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,

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