Better day

After this weeks angst filled days - fretting about M's heart function I worriedly put on his pulse ox - something I haven't done in a while. Check out that 91! Incredible. That is awesome for Macsen and that heart rate is unbelievable  - it never drops below 125 when he's awake. 

His 02 drops with activity - 80 is acceptable though and his heart rate jumps. He was climbing/sliding so still pretty good I think. 

Giving thanks today for this gift of reassurance.

Go!

Macsen is blowing me away with his talking! After 2 years with barely a "Mama" and lots of "uh uh uh" we are now getting "Go!" and "Neigh" and he's attempting more sounds all the time. I think it is due to starting the fish oil regime recommended here  (this is the dosage we are using)and using the Kaufman techniques. Reading up on the same website signs of Apraxia and it is very probable Macsen has it. He has so many of the symptoms but is so young it is hard to officially diagnose him. So happy we are finally able to see some progress though.

I did talk to his cardiologist Tuesday and while it was great talking to her - she really is very kind and a wonderful person in addition to being a brilliant doctor - I feel so unsettled now, so anxious. I knew everything wasn't rosy during his testing in November - his pressures are barely passable at 14 (optimal is 10 or less) and the squeeze/ejection fraction are pretty bad. But I still felt like his surgery was pretty much guaranteed to happen this summer and we started gearing up for that - mentally preparing, arranging our life around "huge surgery in 6 months." Talking to Chitra this week has made me feel it is all up in the air. Some of it is due to his function and uncertainty that the enalipril will help that enough to safely add more pressure to his heart, something the 3rd surgery will do Some of it is from the discovery that Macsen has more pronounced lymph vessels in his neck. Lymph is something heart centers are just starting to  research in relation to the complications post-fontan kids are experiencing. If Macsen is already experiencing issues - he can not seem to clear the lymph efficiently and it gathers around his face making him seem chubby - then another surgery may send him in a downward spiral of complications. But they can't say whether it will or not - it's all a guess. So the new plan is we don't really have a plan - something that makes me feel like I want to vomit and my chest will explode. I hate it. I need a plan. The best she could give me is we do another cardiac MRI and cath this summer - if his numbers have improved and his function looks better then we would plan the Fontan in the following weeks. If they aren't improved I'm not really sure where we go from there? I am ok postponing that 3rd surgery for another 12 months if he seems like he is still growing and his oxygen levels stay above 80. But they seem to be dropping, especially on his feet and he is losing weight and barely growing.
<<<Caution - extreme venting and angst  beyond this point. Sorry. Sometimes it comes to that>> 

To be honest, as terrified as I am at the idea of another surgery I am crushingly disappointed I can't count on it being over by the end of the summer. I was hanging all my hopes on "Post Fontan living " - a magical land where Macsen could be satting in the 90's,where he would want to eat and he would finally grow because his organs and tissues would be getting the oxygen they need. Where we could plan family vacations without feeling like we are using up money and vacation days from Aaron's job - days we may need for extended hospital stays. A place where we wouldn't have to have fundraisers for ourselves anymore! A place where I can sign the girls up for sports and extracurricular activities because we won't have so many out of town dr appointments. A place where I'm not spending 2-4 hours everyday pumping milk- eating foods and drinking drinks I don't like, desperate to keep my supply up.  And even though there is a possibility we cath this summer, his heart function improves and we schedule his surgery a few weeks later I just can't focus on that right now... it's like I can't see it. All I can see is how hard everything is, how impossible this life feels sometimes, how far removed I feel from everyone who are living "normal" lives. I want to be on the other side of all of this - and I know it will never be over but this part will. This "pre-fontan" time. And I know there are no guarantees that post fontan time will be like I am hoping. We know some families that went home 6 days after surgery with a pink child ready to take on the world and we know  many families who are having a really rough time after fontan and wish they could go back to the glen days. It's so hard to know what will happen - true for anyone I know but I have seen the face of it, the months of rehab after a stroke from surgery, the middle of the night life flights because the child has a cast stuck in his lungs and can't breathe, the listing for a heart transplant. And worse. So do I pray he is well enough for the surgery this summer or do I pray for another year with no surgery and a relatively stable Macsen? I know my Christian friends would say"God has got this. He has a plan. Give it all to Him." That is so very, very hard.  

The first rule of baby fight club

Finally got a few more results from Macsen's November Cath and mri. He does have "pronounced" lymphatics around his neck - what this means for his next surgery I am not sure. I would like to talk to a lymph expert - either Dr.Dori at CHOP or Dr.Fishman @ Boston Childrens. My main worry is post Fontan complications like plastic bronchitis or protein losing entropy. Both incurable and both complications from lymph + post fontan circulation. Macsens CHOP card is calling us on Monday to discuss it. 

He had also started losing weight. He is down to 24 # from almost 26 :( I have been trying to get in touch with his GI for weeks when it became evident he was no longer gaining. His poop also looks weird which worries me. His Gi and I keep missing each other and Atlanta childrens  is a pain - I can never just call a direct number and leave a message for the dr. It's all through answering services

In happy news he is talking more - he says "wawa, Nonna, buhbuh (bubbles)" You have to give him the simple version and prompt him to use his mouth to make the sounds but when he's done it he raises both hands in the air and gives a huge smile. 

He got his first black eye this week - in the tub though Aaron likes to say "You should see the other baby." And "the first rule of baby fight club is you never  talk about baby fight club." Love that man . 

2015 is here!

Happy New Year everyone. So far so good. Really (knock on wood - don't be getting any ideas 2015! We had a wonderful Christmas here at home with the most beautiful tree ever, happy (healthy) kiddos and my mom came to visit. She taught the girls chess and now they ask to play almost every day. Santa brought both of them the 2 gifts they really wanted - a mermaid tail and a robot dog. And Macsen got cars, cars and more cars. They were all very happy. Aaron was able to pick up some shifts at work and wasn't forced to use a lot of vacation to cover unpaid lay off days - he is hording his vacation days for Macsen's upcoming surgery this summer.
I have spent all day on the phone with different medical equipment suppliers, lactation consultants from all over, and our insurance company. A few friends suggested I ask about insurance coverage for a new pump and it turns out they will cover buying a new electric pump. Woot! I would ultimately like to rent a medical grade pump that is much better - I use the one at CHOP whenever we are inpatient or have appointments and my supply always goes up. Insurance doesn't cover all the cost of renting one of those though so we have to weigh the pros and cons. I had to research what medical diagnosis necessitate insurance paying for 80% of the rental - insurance company wouldn't tell me of course. I had to go through all Macsen's paperwork citing every code from every specialists\ until I gave them the magic one. Surprisingly HLHS wouldn't work but 783.3 - feeding difficulty would. Nuts. In the meantime a friend gave me her old pump so I wouldn't be without. Never in a million years would I have thought I would be looking to buy a new pump this late in the game but hey - we do what we must.
Macsen seems to be doing well. He's still slowly gaining - almost 26# now and not sure of height but I did have to put up several pairs of pants that are now too short. He is babbling more every day. He now has very clear signs for his sisters and asks for them when they are at school. He says "wawa" in addition to his "mama" and "uhoh." We are working specifically on vowel sounds with his speech therapists and it seems to be clicking better for him. He is running better - not falling as much. He is taking more solid food - infinitesimally more most days but to the trained eye - the desperate eye - it is definitely more. He makes all the animal sounds in his favorite books. If you stop and restart a movie he gets mad if you don't get it in the exact spot it was when it turned off - even if it has been an hour since it was turned off. And he is consistently pooping on the big potty. Not bad for barely 2 :) We are so proud of him.
We are STILL waiting to hear results of the lymphatic portion of his MRI from November - crazy to think we are going on 2 months of waiting. I also had to request all the records from that visit bc we have received nothing cardiac wise - just a letter from his neuro. There is an electronic site you can access to view test results but it has surprisingly little information on it. It's a shame because it really could be very helpful if I could see everything the office has access to - all MY son's medical information. Wasted opportunity and it would make my life easier.
I am struggling to find the motivation/time to work out. All this time at home doesn't help if it's with a tiny cling-on attached to me. 3 surefire ways to make Macsen stop playing sweetly and independently = 1)Try to have an important phone conversation. 2)Try to clean the house. Or 3)Try to put in a P90x video and start jumping and kicking wildly around the room. He comes like a shot "hold me, hold me." followed by tears and lately a full blown lying on the floor, arms waving, feet kicking, face purpling tantrum. He has also perfected the art of making me feel guilty - he drops his head and shoulders and sighs or when he really wants to pour it on he pokes his lip out, makes it quiver and silently sobs. The girls and I are complete suckers and give in everytime. We love that little stinker.