2008 IPF Journey Begins

Pulmonary Fibrosis, Interstitial Lung Disease

ILD is a general term that includes many chronic lung disorders in which the lung is damaged, the walls of the air sacs become inflamed and then scarring (i.e., pulmonary fibrosis) begins in the tissue between the air sacs (interstitium). This causes the lungs to become stiff and smaller in volume. Idiopathic refers to “undetermined cause” for the fibrosis.

Pulmonary Hypertension

This rare disorder in which the pressure in the pulmonary circulation is above normal levels can cause permanent damage to the lungs and become life-threatening. When there is no known cause, it is called primary pulmonary hypertension. Pulmonary hypertension that occurs as a result of other disorders is called secondary pulmonary hypertension. 

Neither of these are terms my husband and I had ever expected to become familiar with and until January 2008 hadn't heard of. That's when he booked his annual physical exam and intended to have his doctor sign the application for his Assistant Dive Instructor certificate. Carman was already a Dive Master having completed the qualifications in 2007 and had been thrilled to dive the Barrier Reef in Australia (2005) several reefs in Roatan (2006), participate in a Roatan shark dive (2007) as well as exploring several dive locations in Ontario including Tobermory and Brockville. Scuba was going to be his retirement project - he and our friend John, a Dive Instructor, were planning to teach together.

Carman had just recovered from a cold but complained about some lingering tightness in his chest. This was the catalyst which started our journey with IPF...a series of x-rays and tests followed in the spring and in summer led to a referral to a wonderful respirologist, Dr. M. 


During a pulmonary function test in July his oxygen saturation levels went from 95 to 77% and then a lung biopsy in Sept. confirmed the initial diagnosis of idiopathic pulmonary fibrosis. His lung function was 70% in Nov08 and had dropped to 58% by Nov09, a year later. It seemed that each time he got a cold the disease progressed a little more (IPF patients cannot take cold medications). By July 2010, we had been referred to the transplant team at Toronto General for an initial teleconference consult. We weren't ready for transplant yet, but Dr. M thought it proactive to get another opinion and the get the team  familiar with Carman's case.

In October 2010 Carman had a Right Ventricular Catheterization to test pressures in the pulmonary artery. This determined that he had some pulmonary hypertension, but at the time levels were still within the "normal" range, just at the upper end of it. His fitness regimen became even more important so that the heart would be strong enough to help his lungs. 

We decided that a personal trainer would help, so in early 2011, Corey became his fitness guerrilla, champion, and friend all at the same time. Carman was dedicated to going to the gym and put every effort into his workouts. Corey did some research on IPF and adjusted his program and its execution to allow for longer recovery periods between sets, since Carman became winded more quickly and took longer to "catch his breath" than others. He was looking great, bench pressing 600 lbs, and feeling pretty good about the results of his regimen!

Then August rolled around...

Carman caught a "flu bug" of some sort and was knocked off his feet for 3 weeks. His weight started dropping and when he went to the gym, just going upstairs to the cardio equipment was more of a workout than he could handle. By mid-September he could no longer manage the workouts at all, so put his gym membership on hold and started oxygen therapy (2.5L/min during any physical activity). Our consultant, Joanne, was very helpful - setting us up with some tanks and a portable pulse concentrator - so Carman could not only have mobility while at home, but could fly to Florida to visit our good friend John. He found the warm weather in south Florida a great comfort and even managed some cart golf while there - although from time to time he did see some stars.

In Nov11, Dr. M corrected Carman on his interpretation of the O2 therapy - Carman was using it to recover from rather than during physical activity - thereby causing his saturation levels to plummet into the 70's. X-rays showed that the disease had progressed somewhat in his left lung as well. At our Dec11 teleconference with Dr. S from the transplant team, it was suggested we do another meeting in Jan12 and discuss arrangements to travel to TO for a pre-transplant assessment. By the middle of February we had a date for his assessment - the week of March 19th.

In spite of another cold in February, we managed to squeeze in a short trip to Florida - actually, my sister, Paula and I "pushed" Carman to come along (literally, as I had arranged for wheel chair transport for all flights to help him conserve his energy). We couldn't have managed without the help from Paula and the wonderful staff at airports in Portland and Atlanta along the way. We spent her March break having one of our own, Carman relaxing by the pool with both feet up (his gout flared up after he recovered from the cold) and enjoying the pampering that was lavished on him by all! John's hospitality was just what we all needed!

The travel really exhausted Carman, our first clue that the pulse concentrator just was no longer providing him with the oxygen levels he needed. This became even more apparent on our trip to Toronto...

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