Causes: Congenital Heart Defect Awareness Week, Sam's Story.

After reading my first Causes series guest post on Noah's story, a friend encouraged me to reach out to Arianne who would likely be willing to share Sam's CHD story, too.  I did and she was.  I think most of us never really consider that things like this could happen to us...to anyone, 1 in 100 babies to be exact.  I've already been reminded by these stories that big, life changing things happen to very normal, unassuming people.  These big things redirect their lives in a very abrupt way as they navigate towards their new normal. I am grateful the opportunity to share Sam's story.

What is a CHD? CHD stands for congenital heart defect. A congenital heart defect is a defect in the structure of the heart and great vessels which is present at birth. Many types of heart defects exist, most of which either obstruct blood flow in the heart or vessels near it, or cause blood to flow through the heart in an abnormal pattern. Some defects don't need treatment, but complex congenital heart defects require medication and/or surgery.

 

I am honored to write this story for my son, who is a heart warrior and had fought hard and strong to be here today!  I also write this story to help spread awareness about CHD.

 

On Thursday June 14, 2012 Joe and I went in for our first ultrasound at 20 weeks.  We decided during this ultrasound we would find out if we were having a boy or girl, not knowing that I should have been asking the ultrasound technician if all 4 chambers of the heart were present, if the heart rate is normal, if she looked at all the arteries, if the heart function was normal, or if the heart and stomach were in the correct position.  The technician performing the ultrasound confirmed we were having a boy and Joe was beaming!  Our family of 3 would now be a family of 4 and we would be tied for boys and girls.  We could not have been happier.

One day later on June 15 we received an unsuspected call from our doctor indicating that the ultrasound pictures of our baby’s heart showed there might be a problem.  Even though we didn’t ask those questions, we were blessed with a technician that just thought the heart did not look right.  Our doctor got us in for a level 3 ultrasound and a Fetal Echocardiogram on June 20 (yes, 5 agonizing days later).  This is a day that changed our life forever.  The doctors confirmed that our baby had Hypoplastic Left Heart Syndrome (HLHS) and words cannot explain the emotions that overtook us trying to understand why.  This is an abnormality in which the left side of the heart (left ventricle, aortic valve, and aorta) is severely underdeveloped. When the left side of the heart is underdeveloped, the right side of the heart must pump blood to both the lungs and the rest of the body.  The exact cause of HLHS and any CHD is unknown but there is a genetic link even though it can happen to anyone.

The doctors gave us some hope and told us about a 3 staged surgery that would mend our son but not cure him.  From this day we moved on to a whole new outlook on life of taking each day as it comes and we had to wrap our minds around our future.

Moving from trying to figure out why us and what causes HLHS, we spent the rest of the summer at numerous doctors’ appointments and trying to learn as much as we could about our baby’s condition and the journey we had ahead of us.  My actual pregnancy continued on pretty uneventful.  On the night of October 28 we made our way to Abbott Hospital, over an hour from our house, to wait for our baby’s arrival so our son would be near the best team of doctors we found for him as soon as he was born.  Samuel Joseph Holicky was born at 40 weeks exactly on October 29 at 4:29pm weighing in at 8 pounds 15 ounces and was 21 ¾ inches long.  The doctors were impressed at how big he had grown and hoped then that this would continue to be true; Sam continues to show them that he isn’t going to let his underdeveloped heart stop him.

 

Sam was immediately transferred with a team of doctor’s under the connecting tunnel to Children’s Hospital of Minnesota’s NICU.  They monitored him continuously to watch his oxygen saturation levels, give him prostaglandin therapy medicine to keep open the Ductus Arteriosus, a hole in your heart that closes on most babies after birth as they needed it to stay open for his first surgery, and fed him nutritional IVs.  From there on October 31, Sam was moved up to the cardiovascular unit where he would be prepped for surgery.  On November 1, at 3 days old, Sam underwent the Norwood procedure.  In this procedure, surgeons re-route the blood flow around some of the defective areas of the heart by creating new pathways for blood circulation to and from the lungs; this is the most critical surgery of the 3.  After almost 6 hours from when we let Sam go with the doctors, the surgery was completed without any hurdles, he was off the bypass machine, and moved back to his room.  Words cannot explain my feelings when I walked in and seen all the tubes, IVs, and monitors hooked up to Sam; it was heart breaking.  Sam’s chest was able to be closed 24 hours later; they weaned him off of IVs and got him onto oral medications and drinking from the bottle in a week.

 

 

 

On Wednesday, November 14 we were released to go home, just in time for the Holidays!  During this time Sam was medically fragile until his second surgery as his heart was working very hard due to lower oxygenation saturations.  This meant keeping up with a medicine routine, checking his weight daily, checking oxygen saturation levels throughout the day, home nurse visits, monthly cardiologist checkups, monthly shots to help prevent RSV (a respiratory virus that would be severe for any infant and especially for Sam with his heart defect), lots of sanitation efforts, and keeping the colds and flu out of our house.  This also meant staying home as much as possible, so my husband was able to stay home with Sam for most of this 3 ½ months, especially after my maternity leave ended.  I would work from home 1 or 2 days a week and our moms would help when needed too.  All of these efforts were fairly successful and we never landed back in the hospital.

 

Sam’s oxygen saturation levels began to decrease over time, as they suspected, and they decided that Sam was ready for his second surgery.  Sam was scheduled for surgery on March 8, 2013.  In this procedure, the Glenn, more rerouting was done to reduce the amount of oxygen-rich blood circulating throughout the body.  This surgery gave Sam an improved quality of life as the heart does not have to work as hard.  Sam’s surgery took about 4 hours and again when I walked back in his room after surgery my heart just dropped looking at my little boy all sedated and hooked up to IVs, tubes, and the ventilator.  Sam was eating again from the bottle the day after surgery and within 3 days he was off all IVs.  Weaning him from the low flow oxygen was another story as the doctors felt his oxygen saturations were too low following surgery, but the actual cause ended up being RSV, keeping us in the hospital for 2 weeks. Finally on March 21, he was off oxygen and the cold was clearing so we got the green light to go home.

Sam was sent home on a home nebulizer treatment in addition to the heart medicines until his respiratory infection was completely cleared.  On April 8, Sam made his first appearance at daycare and besides well-baby checkups, a daily medicine routine, and follow-up Echocardiograms, EKGs, and X-Rays with the Cardiologist, Sam is just enjoying life as a 15 month old and busy getting into everything.  The crawling stage is quickly changing to walking, but it’s amazing to see the progress Sam has made in the last year jumping hurdle after hurdle.  Sam is such a great addition to our family and brings joy, love, and laughter to us every day.

 

The next surgery that Sam will undergo around 3 ½ - 4 years of age (at least we hope he can go that long) is the Fontan.  This completes the repair of the Hypoplastic left heart. The functional effect of this surgery is to allow the rest of the blood coming back from the body to go to the lungs. After this procedure, there's no mixing of oxygen-rich and oxygen-poor blood in the heart, and Sam’s skin will no longer look blue, especially when cold.

With the awareness we spread, we hope it helps to raise money for research and medical developments that will further Sam’s future.  With these repairs that have been done and will be done to Sam’s heart, this does not cure him.  There are many complications that come with Sam’s heart and no way to know now if he will need additional surgeries, a pacemaker, or even a heart transplant.  Sam has a life full of medication and Cardiology appointments ahead of him along with worries every time he gets sick.

 

We have many doctors, surgeons, and nurses to thank for their work with Sam.  Also, we have to thank our family, friends, and community for all their support, love, and prayers.  We wouldn’t be where we are today without everyone helping us along the way!

You can follow Sam at http://www.caringbridge.org/visit/samuelholicky

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.