What do Claire and Darth Vadar have in common?

Not the old Darth Vadar.  But the new one - the young one - the one on the VW Passat commercial that was such a big hit during the Super Bowl.  Well, I was unaware of anything that they had in common except that Claire and the Darth Vadar kid were both cute!  But yesterday several people sent me messages telling me that the little guy who played Darth Vadar in the commercial (Max Page) was also born with Tetralogy of Fallot. 

We had never even heard of Tetralogy of Fallot until August 25, 2010 when the fetal echo cardiogram showed that Claire had it.  Since then, we have heard of so many people who have been born with TOF and you would never even know.  Even Shawn White the Olympic snowboarder was born with TOF. 

I don't remember if we have ever posted anything on TOF.  Most of our posts have been more of a "what's going on today" type thing or focusing on the rare mesenchymal hamartomas.  So instead of writing a big long post about it, here is a link to the American Heart Association's website with an explanation of TOF:  http://www.americanheart.org/presenter.jhtml?identifier=11071 

Claire has all of the classic symptoms of TOF except that she's considered a "pink tet."  Basically the doctors have a scale - instead of it being 1 to 10 where 1 is severe and 10 is good, they use blue and pink since TOF is a cyanosis (blue) heart disease.  Claire is considered pink which means she doesn't have the typical blue hue about her skin.  She looks like a normal pink baby.  She has a high flow in her pulmonary vein.  Most TOF babies have a small PV and so does Claire.  But hers is big for a tet baby and she gets good blood flow therefore making her less blue and more pink.  The catch 22 with this is that the cardiologist said that pink tets are more susceptible to having the tet spell where they turn blue.  Hence scheduling her open heart surgery soon we think. 

Claire also has a right aortic arch.  This is not common with TOF but sometimes accompanies TOF.  It should not affect Claire and will not have to be repaired like the other issues with her heart.  It's just another anomaly.  The right aortic arch with TOF is most of the time a sign of Di George Syndrome - a chromosomal disorder.  But Claire was tested for Di George when she was born and it came back negative.  So she just has a different kind of heart!  A big strong one at that! 

Below is the actual 1st sketch we received of the explanation of TOF from the doctor in Greenville before we went to Duke.  Some of you have already seen it.

I highlighted the areas so you can see where to compare.  The top heart is a normal heart and the one at the bottom is Claire's heart.  You can see where the yellow highlighted area is - this the hole between her 2 bottom ventricles.  This should be closed like in the top heart.  But Claire's is not.  Then you can also see that the Aorta is green and the Pulmonary Artery is pink.  Claire's PA is smaller than a regular PA.  It also has some muscle tissue built up at the opening that blocks blood flow.  You can also see that Claire's AO curves to the right (you have to look at the heart backwards) which is not normal compared to the top heart. 

When Claire has her surgery, the yellow area will be patched and closed.  The pink area will be opened and I think the built up tissue will be removed as much as possible.  We will find out more in detail next week I hope when we meet with the heart surgeon. 

So anyways, just thought it was interesting that kids/people born with TOF are out there and can do big things with their lives.  That's exactly what we are expecting from our Claire Bear and her special heart!

Love,

The Scott Family