Saturday, December 29, 2012

Every Breath You Take

Every breath you take
 
Every move you make
 
Every bond you break
 
Every step you take
 
I'll be watching you.

 

Every single day
 
Every word you say
 
Every game you play
 
Every night you stay
 
I'll be watching you.

Before we had a chance to Google Pompe disease or enzyme replacement therapy, we were introduced to the word Pulmonologist. Now, I consider myself a well-educated man and I admit to a somewhat strange interest in the meaning of words so when these were tossed out I thought for sure I could use some grammar tricks and return with a definition in minutes. However, like many times to come I would discover that I had just entered a world which was not designed for quick answers and simple solutions. We had entered a new world we never knew existed nor expected to discover. While others my age would be focusing on their careers or favorite sports team, I would soon find myself very interested in something I thought was automatic to the rest of us…breathing. I would soon learn that I needed to add the words Pompe and Pulmonologist to my vocabulary because they would have a big impact on every breath Emma and Maddie would take.

I clearly recall that day at CHOP when we first learned that Pompe disease can impact the diaphragm, a muscle used for breathing. I came prepared, like all meetings, with pen and paper ready to write my notes and highlight my action items. But, as the doctors began to explain, I could not write it down. I was stunned. They told us that without proper treatment and diligent follow-ups, Pompe patients could experience a loss of breath after exercise, difficulty breathing when sleeping, and a tendency to develop more severe respiratory infections. In many cases, patients will require the use of a BiPAP breathing device at night. In the worst case, patients could experience respiratory failure, requiring a tracheotomy and the permanent use of a ventilator to help them breathe.

My first response was to sit in disbelief and challenge the doctor’s logic, but thankfully, before I made a fool of myself, my Dad's problem solving style quickly set in. "So, what do we need to do,” I asked. The doctors then explained the importance of staying aware of morning headaches and tiredness due to a lack of oxygen, stacking pillows to keep one’s head up at night to improve breathing, getting the flu vaccine early each year to reduce respiratory infections, about and most important, the necessity to have the girls see someone called a Pulmonologist.

Since I was a kid, I was never afraid to be the one to ask the "stupid question.” I am not sure if it was because I was more inquisitive than the next kid or just a bit slow on the uptake. Either way, I told myself I was asking the questions others were afraid to ask and never hesitated even if I knew my teacher would give me that "are you serious?" look. Over time I am a bit more careful here as I learned that in the working world despite your boss’s statement “there are no dumb questions”, there are. Regardless of my hesitation, I was faced with a term I did not know so threw it out. “What’s a Pulmonologist?” Fortunately, the CHOP team did not give me that "are you serious" look, but rather explained that these doctors specialize in all things respiratory and would be responsible for monitoring my daughter's breathing performance through annual testing.

Since that initial meeting we’ve met a few Pulmonologists across the U.S. and learned more about breathing than I cared to learn. We've also watched our girls undergo breathing test after breathing test while sitting and lying down. They’ve breathed into funny looking machines, sat in science fiction looking air-tight chambers, and competed with each other to see who could have the best results. We’ve also sat quietly by their beds at night listening to them sleep and prayed that they would never have to worry about something that is automatic to the rest of us…breathing.

A couple weeks ago, we had our 2012 visit to the Pulmonology Lab. This appointment was clearly much simpler than our first, even routine. As the girls and I walked down the familiar white hallway at CHOP, passed the posters of medical inhalers, and found our seats, it was all good. There was no nervousness, no anxiety, and no need for my stupid questions. We were comfortable in this world, knew what to expect, and were confident in another great result.

As there were two doctors on staff that morning, the girls were split up for speed. Each would undergo testing while sitting up and while supine (lying down). Each would run through the same schedule they had done many times before and each would do their best because we all understood the importance of the results. One of CHOP’s staff called this week to tell us that they reviewed the results and they were “great”! The results show they are either stable or improving a bit since last time. The combination of enzyme replacement therapy, diet, exercise, and a little thing called luck seem to be keeping things in check.

Looking back it is funny to think of where we started from and where we are today. With attentiveness, education, and hard work, the girls are doing well. However, we know that Pompe is a progressive muscle disease which means that past success does not mean we can ignore the issue. We all need to remain vigilant, educated on new advancements, and on top of our regular visits to the doctors. Nothing in life is a sure thing, but with the right planning and “can do” attitude, our girls will be just fine.

Before I know it we'll be back at the Pulmonology Lab and I'll be peeping over the doctor's shoulder as he completes his tests, hoping to see positive results. Whether the results are perfect or just a bit less than we expected, I'll be there by their side because...

Every breath you take
Every move you make

I'll be watching you.

Breathe on!
Matt

Credit to: The Police, "Every Breath You Take"