Piece of my Heart

Welcome to our blog, we will be using this to keep you all updated on what is going down while we travel the muddy waters of our baby's heart defect.  Some of you have probably noticed the name and are wondering what meaning it has.  Well here it is, the big reveal, we have chosen the name Owen Miles for our son.

Owen Miles Krajnik

That will be his name.  We looked up what Owen means a few nights ago and were quite surprised to learn that it means "Young Fighter".  We think it fits quite well for what is in store for him.

Most of you probably already know the story, but here is a refresher on the story for those of you who might not have heard it in it's entirety.  We first discovered that there was something special with our pregnancy when we had the first anatomical ultrasound on December 5th, 2011.  The anatomical ultrasound is where there look carefully at the developing baby to measure the limbs and organs as well as the head and abdomen circumference, just generally looking for any indicators that there might be an issue with the pregnancy.  Our little guy was being stubborn and wouldn't move into a good position to let them get a good look at his heart, but we did get to see that it was a boy.  After the ultrasound, Dr. Garvin-Cress, our normal Ob-Gyn, informed us that our baby only has one umbilical artery where there are usually two, know as single umbilical artery(SUA).  SUA carries an increased risk for birth defects, especially heart defects, and is also associated with chromosomal defects.  Dr. Garvin-Cress told us that she wasn't very concerned based on the measurements that they got from the ultrasound he appeared to be healthy.  They would try again at the next appointment to get a better look at his heart.

Our next appointment with Dr. Garvin-Cress was on January 9th, 2012.  Again our little guy was being stubborn and wouldn't turn so they could get a good look at his heart.  Something they did see concerning his heart on the ultrasound must have been unusual because when we talked to Dr. Garvin-Cress she referred us to Dr. Hayes, a perinatologist at Aurora Baycare in Green Bay.  Our appointment with Dr. Hayes was the next Friday, January 20th.  Again they started off doing an anatomical ultrasound, our little guy is actually a little bit ahead of the curve in his size.  When the tech got around to looking at his heart, she was having trouble getting a good look at it, so she got another tech that was more experienced at looking at babies hearts.  When that tech got in the room she started looking at his heart from all different angles, and eventually she left the room to get Dr. Hayes.  So now the Dr. and the tech were in the room looking at our little guys heart from every angle they could get, mapping the flow in various spots, throwing out phrases like "that's unusual", "I don't see any flow", and "look closer at the valve".  Needless to say at this point we started freaking out and had a pretty good idea that there was something wrong with our little guys heart.  Finally they were done looking his heart and Dr. Hayes told us that we would discuss what they found in his office.  Dr. Hayes told us that our baby boy had an unusually small tricuspid valve, the one between the right atrium and the right ventricle, which, as the heart was developing, had prevented the septum between the right and left ventricles from closing due to a reduced flow of blood through the right ventricle.  He was telling us that our little guy has a ventricular septal defect.  After informing us of this, he said that he was referring us to the Fetal Concerns Program at Children's Hospital in Milwaukee because they are the experts in the state on congenital heart defects, among the top in the nation in fact.  He also wanted to start doing weekly bio-physical profiles on our baby starting mid-February.  Before we left Baycare, we had already started to process of getting an appointment set up at Children's Hospital.

So that all being said, we have an update for everyone. As most of you know, we had our first appointment at Children's Hospital in Milwaukee on Tuesday, February 7th.  When we first got there we had an ultrasound and he was being stubborn again.  Big surprise, right?  While in the ultrasound we met the perinatologist, Dr. Broekhuizen, and our fetal concerns program coordinator, Barbara.  We discussed how we are going to deliver the baby.  Dr. Broekhuizen wants to keep it as natural as possible, or in other words, he doesn't want to induce unless Cassie will be going over her due date.  He suggested that she come to the Milwaukee area 2-3 weeks before she is due and stay with family, that way she will be closer to the hospital than if she stayed in the Two Rivers area to avoid a pricey ambulance ride down to Children's Hospital.  The next stop for the day was to meet with a lactation specialist.  No ground shaking revelations there.  We are planning on breast feeding Owen, but obviously he won't really be able to breast feed if he has to have surgery, so the plan is for Cassie to pump every three hours and they will refrigerate it right in his room.

After the lactation specialist, it was on to the main event, a fetal echo cardiogram where we would get a better picture of what is wrong with Owen's heart.  We made our way over to the Herma Heart Center for the fetal echo cardiogram.  There we met Dr. Saudek, a pediatric cardiologist, who performed the echo cardiogram. Now Dr. Saudek doesn't like to talk a lot while he is doing his scans, so we sat there for nearly an hour while he looked at Owen's heart from every possible angle, mapping blood flow through every chamber and valve in near silence.  We did chat with Maura telling her a little bit about ourselves and Dr. Saudek did join in the conversation a bit, but he didn't say anything about what he was seeing with Owen's heart.  When he finally had all of the information that he needed, we went into a conference room to discuss Owen's heart.  Dr. Saudek entered the room with diagrams of hearts to help explain what is wrong with Owen's heart.  The bottom line is Owen's heart is worse than what Dr. Hayes could see.  Owen has a cyanotic congenital heart defect known as Type 2 Tricuspid Atresia.  What this means is that there is virtually no blood flowing through his tricuspid valve.  Because there is no blood flowing through the tricuspid valve, the only blood entering the right ventricle is coming through the ventricular septal defect.  Blood also only leaves the right atrium through a whole in the septum to the left atrium.  This lack of blood flow caused the right ventricle to be hypoplastic or severely underdeveloped.  Along with the non-functioning tricuspid valve and underdeveloped right ventricle, there is a transposition of the greater arteries leaving the heart, meaning that the aorta and pulmonary artery are leaving from the wrong side of the heart.  Dr Saudek was very good at explaining how the heart works and what was different with Owen's.

After telling us what is wrong with Owen's heart, Dr. Saudek explained to us what would be done to fix it.  Owen will need three separate surgeries to fix his heart.  The first step will be to give Owen prostaglandins to keep the pulmonary ductus arteriosis, or PDA pathway between the aorta and pulmonary artery open.  This pathway is always present because in utero the lungs aren't really necessary, but closes shortly after birth.  Within his first week, he will need to have a procedure on his heart called the Norwood Procedure.  This procedure consists of first disconnecting the pulmonary artery from the heart and then putting in a patch so both ventricles pump through the aorta.  They will also make the aortic arch bigger to make sure enough blood can flow through it.  Off the top of the aortic arch they will put in a shunt to the pulmonary artery.  Owen will be in the hospital for at least a month after he is born to recover from the surgery.

Keep in mind, he has a hypoplastic right ventricle, not a hypoplastic left ventricle.

As Owen grows, the shunt between the aorta and the pulmonary artery will become inadequate because it won't grow with him.  When this happens between 3 and 6 months, Owen will need his second surgery.  At this point, they will go back in, removing the shunt and putting in a bidirectional cavopulmonary shunt, where they disconnect the superior vena cava from the heart and patch it directly into the pulmonary artery, this is called a Glenn procedure.  His third and final heart surgery will be between the ages of 2 and 4 years.  They will go in and perform a Fontan Completion where they redirect the blood from the inferior vena cava around the heart and patch that into the pulmonary artery.

We would like to thank you all for your support in this very trying time in our lives.  We are very appreciative.  And we will do our best to keep updating the blog along this journey.

Love,
Kevin and Cassie