Thursday, November 17, 2011

Baby You're A Rich Man


How does it feel to be
 
One of the beautiful people?
 
Now that you know who you are
 
What do you want to be?
 
And have you travelled very far?
 
Far as the eye can see. 

Baby you're a rich man, 
Baby you're a rich man,
 
Baby you're a rich man too.

This time of the year offers an odd mix of Christmas joy and New Year's anticipation. You'll find the rare station still playing classic Christmas carols, trying to retain the warmth of the season, while at the same time racks covered with paparazzi magazines listing the best things of 2011.  You'll find lists with the best news stories, best movies, best celebrities, best looking, the best scandals, and the best scoundrels. One list that never misses attention is the richest people.  

We list those who have the most family wealth at the end of 2011, those who were paid the most money in 2011, and even those that lost the most money in 2011. Some of these stories are meant to make us feel good and others are meant to make us upset.  In these times of economic struggle we must find it satisfying to stand on the side of the good rich (aka Steve Jobs) and against the bad rich (aka anyone who works on Wall Street).  I'm not sure this makes anyone feel any better on January 2nd, but such is life.

One thing missing from the list is a definition of the word "rich". Merriam-Webster defines it as "having abundant possessions and especially material wealth".  This is easy to understand.  Dollars and cents are simple to count and compare.  When we think rich we think Bill Gates, Warren Buffett, or some movie star relaxing on a beach in the French Riviera.  I propose this is only a partial definition of the word.  Perhaps there is more than meets the eye.

A few weeks ago we were invited to Amicus Therapeutics to speak about our experiences with Pompe disease.  Amicus (http://www.amicustherapeutics.com) is a biopharmaceutical company, lead by John Crowley, which is working on pharmacological chaperones for the treatment of genetic diseases.  The science is much more sophisticated than I can detail, but the idea is chaperones will bind to a protein to increase its stability and increase its activity.  For Pompe patients, these investigational drugs may one day be used in collaboration with Enzyme Replacement Therapy to heighten ERT's effectiveness in the body.  In other words, if the product is successful, our girls will receive the same ERT via IV therapy, but hopefully each drop will work a bit better than today.        

We've done these types of events for the MDA, CHOP, and others before, so we quickly agreed.  Any opportunity to tell our story and meet others living our life or working to improve it is time well spent.  So, we loaded up the car and drove to Amicus's offices in Cranbury, New Jersey.  We received a warm welcome by members of the staff and were set up in a big conference room just in time of lunch.  Before we knew it, the food and the employees starting rolling in.  You never know how many people might attend these sessions, but we were lucky enough to have seventy employees. Working for corporate America myself, I know how tough it can be to get a few people away from their emails and meetings, so we were honored.

I started off the meeting with a few slides highlighting our story, our learnings, and our hopes.  As soon as I began to talk I couldn't help but move around the room, test out a few one-liners, and make sure my message was as clear as possible.  I was in "work mode", trying to engage the group and make sure their time was well spent. However, as I continued I didn't see people checking watches and Blackberries as is so common in today's fast paced business environment.  Rather, I began to see emotion.  In the midst of a never-ending focus on quarterly financials and clinical trial schedules, they were remembering the reason why they chose this profession.  They are heroes to people who look to science to bring solutions to their loved ones facing disease.

After I finished my presentation, I invited Emma, Maddie, and Carter to take questions. To ease any concern about asking prying health questions to teenagers, Emma stepped up, spoke about her love of public speaking, and her interest in answering anything and everything about life with Pompe.  The crowd seemed to relax and the questions began.  

"Where do you receive your treatment?" 
"Do you find yourself getting weaker or stronger?"
"How do you cope at school?"
"What do you tell your friends about Pompe and what do they think?"
"What is it like to be a brother with two sisters affected by Pompe?"
"How do you plan to transition to college?"

Donna and I chimed in here and there when necessary or when the questions were directed to us, but we tried to let the kids do their thing.  For most of the time, I just sat back and grinned with pride.  

Toward the end of the session, a man raised his hand so he could ask a question. Before he did, he stood up and told us that he too had three kids.  One was a teenager, one was a bit younger, and one was soon to be born.  His voice began to quiver as he said his wish was that one day his three children would be as strong and as close as those he saw before him.  The power of his statement hit me as I looked at our three kids.  Two years ago their world was rocked and now they are standing confidently together fielding questions and displaying their commitment to each other. "Wow!”  

We finished off the meeting with a bit of dessert, a chat with some of the attendees, and a promise to stay in touch.  Just like other events before, I hope we left behind just as much value as we gained.  As I look back, I realize this day offered much more than I expected.  It's been a crazy couple months as I've scurried around trying to finish this task and that task at work.  The busy time has kept me away from some of the things I love such as this blog and made me focus too much on the short term issues of finance and quarter end results.  Looking back, I remember that while all those things are important, they are fleeting.  Just like the definition of "rich", the definition of "life" is so much deeper and so much more fulfilling.  

So, as this year comes to a close you can spend some time searching if you wish, but I'll save you the time.  You won't find me on any of the Best of 2011 lists.  I haven't starred in a top 10 film, been rated as the most beautiful, or been named the richest guy in America.  However, as midnight approaches and 2012 is rung in, I won't be worried by any of it.  Instead, I will be spending the time with my loved ones, counting my blessings, and thinking of the day we visited Amicus and heard the kind words from another father, who saw our children proudly standing together, and remind myself once again that...

Baby you're a rich man,
Baby you're a rich man,
Baby you're a rich man too.

Happy New Year!
Matt

Credit to: The Beatles, "Baby You're A Rich Man"

Wednesday, November 9, 2011

And Your Bird Can Sing

You tell me that you've got everything you want
And your bird can sing
But you don't get me, you don't get me


When I was a teenager I thought my future was pre-determined. I didn't have the athletic ability to become a professional athlete nor the interest in becoming a lawyer. I was going to be a musician, writing songs, playing guitar, and singing in a band. I took the typical first step toward my goal, buying the first guitar in a pawn shop and taking lessons. Once a week I would head down to La Habra Music for lessons and each night I would slide on the turntable and play over the loud music coming out of my cheap speakers. 

With the help of my teacher I was able to learn how to play chords, a couple simple riffs, and eventually my first song called "And Your Bird Can Sing" by some band named The Beatles. I must have played that song a hundred times, each time imagining the excitement of being on stage. Thankfully my parents were gracious enough to let me go at it over and over until I had it down. I'm sure it didn't sound anything like George Harrison, but I didn't care. I knew a song! 

Time went by and that guitar was slowly replaced by college books and a girl I would someday call my wife. As the years rolled by we moved place to place from crappy apartments, to starter homes, and eventually to a nice place we are proud of. Each time we moved that guitar came along even if it found itself stashed away in a garage or basement. Here and there I would search it out and begin to play again just to see if I remembered that old riff and maybe that old song. 

As our kids aged I noticed that we did have a musician in the house, but it wasn't me. It was Maddie. It started with clarinet in elementary school and then transitioned to violin. When she was in third grade she was playing with the fifth and sixth graders. By fifth grade she was first chair, tuning the orchestra, and leading in solos. Over time Maddie added a bit of piano and eventually decided to open her heart to singing.

The first time I really heard her sing a solo in public was at last year's school musical when she played Alice Applebee. I sat in the audience and heard a voice shooting out from a little girl with such joy and strength.  She did a great job and her confidence boomed.  After the play ran its course, she was hooked. She soon started singing lessons once a week and began to prepare for the next musical. 

A wonderful thing she did not consider was singing's importance to her health.  Just like the benefits she'd seen from those nights in the physical therapy pool, singing was helping her. You see, in addition to muscle weakness in arms and legs, adult onset Pompe carries the risk of respiratory complications caused by weakening of the diaphragm and other respiratory muscles. If allowed to progress without treatment by ERT, diet, and exercise, Pompe can lead to breathing issues including the use of bipaps at night and potentially respiratory failure.

People say a muscle at rest usually stays at rest, while a muscle in motion usually stays in motion.  I don't know if Maddie will ever face these challenges with Pompe, but if the chance is there, why not keep those muscles in motion?  After all, what's wrong with the sound of music throughout the house especially if it brings joy to a little girl's heart?

That guitar now hangs in my home office waiting for the opportunity to be used once again.  Maddie has been getting ready for her next audition by practicing lines and singing songs.  Maybe it's time to pick it up, get it back in tune, and play it beside my little bird, who clearly has more musical talent than I ever will.  As I listen to her I wonder if each time she opens those lungs and fills the house with her song, she is looking Pompe disease right in the eye and singing...

But you don't get me, you don't get me!

Encore!
Matt


Credit to: The Beatles, "And Your Bird Can Sing"

Sunday, November 6, 2011

Hello Goodbye

You say yes, I say no

You say stop and I say go, go, go
Oh, no
You say goodbye and I say hello
Hello, hello

I don't know why you say goodbye
I say hello
Hello, hello

Last July, I wrote the post Two of Us where I first shared the story of Mike Stanzione. As a reminder, Mike is a Pompe patient who has faced many of the same struggles other adult Pompe patients do, but with a twist.  While Mike had the same challenges with muscle weakness, respiratory deficiencies, etc., he wasn't able to manage them in the comfort of his home.  Rather, due to his 24 hour dependency on a ventilator and his health insurance, he was required to stay in a hospital.  The twist is Mike's hospital stay lasted over four years with no end in sight.

When I met Mike last summer I was inspired.  I was inspired because despite the steadfast position from his insurance company who said he could not go home, he continued to believe that his stay was temporary. I was inspired by his commitment to wife and son and his family's commitment to him, never giving up, visiting and supplying posters of his young son's achievements.  Most of all, I was inspired by the man's spirit.  After four years in the hospital, he had every right to pack it in and give up, but he refused to say goodbye. 

The day I visited him he had just finished a call from a member of Dr. Barry Byrne's staff at the University of Florida. He was in conversations about a revolutionary procedure where he would be the first person with Pompe to get a diaphragmatic pacemaker. If the procedure worked, it would allow him to breathe without his ventilator for part of the day.  This was the key to returning to the only place he wanted to be...home.

Mike's residence was the long-term care area of Bergen Regional Medical Center in New Jersey.  This was not a hospital wing filled with the joyful sound of newborn babies. This was a serious place where people with long-term health challenges came and stayed.  I don't know what it is like to live month after month and year after year in a long term care center, but I'm sure it is not easy.  During my visit I saw people who seemed full of life and others who seemed resolved to live out their days in quiet. I'm not sure what makes the two of them different, but my belief is hope. 

Mike had that hope - the hope that one day he would be reunited with his family. Rather than listen to the officials telling him to say goodbye, he chose instead to reach out over the internet for a solution.  Through Facebook he found a worldwide network of Pompe patients waiting to help him.  He found new people with new ideas and new solutions.  This eventually led him to Dr. Byrne and the amazing people at University of Florida's Shands Hospital. 

Through the efforts of many, Mike made it down to Florida for that revolutionary procedure.  He recovered well and headed back to New Jersey where a great thing happened.  Over time, it seemed to work.  One day he was able to be off the ventilator for an hour.  A few days later it was two and a half hours.  Then it started to roll.  Four hours became six, six hours became eight, and eight became twelve. His 24-hour dependency was broken and he was finally coming home!

You can read the rest of the story in the web link below: 
If the link does not work, please copy and paste the following into your web browser: (http://newyork.cbslocal.com/2011/10/18/nj-man-suffering-from-pompe-disease-returns-home-in-medical-miracle/)

I have a feeling that when all the hoopla resides, the welcome home signs are put away, and day to day life returnes, Mike will look back.  He will remember those days in the hospital when others told him he would never go home.  He will remember the battles he won and the support from his family and friends.  Best of all, he will be remember the reason behind it all each time his son walks in, throws his arms around his dad, and says... 

I don't know why they said goodbye
I say hello!

Congrats Mike!

Matt

PS: To all my friends at University of Florida, Great job!  It's a good day to be a Gator! 

Credit to: The Beatles, "Hello Goodbye"

Saturday, October 8, 2011

Dig A Pony

I dig a pony
Well you can celebrate anything you want
Yes you can celebrate anything you want
Ooh!

Everything has got to be just like you want it to
Because...


It was a much simpler time when she began.  She was young and with the long flowing hair you expect to see on girls in elementary school.  The horse was at least three times her size so much so that she needed help just to make it up into the saddle. Once up there she did her best to control something that was clearly much larger and much stronger than she was.  At that time we had no idea that the muscle strength she struggled with would later be eclipsed by the strength of her heart to overcome her fear.

I can't remember when she first asked to try it, but I do remember the first time she rode.  One benefit of living in the suburbs of Pennsylvania is the availability of horse stables and riding lessons.  After some research, we picked a place and pulled into a gravel parking lot next to an aging stable.  The stone stable was built unlike anything I saw as a child, but became accustomed to in this area.  It was from the 1800s, was surrounded by vast fields, and seemed to quietly hold stories of the many riders who came through from the Civil War forward.  To this Southern California kid who has developed an appreciation for local history, it was cool.

We first met Jen Bucy, the owner of Hillside Stables, coming off the ring after helping another young girl learn to ride.  This was her stable and came well recommended.  She greeted us with a big smile and walked us through her training program.  We weren't sure if this would be something our daughter would pickup as a hobby or just a temporary fancy, but it didn't matter.  She was looking forward to doing something different and we were happy to oblige. Donna rode horses as a young girl and told stories of great times so it sounded like a fun thing for Emma as well.  I did not expect she would come to love it as much as she would.

In the months and years to come we spent many afternoons sitting on the grassy hill watching Emma make circles around the ring.  These were idyllic times where the world seemed perfectly aligned for our little one.  She was doing something she loved, was in a beautiful environment, and improving all the time.  I came to learn that horseback riding was not just an activity, but a bit of a lifestyle.  There were many afternoons I dropped her off not to ride, but so she could learn to groom the horses, muck the stalls, and do whatever else you do at a stable.  At times I was happy to help out as well, but did my best to let her be.  This was her place and her time to live in the beautiful land of innocence that lay all around.

Over time she struggled to get the horses to jump, canter, and all those other things they do.  This required strength in her arms, legs, and great posture all of which didn't respond, as they should have.  There were other girls that were moving into competitions and even some that got their own horses.  This was not to be for our Emma.  We had no idea her muscle strength was impacting her ability to do what the older girls were able to.  She looked just as good as the others, but seemed to plateau.  That was OK for me, but our little competitive one was frustrated.  

Months past by when she continued to ride, muck stalls, and spend time growing up next to the gentle giants, but over time the desire faded.  We are big advocates of our kids trying many things with the hope of finding a few things they love.  When we were younger we saw too many kids get pushed into one endeavor or another, only to get burned out and never return.  Our job is not to create an athlete, a musician, or a horseback rider, but to create good people who in the process might just find something they love.  So when she nervously came to us asking to stop riding we were surprised, but told her it was OK and recommended we look for something else she might enjoy.  Over time that became the clarinet and music.  

Fast-forward a few years...  These days the clarinet has been replaced by Yoga classes, an appreciation of flower arrangements, and an interest in business and literature.  This past summer she held three jobs, building up a bank account and more importantly new friends and experiences. Then, as summer was coming to a close the question came.  "Do you think I could try horseback riding again?"  She explained that she missed the innocence of those days at the stable, being around the horses, and spending hours just being a kid. She was not concerned with winning competitions or buying a horse.  She just wanted to do something she had walked away from, only to realize later it was something she truly loved.

On that first day back, you could tell she sensed the same excitement a little girl once had years before.  The stable looked the same, Jen was still there with open arms, but Emma was different.  She was not here to relive the past.  She was here to look forward. She was here to prove to herself that a little thing called Pompe would never keep her from the things she loved. So, just as she had done many times before, she walked right out of her comfort zone and into another adventure.  

I stayed long enough to reintroduce myself and take a quick look around, but quickly realized that this day was to be as emotional for me as it was for her.  It meant different things to each of us and she didn't need me around dampening the mood with never ending stories of yesteryear.  I so wanted to watch her get on that horse once again and ride around the ring, but she was no longer that 11-year-old girl with the flowing hair.  She is a young woman. 

I drove away waiting to hear the stories of her grand adventure and thinking.  I thought about how the years have past, but most of all I thought about how proud I was of my daughter.  While she had every justification to sit on the sidelines, she continues instead to choose the playing field.  Who knows where this attitude will take her in life, but I seem to think she will do quite well.

As Lennon and McCartney correctly wrote, 
  Well you can celebrate anything you want
  Yes you can celebrate anything you want

For now she is where she belongs, on the back of a horse amid the grass and trees, quietly celebrating her victory ... over fear.  Congrats! 

Ride on!
Matt

Credit to: The Beatles, "Dig A Pony"




Here's a photo of Emma at Hillside Stables with her 
Pompe InCommon (http://pompeincommon.com/) global balloon,
showing the diverse things people with Pompe can achieve.



Tuesday, September 13, 2011

Every Little Thing She Does Is Magic

Though I've tried before to tell her

all the feelings I have for her in my heart

Every time that I come near her

I just lose my nerve as I've done from the start

 

Every little thing she does is magic
 
Everything she do just turns me on
 
Even though my life before was tragic
 
Now I know my love for her goes on 

The other day I was tidying up the basement and came across a book titled, Don't Sweat the Small Stuff...and it's all small stuff and it got me thinking about our last visit to CHOP. The philosopher in me gets the idea that you shouldn't worry about the little things in life because somehow life continues on past the day to day woes. To be honest, I've preached the same story to my kids over and over again when they would come home with concerns that those of use who are "older and wiser" have overcome without bumps or bruises. Just when I thought I had it all figured out, I learned at CHOP that every little thing Maddie does is magic. 

Most of my concerns about the girls' Pompe condition have been about their major muscle groups. It seemed obvious that their leg and arm muscles should be the priority as they deal with the obvious things... standing, lifting, and carrying. Over time I came to appreciate the importance of the muscles in their chests and their relation to breathing especially as I watched them struggle through one pulmonary function test after another. I never thought about the little muscles in your hands that allow me to type this story, text on my phone, or open a jar of salsa (Yes, I love salsa!). However, as I listened to Maddie explain her struggles to her Occupational Therapist my eyes were opened.

It's funny how we take for granted many of the things our body does on a daily basis. From grabbing that first cup of coffee in the morning, to paging through the newspaper, to drumming away on the steering wheel, to typing that first email, we use our fingers. We use them to do just about everything and usually don't think twice about it. For me, I don't think about them until I get the smallest cut requiring a band aid and I wait for the day when they are free again. I never thought a thing called Pompe could impact their ability. 

I've observed more than a few Muscular Dystrophy kids with weakness since our girls were diagnosed, but one sticks out in my mind. She was a teenager at the MDA Muscle Summit in Camden, NJ who the girls met right after the day's events were complete. When she introduced herself to me with a sweet hello, I reached down for her hand, not thinking twice about it. I was taught early that looking someone in the eye and shaking their hand was the proper thing to do, but in this case it wasn't. As I picked up her limp hand, she smiled and said her hand just wasn't too strong anymore. I felt about 2 feet tall and apologized, but to her it was OK because it had happened so many times before.

Since then I kind of forgot about the event and focused all my energy on making sure the girls were on the right track. Treatment after treatment went well and CHOP appointment after CHOP appointment showed stabilization or even improvement. Then, during last month's appointment Maddie mentioned her fingers. She explained to the OT that sometimes she struggles to button the buttons on her shirts and to open water bottles. Emma then chimed in to say she is challenged with typing sometimes especially when her fingers get lazy and rest on the keyboard when she wants them to move on. Funny, they never complained and I never really noticed.  That's their way, I thought, never complaining, just adjusting. 

The OT did a great job listening to them and giving them some great advice such as slightly cutting the button holes or resetting the keyboard controls. After the practical advice he offered something even more important, understanding. He said he understood their frustration and promised to work with them to improve it. He also explained that sometimes he sees people who are really strong, but have weak hands and other times he sees people who are quite weak, but whose hands work well. While he understood their concerns, he asked the girls to remember that while their bodies are weak, they could still do the majority of what they wanted with their little hands. That was not only important, it was magic.

Tomorrow morning when I wake up, grab that first cup of coffee, button that dress shirt and type away that first email, I've promised myself not to sweat the small stuff, but to think about Maddie Jane. I will, because I know that no matter where I am, she will be there fighting on, and because I know that no matter what life brings her, to me... 

Every little thing she does is magic! 

Stay strong, 
Matt 

Credit to: The Police, "Every Little Thing She Does Is Magic"

Sunday, August 14, 2011

I'm Only Sleeping

When I wake up early in the morning
Lift my head, I'm still yawning
When I'm in the middle of a dream
Stay in bed, float up stream

Please, don't wake me, no, don't shake me
Leave me where I am - I'm only sleeping

Everybody seems to think I'm lazy
I don't mind, I think they're crazy
Running everywhere at such a speed
Till they find there's no need

Please, don't spoil my day, I'm miles away
And after all - I'm only sleeping

As the youngest of six, I looked up to my older siblings for a variety of reasons, but when it came to sleeping in, Michael was the ace.  Of course there were other things that made him cool including his willingness to let his younger brother hang out with the older dudes (as long as I promised not to speak), his skill rebuilding customized VW engines in lightning speed, his fried bologna sandwiches, and his ability to make me laugh so hard my sides would ache.  While each of these deserves their own story, except for the fried bologna sandwiches, I'll focus on that teenage boy's ability to sleep. 

When I was a pre-teen I struggled to sleep past 8 AM, but Mike would regularly crawl out of his room at 1 PM.  Sure, he was six years older and went to bed later than me, but even when I grew up I could not match his skill.  For those of who grew up in the big city, surrounded by a never-ending supply of entertainment, sleeping in may not seem like a big deal.  Trust me, for a kid growing up in suburban LA this was important.  You see, weekend morning after weekend morning his room remained covered in darkness while I ate my Captain Crunch in front of the television wondering when the call would come.

There were many great things about SoCal as a kid, but one of the bad things was the weather.  Bad, you say?  Yes, bad because since the weather was always nice the weeds just never stopped growing. So while waking up early on Saturday meant you had first dibs on the cartoons it also meant you were the first kid dad would grab on the way out to weed the hill.  When I think of that hill now it wasn't that big, but as a kid it was a football field in length and as steep as Everest. Hour after hour I would labor away on the Crowley Chain Gang while my brother slept.  I guess I should have been angry, but I was filled with admiration.  I thought I was good at avoiding work, but that boy was a genius!

More years have passed than I care to remember and so far my kids have followed my sleeping pattern more than Michael's. After a long week at school or a late night sleep over, they are up no later than 9 AM, ready for the day.  Fortunately, our house has no hill to weed so it’s been easy duty for them.  The only battle is who gets to choose the cartoons.  This all changed a few weeks ago when the girls missed out on their Lumizyme.

About a month ago, their treatment day was approaching like clockwork.  The delivery arrived on time, the meds were placed in the fridge, the supplies stashed away in their boxes, and the nurse's schedule confirmed.  But then a sinus infection popped up out of the blue.  We’ve learned that it is important to be aggressive with infections before they spread to the lungs, as Pompe patients don't have the best pulmonary function.  So, off to the pediatrician they went and home they came with a two-week prescription for antibiotics.

We were happy they were on the path to good health until their doctor advised against the Lumizyme treatment because of the antibiotics.  I assumed they could get it the next day or next week, but no go.  The two-week dose of antibiotics meant they would miss their whole treatment and would have to wait another two weeks.   I did not realize how important those bi-monthly treatments were until several days later.

It’s been over a year since the girls started on Lumizyme.  From the first days at CHOP to the recent days at home, they have never missed a treatment.  Because of this, their diet, and exercise, they have clearly shown improvement in some areas and stabilization in others.  We had not seen a decrease in their energy level or ability to get around until we began to see a gradual impact upon on Emma.

It was normal for us to see her slower in the PT pool the day before a treatment, but this four week lull concerned us.  In the mornings, we had to drag her out of bed so she could make it to work and in the evenings drag her down to dinner so she would eat before bedtime. She would sleep in every morning, take a long nap after working a four-hour shift, go to sleep early, and repeat the cycle.  At the same time, she began to struggle up the stairs just like she had 18 months before.  It was concerning.  The girl who always got up early and had the energy to get things done was clearly zapped.

Fortunately, two weeks later our home healthcare nurse arrived at the house, the Lumizyme warmed to room temperature, and the IV drip began.  Within hours Emma’s energy was back.  It was like magic.  Within a couple days her sleep pattern returned and her ability to climb stairs was back to normal.  Don’t get me wrong, the medicine has not enabled her to race Usain Bolt up a flight of stairs, but it has returned her to the active young lady she is.

When I look at my kids I wonder how they will describe their siblings twenty years from now.  Will their older sister be cool because she let her younger brother and sister hang out with her friends (as long as they promised not to speak)?  Will their brother be cool because he repaired his sisters’ cars or made a mean bologna sandwich?  Will their younger sister be cool because her humor made them laugh until their sides ached?  Time will tell.  I'm sure each of them will remember a number of special things other than Pompe because the girls' treatments never allowed the condition to change what makes them special to each other. 

In twenty years I expect I will still be running around after them, but just a bit slower than I do today.  I plan to be there every chance I can to help them out and watch them continue to mature into great people.  However, while I love them all very much, I hope they don’t stop by too early on Saturdays mornings expecting me to help weed their hill.  By that time, I plan to try once again to see if I can match Michael’s skill and will hang a sign outside my door saying…

Please, don't wake me, no, don't shake me,
Leave me where I am - I'm only sleeping

Wake up!  Enjoy the week,
Matt

Credit to: The Beatles, “I’m Only Sleeping”

Sunday, July 31, 2011

Freeze Frame






I could see it was a rough-cut Tuesday
Slow-motion weekdays stare me down
Her lipstick reflex got me wound
There were no defects to be found
Snapshot image froze without a sound

Freeze Frame! Freeze Frame!
Freeze Frame! Freeze Frame!
Freeze Frame! Now Freeze!

I am usually the one taking the photos.  Just like my dad, I find it more interesting to take photos than to pose in them.  I prefer to be the clown behind the camera getting others to smile or better yet, grabbing that innocent pose they weren't expecting.  This interest is helpful, as God did not necessarily grant me a face ready for GQ magazine or the patience to sit and say "Cheese!”  Fortunately, I was lucky enough to marry someone whose good looks and patience were past down to my kids so when Genzyme called requesting a photo shoot I was OK to sit back and watch. 

Our photographer Chris flew straight from recording the tornado damage in Tupelo and arrived at our house Sunday morning at 10 AM with two bags of camera gear and an energetic spirit.  I don't know why, but as soon as I met her I knew this would be great.  From her caring manner and true interest in the girls' condition you could tell she was here to do something special, to capture a place in time which we would never forget.  Carter and I quickly found ourselves moving camera gear and relocating furniture while the girls settled on their clothes, per the photographer's advice.  It was, "Way too much blue," "Green just won't work," "Perfect for you, but what about the location," etc. until we settled outside for the first shots.  

A few months ago Genzyme contacted us to see if we would be open to having the girls be part of their Pompe promotional materials.  At first I was a bit reluctant.  Other than those that follow this blog, their diagnosis has been invisible to most of those around them, which is just fine with me.  We made a decision early on that they would remain their own people who happened to have a condition named Pompe, not a patient who is also a person.  The decision was taken with the understanding that while this condition may increasingly impact more of their life over time, they will never allow it to impact all of their life.  It will never define them.

Our first photos were quick shots of the family - the five of us, the kids, and even a few with the dog thrown in for laughs.  Then, for several hours she took photos of Maddie by her garden, Emma and Maddie playing board games, and others.  One of her goals was to show the girls in different situations, being mobile, using their hands well, and enjoying themselves.  After all, a smiling photo is a message everyone can appreciate.

By the end of the photo shoot we had relocated across our house and backyard several times and shared a few great stories along the way.  In the process we learned about her adventures around the world and she learned about our adventures in suburban USA.  When she departed there were genuine hugs all around, a phone number to call next time we visit Chicago, and a promise that she would do her very best to produce worthy photos.  She could see that our kids held a little magic among them and she wanted to make that come to life.

We recently received a CD of photos and three framed shots for our home.  She was true to her world.  Behind the shading and the dimension, the color and the location, the magic is there.  It is even there among a family (with very good Photoshop skills on dad).  It is there in the confident look of girl with her smiling brother and there with the comfort and happiness shared between two young girls who have faced more worry in their short years than necessary. 

I'm back to where I belong, behind the camera keeping track of life as it goes by.  While I know I will never have the skill of our photographer, the next time I take that shot, and see that Freeze Frame in my view, I'll remember a good day in summer when magic was captured and all was well. 

Thanks for the memories Chris!
Matt

Credit to: The J Geils Band, "Freeze Frame"

Here are a few photos from our day.







Monday, July 11, 2011

Two of Us


Two of us sending postcards
writing letters on my wall
You and me burning matches
lifting latches on our way back home
We're on our way home
We're on our way home
We're going home

You and I have memories
longer than the road
that stretches out ahead

Back in the day before CNN, DVDs, and flat screens, there was something called television.  Not satellite television or on-demand television, just television.  For the benefit of those who are two young to remember, TVs used to be a big box with one dial, rabbit ears, and channels from 1-13, if you were lucky.  Growing up in Southern California’s 70’s, the line-up was simple.  Channels 2, 4, and 7 were the networks; 5 and 9 were the locals; channel 6 was PBS; and the others ran programs I usually ignored.  During the week, you had Sesame Street and The Electric Company in the morning, and The Brady Bunch and Adam 12 after school.  Saturday morning meant Bugs Bunny and Sunday meant Popeye.

In those days you were not able to choose your programming.  You simply followed the TV Guide’s schedule or missed it.  This was particularly important during the holidays when you had once chance to catch your favorite or you waited another 12 months.  From It’s the Easter Bunny Charlie Brown to Frosty the Snowman, you had to watch the schedule or you were stuck watching some cheesy show your mom thought was “cute”.  Strangely, I still find myself doing this at holiday time, checking and asking when this show or that show would be playing.  I am then reminded by my kids that we have them on DVD or they are running 24 hours a day on cable channel 126. 

One program I never missed was The Wizard of Oz.  Each Thanksgiving this hit the little screen and we were sure to catch it.  From a child’s view, the story of The Wizard of Oz is straightforward.  A girl saves her dog, gets caught in tornado, dreams of magical land of strange characters (in color!), and wakes up after learning a lesson.  I did not watch for any life lessons or big messages.  I just liked to laugh at the Lollipop Guild.  However, if you dig a bit deeper you’ll find that the story has a bit more to offer.  It is the story of friends searching for things we all crave at one time or another – to be smart, to love, to be brave, and to go home.  Last month, I was fortunate enough to meet a man who clearly has the first three and is doing all he can to obtain the fourth.

Mike is a man who several years ago started to struggle when walking up stairs.  Like many of us would do, he pushed it aside for a long time blaming it on age, a bad back, and generally a busy life.  After all what else could it be?  Years went by until he finally visited a doctor.  Then came a long line of tests and eventually a diagnosis of polymyositis, an inflammatory muscle disease.  He began treatment and continued his life.  Unfortunately, the diagnosis was incorrect and the treatment was not working.  So he went off the treatment and did his best to manage his condition.  Then came the fateful day in 2008 when he arrived in the Emergency Room.

He was taken to the ER complaining of breathing challenges.  Upon arrival, he was informed that he had toxic levels of carbon dioxide in his lungs and that his weakened muscles were not allowing enough oxygen to be pumped into his system.  They tried an external breathing support mechanism to get him back on track, but it did not work.  Before he knew it, he awoke with something that has changed his life - a tracheotomy.  A tracheotomy is a procedure which inserts an airway into your neck, allowing a person to breathe without the use of your nose or mouth.  The tubing is connected to a machine that controls the level of oxygen and essentially breathes when you cannot.  The immediate concern went away, but what he did not know was that this little incision would keep him from home from that day forward.  

When I first heard of Mike’s situation I was puzzled.  In today’s age of medical innovation, how could someone be forced to stay in the hospital for such a condition?  I did not realize how uneducated I was.  As I’ve come to learn, a trach requires full time care in case of a blockage or failure.  Without on-site care or access to quick emergency assistance, the result could be bad.  The trouble is that full time care is expensive and Medicare only offers it two times a week.  So, without options, Mike waits at the hospital for his chance to go home.  The amazing part is that he does not wait in despair, but with clear focus on his goal.  A goal that takes him home to his eight year old son so the two of them can do all the things a dad and young son should do. 

I visited Mike in the long term care center of a local hospital recently with some hesitation.  Since the girls’ diagnosis we have gone out of our way to meet all the Pompe patients we can and each time I quietly walk away energized or concerned.  Each time I wonder if I am looking at the future of my girls or at what life might have been without their early diagnosis and treatment.  Eventually, I return to a belief that time is on their side, but some people leave a lasting mark.  Mike is one of those. 

I have never been a fan of hospitals (except CHOP!) so each time I walk through one, I am not on my game.  Fortunately, Mike’s jovial hello and warm smile set my fears aside.  When I entered his room, the hospital environment was quickly replaced with connections to his son.  There were photos of his son at home and at church, by the pool and on the baseball field, with his mom and with Mike.  There were letters and artwork, and posters everywhere.  Each showed a young, smiling boy full of life.  Mike’s reason to return home was clear.

During our visit we spoke about his son, his wife, the Yankees vs. the Mets, our mutual Pompe friends, my kids, his plans to get home through new treatment options, and his son, once again.  After a few minutes I forgot about hospitals and trachs.  I forgot about Pompe and long term care centers.  This was more than a hospital visit.  It was two dads who had never met each other, yet shared something unique.  After an hour or so of chatting, Mike’s brother arrived for his regular visit, so I thanked Mike, wished him the best, and made my way home.  As I walked out his room and past the other patients in the building, I thought about being a kid and watching The Wizard of Oz.

If you recall the end of movie, the Scarecrow, the Tin Man, and the Lion finally realize their goal.  Across their whole adventure, they believed only a Wizard could bestow their wishes, but they found they only needed a chance to use their skills to benefit others.  Mike’s chance is not coming in the form of a hot air balloon, or ruby slippers, but in an RV.  Yes, he is headed off soon, away from the hospital, and on his own adventure which will hopefully bring him one step closer to his goal.  I know this is not a Hollywood movie where everything works out in the end, but my hope is that one day he, like Dorothy, will be able to wake in his own bed, with his family by his side, and tell them, “There’s No Place Like Home.”  

Keep up the fight my friends!
Matt

Credit to: The Beatles, “Two of Us”     

Monday, June 27, 2011

Once In A Lifetime


And you may find yourself living in a shotgun shack
And you may find yourself in another part of the world
And you may find yourself behind the wheel of a large automobile
And you may find yourself in a beautiful house, with a beautiful wife
And you may ask yourself-Well...How did I get here?

Letting the days go by, let the water hold me down
Letting the days go by, water flowing underground
Into the blue again, after the money's gone
Once in a lifetime, water flowing underground

Wikipedia defines déjà vu as the feeling that one has already witnessed or experienced a situation. While I don't believe in previous lives, old souls, and all that stuff, I must admit to having that déjà vu feeling before.  While strange, it can be somewhat comforting especially when you try to figure out what to do next.  If I have been here before it must all work out, right?  Unfortunately, I could not find a definition for the feeling you have when you realize you have just driven a few miles on a familiar highway without thinking.  Or, that feeling when you are taken back by sad news you never expected.  When these times happen, you take a look over your shoulder and say, “Well...How did I get here?”  Last Saturday was such a day.

I fondly remember summer camping trips when I was young. Each year, my mom would load up the tent trailer with food, my dad would tie 112 bikes to the top, and we were on our way. Sometimes, we would take a long drive into the woods and spend our nights shooing away the mosquitoes, but most times we would find ourselves at a seaside campsite a few short hours from home. Fortunately between our large family and our cousins, there were always many kids to play with and plenty to do. Each morning began by sitting at the picnic table for cereal, each day filled with adventure out in the water, and each night finished around a campsite listening to the adults talking about the old days. If you were to drive by our site during that week you would not have seen anything special, just an old tent trailer and a tent or two.  It may not have been anything impressive to the eye, but I guarantee you that for a little boy, it was magical.

A few months ago the girls were invited to attend the MDA's local summer camp. In between appointments at CHOP, the rep told them all about the fun they would have, the friends they would make, and the gentle feeling of being with other children all leaving their daily concerns behind.  She said others raved about their stay with terms such as "best week of the year", "well worth it", and "Once in a Lifetime!"  We are not much of a camping family these days. We have spent a weekend or two at the local KOA campground, but not regularly and never in a summer camp environment. As our family resides on the west coast, we tend to spend our summer vacations there, far from tent trailers and far from the Pennsylvania woods. So, when they heard about it, they jumped at the chance.

Last Saturday was drop-off day for the campers. We had prepared over the previous weeks by gathering all the necessary supplies and speaking with the girls about the week ahead. We knew this would not be the camping trip I remembered as a child for a couple reasons. First, we would not be there with them and second, they would be surrounded by kids facing sometimes similar and sometimes much more severe muscular challenges than theirs.  We wanted them to be prepared for what they may see and what they may feel. 

As any parent, it is always a balancing act between preserving innocence and opening the door to the real world. This has become tougher for us over the past year or so as we found ourselves dealing with issues we never expected.  Our kids continue to amaze us with their strength and commitment, but we did not want to leave them on their own, separated into difference cabins, without preparation. There were times they were 100% committed to go and a short time when they were worried.  What I found interesting is they did not resolve this concern by only speaking with us, but by discussing it themselves. While we were busy doing dishes they were upstairs discussing their fears and decided to face them together.  When I was their age camping, I was busy trying to decide between Sugar Smacks or Captain Crunch, never anything like this.  When we heard them we knew it was time to let them walk the balance beam on their own.

When we drove in and parked our car, I saw what I expected: a few kids in wheelchairs, a happy group of volunteers, and a beautiful campsite. There was a grand hall, gymnasium, a pool, and a series of cabins. I told myself to relax; this is going to be great!  But when we entered the registration hall, it hit me.  Suddenly, we were surrounded by doctors and nurses, children of all ages with severe muscle disease and weary parents carrying boxes of medication that their kids must take each and every day. I had met these people before at events and had spoken to them about our story, but this was different. This was not my story.  This was theirs and I felt like a foreigner.

I couldn't stop asking myself how we got here and where the days had gone. It seemed like only yesterday that I was walking Emma to first grade with a huge smile and without a care in the world. Everything was going great. It was the American dream…three healthy kids, a great marriage, good job, and a new house. I never could have predicted this one.

In the middle of my irrational moment, I caught a site of the girls’ faces. They were laughing with their counselors and smiling for pictures with the other kids. They did not feel like foreigners, they felt like they belonged.  I realized then that all the while we had prepared the week for them, I had not taken a moment to prepare the week for me. I had not taken the time to realize that while this would not be the seaside campground I remembered as a child, the magic of camping was still all around.  This was their magic.  The one they would remember for years to come.

I don’t think my experience is unique to summer camps, muscle disease, or Pompe.  Each of us has had our own moment when we look over our shoulder or into the mirror and wonder why.  This could be over the illness of a child, loss of a parent, a job, or any other thing that was truly unexpected.  Each of us has our own cross to bear and the beauty of life comes from how we live with it.

We did not hear from them all week. This was not because they were discouraged from calling home, but because they were too busy having a blast! Unfortunately I was not able to be there for pick-up day, but Donna told me there were lots of tears, lots of hugs, and lots of promises to come back next year. As soon as they got home, they had several new Facebook friends and even more stories to tell. When I asked them to look back and sum up their week, the message was clear. It was...

Once in a lifetime!

Stay positive,
Matt

Credit to: The Talking Heads, "Once In A Lifetime"