July 8, 2012: Hmm... what next...

Heaveno!

Happy belated 4th of July to y'all!  We had an absolutely wonderful time at the home of some dear church friends, the Olivers.  Here's Ali rocking a Wonder Woman costume with their awesome daughter, Madison.



Well, we're back home and settled in (mostly) to life around here.  My main hospital contract ended before we could get a new one signed, so I am technically out of a lot of work till at least the 19th of this month when we have another meeting scheduled to 'get it done.'  Please say a prayer it doesn't get rescheduled a third time! Yikes.  I recognize I need some down time right now, but I don't really like having the down time.  I feel lazy.  You know me... Mr. terminal-doer... It also stings when you write big checks at the beginning of the month for day care and summer camp, then you are at home earning no money while your kids are still at the places they would need to be if you were working, but you've pre-paid, so whattayagonna do?





I have Julian home on Mondays regardless, and tomorrow, I asked Ali if she'd like to stay home with us and she said yes!  I was really tickled.  Her summer camp is cool in that they have lots of cool field trips to great places, and camp is from 7am-6pm ALL summer, unlike most of the other camps I ran across.  However, on the days they don't have a trip, it's a long day, so since they don't have trips on Mondays, I think me and the kids are gonna hang together on Mondays.  Hopefully, I can coordinate some play date Mondays with the other kids from school that she doesn't see at camp.

We had a couple of good meetings this week with team members of PUCK, Minnesota Medical Foundation (MMF), and CCRF.  There is a very exciting buzz around PUCK after the success of Time to Fly.  I have to say thanks again to all who joined our team, or donated on our behalf.  We are half way through the year, and a little under $300,000 of the $450,000 Challenge Grant by JGSF and EBMF has already been raised!

That means that Dr. Tolar has received an infusion of research funds, equipment, and other in-kind donations totaling just under $600,000 since January.

I want you to know just how personal this is to me and Ang.  When we first met with Dr. Tolar, Dr. Wagner, MMF, and CCRF, there was not exactly a lot of funding in house or on the horizon for EB research at the U of M.  In fact, they had recently laid off research assistants - good, well-trained researchers of EB - because there just wasn't enough funding.  The deepest, scariest thought in our hearts was that they would run out of funding, and simply need to work on other areas that were funded, and everything Bella went through would be in vain.

It's such a wicked coin toss.  I stare at pictures of Bella on the wall, and think, "Did we kill her?"  After all, here was this big, fat, happy kid who was 95th percentile height and weight, who didn't look 'that bad...'

...then I remember the rest of the story.

The part about how her left leg had never seen the light of day.  The part about how at a year old, she couldn't stand up or crawl.  The two times that her entire tongue blistered and I had to cut it with scissors so she could breathe.  The time she woke up and her ear had blistered onto her head, and I had to cut the blister to release her ear from growing into her scalp.  The time she almost died from an 'occult infection' at 4 months old.  The 4 other EB kids that died in one month when Bella was only 6 months old.

and that was just the first year with EB.

See, I want to believe that we did what we thought was the right thing, but when it ends up with your child dying, it's sometimes hard to come to that conclusion, no matter how philosophical you wax it.

At least we know that the docs learned from the summer of 2010, when two of their babies died, and a third almost did, all because they used a common chemo drug called busulfan.  Now, they don't use that drug any more, and things are incrementally better.

But that's not good enough.

Our EB community NEEDS a treatment that is EXPONENTIALLY safer than BMT as we know it today.

Today's BMT is like the model-T car, or the first black & white TV set.  It was where we had to start, but it's NOT where we are going to finish up.

THAT is what this infusion of funding is hoping to lead to.  Dr. Tolar has two further ways he wants to treat EB that will literally be exponentially safer than BMT as we know it today.  THAT is the vision we work for.  We don't want parents to have to walk through the valley of death that BMT is today anymore.  I am both hopeful for each new patient going through the trial, but I am also scared to the depths of my soul for them and their family.

Last Sunday we were visiting Charlie in the new BMT unit at U of M.  Two years ago from that date was the eve of Bella's transplant.  It was so bad.  The mucositis which eventually lead to her intubation was unlike any torture I have ever encountered.  It still haunts me.  Two years later, and the pain is RIGHT THERE.  It slithers around my heart with barbed tentacles and clutches it suddenly, almost crippling me from the inside out.  Thankfully, the frequency, duration, and intensity has diminished over time, but it takes very little for those barbs to lance me again and again.

I'll put it this way, when your kid's first (and last) word is, "NOOOOO!!!!" while you wrestle to shove a suction into her mouth, followed by a pain-dripping, "OWWWW..." you don't want to ever give up until something massively good has transpired in the wake of that horror.

Alright.  I'm in a pretty bummed out spot right now.  I can't tell if I was in a funk and then wrote, or wrote myself into a funk.  Either way, I'm going to share with you a quote from our interim pastor from his homily today.  His name is Rev. J. Dale Suggs, and man, he can put together a homily.

"When God looks at us, 
He sees the best of what was, 
the truth of what is, 
and the promise of what can be."

I'm going to practice this right now.

When I look back on Bella's BMT, I see an amazing, dedicated, pioneering treatment team that labored for months around the lock to save our little girl.  At one point, the level of care she was receiving could have only been given at maybe 2 or 3 other children's hospitals in the country, according to the Medical Director of the PICU.  When I look back on the amazing transformation of Bella's skin, and to know - thanks to her biopsies during her autopsy - that her throat was healing just as well, I know that through this treatment, Bella was growing her sister's skin from the inside out.

That is extraordinary.

When I look at the truth of what is, I see some kids doing remarkably well getting a new lease on life through this treatment, while still others die from complications from the very same protocol.  I can only imagine how confounded this leaves Dr. Tolar, and every family approaching transplant.  It's like a roll of the dice.  Are you gonna be lucky, or unlucky?

When I look at the promise of what can be, I really can see a day ten years from now where Dr. Wes Miller is consoling another parent whose child has died of a rare incurable disease during a clinical trial.  He looks them in the eye and says, "You know, 10 years ago, there was no treatment for EB, but thanks to parents like you, we have various treatment options for that disease, and no child has to die from it again."  I can believe in that promise, because that's exactly what he said to me the day Bella died, except he cited Fanconi Anemia, or FA, another metabolic disease the U of M pioneered the first cure for... 12 years ago.

I believe.

Do you?

God night.