Happy Heart Month!
To close out Heart Month I want to share with you Huck's story! Huck was just a few doors down from us when we were on the 15th floor; AKA: the step-down unit. His story and ours have many similarities and... he is so stinkin' CUTE!
To follow Huck's journey, check out his Momma's blog- Southernly Ever After.
We found out we were expecting our second baby when our daughter, Whitlee, was almost two and found out at 16 weeks (selectively) that it was a little boy that we decided to name Huck.
How we found out his diagnosis was a little dramatic. We went to our 20-week anatomy scan, not expecting much action since we already knew he was a boy, which they confirmed. A couple of days later I got a phone call from my OB’s office saying “they weren’t able to get a good picture of his heart, probably just a positioning issue, probably just because he was so active, happens all the time” and they wanted to scan again . In that two weeks, I googled countless images of heart ultrasounds. When she started scanning, I was definitely not seeing what I thought I should be seeing, but I didn’t rely much on my two-week google education, so I asked her, “do you see four chambers?” I knew ultrasound techs aren’t allowed to say much, but they can usually answer yes or no anatomical questions. All she said was, “You’ll have to discuss your results with your doctor.” So I asked again, “Do you see four chambers or not?” She gave me the blankest stare and said, “You’ll have to discuss the results with your doctor.” I was panicking at this point so I said: “Then go get my doctor.” She told me my doctor was unavailable and that I needed to leave, that they would call me with results. In my state of panic and hormones, I made a scene and refused to leave until they told me what was wrong with our baby. My sister-in-law was with me and she said it was like something from a movie. Slightly embarrassing now, but it got me answers. My doctor’s RN took me to a room where she explained that they couldn’t see all four chambers of the heart and they were sending me for a level 2 ultrasound and an echo as soon as they could schedule something.
We had that appointment in Galveston at UTMB, where they diagnosed him with hypoplastic left heart syndrome. We were devastated. I can’t even think about that day without crying. We had an appointment with a fetal cardiologist the next week, where he confirmed a diagnosis of hypoplastic left heart syndrome and gave us the rundown on the plan of care. He told us I would deliver in Galveston at UTMB, and they would transfer Huck to Memorial Hermann Children’s via ambulance and I could join him when I was discharged. Something about this whole scenario just rubbed me the wrong way so we pursued a second opinion at Texas Children’s. We faced a mountain of insurance-related issues, but after a long hard fight, we finally had an appointment at the fetal center in the TCH Women’s Pavilion at 33 weeks. I delivered our daughter at 34 weeks due to preeclampsia, and I was so nervous Huck would come early and we wouldn’t have proper plans in place. Our fetal cardiologist (who is now our pediatric cardiologist), Dr. Lawrence, immediately informed us that he actually didn’t have HLHS. She said he had a rare combination of several different defects. He has tricuspid atresia with a ventricular inversion (his ventricles are swapped), which resulted in a hypoplastic left-sided right ventricle. He also had a hypoplastic aortic arch, congenitally corrected transposition of the great arteries (due to the ventricular inversion), a VSD, an ASD, and an abnormally large PDA. She said most likely his surgery plans would stay the same but we’d have to wait until he was born to be sure.
I thankfully made it full term without preeclampsia this time, and Huck was born at the TCH Women’s Pavilion on May 13, 2017, and I was able to hold him and kiss him before his Daddy went with him to the CVICU. They even let Whitlee come in and meet him before he left.
We had our sit down with our surgeon, Dr. Mery, a couple of days later where he explained with Huck’s anatomy they had some options for the first surgery, but after that, he would be treated as a single ventricle baby and would have a Glenn and later a Fontan. He said he wasn’t sure what surgery he would do until he was in there because he couldn’t get a good measurement on the echo on how restrictive his arch was and how large his VSD was. If he could rely on the VSD to stay open, it gave them options, which included a PA band or an arterial switch. Ultimately he went with a modified Norwood with a double barrel DKS and BT shunt. They also reconstructed his arch while they were in there. Huck had that first surgery at 3 days old, and after nearly 12 hours he came out with flying colors. His recovery went really well and we were sent to the step-down unit a couple of weeks later in preparation to discharge to stay in Houston until his Glenn.
The day before discharge, we were checking off all the things - we had our go home echo, did our CPR training, had our single ventricle training and tools to take home, had meds filled and delivered, and Huck was finishing up his car seat test (which he passed), when his team walked in. They said his echo had shown that his ventricle wasn’t squeezing properly and they were going to RRT him back to CVICU, this time to the heart failure unit. They wanted to intubate and do a CT to check his heart, specifically his coronaries. Everything came back great. The next day they extubated him and put us back on track to go home again. They decided that day during rounds to keep him CVICU one more night before sending us back to the floor. He coded that night. And again . Both times, it was because he got mad and instead of his heart rate rising like a normal person would when they’re mad, he would just crash. Thankfully both times they were in the room when it happened - it’s hard to ignore a Huck scream - but they were able to react quickly and save him. His second code landed us a permanent stay in CVICU for the duration of his interstage. Thankfully the next few months were mostly uneventful, we just had a rough time trying to keep him calm and we dealt with a round of NEC watch with bowel rest and reoccurring blood clots from his PICC line. Anytime he had a rewire, he got a new clot.
Huck’s Glenn was on the books for . Hurricane Harvey hit Houston from about , so we were looking at having to postpone. After his pre-Glenn cath lab a few days prior, he was so angry, more than normal, so they started investigating why. They found pneumatosis on an X-ray, which is highly suggestive of NEC. They diagnosed him as stage two NEC this time, which earned him 14 days of bowel rest. This postponed his Glenn for sure, but it finally happened a couple of weeks later. Everything went smoothly and we were shocked at how fast Glenn recovery happens. Once Huck overcame some eating issues from bowel rest and surgery in general, we were finally discharged home at the end of September! After six long months of Houston life, we were so excited to go home. We had been living in a travel trailer at the Lakeview RV Park, shuffling Whitlee back and forth between grandparents and us, and my husband had been driving back and forth for work every day - two hours each way.
Huck came home on seven medications, including Lovenox shots for the clots, and at 9 months old/5 months post op, we are so thankful that we’ve been able to wean him off of everything except half a daily aspirin, which his cardiologist says he will be on long-term. Stopping Lovenox shots was a celebrated day! We just got released to cardiology visits every 6 months and Huck is thriving at home, meeting appropriate milestones, which we are so thankful for. His ventricle still doesn’t squeeze 100% properly; it has an area of mild depression but has remained stable. We’ve been told it may regain full function at some point or it may stay the same.
I truly believe everything happens for a reason. I don’t know God’s plan for why we’ve been through this and I may not ever, but I know he has a plan for our family. Oddly enough, our neighbors a few houses down just delivered a baby with HLHS at TCH (Hi, G family!) and we were able to help them prepare a little ahead of time with what to expect and some of the things we wished we’d known ahead of time, so that makes me feel useful, like some good comes out of it. I hope in the future, we can always focus on the good and continue to make every day we’re given with Huck count.