Clare did great during her catheterization on Wednesday. We are breathing easier having it behind us as we move forward in what's next for Clare. I always have this feeling that we've cleanly jumped over another hurdle and are gearing up for the next one. But we do get some recuperation time in between!
Clare was such a good sport Wednesday morning waiting for her cath to begin. She was scheduled for 9:30am, so she was cut off food at 11pm and water at 7:30am. However, Clare was scheduled second case and the first case ran late. So Clare did not end up entering the cath lab until noon. She never once complained about being hungry or thirsty and was in fairly good spirits. Since the cath did not begin on time, the anesthesiologist started Clare on IV fluids (at our insistence) while she was still awake. (We have learned from experience that anesthesiologists do not like being told how to do their job, but as long as they keep our child safe, I don't need to like them personally!) They gave Clare a Valium before inserting the IV because she was still traumatized by her lung scan injections from the day before. I don't like seeing Clare so upset and wished she could have already been under anesthesia when they started the IV, but dehydration during a cath while under anesthesia is a huge risk for children with Williams syndrome. So having fluids on board since her start time was being delayed was the safest option for Clare.
As parents, the nerves will never go away, and we will never get used to that horrible moment when you simply have to walk away from your child and literally leave their life in someone else's hands. It's been over four years since Clare has been in the cath lab, but all the old familiar feelings came rushing back. Shawn and I walked Clare down to the double doors before the cath lab, gave her kisses, then went back to the waiting room.
The cath itself was fairly short compared to other caths Clare has undergone. Coming out of the cath, Clare had a comparatively "easy" recovery. In the past, she's had high fevers, vomiting, blood transfusions, trouble with the pulses in her legs, excess fluid and puffiness, high blood pressures, you name it. This time, she looked great, her blood count levels were good so she did not need a blood transfusion (first time that's happened - a sign she's growing up!), and she had no fever or vomiting. She was grumpy the night after and complained of ear pain (she had an ear infection going into the cath), but her ears actually looked fairly clear. Clare had an IV line inserted into a vein in her neck so her ear pain could have been from that as well. Coming home, she had a slight fever and seeping from one of her groin wounds, but those are slowly resolving as well. Today, she is pretty active and feeling good. She will be able to return to school on Monday with restricted activity.
The cath was both diagnostic and interventional. Clare's pulmonary stents were dilated open some more (there was some tissue build-up from the past four years), and the stents still have some growing room. Which means we probably have a couple more years before we have to discuss options for stent replacement. The cardiologist also dilated two of her branch pulmonary arteries, but that was the extent of the dilations. Four dilations is a huge improvement over Clare's last caths in 2007. During those two caths, she had over 15 dilations combined. Her lung scan results post-cath showed improvement in the balance of flow between the two lungs and her systemic pressures in her heart were improved as well. That's the fantastic news!
The not-so-fantastic news is that Clare's ascending aorta and aortic arch has narrowed again. Her echo results over the last four years have shown a zero gradient across the aorta, which is perfect. However, the cath showed that was not the case. Her gradient is actually a 25mm gradient. Without going into the complicated details about gradients and pressures, the basic news is that when Clare had her aorta repaired via open heart surgery as a baby, the gradient in her aorta was 50-60mm. So she is halfway to that point again. The problem is that, unlike the pulmonary arteries, unless the aorta has a distinct "pinch" somewhere, the cardiologist will not balloon-dilate it in the cath lab. And Clare's aorta is narrowing all the way up and through the arch again. It will require another open heart surgery to repair it. Unfortunately, we have no way of knowing how long Clare's aorta has been narrowing again. The last time it was precisely measured was during her September 2007 cath, so it could have been slowly narrowing over the past four and a half years or it could have started six months ago. So we do not know how quickly this is progressing.
We have not gone over these results with Clare's personal cardiologist yet, so we will have lots of questions for her at that visit! Thank you to all for your prayers and supports.