Caridology lesson and revalations about HLHS.

To any physicians who may be reading this, I apologize in advance for my gross generalization. My friends, these are Heart Valves. At least in my home yesterday, they were what I used for a demonstration of what heart valves look like and how they work. Yesterday afternoon I overheard my girls talking about Emily's heart and asking what "exactly" was wrong with it. Their Aunt on their father's side had told them our Emily had died because of a hole in hear heart. This was not even close to the issue, but I give her credit for trying to help them understand. Emily died because of several issues, and she was actually born with 3 separate "Terminal" heart defects. *FYI, the probability of a baby being born with one defect is 1/400. The odds of a baby being born with ONE "terminal" defect are 1/800. Emily was born with 3 separate (and we were told, unrelated) "Terminal" heart defects. To the knowledge of her cardiologist, she was the only baby to ever be born alive in the united states with her specific series of defects. However, for explanation purposes, I tell people that Emily died primarily because she had HLHS, which is Hypoplastic Left Heart Syndrome. Basically the left side of the heart (which is what pumps the blood to the rest of your body) doesn't form, and the baby is born with what is essentially a two chambered heart instead of four. During my demonstration yesterday, I drew the chambers on paper so the girls could better understand. I also drew the narrowed aorta and explained the problem by demonstration with a bendy straw. However, it was my explanation of AV (Aortic) valves (and problems associated with them) that impressed and delighted even me!

Below are my three heart valves:


This is a standard Tri-cuspid Valve, which is what most people have. We ran water through it, and both girls noted how it held water beautifully, releasing it only when I opened the valve. It keeps the blood in you heart moving in the right direction, and Molly and Kate were delighted that they both have THIS type of valve in their heart.

I then explained this next valve to be a "bicuspid valve" and showed them how it works. I have a Bicuspid (AV) Aortic Valve (the girls now know this) which has been giving me some trouble as of late. (The girls do NOT know that, but have seen me wearing a halter monitor, etc.) I used the water demonstration to explain that a Bicuspid valve can work just as well as a tri-cuspid valve. They were delighted with this result.

Finally, I showed them Emily's valve, pictured above. Emily had a Bicuspid Aortic valve (AV valve) with "Aortic insufficiency". Her valve was leaking and very deformed, and the girls were both shocked to see that the water ran right through the valve, and just sloshed around in the sink (which for our purposes represented the heart.) I think the visual representation was very helpful to Molly, and I know Kate just enjoyed playing in the water, and with the valves.... as evidenced by this next picture. Kate and Molly then proceeded to stuff the "valves" down their shirts, and run around the living room screaming "I have a heart valve! I have a Heart Valve!"

I am glad that my girls are processing, and reprocessing the events of our lives. I explained to Molly this is VERY normal, and invited future conversations about the subject. I told her I KNOW her questions will become more and more in depth as she grows older, and let her know I am ready and very glad to answer any questions she has. She doesn't ever have to worry about making me sad by asking because I LOVE to talk about Emily, and feel it's important that they know what happened so they can be educated in the future.

As I was going through my own heart testing back in April, I learned a few things about my heart problems, as they relate to Emily's condition. This information has set me back a bit in my grieving process and honestly, has been very difficult for me to handle - although I continue to try. As a result I have only shared it with a few very understanding people. With that said THIS INFORMATION COULD BE SENSITIVE....

Since April, I have learned:

• HLHS is not caused by a "fluke" as we were told five years ago. Within the past year, they have discovered that HLHS begins at about 11 weeks and is caused by a stenotic AV valve. The AV valve does not open properly, and the left side of the heart does not form because of the issues with blood flow.


• AV valve problems are also NOT a fluke - most often (but not ALL the time) they are genetic. The gene is passed down on the Mother's side, and so my probability of having child with an AV valve defect ( because I have one myself) is 25-50%. This helps me understand why I have had 5 pregnancies, and have brought 2 children home. Apparently, I hit the 50% mark.


• Molly and Kate's probability of having a child with an AV valve defect is still 25%, even though they have no problems themselves. This is due to the fact they have a full sibling who died of this defect. I will have to find a way to explain this to them in the future. I'm still thinking about how I might approach that conversation, when the time comes......


• 7 years ago, HLHS was a death sentence, and there were very few surgeries to repair this defect. The surgeries that were available typically had "less than ideal" outcomes. Just 5 years ago (when Emily was born) survival rates for babies with HLHS was 20%, and surgical outcomes were still "Less than Ideal." TODAY, survival rates for HLHS are up to 70% - which I find amazing.... but I am still sad and even a bit angry at times that my daughter could not have been part of that 70%.


• There is a "fetal surgery" that can be done at about 12 weeks gestation for babies who are diagnosed in utero with a "stenotic AV valve". This surgery will PREVENT HLHS from developing! A catheter with a balloon is inserted into the infant's heart and essentially, it "blows" the AV valve open. At birth, the baby requires one surgery to replace the AV valve! *In 2003, the AV valve could not even be replaced in infants...today it can, and is done routinely.


• There is a push by perinatologists to perform fetal echo cardiograms as standard procedure around the 12 week mark for Mothers who have had a child with a Congenital Heart Defect. I fully support this movement.


• Molly and Katie thoroughly enjoy creative demonstrations about how parts of their body work. I have learned I can explain the death of their sister in a very "matter of fact" way using McDonald's Sundae lids, and not even cry while I am doing it! I have also learned, no matter how strong I am during these discussions with my girls, after they go to bed that night I will inevitably break down, with a pain in my heart I had almost forgotten existed.


• I have learned that after five years of grieving, I still blame myself for the death of my daughter. I have been told that I shouldn't - but I do.


• I have learned that guilt is perhaps one of the most painful companions of grief.