Monday, February 1, 2010

CHOP Visit #1

Where to start.

We had a fetal echo-cardiogram and a level II ultrasound. Then we had lunch and waited and waited. Finally, we met with the pediatric cardiologist and the maternal-fetal OBGYN. Another long, long day.

Diagnosis; Still Going with Truncus Arteriosus with Valve Insufficiency and Stenosis
They (CHOP docs) agree with UVA that it is most likely Truncus Arteriosus. They believe it to be Type 1. That is good news as Type I is the simplest presentation of this complex issue. They still will not know if this is indeed Truncus Arteriosus or not until she is born. But we are still leaning in that direction.

However, the valve regurgitation (it does not open or shut completely) and now stenosis (thickening and stiffening of the valve) is a seriously complicating factor. Most patients with Truncus Arteriosus have some form of valve issue but it minor and the valve is still essentially good enough. This is important because they actually use this valve as part of the "fix" (keep reading).


Major Problem = Insufficient and Stenoic Valve

There are two problems that result from an insufficient valve:

1. The first problem is that it places her on high risk for arrhythmia in-utero, and the longer she is in utero the higher the risk of "sudden death" because of the arrhythmia. Since her heart has to work extra hard to compensate for the valve problem it cannot tolerate an arrhythmia. At least, that is the theory - they simply know that with such a valve, "sudden death" is something that has been witnessed. The theory is that arrhythmia is the culprit, but it they really don't know for sure. The fact that the heart has to work extra hard means an arrhythmia can occur - so the logic is sound. Hydrops does not seem to be something they are even that concerned about now - as that is more chronic and happens slowly enough that it could be caught (what they would do, I don't know). Arrhythmia, in there "limited but scary" experience is acute - and impossible to predict.

2. The other major problem with her valve is that it may not be usable during corrective surgery. The standard "fix" for Truncus Arteriosus involves using this "native valve" as the aortic valve. A synthetic valve is used for the pulmonary artery valve. A well-working "native valve" is very important because the aortic valve must withstand a higher pressure than the pulmonary valve. Artificial Pulmonary valves are well tolerated because the lungs run at a lower pressure than the rest of the heart. Artificial aortic valves are less tolerated by the heart and require blood thinning drugs; not something great for babies and toddlers - it is hard to regulate the drug simply because of their irregular eating habits and since toddlers are often getting bumped and bruised as they "toddle" around, having thin blood is not helpful.


The Plan - Surgery Sooner Rather than Later

Due to her risk in-utero they decided she needs to be born and undergo surgery sooner rather than later. They basically want to induce as soon as her lungs are developed fully. This means they must do an amniocentesis at 36 weeks (second week of March). From the Amio then can determine if and when the lungs are "ready." They also want us to do genetic testing - the OB suggested that the more they know before she is born the better they can plan for surgery. The OB explained they might find some genetic issue that would be important for the surgeons to know.

Lungs are fully developed by 38 weeks; sometimes they are developed as early as 36 weeks (30% chance). So, we now now she will be born somewhere between March 12 and April 2nd. Surgery will, ideally speaking, follow three to four days after she is born.

The plan is for an induction - this is better than a c-section in her case. The hope is a c-section will be avoided and that the induction will flow well. I'm still holding out for an all-natural birth (beyond the induction), as meds can slow things down and lead to c-sections. We won't get to hold her - she will be whisked away right after she is born and once she is "ready" in cardiac intensive care unit she can be touched but not held. She'll have a feeding tube and lot's of other things hooked up to her.

Our next appointment at CHOP is the Monday following our temporary move to Philly. We'll do the same thing we did today (echo and ultrasound) and meet more docs and see more of the hospital.

10 comments:

  1. Still praying for you all. My heart is heavy for Gwenyth and for you two as well...but God's grace is sufficient and you are all in His hands. Thanks so much for the blog, it's great to know what to specifically pray for and to keep updated. Please let me know if we can do anything for you. Love and blessings!

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  2. Oh, how my heart aches for y'all. Praying you will know His peace that passes all understanding as you walk through your dark tomorrows... praying for miracles and healing. Much love.

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  3. Wow. Thank you for keeping all of us updated. You're in our hearts and prayers.

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  4. We are praying for you all ... for peace and strength as you go through the unknowns, the preparing and move to Philly, the fears. Praying daily for a strong little Gwenyth.

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  5. I love you and I know you will get through this! Stay strong!!

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  6. Myers and Laura,
    I'm so sorry to hear that you have been going through all this with Gwenyth. Brian, Maddy, Lily and I all have you on our minds and wish you much love on this journey. Patricia Ochs

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  7. Laura and Myers,

    Thank you for taking the time to write out these details so we can pray. Your daughter is already loved by many.

    Adina

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  8. I just came across your blog searching for some Truncus info. I will be praying for you little girl. My son is 3 and he too, has Truncus. If you have any questions or would like to talk, please feel free!
    www.caringbridge.org/visit/mittelstaedt
    Gage's Mommy, Chantae

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  9. http://www.mycharmingkids.net/search/label/Stellan%27s%20story

    I thought you might like this story of a baby with a sick heart. It was a roller coaster ride, but it has a very happy ending.
    God bless you and your family.

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  10. Praying for you guys and little Gwen mightily!! {{HUG}}

    Love,
    Brooke & the rest of the Chaos.

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