do my own driving, shopping, cooking, housework, and one or two 2-mile walks per day on the farm property — over hilly landscape — because I’m de-conditioned after this past winter. I’m currently having to use 8-10 lpm for those walks but I’m doing them. Use it or lose it!

What made you decide to get so involved in the PatientsLikeMe community and how has it helped you better understand your own PF?

Involvement with PatientsLikeMe was more of a knowledge-based decision. I believe that knowledge is power and knowing as much as I can about my disease helps me to manage the disease. For me, support is sharing what I have found and providing directions to that information for others. Then it is up to them to read the information and decide how, or if, it applies to them.

I believe in being my own medical advocate in charge of my medical team. I’ve probably had a natural propensity for that but my way of thinking in not being a traditional patient was affirmed by Dr. Devin Starlanyl, a doctor with fibromyalgia who wrote The Fibromyalgia Advocate. Fibromyalgia is a matter of living with and managing the symptoms and dealing with different medical specialties to achieve that BUT also accepting that you as the patient are central to treatment and management.

I believe that living with PF is that way as well. The doctors can only do so much. There is no single silver bullet that they can give us, no matter what type of PF, to make it all go away. We have a core set of symptoms BUT we don’t all have all the same symptoms. We have to take ownership for our disease management.

So at PatientsLikeMe, I seek to not only learn but to share what I’ve learned. If I can help one other person shorten their learning curve then perhaps I’ve helped.

On your PatientsLikeMe profile, you reported using a pulse oximeter in 2013 – how did you like it? What did it help you learn?

I found that I was having to slow down too much or struggle too much to breathe. My walking test was not yet indicating that I qualified for oxygen therapy but rather was on the cusp of requiring supplemental oxygen. I was concerned about the damage to my body.

I purchased an inexpensive pulse oximeter to check my saturation. I soon realized that being short of breath was not a reliable indicator that my oxygen saturation had dropped below 90%. Having the oximeter to give me a measure of my saturation helped me to better interpret and listen to the other biofeedback that my body was giving me.

The oximeter helped me to manage my activity so that fear didn’t turn me into a tortoise that either slowed way down or seldom moved. I got a better handle on just how much and how fast I could do things to keep active, to keep my body healthy, to exercise all the parts of my respiratory system, and yet to do it SAFELY!

Looks like you use your profile tracking charts and reports a lot on PatientsLikeMe- why do you donate so much health data, and how do you think that will change healthcare for people living with PF?

Again, my propensity. I love learning! I love sharing what I learn! I keep my own spreadsheets with my medical data but that only benefits me. I know that one of the problems for researchers is accessing a sample population large enough to make meaningful inferences from their findings. And finding a large population in a given geographical area for a rare disease is difficult. Going outside the geographical area is expensive. So hopefully the remote sharing of information will be the answer.

We are all so very different and so many of us also have other health issues on top of the PF. So who knows what comparing us will show? But throughout life I’ve been amazed at how seemingly inconsequential, seemingly totally unconnected pieces of information can come together at a later point and TURN ON the light bulb!

So why not share my health data? It really is anonymous. Unless I provide more identifying information, I’m just a name and a face but maybe with enough names and faces we can get some answers that will benefit us all.

 Share this post on twitter and help spread the word for pulmonary fibrosis awareness.


11 Comments

  1. Nice job Barbara.

    I especially like: “I believe that living with PF is that way as well. The doctors can only do so much. There is no single silver bullet that they can give us, no matter what type of PF, to make it all go away. We have a core set of symptoms BUT we don’t all have all the same symptoms. We have to take ownership for our disease management.”

    Sums you up to a T

  2. Just one correction. Not a Montreal Canadians fan. Not a big hockey fan and my heart belongs to the Leafs but as the remaining Canadian Team in the hunt for the trophy for a Canadian game, I’m rooting for the home guys…even if it’s Toronto’s mortal enemy 😉

  3. Barbara
    Great interview. You have been such a plus in my life helping with my knowledge of our disease.
    Thanks
    Laura

  4. My husband has been diagnosed with this dreadful disease getting information and treatment has been very difficult this has been going on for four years and has had four chest tubes because of his right lung collapse.

  5. I believe I have PF but I got it from taking microbid for 9 years. I have a chronic UTI that was totally controlled by 50 mg microbid daily which I never questioned because I had no side effects like yeast infections or intestinal upset. Do you know if this kind of PF is different from IPF? I was able to suppress my cough last Sunday but today I cannot. I am coughing a lot and losing my voice when I talk on the phone. I am 72 years old and have no other health issues. My blood test are perfect and my blood pressure is excellent and I have never been overweight. Any information you have for me will be appreciated.

  6. Hello Barbara: I would like to talk about my husband John whom had IPF. He had quit smoking some 25 years before he got sick. We took him to the hospital in Aug 2007 with shortness of breath. They were going to send him home but a nurse had the insite to take his blood gases which were 48. He had worked that day. They never gave him any treatment til Dec when he was admitted for further shortness of breath. They gave him a heart pill as the lungs were affecting his heart. This disease was horrendous for him from the start to finish. The Dr. in Halifax even said he could have a transplant if he lost weight, he did this but there never was a plan to have a transplant as we later learned. Our family Dr wanted to send him to Toronto or the mayo clinic for further tests but he said he was slapped on the hands (figuratively) by the specialists in the Halifax hospital. Everytime my husband would get worse with his breathing, we would have to fight to get to see the Dr. Finally in Dec 2009, he went in hospital to have a new medication but he died 4 days later and had every side affect from the drug but yet they would not stop it. I am glad that you are living with this disease and pray you will continue to live a happy life. I am not certain many manage as well as yourself. Besides the disease, the one Dr. was so horrid and I will never forgive him for the treatment of my husband whom was not a complainer and was always healthy all his life and played sports thru out his life. Another thing is my husband never had a cough. His only problem was he could not get his breath. Take care. Maryann

  7. I was diagnosed with ipf and diabetes a few months ago am am so afraid. I so this and am reading all I can about it.What is the normal lifespan after diagnosis? Just need some answers from people like me.

  8. I was diagnosed with IPF in December 2011 which was accidentally discovered on a CT scan because of a different problem, I had had a cough for about a year and really thought nothing of it. I started reading everything I could find via computer as I know of no one with this disease to talk with. I have found that PF symptoms and life span varies tremendously with people, the cause is unknown therefore a cure is not known. I did go through pulmonary rehab in early 2014 but the large majority of the patients had COPD. My days vary, some days I barely have the energy to care for my necessities and on rare occasions I actually feel good and oh how I treasure them. I had my first exacerbation on May 11th which lasted for near six weeks, I feared it was the beginning of the end as I got very ill but I did improve. Thus far my oxygen level is still good, still do my errands except for assistance in house cleaning, I get short of breath very easily even talking, tire easily, do not have much of an appetite, am sensitive to fragrances and cleaning chemicals, also have Type 2 diabetes, a GI problem and will see a rheumatologist next week to rule out any immune disease.
    I most long to continue living independently. I only discovered this web site a few weeks ago and sincerely appreciate the efforts of those who oversee it. Thank you.

  9. To Barbara: After my diagnosis in the fall of 2010 – I sort of fluffed it off since I did not really feel ill and always counted on my friendly family heart attack to end my life. I then became occupied with death and fear and felt urgency to get my will, living will, power of attorney etc in order. Under a cloud for 4 years getting more depressed and less able. My doctor said that I should not push myself and that there was no benefit in exercise. This year I have asked for a referral for pulmonary rehab (after 4 years!) and found that seeing that I could walk a treadmill and do the other exercises made me stronger and more confident, fought depression and after 4 months I am able to do things I was not capable of 6 months ago. I park far from the big box stores and walk their length, no longer need the electric cart and am being aggressive in seeking out meditation and services from places like cancer services so I can borrow on the bravery of others. Can’t describe the improvements!

  10. Bless you for your concerned contributions. I’m newly diagnosed with IPF (6 months mid 2015) and am trying to get up to speed on information …, and to find how I might contribute to others as you have with your posts. Questions please.., do you take Esbriette and what,s the cost in Canada? Please feel invited to Florida for a respite if you could use the break (I love Canada in both of the months in the summer when it,s bearable

  11. diagnosed 4-16 doing well no oxygen needed yet but cant do lot of physical activity had to give up my bike but still walk. my biggest issue is lack of energy I sleep 10 hours at night and usually nap in the afternoon is this normal?

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