Although her story really began when we found out that we would be blessed with her on February 13th earlier this year, our world was really turned upside down at our 22 week anatomy check-up at the OB/GYN on June 11th. For those of you who don't know, this appointment is when an ultrasound technician does a full scan of the baby's organs, takes measurements, and usually determines the gender of the baby. We already knew ahead of time that we were having a girl because we went to a 3D/4D ultrasound place to get pictures done at 17 weeks, but we couldn't wait for this 'official' appointment at the doctor's office.
The ultrasound technician went through all her checks and told us we were having a girl among other things. The tech spent a lot of time looking at her organs when she was doing her checkup. She told us the baby was in a bad position to get some of her pictures, but we didn't think much of it. After we were all done with the ultrasound, we went back to an exam room to wait for the OB. The OB came in thereafter and quickly delivered the news that would change our lives. She told us that the technician saw the signs of congenital heart disease in the baby, and that the 'best case scenario' was that our little Audrey would need to have heart surgery. She told us that the technician had spotted a single artery umbilical cord during her scan (usually there are 2 arteries in the cord), which led her to take a closer look at her heart. Needless to say, we were left stunned and heartbroken. I just remember feeling sick and like the world was coming down on me, especially because we were offered absolutely no information on her condition.
We were set up with a perinatologist (high risk pregnancy specialist) who would see us in two days. The two days between the time we got the news of her heart defect and the time we saw the perinatologist were the longest and most agonizing two days of our lives due to all of the unknown and speculation of what the diagnosis could be.
We finally met with our perinatologist two days later. She did another full ultrasound with a detailed check of all the organs. What we would later find out is that sometimes congenital heart disease can be associated with other complications outside of the heart (ie, chromosomal abnormalities). After the doctor's first scan, she told us what she thought she could see as far as heart defects. The doctor told us she could not see anything else physically wrong with Audrey, but that we wouldn't know for certain if she had any chromosomal abnormalities until after she is born (or if we chose to have an amniocentesis performed, which we decided to decline after much thought). Given that the perinatologist is not a heart expert, she referred us to a pediatric cardiologist who would perform a detailed echo-cardiogram on Audrey and hopefully provide an initial diagnosis. Our perinatologist ended up being the doctor most frequently visited by us (about 1-2 times per week since Audrey's condition was spotted). She also referred us to our new OB, (whom we absolutely love) since Audrey would now need to be born at the Methodist Hospital in the Medical Center because of their specialized NICU and because that is where her heart condition can best be treated after birth.
The next week we met our pediatric cardiologist for the first time- after another extremely long stretch of sleepless nights and abundant tears. She is a young doctor whom we both loved from the start. Our cardiologist did a long and detailed echo-cardiogram focusing in only on Audrey's heart. It took about two hours and afterwards we were finally given an initial diagnosis. Our cardiologist diagnosed Audrey with Tetraology of Fallot. It was the first time either of us had ever heard of this condition.
Tetraology of Fallot is a diagnosis which typically has 4 different heart defects coinciding with one another. The 4 different heart defects are 1) a narrowing of the pulmonary artery which carries "bad" de-oxygenated blood from your heart to your lungs 2) a ventricular septal defect (ie a hole) between the bottom two chambers of the heart 3) an "overriding aorta" (meaning the aorta is sitting directly over the whole in her heart 4) an enlarged right ventricle.
What all of that means is that essentially Audrey's pulmonary artery can't pump enough de-oxygenated blood ("bad blood") from her heart to her lungs in order to be re-oxygenated, good blood and bad blood are mixing through the whole in her heart, and the aorta is thus delivering poorly oxygenated blood to the rest of the body due to bad and good blood mixing through the hole between her two chambers. Luckily, fetuses have several "holes" in their heart while in utero, so our cardiologist explained that she is pretty much unaffected by her condition while she is in the womb. And although a single artery cord can be of concern too, she seems to be getting good blood flow through it to this day, so both of these pieces of information were of huge relief to us.
The good news in all of this is how far medicine has come over the last 50+ years, and the large amount of knowledge that has been obtained regarding Tetraology of Fallot specifically. At our appointment, our cardiologist proceeded to outline how the surgeon would likely go about repairing everything...
To make a very long story somewhat short, once Audrey is born, she will immediately be taken to the NICU where she will be given a medicine via IV so that a natural hole between the pulmonary artery and aorta that all fetuses have (but typically close up within 5 days of life) will stay open so that she will continue to have decent blood flow to her lungs. She will then immediately be given an external exam and ultrasound to determine if her diagnosis will stay as is, or if they need to adjust it. Her blood will also be taken for genetic testing so that they can determine if she has any genetic abnormalities (that may have led to this condition). She may be blueish when she is born due to her poorly oxygenated blood, but hopefully with this medicine (and resulting increased blood flow to her lungs) she will pink up a bit.
Assuming her diagnosis stays as it is now, after she has about 3-7 days to get acclimated to her new world, she will undergo her first heart surgery in which she will have a shunt placed to connect the pulmonary artery and her aorta so that she will have increased blood flow to her lungs. At his point, she will be taken off of her IV medicine and that hole can now naturally close up because the shunt is in place. Barring any complications, she is expected to be in the NICU for at least 3 weeks after birth. Unfortunately, this means that nobody will get to meet our love bug except for us and our parents. They have very strict rules about visitors in the NICU at Methodist, but I know they exist for a good reason.
Once she has healed, can handle feedings, and has a blood oxygen percentage of 75% or higher, she should be discharged and allowed to come home with us from what we understand. Once home, we will work on "normal" things such as feeding, gaining neck strength, bonding, tummy time, playing, etc. We will continue on healing and gaining strength until she is about 4-6 months old. During this time though, she is supposed to be a 'germ-free' baby, which means that she will only be leaving the house for doctor appointments and we are advised to only let immediate adult family members who have been vaccinated for the flu and pertussis have contact with her. This was a pretty hard pill to swallow because the last thing you want to do is keep your newborn baby from meeting your friends and family, but we know it's for the best. The reasoning behind this recommendation is that because her blood oxygen saturation levels will be so low (around 75-80%; they are normally 98% or so for a healthy person), if she does get sick, she will likely have to recover in the hospital because she will have a harder and longer time recovering than a healthy baby would.
Then, hopefully close to 6 months (which will be after flu season), she will undergo her first open heart surgery in which she will be placed on a pulmonary bypass machine for the duration of the surgery. This is considered her 'full repair' surgery, whereas the shunt surgery was just a temporary solution. In this open heart surgery, the hole in her heart will be closed, and her severely narrowed pulmonary artery will be removed and completely replaced with an artificial artery. Her shunt will also be clamped shut since it is no longer needed with a new pulmonary artery in place.Unfortunately, they don't have pulmonary valves small enough for infants, so she will be missing a valve, which will allow some back-flow of de-oxygenated blood back into her heart. Despite this fact, her blood oxygen levels will be much higher after this repair and since she has never known a higher oxygen level, this will suit her just fine. Once again, barring any complications, she is expected to recover in the NICU for another several weeks until she is ready to come home yet again. At this point, she will pretty much be ready to make her debut to the rest of our family and friends, and start making her own baby friends too :) We can't wait for this day, and wish we could just fast forward to it, but we know with plenty of faith, prayers, and support, we will make it to this day eventually and joyfully!
Eventually she will need yet another open heart surgery, but hopefully not until her early teens when she outgrows her artificial artery. During this surgery she will have her artery replaced again, and will finally receive a valve. She will likely require a few valve replacements over her lifetime, but luckily these are done endoscopically and will be much less invasive than any kind of heart surgery. Who knows how far cardiac care will have come by then though. We're already amazed at everything they can do now, but we're positive it will only get better with time. And although this condition is devastating for our little girl, we can't help but be so thankful for the technician that caught this problem early on in the pregnancy. A lot of children aren't diagnosed with heart defects before birth, which can obviously lead to serious consequences afterwards. Yes, we have suffered by knowing what she will face all of this time, but both of us agree that it is much better to be prepared for everything that is about to come.
Sorry for the ridiculously long and detailed first post; it was our chance to introduce everyone to Audrey's condition and our understanding of what her medical treatment will be based upon the information we have from her doctors as of now. Of course we will do our best to update the blog with any changes as they come about.
We want to end by thanking all of Audrey's supporters for their prayers. We know that the love for her already extends beyond our family and friends, and we couldn't be more grateful. We have been heavily relying on the power of prayer and our faith that the Lord will help our baby girl get healthy over the next several months, and additional prayers for successful surgeries and quick recoveries are all we could ask of anyone.
Much Love,
Charlie and Christie
Audrey at about 29 weeks :)
No comments:
Post a Comment