A Ray of Sunshine via the Phone

So, we just got off the phone with CHOP's Fetal Heart Program's super-helpful Coordinator Denise Donaghue and super-knowledgeable Program Director Dr. Jack Rychik... and they gave us more HOPE. Despite the fact of an abnormally elongated Tricuspid Valve, Dr. Rychik was optimistic that since the valve has NO LEAKAGE as of yet, there is little probablility that it will develop any leakage (or at least enough so that it endangers the functionality of Macsen's heart). This statement was based on the 250+ cases (over the past 4 years) of HLHS experience that these guys have to draw upon. Granted, NO OTHER previous case entailed an elongated Tricuspid. But still. Everything hinges on this valve doing all the work. And while they roughly estimate 50%, he said he feels it's probably higher. >SIGH OF RELIEF<

So, no two HLHS circumstances are the same. Ours is just a little more unusual than most. OK, than ALL. Macsen means the Greatest... I guess he had to be the most unique fetus with HLHS. What a superstar. I swear, he's had more pics taken IN UTERO than both our other kids in the last 5 years combined. (I may be exaggerating, but we're counting in the hundreds now.)

He will, of course, have to be monitored throughout his life to make sure there is no significant leakage. The doctor told us of a recent case where leakage occurred in the teenage years. Well, if he makes it through all this, a few check-ups are totally worth it. I will thank God for his life every drive to and from those hospital visits. And also every day he wakes up... and goes to bed... and every time I see him. Pretty much on a constant basis.

Here's a diagram from the CHOP website that gives a general view of typical HLHS features. Macsen has all of these and then some. His left ventricle (LV) is squished pancake flat. The mitral valve (LA to LV) is almost a solid wall. And the Aortic Valve (LV to Aorta) is non-existent.
Next step, another viewing on June 7th @ SHH in Pensacola. Praying for no leakage starting.


Here's the full diagram with explanations of the 3 open-heart surgeries.
http://www.chop.edu/img/cardiac-center/hlhs-surgery-three-stages.html

Feeling better today

I've realized something about myself through all this - when faced with horrible news I absolutely shut down for 24-48 hours. I can barely function without tears leaking out of my eyes at the slightest thing. And after those 48 hours I'm ok-ish. I can not cry every time I try to speak. 
So I called Denise (the fetal heart coordinator @ CHOP) back today to tell her thank you for checking up on us - making sure we got home ok. And I was able to ask questions.
I asked her if we could send Macsen's images to Childrens in Boston or MOTT Childrens Hospital in Michigan. Just because if the expert @ CHOP hasn't ever seen anything like Macsen's heart maybe another expert somewhere else has. She assured me they most certainly would do that and that today they have a big Wednesday meeting to discuss cases and all the CHOP experts would be discussing Macsen's case. She did say she felt there was still hope for him. We'll take that! That everything really depends on his tricuspid valve which they are calling dysplastic which just means "abnormality of development." And we just don't  know what that valve will do through the rest of pregnancy and (if he makes it) after birth. We can't make any decisions now - we can't plan. We just have to wait and see. But we can still hope. I never realized how important hope was before I saw things as truly hopeless.
I told her I do want the reality of it though. I don't want it sugar coated. If he has no medical chance - no intervention that can be done or surgery that will help I want to know. I want to know now that we'll only have  few days with him. I'll want to prepare myself, our girls, our families for it.  Maybe some wouldn't want to know but I do. She assured me that they would be honest with me. 
But for now until I'm told differently I'll hope that his heart can change, grow, still function. And if not he can maybe get a transplant - not a be all end all to his struggles as transplant comes with it's own set of problems - but it is still an option. 
It's amazing what 48 hours can do.

And the hits just keep on coming

Just got back to the hotel after 8 hours @ CHOP. 2 hour ultrasound, 2 hour echo of his heart, genetic counseling and then the culmination - meeting in a tiny room with 8 experts/fellows/counselors etc.  So I didn't get my wish - Macsen doesn't fall into those "normal" stardard risk HLHS patients. In addition to having an almost non-existent left ventricle, non functional mitral valve, he also has a severely abnormal tricuspid valve and deformed right ventricle. This is really bad. We were counting on the right  side of his heart to do all the work after his surgeries. All the surgeries doctors have developed for HLHS rely on  a functioning tricuspid valve and right ventricle. Macsen's tricuspid valve is elongated and juts pretty far into the right ventricle and the doctor said there are "things radiating off of it." He's never seen anything like it and he's the expert. He can't tell us if it will hold and continue to function because he's never seen anything like it. He did say that if the valve starts to leak there is nothing they can do for Macsen - nothing.... sob. He may even die inside me if that happens. And after he's born if his valve fails then our options are palliative care (a term I didn't even know existed until 2 weeks ago that means watching your baby die) or trying to get a transplant - which he didn't think was very probable. And these are the guys that give everyone hope when other doctors tell parents to give up.
So if Macsen makes it to birth and if his valve holds and somehow functions then he will get his first surgery within  a few days of birth and then he has maybe a 50% chance of surviving until his next surgery.... But because his valve is so unusual who knows what will happen.
Just when I had kind of gotten a handle on this HLHS diagnoses we get this one... it doesn't even have a name I can google... there are no websites devoted to hopeful stories of kids that have beat this diagnoses. The team couldn't really give me any hope... I need some hope. I need some to be able to face 3 more months of carrying my little boy who I may never get to know.................................

More good news today!

Just got off the phone with Sacred Heart and my amnio came back all clear 100%! We received the preliminary results last week and now we have the rest of the results! Sigh of relief!
Besides the obvious reasons for being happy at the results - the baby doesn't have Spina Bifida, Downs, or other genetic issues- maybe this means Macsen falls in with the HLHS babies categorized as "standard risk" who have a significantly higher chance of survival after the first surgery - 93% vs 57% for high risk HLHS. We may find further problems in his heart that would make him high risk but until then we'll be hopeful.

CHOP appointment made!

So we're on our way down this HLHS road now. Heading to the Childrens Hospital of Philadelphia on Monday. Have a whole day of tests planned and meetings with specialists. We even will have a sit down meeting with Dr Jack Rychik - the guy who actually/literally wrote the book on HLHS.  We are so lucky to know about Macsen's defect before he's born. So many parents take their HLHS baby home only to have them go into distress and start shutting down days later with no idea what happened to their seemingly healthy baby. I am thankful to know - even though it makes this pregnancy sort of bitter sweet.
 I love being pregnant - I'm one of the weird ones that doesn't beg to be induced. One of my favorite times of the day is when I'm reading bedtime stories to my girls - Macsen kicks up a storm during the first story. He always falls asleep by the second :)

Getting the ball rolling

So after numerous phone calls and emails the doctor that diagnosed us FINALLY sent all our info to the Childrens Hospital of Philadelphia. I was hoping they'd take one look at the ultrasound and fetal echo and say  Macsen was the perfect candidate for the balloon surgery. But they couldn't tell :( So , we have our first appointments in 1 week in Philadelphia - a mere 1000 miles away. Now we're researching plane tickets (which are ridiculously expensive with only a week to plan) and who can watch our girls here over night while we visit the hospital where the baby will have his surgeries. I'm excited to meet part of the team that will help our baby but so nervous too. This is all so different than my easy pregnancies with the girls.
Tonight I did go to my mixed martial art class I've been going to for the past 3 years. My doctors assured me some exercise wouldn't hurt the baby and it sure made me feel better. It was great to see my instructor John and have my  husband Aaron in  there with me - he always makes me laugh and he hasn't been able to come because of shift changes at his job. Felt a little like ... normal. Something I'm beginning to appreciate.

Monday morning and I'm the only one up

I have high hopes for today! Today we're going to get the ball rolling. Today CHOP will finally get to see my ultrasound reports and they will be able to help us make a plan for Macsen's birth and the surgeries he will need. They will tell me whether Macsen's heart can benefit from a surgery they perform in-utero called a valvuloplasty where they insert a tiny balloon in my baby's strawberry sized heart and stretch it. It can really help some babies - decreasing some's need for any subsequent surgeries, having very little affect on others. I still pray he qualifies.

What comes next.....

   So, it's Mother's Day. Aaron got it just right:) Homemade pumpkin waffles in bed, steaks on the grill, and a heart shaped cookie cake. I know, very food-centric day. I've been forcing myself to eat since the diagnoses - Macsen needs food. With this rare defect, with no known cause - I can't help think it was something I did, something I ate, did I work out too hard? did I spend too much time editing photos in front of our wireless router. What's ironic is that during this pregnancy I've eaten almost wholly organic foods, bought a juicer and was drinking juiced kale and spinach - my husband said it was like licking a lawn mower. I've only colored my hair once and used non-permanent non-ammonia containing stuff at that. Sigh. I admit to eating sandwich meat and soft cheeses. And I admit to not being worried - not one day I was pregnant - that anything could ever be wrong with my baby. 

So what comes next? 

 I've been reading everything since our ultrasound. Hospital reviews and surgery information. I am now fairly knowledgeable about the 3 open heart surgeries Macsen will need - 1 within days of his birth, one within months and another while he's a toddler.

I have even found the hospital - Children's Hospital of Philadelphia (CHOP)- a mere 1000 miles away- that I think will give Macsen his best chance at life. I've read the sheer number of these type of operations a hospital performs each year directly correlates to their success rate - the more they do they better they are at it. If we don't do our best at finding the best place for Macsen and something happens to him I could never forgive myself. 

 So now the questions come and more tears - tears from my 5 year old at the thought of leaving her home - her room with the sparkly paint and butterfly stickers ,her bunk beds we were so proud to find on Craigslist and which doubles as a fort on many occasions, but mostly her daddy who we think will have to remain behind during the month or so before the baby is born and then for who knows how long after Macsen is born. It's all an unknown. I don't know if we'll ever be able to come home again. Will he ever be well enough to brave living several hours away from reputable pediatric cardiologists with experience in this rare defect? 

Always a lover though my 5 yr old wanted to name this blog "I Love My Brother"