Archive for November, 2014

Two By Four and Transplant

Waiting for a very important page from Catherine for his thickener

Waiting for a very important page from Catherine for his thickener

We are home! Our flight landed in AZ at 10pm MST, 12 EST… our body time last Wednesday. Bronson loves it here. He is thriving since being back. His weight remains stable, along with his oxygen saturations, and his appetite has improved. When Dr. Blume first met with us, Bronson was “Failure to Thrive” and dying, essentially. He’d lost 8lbs, was in respiratory distress for the 7th time, and IV diuretic/ oxygen dependent.  His life was on a “tight rope”. Her goal was to progress him to a “two by four” and then finally to a platform. Her team indeed saved his life by restoring his nutrition via continuous calories through his newly placed feeding tube, calculating a reasonable fluid restriction, and successfully transitioning him from IV diuretics to oral which then led to weaning oxygen. From our perspective, he’s for sure on that “two by four”, figuratively and well, literally too. He has two ventricles and four chambers. 😉 He’s not quite a platform though. We were discharged from a four month hospital stay with about an 28oz fluid restriction in a 24 hour period with fierce diuretics and potassium supplements. Platform status would only be achieved with free flowing fluids and less diuretics. With time, that could be a reality for Bronson. We’re not counting on it though. Just simply hoping … and praying too. We continue to submit to the Lord’s will, just like we did when this “whole heart” option was presented to us. Currently, it could be the Lord’s will for Bronson to have a new heart entirely. The probability of transplant was apparent to me when I became connected with a group of moms/dad’s on Facebook who had already journeyed the Bi Ventricular repair with their child. I learned that the repair could fail even risking loosing transplant as an option. For those that remember (Bronson’s groupies 😀 ) I posted an update detailing our emotions from our risk/benefit analysis. Here’s that post for those that missed it and those that want the recap: http://bronsonbeckman.com/wp-admin/post.php?post=962&action=edit

During our time in the hospital when Bronson was sick I couldn’t look at pictures of him healthy. Now it’s a task to look at pics from when he was sick. There’s benefit in living in the moment. It helped me to be mentally impaired to the past, avoiding looking back. Currently, the moment that I’m in is gratitude. I am thankful (along with Eric) for the friendships we molded and the “family” we acquired from our “whole heart” adventure we began in Boston. The one that we are going to continue in Palo Alto/San Francisco CA at Lucille Packard Children’s Hospital aka “Stanford”. I had always known that if transplant became our reality that I’d want to ride that coaster close to home! Stanford is about a two hour flight from AZ and an 11-12 hour drive. For Bronson, the transplant process could be a three year venture. He had 8 blood transfusions after his bi ventricular repair. Multiple transfusions lead to high antibodies in his blood which means any heart he receives would be rejected by his body. An aggressive plan to neutralize those antibodies could involve a multitude of IV anti rejection therapies over a course of three years, along with intense monitoring for rejection via multiple heart catheters. We would be required to stay close to the hospital before and after surgery. A 2 hour flight versus a 6 hour flight to Boston or to even have the option to drive makes CA the most practical place to be. It’s also reassuring to know that the hospital has successfully recruited several of top talent from Boston! We’ve been referring to it as “Boston West”.  We agreed to complete the full evaluation with Dr. Blume’s team when the results from the last heart catheter revealed that we would be need to prepare for this new journey.  This four chamber heart is pumping with such high pressures that it’s creating potentially dangerous resistance against his lungs. That high resistance, along with his elevated anti bodies, had Dr. Blume concerned that other centers would deem Bronson too high risk and decline him for transplant.  We completed a full transplant evaluation so she could have data to send.  The team at Stanford already confirmed their confidence with transplanting him successfully. They still require their own data though which is why we need to complete their protocols for evaluation too. We have a tentative date for Dec 12 that will be confirmed today. Meanwhile though, he is on a prescribed .25 Liter of oxygen  at night to relieve that resistance temporarily while he sleeps. It essentially gives his heart and lungs a break. There is still a very dismal potential for this heart to succeed. We’ll know in three months after the first heart cath in Stanford to re measure the resistance from his lungs. If it is reduced then we MAY detour the transplant train and continue to monitor him closely with frequent catheter’s. Based on the first appointment with his AZ cardiologist yesterday, the hope that this heart is not “completely toast” does have some glimmer. His average heart rate has come down from about 125-135 when we left Boston to now it’s about 121-135. His red blood cells continue to reproduce at a rate high enough to compensate for the ones breaking through his valve and his O2 saturations increased to 94-98. Each of these are clinical markers of some improvement. Still, until his function is on a “platform” and providing a desired quality of life, one where he can drink leisurely (juice, water, and milk) preferably not alchohol … leisurely ;-),  we’re mentally and emotionally prepared for a new heart. I know I can’t express enough humility for everyone who has unselfishly and generously offered unconditional support financially, emotionally, and mentally. I honestly feel like “thank you” is too simple to encompass our appreciation. For now, it’s all we have though. We do have pictures to share!

 

November!!!!

IMG_20141031_181918_149Here we are, November 1st as I’m typing.  We’re still moving along day by day and finding some joy in the little accomplishments.  Bronson’s weight has been about the same over the past couple weeks (13.2 to 13.5 kilos) with little moves up or down recorded every day.  The climbs bring less satisfaction when the falls bring frustration. Over all it’s ok as the team isn’t too concerned. He’s it least not loosing big chunks of weight, just little bits that he picks right back up, so overall stable.  The greatest excitement is that he has been breathing without O2 and on room air only, (no oxygen tank to drag around)! He’s also detached from Milrinone (the continuous IV heart therapy). With both of these methods of support being gone, his energy levels have remained constant.  He has been all over the place since being “UNHOOKED”. He’s been outside more to the garden, to the play rooms, learning to ride a bike (well technically it’s a trike but just getting the pedals going has been great to see), and the biggest one is going to the playground! It was where we celebrated being detached from his IV tree.  Quite a while ago, when he was almost at his lowest sick point,  Tabatha took him down there and after climbing on one platform he said that it hurt and since then he’d refuse to go back.  These last two trips have been completely different.  He has been climbing steps, sliding down slides, playing with other kids….a lot like his regular old self.  He is still pretty weak as far as his endurance goes, but that’s improving every day with the help of Pysical Therapy and some spiffy new leg braces with construction vehicles on them. The Rehab supervisor observed that his leg muscles are so deconditioned from too much time in bed, that he was using his hips and his lower back to compensate which was making him wear out faster than he should be. Basically, he was doing a full body workout just to walk. Now he wears the braces to walk any distance aside from just hanging out in the room.  Since wearing them, he’s been walking significantly farther and for increased periods of time. We were told to expect about 6 months to see complete improvement.

As far as technical stuff; he got his 6th or 7th (we lost count) blood transfusion about a week ago. Within in the same timeframe that he got his feeding tube, along with starting the IV heart therapy (Milirinone), the team tested for a condition in his blood called Hemolysis.  His red blood cells are breaking at a faster rate than his body is able to produce more to maintain a high enough red blood count. The cause is uncertain, with speculation though that his red cells are breaking as they pass through a “paravalvular leak” in his Melody Valve in the Mitral position. A “paravalvular leak” is a gap between the circumference of the valve and the ventricle wall. The team has just been monitoring the rate that his blood is breaking and using that to determine the timing of his transfusions before he’s anemic, until there is more data to support what the next step will be. He has a Heart Catheterization that is set up for this Wed the 5th to gather that data. They will be going in with a catheter through his groin and his jugular to check his heart pressures. There is a remote possibility that they could do something then, regarding the leak around the valve. So overall The Cath will tell us what the next course of action will be. We will update everyone later in the week with that news. Aside from the Hemolysis being the greatest complication we’ve been dealing with, he continues to heal, grow, and get stronger. He’s still getting continuous formula into his g-tube 14 hours a day to the tune of about 1000 calories a day which is calculated to maximize weight gain.  We are in the process of getting that peeled back. Tabatha with her super blender has been whipping up smoothies for him all along and has now cranked out an amazing concoction that we are transitioning him to.  She has done such a great job growing him througout his life by making really good, high quality, high calorie foods for him. This is no different, except for the method of delivery is via the tube.  The formula that B has been receiving served it’s purpose but has now outlived its benefits. He pukes every morning after being having it pumped into him through the night and also once during the day from the intense reflux it has created in his GI system. We are very tired of it. As long as he continues to require the feeding tube to grow, he’ll continue to have a very tight oral fluid restriction. Currently he gets most of his fluid from the formula through his tube which leaves about 6 oz of “honey thick” fluids that he can drink in a day. He is still aspirating. That was confirmed to have improved a little though with a repeat Barrium Swallow Study a few weeks ago. We’ll take ANY improvement!

On a lighter note, Bronson experienced his first Trick or Treating in the hospital. Tabatha found a costume set for kids from 3 to 8, that had 3 super heroes in it; Superman, Batman, and Robin, thinking that B could choose one and the other two might be donated to other kids in the hospital.  As it turned out, Bronson wanted to wear the mask (so Batman) and that left us wearing the other two costumes. Super dad and Robin mom.  Nurses and Doctors all over the hospital dressed up and several of the floors had trick or treat stations set up.  We made the trek to two other floors and then about half of our floor, before his little legs were just too tired to keep going.  I just carried him the rest of the way back to our room.  This being only his second memorable Halloween, we couldn’t figure out at first why he kept roaring at everyone. After about a half hour in, it finally dawned on me.  Last year we went to Tabatha’s brother Mike’s house for the trick or treat evening. That evening was full of treats and tricks… LOUD ROARING tricks! Our friend Mark dressed up as a shrub and kept jumping out and scaring kids when they approached the candy bowl while we all laughed hysterically. Bronson caught on to the fun and started hiding in the bushes so he could jump out and join in the roaring. Apparently he brought the tradition all the way here to Boston Childrens Hospital!! Uncle Mike and Mark would be PROUD! We’re anxious to see what “Tricks” the three of them carry out together next year.

We continue to be abundantly blessed with all of your donations to Bronson’s Go Fund Me http://www.gofundme.com/d9lnyc account. The care packages are still coming and we are forever grateful for each of your thoughtfulness with the items you’re sending. Along with each of your support and encouragement via; text, fb, emails, calls, letters, cards, we are also humbled by the genuine care, concern, and total treatment by the entire staff here. And I mean a wide range of staff from the head chef of the cafeteria and manager of room food service to the cleaning crew to the team of docs, nurses, and therapists (music, art, pt, ot, pet, etc ) who are sincerely joyous with every improvement that they see Bronson make. He’s recently earned the title, “Mayor Bronson”, because he walks through the halls waving at everyone! The people here not only make this experience tolerable, they often make it enjoyable… relatively of course. Until the next major announcement, hopefully by the end of this week after his cath on Wed, enjoy the pics!