Workup Results, the First Issue, and the Hurler's Gang

At the Minnesota Science Museum
Sarah, Ethan and I met with Dr. Lund this morning for what's called the pre-transplant "exit interview."  This is where they walk through the results from all of the pre-transplant evaluations and chart the course of the admit, transplant preperatory regime, etc..

Basically, everything looks good, except for the one thing that doesn't look good.  And we're going to have to deal with that thing before we can move to transplant.

The CT scan showed that Ethan has two nodules in his lungs.  The nodules are "presumably infectious, possibly [granuloma]."  A granuloma forms when the body is unable to fight an infection, typically bacterial or a fungal.  When the body is unable to fight the infection, it forms a protective wall around the infection and just leaves it there.  The trouble is, you can't tell from the scan if the infectious agent is still active or not.

During a bone marrow transplant, when the body's immune system is wiped out, granulomas with active infectious agents are, so Dr. Lund tells us, 100% fatal.  

100%.  

Chew on that number for a while.  I know we did.

So, we've got to deal with it before transplant by finding out what the infection is and then killing it.  To find out what it is, they are going to perform chest surgery.  The surgeon will go into the lung with a scope--and I assume something to collect a sample.  They'll take a sample and culture it, which means put it in various growth media to see which ones it responds to and thus what it is.  Once they know what it is they'll put Ethan on a course of medication to wipe it out. 

In typical doctor fashion, Dr. Lund was sure that they could kill the infection, whatever it is.  It's just going to take time (could be four weeks or so if it's a fungus--they take a long time to culture), and some range of drugs.

After the surgery, Ethan will have a chest tube placed and will be inpatient for 2-3 days. And then he'll be outpatient on some course of drugs for a number of weeks, because they'll take a guess at what the granuloma is hiding and treat him for that. If we're lucky, once they figure out what it is, they will have guessed right and it will be dead and gone. 

To me, this just goes to show that there is no such thing as an uncomplicated bone marrow transplant.  BMT's are utterly complex and completely unnatural procedures where the body's natural protective response is hammered into submission through drugs and radiation so new DNA can be secured in place with a hot glue gun.  Each step brings its own range of potential complications and side effects.  Thereupon additional processes and medications are utilized to lessen those complications and side effects. And so on, and so on.  This will undoubtedly be the first of a number of complications.  Some may be major; hopefully most will be minor.

There are two pieces of good news.

To repeat myself, the first piece of good news is that Dr. Lund is sure, in typical doctor fashion, that they can kill whatever it is that's causing the granuloma.  

The second piece of good news is that Ethan is otherwise in very good shape.  Aside from the adrenal insufficiency, which seems manageable, all of the other departments said that everything looked fine.  His heart's in good shape, his vision and hearing are in reasonably good shape for a Hurler's kid, his neuropsych results were at least as good as they were over the summer (though it's hard to be precise, because he was uncooperative for some of the testing), his fine motor skills are on par with a normal 29 month old.  

Just as important as all of the medical opinions though, may be the fact that in our opinion, he's strong enough to get through this.  If the science works, Ethan's strong enough to come through the other side.
Big brother, little brother

In non-medical news.  Ethan is behaving more and more like a typical two year old.   He knows what he wants and what he wants to do, he doesn't mind throwing fits--though I won't say they rise to tantrum level--when he doesn't get his way and one of his favorite words is "mine".  But even with the terrible twos, he's still a happy, fun and smiling kid.  He loves to interact with folks, kids and adults alike, he likes to play games, he's got a great sense of humor,  And slowly, but surely, he's learning new words and communicating just a little bit better.

In house news, there are now three other Hurler's kids in residence, not including Ethan, and we met another one in clinic today.  You can't spit without hitting a kid with a rare disease.  One kid is post transplant and they've been here basically for a year dealing with complications.  Hopefully, they'll be able to go home soon!  The other two kids are 13 and 17 months old and are here for transplant.

I think we need to get leather jackets for the kids with a logo on the back!  Now what would a biker gang of Hurler's kids be called...



Comments

Anonymous said…
Thanks for the update. So sorry to hear about the granulomas, Todd, and the additional procedure Ethan will have to go through. As always, you are all in my thoughts, and I hope the infection is cleared quickly.

Barbara Thurlow
Anonymous said…
Everything is crossed for you guys and that they kill this infection quickly. So glad you've got to meet other Hurler patients. Wish we could be there to give you all a hug but you are constantly in our thoughts. Much love xx Teresa, Shane and Corey x
Anonymous said…
I hope the other Hurler's families can help you as I'm sure you will reach out to them and lend support too. Sobering news about this new glitch - continued prayers and good vibes going out to all of you! Hugs to the boys! Love, Marian
Anonymous said…
Thank God the doctors found the nodules!
Thanks for keeping us posted Todd.
We pray for Ethan and all of you everyday.
Love Mary Anne
Wendy B. said…
Hmmm...lots of news to digest......Granulomas....chest tubes...... daunting info at the very least. But you have a strong son, a fighter. And positive energy all headed your way. As for the biker gang....Hurlers Harleys??.....Hurlers Hurdlers???.......The Hawgs of Hurler??......Hells Hurlers?? It's late after a busy week for one and all.....may the dawn bring new hope and renewed strength to all of you. Nurse W.
Todd Waddell said…
Ohhh, "Hurler Hawgs" I like that.

I just have this great mental picture of a buncha Hurler's kids on Haeley's. Too funny!