Causes: Congenital Heart Defect Awareness Week, Noah's Heart.

The first guest post in my Causes series is about a sweet little boy named Noah.  I "met" Noah's mom, Aubrey, in an online group of about 40 women from all over the US and Canada who were all due with babies in January 2013.  We began chatting when we were pregnant and have continued frequent communication as our babies have grown.  For perspective, Noah was born just 11 days before Myra.  I have naturally followed Noah's story, but it was an entirely different experience reading it all at once.  It is important to note Noah has overcome a lot in his first year of life, but in many ways this is just the beginning for him.  I am very grateful Aubrey was willing to share her family's story.  (Note: Aubrey sent me many photos and asked me to choose.  Clearly I had trouble choosing, so enjoy the plethora of Noah photos!)

February is Pediatric Heart Awareness month! 1 in 100 babies are born with a heart defect. That is one roughly every 15 minutes and a total of 40,000 born this year alone in the United States. I am honored that Ali asked me to share my son Noah's story in honor of this month.

My husband, Sean, and I found out something might be wrong with our baby's heart when I was 21 weeks pregnant. That was the most devastating phone call I have gotten so far in my life. It was a week long wait from that phone call until we were seen at Maternal Fetal Medicine and a fetal echo done. Our sweet baby boy, our Noah, was diagnosed with Tetralogy of Fallot (ToF); a heart defect characterized by a large hole between the lower chambers (ventricles) of the heart and an obstruction from the heart to the lungs. With ToF patients, the aorta lies over the hole in the lower chambers, and the muscle of the lower right chamber becomes thickened. We were told he would need at least one open heart surgery sometime after birth but they couldn't tell us when he would need it until after he was born. He would need to be born at a hospital 3 hours from our home, where they would be capable of handling his care. The rest of 2012 was a roller coaster of emotion as we prepared for our sweet boy to come into the world.

Noah was born January 11th, 2013 weighing 7lbs 12oz and 21 inches long! A great size for a heart baby! His echo after birth confirmed the Tetralogy and also showed he has a small atrial septal defect (ASD) and an anomalous left coronary that came off his right coronary. He is thankfully what is considered a "pink tet" and only spent about 2 days in the NICU. Then Noah  was moved to room with me and brought home on January 14th (his due date!). We were told to expect surgery sometime between 2 and 4 months old.

Noah had regular appointments with his cardiologist. At one month old he was observed to be having retractions when breathing and his liver was slightly enlarged. He was diagnosed as being in heart failure and started on lasix to help combat that so he could grow and have more time before needing surgery. He was thankfully still keeping his oxygen saturation's in the 90's despite his heart having to work so hard.

Noah's weight gain slowed quite a bit around 3 months old, but he was still doing well holding his oxygen saturation's in the low 90's. With that, his surgery was pushed out until June 17th, to allow him more time to grow bigger and stronger before surgery. At his appointment the next month though his saturation's had dropped significantly, reading 78-79%. His cardiologist was concerned and had us begin monitoring his oxygen with weekly checks at our local hospital.

On June 2nd, we grew concerned because he had begun to have a lot of blue spells. It is hard to draw the line of when it's time to call it in. He had one spell where while we were able to pull him out of it and he didn't lose consciousness. He did become very floppy and listless. It is a frightening thing to see your baby blue and floppy like that. We called his cardiologist and were told they wanted to admit him to the children's hospital to be monitored and observed over night. He thought it was probably not much going on and we'd be back home after a night stay. We made arrangements for friends to watch our two year old daughter while my husband worked and I stayed with Noah at the hospital. I packed a small bag for Noah and I.

When we got the hospital we were to go to the ER to be admitted there. The nurses loved Noah and cooed over how cute he is. They got him all hooked up to the monitor and he was of course stating in the 90's, ha! I told Sean it figures and we must have jump the gun on being nervous parents. His 90's saturation's didn't last long though before he began having spells where he'd dip into the low 70's. After a couple spells they decided to get him on oxygen and give him propranolol. Taping the oxygen tubing to his face really agitated him though and acerbated the spell. He went from low 70 oxygen saturation's, to 50's, then 30's, I looked away from the monitor then, my husband (and Noah's medical records) say's he bottomed out at 29% though. Our room was full of nurses, residents, and doctors. They were giving him blow by oxygen to try and pull him out of the spell and calling . I couldn't get the doctor to make eye contact with me. You know things are really serious when the doctor won't even look at you. I remember seeing a nurse grab a face mask and bag. That imagie I think will haunt me the rest of my life, someone preparing to bag your baby, not something I'd ever wish on anyone. Noah thankfully began coming out of the spell right about then. I am beyond thankful for that.

He earned himself the biggest room in the ER and his own nurse who was only assigned him as a patient from there. Plans changed quickly and we were told he was being admitted to the PICU instead of the floor. We knew then this wasn't going to be just an overnight stay. Once we were moved to a room in PICU his cardiologist came and talked to us. They had bumped the patient who was supposed to have surgery that next day and now Noah would be having his. He had plainly shown us he couldn't wait any longer.

On the morning of June 3rd we handed out sweet boy to the anesthesiologist at the elevator and said our "see you soon's" as the doors closed and separated us. It was an agonizing wait, but at 10:30am we got the news he was out and had done awesome! 

 

We were told he would probably need a conduit, because of his coronary anomaly would need periodic replacing, from his right ventricle to his pulmonary artery. Though his amazing surgeon was able to complete the surgery without using one! We spent 3 days in the PICU and 2 days on the regular floor, from there when we were able to bring Noah home again. That was a beautiful day.

Noah is doing well now. He still has the ASD (Atrial Septal Defect) and because of how his repair was done due to his anomalous left coronary there is some risk of the muscle that was cut away in his ventricle growing back and him developing double chamber right ventricle so he is being watched for that. While they call the surgery for tetratology a "repair," it is not a cure. There is no cure for Congenital Heart Defects as of now, though we hope that with the spread of awareness funding for research can be increased and more lives can be saved.

Noah will have to be followed by a cardiologist all his life as there are many complications that can arise with his heart. We are hopeful though that he will continue to thrive and his heart will continue to work well for him. He is the happiest baby and so full of love and joy, he has given us a new outlook on life. Every day is precious, none of us are guaranteed tomorrow so it is important to live today.

Click the link to follow the Noah's Heart Facebook page.  

Also, Aubrey just posted this: Please read this. It is a blog post by a fellow heart mom about why it matters that we spread awareness, she says it more eloquently than I can. I know I am probably annoying some of you with all my posts. I know some of you do not understand and think Noah is "fixed" now and I need to "move on" but that isn't how it works. He isn't fixed. And I will continue to spread awareness until there is a real fix for all of these kids that suffer with CHD. Because it matters. These kids matter. Please click this link and read.