CHOP Would….

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Norah's Echo

Norah's Echo at CHOP

I’d never driven further North than West Virginia. But Norah has taken me down many roads I may otherwise have never travelled, both literal and figurative. In fact, I drove very little on our trip to CHOP. Stu and Nana Kim did the driving…and the holding it together. The ride was scenic and lovely. I particularly loved the last long stretch into Philadelphia on I-76. It was lined with tidy picturesque farms, the kind they take pictures of for jigsaw puzzles. Inside the car the three of us worked hard to keep Norah Bug amused. As her Uncle Joni and Nana Kim always say, she requires three attendants at all times. She did so well, though. After 11 long hours in the car we checked into the hotel just in time for dinner and an attempt at rest before Norah’s assessment on Thursday.

Outcome-wise Thursday went very well. But getting through it was trying. Norah was scheduled for an echo at 10 am. They didn’t even get us checked in until 11 am. So, during the hour and a half echo we watched the clock tick past our 11:30 am meeting with the surgeon and then our 12:30 pm meeting with the cardiologist. I was a nervous wreck. Scared that we had come this far and wouldn’t get to meet the surgeon, Dr. Thomas Spray. Norah was napless ALL day. We didn’t get lunch. I went  8 hours without being able to pump (ouch is all I can say about that). But as ever Norah was strong. And she surprised us by being amazingly tolerant through sleepiness, strange noises, bright lights, long tests and nervous parents. She fussed here and there but nothing, NOTHING near what the situation deserved. I cried more than she did. Stu and I were so thankful to have Nana Kim there. She helped with Norah, listened, calmed us (well, me, Stu was calm as ever) and really allowed us to focus on our purpose. Thank you mom!

We did finally meet with Dr. Spray at the end of the day. We first had Norah’s assessment with Dr. Rychik, who has been following Norah long distance for a couple of months now. But there’s nothing like seeing a patient in person and he commented that Norah looks happy, healthy and chubby. After studying her echo he told us that her ASD (Atrial Septal Defect) has nearly completely closed on it’s own. Phenomenal news! He is of course still suggesting surgery to repair her pulmonary stenosis, the business at hand. However, he believes that the problem is not the valve itself, but the area just above the valve where it branches into two pulmonary arteries. There’s narrowing there as if it were being constricted by a tie wrap.

Pulmonary Stenosis

Pulmonary Valve and Branch Arteries

The surgical repair would be a patch to enlarge this area. That would relieve pressure on the valve and improve blood flow to Norah’s branch pulmonary arteries hopefully increasing their size, perhaps even immediately. We discussed the widespread narrowing of  Norah’s blood vessels and Dr. Rychik, of course, doesn’t know what the future holds there. He sounded optimistic that we would  see growth. This is something we’ll follow closely and that may eventually need to be addressed. But not now.

Now, on to the SVAS (Supravalvular Aortic Stenosis). If you remember, this is the defect common in Williams Syndrome patients that Norah doesn’t technically have yet. I say “technically” because this area of her heart does look like it has a kink in it, but blood flow is normal there. Dr. Rychik reiterated their original opinion that they should go ahead and patch this area in anticipation of this defect developing. So, you can imagine our surprise an hour later when Dr. Spray told us he wouldn’t touch this area right now. At first we were uneasy that the two didn’t seem to be on the same page. But then we realized that this was an indication that Dr. Spray was keeping his eyes open and not taking our daughter’s surgery lightly.  And frankly, this has been a piece of this decision that has given us much worry. Could we be causing scarring and narrowing where it might not have developed on it’s own? Were we making the surgery more perilous unnecessarily? We are so glad that this is off the table for now. We know that this may need a surgical repair in the future. But we also know that Norah’s heart is functioning well now against all odds. It gives us great hope!

So, that’s the official recommendation. Surgery to fix the pulmonary stenosis as soon as our schedules permit. And we are embracing this opinion. We feel this is the  place for Norah’s heart surgery. The facilities are clean, open and bright. I mean bright with real sunshine! The staff was great. Most importantly, Dr. Spray is internationally renowned for his skills as a cardiothoracic surgeon. We met a surgical assistant named Christie who was so proud to work with him that she became visibly animated when talking about him. He came across as kind and careful. We also toured the CICU and much preferred the layout to that of MUSC’s.

The one drawback is of course, the distance. But there is nothing more important than getting this step in Norah’s journey right. And in that light CHOP doesn’t seem that far at all.

Again and always thank you to all of you for wrapping us in love and prayers. We feel it and it gives us peace.

Norah Flowers

Norah at a rest stop on our journey

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Philly Bound

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Children's Hospital of Philadelphia

Children's Hospital of Philadelphia

Well the week has arrived. We’re driving to Philadelphia on Wednesday for Norah’s assessment at CHOP (Children’s Hospital of Philadelphia) on Thursday. Horrible acronym aside they have a great reputation in pediatric cardiac surgery and also house a William’s Syndrome clinic, one of only eight in the country.

Norah hasn’t had an echo in nearly 6 weeks. That’s the longest stretch since she was born. Her last few have shown no negative changes and perhaps show a bit of growth. I have spoken by phone with her cardiologist a couple of times but mainly we’ve been avoiding thinking about the upcoming surgery.

We’ve heard opinions now from CHOP, Levine here in Charlotte, UVA, Duke and MUSC. Everyone seems to agree that surgery, at least on her pulmonary valve,

is in the future. But when? And how much? Some say, wait until we see a change on her echos that would indicate it’s time. Some say do another heart cath or ct scan around 9 months to take a closer look. Some say do the surgery this Fall. Some say don’t touch the aorta until necessary because scar tissue can form and need further repair. Some say patch it now to avoid another surgery. Who’s right? There is no way of knowing because these are just opinions. And they all have their compelling points.

I know you are all keeping us close in your prayers and thoughts and we appreciate you so much. Right now we can certainly use that loving energy directed specifically at this decision, Norah’s continued strength and our safe travels this week. Thank you!