As previously described in “D-Day,” we received Daniel’s diagnosis on Friday, March 30th at our 20-week ultrasound. It was Easter weekend, and I walked around in a semi-conscious fugue for days. All I could think about was Daniel’s heart; all my thoughts and emotions were filled with fear and the unknown. I couldn’t focus on the Easter services, I couldn’t enjoy my children and their cousins playing, and I certainly couldn’t hold a conversation that involved something other than HLHS and Daniel.
Thankfully, I have two highly connected family members in the medical field in Houston– my brother and his wife. Somehow my sister-in-law was able to pull some strings and get me in with a neonatal cardiologist to do a fetal echocardiogram just 5 days after we received the original news. A fetal echocardiogram is when the ultrasound just focuses on the heart for 2 straight hours. It maps the blood inflow and outflow and evaluates the structures in and around the heart, like the aorta, pulmonary artery, pulmonary veins, valves, etc.
Our appointment in Houston was on Wednesday, April 4th at The Fetal Center at Memorial Hermann Hospital. We had our fetal echo from 8-10 a.m., another full body ultrasound to be sure that there were no complications with Daniel’s other organs, and an appointment with a genetic counselor.
To say I was a nervous wreck would be a huge understatement. First of all, I was going through denial. I had convinced myself that there was NO WAY my baby had HLHS. The ultrasound was unclear, he was in a weird position, and these tests are wrong ALL THE TIME. Surely this was all some horrific mistake. I was praying that it was, anyway.
Secondly, I had only been to Hermann Hospital a few times, and I was extremely nervous about navigating the medical center to get to all of my appointments. Thankfully, the Fetal Center is all confined to one area. All of the departments within work together; all are aware of what the other is doing. This was so shocking and relieving to me; I have no words to convey how much it made a difference on one of the most stressful days of our lives. We met with ultrasound technicians, multiple neonatal cardiologists, the fetal medicine specialist, the genetic counselor, and our surgeon. All knew about the others, they all worked together to be sure we got to the right place at the right time, and they all bent over backwards to make us feel comfortable.
The echocardiogram was the longest 2 hours of my life. I laid on my back in silence as the neonatal cardiologist fellow looked at Daniel’s tiny heart on the screen. I had no idea what I was looking at, and he couldn’t tell me what was going on. So it was two hours of heartbreaking silence. Very quickly I realized that it wasn’t a bad dream. Daniel really did have HLHS.
There were many lights in the darkness, though. I knew without a doubt that this was the right place for us to be. We were doing everything we possibly could, and that gave me tremendous peace. About halfway through the appointment, Daniel decided he wouldn’t cooperate anymore. At one point, the cardiologist said, “Where did he go??” Daniel literally hid from the ultrasound wand. Then, when the cardiologist found him again, Daniel kept moving away, refusing to cooperate, and kicking the wand in protest. It made me so proud, honestly, and amused to see his little personality in play already. It also reminded me of his sister, Hannah. Hannah is the most stubborn, hard headed, strong minded child I know, and I seriously hope Daniel has her fighting spirit. His protests in the womb suggest he might be just as stubborn as his sister, and he will need all the fight he can muster.
After two hours, we were funneled into a conference room, where the cardiology fellow and the pediatric cardiologist told us the news. They didn’t use scientific jargon or talk over our heads, though. They gave us two diagrams. The first one was a normal heart. They patiently described how a normally functioning heart works: the unoxygenated blood from the body travels into the right atrium, then through the valve into the right ventricle. The right ventricle pumps the blood through the pulmonary artery, where the blood then gets oxygenated in the lungs. The newly oxygenated blood travels from the lungs through the pulmonary veins into the left atrium. Then the blood travels through the valve into the left ventricle, the strongest muscle in the heart. The left ventricle pumps the blood through the aorta to the rest of the body.
Daniel doesn’t have a left ventricle. His valve is completely closed, and his left ventricle is completely unformed.
In the womb, there are two holes open in a baby’s heart. There is a hole called the atrial septum, and there is one called the ductis arteriosis. These holes allow blood to flow from the heart to the rest of the body. In utero, the placenta provides the oxygenated blood to the baby, so as long as Daniel is inside me, he is safe and healthy. Within two days after birth, however, the two holes close up. If the two holes close up, then blood can’t get to the rest of the body, and Daniel dies.
In order to prevent the holes from closing, Daniel has to get an IV immediately after birth with prostaglandins. This will prevent the holes from closing. This is only a short term “fix” until Daniel can have the Norwood procedure, the first of 3 surgeries Daniel will have to have.
A note before going any further– Daniel’s surgeries are “palliative,” not corrective. Palliative means they can’t FIX Daniel’s heart. There is no way for humans to magically create a left ventricle. The only thing they can do is re-route the heart to make it as functioning as possible. This is done through a series of 3 surgeries:
The Norwood Procedure: A 6-8 hour open heart surgery. Daniel will have to have this within a week after birth. The details are all very complicated, and I will post more about this procedure when Daniel is having it done.
The Glenn: a shorter, less complicated surgery that is scheduled when the baby is 4-6 months old
The Fontan: the final surgery that occurs when Daniel is 3-4 years old
From my still very limited understanding, the eventual goal of these surgeries is the following:
Instead of unoxygenated blood flowing into the right atrium and ventricle, then being pumped into the heart, the unoxygenated blood will now flow directly into the lungs. As a child gets older, gravity can make this happen somewhat effectively.
The oxygenated blood then flows into the right atrium and right ventricle. In the Norwood procedure, the Aorta and Pulmonary Artery are fused together. This allows the right ventricle to pump the blood to the rest of the body, not the lungs.
Basically, guys, this is an amazing MIRACLE of modern medicine. I can’t fathom the brilliant surgeons who thought of this. The idea that Dr. Salazar, Daniel’s surgeon, sews tiny blood vessels together and works on baby hearts regularly as his day job BLOWS MY MIND.
I am so incredibly thankful to live in the United States where these techniques are developed and explored. In some European countries, 80% of babies identified with HLHS in utero are aborted, and most of the remaining babies who are born with it are just given “comfort care,” which means they are made comfortable until they die shortly after birth. I have met and taught (a story for another day) adults with HLHS, and these people can have amazing, productive, happy lives. Dr. Salazar says that the survival rate for all three surgeries with some of the new improvements made in recent years is over 90%.
All of these details, and more, were explained to us by two very wonderful cardiologists. They patiently endured my ignorance, answering any and all questions I had in words that I could understand. The explained the good news– right now, Daniel’s pulmonary veins look great, his right ventricle is pumping well, and his valve looks like it is not leaking! All amazing news.
There are SO MANY complications that can arise in babies with congenital heart defects. The heart structure and the lungs are so intimately connected. Very frequently babies develop pulmonary vein stenosis (from what I understand, it is narrowing or closing of the pulmonary veins). This means that blood can’t get from the lungs back into the heart. Babies also can have a restricted or closed atrial septum (one of the vital holes in the fetal heart before birth), valves can leak or be nonexistent, the aorta can be absent. The number of accompanying defects Daniel can have are incredibly numerous. When I think about what can go wrong, I feel like my head and heart will explode.
Next we went to the full-body ultrasound. As I mentioned previously, there are many other problems that can arise when there is a flaw in the heart. The ultrasound helps the doctors to assess all the other organ systems. There, we met the fetal specialist, Dr. Johnson. He doesn’t mince words, and he doesn’t sugar coat truth. This was such a breath of fresh air. He was clear, confident, and competent.
We found out that we would have to temporarily relocate to Houston around 32 weeks gestation (June 18) to be sure that Daniel is born in Houston. The fact that I had a previous premature delivery is very concerning. A newborn baby’s heart is already tiny. A premature baby’s heart and lungs are even tinier and still undeveloped. It is not a “deal breaker” if Daniel is born early, but it certainly isn’t optimal. I am getting progesterone shots weekly to help bring Daniel to term. Please pray that Daniel stays in utero as long as it is safe!
Thankfully, the ultrasound looked perfect. There are no other concerning features at the moment. We go back May 30 to get another ultrasound. In that ultrasound, they will be sure the atrial septum hasn’t narrowed, look at the pulmonary veins and other structures of the heart, and see how Daniel is growing. So far, he is a big boy. When I go in for my routine OB-GYN appointments, he measures 2 weeks ahead of what he should be, so at least the HLHS doesn’t mean he is growth restricted.
After the ultrasound, we met with the genetic counselor. Basically, there are genetic causes and environmental causes.
Genetic causes would mean that some chromosome or letter in his DNA is missing or switched. The only way to assess with 100% accuracy if Daniel has a genetic disorder is to do an amniocentesis. A needle penetrates the womb and draws out amniotic fluid. We decided not to do any further genetic testing because the outcome wouldn’t affect anything we are doing. To risk a miscarriage or still birth to know the answer the a question that we still couldn’t do anything about makes no sense for us. So we will have to wait and see. At the moment, there are no tell-tale signs of any other genetic abnormalities on the ultrasound. Typically with certain genetic conditions, certain signs can be seen on the ultrasound. Daniel has none of those.
Environmental causes would mean that I took some medication or was exposed to something that may have caused this. Nothing I have taken during the pregnancy (omeprazole for unrelenting heartburn) has been shown to cause this defect, and I, to my knowledge, I haven’t been exposed to anything.
The genetic counselor said it was probably a combination of genetic and environmental factors. No one really knows why this happens to 1 in 100 babies nationwide. If I had further pregnancies, I would have about a 3% chance of having another baby with a congenital heart defect. Daniel also has a 3% chance of passing this on to his children one day (it is only 1% for people without CHD).
After the genetic counselor, we met our surgeon, Dr. Jorge D. Salazar. What an amazing man! Here are two links if you want to know more:
Upon meeting Dr. Salazar, the first thing he said to us was, “I have 7 children, so I know what it means to love and cherish your child. Know that I treat every single baby I operate on like my own, and I will do everything in my power to give Daniel the best results possible.”
Dr. Salazar also gave us some amazing advice. He said, “Keep your eyes on where you want to go. Don’t look at the trees. When you are driving down the highway at 80 miles per hour, you aren’t studying the trees along the road; you are looking in front of you at where you are going. Don’t think about all the things that can go wrong. Keep your eyes on where you want to go.” He also said, “Miracles happen, folks. There have been times when the baby is delivered that there is a left ventricle there where there wasn’t one before. There is no limit to what God can do.”
There have been so many times over the past few weeks that I have literally shaken my head and said aloud, “DON’T LOOK AT THE TREES!”
After meeting with Dr. Salazar, I had such peace of mind. Not only does this man have the incredible technical skill to perform these operations successfully, he has the heart for people and is a believer in God as well! We even prayed together after our meeting.
During the meetings that day, I kept thinking, “What if…?” Almost as soon as I said that, words that I had to memorize from the small catechism during confirmation in 7th grade kept coming back to me, words I haven’t thought about in 15 years. Each time I got scared, when fear threatened to make me crazy, part of the explanation for the first article of the apostles creed repeated in my mind: He richly and daily provides me with all that I need to support this body and life. He defends me against all danger and guards and protects me from all evil. All this He does only out of fatherly, divine goodness and mercy, without any merit or worthiness in me.
So often I have gone through life unaware, apathetic, and unmoved by the ways God works. During this most difficult day, I kept seeing incredible, TANGIBLE ways God continually, richly provided for us.
The fact that we even know that Daniel has HLHS before he is born! Only 40% of babies who have HLHS are identified while they are in the womb. Much of the time parents don’t know until their babies are born and start dying.
The fact that my brother and sister-in-law were able to recommend and get us to such an amazing place, The Fetal Center. Dr. Salazar and our cardiologist just came to Hermann 6 months ago.
The fact that so many improvements have been made to Daniel’s operations in the past 3 years. The improvements made to the surgeries mean, possibly, fewer complications for Daniel and a better outcome.
The fact that we even know that I should be taking progesterone because of Hannah’s premature birth. Thank GOD Daniel wasn’t born second and premature. At least now we know of the potential for preterm delivery and can prepare accordingly.
The fact that John and Christi live in Houston and are so close to the RV park where we will be staying. Their continued support means so much to us.
The Holy Spirit reminding me over and over again of something I memorized YEARS before
There are SO MANY blessings and miracles I have experienced first-hand because of this diagnosis. I was and am so in awe that God’s power, grace, and mercy.
Here is the plan going forward:
We return to Houston for another round of appointments on Wednesday, May 30th. We will have another ultrasound, an appointment with the doctor who will delivery Daniel, and we will tour the cardiovascular and neonatal ICU.
We are supposed to relocate to Houston beginning no later than Monday, June 18th. We are pulling my aunt Pam’s RV down. There is an RV park conveniently located minutes from the medical center, and there is a shuttle to a from the medical center. My mom, the kids, and I will be there until after Daniel is born. Brian will be splitting his time between Houston and home. My dad is staying home with the dogs.
I am so thankful that my mom is willing and able to do this for us. Without her, I would probably have to be there alone and rarely see my children. Because of her willingness to come along for the journey, I won’t have to be separated from my children for months.
A word, also, about my amazing husband. Brian has been the most amazing partner during all of this. I wish I had the words to express how thankful I am for him. I think God allowed our paths to cross and has kept us together for ten years because he knew I would need Brian’s quiet strength, selfless love, and constant support.
Please let me know if you have any tips and suggestions for surviving Houston heat with two toddlers in tow or for an extended hospital stay with a newborn who has just undergone surgery. I am so ignorant at the moment. I have no idea how I should even prepare for this. I appreciate any and all insight.