Huck Inlow | 4 Months


Late posting, as usual! But in our defense, with Huck’s last surgery and recovery, things have been a little hectic! 

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

He is wearing size 2 diapers and currently not wearing clothes since he just had his Glenn surgery. Was wearing 3-6 months right before though! He has almost completely outgrown 3 month onesies. 

Eating: Huck was eating anywhere from 120-150 mls per bottle, every 3-4 hours up until the point he went for pre Glenn cath lab at around 3 1/2 months. After that, he developed necrotizing entercolitis (NEC) and was put on bowel rest and antibiotics for 14 days. They ran IV nutrition during that time. He was released to restart feeds the Sunday after his Glenn and we started with 15 mls and are up to 30, but so far he is not tolerating his feeds very well. He has taken 7 bottles in the last 24 hours and puked up the entire volume of 3 of them. Eating is the main thing he has to figure out again post op in order to be able to go home. 

Sleep: Again, pre Glenn, he had a pretty decent routine, but it kind of all went out the window post op. He is starting to sleep more at night and nap less during the day, though. He still sleeps a lot in the mamaroo which helps his reflux, but will also sleep pretty decent in his bed at a pretty steep incline. I’m still brainstorming ideas for sleep when we go home.. pretty sure we will be getting a mamaroo, but trying to think of other options too. We had an arms reach co sleeper that I’m trying to figure out if there’s a safe way to elevate the head of it. If you have any ideas, let me know! He will be sleeping in our room for quite a while because I’ll be a nervous wreck for … oh, the next 18 years. 

Milestones: He rolled belly to back a few more times before he went back on chest precautions post op, and when he’s on his back, he rocks really hard to one side. Wants so badly to flip to his tummy! Has really great head control and can sit unassisted for up to 20-30 seconds!

He also went through his first hurricane. We are still in Houston, so we were here throughout Hurricane Harvey and it was pretty intense here for a little bit. 

Medical Milestones: He had his Glenn on September 7! This was his second open heart surgery. Post op recovery is going well! He was extubated the day after surgery and has been on room air since then. We have been working on getting back up to full feeds so that he can get off tpn and lipids and hopefully go home! His reflux is back with a vengeance so we’re trying to work around that. He is still on lovenox shots, clonidine, a diuretic, reflux meds, diazepam, and methadone for opiod withdrawals, but we are actively weaning methadone. They turned milrinone off a few days post op. So far, labs look good and indicate a successful Glenn! 

Currently satting in the high 70’s to mid 80’s, heartrate is 120-150, blood pressure is usually 90s/40s. 

Likes:

  • Being held / rocked / snuggled
  • Mamaroo
  • Watching videos of sister 
  • Watching movies, really likes Sing 
  • Being tickled
  • Listening to Elvis 
  • When you blow kisses at him
  • The lights that reflect off his monitors
  • Warm bottles
  • Chewing on toys
  • Field trips out of the hospital room

Dislikes:

  • Diaper changes
  • Picc line dressing changes
  • Baths
  • Lovenox shots
  • Getting his blood pressure taken
  • Getting his temperature taken
  • Being listened to with the stethoscope
  • Pretty much anything hospital related anymore, haha
  • Pacifier
  • Being restrained in any way

Whew, another month down. 123 days in the hospital and 128 days in Houston.. we are so ready to go home. We basically missed the whole summer being down here with Huck and we’re ready to get out of here, get back to the country, and enjoy the fall. Even though our stay has been long, I couldn’t imagine how things would be if we were anywhere else but TCH. We’ve now seen what happens with babies that start out where we were supposed to start out (that’s a story for another day), so that alone makes us thankful to be here, but we are also so thankful for how so many people have taken what seems to be a special interest in our boy and our whole family. I’ll actually miss a LOT of people when we finally go home! 

Huck Inlow, you are so loved! I hope one day I can explain to you how much not only your daddy, sister, and I love you, but how much our entire community and family and friends and nurses and doctors and PCA’s and therapists and complete strangers love you. You have so many people supporting you and we have been shown so much kindness from all of those people. I hope you always know that kindness. And I hope you know how many people have rooted for you, prayed for you, and fought for you. You have love from and support from all over! 

Happy 4 months baby boy! We can’t wait to show you the world! 


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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’s Heart | Counting Chickens 


What you don’t see in these pictures – Huck basically hugging my hand with both of his hands, this momma losing her mind, sobbing uncontrollably over what might be happening, and the team of doctors flying around us setting up machines, placing lines, and making a lot of very fast paced decisions. 

Earlier this week, we started the discharge process with plans of getting to go home today (Wednesday). It’s basically the same situation with switching from CVICU to step down unit; they have a single ventricle checklist to make sure he’s ready to go which is really just a lot of testing, blood work, and regular baby milestones. When we left CVICU the first time, a week ago, one of the checklist items was an echo (an ultrasound of his heart if you’re new around here), which looked great, so off we went to our new digs. To get discharged, it’s a similar process – he had to gain weight appropriately, eat a certain percentage of his bottles, get an echo, get a brain MRI, get a chest X-ray, we had to get various education sessions, pass a car seat test, I mean really the list keeps going. But these items are in place for a reason and thankfully, Huck had his “go home” echo yesterday afternoon, just to make sure his heart was functioning okay. 

While we waited on the echo to be read, we proceeded with the next item on the list, his car seat test, which he passed! This is just a test where they make him sit in his car seat for two hours and make sure he won’t lose oxygen or stop breathing or anything strange. 

While he was getting car seat tested, Nolan went to get us some dinner from Cliff’s Grill. (Which I highly recommend if you’re in the Medical Center area. Their southwest burger is amazing!) We hadn’t even taken the first bite of food when suddenly, half of his single ventricle team came walking in the room. 

They explained somewhat quickly that between the last echo a week ago and the new echo, they noticed part of his single ventricle was not squeezing the way it should. They called it “mild to moderate”, with “severe” basically being heart failure. Thankfully it was noticed before the point of heart failure, and additionally all of his stats and monitoring were considered perfect and he acted completely normal and calm, which they said was all very confusing to them. They wanted to do a CT scan to figure out exactly what was going on. They thought there might be an issue with one of his coronary arteries which would have been indicated by the ventricle not squeezing properly. They explained that for the CT scan, they had to do a breath hold to get the perfect picture, which means he had to be reintubated so that they could manually make him hold his breath. To do all of that, he would also need to be sedated, so via standard protocol, they called in the rapid response team to transport him back to the CVICU. 

At that point, we were told a broad spectrum of what to expect – this could end up being nothing, if there was something wrong with the artery, they could possibly do a catheter procedure to fix it, if there is a major problem (which they did say was unlikely based on his appearance, stats and behavior), they’ll have to take him back into surgery, open him up and fix it. We knew in the back of our minds that he could also crash at any time during this process if there was truly something wrong. So I stayed with him the whole time – while they transported him, sedated him, intubated him and took him for his CT scan. I will truly know this kiddo inside and out before he’s even a month old. 

The CT scan was very quick. Apparently TCH has a top of the line CT machine and people come from all over the world to use it because it captures things in mere seconds. Huck’s entire heart function was captured in less than 5 seconds. They told me in the CT room that the radiologist was at home but waiting on our scans to come through and would read them asap and get back to us within an hour or so. 

About 15 minutes after we got back in the radiologist called and said his CT was perfect! Our baby boy has pulled out the stops once again! I cried for the hundredth time that evening, so thankful that he was okay. They extubated him late last night when the sedation started to wear off and he was put back on an oxygen cannula for the time being. He is doing well right now, a little grumpy and uncomfortable but I’m hoping he’s back to his normal sweet self very soon. 

We are incredibly thankful for the team of people at TCH that double check and triple check before sending you on your way and for our surgeon and his team who are absolute angels for saving our baby boy’s life. My sweet husband also deserves some recognition for being my rock through all of our tough times. He has taken on our wild Whitlee so that I can be with Huck right now and he’s managed to keep his sanity through all of it, while keeping me in the right mind set too. 


We’re currently waiting on the doctors to round so that we can find out exactly what they think they saw, why it’s okay now, and what the plan is going forward. This was a temporary setback in our journey home, but I can’t even tell you how relieved we are right now after a whirlwind emotional night. 

Huck’s Heart | Two Weeks Post-Op

Huck was two weeks post op on Tuesday evening, so I thought I’d do a comprehensive post on where we’re at, how he’s doing, how we’re all feeling about things. I normally do a postpartum update for myself but it seems my blog has been getting a little more traffic than normal so I’ll spare the talk about my after birth pains and perineal stitches. (Which have subsided and all is well in that department if you’re interested.)

We are so happy to report that everything has been going really well. It’s been such a blessing to have him progress each day. At one week, we were down to one central line left (a neo picc) that he is currently getting one single IV medicine through. He started with two umbilical artery lines plus three additional artery lines – one in his groin area, one in his wrist, and one in his ankle but they’ve taken them all out one by one. And he finally got his chest drainage tube out late last week!

As far as feeding, they started feeding him milk through an NG tube a few days after surgery to make sure his tummy could tolerate it and then they had occupational therapy come work with him on taking a bottle. A lot of heart babies will have feeding issues because there was a delay in feeding, because they developed an oral aversion from intubation, or sometimes because there was temporary damage to the nerves that control swallowing. In Huck’s case, he is doing exceptionally well (doctor’s words) and making beautiful progress (also doctor’s words). By day 3, he was already attempting bottle #5 and was taking about half of it before he exceeded the time limit and they put the rest in his tube. At 14 days post op, he was taking all feeds by mouth and was up to 50 ml. On day 15, he pulled his ng tube halfway out on his own and they let him leave it out. And then on Wednesday this week they gave the green light for him to start trying to breastfeed. The timing was terrible though so he was a little too sleepy after 7 attempts earlier in the day at a blood draw (s e v e n!!), but we tried again the next day and he did really well. Eventually I’d love to just breastfeed him for convenience but we may be alternating fortified breastmilk by bottle with actual breastfeeding. 

Back to it – so late last week the single ventricle team in the CVICU started their checklist of items to approve him to leave and go to what they call their step-down unit. Then on Tuesday, at 2 weeks post op, they gave the all clear. We are officially out of the ICU!

In the step down, Huck has his own room and this is basically treated as his transition before going home. I’m actually staying in the hospital with him and learning all of his care and more about his temperment so that we know more about how he tolerates things and how he normally acts. I have actually done all of his feedings, diapers, comforting, and oral meds by myself for the last three days, aside from earlier today when I had to run and take care of some stuff in preparation for us leaving the hospital. 

As for going “home”, a lot of it will be dependent on him and how well he progresses with weight gain and taking his feeds by bottle in a timely manner. That, plus straightening out a few arrhythmia issues is our current hurdle but that part is kind of trial and error, treat and wait to see if it’s fixed. But I always say “home” because in our case with him having a Norwood surgery, they’re 99.9% sure he will not go home home until after his second surgery, which is scheduled for mid September. They want us to remain close for what they call the “interstage” period, the time between the Norwood and Glenn surgeries. But they have talked more and more about us leaving the hospital the past few days. They also needed to take him off one more of his medicines, which they did two days ago, and then decrease and transition the last IV medicine to oral, which they did yesterday. He also has to continue to gain weight and not have any other hiccups, but they are tentatively planning for us to go home early next week. Fingers crossed!!

A lot of people have asked how we’re doing and I can say for the most part, we’re okay. We are deeply thankful for how well he’s doing and the progress he’s made every day. But I still have days where it’s hard. Every time we change the new routine, I have an adjustment period in my emotions. Nolan went back to work, I cried. We switched to the step down unit, I cried. I’m sure when we finally get to go home, I’ll cry (although that time may be more from relief). I don’t really do any of that in front of people though.. I tend to have my breakdowns in private, thank God. Just don’t ask me if I’m okay lol.. you have about a 50/50 chance of my eyes filling up with tears before I can answer. I sort of just get a little overwhelmed occasionally until we adjust our sails and move forward with whatever new normal we’ve been given. I think one of the hardest parts is navigating how to be the best mom I can be to both kids. Whitlee came down with a mystery virus last week and thankfully she was at my moms house and we weren’t exposed to it. But we had to leave her with my mom and then my in-laws until she was symptom and fever free for a considerable amount of time before she could come back. I hated not being able to snuggle her while she was sick but we have to be extra extra careful right now; a common cold or stomach bug could be life threateningly dangerous for Huck. Thankfully she came back to Houston a couple of days ago and I am so happy to have both kiddos in one place again. 

So now we’re just learning how to take care of our special little boy and learning his habits so we know what’s normal for him and what’s not. We’re getting a lot of education the rest of the week in preparation and we’re just praying he stays on the right path to get to go home soon!