But the real purpose of the day wasn’t the echo itself, but the appointment after it – meeting with Namine’s cardiologist, Dr. Camarda, to find out the results of the echo. We expected a longish wait time, but he met with us almost immediately.
Before Dr. Camarda came into the room, the nurse took Namine’s oxygen saturation and heart rate. Her heart rate was less than 110, and her O2 was 91. I could hardly believe it. We really can’t take Namine’s sats at home – even though we do have a pulseox machine, it’s unreliable at best. (It tends to be off by more than ten points when it reads O2.) So to be honest, I expected that Namine’s O2 would be in the mid to low 80s range. Seeing her O2 that high even now, at the age of three, meant that her heart is likely still doing fine.
When Dr. Camarda came in, he was impressed – more like astonished – by how verbal Namine is. The people we had met for Namine’s IEP would have been impressed by not only the amount of talking she did, but also by how understandable (and not just to us, but also Dr. Camarda) she was.
The doctor explained to us that Namine’s heart is doing just fine. More than that – her heart is healthier than any post-Glen patient he has. Namine’s good skin color, her high O2 level (for a post-Glen, at any rate, and that’s what matters), and her echo results all point to a healthy heart which has absolutely no need to be operated on. He expects that perhaps in a year from now, Namine will need it – eventually every Glen patient needs the Fontan to complete the series – but not now. We scheduled a checkup for six months from now, but Dr. Camarda sees no need for another echo for another year. And certainly not a cath; he sees no reason to poke and stick her more than necessary.
Namine had been on an iron supplement, prescribed by Dr. Gordon, her special needs doctor, because there was a possibility that she was anemic. That fear, however, has been dispelled; Dr. Gordon feels that Namine’s excellent test results after the echo indicate that she is not anemic after all. So no more iron supplement, either. Good thing, too, as iron really messes with going potty. (Yes, I am the father of a three year old, and I say “potty” without shame.)
Some time ago now, it was explained to us by Dr. Camarda exactly why Namine’s heart is doing so well. If you don’t recall, allow me to refresh you.
Being born with caudal regression syndrome (CRS) can mean many different things, as it differs so vastly between patients. It is not very well understood, because it is a rare condition (1 in 25,000 live births is the current statistic). In Namine’s case, it meant a partially formed sacrum (tailbone), severely shortened and bowed femurs (thigh bones – the right in particular is almost crescent-shaped), no patellas (kneecaps), and clubfoot (club foot? clubbed feet? I don’t know – the term seems to vary depending on who you talk to). We had been told since Namine’s birth that her hips were dislocated, but on closer examination, Namine’s new ortho doc revealed to us that they are indeed located. (This explains why Namine can bear weight and is able to learn to walk.) We also found out that Namine’s tibias (shin bones) are corkscrewed, further twisting her feet inwards (also explaining why her feet are still turned so far inward, despite having had clubfoot repair surgery).
But that information is probably overkill. The important thing to note is Namine’s extremely short legs – meaning much less muscle for her heart to support, much less blood for her heart to pump. This ultimately means that her heart does not need to work nearly as hard as her fellow DILV (abbreviation for Double Inlet Left Ventricle, the type of congenital heart defect Namine has) patients. And this is the reason that Namine’s heart is doing so well now, at a time in her life when, had she been born with “normy” legs, she would sat lower and in all likelihood require the Fontan now.
Some say there is no design to the world. Life is mere chance, and death is ultimately meaningless. I, however, have faith in a God who saw fit to save my daughter’s life by blessing her with caudal regression. Her strength and determination are born of survival, and she is proof to me that God exists. And not only exists: He loves us and does more than we will ever understand. So I praise Him for my daughter, whose heart He has made strong.
This post is part of the timeline: Heart Repair – an ongoing story on this site. View the timeline for more context on this post.