Our sweet Liam was born with a very rare congenital heart defect called Hypoplastic Right Heart Syndrome (HRHS) and Pulmonary Atresia. Typically a set of several open heart surgeries over time can fix this defect. After Liam’s first week of life, his heart showed signs of serious distress. After several procedures, a new diagnosis was made regarding his coronary arteries. This changed the course of his treatment as we learned this is not repairable. They considered him critical and listed him as status 1A on the heart transplant list, the highest priority. We were told that Liam can’t go home and has to remain in the hospital until transplant because of the risk of having a lethal heart rhythm that could result in sudden death. He needs to continue to be monitored closely until a heart becomes available. The typical wait time for the heart of a small baby is 4-6 months.
Over the past few months, so many of you have been asking how you could help us. Thank you. Many of you know that Josh is in his first year of PA School and I am in my 13th year as a NICU nurse. I intended to go back to work after my maternity leave ended. Since we have had to shift gears from the surgical to the transplant path, I can't imagine leaving Liam in the middle of the process and returning to work in the very near future, leaving us in tough spot financially. Our most practical need and a deep concern of ours is the temporary replacement of these financial demands and the full cost of maintaining our benefits until after Liam's heart transplant recovery.
Our precious little warrior has already been so strong, and he has only just begun his lifelong journey related to his congenital heart defect. We can't thank you all enough for the outpouring of love and support and constant prayers during this difficult season in our lives. There is so much unknown on the road ahead. There are times when fear creeps in and there's so much about this situation that we don't understand, but our story is a story of faith and not a story of fear. We know that God is with us every step of the way. He gives us strength as we need it and His plans are for good. There really isn’t a way to express the deep thanks that we have for the support that we have already received. We are thankful to have every one of you alongside us on this journey.
I am going to tell Liam’s full story below for those who would like a more in depth update on our journey so far. We will be sure to provide updates here as we are able. Thank you for believing with us!
“When you go through deep waters, I will be with you."
Liam’s journey since birth
On September 16, 2022 Josh and I went in for our anatomy scan so excited to see our baby boy and how much he had grown! Everything looked great until the tech got to looking closer at his heart. After scanning it more in-depth for what felt like hours, she felt the need to go out and show the images of the heart to the doctor. Josh and I were left in the dark ultrasound room for about 30-40 minutes just waiting, trusting, and hoping it was nothing serious. After the doctor came back in she said our precious baby had a very complex and serious congenital heart defect. We were told that he had hypoplastic right heart as well as pulmonary atresia. She went through percentages of survival and talked about terminating our pregnancy because of how significant the defect was. We left the hospital heartbroken. Confused, yet hopeful - or trying our best to be.
She referred us to a pediatric cardiologist who did a fetal echo on him which did confirm that he did indeed have a hypoplastic right heart with pulmonary atresia. 4 out of 100,000 live births. The right ventricle was extremely small and the pulmonary valve didn’t form. He and I were followed closely throughout my pregnancy to monitor the growth and size of the right ventricle and his heart valves, vessels, etc. In any circumstance, we were told that he would need surgery after he was born, but depending on the growth would determine the number and level of treatments and surgeries that he would need. We were told that we would be in the best spot if we saw that right ventricle grow in size with him.
Josh and I had been struggling to find a boy name that we agreed on. As we were in that dark room waiting for the doctor to come back in, we looked back over our list of names. We decided that we both liked the name Liam. We looked up the name and saw that it meant "Strong-willed warrior; Guardian; Protector". It was perfect.
They induced me at 39 weeks on the night of January 16, 2023. Liam quickly let us know that he could not tolerate the induction process. He had significant decelerations in his heart rate. The decision was made to go for a c-section first thing in the morning. Liam Joshua Wahl came out pink and crying at 8:49 am on January 17, 2023 weighing 7 pounds 7 ounces! His primary NICU doctor said that he looked so good that if she didn't know he had a congenital heart defect, she would have sent him to the newborn nursery. He was stabilized in the NICU at West Penn by my amazing co-workers. They placed central lines and started a medication that allowed him to still get oxygenated blood to where it needed to go, then he was taken by ambulance down the street to Children's Hospital’s cardiac ICU to determine the next steps. That night they did an echocardiogram and CT Scan which confirmed the prenatal diagnosis.
A week later, Liam had his first trip to the cath lab. They were able to get the stent into the PDA and the right pulmonary artery to feed blood to the right lung. They put a balloon through the left pulmonary artery because it showed constriction. The surgeon warned us that it would be complex and an abnormal route because of his specific anatomy. The procedure was successful praise God. We were told that the right ventricle can’t be used at all for circulation to get oxygen from his heart to his lungs. We were also told that the stent would help him until he grows enough to have open heart surgery in a few months to perform a longer-term solution with an additional open heart surgery at several years old.
On February 1, 2023 Liam was taken to the cath lab again due to some concerning episodes. During that time, a new diagnosis was given that ended up changing our entire course. They noticed that he had right ventricular-dependent coronary circulation and narrowed/interrupted coronary arteries. This prevents necessary circulation and we were told is not repairable. At this point, the medical team determined his status as critical and deemed it necessary for them to begin the transplant listing process asap.
On February 8, 2023 he was listed as status 1A, the highest priority listing. We were told that it would not be safe for Liam to be discharged home until after his heart transplant because of the risk of sudden death. He would need to remain in the hospital throughout the entire wait time, which they estimated to be 4-6 months.
Over the next week, Liam's episodes got more severe. He had no reserve. His oxygen levels revealed he was struggling and his heart was experiencing extreme and at times dangerous stress. This led to a third visit to the cath lab where they inserted an additional stent into Liam's right pulmonary artery, as well as a new stent into the left pulmonary artery. The next few days were difficult as he developed a blood clot, battled an infection and remained on the ventilator. His vitals were concerning over these 48 hours. After many interventions, thankfully, he stabilized.
Since then he has remained on a steady, stable course. The additional stents proved to take the stress off of his heart and he is now in a phase of eating, growing, and waiting for his life-saving heart. There are times when he looks so well that we can't even believe he is sick. However, the doctors remind us that his heart is very fragile and he needs continual monitoring in the event of anything changing at any time before his new heart becomes available. Our prayers now need to be that he can stay stable as we wait and that God would provide the perfect heart for Liam!