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"

Saturday, November 3, 2012

Let it Be

When I find myself in times of trouble, Mother Mary comes to me
Speaking words of wisdom, let it be
And in my hour of darkness she is standing right in front of me
Speaking words of wisdom, let it be
Let it be, let it be, let it be, let it be
Whisper words of wisdom, let it be

To some it was just a spreadsheet, but to me it was a thing of beauty. Spreadsheets only have numbers and calculations. This had names, dates, times, events, travel details, all in color. I’m not talking just any color. There was a rainbow of colors to separate one item from the next. After all, for a trip like this, the standard white background would not do. This was the Olympics and if I was going to be the planner, I was going to do it in style.

Over the years I’ve met a few people who claimed to be experts in organization. They’ve held seminars, written books, and sold recordings. They’ve inspired millions of the organizationally challenged, but none could hold a candle to my mentor. You see, my father was the master. When the others were promoting their skills for money, my dad was quietly passing his secrets to his kids. When grown men wondered how to organize file cabinets, I did it with ease. When others froze in the calendar aisle of office supply stores, I created my own. And, when I needed to plan a vacation the pre-Excel color coded organizer was there as a reference. You see when my sister graduated from medical school back in the day my dad had our trip detailed to the minute. Each family was color coordinated and each event carefully defined. Scoff as they might, this man got us there and back on time, on budget, and as planned. It was a thing of beauty.

As we began to plan for our summer trip to London, I jumped at the chance to organize the travel details and my friend took on the challenge of the “one thing”. The breakdown was simple. I was to record all the details around travel, events, reservations, and prices while he developed something special. The “one thing” was the opportunity for each of us to squeeze in something we wanted to see, do, or experience while we were in London. There were no limits and only one ground rule. No matter how wacky it sounded, we would all make time to participate . There would be no schedule or clock, just the opportunity for magic and memories. What I did not realize until later was that while the planning was necessary, sometimes magic can only happen when you Let it Be.

Over time, each of us chose something different. The list included the London Eye, Buckingham Palace, Shakespeare’s Globe Theatre, and even swimming at this crazy pond at Hampstead Heath. For me the choice was simple. If you’ve read this blog for any period of time you know I like music and love the Beatles. While my musical taste is quite eclectic, they just seem to spark my creativity. As such, my “one” choice was simple…Abbey Road. For those who may not know, Abbey Road is the location of Apple Studios (not the Steve Jobs version, but the Beatles version) where the Beatles recorded and the name of my favorite Beatles album. The cover of the album is a simple photo of the group crossing a street. While I don’t believe they understood the importance at the time, the photo has become iconic. It is so much so that people from around the world travel to the site just to walk the same steps and recreate a moment.

I don’t recall which day it was that we decided to fit it in, but it came together perfectly. We had a free afternoon (thanks to my excellent scheduling – Ha!) on the way to a soccer match and took a subway detour. When we came up from the tube station I began to feel the magic. There were people of all shapes and sizes sharing what appeared to be a strange pilgrimage, just to cross a street. As we walked around the corner on that blustery day, the famous crosswalk came into view with the studio just behind. It may not have been special to everyone in our group, but to me it was cool. It is a strange feeling when after looking at a photo for many years, you find yourself next to it.

Within minutes cameras were out and my family was ready to cross. As our friend waited for traffic to clear Emma removed her shoes, to recreate the Paul McCartney pose, and we readied ourselves. Amongst the honking horns of delivery vehicles and taxis, we crossed, stopped halfway, took a few photos, and ran to safety on the other side. We then stopped by the studio to take a photo, write on the wall, meet a few people, and share a few stories. Then, just like that, it was time to go but before we got too far, our friend pulled out a sign and handed it to Donna and I to hold up in the street. The written message was in bold letters and clear as could be…Let it Be.

As we again began to leave, a gentleman asked Emma to take a photo of him and his son on the crosswalk. Once that was done Donna started up a conversation and found out that this man was from the same area in California as her. After minutes of small talk about towns and restaurants he asked if we needed access to more Olympic tickets. Sure we said, as long as it was legal and not crazy expensive. It went something like, at 8:30 AM, go to Westminster tube stop, enter x hotel, go to x floor, find x room, and line up. Each day x offered a series of tickets (at face value!) which its government was not using for the day.

It sounded a bit crazy to me, but then again it wasn’t on my spreadsheet. The great part was it immediately added adventure and actually worked! For a few days our group acquired tickets to events we had given up on months before. Each day a visit to x hotel on x floor took place and we were able to pass on a bit more of Olympic magic to the kids. I can’t calculate the odds of us being on the right corner and at the right time, to get that tip? I can’t tell you because it can’t be calculated on a spreadsheet – with or without color. All I know is it brought smiles to the faces of our kids and left us with a story that they will tell long after we’re gone.

Later that evening as we finally took our seats at Wembley Stadium, I looked over to my kids, and smiled. I was glad that all our planning had allowed us to be there on time, on budget, and as planned. However, I was happier that my friend took charge, created the “one thing”, and allowed each of us to experience a bit of magic throughout our vacation. While some things can be scheduled, organized, and planned, sometimes you just need to set aside your calendars, set aside your stress, open your mind, and…

Let it be

All the best,
Matt

Credit to: The Beatles, “Let it Be”

Here are some photos from our day at Abbey Road which you might enjoy.


The kids in front of Abbey Road Studios


The famous crosswalk



A simple message 


 to be shared...



 and shared once again...



A memory left behind

Sunday, October 21, 2012

Homecoming of The Boys of Fall

When I feel that chill, smell that fresh cut grass
I'm back in my helmet, cleats, and shoulder pads
Standin' in the huddle listenin' to the call
Fans goin' crazy for the boys of fall

They didn't let just anybody in that club
Took every ounce of heart and sweat and blood
To get to wear those game day jerseys down the hall
The kings of the school, man, we're the boys of fall

Well it's, turn and face the stars and stripes
It's fightin' back them butterflies
It's call it in the air alright yes sir we want the ball
And it's knockin' heads and talkin' trash
It's slingin' mud and dirt and grass
It's I got your number, I got your back
When your back's against the wall
You mess with one man, you got us all
The boys of fall

A few years ago Carter came to us and said he wanted to play football. I quickly recalled my middle school days at St. Angela's playing flag and pictured a field covered with nice catholic boys wearing matching shorts and t-shirts. "Not that kind of football,” he said. "I want to play real football, the kind where you wear helmets and actually tackle kids." I wasn't so sure Carter was ready for the roughness of football, but since competitive chess was not an option I agreed.

A few enjoyable years of Pop Warner at Marsh Creek were followed by a year of middle school football and a year of Freshman football. Freshman football at Carter's high school means only freshman play (no bigger kids sneaking in) and all of them stay safely clear of the man-child players who could be seen frequenting the varsity field. Freshman football was controlled, designed for fun, organized for learning, and a testing ground for the big time. Lots of boys play Freshman ball for those very reasons. However, when the idea of graduating to JV to play scout team vs. boys that weigh 250 pounds and squat 3-4 times your weight enters their minds, they decide to pursue their passion in one of the many other sports the school has to offer. Not Carter. He loved it too much.

As a sophomore at D East, unless you are a stellar player, you play JV.  You practice with the varsity, act as scout team, and play games on Monday afternoons. For those that don’t know, scout team means you run the opponent's offense and defense against a fired up varsity team who goes full force. This is not flag football or even rough touch; it is tackle football with a vengeance. As a tight end or linebacker, Carter frequently found himself eye to eye with his varsity counterparts who only saw the opponent’s jersey and held a desire to prove to his coaches that he was ready for Friday night lights. Scout team brings no glory, but is a rite of passage for each player. There is no sympathy from the older kids, but for those who play their part there is one thing more important...respect.

Most evenings I pick up Carter from school about eleven hours after he's left in the morning. The somewhat quiet, non-morning boy who left the house with combed hair, pressed shirts, and lunch in hand is not the same one I pick up in the evening. The one standing outside the locker room is donned in filthy practice pants, a soaked shirt, tussled hair, and a giant smile. After he drops his bags in the car, flops into seat, and asks what's for dinner, I ask about practice. He always starts with the story of that day's scout team. He would talk of how he caught a pass and one kid "destroyed him" and how he tried to tackle another and "took him down".  The details go on and on.  While we live five minutes from school, on those evenings I wish we could drive for hours. There are few things better than listening to your son as he is maturing right in front of you.

During the high school football season, few things are more important or more exciting than Homecoming week. This is the time when alumni return to the stands and former players walk the sidelines with their teammates. It is a time when the school and its town come together around a football field to celebrate the fall and reinforce tradition.  During this week, the seniors nominate girls to the Homecoming Court and then choose a Queen.  Rather unexpectedly Emma said, “I’d like to be on the Homecoming Court”.  She ran, was nominated and we were excited for her. 

Homecoming week started with the Homecoming game itself. This year our high school team is doing quite well, so by half time the game was under control, but there was still electricity in the air. It was time for the Homecoming Court to be escorted across the field.  When the girls and their escorts lined up I could faintly see Emma until her name was announced.  There she was, walking with her head high, confidence in her eyes, and a giant smile on her face. I was in the stands with my camera flashing and wondering how lucky I was to see both my son and daughter on the same field that special night.

Our team went on to win the game and the rest of Homecoming week was underway. Our kids had a great time at the dance, the annual bonfire, and the pep rally. As a member of the Homecoming Court, Emma participated in the bonfire and pep rally helping to lead the school in cheers.  They didn’t allow this old guy to attend, but according to Emma and Carter, it was awesome.

Last Friday night Homecoming week came to a close with the annual rivalry game of D East (us) vs. D West.  We arrived extra early, grabbed our seats, and watched the crowd of 7,000 slowly assemble. The action started fast with 14 points for D East followed by a fast 14 for D West.  As the game continued we saw lightning off in the distance and rumblings that it was headed our way.  As the second quarter rolled into the third, D East extended their lead, and the weather continued to roll. 

At the end of the third quarter a delay was called. No matter how much everyone wanted the game to continue, safety was key so the stands were cleared and people headed to their cars. While others lined up to head home, we just couldn’t. We had hope the game would somehow continue and wanted to be there rooting on our son if it did.  Suddenly, we heard they were playing and raced back in time to watch the last few minutes with a small crowd of students and parents.  Standing in the rain and listening to the kids celebrate by singing the song "Hey Baby" seemed like the perfect ending to a perfect week.  But, it appeared I had missed a bit of magic.

When we got home Carter told us that after the decision was taken to restart the game, the teams walked out of the locker rooms and onto the field in silence.  Since the security staff had cleared everyone from the stadium, all 7,000 were gone. With nothing but the sound of rain droplets against helmets to break the silence, the boys walked out as one.  All season long their coach had preached the importance of their team as family and due to a strange set of circumstances, here they were during one of the biggest games, alone but together as brothers.  Everyone they needed was there.

While the symbolism of that moment may play more to me than others, I just love that moment.  Just as our family and many others have faced the silence of the unexpected, but rallied together, these boys did the same.  It did not matter that the boys came from different backgrounds or different faiths, when it was necessary, they stood together.  Nothing had to be said because they already knew that despite the challenge...


It's I got your number, I got your back
When your back's against the wall
You mess with one man, you got us all
The boys of fall

1, 2, 3...FAMILY!
Matt

Credit to: Kenny Chesney, "The Boys of Fall"


If you haven’t had the opportunity to see "The Boys of Fall" video, I recommend you spend a few minutes and check it out the following link.  With a little luck, it may leave you with the same level of inspiration it left me.

http://www.youtube.com/watch?v=AlXDo5WhQXI

Here are a few pictures of Homecoming which we hope you’ll enjoy.


 Carter #38:

 Emma walking across the field

 One proud girl

The Boys of Fall ... Post Game Celebration


Saturday, October 6, 2012

She's A Rainbow

She comes in colors everywhere;
She combs her hair
She's like a rainbow
Coming colors in the air
Oh, everywhere
She comes in colors


Have you seen her dressed in blue
See the sky in front of you
And her face is like a sail
Speck of white so fair and pale
Have you seen the lady fairer

She comes in colors everywhere;
She combs her hair
She's like a rainbow
Coming colors in the air
Oh, everywhere
She comes in colors



Does she always run that way?

It seemed like an odd question, but then again everything about that day was odd. There I was, standing in the colorless halls of the Neuromuscular Clinic at Children’s Hospital of Philadelphia, listening to doctors and wondering how fast I could escape back to our quiet life. My logical side told me there was no doubt this was the right place to be, but somehow it still did not make sense. Somehow our lives took a detour we weren’t expecting and I was confused.

I was jarred when it came once again, "Does she always run that way?”

His question was clearly an indication that something was not right and I was afraid to answer. Fortunately, Donna spoke up and told the doctor that it was just the way Maddie runs and asked if something was wrong. He didn't have much to say, just noted something down and moved on to the next test. Once he was done, he laid out our situation. There were a couple things to consider, but Pompe Disease was the most likely candidate and later proved to be the diagnosis.

For those of you who are new to this blog or don't recall the details, Pompe disease is a rare, inherited neuromuscular disorder that causes progressive muscle weakness. It is caused by a defective gene and results in a deficiency of an enzyme which leads to excessive buildup of glycogen in the muscle tissue. For us, it was a foreign word that meant nothing, but soon proved to define much of how we lived our life and what occupied much of this author’s mind.

One of the interesting impacts of Pompe is on exercise. It was recommended that our girls stay away from exercises which might negatively impact muscle breakdown and steer towards those which would not. The thought is that the breakdown and rebuilding of muscle which is inherent in things like weightlifting was not good as the rebuilding would not occur as in a normal person. So, we were steered towards limited, low impact exercises like swimming. To the girls who always seemed “to run that way” it wasn’t too much of an issue, but to me it was. You see, I love to run. I love the freedom of running through our town, the energy of a race, and the thrill of crossing the finish line. It may sound silly to some, but I hoped I could share that love with my girls, but due to Pompe it was not meant to be … or was it?

A number of months ago Donna was cruising through status updates on Facebook when she came across this thing called The Color RunTM. When she clicked on the website it said the following:

“The Color Run™ is a unique experience focused less on speed and more on crazy color fun with friends and family. Color runners come from all different ages, shapes, sizes, and speeds; but everyone toeing the start line has a blast. Whether you are a casual morning mall walker or an Olympic athlete, the 3 miles of The Color Run™ course will be the most memorable and colorful run of your life!”

She then watched the videos on the site which showed runners, but not the way she expected. It showed people of all stripes – fast and slow - covered in bright colors, laughing, dancing, and celebrating. They were full of energy, celebrating their freedom, together. Perhaps, she thought, this was the run for us.

By chance this was the inaugural year for The Color Run in Philadelphia so Donna signed up our whole family and marked it down on the calendar. We were not sure how our girls would last walking three miles, but it did not matter. It did not matter because the thrill of this run was not with the time at the finish, but in how long you could enjoy the process. It was perfect.

As the days drew closer I discovered that Carter and I would not be able to join the race due to another event so we passed our tickets onto the girls’ friends. While I wished we could have enjoyed the event together, I was glad it would be infused with the additional energy of their friends.

On race day, I stood on the sidelines of Carter’s game in Princeton and received text after text with photos from the race. Each one showed the girls covered with greens, blues, pinks, and purples and most importantly … smiles. When I arrived home later that evening I was greeted with stories on how much fun it was during the run and what a great party they had after thousands of people crossed the finish line. I was told there were runners and walkers, old and young, and all had a grand time. Finally, much to my delight, I was told we needed to do it again next year and that I needed to attend so I could cross the finish line with them.

I’ve checked the site and was pleased to see the race is headed back to Philly in 2013. We’ll be signing up as a family again and I hope more of our friends will be able to share in the fun. Either way, I’ll be thrilled because one my hopes will come true. I will finally be able to cross that finish line hand in hand with my girls.

Over the years I’ve learned that life can place you on detours you weren’t expecting. However, if you keep looking forward you may find the road you're on is much better than the one you expected. In our case, colorless CHOP turned out to be a haven of comfort and the answer to "Does she always run that way?” was no longer greeted with confusion.  Although I knew it all along, it just took a little race to remind me that doesn't matter whether they run, walk, win, or finish.  It doesn't matter because wherever they go their inner color can be seen from far and wide.  All you have to do is stand back, look in their eyes, and you realize...

She’s A Rainbow!!

Best,
Matt

Credit to: The Rolling Stones, “She’s A Rainbow”

If you are interested in this run in your town, check out their web page at http://thecolorrun.com/.

Here are a few pictures of the adventure.


Donna and the girls at the starting line.

 Maddie and her friends before the color (minus the cool matching socks).


Emma and her friend post race.

After the race ... smiles, laughter, and celebration.  All before 9 AM!

Sunday, September 9, 2012

London Calling

London calling to the faraway towns
London calling to the underworld

London calling, yes, I was there, too
An' you know what they said? Well, some of it was true!
London calling at the top of the dial
After all this, won't you give me a smile? 

London calling

When I was a senior in high school I had this outlandish idea.  I wanted to celebrate the upcoming step into adulthood (aka graduating) by gathering a few buddies and driving across the US.  I’d read a few books in English class about young men and adventure and I felt it was our time.  I could picture my friends and me cruising along Route 66 at daybreak, visiting distant cities, staying at strange motels, meeting interesting people, and eventually overlooking the Atlantic Ocean.  For a kid that never left California, it seemed magical.

Like many things at that age, excitement can somehow get in the way of reality. Despite immediate promises and energetic planning, realization of college requirements, parental concern with teenage boys on their own, and a little something called gas money got in the way.  Graduation came and went and life moved forward. I told myself that the opportunity to throw caution to the wind and go somewhere outlandish was not lost, just postponed.  And then, many years later, the opportunity returned.

It was the summer of 2008 and we were sitting on the sofa enjoying the Beijing Olympics' Closing Ceremonies when the phone rang.  A bit irritated by the interruption during this family time, I abruptly picked up the phone.  I assumed it was someone asking for us a political donation or that guy that kept trying to sell me lawn service. Fortunately for us it was neither, but a good friend.  Before I could so much as say hello, the question came.  "Do you want to go to London for the 2012 Olympics?"

At first I had nothing to say.  After all, I thought driving across the US was outlandish, but this seemed crazy.  Don’t get me wrong, by this time I was a seasoned European traveler for work, but the challenge in such a trip seemed too large.  First off, my family had never been on such a trip.  They didn’t even have passports.  On top of that, I wondered how in the world we could coordinate a trip for two families to the Olympics and how much would it cost.  All the caution I didn't have as a teenager was thrown to the wind when I asked the family and received a resounding, “YES!”

A few weeks later, we held our first “war room” meeting so the adults could outline our plans.  As we sat down, I saw the same wide eyed excitement that was in the eyes of my former high school friends.  This trip was a wonderful opportunity to not only see the Olympic Games.  It was an opportunity to see incredible sites, meet interesting people, spend time with great friends, and watch life long memories develop in the eyes of our children.  There was no turning back and I was thrilled.  

Weeks became months and months became years and war room after war room, we prepared.  Planning a trip like this for ten people seemed a bit daunting at times.  However, we quickly realized that the planning was not a chore, but exciting in its own right.  It was fun to talk about what we may do and what we may see.  It was fun to use Google Maps to explain the world a bit to our kids.  And, it was fun to imagine how this one trip may impact their lives forever. 

Along the way we did our best to integrate the girls’ needs vs. potential challenges in London.  First off, we worked with our IV nurse and the insurance to allow the girls to receive their treatment just before we left and as soon as we got home.  Because of the way our trip fell, they would be going longer between treatments.  Second, we anticipated a lot of walking during the two weeks, so were sure to find an apartment close to a tube station so they could spend more time on the train and less time on their feet.  Finally, we were sure to allow them as much rest as possible as we assumed they would get more tired with time. 
        
So, how was it?

The trip was fantastic.  That’s a lie.  It was beyond fantastic.  It was amazingly fantastic!

It was amazing to stand in front of Big Ben, ride the London Eye, cruise the River Themes, tour Buckingham Palace, walk across Abbey Road, visit Stonehenge, and also watch some of the best athletes in the world compete at the top of their game.  It was simply everything we had planned for, everything we had hoped for, and so much more.

Because of some good connections, some hard work, and a bit of magic, we were able to attend a lot of events.  As a group, these ranged from soccer, to tennis, to indoor volleyball, to beach volleyball, to basketball, to gymnastics, to swimming, and a few others.  On top of all that, we were able to experience so many moments which may never show up in a tourist guide book, but will be saved in our hearts forever.

When we arrived home, we learned that the recovery time for our girls would take a while.  Despite receiving their Myozyme treatment the day after flying in, it took at least a couple weeks for them to get back to normal.  We all fought through the exhaustion that comes with jet lag, but theirs was a bit more challenging.  All those days on their feet, climbing up and down tube station stairs, and not being on their regular diet, had an impact.  However, you would not have recognized it in their face or their attitude.  They embraced the opportunity to be in London and were not going to let Pompe slow them down.

So in the end, life is funny.  Years and years before, a young man read about adventure and wondered what lies beyond the California shores.  While a trip on the road with his high school buddies seemed like the best way to quench that desire, he was wrong.  Years and years later he was to find out that a trip across the ocean with his family and wonderful friends was the best way to do something outlandish.  Now that the planning is over, the Olympic flame has been extinguished, and normal life has returned, that same man is so very happy he picked up the phone when it was…

London calling!

Cheers,
Matt

Credit to: The Clash, “London Calling”

Day one with the 2012 Olympics Mascot


 First event at Wimbledon...Tickets anyone?


USA vs. Brazil in Volleyball


Two dads at the end of a successful trip...Happy as Larry!


Monday, June 18, 2012

Defying Gravity

Something has changed within me
Something is not the same
I'm through with playing by the rules
Of someone else's game
Too late for second-guessing
Too late to go back to sleep
It's time to trust my instincts
Close my eyes: and leap!

It's time to try
Defying gravity
I think I'll try
Defying gravity
And you can't pull me down!

I'm through accepting limits
cause someone says they're so
Some things I cannot change
But till I try, I'll never know!

I never committed to having the steadiest hand in the crowd, but was sure I had this one. I thought about getting a tripod, but this was just another school concert. I’d been to many before and would attend many in the future. I expected her to sing just another song, but as soon as the first words came out it was clear I was wrong. What I heard was more than a solo. It was a statement. A statement of courage from a little girl who decided to look life in the eye and say, I will not be held down; I will Defy Gravity.

If you have followed this blog for any period of time you’d know that I am not an impartial judge when it comes to my kids’ performances. While I might be able to be tough on adults trying to break through on American Idol, America’s Got Talent, or X Factor, I’d give my own a 10 at the first note. One touch of emotion, one look in my eye, and I’m done. Yes, I admit it. All that aside, I was stunned that Thursday night, when Maddie took the stage. She’s taken a few singing lessons, belted out a few tunes in plays, but I did not expect this message, or then again, maybe I did.

When I was 11, I spent my time with pickup baseball games, paper football on the lunch tables, and generally doing my best to make it through Catholic School. My biggest challenge was math tables and making sure I kept my jokes quiet during weekly mass.  I faced no serious burdens only the wrong side of Sister Sarah’s wrath. This was no ruler or harsh word. It was much worse; the infamous, single space, handwritten, five page essay due the following day.  (Mine was on John Wayne.)  At the time I thought I was living on the edge (inside my carefully crafted suburban bubble), but boy was I wrong.

In contrast, when Maddie was 11, she was told by medical experts that her life would be filled with limitations. Her long term ability to walk was in question. She was to start a lifelong treatment with unclear results. She overheard others say they accepted their diagnosis and were not willing change their lives because of it. She felt alone and confused. Never once did I ever face such a challenge and am not sure what I would have done other than crawl up in a corner.  That is not Maddie's style. 

She decided to fight. She learned what she could understand, trusted her parents and caregivers, walked into Day Medicine with a smile on her face and bravery in her heart, and never, ever complained. Since that day things have gotten a bit clearer, the picture a bit rosier, and the future a bit brighter. I guess you could say it happened on its own and that she just had to wait for it. Or, you could say that in making the choice to be brave she changed her outlook.

That night when I started to video tape, I lost my steady hand. I didn’t shake because of what I saw, but because of what I heard. As I passed the camera to my son I realized I did not want to watch this through a lens. I wanted to take this in for myself. For that night a young girl was defining the way she planned to live and I didn’t want to miss it.

This past week the girls attended MDA Camp. I was pleased to hear Maddie sang again, this time at their talent show. I heard from a couple counselors that the room was silenced as she belted out her tune. This time it was a different crowd and a different song, but I hope the impact was the same. I hope at least one person was touched as I was. I hope at least one child who attended the camp, heard her voice, and quietly told themselves that despite their burden they too were going to make a statement. They too would put themselves in front of a crowd one day and tell the world that it is their turn to begin…Defying Gravity!

Bravo my dear. Bravo!
Matt

Here is a YouTube link to Maddie’s performance. Thankfully one of our friends had a tripod and took a great video. For some reason the video is a bit faster than the audio, but I promise you it is much better than the shaky one I took! I hope you enjoy it as much as we did.


Click here:
http://www.youtube.com/watch?v=4wKZsT7xp1s


Credit to: Wicked, “Defying Gravity”

Sunday, June 3, 2012

Across the Universe

Images of broken light, which 
Dance before me like a million eyes,
They call me on and on across the universe.
Thoughts meander like a
Restless wind inside a letter box
They tumble blindly as they make their way across the universe.

Nothing's gonna change my world
Nothing's gonna change my world
Nothing's gonna change my world
Nothing's gonna change my world


Many years ago he told me it took him his whole life to find out what he really wanted to be. At first it sounded a bit sad, but when you saw the joy in his eyes and heard the excitement in his voice, any hint of sadness was washed away and replaced with a broad smile. Years after playing the corporate game with the politics and repetition, he had found peace in a new profession. Who could have guessed it would be at age 70 and as a volunteer fireman?

As you may have read in blog posts before, we have become active supporters of the MDA: speaking at events, gathering with friends to raise money, and meeting some wonderful people from all walks of life. Recently Maddie and I were asked to join their Fill the Boot fundraiser kick-off meeting in Harrisburg, PA. The Fill the Boot campaign began back in 1953 when a Boston fire fighter inspired his colleagues to raise money for a friend who had two boys with muscular dystrophy and has expanded since. (That friend's name was Charlie Crowley, which is odd, but no known relation.) Over the years, fire fighters have stood on street corners, boots in hand, raising money for kids with muscular dystrophy. Since 1953 the International Association of Fire Fighters have raised $435,000,000!

When Maddie and I were asked to come at speak at the event, she was excited and I was nervous. Maddie had recently been named the Pennsylvania State Goodwill Ambassador and was eager to get to work. I had spoke about our story in front of corporate groups, families, and others, but never in front of this group. My default was to present the same slides I had done so many times before, but something was driving me to tell a different story so I could not only motivate this group to hit the streets with vigor, but also offer them a connection to what they do. At first I did not understand, so set is aside.

I sat for some time staring at a blank computer screen trying to come up with the right words, but they did not come. And then, out of the blue, it hit me.  My normal story just would not do. I had an important story to share and this just might be the only chance I'd have to share it. So, I opened up my photo files and searched.


On the drive to Harrisburg Maddie and I talked about life, school, friends, and any old thing she wanted. We played the alphabet game, watched one town pass after another, and enjoyed the quiet. For those of you who have teenage children, you can appreciate how busy life can be going from one event to another. The opportunity to share moments of solitude with your children is a treasure.

When we arrived at the hotel and started to meet the fire fighters from across Pennsylvania I became nervous. I had practiced my speech and had to make sure it was right. I felt an obligation to ensure my message was passed on clearly and that it left the group with the same level of inspiration that it gave me.  When it was our turn, Maddie and I stood up and off we went.

I started the presentation with a short introduction and explanation about Pompe.  I then told them that I would not share the usual story of our experiences since diagnosis. Today was special.  Today I would share the story of another fire fighter who once said, "there is something special about that one" when he referred to our Maddie. I offered them four dates: 1928, 1993, 1998, and 2004. The first was the date of his birth, the second was the date of his retirement, the third was the date he discovered what he wanted to be, and the last was his passing.

I told the story of a man who got involved in his little retirement area, in California's high desert.  Through doing so, he noticed something was missing and just like he had done so many times before, he went about to fix it. He raised awareness, raised money, and helped the community invest in a new fire vehicle.  He went further by inspiring others to become volunteer fire fighters and then decided it was the thing for him as well. After standing on the sidelines, he became a volunteer, fought mountain fires, house fires, saved crash victims, and became a member of the fire fighter brotherhood.  Years later, those brothers stood by him when he fell ill and later by his family as their trucks lead us all to his resting place.

My story did not end there. It continued with a story of inspiration. By sharing photos, I explained that his dedication to fire fighters lives on. I told them how his inspiration guided his grandson's decision to become a San Diego fire fighter. I also told of his grand daughter's commitment to help inspire others including here today. While she may lack the strength to match the physical challenges of fire fighting, she was dedicated to help where she could. I stood at the podium with pride as the photos were revealed one after another. This was the story I wanted to tell because this man was more than a firefighter. He was my father, John Crowley, and one of their brothers.  While he wouldn't be there to stand on the street corners with them, he would be with them in spirit.

Maddie and I finished our presentation together. I mentioned a few lessons we have learned along the way. Maddie said a few words about herself and then what the MDA means to her, her sister, and to her friends. She spoke about fund raising for treatments those she knew that had none. She spoke about her anticipation for MDA Camp and that she loves when the firefighters come there to make dinner. Best BBQ ever! She then thanked them for what they do and for offering hope to everyone with muscular dystrophy.

Here are some of the photos I shared at the end of my presentation:

Fighting a house fire.

 
In uniform.


His son and grandson, the San Diego fireman.

 
His grandson (middle) at work.

 
                                His grand daughter with all the gear.

A few weeks past and I moved on to the other issues of life. There were school events to attend, work to be done, and blogs to be written. We then met up with some of the MDA staff that attended the event. As we relaxed, one approached me and told me she had a photo from my speech, which she wanted to share because there was something special about it. She had taken it at the Fill the Boot event when I was talking of inspiration and sharing photos. When she showed it to me I just smiled.

As you can see below, it's a photo of me at the podium and my dad on the screen.



Maybe it was the lighting in the room; the angle of the picture, or just maybe it was a little divine touch. Either way, that photo showed a son proudly speaking of his father and a dad whose face seems to have taken on extra color and come to life. I'm not saying it was hocus pocus or mystical magic. All I know is that it was a moment, captured in time, when a man appeared to reconfirm he was right all those years ago when he looked at Maddie and said, "there is something special about that one". From Across the Universe he saw that his little grand daughter was working hard to inspire at least one more fire fighter to volunteer to help others, just like he had done so many years before.

So, the ending of the song says, "Nothing's gonna change my world," but I don't believe it. I don't because I've witnessed a team of dedicated firefighters, a grand daughter, and a father do just that.

Thanks for reading,
Matt

Credit to: The Beatles, "Across the Universe"

Sunday, May 27, 2012

Do You Believe in Magic?

Do you believe in magic in a young girl's heart
How the music can free her, whenever it starts

And it's magic, if the music is groovy
It makes you feel happy like an old-time movie

I'll tell you about the magic, and it'll free your soul

I was in 5th grade when I first saw magic happen in Philadelphia and I never thought it would be repeated. Boy was I wrong!

As a young boy, living in Southern California, my knowledge about Philadelphia was limited to two things. The first is what we had learned in Social Studies class and the second was what was happening on the basketball court. Like everyone else, we learned all about the Liberty Bell and the Declaration of Independence. While interesting, something that happened 200 years ago seemed like ancient history considering that every LA building he saw was no more than 30 years old. However, the second thing about Philly was very real to a kid who loved basketball: his name was Dr. J.

During basketball season, the five Crowley boys had one team: the Los Angeles Lakers. I recall nights watching them on TV and hours in our driveway trying to reproduce Kareem Abdul-Jabbar’s famous sky hook. The Lakers were our team and there was no room for another. However, when no one was around I would also quietly pretend I was a certain 76er. Dr. J. was one of the most amazing athletes I had ever seen. He seemed to soar above the rim and make the unbelievable happen. Over and over, I would attempt his famous behind the backboard layup, but I was a little too small and honestly a little too uncoordinated. I quietly thought he was the best, but I was soon to see better.

It was the 1979-1980 NBA season and the Los Angeles Lakers were playing the Philadelphia 76ers in the Finals. I loved watching Dr. J, but there was no doubt my allegiance lied with the Lakers. It was a hard fought series and at the end of Game five I was worried. Kareem injured his ankle and could not play. With Game Six back in Philadelphia, I thought the Lakers were in trouble. That was until a rookie player decided to step up and play center. In front of the 76ers home crowd, he scored 42 points and recorded seven assists covering the court like no one I had seen before. From that day forward he was my new favorite player. His was named Earvin Johnson otherwise known as “Magic”.

Over the next several years the Lakers continued to get better and won a series of NBA Championships. Dr. J retired from basketball and the 76ers took a step back while other teams like the Celtics returned to glory. The year we were married Magic retired from basketball and took a step away from the limelight. These days Magic is an owner of the Los Angeles Dodgers and a TV commentator during basketball season. He looks good, but is defiantly not in shape to run up and down a basketball court, but then again neither am I. It seemed that the magic I once saw in Philadelphia was never to be repeated. That was until last Friday.

As we drove in CHOP at 8 AM it was hard to believe it was almost a year since we’d last done it. The girls’ last checkup had gone so well that the doctors told us we could wait a year to be seen. Over that year life seemed to settle in and our constant talk of CHOP faded. We transitioned to administration of Lumizyme at home, the girls kept up their swimming and exercise regimen, mom kept close eye on their diets, and I typed up blogs. Pompe was always on our mind, but just became part of our daily life. Every so often something would pop up of concern, but they were fewer and farther between. Regardless, that morning we were nervous parents.

There was nothing specific to be concerned about, but since we could not see what was going on inside our girls’ muscle cells, we never really knew if what we were doing was making a difference. We continued to follow other Pompe families on Facebook and watched as some improved while others struggled. The disease is a bit tricky to predict and we were just hoping we landed on the good side of luck. We were not prepared for what was coming next.

Each appointment starts with the usual height, weight, and blood pressure followed by an introduction to some old faces and some new ones. Then it was time to settle in a little room and get down to business. The first up was physical therapy. Maddie went first, followed by Emma. The PT specialist used her tool to test the strength in one muscle after another. She tested neck flexors, elbow extensions, wrist extensions, hip flexors, hip abductors, knee flexors and more. She had the girls pinch their fingers together, hold a fist, do a 10 meter run, stand up from laying on the floor (Gower’s test), and run up four steps. Every muscle they had was tested, retested, and timed. She said everything was looking good and then printed the results.

In many of the cases the results were what we hoped for, things were stable, but in others we were amazed. Their hip muscles were stronger! Their lower leg muscles were stronger! Their 10 meter run time was faster, their Gower’s time was faster, and their four step time was faster! Something was clearly going in their favor, but before we got too excited we wanted to get the doctor’s input.

This time we met with two doctors. The first was the one who replaced Dr. Finkel, who moved to run a new hospital division in Florida, and the second was Dr. Bonnemann. He was the one who diagnosed the girls, but had since taken up a position at the National Institutes of Health. After each talked to the girls, completed a few tests, and reviewed the results from the Physical Therapist, they were pleased. The girls still had distinct areas of weakness such as their upper body, but the results were clear. They were stronger!

On top of that, the doctor commented that they did not believe there was much glycogen stored in their muscles. All those nights in the pool, all those days watching their diets, and all those afternoons receiving their Lumizyme infusions were paying off. But in addition, their early diagnosis had made a big difference. Catching this before much of the muscle damage had been done has allowed their efforts to be that much more successful.

I’m not sure if our feet touched the ground as we left the building. This was such great news and to be honest, unexpected. We had hoped to hear that things were stable and really hoping to hear that things had not gotten worse right under our eyes. Somehow we heard something we had only wished could happen something that could only be magic.

So as I sit here and type away on my laptop I am quite pleased to admit that I was wrong so many years ago. When I was 11, I thought I had seen a once in a lifetime event. I thought magic could never return to Philadelphia, but last Friday I found out that, at least for one family, it did.

We’ll be heading back to CHOP in six months to have the same set of tests and will see the same doctor. That time I’ll be a bit more confident on the ride in. That time I will have a smile on my face as we enter the building and that time I’ll be humming…

Do you believe in magic?

All for Two and Two for All,
Matt

Credit to The Lovin’ Spoonful, “Do You Believe in Magic”

Saturday, April 28, 2012

Golden Slumbers

Once there was a way to get back homeward
Once there was a way to get back home
Sleep pretty darling do not cry 
And I will sing a lullaby 

Golden slumbers fill your eyes 
Smiles awake you when you rise 
Sleep pretty darling do not cry 
And I will sing a lullaby 

It must have been that discussion with the UPS driver that made me think about it. After all it was just another Saturday morning.  I was up earlier than the rest of the clan so I could enjoy the quiet house and for some reason decided to start early yard work.  Don't get me wrong, I was more than happy to get outside because it was spring.  There is something about the combination of cool spring air, morning sun creeping through the trees, and fresh cut grass that has always helped cleared my head.  The worries of the week are left behind as the weekend world comes alive.  It was just like so many suburban Saturday mornings with one exception, the UPS delivery.

Every other Thursday we receive a call from the pharmacy to confirm that our shipment will be on the way and every other Saturday the same UPS driver delivers two boxes better than any Christmas present I could receive.  For almost a year, he's driven up our street, stopped his truck, carried the two white boxes up our driveway, and requested a signature.  There has never been a question, just the joyful hello you would expect from any UPS driver.    

Maybe it was because I was alone outside the house so the kids couldn't hear or maybe he just figured it's been months I've been meaning to ask so today's the day. Either way, when he walked up to me put down boxes and handed me the document to sign he asked.  "If you don't mind me asking, what do you guys get every other Saturday morning? Each week, my truck is empty and you are my only delivery."  So I explained.  I explained our girls have a rare, genetic muscle disease called Pompe and that these boxes hold their medicine.  

He looked at me with a sad face and said, "So is this going to make them better, like will this make them recover?"  I told him it would make them better, that this was a lifelong treatment they would be on for all their lives, and that it was OK.  He awkwardly apologized, not knowing what to say.  I then explained to him how much we looked forward to hearing the roar of his truck on Saturday mornings.  To us it means independence and a better quality of life for our girls.  This seemed to lift his spirits because he smiled, shook my hand, and jogged off to his truck.

In May 2012 we will mark one year since that wonderful day when the girls first met their nurse, first watched their medicine mixed at our kitchen table, first had the IV set where they ate their dinner, and first sunk into their own sofa as the medicine seeped through their veins.   When we received the green light to have the infusions at home, we were nervous, excited, tense, thrilled, busy, and ... did I mention nervous?  Now, it is a day we look forward to.  By the end of two weeks the girls are a bit more tired, a bit slower, a bit achy, and ready to get their meds.  To make it even better, we love our home IV nurse Adam.

As you can imagine, the thought of having someone come into your house every two weeks and sit for hours with your children was a bit unsettling.  Would they be reliable, would they be respectable, would they be able to manage any crisis, and most important to me - would they have a sense of humor?  Now I recognize having a sense of humor is quite low on the totem pole behind capability here, but to survive in our house, it's a must.  Fortunately for us, Adam is awesome.  His medical skill and knowledge are top notch, he relates well to the girls, and has a great sense of humor.  

Having Adam at the house is like having a good friend pop by every other week.  When he arrives it is all business.  He gets that medicine mixed, gets those IV's going, connects the IV pumps, and ensures the girls meds are flowing well.  Then, things calm down a bit.  At all times he is like a bear watching his cubs to make sure they are doing well, quietly checking IV lines, taking blood pressure, and the like.  In between we all talk.  We talk about food, we talk about medicine, we talk about politics, we talk about the kids' events, we talk about how cool it must be to be Carter, and then we talk about food again.  Adam is quiet the food connoisseur and Donna and him love to talk about this recipe, that recipe, etc., etc.  By this time I usually step back into my office, close the door, and catch up on work.

One of the benefits of what I do is that I am able to work from home often.  This comes in handy on treatment days in case the girls need to be picked from school or I have to let Adam in to get set-up before they arrive home.  One downside can be getting things done in a loud house.     At first this concerned me with the treatments at home, but over time I've noticed that after the rush of the set-up, our house turns into a gentle, quiet place.  Quite often the girls move to the sofas to do homework or read as they get their meds.  The portable IV pump enables them to move around the house with ease, but Adam likes them to be together so he can monitor them easier, keeping an eye out before anything becomes an emergency.  I'll turn on some background music or a fan and work away without problem.

The other day I came out of the office and the house was silent.  Adam was reading the paper, Donna was at the grocery store, Maddie was reading, and Emma was nowhere to be seen.  I asked Adam how things were going and he said, "Great!"  I then asked where Emma could be and was directed to the front room.  There I found her, under a blanket, fast asleep with her IV pump at her side.  In the old days, we would drive home from CHOP with two kinds of kids.  The first kind had the giggles as their body was filled with a strange release of sugar, which made them laugh uncontrollably.  The second kind were exhausted, fast asleep from a long day as their medicine quietly did its magic.  For Emma, today was the latter.  She lay fast asleep with golden slumbers filling her eyes.

It's been a long time since I have been a junior in high school and don't recall if I worked as hard as she does.  Every class she takes seems to be honors this or advanced that and every week there are discussions about SATs and colleges.  Some of the time they come from her to us, but most of the time they come from others. The usual questions range from, "Where do you want to go to college?" to "What do you want to be?" to "What do you want to do?" and "Are your grades, SATs, ACTs, etc., good enough?".  I appreciate all of this, but also recognize it's a lot of stress on a 17 year old who also has to deal with being a bit more tired than some others and worries at times what the future with Pompe may bring.  

When I saw her peacefully sleeping I couldn't help but sit and smile.  For those few moments the best part of having home treatments was realized.  Despite all the struggles or worries she may face, she is able to curl up on her own sofa, with her own pillow, and be swept away to slumber with the confidence of knowing that smiles from her parents, her siblings, and her awesome nurse Adam awake you when she rises.

So as I got up and tip toed back to my office, I left a small kiss on her forehead, and thought how fortunate we truly are.  We have wonderful kids, a great support team in our area, and a wonderful nurse that every two weeks fills our house with laughter and brings comfort and hope to our family.  With that, I smiled and hummed...

Sleep pretty darling do not cry 
And I will sing a lullaby

Happy Spring,
Matt

Credit to, The Beatles "Golden Slumbers"

*If you have never taken the time to listen to side two of Abbey Road, I recommend you do.  A sunny, spring morning is the perfect time.  Leave the mower in the garage.  A hot cup of coffee is a better plan.  Enjoy!