Kaleb Prinz: March 2012

The results are in, Kaleb's first heart surgery will be installing a shunt (which will also fix the pulmonary branch obstruction) and connect the pulmonary veins to the heart (which will fix the pulmonary vein obstruction). No two ventricle repair, at this time.

Once Forbess reviewed the findings of the Cath it was very clear that a two ventricle repair was too risky at this time.

Good news is that Kaleb will be able to grow more before we make a decision on either a single or two ventricle repair. Fixing the obstruction in the veins will also help with the lung issues we're currently having.

Bad news is that we'll have to still be on safe at home program for a much longer time than expected. Being a shunt is high risk for infection. We'll have to have the Ladds done earlier which is not favored by Forbess.
And this may add an additional heart surgery.

It's every emotion you can think of and honestly there was a little lump in my throat. I had high hopes for the 2v repair. But honestly Kaleb needs to be a little bigger and there was just too much to do at one time. Real-estate is a big issue in Kalebs case for the 2v repair. Because his heart is on the opposite side of his body the aorta is in wrong place. Forbess will have to install a conduit "in" the heart (to connect aorta to correct ventricle), which takes up room.

There are so many positives to this when you really think about it. Forbess will do half the work so less time on heart lung bypass. His veins will be fixed allowing better recovery on next surgery. And he will be able to be much bigger to give us that chance of a 2v repair instead of a single ventricle. Biggest downside is the infection risk with the shunt. It's really no different than the stents we have, so quarantine status will remain for another 4-8 months. Yes, it could be that long.

We have a plan and no plan in this situation comes without risks and rewards, but it's nice to know what we are going to be doing. His surgery will
probably be in 3 weeks.......

Hi everybody! The last several weeks have flown by, and we have truly enjoyed having our precious Kaleb at home. It's hard to believe he is almost 7 months old, just about 14 pounds, and absolutely HUGE(from our perspective anyway!) Kaleb has been developing by leaps and bounds, as well. He sits up much better, with some assistance of course. He's taken solids-tolerated rice cereal...liked avocado, peaches, and carrots...hated sweet potato and applesauce. Who hates applesauce?! We recently introduced Mr. Juice bear and he will use it with breastmilk only. What can I say? He likes the good stuff! He has amazing directed hand movements! It's amazing to watch him reach for and grab whatever he wants...including his NG tube. In that respect, he has "earned a mitten" on more than one occasion. ;) There have been many walks in beautiful weather for Texas, lots of time spent entertaining us from the crack of the couch, and he even helped us plant the garden. He can put both feet in his mouth at the same time, talk more than his father and me, and his laugh is our most beautiful sound on Earth. Kaleb thrives at home, and we are beyond blessed to have had him there for almost 5 months (give or take a few hospital admissions in between.)

Of course, the time passing quickly means the time for his surgery has been fast approaching. Yesterday, we brought him to Children's Medical Center for the Cath Lab procedure that prepares him for his big heart surgery. The plan was discussed many times, and fairly well understood that Dr. Nugent would: go in, record and analyze pressures and measurements, and a surgical plan would be decided. For some time now, we have been excited that a "2-ventricle" repair is within reach. That means that at the end of Kaleb's heart surgery, Dr. Forbess (our heart surgeon) will have corrected his many defects to become a heart most like yours and mine. If you've looked at Karl's diagrams comparing a normal heart to Kaleb's current condition, this is nothing short of miraculous. With the pumping action of a 2-ventricle heart, Kaleb's life expectancy and quality of life are vastly improved. I won't go into more detail than necessary here, but understand this outcome is a miracle we never thought to pray for. Up to the point this was even mentioned(a little more than a month ago), his survival and quality of life was our entire concern.

So how did this amazing surgery even become an option for Kaleb? Back when he was born, Kaleb was so tiny and his heart defects so complicated, no one thought we could make it to 6 months and 13+ pounds without requiring surgical intervention. At the same time we've spent the past 5 months enjoying every moment with Kaleb, we've also been carefully observing his behavior, monitoring his oxygen saturation, doing everything possible to put weight on him, and taking on his unbelievable reflux. It's been an incredible battle, and as Dr. Nugent says, "we've already won" getting this far. At almost any point before this, the only surgery with a good chance for survival was the single ventricle path that Karl has already described in an earlier post. We were prepared for a single ventricle option, and we were blessed to have it. But a 2 ventricle heart means our baby RUNS without tiring, throws a terrible two size tantrum without turning blue, gets the tube out of his nose and enjoys food for maybe the first time, plays sports if he wants to(and mommy lets him!), and lives a life Karl and I could only dream for him since I was 19 weeks pregnant.

Of course, our top priority is what is best for Kaleb, whether that involves a 2-ventricle repair or a single. Though our first option for surgery is the 2-ventricle repair, if something goes wrong, we need a back-up plan. The single-ventricle path was the back-up plan we hoped to confirm with the measurement and pressure results from yesterday's Cath procedure. From the very beginning, nothing has been according to plan where Kaleb is concerned, and this procedure was no different. The good news gleaned from the Cath is that almost everything the doctors believed about his condition is as they thought-with just a couple of notable exceptions. First of all, the 2 SVCs that Kaleb has are actually connected in a way that one of them can be removed and he will be just fine. Amazing, surprising, and great news for a 2-ventricle repair! Secondly, and the not-so-good news is that his pulmonary pressures are WAY too high for any chance of survival for either of the single ventricle stage surgeries. This is incredibly unfortunate because it leaves us without a back-up plan. I compare this to the idea of jumping out of an airplane knowing full well you have no reserve chute. No sane person would do it, and certainly never allow it for your child.

What does this mean? Probably much more than we know right now, but it absolutely means our doctors are furiously working on a new back-up plan. It also means that his huge surgery will take place in the next couple of weeks, not months. There is nothing but risk associated with waiting at this point. There is also a conference being held as I type, between the best minds of the Cardiac Unit of this hospital, and the goal is to solve this issue. Karl and I are understandably hopeful, as well as, terrified for the outcome of this meeting. Please pray that these incredible doctors are able to design a surgical option that will have a favorable outcome in the event we need it. Please also continue to pray that our little man improves and is able to go home with us before his surgery. As I posted on Facebook earlier, he is having a hard time keeping his oxygen levels up and we have been unable to take the breathing tube out. To further complicate things, he is running a reasonably high fever, so they've run several tests to make sure he hasn't acquired an infection. Hopefully, we will have good news by tonight and will be able to post a follow-up to this post quickly. Enjoy the pictures of our sweet baby K!
One day, one hour, one minute at a time,
Jenifer