The Day Everything Changed

Today marks one year ago that we got confirmation of Huck’s heart with the fetal cardiologist. But the day everything changed was January 20. That day, we made that hopeful drive to Galveston, praying there was some mistake. That maybe our normal OB’s ultrasound machine wasn’t clear enough. Or maybe Huck was just laying weird and they couldn’t get a good shot of a whole heart. I remember thinking, “This isn’t really happening. This doesn’t happen to people like us. This is all just a mistake. A fluke.” While we sat on the ferry, we didn’t get out and sightsee like most people do. Instead, we sat in the car and googled heart defects. We knew what worst case scenarios were and we read words like “mortality rates” and “survival rates”. We vaguely knew what hypoplastic left heart syndrome was and we knew that a single ventricle defect still has mortality rates. I had a copy of the insurance paperwork from my OB’s office but I couldn’t make myself look at it because I was scared of what it might say. I didn’t know it until much later but the diagnosis codes were listed there, one of which was “apparent life threatening event”. We went to that appointment hoping for the best but prepared for the worst.

They called us back and we had the most in depth ultrasound ever. The tech took the time to show Nolan the things he’d never really gotten to see before, chatting with us the whole time. We saw his hands and fingers, feet and toes, his face and lips, all of his bones. And when she switched over to his heart for the echo, she went dead silent. We squeezed each other’s hands and we knew something was wrong.

They told us before that appointment that we would have the scan and then the maternal fetal medicine doctor would talk to us – good or bad, we would have answers before we left. The tech got up and left the room and we talked about how strong his heart looked. That there was one part that was just ticking away, so strong. We found out later that was his one good valve, which was strong. The doctor came in, sat down right in front of us so that he was at eye level, and said “we think your baby has hypoplastic left heart syndrome”. I swear it was like something in a movie. It felt like everything got really fuzzy around the edges and I was hearing him but I was simultaneously scrolling through my brain of everything I’d read about HLHS and how everything in our life just shifted. In one sentence, our world changed.

We were told that we would be referred to a fetal cardiologist, who confirmed HLHS one year ago today, but like most of you know, we later found out with our rockstar fetal cardiologist at Texas Children’s that Huck has his own rare combination of defects making up his single ventricle physiology. He’s not a straightforward HLHS baby; he has most of the same characteristics, but with a few additional defects complications. (As a reminder, Huck had ventricular inversion with a hypoplastic left-sided right ventricle, tricuspid atresia, a hypoplastic aortic arch, congenitally corrected transposition of the great arteries, a vsd, an asd, and a very large pda. On paper it was listed as 7 different defects.)

We left there in rough shape. I don’t think we’d ever been so sad before in our life. I remember we tried to eat lunch but neither of us had much of an appetite. We were driving down the seawall in Galveston and I think we both saw him at the same time. I heard Nolan say “oh no” but it was too late. I’d already seen him. There was a dad waiting to cross the street, with a happy little baby boy in his arms. Something relatively small, but we both completely lost it. Nolan was on the phone with his dad and he just handed me the phone and I managed to choke out that we’d call them back. We had no idea how we were going to make it through this. But here we are, one year later and we’ve survived so far. And we’re home and happy and Huck is doing great, all things considered.

Yesterday he had a cardiology appointment and an echo to recheck the potential arch narrowing and his heart function. I’m so relieved that we found out that his echo showed almost zero gradient in his arch, which means no narrowing and no cath lab! His ventricle function is still the same – mildly depressed – and that may either stay the same or may still return at some point. His cardiologist was able to check out his consistent retracting that we’ve been noticing for a while, even when he’s very clearly not in distress, and she determined it’s not from respiratory distress, but mechanical from the way his sternum and ribcage healed from his two surgeries. We’re weaning his enalapril, discusses plans for the future, talked a little about his Fontan, and got released to visits every 6 months. Oh, and his saturations were holding steady at 89! I’ve been so anxious for this appointment, especially with it basically being the one year mark from THE day, but I left there feeling like a huge weight has been lifted off my shoulders.

This time last year was so hard on us. But this year, things are looking pretty bright. ❤️

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rt #ToughLikeHuck #1in100

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Huck | 7 & 8 Months

Stats:

Birth – 3035 grams | 6 lbs 11 oz

1 Month – 3950 grams | 8 lbs 11 oz

2 Months – 4420 grams | 9 lbs 11 oz

3 Months – 5620 grams | 12 lbs 6 oz

4 Months – 5889 grams | 12 lbs 15 oz

5 Months – 6840 grams | 15 lbs 1 oz

6 Months – 7530 grams | 16 lbs 10 oz

7 Months – no official weight check, but somewhere in the neighborhood of 17 lbs

8 Months – 18 lbs even 🙌🏼

Finishing out our last box of size 3 diapers, and moved into size 4’s. He kept blowing out of the 3’s. 🙈 Fitting comfortably in 12 month clothes and got some 18 month outfits for Christmas!

Eating: We had been using the European Holle formula for a while and I swear there was something in it that was magic because Huck was the happiest baby on it. Then some time around 7 months, he had something weird happen. He ran a low grade fever and had a runny nose, seemed to be cutting more teeth, and then started pooping like c r a z y. Up to 13 times in one 24 hour period. Several times a night. Blowouts galore. And it wasn’t watery, just loose and super frequent. This went on for 3 days. Then I started seeing specs of blood late on a Saturday on day 4. I was immediately worried about NEC (he’s had it twice, before his Glenn), but I knew the likelihood was extremely low, plus he didn’t have any other symptoms. Wasn’t very fussy, wasn’t refusing feeds, belly was soft and normal roundness. I didn’t have the gut feeling to run to the hospital so we waited it out until Monday, day 6. I was thinking maybe so much poop had irritated him and caused a fissure. But by Monday morning it was still there, a spec or two in every diaper, so off we went to the pediatrician. Thankfully, almost immediately after seeing one of his diapers, she diagnosed him with a milk protein allergy and switched him to Nutramigen. I think it was two days in that his poop cleared up from blood, and the frequency started to decreased about that same time and now we’re back to normal! He eats quite a bit more of the Nutramigen too, sometimes taking up to 6 ounces at one time. This is big for him!

Sleep: I think really the only thing that has changed in this department is that he’s maybe the tiniest bit more predictable. He still wakes up several times a night to eat but I noticed recently he doesn’t eat very much before he rolls over and goes back to sleep, so I think it’s more habit than anything. He normally goes down around 6:30/7, wakes up every 3-4 hours and is up anywhere between 5 and 7. He usually takes a small cat nap in the morning not too long after he wakes up, which from everything I’ve read, is a sign he’s not getting enough sleep at night, but we haven’t been able to get that figured out just yet. Then he normally takes two solid 1.5/2 hour naps, one in the morning and one in the afternoon. Sometimes it’s two shorter naps for morning. He still sleeps in the co-sleeper next to our bed and I really think we’d be okay moving him to his crib, but if you’ve been around here a while, you know our house is old and awkwardly laid out, so you actually go through his room to get to Whitlee’s room, with just a doorway separating them. So I’m worried he’d either wake her up or vice versa, so we haven’t taken the plunge yet.

Also, he started flipping over to his tummy to sleep and prefers to sleep either on his right side or on his tummy with his butt in the air. So cute!

Milestones: Sitting up really solid

  • Can go from sitting to laying down on his tummy without crashing (usually)
  • Scoots around on his butt if the floor is slick
  • Standing if he has something to hold on to and acting like he wants to pull up on things he can get next to
  • Got his 3rd and 4th teeth – upper lateral incisors before his upper middles, so he looked like a vampire for a minute
  • 5th and 6th teeth also coming in – upper middle teeth
  • Had his first Christmas
  • Started real solids, prefers them over traditional baby food
  • Finally started babbling and of course, “da-da” came first – just like his sister
  • Got his first big boy haircut
  • Army crawling has started
  • Blows kisses intentionally (without the hand motion)

Whitlee never did the traditional crawl, up on her knees. I think it’s because our floors are so hard. Shoot, I don’t even want to crawl around on them. But she went straight from army crawling everywhere (fast!) around 7 1/2 months, to pulling up around 8 1/2 months and cruising the furniture and then took off walking around for good around 9 1/2 months. I’d say little brother is hot on her heels! This makes us so proud because we were told he would most likely have developmental delays and even possibly some severe delays but he seems to be pretty much on par with definitely what could be considered “normal”.

Medical Milestones: Nothing too major this month except diagnosing a milk protein allergy and switching formulas to alleviate that issue. We go back to the cardiologist in a couple of weeks for a check up and another echo. I’m hoping he’s calm enough to get a good look at his arch and they can make a decision on whether it is narrowing or not. We’re also praying his decreased function shows to be fully restored which we’ve been told can happen after the Glenn. His cardiologist will probably make a move on weaning his enalapril or tell us how much longer she wants him on it. We’ve been told he’ll be on daily aspirin forever (he’s on half now), but that the enalapril probably won’t be long term.

He also got a referral to physical therapy because he has some mild torticollis. I’m not sure when we’ll start PT for that, but it shouldn’t be too long and I’m hoping it’s not too bad. It’s not severe torticollis so maybe just some stretching or a tot collar for a bit. Our occupational therapist from the hospital said torticollis is super common in their cardiac patients. I guess maybe from being in a hospital bed for so long, but Huck has always preferred everything to one side. I don’t know if the torticollis came first and pushed the preference or if the preference is what caused the torticollis. Regardless, it can be corrected and should be while he’s still young.

Likes: Eating big people food

Whitlee

Maggie (our dog) – oh my gosh, he’s obsessed!

Riding in the car

Baths

Trucks

Anything that lights up or makes noise

Chewing on anything but loves Sophie the giraffe and his squeaky donkey

Snuggling with a blanket

Being held IF you’re standing up

Dislikes: Being cold

Being held if you’re sitting down

Being fed, wants to do it himself

Having a dirty diaper, he wants to be changed almost immediately

Getting his face wiped

His head being rubbed

It blows my mind that we are rolling up on a year old so soon! Things have started to settle down just a little bit but we hopefully have something good happening this year and things will more than likely become hectic again. But I was reflecting recently on where we are now. Life with Huck started out so different than your typical newborn stage and there was a lot of focus on “what needed to be done” and survival mode. I had heard that it takes a little while, but soon you settle into normal baby life, and the heart part becomes a little more distant. It’s always there, kind of like background noise, but it’s a little less front and center. I think we’re finally starting to get there, given everything goes well at the cardiologist next week. I remember looking back in the car the other day, seeing him in his car seat mirror and thinking how much I love him. It sounds weird, but for once I didn’t think about how thankful I was that he’s alive (of course, I’m insanely thankful), or wonder if he was breathing okay, or wonder what his heart rate or saturations were. I just looked back and thought, “goodness, he sure is cute. Oh, I just love him to pieces.” It was a nice normal moment.

Happy eight months, little boy. We are so lucky to be your parents and can’t wait for so many more normal memories with you!

Huck | 5 Months & 6 Months

Well it finally happened. The inevitable second kid problem where you don’t have nearly the time you had with the first kid and you almost miss TWO months of posts. But I’m finally getting 5 and 6 months posted just in time for 7 months.

Stats:

Birth – 3035 grams | 6 lbs 11 oz

1 Month – 3950 grams | 8 lbs 11 oz

2 Months – 4420 grams | 9 lbs 11 oz

3 Months – 5620 grams | 12 lbs 6 oz

4 Months – 5889 grams | 12 lbs 15 oz

5 Months – 6840 grams | 15 lbs 1 oz

6 Months – 7530 grams | 16 lbs 10 oz – 32nd percentile! 🙌🏼

Well since I last posted at four months, we went from size 2 diapers up to size 3 diapers and he pretty much blew right through 6 month clothes within a few weeks and we’ve moved on to at least 6-9 and a lot of 9 month clothes. Baby boy is growing!

Eating: Whew, so much has changed since my last post. We were still inpatient at that time and eating was Huck’s biggest obstacle to be discharged home. He was really struggling with acid reflux and vomiting large volumes of milk. He was still on breastmilk but my supply completely crashed a couple of months ago so he was on the back stash I had pumped for him. We finally ended up doing an upper GI study to check for a few things and another heart mom pointed out in a blog post, it sounds odd, but we prayed for a bad outcome. Because a “bad” outcome means there’s an answer and a reason for his struggles with eating. And a reason typically comes with a solution. In our case, Huck’s GI study showed that his stomach wasn’t emptying properly and he was actively refluxing at random times. This meant that his poor motility was causing him to reflux more and was most likely causing his disinterest in eating. We started him on a motility agent and slowly but surely, he started eating more, keeping it down, was able to come off tpn and lipids, and eventually ate enough to earn his ticket out of the hospital!

Since we have been home, we transitioned from the last of my breastmilk to Enfamil Gentlease. We had a good experience with EG with Whitlee, so it was familiar territory to us. Huck did okay, but he still seemed so uncomfortable while eating and I know him well enough to know he was still silently refluxing. He had stopped spitting up but he still had all of the signs. Another heart mom had told me that they had tried a European formula and her daughter loved it. I did some research on the difference in European formulas and American formulas, how to actually get it, and decided to give it a try. In the first week, Huck’s entire disposition changed. He was much happier, seemed so much more comfortable, stopped looking so reflux-y and his bowel movements even changed back to looking like breastfed baby poop. We have been on this formula for a while now and loving it! I’ll probably make a separate post about the details but if you’re curious about anything, feel free to ask! We chose to go with Holle Stage 1 and tried the Stage 2 but he seemed to prefer the Stage 1, so we went back.

We also started baby food between five and six months and he loves the vegetables and does not care for the fruits at all. He really likes to eat but I’m a little worried about the constipation that seems to be coming with it. I’ve been reading up on probiotics and we may start that if we get the all clear from our doctor and cardiologist.

Sleep: We finally seem to be making some progress in this department. At our first visit with his cardiologist, who is very well versed in single ventricle babies, she asked about his disposition and certain behaviors. We told her about how fussy he is and that you really can’t leave him to play for even a couple of minutes without him losing his mind. She reminded us that in the interstage for single ventricles, they absolutely cannot let them cry (their BT shunt is so dependent on different variables and crying can cause restriction to his lungs), so any time he ever made a sound, someone was always there to comfort him – rock, pat, shush, bounce, etc. So when you are post Glenn and stable, there’s a whole new world for these babies where they can actually cry without it being life threatening. She said that it’s one of the most common concerns she hears from single ventricle heart parents, that their babies cry so much and it’s exhausting having to constantly console them. So she not only gave us permission, but encouraged us to start letting him cry it out a little. Self soothing and entertaining themselves with toys is a learned behavior from a very young age, and he never had that opportunity, so here we are at 5/6 months with a baby that has no idea what to do with himself. We attempted the same mild version of cry it out that we did with Whitlee and he at least will put himself to sleep now. He still wakes up one million four-six times a night to eat, but it is what it is. Whitlee didn’t sleep through the night until 18 months, so I guess that’s evidence that we’ll get there eventually.

Milestones: Rolling like crazy

  • Standing with support
  • Sitting unassisted longer stretches
  • Self weaned his swaddle (thank God for that. Weaning it with Whitlee was a nightmare.)
  • First baby food
  • First trip to Galveston and rode on the ferry
  • First Halloween – he and Whitlee were both puppies, per her request!

Medical Milestones:Finally discharged from the hospital just before 5 months!

  • Had our first outpatient cardiologist visit and echo – ventricle function has remained the same (mildly depressed), but his arch may be narrowing again. We go back in January to check again.
  • Weaned several medications
  • Got his first round of vaccinations
  • Got his flu shot at 6 Months
  • Got approved and received his first synagis shot (rsv prevention)
  • Had his first hospital readmission for a random virus that had him running fever and a high white blood cell count, the day of 5 months. Thankful to report it ended up just running its course and being done. Sats and blood pressure all held steady throughout.

Likes: Being held

  • Eating
  • Whitlee
  • Maggie (our dog)
  • Riding in the car
  • Baths
  • Vegetable baby food
  • Big people food – constantly trying to eat my food
  • Chewing on anything!
  • Snuggling with a blanket

Dislikes:Diaper changes

  • Lovenox shots
  • Being strapped into his car seat, totally fine once he’s in and the car is moving
  • Fruit baby food

Whew! Life has been so hectic. Actually, I think hectic is the understatement of the year. But even on our craziest days, we are so incredibly grateful for the gift of our two babies.

Happy 1/2 Birthday, Huck Inlow! We are so proud and so blessed with every day we get with you. Forever thankful for life at home together as a family. ❤️

Five & Six Month Photos

Huck’s Heart | The Grand Explanation 

I know I’ve been promising a video, but that has actually proven to be a little more complicated than I think I can take on right now, so I’m hoping this will help explain his anatomy and surgeries just as well. Also, disclaimer – I’m clearly not a doctor or a nurse, we’ve just learned as much as we can about Huck’s diagnosis and surgeries. If you are a doctor or a nurse and notice that I don’t have something quite right or maybe worded improperly, I apologize in advance! 🙂 The term “hypoplastic” will be used quite a bit. This just means “underdevelopment or incomplete development”. Okay, I’m done with the disclaimers. 

Huck was originally diagnosed with hypoplastic left heart syndrome (HLHS) when I was 22 weeks pregnant. This was diagnosed at UTMB. When we transferred care to Texas Children’s at around 33 weeks, it was later found that he actually didn’t have HLHS, but something similar. There’s a whole category of heart kids that are “single ventricle”, which includes hypoplastic left heart syndrome, hypoplastic right heart syndrome, and quite a few others. The common denominator is that all of these kids only have a single ventricle, and that most will require the same palliative three stage surgery series, but Huck’s heart is different in its own way; a rare combination of several different defects. For simplicity’s sake, you could say that his heart is fairly similar to hypoplastic left heart syndrome, but he has some other characteristics that make it different. 

So, I’ll take you back to anatomy class for starters. (Wishing I’d paid a lot more attention in college A&P these days!) A normal heart has two sides – a left side and a right side, made up of four chambers. The right side recieves deoxygenated blood “blue blood” from the body through two veins called the Inferior Vena Cava and the Superior Vena Cava. (Keep these two guys in mind for later.) The blue blood travels into the right atrium, down to the right ventricle through the tricuspid valve, and then is pumped into the lungs through the pulmonary artery. The blood is then oxygenated in the lungs to become red blood, and is pumped from the lungs into the left atrium, down to the left ventricle through the mitral valve, and then back out to body through the aorta and the aortic arch. 


First thing to address is that Huck only has a single ventricle, which means where a normal person has two lower chambers, he only has one, which is why, along with HLHS and HRHS kids, he essentially only has “half a heart”. He also has ventricular inversion, meaning his ventricles are swapped. His left ventricle is on the right and his right ventricle is on the left. But also, and this is where it gets a little complicated, add in that his “left sided” ventricle (which is actually his right ventricle) is hypoplastic. So he has what they call a “hypoplastic left-sided right ventricle”. 

He also has tricuspid atresia, which means his tricuspid valve never developed. (A little backstory – his tricuspid valve would have originally been on the right side of the heart, but with the ventricular inversion it ended up on left side with the “left-sided right ventricle”. The valves were actually what caught our fetal cardiologist’s attention, because she noticed that the mitral valve was on the wrong side of the heart, and then she realized that the characteristics of his single ventricle were more on par with a left ventricle but was on the right side. This led her down the line, hammering out each detail, until she pretty much diagnosed him prenatally spot on with what they determined after he was born. She’s a rockstar!)

So continuing on, he also has a hypoplastic aortic arch, also known as coarctation of the aorta, which means his aortic arch was severely underdeveloped. 

Then he has something called “congenitally corrected transposition of the great arteries”, or CCTGA, which means his two main arteries are actually backwards and would normally be considered a defect in itself called transposition of the great arteries (TGA) but because of his ventricular inversion, his arteries are connected to the correct ventricles, making it “congenitally corrected”. 

He also had a very large Patent Ductus Arteriosus, or PDA. A ductus arteriosus is a blood vessel that, before birth, exists in the aortic arch that connects the aorta and the pulmonary artery. This is how a baby like Huck can survive in the womb. It provides a route of blood flow that would have otherwise been restricted by his anatomy. Within minutes or up to a few days after birth, the ductus arteriosis is supposed to close as part of the normal changes occurring in the baby’s circulation, unless a baby is given prostaglandin to keep it open. In Huck’s case, his was very large and would have most likely remained open (which is why it is referred to as a “patent” ductus arteriosis), but he was on prostaglandins just to be safe. His PDA was actually assisting his heart function by allowing systemic blood flow to the body, where as his hypoplastic arch would have restricted it. The PDA was removed during the arch reconstruction portion of his first surgery. 

And lastly, he had a moderately sized ventricular septal defect (VSD) and a small atrial septal defect (ASD). The two septal defects are something a lot of people have heard of, commonly referred to as a “hole in the heart”. This picture below is the closest I have found to Huck’s heart. It shows the ventricular inversion, the CCTGA, the hypoplastic left sided right ventricle, the tricuspid atresia, and both septal defects. It is only missing the hypoplastic arch, which is shown in the second picture.




So how does a heart with these defects survive?

In a normal heart, the basic path of blood is oygen poor (blue) blood flows into the heart from the body, out to the lungs, where it is oxygenated (red blood) and returned to the heart, and then sent back out to the body. Red blood and blue blood are kept seperate. Since Huck is missing some of the key components to make this work and some of his anatomy is swapped, his blood flow is different. You can follow this in the picture above – blue blood flows in from the body and into the right atrium, red blood comes in from the lungs and into the left atrium. At that point, because the left sided ventricle and tricuspid valve aren’t there to do their job, it forces the blood back over to the right side of the heart, and all of the blood goes into the same area and mixes to become “purple blood”. Then the mixed blood is pumped both out to the body and out to the lungs. Purple blood is okay but not ideal long term. 

So for Huck’s first surgery, which was a modified Norwood, they had to make a few changes to Huck’s heart so that his blood flow to and from the lungs was adequate, and the blood flow to and from his body was adequate. The Norwood is usually done as a bridge to get a baby big enough and stable enough to support the circulation of a Glenn (second stage). The main issue for Huck was that because he doesn’t have a working valve on the left side of his heart, the blood doesn’t have a way into the left sided ventricle, and therefore it doesn’t have a way into the aorta and back out to the body. His VSD could have been used to their advantage because it allowed blood flow into the very small left sided ventricle from the back side, and would have allowed blood a way into the aorta, but his VSD was considered to be moderate and they didn’t trust it to remain open and a proper source of blood flow. To alleviate this issue, they connected his two main arteries (using a “double barrel” DKS procedure) and added what’s called a “BT shunt” to help control the blood flow to the lungs. They also removed the PDA and reconstructed his aortic arch (previously hypoplastic) so that blood flow from the heart out to the body was not restricted anymore and didn’t require prostaglandins long term. And they cut a little more out of his atrial septal defect to allow blood to flow more freely from the left atrium to the right, giving him more of a “common atrium”. This picture shows hypoplastic left heart syndrome and a homograft patch instead of a DKS, but it’ll give you an idea of how they join the two arteries into one big artery and where they place the BT shunt. 

The end goal for a single ventricle (a completed Fontan circulation) is to eventually bypass the heart completely for oxygen poor blood from the body, which will then go straight to the lungs. Then the heart’s only responsibility will be to receive oxygen rich blood from the lungs and send it back out to the body. 

For the next step in the palliation series, Huck just had his second surgery at 3 months 25 days. This surgery was a bidirectional Glenn, and they basically removed the BT shunt and connected his Superior Vena Cava (SVC) straight to the pulmonary artery. This is the first step in bypassing the heart for oxygen poor blood to the lungs. So, in a normal heart, oxygen poor blood from both the upper and lower body would normally go into the right side of the heart, and out to the lungs to be oxygenated, pretty simple. But in a Glenn, which addresses the upper body’s blood flow, the oxygen poor blood from the upper body goes into the SVC like it should, but the SVC is now connected straight to the pulmonary artery, which bypasses the heart and goes straight to the lungs. From there it will receive oxygen from the lungs, and go from the lungs into the left atrium, over into the right atrium where it will meet with the oxygen poor blood still coming from the lower body, down to his right sided left ventricle and then back out to the body. So essentially, he’ll have less “purple” blood (red and blue blood mixing). Also, not a standard part of a Glenn, but his surgeon performed a “right pulmonary arterioplasty”, which placed a patch on his right pulmonary artery because it was mildly narrowed in one spot.

During the third surgery, the Fontan, which will happen at about 4 years old, they will go back in and do something similar to the Glenn, but at that time, they will connect the Inferior Vena Cava (IVC) straight to the pulmonary artery like they did with the SVC. This will complete the Fontan Circulation and bypass the heart for all oxygen poor blood from the body to go straight to the lungs. At that point, Huck will have the closest thing to “normal” blood flow (blue blood from the body to the lungs, red blood from the lungs to the heart and back out to the body) that his body can have with a single ventricle.

Will he ever be “cured”? 

The thing to remember with all of this is that for a single ventricle baby, there is not a “cure”. They call this a palliative fix. Palliative generally means “providing relief from the symptoms and stress of a serious illness with the goal being to improve quality of life for both the patient and the family.” While they can’t reconstruct his entire heart or ever give him two ventricles, they can alleviate the problems associated with only having one ventricle and hopefully give him a long and happy life.

How long will he live?

Currently there are only people in their 30’s that have had this surgery series so there is really only data to that age, but our hope is that by the time Huck is in his 30’s, those people will be living well into their 60’s. In some cases, single ventricle babies end up needing a heart transplant and it’s even possible that some kids that have the whole 3 stage series still end up needing a transplant. That is something that will always be in the back of our minds. Huck’s single ventricle is doing all of the work, so there’s always a chance it could lose function and he could end up in heart failure. Huck does have a small advantage in that his single ventricle is a left, which is considered to be the “workhorse” of the two. He also has a small disadvantage in that his single ventricle has an area of “mildly depressed function”. We have been told it is very possibly for him to regain full function in his ventricle at some point post Glenn, just due the overall workload being reduced on the heart in comparison to a BT shunt circulation. But for factual sake and the statistical side of things, studies show that survival after the bidirectional Glenn and Fontan operations is nearly 90-95%. The mortality rate for single ventricles is usually highest between the Norwood and Glenn, what they call “interstage”. So when a baby makes it to their Glenn, it’s a big deal. Usually a pretty celebratory day around the CVICU! 

Will he have a normal life?

From our perspective, yes, we absolutely hope so. With that being said, living any part of your life in a hospital is not normal. Having 3+ open heart surgeries is not normal. Taking medication for the rest of your life is not normal. BUT we hope that Huck will be able to do anything he wants to do. Ability is individualized. It’s very possible that he will get out of breath a little easier, and pretty unlikely to participate in heavy contact sports (in protection of his sternum), but I have heard of many kids that play sports and have exceeded expectations. I posted a while back of a girl named Meghan Roswick, who is a 25 year old Fontan with HLHS that competed in gymnastics as a child and is a competitive skier now. It will all depend on him and how well his heart holds up. We plan to do all the normal things we would have done anyway – trips to the lake, playing chase around the house, family vacations, swimming lessons, preschool, normal school, just “life”.

It’s truly amazing what modern medicine can do now and we pray that it continues to progress. Texas Children’s has recently been ranked as #1 in the country for pediatric cardiology and heart surgery, so we feel confident that throughout Huck’s life, he’s in the best hands possible (we literally trust our surgeon with his life!) and they will do everything they can for our little boy. He is currently doing very well with his Glenn recovery but we are still working on crossing some things off the list before the “h word” is discussed. 

Huck | 3 Months


Stats: 

  • Birth – 3035 grams | 6 lbs 11 oz
  • 1 Month – 3950 grams | 8 lbs 11 oz
  • 2 Months – 4420 grams | 9 lbs 11 oz
  • 3 Months – 5620 grams | 12 lbs 6 oz | 62 cm

He is wearing size 2 diapers and mostly 3 month clothes, but a few of those are starting to get snug length wise. He basically just blew right through that size.

Eating: Huck is currently eating 85-100 mls of breastmilk (roughly about 3 ounces, give or take a few mls) about every 3-4 hours. He was made NPO (nothing by mouth) for a week due to a bloody poopy diaper and that hindered his volume capacity a little but he’s finally doing a lot better.

Sleep: His daytime sleep is still fairly consistent. He’s normally sleeps until about 8 AM, gets a bottle, then meds, and lovenox about 9 AM, takes a shortish nap, gets his weight done, a bath, and lead sticker change, followed by another bottle and meds, then a really long nap from about 12-2/3. He’s usually awake and happy around 3 PM where he will eat/play/cat nap until around 5:30. He’s been eating less and sleeping more at night which the doctors and I are both good with. He’ll normally eat again about 9 PM, sleep a good stretch, eat again around 2-3 AM and sleep another good stretch.

He still sleeps a lot in the mamaroo which helps his reflux a lot, but will also sleep pretty decent in his bed, in his “nest” that they make for him.

Milestones: He rolled belly to back for the first time and has had a few little laughs! He also sits up assisted and can control his head and body really well.

Medical Milestones: Thankfully, nothing has changed majorly since 2 months. Currently still inpatient on the CVICU heart failure unit while we wait on our Glenn surgery. We will be here until then. He is on milrinone and clonidine + reflux medications + a twice daily diuretic. He is not on any respiratory support at the moment and taking all feeds by mouth. He is finally off tpn and lipids, but still getting therapeutic doses of lovenox for a blood clot in his picc line. The Glenn should be happening in the next few weeks!

Likes:

  • Being held / rocked
  • The mamaroo
  • His playmat
  • Anything with lights and sound
  • The bumbo chair
  • Warm milk
  • Listening to Elvis (it seriously calms him down immediately)

Dislikes:

  • Diaper changes
  • Picc line dressing changes
  • Baths
  • Lovenox shots
  • People messing with his feet

I can’t believe this little guy is already a quarter of a year old. It seems like it’s both flown by and crawled by. I love getting to know him and spending time with him and seeing him with his daddy and his sister. His next surgery should be very soon and if everything goes as planned, we should be home not too long after that. For the first time, all four of us will be together under one roof. I can’t hardly wait! ❤️

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Huck’s Heart | April Update Part 2 

In honor of making it to 34 weeks, here’s part 2 of this crazy journey so far! 

Read Part 1 here 

I started calling on Monday of the following week, asking for the status of our referral. We were told it could take 5-10 days for approval but if we called in, they could possibly expedite the process. Again, trying to shorten the insanity of what we went through, I made phone call after phone call; one to the OB’s office, then another to our coordinator with Texas Children’s to relay information and ask questions, then another to the insurance company, rinse and repeat, until we finally got approval for all our visits the afternoon before our first appointments with Texas Children’s. Talk about cutting it close! 

On Friday, April 7, I was up at 3 am partially because I couldn’t sleep and partially because we had to leave super early to be in Houston for our 8 am appointment and we weren’t really sure about traffic. It was a whirlwind day, with 3 different appointments, each lasting approximately 2 hours. We were basically starting from scratch and letting them build the information from the last 8 months and Huck’s heart in their own systems and records. 

First we met with the MFM (maternal fetal medicine) ultrasound department. We had a full anatomy scan, which at 33 weeks is kind of strange because he’s so big at this point. They measured all of his body the best they could, let us know he was measuring 5 lbs 4 oz, in the 68th percentile I believe, and confirmed for like the 15th time that he is indeed a boy. 🙂 Oh and that his head and belly were measuring in the 90 something percentile. The words “fat tummy” were used lol. He’s a full pound bigger than Whitlee at this point. 

After that we met with the Maternal Fetal Medicine doctor that we will see until Huck is born and who we thought would be delivering him (I’ll get to that part). She went through my entire medical history, looked at my blood sugars, talked about preeclampsia and symptoms to keep an eye out for, plan for monitoring going forward, the delivery process, what to expect the next 6-7 weeks, gave me an updated tDap vaccine for this pregnancy, and did a cervical check. And then before we signed all of the consent forms for birth and treatment, she asked “do you want another c section, or would you like a vbac?” She was so casual about it, like asking if I wanted water or tea. I asked what she thought and she said that as long as I’m stable with my gestational diabetes and blood pressure, she has absolutely no problem letting me go into labor on my own, when Huck is ready, and letting me do a trial of labor after cesarean; that they do tons of them there. She said it would be easier and faster recovery for me, as well as beneficial for Huck to travel through the birth canal and help squeeze extra fluid from his lungs, especially in this case. She reminded me I’ll need the ability to go see him in the nicu and a c section recovery might hinder the speed in which I’m able to go there, plus having a 2 year old to handle. There were discussions about the risks involved and reasons why we might still end up having a c section but for now, we agreed to keep a close eye on things and proceed towards a vaginal birth. She may or may not be the actual one to deliver him though since it will be spontaneous. 

I’m so nervous and excited for that part, but seriously so at peace that I know she fully supports the idea and that if she moves toward a c section, its because I truly need another c section. She did confirm during my cervical check that I was not dilated yet (good thing since we’re not quite ready yet) but that he was low and she could feel his head, which at this point was excellent because this is what would begin to soften and efface the cervix when it was time. She would prefer I spontaneously go into labor on my own but was open to inducing if we should reach that point. 

The last appointment of the day was for the echo and to see the cardiologist. They did a full echo, lasting about 45 minutes. Nolan and I kept both drifting off during because she was super quiet. She said that while she did the scan, the cardiologist was watching from a different room. I noticed she went back over some of the same areas over and over, very very slowly. I remember wondering if the cardiologist was asking her for second and third looks at some things. Afterwards she left and the cardiologist came in. She said that Huck has a very complicated little heart, but that it is not hypoplastic left heart syndrome. She did a few scans of her own and then had us sit down for a consultation where she explained the complex anatomy of his heart and how we would proceed. 

She explained that he does actually have two ventricles – one on one side that is large and fully functional and another that is very small, sort of like a pocket ventricle. He also has both of the main arteries that are very important for function of the heart. Then she told us that the large ventricle, she believes is the left ventricle, making him have a ventricular inversion. (This explains why our first cardiologist was hesitant to commit to saying right or left.) This also means that the aorta is attached to the left ventricle and the pulmonary artery is attached to the right ventricle, which is called Transposition of the Great Arteries or TGA. He also has Tricuspid Artresia, meaning his tricuspid valve never developed, and he has a ventricular septal defect, a large hole between the two ventricles, making it look like one ventricle. I know this is super confusing and it still is to us too but this picture explains it pretty well. 

This diagram shows a “hypoplastic” right ventricle because the term hypoplastic means “underdeveloped” and he will still be considered (and treated like) a single ventricle baby. But in our case, if she is correct about the complex anatomy of his heart, he has the stronger of the two ventricles. The left is usually considered the “work horse” of the heart, having stronger muscle walls and capable of regulating blood pressure better than the right. 

So what does all of this mean? It means that he will for sure have open heart surgery at least twice in his life. But for now, pending anatomical confirmation at birth, he is clear of the Norwood procedure that happens almost immediately after birth. Reminder, the Norwood is the most complicated and severe of the surgeries and has the highest mortality rates. They said he will go to the NICU somewhat immediately following birth. I say somewhat because as long as he’s stable when he comes out, they will do his initial assessments in the room with us and that he would most likely be with us for around 30ish minutes before leaving for the NICU. Some of this has been a little strange to explain to people because the natural reaction is “we’ll won’t he be in critical condition when he’s born? I mean he’s missing part of his heart… and some of its backwards… and I just don’t understand.” And the best answer I can give that we’ve had to learn over time is that there are some unique features to a baby’s heart while they’re in utero, like some ducts and passage ways, that allow blood to freely move about the heart in a way that is actually “okay” for them at first. It’s not a perfectly functioning heart but it gets the job done. Those things remain open for quite a while giving care providers plenty of time to do whatever needs to be done. It’s all pretty miraculous. 

Where we’re at now is that my care has officially been transferred to Texas Children’s Hospital. I have appointments there once a week, but they’re usually 3-6 appointments crammed into one day since we live 2+ hours away. We won’t know much more about his heart now until he’s born and they’ll able to do a full echo on him to confirm the anatomy of his heart but if everything matches what they believe currently to be true, he may be able to wait a few months before his first surgery, making him much more stable and tolerant of the procedure. We will be temporarily relocating to Houston in a couple of weeks per the recommendation of our team of doctors at TCH and we’ll remain there until Huck decides to make his appearance. We’re not quite sure how long we will be there after he’s born but our prayer is that everything the doctors believe right now holds true and we know they’ll get us home as soon as it’s medically safe for him to do so. We definitely believe he’s in the best place possible now! 

Again, thank you so much to everyone for continued thoughts, prayers, and donations. There have been so many local fundraisers and it makes me cry every time I think about how everyone has helped. We can’t even begin to put into words how thankful we are for everyone’s continued generosity! 

Huck’s Heart | April Update Part 1


Trigger warning .. if words like mortality rates and information regarding insurance diagnosis codes or hospital outcome data scare you, I would tread carefully through my post. It is all mostly good news for us though. 

One thing I can tell you for sure, being pregnant with a baby that has a critical congenital heart defect, you get to experience ALL the emotions. We were initially told it was “probably nothing, but they needed to check to be sure” and then we were told there was definitely something “not right” so we were sent to a maternal fetal medicine specialist to take a better look. You can read a bit more about all of that here.

From there, Huck was diagnosed with hypoplastic left heart syndrome or HLHS. And then the endless researching commenced. We read things about how 20-30 years ago, this was considered fatal. And that even now, there are still babies that die from it. Some die from not knowing they have a defect so we were so thankful that we knew about Huck’s so early, but some babies die much later from surgical complications or illness that affects them too greatly. The fact that data exists for each hospital on the mortality rates for the first surgery, the Norwood, was scary. Would our baby be one of those numbers? It was terrifying territory not really knowing the life ahead for our baby. 

As mentioned in the first update, we had an appointment with the pediatric cardiologist and he confirmed HLHS and we talked a bit about the plan. Some of you know I lost my job last year when the oil field market plummeted and I was the one that carried insurance on our family. We were forced to obtain insurance through the marketplace just so that our family didn’t go without insurance. We pay a lot of money for very little coverage. Without going on a very emotional political rant and to maybe make a long story a tiny bit shorter, we started finding out that we had some pretty intense insurance network issues. We were sent to a specific maternal fetal medicine and pediatric cardiologist because they were in network but the recommendation from the pediatric cardiologist for standard HLHS care would be at least 3 open heart surgeries, starting the week Huck was born. He said that we would deliver at the hospital he was associated with, UTMB in Galveston, and then Huck would be transferred to Memorial Hermann Children’s in Houston which is affiliated with the UT medical system. UTMB and UT are contracted with each other. I made phone call after phone call ensuring that this was the best option for us and to make sure of who we were/weren’t in network with. I was told we were in network with both UTMB and Memorial Hermann. So we proceeded down that path. 

Now, as a heart mom, you start to learn a lot about hospital rankings and why they are ranked the way that they are. You also see the scariest things like the hospital outcome data that I mentioned. Texas is so blessed to have the #2 ranked hospital in the United States for pediatric cardiology and heart defects, Texas Children’s. It was assumed by pretty much everyone (including us) in the beginning that with such a serious defect, we would be going to Texas Children’s… until we found out we were not in network with them. Again, I made phone call after phone call and even got some help from some family friends that are on the board of directors but it was determined that if we were in network with another children’s hospital that could perform the surgeries, hospital ranking didn’t matter to insurance. We had to go where we were in network, just to simply be able to come out of this not millions in debt. (Yes, millions. Literally.) 

We ended up researching our specific surgeon and found that his credentials were excellent (he came from the #6 ranked hospital in the US for pediatric cardiology) and we loved our current pediatric cardiologist we’d been seeing. We decided it would all be okay, we were placed on this path for a reason, and we felt confident that they would take the best care possible of Huck. We also decided that we didn’t want to do a transport at all so we were going to go ahead and deliver at Memorial Hermann, so that Huck would be where he needed to be. 

And then another bombshell happened. We found out that there was some kind of misunderstanding during all of those calls and were NOT in network with Memorial Hermann either. Come to find out, the insurance plans offered in the marketplace exclude coverage for care at tertiary facilities… like children’s hospitals. We were not in network with a single children’s hospital. Not a single one. I was not prepared for the emotions of having our baby be diagnosed with a critical heart defect but I was damn sure not prepared for the road blocks we were facing. I had no idea what to do from there. I cried ugly ugly tears that day. 

So I started making phone calls again. Phone call after phone call. I was getting pretty good at making phone calls. I made 43 in a single day one day. I finally figured out that in order for Huck to have his necessary care, we would have to request out of network coverage for a tertiary care facility. At this point, we realized if we were about to go down this path, we might as well go for Texas Children’s. So I reached out to all of my previous contacts and got more information and tried to help my doctors office coordinate with my insurance company to get the referral done correctly. Everything seemed to be moving in the right direction. Then we found out that our current pediatric cardiologist had not officially made his recommendation for care. He normally does that after the follow up echo, which we were scheduled for the week following all of the insurance chaos. So it was decided that we would see him for our follow up and get his official recommendation for care in order for the insurance referral to be finalized. 

On March 30, we went to our follow up echo, hoping for at least no change. We were praying that they didn’t see any additional defects or any less function in his heart. We were met with good news! They were able to see a bit more on Huck’s left side than they originally thought. Originally they could not see a significant left ventricle, and could not see the aorta (the main artery that comes out of the left ventricle). This time, they could see both main arteries but they looked to be feeding from a single ventricle. It was explained to us that his potential diagnosis could change. (Side note, all diagnoses given during pregnancy are considered potential because he won’t be officially officially diagnosed until birth and they can get a good echo on him.) He said that instead of hypoplastic left heart syndrome, we could be looking at a variation of double outlet single ventricle, and that he was leaning towards saying it was the right ventricle, (DORV) but wasn’t quite sure. (Remember this part – the fact that he couldn’t commit to saying which ventricle other than it looked to be a single ventricle with a tiny second ventricle was a bit of foreshadowing on what came later.) He said this doesn’t mean anything major except that the urgency of Huck’s first surgery may change. HLHS babies absolutely need surgery within the first week to be able to live, but DORV babies can sometimes wait a few weeks and in less extreme cases, a few months. That would all have to be determined for sure after he is born. But we left that appointment with two things accomplished – we were so glad for even the slightest improvement in potential diagnosis and we also got his recommendation for birth and surgeries to be performed at a tertiary hospital. We got the phone call from my OB’s office that afternoon that the recommendation had been submitted to insurance and that finalized the first referral. And then we waited.

To be continued…