This blog is dedicated to my 2 year-old son Ian who was diagnosed with HLHS in utero, and to the thousand parents that find out each year their child has HLHS (Hypoplastic Left Heart Syndrome). I put a FAQ section at the bottom with practical stuff.
When I found out our son had a broken heart, my heart broke too. Why did God allow life to put such a heavy cross on such a little boy? It’s not fair. But it is what it is, and we will do what we must. It is like running a marathon, but not knowing how many miles till the finish line having to push yourself to keep going. Currently, our son is doing awesome in every sense, and no one would guess what he’s been through.
Did you just find out? Are you anxiously awaiting the arrival of your little heart warrior? I know how you’re feeling, I’ve been there. I want to offer you hope and encouragement. You are going to need to hunker down and muster up as much strength and courage as you can. Your heart warrior is going to be physically fighting for his/her life. You are going to be fighting an emotional/spiritual battle of your own. You will get overwhelmed and frustrated at times. It is going to be an intense roller coaster, kind of like one of those insane ones where it is pitch black and you don’t know when the drops or turns are coming! But you will be so proud of your little fighter! You will feel blessed to call him or her yours.
The good thing is, even though this is your first “ride” the medical team has been on this wild ride many times before and there aren’t many twists or turns they haven’t seen before. This is why it is important to go to a center where you have complete trust in their experience. When Scott (hubby) and I first found out, we spent weeks agonizing over the decisions regarding the how- Hybrid vs Norwood; and where- CHLA, Lucile Packard, Las Vegas, or Spokane (our cardiologist did not have any strong recommendations, but he did say if it was him, he would go to an elite program). We live in a state that does not have any pediatric cardiothoracic surgeons so we had to go out of state. I am an optometrist, so with my medical background I spent countless hours and evenings reading journal articles, taking notes about IQ, survival rates, ADHD, the Sano Shunt vs the BT shunt, etc. My husband read blogs and figured out how to get double coverage for the insurance and make sure we had access to Life Flight. We looked at the breakdown of the rankings and ratings of the US News & World Report for Pediatric Hospitals and Cardiac Surgery, http://health.usnews.com/best-hospitals/pediatric-rankings/cardiology-and-heart-surgery and even called a few centers making inquiries about Interstage Programs (Interstage is the time between the Norwood and the Glenn- the first two stages of surgery, Interstage has the highest risk of death, in our program a nurse called weekly to check in and we monitored oxygen saturations 3x/day. This was a huge help as our local doctor was unsure why he had such saggy sats and he ended up on continuous oxygen, where the nurse from the big center suggested checking his hematocrit and if low to give him a blood transfusion. Turns out the blood transfusion worked wonders and he no longer required oxygen at home!)
Every single heart warrior has a different journey. But I think unanimously every journey has some smooth places and some rough patches, REAL rough. When we first googled “HLHS” the first thing that popped up was an obituary of a 3 month-old girl from the town we lived in that had died a few months prior. In case we didn’t know it was serious. Ouch. I know nothing involving the particulars other than she went to the small center and was on the transplant list. What was helpful for us was not to focus on the all the negative “what ifs.” There are just too many of them. We avoided social media groups that sometimes tend to focus on all the struggles. We were able to get in touch with a few different families and talk to them. And received a care package from Sisters by Heart. We knew there was a chance he might not make it, but we didn’t see much merit in entertaining that line of thinking at this point.
The majority of kids make it. There are HLHSers that are in their mid 30s. Their IQ is typically in the normal range. I’ve seen a picture of an HLHSer that became a pediatric cardiac nurse, and a young man who got accepted into medical school. Everytime fear or panic would creep in we would try to remind ourselves to keep our eyes on the prize. Despite the rough patches we were getting one of God’s greatest gifts-a baby! And though these first few years would be so traumatic we’ll never forget them, luckily they are times he will never remember!
While we were in the hospital, I read an article about the keys to happiness. One point really resonated and that was to focus on what you have, and not to dwell on what you don’t have. Makes sense, I remember when learning my prayers as a child you first thank God for all the blessings in your life and then you ask Him for your petitions. We stayed at the Ronald McDonald House near the hospital. While there I met a dad whose son was born with 11 broken bones and would likely be a wheelchair-bound dwarf, it gave me a stronghold on perspective. There were also several families with children who had different sorts of deformities, some facial. And I think we all know people who struggle with infertility and how unimaginably hard that would be. My son is beautiful and whether God gives us days or decades we are going to count each day as a gift and learn to enjoy the moment! I strongly recommend “Welcome to Holland”http://www.our-kids.org/Archives/Holland.html a short essay that is so applicable. We didn’t pick this journey, but that doesn’t mean it won’t be full of beauty, joy, and laughter. We appreciate those things all the more because of the hardships we bore to get here. I heard a quote of Bear Wosniak today on the radio that said, “Life begins at the detour.” Seems fitting, since no one’s plans include something like HLHS. Like it or not you will never be the same and you will realize that you and you family are stronger and better because of it.
Sometimes it takes hitting rock bottom to really trust in the Rock. My husband and I have always been people of faith. But this experience brought us so much closer to God and each other, and I think that is why God allows such things. I have never been really afraid before our HLHS journey. Fear was just not a real thing to me, sure I worried plenty about tests and stressful work stuff, but I never knew anything that left me shaking with my stomach in such knots like this. Courage, of course isn’t that you are not afraid, but it is facing that fear. So I would try to punch it in the nose! I would scream-sing praise and worship songs if I felt like I was starting to despair. (Like this Lauren Daigle song) We put our trust 100% in God. We placed our whole family in God’s hands and had to walk by faith, not by sight. We had no idea how it would all work out, so many details. We have two older daughters we ended up being away from for almost a month with each of his two major surgeries. They stayed with my husband’s side for one stint and mine for the other. The girls handled it really well, they were 4 and 2, but once we were reunited you could tell emotions were running high. It was so hard for us to be away from them for so long! But when our son was hospitalized closer to home it was harder in many ways because it seemed like we were constantly having to tell them good-bye and tear ourselves away. It was easier for me to just stay with our son and for my husband to stay with our girls full-time and visit us, much less traumatic on everyone. It was really hard for me to be anywhere but the hospital when he was in the hospital, plus I was near constantly pumping. I even rented a hospital grade pump to keep my supply going as long as I could.
There are going to obstacles, but I hope that you are able to find like we did that when you give God your trust He gives you his peace. No one understands the pain of watching your son suffer like Mary, Jesus’ mother. So if you are in the practice of asking family and friends to pray for you, I would suggest that you also reach out to your Blessed Mother, she will offer her powerful prayers for you too! We have seen so many of God’s fingerprints throughout this journey. And although I wished we didn’t have quite so many twists and turns, God gave us a blessed assurance that He has a plan for our son’s life, not his death. I know all the prayers we received helped. We unashamedly asked friends and family members living and dead for prayers and for their whole church to pray too! One particularly rough time was when Ian came out of anesthesia not moving his left arm and barely his left leg and blind on the left side of his vision. It was scary not knowing if these stroke-like symptoms would resolve or not. But my husband kept his calm and could see so clearly that it did not matter. Our job is the same no matter what. If he comes home with an NG-tube, a g-tube, on continuous oxygen, or a hemiplegic our job is the same. Our job is to love that little boy 100% till death do us part. So we just poured our hearts out without reserve and always hoped for the best. I say it is okay to cry, tears are how our love manifests itself. If we didn’t care so much, the uncertainty wouldn’t tear at us so much.
Lastly, a note on mental toughness. Sometimes when you don’t feel strong or brave, you act strong and brave, and without realizing it you actually become it. I brought make-up and jewelry and got dressed everyday in the hospital. Look good, feel good, right? I found this works with being nice too. I find that I like myself much more when I am nice to people, even though they may grate on my nerves eventually I find I do have genuine feelings of kindness without the effort it took in the beginning. Of course, there are so many well-meaning people that just plain say the wrong thing. And then there are the people that don’t know what to say, say nothing, and leave you wondering if they even care. Personally, there is no better place to be than in someone else’s prayers, so I think the best thing to say is that I’m praying for you all.
You can do it! You will persevere! Sometimes you get through one day or one hour at a time. You will cry tears of joy and tears of sorrow and worry. No one said it would be easy only that it would be worth it. But know that no matter what happens there is a God that loves you and your child more than anything, so lean on Him hard. Lean on your family and friends. Lean on me! Leave your contact info in the comments and I will delete it before I approve the comment. By the way Ian did recover from his left-sided blindness and weakness a few days later! God is good.
Frequently Asked Questions:
- Where did you deliver?
- Although we initially assumed we would deliver down in LA (CHLA is where Ian had his surgeries) after discussing all the pros and cons with our cardiologist we decided to deliver in Boise and then life flight him to LA. From a medical standpoint it gave us the flexibility to change our minds about which hospital. For example, at Lucile Packard/Stanford they have surgeon renown for his work on preemies. Or if he had unforeseen complications with his lungs or something and we’d want to opt for the Hybrid instead we could go to Las Vegas or Spokane. Our cardiologist told us that the centers that are the best for the Norwood are not as well practiced for the Hybrid, and so if we wanted a Hybrid we’d want to go to a high volume Hybrid center. Practically, delivering at home allowed us to stay local with our family (other children) and jobs for ~3 weeks on the front end, since we knew it would be about a month with surgery and recovery after birth. This also allowed big sisters to get to briefly meet him before we flew off. This was good for their little minds to understand why Mommy and Daddy had to go, and heaven forbid if we lost him they’d at least gotten to meet him. I was also able to keep my OB/GYN for my 3rd cesarean and he is so amazing (my recoveries have been cake compared to others). Ian’s birth was really special, I was able to have a few moments of “skin on skin” before he was whisked away to the NICU. Easily in my top 5 best moments of my life!
- Where did you stay while he was in the hospital?
- One of the things we liked about CHLA was that they have an entire floor of heart kids with mostly private room accommodations. There is the CT-ICU (Cardio-thoracic Intensive Care Unit) and also the step down unit CV-Acute (cardio-vascular). The rooms have a bench that functions as a bed as well and a shared bathroom. The bench is really quite uncomfortable, and we really wanted to try to get good sleep to help ease our stress levels. So, initially we would stay with him till 11pm, but sleep at RMH then go back at between 7 and 8 am and he pretty much slept the whole time. Then as we were there longer we realized that the nurses are very attentive to his medical needs, but really are not disposed to do anything if he is crying unless it affects his vitals. We were kind of horrified that soothing a baby meant medication! So, we adjusted. We kept our room at the Ronald McDonald House ($25/night suggested donation if you don’t get financial assistance), and my husband and I alternated nights on the cot/bench and a real bed at RMH. That worked better, it kept us completely in the know at all times and able to soothe him.
- How long was he in the hospital?
- We were told to expect a month long stay for the Norwood, but that it can widely vary. It is about 2 week recovery from the surgery and then another few weeks to establish feeding. Our little guy Ian, ended up needing a “shunt revision” where two weeks after his first surgery (Norwood) they did another surgery to replace his shunt with a bigger one. As a presumed side effect, his left vocal chord nerve was damaged, this put him at a high aspiration risk (inhaling milk into his lungs instead of stomach) so after swallow studies (a swallow study is where they have the baby drink a dye while taking a video with x-ray) he was restricted to only being tube fed. At 6 weeks old we discharged on an NG-tube and a little over a month later we had a g-tube put in. So sadly, our little guy was unable to nurse because of his aspiration risk. When he did get to drink by mouth (~4mo old) it was thickened with rice cereal. On a side note: We also had to cross a bridge where we had to decide whether we wanted a Nissan fundoplication, most of the local doctors were recommending it, but we did not feel like it was necessary, since he didn’t have any reflux issues and that would be another invasive surgery wrapping the top of his stomach around the bottom of his esophagus. He was too small for laproscopic and would have a scar from nape to navel. I did research again and we decided against it, but the team encouraged us to wait to be sure, so that is why we waited the month to get the g-tube -to be sure he didn’t have a reflux issue and really need the fundo. We are so happy the way things turned out. Every journey is different!
- What did you wish you knew ahead of time that you didn’t?
- I had a young naïve nurse warn me about “delayed sternal closure.” I wish someone else had told me with more sensitivity to my feelings. Her timing and approach were terrible. It will be really hard to prepare yourself for what your little one will look like coming out of surgery. They look really rough, they have dozens of “lines” usually multiple IVs, drainage tubes (one for each lung and one for the sac around the heart). They will of course be intubated, generally through the nose, and then there are many many sensors. The oxygen numbers, “sats” for short, are some of the most important. But the most disturbing aspect is that in order to give the heart room to swell, because the swelling of injury to a muscle is delayed, it is standard procedure to leave the chest open post op. They will then sew together the sternum or breast bone and overlying skin several days later. They keep the babies very comfortable with some very strong pain meds, we rarely felt like Ian was in pain. Little by little the babies are weaned off of the support. We were finally able to hold him again after 5 or 6 days!
- I would recommend bringing things to decorate the room/bulletin board behind his/her bed. A lot of people had names in the window on little banners, etc. I didn’t have any good scrapbooking stuff, but made do! And although I thought I would spend downtime reading-it never really happened. It just took too much mentally to do that. So I found myself coloring pictures to send home to my 2 and 4 year old. Or buying colorful books and things off Amazon and shipping to RMH.
- Lastly, I never would have guessed how much I enjoyed having a CaringBridge page! One of the other HLHS families had used it to keep people informed. CaringBridge.org is a website that lets you enter journal entries and post pictures. This was critical to keep all our prayer warriors across the country informed. We had way more people than what I would have been friends with on Facebook or emailed easily. It was much less distracting than something like that, but could be easily share on social media too. People commented and followed along with his progress daily during the touchy times and even still I post updates and am humbled to hear how many still pray for him. No wonder he is doing so well! Search SebastIAN Naugle if you want to check out Ian’s page.
Feel free to comment anything! I know this got long, but I know when we were expecting, we really liked having as much information as possible.
Love and Prayers,