Tuesday, September 27, 2011


Yesterday Ryan and I needed to bring the two youngest to the cardiologist for checkups.  Teagan had a pulmonary valve that wasn't working perfectly when she was born, but the doctors believed she would out grow it by two.  Her doc could still hear the murmur so we had to go check things out and thankfully she was given a clear bill of health and we were told that the sound persists, but the valve is working perfectly! I was so relieved as out of the five kids, four have had to see the cardiologist and now all the girls are cleared.

For Finn we are monitoring a more significant defect.  We are thrilled the little guy has grown even more, pushing 20 lbs at long last!  We told the cardio that he was being tested for elastin deficiencies related to his heart defect and cutis laxa which impacts multiple organs in the body including the skin, heart, lungs and gastrointestinal system.  She examined him and noted the same things the geneticist saw, his skin texture, his hypermobility and low muscle tone.  The form of cutis laxa they believe he has is very rare, and seeing it combined with his heart issue which we believe to be genetic is even more unusual.  I am glad we are getting the testing done so we now what might be coming down the path, sadly elastin is in almost all our organs and when multiple organs are impacted, we need to be vigilant in our care for him,  and proactive in his health.

Sadly when they did his echocardiogram we did not get the same good news that we did with Teagan's.  They measure the severity of his narrowing in his aorta using a gradient which correlates to pressure.  There are different levels of SVAS and while his is still in the mild range, his gradient as a whole increased 5 full points in less than 4 months.  With the rapid growth babies go through, we are anticipating this happening again and we may be looking at stepping up into the moderate range of his blockage when he goes back in February.  As I have the same heart condition, I know the importance of the gradient and had hoped his had remained stable, seeing such a big jump in such a short time frame worries me.  While I knew that surgery is more of a when and not an if, I really hoped he would be more like me and get to wait until he was well into adulthood to face the surgery, but it is not looking as promising.  The good part is he is still considered mild and there is the possibility that we will not see a change in February, but I believe we may be facing surgery before his tenth birthday and possibly even sooner than that if the gradient continues to rise or further impedes his valve from opening and closing properly.

The surgery he would need is pretty invasive.  It is open heart surgery and has all the complications and healing issues associated with that type of surgical intervention.  The surgery works well and is performed enough that I am confident he will be fine once it happens, but it just stinks knowing it is coming.  Add to that his other concerns, one of which is if the cutis laxa will impact healing for him, or if it will affect his lungs in any way (emphysema is common in kids) makes me a little scared, but we are focusing on the positive and will handle the rest when it comes at us.

Developmentally Finn is on track.  He pulls up on everything and cruises the furniture.  He self feeds, he plays well, he is reactive and silly, so the rest is secondary.  I wish I could express the joy he brings us every day, he has a huge smile with big squishy cheeks, he laughs and hugs and loves to be played with.  He is my snuggly baby, happy in my arms at any time and he finally, finally, FINALLY is sleeping a bit better!

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