Our little daughter, Hope Joanna, was born on November 16 at 4:13 am. She weighed 7 lbs 2 oz and was 19.29 inches long. Hearing her cry when she arrived was such a wonderful sound and seeing her beautiful little face brought such joy.
After birth Hope was doing better than expected and I am so thankful for the moments we had together – moments I wasn’t sure we’d get to have. Natalie not only got to meet but also got to hold her new little sister and I even got to nurse her a bit.
As time passed since her birth, Hope has experienced some difficulties that, while expected, are still tough to watch as a mom. She went from needing no assistance with breathing or eating to being on higher flow air and getting all food fully through a feeding tube.
Through echocardiograms, x-rays, and monitoring, the doctors have confirmed the diagnosis for our little girl and started to make a plan moving forward. Hope has been diagnosed with heterotaxy and an unbalanced atrioventricular septal defect (AVSD). It’s crazy, to look at her on the outside you would never be able to tell how complicated she is inside!
Heterotaxy means that organs are in different places than they should be. In Hope’s case, her stomach is on the opposite side, her liver is displaced, her intestines are malrotated, and she does not have a spleen at all. The biggest concerns as a result of this are based on the malrotation and the spleen.
Since the spleen plays a big role in the body’s immunity, Hope is on amoxicillin daily and will continue to be for potentially 5 years or so. The malrotation may not be an issue, but can become one if the intestines become twisted on themselves. The doctors are still deciding whether or not this will be addressed surgically. If it is, it would be sometime down the road.
The heart defect – unbalanced AVSD – is what we had expected from the prenatal ultrasounds. Hope’s left ventricle is smaller than it should be and not completely separate from the right. So instead of 2 ventricles hers are kind of combined into one. Also, while a normal heart has a valve for each ventricle, Hope’s heart has one large valve that is off-centered to the right side.
In addition, her aorta is mildly narrowed and has a bicuspid valve instead of a tricuspid. Her inferior vena cava (the large vein that brings blood from the bottom half of the body to the heart) also comes up on the opposite side of where it should be.
Because of the combined nature of her ventricles, the oxygenated and deoxygenated blood in Hope’s heart mixes together and a larger amount of blood flows to the lungs that it should. At this point in time, the impact of this is mainly on her breathing. She breathes very fast – to watch her it’s like she is panting all the time, as if she were running on a treadmill or something. This expends a lot of energy which is why it is too difficult for her to eat by mouth and to gain weight.
In the long term, Hope’s body cannot function with her heart the way it is. The general consensus right now is that she will very likely need a single ventricle repair. We had hoped for a chance at a biventricular repair – to make the heart eventually more like a normal heart – but that seems unlikely.
The single ventricle repair uses staged surgeries to rework the heart in such a way that it can function with just one combined ventricle. The surgeries would likely happen around 6 months (the Glenn procedure) and potentially 5 years or so (the Fontan procedure).
Prior to those, however, the lungs need to be protected. So the first thing to be addressed is decreasing the amount of blood flowing to the lungs. As of today, it looks like that surgery will take place at the end of this week. The plan right now is to do a pulmonary band – pretty much a band around the pulmonary artery that decreases flow to the lungs – which should help with the breathing issue. They also want to take care of the narrowing of the aorta at the same time.
This means that our sweet little girl will be headed into open heart surgery, the first of potentially 3 or more that she’ll experience in her life, at just 2 weeks old.
This is not the path I would have chosen – for Hope or for us as a family – but it is the road we’ve been given.
In the Bible, the word hope means confident expectation. While we can’t be sure of what will happen in the coming days, weeks, months, or years for our sweet girl, we can hold fast to our confident expectation of who God is and what He has promised. Whatever happens, He loves Hope and He loves us and He has a purpose and plan. And someday, when all the trials and suffering of this world have passed, we have the confident expectation of eternity in heaven… where there are no tears and no pain and every heart works perfectly.
We chose the name Joanna as a middle name in honor of my grandma who passed away last year and because it means God is gracious. Joanna was also a woman in the Bible who was healed by Jesus and was one of the first to discover that He had risen from the dead (Luke 8:1-3, Luke 24:9-10). Despite the difficulties that remain we are thankful for the healing work God did to remove the hydrops during my pregnancy. We were initially told that our baby would not live and here she is, in our arms. Whatever may come, we praise God for His graciousness and the healing that He did.
I know this is a long post and heavy on the complicated medical information but I wanted to share everything that we have at this point. We are so thankful for all the support and prayers we have received along the way and ask that you continue to pray for our little girl and for us.
Please pray for wisdom and skill for the surgeons, doctors and nurses that care for our daughter as well as for peace and strength for us. Watching my child struggle and not being able to fix it is probably the hardest thing I’ve ever experienced. I know God can and will use all things to His glory, but that knowledge doesn’t necessarily make things easier for me!
During my pregnancy the song “I Have This Hope” by Tenth Avenue North was one that Steve heard over and over again. It makes me cry every time I listen but the words are so applicable to our lives right now. If you haven’t heard it you can find it here.
I have this hope, in the depths of my soul. In the flood or the fire, You’re with me and You won’t let go.
I cling to this truth… He is our hope.