Tuesday, April 26, 2011

I Love To Laugh

I love to laugh
Loud and long and clear
I love to laugh
It's getting worse every year

The more I laugh
The more I fill with glee
And the more the glee
The more I'm a merrier me

Most of what I've read about Pompe Disease and fatigue relates to respiratory issues.  According to pompe.com, "Most people with Pompe disease eventually experience breathing difficulties. These difficulties may start out mild, and it may take some time before patients or doctors recognize them.  Pompe disease often results in a weakened diaphragm, the large muscle located just below the lungs that plays a crucial role in breathing. A weakened diaphragm makes it hard to breathe deeply and get enough air into the lungs, especially when lying down, ... eventually leading to respiratory insufficiency."1   

Fortunately, our girls have performed well on their PFTs (Pulmonary Function Tests) to date and have even shown improvement in some areas.  However, we have occasionally seen Emma affected by fatigue between treatments.  This seems to follow a regular pattern.  She starts out with lots of energy after her Lumizyme treatment and then begins to feel tired a few days before her next.  As soon as her treatment is done, she's back to old Emma, full of energy.  Along with her bounce of energy comes another side effect, laughter.

If you've seen the movie Extraordinary Measures you may remember the scene when the kids receive their Myozyme treatment for the first time.  The parents and Han Solo, sorry Harrison Ford, are standing outside the room wondering if the treatment will work and off in the distance they hear laughter.  When they rush to the room, Indiana Jones, sorry Harrison Ford, tells them they are laughing because the glycogen is being released from their muscle cells.  They are laughing because they are experiencing a sugar high.  We have never experienced this result at the hospital, but have seen our share of giggles on the way home.

Growing up as the youngest of six kids with a dedicated mother and a serious, yet witty father gave me a few strengths.  One is resilience and another is humor.  My dad was a serious guy when it came to solving problems, but had a dry sense of humor the rest of the time.  He was famous for his outrageous questions at the dinner table especially when a new friend or fiancĂ©e decided to stay for a meal.  Out of nowhere came questions about the day’s events or his kid's past that were delivered so logically that our guest was almost forced to agree.  This was followed by raucous laughter, a humble apology by my dad, and a lighthearted dinner.  Somehow this has made its way to our dinner table many years later and many miles away.

We are not sure what respiratory challenges Pompe may bring to our girls in the years to come.  We do know that many of the adult patients we've met use a BiPAP machine while they sleep to ensure proper breathing.  At the same time, we hope our combination of exercise, diet, enzyme replacement therapy, and a fighting spirit will keep that BiPAP machine at bay.  Whatever happens we will face it with strength and with a little humor.  While it may sound silly to some, laughter may not be the best medicine for all cases, but it sure does help.

So, the next time you're a bit tired, feeling a bit down, or maybe even have some fresh Lumizyme rushing through your veins, stop by the Crowley house after the girls' treatment.  We'll likely be sitting around the table, laughing about stories from our day, and maybe even singing...

We love to laugh
Loud and long and clear
We love to laugh
So everybody can hear

The more you laugh
The more you fill with glee
And the more the glee
The more we're a merrier we!  

Credits:
Richard M. Sherman and Robert B. Sherman (from the movie Mary Poppins), "I Love To Laugh"

Sunday, April 10, 2011

Sunday Papers

You can read it in the Sunday papers,
read it in the Sunday papers

Just a short update this time around.  

As mentioned in my last post, Whats So Funny 'Bout Peace Love and Understanding?, Dr. Lisa Sanders of the New York Times has written an article about Maddie's diagnosis.

The article was released in today's New York Times Magazine.  Please check out the link here, New York Times MagazineBetter yet,  go pick up a fresh copy, a large coffee, and enjoy the read!

All for two and two for all!
Matt

Credit to: Joe Jackson, "Sunday Papers"