Snow Day 2014

No go from Boston Childrens

Talked to Dr.Emani and unfortunately, but not surprisingly, Macsen is not a candidate for the bi-ventricle repair - mainly because he has no mitral valve to work with which is a big part of rehabilitating that undeveloped left ventricle. Sigh. And promising research they had to build a new valve apparently has not panned out and they are back to square one.  I was worried after coming across this http://www.annalsthoracicsurgery.org/article/PIIS000349751300372X/abstract that states "Management of unbalanced common atrioventricular canal (UCAVC) defect by a single-ventricle (SV) approach frequently results in poor outcomes." Macsen has unbalanced av canal in addition to HLHS and the surgery track he is on, known as Fontan circulation, is a single ventricle approach - meaning despite undergoing 3 open heart surgeries, he will still only have 1 working ventricle and according to this research will result in "poor outcomes." :( After reading this research, I kind of freaked - other than transplant which is so very tough to get for someone so tiny, is not guarenteed he could even get a heart, and opens him up for a whole host of new problems the Fontan is the only option for Macsen. But after talking to Dr.Emani, who participated in this research, he feels Macsen will be/should be ok as opposed to other children in the study, because to date M's tricuspid valve is still holding strong. His survival seems to repeatedly come down to that valve - the only one he has. Bright side?  It was good to hear after studying M's recent echo he felt Macsen's heart looked really good considering his defects and he wouldn't change his treatment course. To read an interview w/ Dr.Emani about the single ventricle recruitment program  http://vectorblog.org/2012/11/building-a-whole-heart-for-children-born-with-half/
So we are on track to visit CHOP in 2 months to get a full work up and start talking dates for his next open heart surgery. It will be an exhausting trip with visits with many specialists spread over several days but I look forward to seeing his cardiologist up there and the people that have been helping him since birth. We usually go visit the special delivery unit as well to show him off. It's great to see the midwife that delivered him - babies born in the SDU are always critical but the staff do their best to make the experience of childbirth as normal and wonderful as possible and I love to show off our little success story.
Macsen is doing well. Cruising more on the furniture and working on his balance. Picking up cute little tricks every day - his new one is "the cuddle" - he lays his head on your shoulder and sinks into your body - sometimes adding a little pat, pat, pat for added heart melting affect. He can now scoot forward and backwards on his little ride-on, can crawl on all fours instead of the army crawl, and has started to eat a tiny bit more. He has started putting more food in his mouth and although he mainly sucks it or chews it to a pulp then spits it out he MUST be getting some nutrients right? Excitingly, 2 days ago he ate 3/4 of a baby food jar of bananas and mangos! We cheered for every bite and he would wait until he had his Dad's attention before opening up for the next bite. You know how families bronze baby shoes? Well, I am so proud I think I could bronze that Earth's Best jar of baby food - it sits proudly empty on the kitchen table to remind me he CAN do this! We just have to keep at it. We have also found a solution to my drop in breast milk - still so important as it is 99 % of his daily intake. That solution is DONOR MILK - provided by my best friend no less who happens to have a little one 1 month younger than M. She eats organic and I know her whole medical history so feel very comfortable - and so very grateful- giving M her liquid gold.
I also feel I should announce - for the first time ever Aaron and I BOTH left M with a sitter so we could do something together. She is one of our best friends and the kids love her and we were only gone 2 hours and were less than 5 miles away BUT it was a good first step - deep breath! He had a good time and only fussed when he saw us walk in. Aaron and I had an awesome time at Body Combat though I am still sore 4 days later :)

Sucking Bacon Essence or showing off his Bacon tongue

Signing time

After months and months of using sign language when we think Macsen wants "more" or "milk" today he signed that he needed "help!" Awesome! Silly boy had a straw and was pushing it though the rake his Daddy had in the kitchen (the company mailed Aaron a new one when our other one broke after only a season so it is sitting , pristine, in our kitchen. Like the lamp from A Christmas Story.) The straw was stuck on the other side and he needed help to get it back. He still "unnnnnhhhs" a lot when he wants something or needs something but it's a start. The only word he says now is "hot"and he uses it correctly so I know he can speak. I honestly think he is just storing everything up until he feels confident he can come out with whole phrases. 

We have had 2 deaths in the heart community that I follow - I am sure there were many more that aren't in my facebook groups. One was Tin Man Flynn Holden http://tinmanflynn.blogspot.com/who was waiting for a heart. His father was always so upbeat and hilarious in his postings it was a joy to read his updates even when you knew times were tough - he always put such a brave and funny spin on what they were going through. He was a very tough little man and reading he had passed suddenly - on day 65 on the transplant list.... my heart broke for his family. Another was Brian. Both children I followed while I was pregnant or right after Macsen was born. 1/100 is born with a heart defect. There are so many children, so many families fighting to love their babies another day. It is so hard when one has just had enough and their little bodies give out. Iam praying for peace and strength for them in these impossible times.
Macsen's records have arrived at Boston and we have gotten an email that the surgeon, Dr Emani, is reviewing his echo. I don't forsee any drastic changes in the course of his treatment. I expect they will also recommend he move forward with the fontan, his 3rd open heart surgery,  in 6 months - 1 yr.  But just in case they see something different, something better... I had to ask. This 3rd surgery still leaves him with half a heart - it will off load the rest of the de-oxygenated blood from his lower body from his heart to be moved via a passive route to his lungs to get oxygen. Not a perfect system, not a cure, and a process that puts a lot of pressure on his liver and kidneys when the blood backs up. Still praying for a breakthrough in stem cells, in 3-d organ printing, in converting the ghost scaffolding of a pigs heart covered in his own stem cells into his very own new, perfect heart. And praying for him to grow. He hasn't in several months - not height or weight. Still stuck at 27 inches and 18 + lbs. By the next cardiology visit in March if he hasn't grown again we will have to see Endocrinology. I hope he grows. come on! Grow! I now add fractionated coconut oil (liquid) to his breastmilk but still no growth. And - despite the singing, dancing, and everything short of standing on my head - he is not actually eating very much. A few nibbles here and there. He loves to hold a croissant and take a tiny bite - but he always pushes it out with his tongue when it comes time to swallow - sigh. He did eat several bites of - all things- a coconut/salsa/peanut butter crock-pot dish. He loved the sauce as long as it was his daddy feeding him. And he'll drink anything from a cup as long as it's water :( No calories there but little dude LOVES water.
We have our dates for our next trip to CHOP set in March - just for a check up with his team up there, a developmental evaluation w/ CHOP's Neuro Cardiac Care program http://www.chop.edu/service/cardiac-center/our-services/neuro-cardiac-care.html, and follow up with an environmental study he is in. Should be a few days filled with tons of tests but hopefully we will get to see some of our family to help break it up. I hate to fly with Macsen - our last trip was a nightmare with every other person on the plane hacking up a lung in  what I imagined the last moments of their life battling TB or swine flu - all with a fragile baby who cannot get sick. I was ready to come home and get legislation passed that mandated every sick person on a plane be required to wear a mask at the very least. Or invent an instant, conveniently collapsible, GERM REPELLING BUBBLE. It might work :)
Excited to hear from Boston - even if it is just that we are on the right path and to stay the course. They are the #1 heart center in the US and to know their top surgeons are looking at M's heart is so reassuring. We love CHOP and have total faith in his care there but I would not feel I had done everything I could for M if I didn't pursue this tiny, and very unlikely, chance that he may be eligible for the bi-ventricle repair. 

Macsen playing "take the object out of my mouth"

I caught a little of M's favorite game on video - something he came up with all on his own. He puts things in his mouth and waits for me to come fish them out. I haven't been too worried about him swallowing anything - with his insane gag reflex as soon as anything gets near the drop zone he "huah"s and spits it out. Pureed fruits and veggies? Huah! Disintegrating baby puffs? Huah! Googly eye from his sister's craft project? Huah! Well, we have found his gateway solid - the solid that has bridged the gap between milk and eating real food and that solid is sequins. He can't get enough of them and like I said I wasn't too worried about him swallowing them because he never swallowed anything. But our little non-eater passed 2 in his poop in the past 24 hours! And just like that he seems to be tolerating other things - bits of croissant he rips off with his new tiny teeth, shredded mozzarella (no vomiting so hoping he is indeed growing out of his dairy intolerance - oh cheese! How I've missed you!) But his most favorite food he never wastes or spits out (besides sequins apparently) is chocolate. He will nibble little bits off until he has consumed a whole square! My goal tomorrow is take a pic of his smiling chocolate smeared face - his little pearly whites peeking out- it is the sweetest face you'll ever see :) Besides swallowing he has also gotten a few other skills I will need to capture on film. He now scoots on his bottom instead of army crawling - apparently army crawling is sooo 2013 and scooting is where it's at in 2014. And he says "Hot!" and smacks his hand down whenever he thinks something might be hot. AND he can now mostly lower himself off the couch or bed and he is so very proud of myself. The word "beams" hardly contains the happiness in his smile when he looks up at me. Gah, we love that little man!