By Abbie Engelhardt, R.N. for Lee’s Summit Physicians Group
This is a personal story about my niece and congenital heart defect awareness. I wanted to share this story to raise awareness… congenital heart defects week is February 7- 14.
My sister, Lori and I were both going to blessed with baby girls around the same time, how exciting!
Everything seemed to be fine.
My beautiful niece, Isabella Rose (aka Izzy B), came early at only 33 weeks, born on September 17, 2009. She weighed 3 lbs 6 oz. I visited her in the NICU before leaving for a cruise and she was doing well.
Upon returning to American soil a few days later, I had heartbreaking messages from my family. Isabella was diagnosed with a congenital heart defect called Hypoplastic Left Heart Syndrome (HLHS).
She now had 3 options.
1. Comfort care.
2. A series of three heart surgeries to correct the defect.
3. A heart transplant.
This was devastating news. She was only 13 days old when it was discovered.
At that time, there was no cardiac screening for newborns.
Luckily, she was premature and being monitored in the NICU or she would’ve died before they found out she had HLHS.
Beginning January 1, 2014, Chloe’s law came into effect, which required all babies in Missouri to have mandatory screening for critical congenital heart disease (CCHD). CCHD screening is a simple bedside test to determine the amount of oxygen in a baby’s blood. Low oxygen levels can be a sign of CCHD. The test is done using a machine called a pulse oximeter. It’s painless and takes just a few minutes.
Isabella’s parents chose the surgical route.
Since she was so tiny, she spent three months in the NICU at Children’s Mercy with a feeding tube to help her gain weight. She needed to gain weight before she could have her first surgery. She had to be 7 lbs before they could do the Hybrid Norwood procedure. Surgery was done on December 9th, 2009 and everything went well. We spent the day in the Ronald McDonald room, which is a huge blessing for families. She spent about two weeks on the vent while her body rested and recovered, she came off it on Christmas Eve.
Isabella’s family tried to have some normalcy, which was hard with numerous appointments (at Lee’s Summit Physicians Group and Children’s Mercy), home health, feeding tubes, medications, etc. She was a fighter, and continued to grow and make an impact on those around her.
She was able to go on vacations, go to the zoo, try ice cream for the first time (her dad snuck it to her) and get some snuggles. She was spunky. I’ll never forget the sound of her cry. We enjoyed watching the girls play together (they loved their Johnny Jumpers), along with their cousin, Blake, who was glad he was no longer the only grandchild.
Time went by and the second surgery was booked for July 6, 2010. It was scary leading up to surgery as you never know what the outcome will be. We soaked up every moment we could with her. This surgery is called the Hybrid Glenn and is an open heart surgery. We again spent the day in the Ronald McDonald room waiting for updates.
Isabella made it through surgery but had some rough moments. After surgery, she wasn’t tolerating movement, her oxygen was lower than they wanted, and her heart rhythm was more irregular. Over the next few days, while her chest remained open, they made more adjustments. She was so swollen. On July 15, 2010 I got a call that she was crashing. The rest was a blur.
We all headed to the hospital. When we arrived, they said she was stable on ECMO (life support). Their plan was to keep her on ECMO for a few days to let her body rest. While she was lying there struggling, my daughter was winning a baby contest at the Cass County fair. It was so hard to be happy for my daughter and glad that she was healthy while Isabella wasn’t. They were supposed to be healthy and grow up together.
On July 19, 2010 they began to wean Isabella from ECMO. They were able to get her completely off ECMO on July 21st at 9:47 a.m. They said she was doing well. Later that evening, my sister and her husband went to the cafeteria for a brief time. When they came back to the PICU, they were coding Isabella. Her sweet little body couldn’t take it anymore. She earned her angel wings on July 21, 2010.
We all held her and cried. It was too soon. She was only 10 months old, but her story doesn’t stop there. She touched so many lives in her short time on earth. She will always be remembered, and I’m so thankful that more people are becoming aware of Congenital Heart Defects. There has been more than a 33% decline in infant deaths from states mandating screening. And as of 2018, all US states have policies supporting newborn screening for CCHD.
Fly high Izzy B! You will always be loved and missed.
More Information on HLHS
HLHS is a birth defect that affects normal blood flow through the heart. As a baby develops during pregnancy, the left side of the heart does not form correctly. It affects a number of structures on the left side of the heart that do not fully develop.
- The left ventricle is underdeveloped and too small.
- The mitral valve and/or aortic valve is not formed or is very small.
- The ascending portion of the aorta is underdeveloped or is too small.
The CDC estimates that each year 1,025 babies in the United States are born with HLHS. In other words, 1 in every 3,841 babies born in the US annually is born with HLHS.
Hypoplastic left heart syndrome, along with other CHDs, are a hidden condition and most kids look completely healthy.
As I mentioned previously, cardiac screening is simple and inexpensive. If the results are negative, the baby’s test results didn’t show signs of CHD. This type of screening doesn’t detect all CHDs, so it’s possible to still have a CHD with a negative screening result.
If the results are positive (fail or out of range), it means that the baby’s test results showed low levels of oxygen in the blood, which can be a sign of CHD. This doesn’t always mean that the baby has a CHD, it just means that more testing is needed.
Pulse oximetry screening is most likely to detect seven specific CHDs.
- Hypoplastic left heart syndrome
- Pulmonary atresia
- Tetralogy of fallot
- Total anomalous pulmonary venous return
- Transposition of the great arteries
- Tricuspid atresia
- Truncus arteriosus