Gall bladder leads doctors to PH diagnosis
By Edward Freundl
It sounds funny to say a gall bladder attack saved my life, but it’s true.
Had it not been for gall bladder trouble in late April last year I would not have found out that pulmonary hypertension — compounded by scleroderma in my lungs — was the condition that was making me short of breath and ultimately cutting short my life.
My doctors tell me this had probably been coming on for a number of years. It was so gradual that I really didn’t notice it; like many undiagnosed PH patients I had attributed my fatigue and difficulty breathing to getting older and being overweight.
In any case, it became clear during the winter and spring of 2010 that something very wrong was happening in my heart and lungs. I was unable to climb even a single flight of stairs at work without stopping to catch my breath, and the first time I tried to mow my lawn last spring I could only walk about 50 feet before my heart began pounding dangerously hard in my chest.
Because of this I had made an appointment with my regular doctor to find out what was going wrong, but fate interrupted what would have likely been months of medical tests and visits to various specialists before coming up with an answer.
Although I’d never had trouble with my gall bladder before, I suddenly began showing symptoms of a gall bladder attack in late April. This coincided with the doctor’s appointment I had already made, and he determined I needed surgery to remove it the next day at our local hospital.
Unfortunately, during the surgery the oxygen level in my blood and my blood pressure both suddenly dropped dangerously low. The surgery was stopped immediately and I was airlifted to St. Joseph Mercy Hospital in Ann Arbor, where I spent more than a week in the surgical intensive care unit.
That first night was the worst. I was in a coma, and doctors told my wife they weren’t sure I would make it until the morning. I made a remarkable turnaround in the middle of the night, however, and I fought hard to do what was needed to leave the hospital as soon as possible.
That turned out to be days ahead of when the doctors thought, and I was sent home after being set up with oxygen 24 hours a day and a fistful of appointments for pulmonary specialists and tests over the next two months to determine what was wrong with my heart and lungs.
Discovering that during the previous few years I had shown signs of some autoimmune deficiencies, Dr. Kristine Phillips, a rheumatologist at Ann Arbor’s University of Michigan Hospital, diagnosed the scleroderma in my lungs.
In addition, one of the pulmonary doctors finally suggested PH as a diagnosis and sent me to Dr. Vallerie McLaughlin, a world-renowned PH specialist and researcher at the U-M Hospital.
A right-heart catheterization and a six-minute walk test at the beginning of August confirmed the diagnosis, and Dr. McLaughlin started me on Flolan due to the severity and quick onset of my symptoms. When I showed progress after the first few months she added Revatio.
Dr. Phillips prescribed a high dose of Cellcept, which acts on my immune system to slow down the scleroderma. And just recently, I made the switch to Veletri, which is a new version of Flolan that is more stable at room temperature. The big advantages are being able to make up more than one dose at a time, and not having to keep the pump on ice packs constantly.
Make no mistake: it was difficult to make peace with being told that my condition was incurable, and that while the damage to my heart from PH was reversible, the damage to my lungs from the scleroderma was not. Over the past year I have had plenty of time to think about the phrase, “life-changing disease.”
By all accounts I have responded well to my treatments, but it certainly seems like it has been a long road to get to this point.
As a result of my doctors putting me on permanent disability, I’ve been away from my job as a newspaper editor for more than a year. I’ve had to watch out the window while my wife and elderly in-laws do all the work in the yard and garden that were once my domain. I’ve had to carefully choreograph simple things like a trip to the grocery store, and my wife and I have had to cut short visits to family and friends to make sure we get home in time to make up my next dose of Flolan/Veletri or to get another tank of oxygen.
On the other hand, I also know I have made great progress and have come to realize that the things I have lost certainly don’t compare to the many things I still have.
Like all chronic disease sufferers, I have good days and bad days, but I’m thrilled to say the bad days are now few and far between.
A year ago I was pretty much stuck in a chair, watching TV and waiting for my next doctor appointment or medical test — and worse, wondering why this happened to me.
Now I’m able to drive, run quick errands, make short shopping trips, do light housework, resume cooking and baking, and even get out in the garden for short periods. This may not sound like much, but it helps me feel more “normal” and less like a liability or burden to those around me. I simply have to be careful not to do too much in one day, and it’s been an adventure learning what my new limits are.
This has also reminded me to be more grateful for the new perspective on life that I’ve developed. I’m grateful to be treated by a world-class team of doctors who are only 40 miles from my home; I’m grateful that my only real “handicap” is an inability to breathe normally; and I’m forever grateful for the love and prayers of my friends and family. I’m certain that I couldn’t have made it this far without them.
Edward Freundl, age 50, is a journalist who was diagnosed with secondary pulmonary hypertension in August 2010. He lives near Jackson, Mich., with his wife Patty and their terrier/boxer mix, Daisy.