Friday, June 3, 2011

be still my beating heart

People we see on a fairly regular basis, as well as most family members, already know that Emma has several congenital defects in her heart. To everyone else: Emma has several congenital defects in her heart. (Surprise!)

The worst of the defects, called Coarctation of the Aorta, was repaired when she was just 17 days old. Basically, it was a very narrow section of her aorta, and prevented adequate blood flow to her extremities. Since we were still brand-new parents wrestling with the reality of actually having two real crying, hungry, pooping, helpless babies, the whole thing was kind-of a blur. It wasn't until Emma was back in the PICU, puffed up on IV fluids and hooked up to every machine in the hospital (including the most expensive one and the one that goes "ping!") that we really felt scared.

Since infancy, Emma has had annual visits with a pediatric cardiologist to keep tabs on the next most serious defect, called Subaortic Stenosis. This defect is basically a membrane growing across the aortic valve, between the left ventricle and the valve itself. The membrane affects the bloodflow into her aorta; instead of flowing straight through the valve like it's supposed to, the blood swirls around and hits the valve itself. The effect of the blood hitting the valve day after day, heartbeat after heartbeat, eventually causes it to start leaking. In Emma's case, her aortic valve is also bicuspid instead of tricuspid. (For the record, Emma's fourth and final heart defect is a stenotic (narrow) mitral valve.)

This year, after reviewing her echocardiogram (ultrasound of her heart) Emma's cardiologist and surgeon agreed that the leak is starting to get worse. In the past year, it has gone from holding steady at "trivial" to deteriorating to "mild-to-moderate." In order to (we hope) slow or possibly halt the deterioration of her aortic valve, the doctors want to remove the membrane now, before the leak progresses to "severe."

Removing the membrane will require open-heart surgery. On my Emma.

This past Tuesday we met with her cardiologist again, had all her pre-op work done, and had a brief visit to the PICU at Tufts Floating Hospital for Children in Boston. Emma got to see a room similar to the one she'll be in, and was reassured to know that she'll have me either in-room with her or right down the hall (while she's still on a ventilator & sedated). She also got to meet her surgeon, Dr. Warner; he's the Chief of Cardiothoracic Surgery and the same surgeon who repaired her coarc eight years ago.

It's bizarre to consider the reproductive future of your eight-year-old daughter, but we want to keep Emma's factory-original parts in and working for as long as possible. If she ends up with a mechanical valve, it will complicate any future plans for pregnancy. Likewise, biologic valves come with their own risk of rejection. The longer Emma's own valve -- defective and misshapen though it may be -- can remain in her heart and working well, the more control Emma will have over her adult life.

Right now, we're trying to strike a balance between quarantining Emma completely (thus scaring the piss out of her) and maintaining the status-quo. Some of the things we would normally only be bugged about (like playing with a neighborhood kid whose nose is running like a soak-hose) are things we need to prohibit until after her surgery. We feel a little paranoid, but this is one of those times when paranoia is a good thing. According to the surgeon, once Emma is home, she can "resume normal activities" with the temporary exceptions of roughhousing and contact sports.

It's a good thing she's been considering taking a break from wrestling. See? It all works out!

1 comment:

  1. Aaaaah! So stressful! I'll be thinking of you guys ... I'm sure everything will be FINE, but I hate seeing ANYTHING painful/scary happen to my kids, so I can only imagine .... good luck!

    -- Anne Gowen