Catching up...

 Below is a post from my Facebook "notes" page from back in early February when we were first given details about Koal's heart condition and what may lie ahead. Some info and expectations have changed slightly (and I will update with that soon), but this is a bit of background from our initial diagnosis and doctor visits.

***At the beginning of January (around 22 weeks) we had an ultrasound that showed our little guy (expected to arrive at the end of April) had some issues with his tiny heart.  We were of course referred to a cardiac specialist at Children’s Mercy, but the perinatologist who first noticed the problems would tell us very little.   We did, however, overhear some things and were able to piece together what they THOUGHT we might be dealing with, and we OF COURSE Googled everything and tried to learn as much as we could with as little information as we were given. 

This past Friday (Feb 4th) we finally had our appointment at CM (yes, we waited nearly a month!) with the pediatric cardiologists to have a fetal echocardiogram and find out what they saw in our little guy’s heart.

The appointment went well. We were, again, so impressed with CM and their staff.  The cardiologists confirmed that our little guy has something called Tetralogy of Fallot, along with a severe variant called Pulmonary Atresia.   In short (and non-medical terms) TOF is a four part heart defect that includes a hole between the bottom chambers, an aorta that attaches in the wrong way, a pulmonary artery that is also malformed (and in our little man’s case, nearly completely closed off, so no blood can flow through to the lungs) and an abnormally large right ventricle.   This site does a fairly good job explaining TOF-PA if you want more details:

http://emedicine.medscape.com/article/899368-overview

TOF-PA is very rare (something like 7 of every 100,000 babies are affected), and quite serious.  The survival rates are, however, very good.  90-95% of kids reach adulthood after multiple surgeries as infants, and many go on to lead normal healthy lives with very few restrictions.  We of course pray that our little man is healthy enough for surgery and that it goes well and corrects all the heart problems as needed.  The cardiologists at CM assured us that while his condition is so rare, they have still seen and treated many infants with the same defects and the surgeons are very comfortable and successful with the surgeries he will need.

TOF is often associated with or caused by a chromosomal abnormality called DiGeorge Syndrome.  Because we declined an amniocentisis in the first trimester, we will wait and the baby will be tested for this once he is born.  We know little about DiGeorge Syndrome, except that it is similar to Down Syndrome, with multiple physical and developmental issues that can go along with it.  We PRAY our little man has no other hurdles physically or developmentally than what will already come with his heart condition, but we trust that God knows what’s in store and will guide us through whatever is to come.

At this point there are still quite a few unknowns for us, but we DO know that immediately after birth, our little guy will be placed on IV drugs to keep his fetal blood vessels open and working so his heart can stabilize.  This will require him to remain at Children’s Mercy Hospital from birth until after his first open heart surgery is performed and a shunt is put in (a month or two?).  Once he reaches approximately 6 months of age, he will undergo his second open heart surgery to repair the TOF-PA defects.  We PRAY that he will be able to spend a few months at home between the surgeries, but that is also unknown right now.  We will meet with the surgeon and other CMH staff in the next month or two to learn more.  All the doctors working on our case (2 pediatric cardiologists, CM surgeons, my OB, the CM OB/Maternity docs, the cardiologist from our OB’s hospital) will also make a decision in the near future as to where I will deliver the baby and discuss what needs to happen immediately after the birth.

We are SOOOOOOOOOOOO thankful for how God has worked through this already and how we KNOW He will continue to go before us through this journey.  We are thankful this was discovered during pregnancy and not because our newborn was turning blue or had immediate heart failure! It is also a HUGE blessing to know that he is at no risk while he is in the womb – safe and sound as can be – and my pregnancy should progress fine with no complications. We are so thankful to have Children’s Mercy here in our city, with such amazing doctors and staff and the incredible technology to take care of our little guy once he is born. We are BEYOND blessed by family and friends who are coming along side us to pray for, support, encourage and walk through this with us as well.  Thank you all SO MUCH…we would never get through this without you!  More than anything, we are so thankful for our loving Father: the Great Physician, the Creator and author of life, our Comfort and Strength. We have felt His peace and presence from the beginning and we are confident He will continue to work in each of our lives and each of those on this road with us.  We pray that we will continually seek Him and rely on Him for every need. We pray that no matter what is to come, that HE is glorified and that each one of us would know Him more because of this.***