Open Heart Surgery and Down Syndrome

I posed some questions to moms who have been there, ones who have seen that the heart defects that their little T21 designer babies have, would likely cause them to leave this earth far too soon without life-saving Open Heart Surgery.

These are the moms who have had to give up control, because the situations were out of their hands.  These are the parents who have to rest in the prayer and hope that their little ones are in the best hands, with the best surgeons.  They pray that their precious little babes will return to their arms with a repaired heart that will allow them to live a full and happy life.

I posed the questions; they gave the heartfelt and true answers…

Sheila is the mother of 9 children, her youngest; Dianna was born with that little something extra, 47 chromosomes instead of 46.

This is Sheila’s story of walking through OHS (Open heart Surgery) with Dianna.

My daughter is Dianna Lee. She was born on April 5, 2012. She is turning five this year. Dianna has 5 brothers and 3 sisters; she is the youngest.

Dianna has had two surgeries. One at 4 months of age and the other at the age of 3.5 years. Her first surgery was at INOVA Fairfax Hospital (now INOVA Children’s Hospital) in August of 2012. We believed that this would be her only surgery. But we learned within two days that she would most likely need a second one at some point in the future, perhaps by age 10.

However, while being monitored every few months, it was decided that she should have surgery sooner while she was still healthy and with no severe symptoms from her leaking valve (the one that was built during her first surgery). She had a repair made to her mitral valve at Children’s Hospital of Philadelphia (CHOP) by Dr. Thomas Spray in October of 2015. It looks to have been very successful.

Going back a bit…

We did not know of Dianna’s diagnosis of Trisomy 21 (Down syndrome) until her birth. We also found at her birth that she was born with a complete AVSD, meaning she had one heart valve instead of two and only two heart chambers instead of four. We had sonograms before her birth that needed some closer attention but in the end, everything came back ‘normal’.

What a funny word.

During my labor, Dianna’s heart rate drastically dropped. The doctor kept a close eye on her and had me do some things to try to improve it. It worked and we thought all was fine. Once she was born, there were many markers for T21. She had swallowed meconium and needed some attention for that. We were told that they believed she had Down syndrome and needed to give her a more thorough check, during which they discovered her heart defect.

Approximately 50% of babies born with T21 have some type of congenital heart defect.

We learned that Dianna would need surgery probably around 3 months of age. Until then she would be put on an oral medication to keep her blood pressure down. With the AVSD, the heart pumps harder. With the increased pressure, not only does the heart muscle work harder, it pushes excess blood to the lungs which can cause irreversible damage.

Left untreated, Dianna would die from heart failure or lung damage most likely by the age of 12.

Dianna’s pediatric cardiologist told us that until 20 years ago, children with Down syndrome were not considered candidates for heart surgery. In his words, they were not ‘worth’ it. The life expectancy of someone with T21 in the late 1980s was about 30 years. We are so thankful that we live in a different time and that Dianna doesn’t have the struggles that some in the past had to face.

Back to her birth…

Dianna and I both had about a 10 day stay in ICU. The hospital wanted to watch her oxygen and get it regulated. Once that happened, they mostly were keeping her since I was also at the hospital still. She was a great eater from the start so had no issues for her weight. Even at birth, she was a good 7 lbs! I’ve heard that is a good weight for a baby born with T21.

Immediately after Dianna’s birth, I had trouble with bleeding. I was rushed to surgery and Dianna was taken to the NICU and my husband was left waiting to find out about both of us. It was a long night.

Dianna was born at 7:30 P.M. I was not in stable condition until 3:00 A.M. the next morning. It was a lesson in not being in control, for all of our family. I spent the first few days of Dianna’s life not even seeing her. I was mostly out of it and didn’t completely realize it. Once I was mostly awake, I just wanted to get home to be with her and the rest of our family.

In the Beginning I really did not understand the complexity of Dianna’s heart issues. We learned as time went on and probably still have lots to learn even after 5 years! I didn’t make it to Dianna’s first couple of cardiologist appointments as I was still recovering. Her cardiologist was great at explaining her condition though, and would take lots of time with us.

We actually learned a lot from the Physician’s Assistant at her pre-surgery appointment just days before her first surgery. Dianna slept, I mean REALLY slept, all the time. Much more than my other babies. She also seemed to be ‘salty’ on the forehead, like she had a good work out and dried sweat on her face. Her coloring was always a little bluer or mottled, which wasn’t completely different than my other children because we are so pale.

 

At the pre-surgery appointment, we found out these were symptoms of her heart defect. Her heart was working so hard, using lots of energy, so she slept more. The extra work also caused her to sweat, not drops, but always a little bit of perspiration that gave her that salty taste. Almost immediately after her surgery her color was far better, too.

As for Dianna’s Specific case…

Dianna had a complete AVSD, only one heart valve instead of two and two heart chambers instead of four. Instead of her blood being separated into oxygenated and de-oxygenated, it would all mix together. What a mess!

During Dianna’s first heart surgery, the surgeon built a wall and a valve using her own tissue. The hope was that this would be the only surgery. But within a day or so, the echocardiogram done in the hospital showed a severe leak in the newly built valve. She remained on blood pressure medicine until her next surgery.

Her second surgery was called a Mitral Valve Repair. After discussing things with her cardiologist, and learning what we could, it was decided to be best for Dianna to keep her own heart valve rather than work with an artificial one or eventually an animal one. There are only a handful of surgeons who even will try to do this anymore. Many prefer (and only have training for) a valve replacement.

Dianna’s second surgery was done by Thomas Spray at CHOP. It has been considered by all who have followed up with her to be a “complete success.”

What did the hospital do to help you prepare for surgery?

The first surgery, we watched a video that was made by the surgeons and the hospital. It helped prepare us for what would be happening during the surgery, but more so the physical condition immediately after surgery. We also were advised to take a CPR class.

I was really concerned with how Dianna would feel about having a large scar on her as she got older. Before her first surgery, I even took a picture of her showing that she had no scar. I thought that I would miss her ‘flawless’ skin. The funny thing is that I have only looked at that picture once since her first surgery! It was not as troublesome as expected. Now, often when I bathe her or rub lotion on her, I tell her it is beautiful and that it is her fixed-heart mark.

During her first surgery, I realized minutes before the surgery began and AFTER we had left her in the care of the nurses and medical staff, that they would be going into her body and that a machine would actually be pumping her blood, not her heart. It was a terrifying moment. I’m certain they mentioned it but there were just so many things and I probably didn’t want to think about it.

 

I feel that we were more prepared before her first surgery, surprisingly. You learn as you go through the experience. So much is going on that you can’t remember everything. So with the second surgery, I was a bit more knowledgeable, but there were still things that were new.

Dianna had a paradoxical reaction to the medication they gave to sedate her before anesthesia. She was like a drunk 3 year old! She flopped and wouldn’t sit still. She was very strong and hard to restrain. I was so worried that she would throw herself on the floor off of the hospital bed. The anesthesiologist came in to check on her and was concerned but professional. She said, “We’ll put that on her chart to never give her that again,” then proceeded to pick her up and carry her to the operating room. We said goodbye at the door.

I think saying goodbye is the hardest part. It still makes me cry when I think of it. You are handing your beloved child over to someone. They’ll cut her open and and literally take her heart into their hands. It is on complete trust that you do this and hope that you will see her again, alive and healthy.

After her surgery, we had to take another CPR course, there in the hospital (CHOP). It was more informative and detailed than the first one. I actually got a certificate. It was helpful to know that if your child is choking on food, it’s more important to get the food out than worry about their chest wounds that may still be healing.

This may all seem shocking, but we rarely think of these moments now. It’s in the past and even as it was all happening, it was manageable, by the Lord’s grace. As my husband would say, we have the hope that if something did happen to Dianna that we would be reunited with her one day, eternally.

In the Present…

Dianna is doing terrific today. Her cardiologist says she’s good for 80+ years, in other words, her heart is as healthy as most. She does still have a slight mitral valve leak (this is the valve that was originally built). As her doctor has explained, many people have a minor valve leak and never have any issues. We hope this is true for Dianna. She did very well throughout both of her surgeries, including the post-op healing, but we would rather avoid any future surgeries.

The biggest thing we noticed was improved stamina. Even after her first surgery, Dianna would take long naps and sleep for a very long time. I almost miss this! Before her second surgery, she would get quite tired during her physical therapy sessions. She would get extremely tired when she would cry; it was a huge workout for her.

 

Almost immediately after her second surgery, she quit taking naps. She couldn’t be slowed down! Even in the hospital within 24 hours of open heart surgery, she was trying to climb out of the crib and playing at full speed.

She is full of energy, far more than I have.

Right now Dianna sees her cardiologist every 6 months. She is a year and a half post op from her last surgery. At her next visit, if she looks as good as she did at her last visit, her cardiologist will want to see her once a year for routine checks. She doesn’t take any medicine now.

One of the things we were quite worried about with an artificial valve was the need to take blood thinners. I know that some have to go this route, but I tend to worry about everything. When we brought Dianna home after her second surgery (she was 3.5), I bought playpens for each level of the house so I could leave her long enough to use the bathroom without her hurting herself.

Within 2 weeks of her “2 month” recovery period (picking up under the arms, keeping her safe, etc.), she fell and knocked her front tooth out. It was a bloody mess, literally. Then two weeks later she fell and knocked the second front tooth out! It would have been terrible if she was on blood thinners. She hasn’t slowed down and doesn’t have much fear of falling.

My Advice to a mom walking this path…

Dianna’s cardiologist told us from early on that we, her parents, are her biggest advocates. Every parent needs to learn what they are able and not just accept what is told them, even if it is by medical professionals. We even went to multiple cardiologists to get second opinions (although many opinions are built into a pediatric heart surgery as each patient is presented to a panel of cardiologists).

Talk with other parents who are in your situation and learn from them. They will all have different stories, but they are all part of educating yourself.

Also, look into the potential surgeons. It is good to know not only their success rate, but the success rate they have of the particular surgery that is being done on your child’s heart. The second surgeon Dianna had was chosen for her specific condition of a Mitral Valve Repair.

If you will have to stay near the hospital during your child’s surgery, ask the hospital. I found that during both of Dianna’s surgery, I could actually sleep right in her room. With the exception of her very first night of her first surgery; at that time I just sat in a chair for the first 24 hours. I wanted to be right there with her the whole time. My husband gave me breaks when I let him.

 

I hope that someone will find these words helpful and will be encouraged if they too are walking this path.

My name is Sheila and I have 9 children (yes, 9) ranging in age from 26 to 4 years old (my extra blessing with Trisomy 21 will be 5 in April). I am a maker. I love to create with yarn, fabric and even food! I began an Etsy shop shortly after my youngest was born, in hopes of helping with therapies and educational materials to use with her.