Happy Holidays

Home!!

***I wrote this Dec 5 but it didn't upload

We made it home after Thanksgiving. The kids were thrilled to spend so much time with their cousins in Maryland. We were even able to celebrate K's birthday with them at a huge ice skating rink - so fun. On the way to Alabama they had a great time playing in all the snow at every pit stop in the mountains and were really very good for such a long drive.

Today we picked out a beautiful Christmas tree. While we were waiting for  Aaron to tie it to the top of the ban the kids and I went inside to see what decorations were left. Macsen fell in love with a train designed to ride around the tree. Lucky boy caught the eye of a manager who was just thinking he needed to clear the tracks to put up something else. He sold us the train for $5! Macsen was thrilled he got to carry the train out and I was thrilled I didn't have to make him put it back. I'll post pictures when we get everything up. 

Chugging along

We are still waiting to hear the results of Macsen's  cardiac MRI - waiting on test results is so awesom (said no one ever.) 

Macsen has kind of exploded lately though. According to my measuring system, which consists of sitting Macsen on the counter to see if his head touches the upper cabinets, he has grown half an inch. He is almost 26 lbs too! I am not sure if it is the appetite stimulant med he is on or the fortifier I am adding to his milk but something is working. He has been going through growing pains at night too - crying out and rubbing his legs. He wakes at least 5 times/night and I rub his legs or rock him. 

He is also trying to eat more real food though 99% of it still gets chewed up and spit out. Last night he chewed up 1/2 a meat ball and some rice noodles. This morning he swallowed about 1/2 a gogurt tube. Slow but progress. I still haven't turned in his paperwork for the intensive feeding clinic in Atlanta. I just don't know how we will swing that just yet with the girls in school and Aaron working. 

A company called Orgain that makes organic nutritional shakes mailed us 2 boxes of samples of their shakes - so generous- in the hopes that's Macsen would like them. 😔 Not looking good though he did take about 5 sips of the chocolate one. Vanilla was an immediate no way. 

Sigh. And after 2 years of running 4+ times per day I wore out my breast pump.  It started loosing suction so I opened it up and the inner membrane is popped. Not sure how that happened or if it just was its time to die but that stinks. Those things are not cheap and after 2 years I hate to buy a new one now. I have a really old one I am using now - grateful it is still running. 

Macsen - out!

Discharged!! Though the doc urged us to stay close to the hospital tonight. We have to keep an eye out for fevers, any grossness at his cath sites when we take the dressings off tomorrow. He has been very puffy from the sedation - couldn't even open his eyes he had so much fluid-and we are hoping a dose of Lasix will clear some of it up. He was also very very junky when they sedated him - they suctioned lots of gunk out of his lungs. Hopefully it is not a sign he is getting sick.

His cardiologist came to talk to us and she was reassuring. The good news is his heart numbers look better than they did before his last surgery. His tricuspid looks strong and the patch sewed on during surgery #2 is only leaking minimally His ejection fraction isn't good though - it measures how well his right ventricle squeezes- what percentage  of blood it is ejecting with each squeeze. A good number is anywhere from 50-70 so Macsen's EF of 30 is worrisome. His ventricle is stiff and just isn't working very well. So the plan is to try meds to help his heart squeeze better. And then a tentative date for surgery Summer 2015 if the meds seem to help him. They are still evaluating parts of the MRI. We hope to get more heart info and some information on his lymph accumulation. 

Tonight and for the next few days we hope to get lots of rest. None of us have slept well in 5 days and with the stress of everything we are just exhausted. Looking forward to the king size bed at my sister's house and seeing our girls.

Chiari?

Based on Macsen's spinal MRI today his neurologist DOES NOT think he has chiari malformation. The fluid around his brain is flowing freely and unobstructed. It is great news to know he will not need brain surgery - fantastic news. He also felt Macsen has all the precursors for speech - it's just going to take lots more work. Definitely doable. 

So great news so far

He is still getting his cath. Will update when we know something

Macsen's auntie G is here waiting with us. She was so great during his appointments/consultations yesterday bc she played with him so Aaron and I could direct 100% of our attention to the Dr. 

***Update at 11. Cath is going well. Another update at 12

Cath time

They just took him back. Not a second too soon - he was signing "water" and starting to get upset. They found an ENT that verified the tubes were blue plastic so M could get the MRI. Apparently older tubes were made of metal. Very annoying though because we had all his records sent - that piece of information wasn't anywhere to be found. I held him while they held a mask to his face - it was strawberry scented to mask the gross smell of the gas- and he was asleep in seconds. Then we had to leave him while they place his Ivs. It will be several hours before we get to see him again. I am praying all goes well - no reactions to sedation, good numbers in his cath and no complications.

In Philadelphia

Our drive up went very well. Kids watched lots of movies which helped a lot. 

Macsen did well with the neurologist - really impressed him with how smart he is. Macsen was really showing off - making all the sounds he refuses to do for his speech therapists. Pointing to all his body parts. Even playing jokes on the doctor. 

Now we are waiting on an ENT to look at Macsen's ear tubes - have to make sure they are plastic before he can get an MRI. It's been 7 hrs since he's eaten and he's going to freak out soon. 

Apraxia anyone?

Macsen's speech therapist threw out a possible reason Macsen doesn't talk yet. She said some of the mannerisms I have brought up are consistent with Apraxia.
"Childhood apraxia of speech (CAS) is a motor speech disorder. Children with CAS have problems saying sounds, syllables, and words. This is not because of muscle weakness or paralysis. The brain has problems planning to move the body parts (e.g., lips, jaw, tongue) needed for speech. The child knows what he or she wants to say, but his/her brain has difficulty coordinating the muscle movements necessary to say those words." (From the American Speech Language Hearing website.) 
I have been looking here http://www.apraxia-kids.org/  for more info as well as doing what I always do when we get a new diagnosis - consulting Dr.Google, reaching out to my heart community. I"ve already "met" several heart mom's whose little ones are also battling Apraxia. 

Sigh. I hope she's wrong and Macsen is just stubborn and any day now he will just magically start spouting out full sentences. But a lot of the symptoms fit. Very little babbling. No mimicking sounds. Eating issues. Normal/age appropriate understanding of verbal language but severe delays in oral communication. Today I told him we could go get Daddy if he would just say Dada - he tried but all that would come out was Ma Ma and he started to get upset, doing his sign for "Daddy" and pointing at the door.
He's a little young to be officially diagnosed but I will discuss it tomorrow with his other speech therapist. I will also talk to his neurologist about it next week. 
Treatment includes intensive speech therapy - 1 hr/day  3-5 days/week w/ a therapist experienced in apraxia. In severe cases some children need a communicating box that speaks for them. It is kind of rare so finding someone experienced is important. Also, I have been reading up on how the use of fish oil has been helpful for some children with Apraxia so I ordered the ones I found recommended. Hopefully his cardiologist won't say they're not good for heart kids - they do have blood thinning properties.
Macsen is also still coughing and snotty:(It has been 2 months now and even though his cough sounds ugly his pediatrician says his lungs sound clear.  I am doing everything I can think of to get him well for his appointments/procedures next week. We really need some prayers. I'm so afraid we'll get all the way to Philly, he'll get his pre-op xray and they'll tell us he has pneumonia or something and all procedures are cancelled. We need answers. We need these tests so we can know what is going on with his brain, his heart, if his heart looks good enough to be a candidate for the 3rd surgery and when that will be.
The fundraiser went well at Nantuckets coffee shop. We met some amazingly nice people - the majority complete strangers- and they were able to raise several hundred dollars for Macsen. He was such a little charmer the whole night - waving to everyone, blowing kisses to say "thank you" and signing please if he wanted something. He also doled out hugs and smiles everywhere. He was just perfect and his sisters were very sweet too. The delicious hot cocoa and brownies helped, as did the products some of the vendors "sold" them for a quarter or a dollar - thank you Kristina from Perfectly Posh, Alex from Uppercase living, Angela from ThirtyOne, Nantuckets and everyone else who came out in support of our family. You are amazing humans

Happening tonight

The family will be there tonight. We will also have Macsen's T-shirts and car magnets. 

Onion backpack

Desperate times call for desperate measures. Antibiotics, steroids, and now .... Onions. A fellow heart mom shared a treatment she uses for her hlhser when she gets a cough. Onions slightly cooked, warm in a cotton towel and tucked into his shirt. We did it twice yesterday - he slept last night finally and didn't wake up in a pool of coughing induced vomit so I declare this old home remedy effective :) He is junky coughing now so I made another onion poultice and slipped it in his shirt while he watches Daniel Tiger. 

Happy Fall

It was 74 degrees today - hence the barefeet. Macsen needed some Vitamin D so we spent some time soaking it up outside - his favorite place to be. He is till battling this virus. I have been keeping his pediatrician in the loop - he just couldn't seem to kick it - and Monday it seemed to be getting worse so we brought him in. No wheezing but he was really rattly and working hard to breathe around all the junk. She put him on Zithromax and an oral steroid that is -HALLELUJAH!!! a dissolvable tablet I can syringe with a touch of water so he doesn't vomit it up. We started Monday and today he seemed slightly better. He wouldn't nap - maybe the steroids? He ate bone broth with a tablespoon of baby puree mixed in so it was still really watery. Some yogurt melts. A few bites of croissant, a few bites of pizza.And he downed 8 ounces of milk at bedtime - a first in his 2 years. He never eats more than 4 ounces at a time. I was so afraid he would vomit I stopped him 1/2 way through and burped him and walked with him upright, trying to get the milk to settle down. He zonked right out after that and hasn't moved for the past 3 hours. It's probably the steroids making him so hungry but I'll take it. He hasn't been cranky or mean -no 'roid rage this time- just seems to have more energy and is sillier if that's possible. He gets such a strong case of the sillies his eyes crinkle shut, his mouth stretched in a huge grin, and he grabs my face and squishes his cheek to mine. Aw, these moments are pure magic. Love that boy. 

He's still working all his speech therapists - he refuses to make their lives easy. He will show off for physical therapy and his occupational therapist who also works on feeding - but for his speech therapists he looks at them and shakes his head no when they ask him "Can you say'Boo? Moo?Mama?'" All sounds he makes everyday for his oldest sister - that girl is a natural speech therapist, a born teacher. Now if we could just bring her to all his appointments we might get somewhere!

Happy Thursday

* this post is from October 7 but failed to upload* 

Today was a good day. I picked up donations from 2 Moms Club mommies - stuff for the big yard sale this Saturday. Macsen even got an impromptu play date out of it - he had a ton of fun playing with a one of their little girls. He's so smart - he just kept giving her toys, keeping her happy, smart man. 

I was able to cancel another appointment in Atlanta by talking to the doctor on the phone - it's so crazy if I hadn't called and asked if this was a possibility we would have another 8 hr round trip to discuss everything we discussed via phone. Hello modern age! Sheesh! We are putting Macsen on an appetite stimulant - if we make him hungrier he'll try more food or at the very least take more volume of milk. He still needs to put on about 10 lbs for his next surgery. Keeping fingers crossed this will help. It will also make him sleepier - maybe we'll get some better rest at night - a girl can hope right?

He played really well all day - rubbing his face in the cat's fur then running tight circles around her, running down the driveway yelling "ahhhhhhhhh," getting pushed around the yard in his push car so I could talk to the Dr. He played with his daddy - lining up all like colors on his little gear toy all on his own. Aaron was so proud. 

He was so exhausted tonight though. We visited the Derby team at their new practice location - they are still looking for a place to have games. Macsen ran around in the heat with his sisters for about 20 minutes then he was done - sweaty and tired out. His older sister gave hima bottle on the drive home and sang "Moondance." 

He had an awful time at speech therapy yesterday - for 30 minutes he looked at the therapist like she had 2 heads, cried and pointed to the door. He was not impressed by her flash cards or her repetitive sounds. He refused to show her any of his signs, say any of the few words he says. He did the same for his other speech therapist a few days ago - he will not perform if he doesn't want to. He is not about pleasing these people. If they refuse to give him back his toy until he makes the sign for please or ball he will simply give them a look, pointedly turn his back on them and move on to another toy. He will not be bullied. Sigh. Makes me think he knows exactly wha we want from him he just doesn't want to do any of it yet. He does sophisticated things sometimes - pointing out when 1 small piece of a game is missing, following directions in a song he's never heard before, lining up game pieces by color. He is very selective about his books too - his new Cars stories are a favorite but he always goes back to his first book "On the night you were born" from Aunt Judy. 

Where's Macsen? 

Running down the driveway 

Racing me in his push car

Cool dude

Super boy

Macsen is still battling the cold he caught 2 weeks ago. We are managing symptoms with the humidifier, saline nose spray and daily torture sessions with the snot sucker. He's been vomiting more - sorry about your rug Danni- and sweating more but it's all hopefully just related to this nasty virus. 

His cardiology appointment went well last week though. They weighed him at over 23 lbs! And he ran circles around the staff - literally! They haven't seen him in a few months and he wanted to show them how he can now run. He also used the exam table as a slide, grabbed his own Echo probe to perform his own echo, and thwarted the blood pressure cuff in its quest to get a good read by wiggling and refusing to sit still. He was a wild man. 

We went for the appointment and came home the same day. - all the kids were coughing and snotty so we opted for our own beds and we all survived the almost 6 hr round trip road trip. 

That's good to know bc we have to drive to Philly next month - Thanksgiving week- for a cardiac and brain MRI and a heart cath. Not looking forward to the cath:( I 'd take a cath over surgery any day but they are not without their own consideral risk. Thankfully it will all be done at the same time so we only have to hand him over once. 

We are ready for Halloween. We've had tons of fun at a few Fall Festivals. We needed to have a little fun as a fam - the kids were sick their entire Fall break and we stayed in, watched movies and drank lots of honey tea. We were supposed to do more fundraising at the Downtown Fall Fest but the kids needed a dose of normal where they could participate in the costume contest, eat festival food and not think about their brother for a while. 

The yard sale went really well - the girls sold lemonade and told everyone about their brother 's half a heart. They are wonderful advocates. They are so matter of fact about it but occasionally they stop to think and ask me the hard questions- "is he going to be ok? Will he die?" For now I am usually able to get them to focus on how well he is doing and that is enough for them. I still believe looking at the positives is the best way for us to keep moving forward. It's harder to do at times - like when I realize he's wearing a 12 month outfit at 24 months, or I hear other 2 years carrying on a conversation and he won't even say Dada, and especially when he spits out of chokes on the one and only bite of food he's attempted that day- but focusing on all the cant's only shoves me deeper into a dark place where I can't live. So I climb out, put him in his push car and we race down the driveway while he enthusiastically yells out "Ahhhhhhhhhhh" and we both laugh our heads off.

Fundraising yard sale

Some sweet friends have put this yard sale together for us. Our family will be there - the girls want to have a lemonade stand for their brother. Many families have donated items and it should be a great event. Come by if you're local!

Macsen is 2!!!

Macsen was sick for his birthday poor little man. He needed some breathing treatments because he started wheezing and then to top everything off his toe nail came off which really hurt - the pic is the before shot with what his sister called his "button holes" the dr put in to relieve pressure right after he busted it. 

But Macsen still enjoyed smashing his cake and rubbing it in his hair. He loved riding in the remote controlled car his nana sent and reading all the Cars books. Thank you everyone who sent cards and gifts - his favorite movie Cars, a Charlie Brown he likes to hug. He loved his Cars pjs  too but he puked on them - poor guy is having a tough time keeping anything down. He vomited all day today - right after leaving our neighborhood on the way to the pediatrician, outside the pharmacy, all over our bed and right after getting his steroids - twice:( 

Needless to say I haven't had much time to sit down and really think about these 2 years we've had with Macsen - two years we never thought we would have. 2 incredible years filled with amazing memories - painful ones and miraculous ones. And yes sometimes I feel we have lost a lot during this time - years off my life, the hope of ever getting more than 3 hours sleep at a time, many friends, our savings, our innocence at the suffering so many go through on a daily basis but we have gained so much too. Friends and family that fight for Macsen with prayers and emails and calls of support. Other heart moms from around the world and their awesome advice - so much knowledge, understanding and acceptance just a Facebook post away. Our faith that miracles can and do happen every day. And of course our little miracle Bubs who chose us and we can't imagine living without. 

Happy Birthday my love. 

So the neuro sez

Rocking his 1 sock (we put it on b/c the sight of his bandage was freaking him out)

Finally got the call from the neurologist today (maybe the 3rd email I sent to M's cardiologist this week did the trick - sigh - squeaky wheel and all that.) He was very pleasant and began by saying the neurosurgeon he discussed M with doesn't think he has Chiari - yay- BUT wait!  He, himself, is not convinced and wanted to hear me talk about M. I told him how he can't eat, couldn't tilt his head back for the longest time, complains of headaches, terrible balance with constant falling, delayed, etc etc. We talked about a lot of things - the findings that Macsen has some white matter damage that he said is common in about 50% of children with heart defects, it happens during surgery/bypass and was probably a lot more significant when it happened vs now when a lot has healed. I was so worried when I read the report and it said "damage conducive with injury" thinking did he fall a lot harder than I realized? Should I have brought him in when he face planted off the couch a few months ago? But no - normal for a heart kid (as normal as any of this is.)
He needs to see Macsen - examine him and observe him- in addition to getting another MRI , a special one that watches the spinal fluid and how it circulates, or doesn't, around the brain. He said they can do it at the same time he is to get his cardiac MRI which is great so he doesn't have to be sedated yet again. We just have to get up there! So no official diagnosis yet.
Yet another trip to Philly is now in the works - hopefully for next month.  Of course the week the kids are out of school, the week I have been been mentally labeling "CHOP week" also happens to be the week this amazing neurologist highly recommended by other CHOP heart/neuro families and Macsen's cardio IS GOING OUT OF TOWN! Ugh. Never simple. So now the girls will have to miss school or we'll have to leave them which I hate doing. We still have to schedule everything with his cardiologist so all the dates are really tentative - no idea what their schedule looks like in October. I do know I need to get Macsen seen soon - nothing like the threat of permanent brain damage to make you anxious.
The neuro said no more chiropractor for now(which I get but hate b/c M's retching and gagging is in full force again) and no jumping on trampolines, or bouncy houses. Good to know since I was going to buy Macsen a little trampoline for his 2nd birthday on Sunday :( He loves getting on our big one with his sisters - horrified that I could have done damage to his brain just by letting him play on there. This sucks. I don't want him to have any more complications. I don't want to learn another specialty, another set of abbreviations and painful detail of another major organ I always took for granted. Ok he's got half a heart - don't give him more to deal with please.
Pity party - table of one, just one... bc Macsen won't join me at it. He just keeps moving forward, crinkly"happy eyes" squinting at me, flashing his wicked little grin - reminding me I can't stop believing in him now.

Poor Macsen

Macsen has had a bad week - all clumsy bad luck and not heart related. He fell flat on his face and bloodied his nose, got his fingers slammed in the door then today the freezer door smashed his big toe. Blood everywhere and the nail is coming off:( Pediatrician cleaned and bandaged it but left the nail on. Poor guy screamed and cried so much he passed out in the car on the way to the office and was so purple the dr looked pretty worried. Pinked back up once we left. Vomited up the first dose of Tylenol immediately - he HATES syrupy meds. I wrapped his foot in a baggy to give him a bath (he had blood everywhere and vomit in his hair) and water still wet his bandage. He was ok once the meds kicked in and is sleeping pretty well now. Praying he isn't in a lot of pain. He still wakes many times a night, crying and squirmy so this isn't going to help. Just can't help feeling so guilty and thinking he needs a helmet and a padded box to live in. He has the worst luck lately

He did great

Headed home!

Waiting for them to bring him back

He is done. He is apparently waking up and not happy but they haven't brought him to is yet. They called to ask if we want them to give him more sedative to calm him. Um no. Just let him see his mama. Poor little one. Hate he's waking up back there alone :( I thought he would wake up here with Mom and Dad. Keep staring down this hall waiting for them to wheel him to us

Ear tubes today

Nurses just took Macsen back. Should be about an hour for the surgery and scope 

On our way to Atlanta

Aaron and I are on our way to Atlanta with Macsen. He is finally getting his ear tubes tomorrow and a scope. Everything should start at 7 am. We have a wonderful friend watching the girls for us so they don't miss school again. We are also thankful we got into the Ronald McDonald House - we have never stayed at the one in Atlanta. We hope to only have to stay tonight and be able to come home tomorrow after his procedures but we'll have to play it by ear ... Get it. Ear tubes. Play it by ear. Ahhhh...

Some news

Big breath. Some results are back from Macsen's MRI. The Gi dr who ordered it is out of the country so I had to keep calling her office and bugging them to get someone else to give us some news. Her partner was extremely hesitant to give me any info - saying there were some abnormalities but neurology is just not her specialty and she was afraid to say something wrong. Call neurology and make an appointment so you can hear what they found. ?!!! Neurology message said expect to wait 12 weeks.!!!?! Texted his pediatrician who received a copy and read it said "possible Arnold chiari malformation and low lying cerebellar tonsils." If you are at all like me you probably know very little about these and went immediately to Google. It's an abnormality that forces part of the brain into the spinal cavity, sometimes blocking fluid, causing chronic headaches and other complications. We are floored that Macsen's little body may have yet another rare diagnosis. It is almost more than I can bear at the moment so I am doing my best not to dwell on it or the implications to Macsen and our family. If he does have it then we will deal with it as well as we can. It means more visits to specialists and probably a different type of MRI just to determine if he does have it. Macsen is suffering from chronic headaches which wake him - crying, eyes closed, little finger stabbing at the side of his head. Several times at night and during the day. I was so hoping they were caused by his anemia and once we got that resolved he would get some relief. 

In the next 2 weeks we have another trip to Atlanta for his ear tube surgery which is still on as of now and another trip to Pensacola to see the hematologist. Not super thrilled about that one bc after realizing Macsen couldn't  take the ferrous sulphate (he would vomit) the dr prescribed a tablet... For a 1 year old... Who doesn't swallow solids and you want him to swallow an iron pill? Hmmmm. I have very little patience lately for drs that don't listen, are unwilling to step out of their comfort zone and try to find solutions for Macsen. I know they exist and it is my job to find them. For now he is taking the iron supplement I buy at the health food store and we will see how well his levels have come up when we do blood tests before that hematology appointment. I refuse to do iv iron infusion on him unless his life depends on it. I think the process would traumatize him and ruin his already troublesome veins.

Today I am praying he doesn't have chiari. We are stretched so thin these days and were hoping the Fall would bring us some answers, solutions for his inability to eat, and relief from the constant traveling and specialists.  We are extremely grateful to have our little guy with us, so many children have been lost this summer to chds, that I hate to complain about the cost of having him here. But some prayers for strength would be greatly appreciated. Thank you to my friends who text to check on us, bring us dinner, pray for us daily. Your continued kindness and thoughtfulness bolster us when everything seems impossibly hard

Recovering now

He's back and recovering now. He looks so tiny. His heart rate is in the 80s - still trying to get that anesthesia out of his system. He has some blow by oxygen to boost his SATS because they were dropping below 75 without it. He is still deeply asleep. We won't get any results until the referring dr reviews everything. It could be several days. So glad this is over. Can't wait to get my hugs and "pat pat pats" when he wakes up

Atlanta appointments

We are in Atlanta again trying to get to the bottom of Macsen's inability to swallow. Swallow study yesterday showed no aspirating and speech therapist thinks there is not a physical reason. We'll see.

At 7 am, after some oral happy meds kicked in, they took Macsen back for his MRI. They told me it would take an hour and it's already been 2. I feel nauseous. This is taking too long. All the risks are playing on a loop in my head and I can't turn them off. Ready to have my baby back

What a month

We have had such a busy summer - lots of driving to and from Atlanta and Pensacola. Macsen picked up a few new doctors - in addition to Cardiology, ENT, GI and endocrinology last week he added 1 more - Hematology. The blood tests for endocrinology revealed abnormal results and I got a message they believed he had thalassemia - a genetic blood disorder I had never heard of. Dr.Google revealed very mild case with no treatment needed all the way to extremely severe cases that result in organ failure around the 20 year mark. Before our adventures with HLHS I would have immediately assumed our child had the mild version and not worried - nothing like that ever happened to us. Blame it on the PTSD from prenatal diagnosis or just our life since then but I jumped to "Oh my God! He has the severe kind - he has another diagnoses on top of HLHS and all the other stuff he has to deal with. How are we going to manage another stumbling block to a healthy child?" I was in full freak out mode - searching the internet, contacting all his doctors. His pediatrician tracked down his newborn screening in Philadelphia - it was normal. How could this have been missed? All the genetic test he has had - and us too! We met with the hematologist and after some discussion of history etc he says "Well, just looking at the numbers I would say it is very likely he has thalassemia but since you just said he is still only taking breastmilk and no food it very well may be only anemia." !!!!#$%^&*%$%^#$ Really? 3 harrowing attempts to find a vein in his tiny arm to yield enough blood to do the test later and the verdict is in - severe - SEVERE- anemia. He almost has no iron in his blood at all. So I start googling it and my first thought is why none of our battalion of doctors ever told me to give him - my child that exists solely on breast milk- a fact I repeat over and over to every dr he has- no one told me to give him iron. I feel like such a failure. His lack of growth, delayed motor skills, extreme fatigue, higher heart rate, nonexistent appetite, and just general discomfort in his own little body could have a lot to do with this anemia :( And I didn't see it. I feel sick. google reveals all kinds of awful side effects of severe anemia for normal, whole hearted people. Macsen is already struggling because of his low Oxygen levels and here I have made it worse - shorting him red blood cells he desperately needs to carry oxygen.
And with all the marvels of modern medicine treating anemia is a very slow and kind of outdated process. He was put on oral iron - ferrous sulphate - a disgusting metallic mixture that not only constipates but also is barely absorbed - I think I read only 2% is actually absorbed... awesome thought as I'm holding Macsen's retching mouth open, forcing in 3 ml 2x/day while he sobs "Mama mama mama"then vomits. It wasn't happening. The kid won't eat applesauce for goodness sakes - how am I supposed to get liquid metal down his throat? I found something else through the suggestion of the health food store owner. It still tastes slightly metallic but is a food based supplement that is not nearly as vile and about 25% is absorbed vs the 2 from the other stuff and not constipating. He still struggles to push everything out with his tongue while I squeeze his cheeks together and beg him to swallow - often wiping most of it off his chin and neck where he spit it out, but he doesn't vomit which is a plus. He has been complaining of headaches too - pointing to his head in the middle of the night, half asleep and crying. The hematologist thinks this could mean the iron isn't being absorbed through his gi tract and we need to start iv iron infusions -in Pensacola, 3 hours away- a slow, 2 hour process where my 22 month old would be "sitting still" with an iv in his arm. The process of finding a vein terrifies me already - flashbacks of him screaming, his fractured heart pounding - his body so slick from sweat it took 3 people to hold his arms the last time - they looked in one arm, then the other, then back to the first arm to take blood. And I have to do that to him every week? Not to mention his veins are already pretty wrecked - I can't imagine what this would do to them? Would they end up needing to place a port again - a PICC line like he had before his 1st surgery because his veins kept blowing and they couldn't keep a good iv? And his team of doctors is not in agreement at all on this - 1/2 say iv iron the other half say too many side effects. What do we do? The pediatric infusion scheduler called me twice today to set up the appointments and I told her I would have to call her back.
Today Mister seemed a little better.  I got a great video of him rolling on the carpet and giggling. He asked to try some of the stir fry I made for dinner-  chewing the tiny pieces of ground beef for just a second before poking his tongue out and saying "ehhh" until I scraped his tongue. But he tried it! He is learning new signs - his favorite is "book." He loves lift the flap books and will ask me to read him 10 at a time - it's impossible for me to say no when he's signing "more book please" over and over. I'm so grateful for sign language. Praying the tube surgery in September will help him hear better so he can start talking more. It will be so much easier when he can tell us exactly how he feels.
We have another trip to Atlanta coming up this month. The girls will have to miss a few days of school but Macsen has a few days of tests and I didn't want to have to leave them with friends. We will have fun the weekend before - it is our oldest's birthday and she wants to visit the aquarium in lieu of a party. YAY!!! Parties are fun but I admit I am relieved when she choses to do something else for her birthday. And we will see Nana and Grammy who will fly in from Maryland. We are very excited to see them and celebrate her big day with family.

Macsen misses his sisters when they are at school

 

Atlanta appointment #2

Big sister keeping Bubs happy during his appointment

We just got back from HOTlanta - and boy was it ever hot. In the 90's. We were very grateful for the pool at the hotel after the 4 hour drive there. We saw GI this time. I feel like for the 1st time this GI doctor actually LISTENED to us- I really, really liked her. She has ordered an MRI to look at his head and neck. It's another hour of sedation which is terrifying with Macsen's history - but the only way to see if that lymph is affecting him in ways we cannot see via xray. They also want to see if insurance will pay for an intensive 6-8 week inpatient feeding clinic There is a long waiting list so maybe after getting the MRI, ear tubes, and ruling out any physical reason Bubs can't swallow we can get him to eat without needing to go to such an extreme method. It would be tough with the girls in school, Aaron working and me in a different state away from them for so long. We would need tons of help. I have faith we can make it happen if that's what needs to be done but praying we don't need to. And if insurance won't pay I'm afraid the clinic is out of our reach at $50-$80,000 so there's that.
The trip was hard on Macsen - he was irate and uncooperative for the first time at a doctor appointment :( He screamed through blood pressure check (so they were unable to get anything,) screamed through weight and height check, and screamed for about 5 minutes after getting into the room. Finally Nurse Mimi  went and got the rest of the family in the hopes it would calm him down. It seemed to help -  his older sisters played cars with him so I could actually talk to the dr without him screaming and turning my face with his little hands. He turns it so I have to look at him, like he's saying "I don't think you realize how upset I am - look at this face!"
I don't know if he is just putting 2 and 2 together that doctor appointments are scary or if it's his age or if it was his canine teeth coming in (you can just see them peeking through) but he has not been a happy camper. I hold him 105% of the time - even in the middle of the night he crawls onto my chest and rocks himself back and forth. It is all I can do to get a shower every 2 days and sometimes I just bring him in with me. He may have grown 3/4 of an inch - I never 100% trust his measurements when they are done in different offices - but a growth spurt could explain his crabbiness too... I think. Could be many other things - heart things, gi things, ear things.... so many things. Praying in the next month we can lessen that list - at least the treatable ones to make him more comfortable and give me a tiny break. You know to shower and eat breakfast.
We also met a nutritionist that was open to my need to stay away from corn syrup. Macsen won't drink any of those sugary calorie drinks anyway. We are going to try adding 1 tablespoon of gelatin to my breastmilk (spread over many bottles to mask it) to get in an extra 6 grams or so of protein in his diet. Hopefully he won't boycott eating all together which he has been known to do when I hide stuff in his milk.
I will keep everyone updated on the MRI and tube situation. I think it will be soon. Thank you everyone for keeping him and our family in your prayers. Sometimes all of this seems so overwhelming but then I look at how far Macsen has come and I know we can and will do whatever needs to be done for our little miracle man.
On a good note, we got to spend some time with one of Aaron's best friends from Maryland. We ate a nice dinner with him on his way through town. It's always nice to squeeze in a little normal on our trips and we appreciate Kenny and his buddy Lucky driving 200 miles out their way on their 1800+ mile roadtrip to come see us.

Our little clown

New day, Different doctor

Macsen had a scheduled cardiology appointment yesterday in Pensacola. Everything looks good according to his cardiologist. Still very minimal leakage with his 1 valve - his tricuspid - and no changes. He fussed for the first minute of the echo but calmed down and let Cindy get the pictures she needed - bubbles by Daddy helped. Strange to think I have been lying on this bed, looking anxiously at that same screen of my son's heart since 20 weeks before he was even born.

His oxygen saturations have started to slowly drift downwards - no longer hovering around 84% he now is luck to see 79/80 with dips into the 50's and 60s when he cried and several minutes in the lower 70's recovering from the briefest exercise. We are still on the whole "wait and see" path for when to schedule his next surgery. When he starts staying below 75 is what his cardio said yesterday - maybe around 3 but who can say. Could be next year, could be next month. My hope is we can do it next summer - away from all the cold and flu germs that plague us with the girls in school, and maybe we can have the girls close to help him recover. Getting him up and walking is a big part of the recovery process.
He is 21 months now and since his 1 yr bday his height has slowly been falling off his growth curve. He's always been pretty consistent with weight and height being around 3-4 percentile for children his age but now he has fallen off the charts for height and is around 2 percentile for weight. So we saw an endocrinologist yesterday as well. I think we love him. Probably the best thing he told us was that he doesn't think MAcsen's lack of growth is related to his inability to eat solids and that he is getting great nutrition from my milk. For his height his BMI is in the 90 percentile range and his weight for ht is 50 percentile. So he's a good size girth wise just not height wise - he's vertically challenged. A feeding tube won't make him grow taller - probably fatter which wouldn't be a good thing according to this dr. Music to my ears - seriously. So many experts have told me he's not growing b/c of a lack of nutrition but this expert says not so. I can stop beating myself up now. There must be another reason. Macsen was supposed to have all these labs done - ie blood drawn to test his complete blood count (CBC,) thyroid and levels of growth hormone but the tech, despite digging around for a vein for several agonizing and heart breaking minutes in both M's arms, was unable to get even a drop of blood. I refused to let her try again and we are going for round 2 tomorrow at a different clinic. Hopefully the next person will listen when I tell them he is a hard stick and heating packs really will help find the vein - this one obviously knew better than me - insert expletive here.

Blood draw fail = passed out from screaming

We had a truly magical day at the beach the day before M's appointments. We all spent so much time in the ocean - Macsen sitting on a boogie board riding calmly and happily over the top of all the big swells, the girls bobbing along in the salty water. We made a mermaid out of sand and shells and seaweed  then had a great dinner at Peg Leg Pete's. Our hotel was beautiful - the Hyatt near the airport is just gorgeous and the kids LOVED the indoor heated pool, modern decor and breakfast. A perfect day

Sandy the Mermaid

Trip to Emory Childrens in Atlanta

We brought Macsen to Children's in Atlanta to discuss the consistent fluid problem he has in his ears. They want to do surgery to insert tubes to drain the fluid. He has failed several tympanic hearing tests and has visibly bulging ear drums but no recurrent ear infections. The worry is leaving fluid for more than 3 months will cause permanent damage. It's probably the most performed procedure on children these days - in fact I just drove myself nuts googling it. I am not sure tubes will help M. He hears well - follows directions, responds to all kinds of noises, hears his sisters come in the side door from all the way across the house... I worry the fluid is tied into the lymph fluid he carries around his face and neck/throat but the eNt assures me it's not connected. Eh. Not convinced . At the same time he is sedated they want to do a bronchoscopy to see if they can find any physical reason he is unable to swallow solids. We will have to be careful and work with a cardiac anesthetist - Macsen reacts strangely to anesthesia. He wakes up mid procedure and then takes 12 hrs to wake up when they redose him. He also has a crazy reaction to pain meds - trying to rub all the skin off his face like he's got insects crawling on him. That's why we have come to Atlanta for these simple procedures - bc if he needs extra support it will be there ready for him. Little man still won't/can't eat. I can get him to take some bone broth I make. I call it his soup and he often wants it for breakfast. It's solid at room temp bc of all the gelatin so I know he is getting some protein and I add coconut oil or his favorite bacon grease for a little fat but any more than a super thin consistency and he won't eat it. 

It wasn't all work though. We also were very fortunate to be treated to a show at Medieval Times. The girls yelled themselves hoarse cheering on their uncle the Green Knight. Thank you Jean Mathieu! We had a wonderful time watching you work and stealing your eggs. Our middle little wanted a baby chick so badly after visiting my brother's back yard coop she smuggled an egg into the van. We found it thankfully before it was forgotten and crushed by the water bottles, leap pads, stuffed animals and coloring books that always bury the back seat. And the kids all swam at the hotel which is a huge plus for them. 

 He's about 20 1/2 lbs now and is slowly outgrowing his 12 month stuff - yay! Never more happy then when I get to put some well worn shirt or onesie in the  give away box because it is FINALLY too small.

He is getting more and more steady on his feet - taking 5 steps before sitting down. He is also getting more upset when I insist on carrying him places. For example today in Ikea because even though he's adorable scooting on his butt around the 432 sg ft apt he gets tired and will just lay his head down... On the floor - often accompanied Linus-style by his favorite blanket, making sure to mop up any grossness he missed with his hands and face. Sigh. Course that's only mildly grosser than when he French kissed my brothers new puppy. Double sigh. Boys

April 2014 Check-ups in Philly

Finally sitting down to write about Macsen's appointments at CHOP.
We were very grateful that a research study we agreed to do - "Prenatal exposure to environmental contaminants and Neurobehavioural outcomes in newborns with congential heart disease" paid for our flight and hotel stay. We were able to squeeze in a visit with his cardiologist and with numerous family members for the 5 days we were there. The girls stayed home with their Nonna.
Overall, M is looking good. His cardiologist was pleased with the way his heart looks and we will plan on having his next surgery sometime in the next 12 months if everything holds steady. So, hopefully we can push it to summer 2015 - I would like to do it when the girls are out of school and everyone is healthy. Fall and winter were brutal this year for colds around here and M absolutely has to be healthy going into this surgery. Plus he will recover better after surgery if his sisters can be there -getting him to walk, laugh, providing some normalcy - or so it seems from the other children we have followed who have had the 3rd surgery. Could be he will need the surgery sooner - he is not growing well and is so tired all the time. Playing for a few minutes before needing to lay his head down and rest. We will keep everyone posted.
Part of the research study is a meeting with the genetics team. They reviewed all the testing he has had already -still saying no noonans, no charge syndrome. And they do a physical exam - they remarked on how beautiful he is :) One new study going on (somewhere in Europe) is looking at certain genes that may cause heart defects. They will be sending his DNA so it can be added to the research and because it is a study we won't have to pay the $17,000 price tag for the test. YIKES! Its scary to think about the scrutiny his genes are under - I am positive there are strange variations in everyone's DNA on the level they look. It is all so new, so unknown. I struggle with the idea that maybe looking at things this closely this soon in the science of genetics may hurt Macsen. But it might help too - what if they find a gene that caused his heart defect? This puts the idea of "protecting your children" on a whole other level - protecting their DNA, their blueprints that will lay out for all that can understand them the essence of my child.
He had an audiology test - he failed it. The audiologist said his test results are consistent w/ fluid in his ears and he needs to be tested here in Alabama. We are scheduled the first week of May. I know he hears well - he has favorite songs he bops too, ones he screams during until you change them, he hates Radio DJs and will cry until you change the station to music. He hears birds singing and makes the sign for birds when he hears them. He hears helicopters fly overhead while we are driving in the car and makes his sign for helicopter. He still does not talk though. He has recently started saying "MAmamama" again and does say approximations of "on, off, Elmo, apple, Dada, bath." Today I was singing Britney Spears song "Ooh la la" and he was saying "ooh la la" - funny boy. I'll try to get it on video.
He loved the developmental testing - asking/signing "more" for more tasks to figure out. How to get in a 1 sided box, finding hidden objects, stacking blocks, pointing to body parts, etc. He scored in the average range which is fantastic - lack of oxygen not slowing his noodle down. In fact they said he was very organized, never panicked or shut down when approaching a problem and just calmly figured it out. When given a peg board and little pegs he filled in the holes from the left to the right - in order. They said he could only stack 4 blocks - stupid tiny slippery blocks, seated at a table already chest high- but at home he has stacked more than 10 high of the chunkier wooden blocks - higher than  he is tall. And even though he wasn't walking at the time of the test they said he had "quality movements." All good things.Official findings were he "was delayed in his expressive speech and gross motor skills" but in all other developmental domains he is at an age appropriate level. Awesome sauce!
My least favorite part of the whole thing was his examination by a pediatrician. She wanted me to start him on soy formula - said my milk was not enough for him and he needed supplementation. I am fine with supplementing - in fact I have bought several options he refuses to drink- but refuse to give my son soy. I have researched the estrogenic properties of soy - how some male babies lactate while on soy formula, how it has been proven to shrink testes- not to mention the recent research linking unfermented soy and cancer. When I voiced my concerns to her she rolled her eyes and said (wait for it) -"That doesn't happen ALL the time. Very rarely." I'm sorry - really? Rarely? Not good enough. Then she recommended all the Pediasure type drinks that are 56% corn syrup + soy - GMO crap. Nope. I know lots of families use these drinks and they have worked for their little ones - helped them put on weight and thrive. But I just don't want to do this to M. My healthy girls don't even eat corn syrup - it makes them crazy - and to put my guy w/ an elevated heart rate on hummingbird food just seems like a bad idea. But I am quickly running out of breastmilk and am desperately trying to find something he will drink that will be help him grow. I have had some luck adding organic whipping cream to his night bottles (when I add it to his day bottles he doesn't drink as much.) So my next step will be trying to make my own formula I think. M still won't eat more than a nibble here and there and totaly depends on breast milk for his calories. We see speech for an evaluation in 2 weeks so we will see what they recommend. After that I may have to enroll him in an intensive feeding therapy program which would probably entail us relocating somewhere near a big center temporarily. Or maybe when he really starts walking, really has enough strength to maneuver around he will have enough strength to chew and swallow... just a theory I have. When my girls started walking they started eating. Granted they were walking at 10/11 months.
So more evaluations, more testing in his future :( He needs to start eating. His growth has really slowed,  he definitely isn't taking enough calories. I don't want to do a tube and will exhaust all other options before we go there. CHOP also recommended we see an endocrinologist to see if he has enough growth hormones though they don't recommend growth hormone supplementation for heart kids. Seems there may be a link between it and Protein losing entropy (http://www.tchin.org/resource_room/c_art_04.htm) which can happen after the Fontan and can be fatal.
He has been sick for several weeks now with congestion, coughing, excessive amounts of phlegnm and I think he may finally be shaking it. The pollen has mostly passed and I think it was aggravating him. If he's not mostly better by tomorrow though we will need to have him checked out by his pediatrician again just to rule out any infections and to ease my mind that he is ok.

Dr.R, audiology test, gazing at Daddy on the plane, and his favorite thing in Philly? Watching cars and trucks 7 stories below our hotel room

Macsen walking!!!!

I have been getting my photos together and was ready to write our update about our trip to Philly last week and LOOK WHAT HAPPENED! HE WALKED!! I am still in shock. He kept wanting to do it over and over - walking a few steps to each of us then plunking down on his bottom then trying his hardest to get up to a stand unassisted. He has been sick this week - virus or allergies we are not sure- but had to be put on steroids b/c he started to wheeze and struggle to breathe. The same thing happened before he crawled too which is kind of weird. Only on steroids twice in his life and both times he makes a huge leap in his physical development. I know steroids are not good for him but man! They sure seem to help him. I don't know if they give him extra strength, or extra energy, or if by decreasing inflammation somewhere in his body he is finally able to move with less effort or if the "roid rage" fuels his desire to get around on his own. Or maybe it's just a strange coincidence. In any case I am thrilled and have watched the video at least 20 times tonight.

18 months old

Macsen is 18 months old today! He has a cold so was a  little crabby but he picked out a GIANT Elmo balloon. He loves Elmo - it is one of the few words he says. "Elmo, Uh Oh, Apple, Off and On, and HOT!" You know, only the necessary words :) You can make some interesting sentences with those words! He's cruising pretty well from leg to leg - or table to chair. He had therapy yesterday and his PT thinks he's still a little wobbly/weak to be walking anytime soon. But he likes to walk by holding your fingers and tries to make me walk him to the door all day so we can go outside. He loves it outside - he wants to ride his spring horse, ride in his little push car, or sit on the trampoline while his sisters gently (and not so gently) bounce him. He laughs the whole time he is falling on his face.
Today at the Publix Deli counter I was buying sandwich stuff and our favorite Publix employee- Ms Donna - gave me some cheddar to try. I haven't had cheese in ... over 18 months so I handed it to Kissy. Macsen wanted it so badly I decided to tempt the Dairy Intolerance Gods that have plagued him his whole life and I gave him a nibble. He ATE IT!!!A nibble at a time but he ate it - only spitting it out if I gave him too big of a nibble. He ate maybe an 1/8 of the slice as we shopped for the rest of our stuff. He didn't puke and other than the extra crankiness I think he tolerated it ok. We'll try again another day but I would love to be able to give him cheese! All those calories. He's almost 19.5 lbs
We are gearing up for our next trip to Philadelphia. Check-ups and testing. Maybe we'll get more of an idea for his next surgery. He also has a cardio appt coming up in Pensacola on Monday. No matter how many times we do this I still feel myself tensing up the closer we get to looking at his heart. I feel the stress like a shadow - I don't know it's there until I look for it and then I realize it's been following me all day/everyday and I just can't shake it.
It will be good to get all these appointments over with. I'm hoping we get lots of reassurance/good news/ and a vague surgery date for somewhere more than 12 months away so we can enjoy this age - this 18 months -2.5. I love this age. Macsen woke me up the other day giving me bug "SWAK" kisses all over my face interspersed with gentle Eskimo kisses. It was truly one of the best mornings of my life - despite the fact that he woke up his normal 5+ times to eat overnight (still taking in over 1/2 his calories overnight) and it was still dark outside. He's a loving baby boy who's hugs are a gift to everyone he bestows them upon.
We just got back from a Medical Conference in Pensacola for Heart Families where we met other CHD survivors and their families. We also met a charismatic 20 something double heart transplant survivor named Bill Coons who spoke about his journey. We all had fun and Macsen actually spent a little time away from Aaron and I in their nursery. He loved it and charmed the women volunteering in the room.
 I keep forgetting to post a link to the Heart Walk we are participating in. It is in Atlanta Mother's Day Weekend and we are raising money for the Children's Heart foundation. They are a wonderful organizarion that funds a lot of research in the CHD world. http://www.childrensheartfoundation.org/research/chf-funded-research
Please consider donating to our walk. We appreciate every single donation - be it $1, $10 or more. We are trying to raise $1000 in Macsen's name.
Miracle for Macsen Heart Walk

Macsen's first Ball

M had a great time at the Cinderella ball. We go every year (though Aaron and I missed it last year b/c we w were in Phillly w/ Macsen getting his 2nd surgery.) I could hardly keep the girls still long enough to take a picture before we went in. Macsen bopped his head - very Night at the Roxbury in his shiny grey suit dancing only with his head. We danced all night and all had a wonderful time

17 months old!

Happy Heart Day!

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.