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