What Really Matters

3 days after we got back from Boston, we had a routine cardiology appointment with Dr. Nugent, and we were ready to discuss everything we learned while we were there.  To say the appointment didn't go well is a gigantic understatement.  We love Dr. Nugent...like he's part of our family love him.  But we showed up on the wrong end of the ice storm....his 2 weeks in the ICU...one of his patients took off to Boston for a 2nd opinion...and who knows what else we didn't even know about?  BAD....as in he used phrases like "ending up with a dead 3-year old" and I remember crying for most of the appointment.  We walked out of the hospital in shock, feeling very much like our support system from the past 2 years had evaporated.  And I know what you're thinking...what did you expect when you decided to get the 2nd opinion?  This business of raising a critically/chronically ill child is not about making friends with your doctors or loyalty to a medical facility...even if they've given you the most precious gift anyone can imagine--more time or any time with your child.  It's about making sure your child always has access to the best care possible, and the latest information/technology/surgical techniques/etc regarding his/her medical condition(s).  In Kaleb's case, we need a hospital that is most experienced in Heterotaxy Syndrome, an amazing cardio-thoracic surgeon, and a fantastic cardiac-specific ICU.  Although we're having a really hard time with it, those are the ONLY things that matter in this decision.

A week after the disastrous cardiology appointment, we met with Dr. Forbess to go over the results of the MRI/3-D echo from Boston and get his opinion on the best way to proceed with Kaleb's care.  Though he chose not to review any of the new scans/reports, he spent a lot of time with us, and was mostly very kind in his approach.  We are especially appreciative of his time because there was an emergency surgery in progress, and he was waiting for a page to head to the OR.  Although we hear differently from Nugent all the time, Dr. Forbess' approach to Kaleb's surgical future is very conservative.  He not only recommended the Fontan(single-ventricle option), he went as far as to say he sees a heart transplant in Kaleb's future no matter what surgical approach we take.  He spent a great deal of time reminding us how far Kaleb's come, and seemed to suggest that we should just be grateful he's still alive and is a Fontan candidate.  Believe me...we are incredibly grateful on both counts.  But at what point in your child's medical journey do you just say....okay, let's just "cut our losses" and ignore the possibility that there could be another way?  He was very careful to say a 2-ventricle repair was possible...just not, in his opinion, the best answer for Kaleb.  He gave us a much-too-rosy picture of life post-Fontan, and even seemed to dismiss the idea of liver complications due to the single-ventricle circulation.  He encouraged us to meet with an adult cardiologist, like his wife, who treats adult Fontans to see how well they're doing.  It sounded good.  It didn't feel right.  We were also very concerned that Dallas wants to wait until May or June to do the surgery.  Another 4 months with oxygen saturation in the mid-70s at rest(mid-60s during activity), activity level way down, and vomiting way up.  We left the meeting wanting very much to stay in Dallas, but just couldn't shake the uneasy feeling of that reality.

The following week, probably the 3rd week of January, we finally got to speak with Dr. Del Nido, the surgeon from Boston whose opinion we had waited so long to hear.  He sounded COMPLETELY different than he looks(like he's in his 20s and he's not!), was incredibly kind, and spent a bit more than an hour on the phone with us.  It absolutely wasn't the same as getting to see him in person, but we are incredibly grateful that he spent the time making sure that (at that time) we completely understood our options for Kaleb and his opinion about the surgery.  He started by telling us he believes Kaleb should have the bi-ventricular(2 ventricle) repair, and he detailed every step he would have to take surgically to accomplish this.  It's such an enormous task, it's hard to imagine it can all be completed in one surgery.  He'd be creating a septum(the wall down the middle of the heart), separating a functioning valve into 2, re-routing major areas of blood flow, creating anatomy Kaleb wasn't born with from cadaver tissue,and taking down surgical work that's already been done.  In the end, Kaleb would have a beautifully unique, 4-chamber, pumping heart, and oxygen saturation of 100%. 

It sounds incredible, and it is.  They are doing this so successfully in Boston, they recently appointed a director to the newly created Complex Bi-ventricular Repair Program.  But Kaleb is much more complex than most of the kids Boston repairs through that program.  By his own admission, Dr Del Nido only sees 6-8 kids anywhere nearly as complex as Kaleb in a year.  This will be a long surgery, with about 2 hours(minimum) on the bypass machine(with his heart stopped).  Kaleb has Heterotaxy Syndrome, which makes the way he responds to his new circulation/repaired heart entirely unpredictable.  The valves that he essentially creates from the one Kaleb has now might leak, and eventually have to be repaired/replaced.  The nerve pathway inside the heart that controls conductivity(the part that keeps your heart beating regularly) could be irreparably harmed, and a pacemaker might be necessary.  I could go on, but I know you get the idea.  It's a terrifyingly huge open-heart surgery, and the risks are many.  And if you're going to let anyone perform it, you don't want your child to be on anyone else's table but Pedro del Nido.  But when I really think about everything that has to go perfectly to achieve success, I can't for the life of me figure out how to consent to the attempt. 

That was several weeks ago, and we have seen Dr. Nugent for 2 Cardio appointments, a coffee, and a Mended Little Hearts event since then.  Thankfully, we have recovered from the December appointment, and we truly feel that he is with us and being as helpful as possible.  We have spoken to Dr. Marx(the Boston cardiologist) at least once more, sent follow-up questions to Dr. Del Nido that were answered, and spent countless hours discussing information that we sometimes feel seriously under-qualified to process.  I have also had the help of some very brave heart moms, that I'm so proud to call new friends and supporters.  And, of course, we have the most supportive families anyone could ask for.  This is, by far, the hardest decision we've ever had to make....and multiple times a day, I change my mind.

We're going to Boston next Thursday, February 27th. Kaleb has a heart cath March 3rd and surgery March 5th, with Dr. Del Nido.  We're not entirely sure about the surgery Kaleb will have until the Cath confirms that he's a better candidate for one or the other.  I've begun praying not for a specific surgery, but for the BEST SURGERY FOR KALEB...and I know God already knows which one that is.  Please pray with us, send us positive thoughts and energy, and continue loving our boy.  We need it all now more than ever.  

One day, one hour, one minute at a time,

Jenifer

How far we've come:

This picture was taken by my mom on the morning we prepared to say good-bye to Kaleb.  It was 24 hours after he was born, and we were just minutes from meeting Dr. Nugent and hearing about the possibility of Kaleb's miraculous Cath intervention.

April 2012-Kaleb's first open-heart surgery

They told us to prepare for the worst, but God had bigger plans.

August 2012-Kaleb's 2nd open-heart surgery The Glenn

Discharged just before his 1st birthday

 

Sept 2012-LADDs to fix mal-rotated intestines and G-tube acquired

 

Being at home for several months agreed with me!

GI defects discovered, tube converted to GJ, and many nights at Children's

We made the most of that summer-swim lessons, family, and fun!

1st flight to Grandpa & Nana's-so much fun!

1st time in Grandpa's pool playing with his cousins-so much fun!

Flight home straight to Children's-not so much fun...

Beach trip 1 month later(still can't believe we were that brave so soon after his transport!)

Pure JOY!!

Our MIRACLE boy turned 2!!

5 weeks at Baylor Inpatient Feeding Program starting to eat and making friends!

Super big ICE fun at Mimi & PawPaw's

The Boston Adventure begins!

Christmas morning at Mimi & PawPaw's was extra special this year!

Valentine's Day fun in the leaves