Friday, April 11, 2014

The Year I Missed It

This is posted over at Down Syndrome Blogs:

Every year, I have celebrated Elise's Heart Day, except this one.  It has been 12 years since she had her open heart surgery repair.  Twelve.  She's 11.  It somehow slipped by me this year, as we were celebrating at the orthopaedist office being freed from her boot after breaking her foot.

When she was born, we were surprised by the fact that she had Down Syndrome.  She had 2 ultrasounds while I was pregnant, one to confirm that I was pregnant with her and one to tell us that she was a she and that she was breech.  At the second one, they tried to tell me that she wasn't as far along as previously thought...because she was so tiny.  And they spent an inordinate amount of time looking at her pinky finger.  When they finally confirmed that she had the usual 2 joints in it, they looked no further for any evidence that she wasn't perfectly normal.

Had they looked further, they probably would have caught the Down Syndrome.  Had they looked further, they would have discovered the mess that her heart was in.

When she was born, she was shuffled home with massive apologies for missing the Down Syndrome.  And by apologies, I mean my obstetrician almost wept for not catching it, when he suggested that "other people liked to adopt Those Babies" and we didn't have to keep her.

At the pediatrician, they seemed in over their heads in dealing with Down Syndrome.  They had to look up everything like she was a foreign object, rather than a standard issue baby.  At her 2 week check up, they caught a slight murmur and suggested that we go in for some testing and visit a cardiologist, "just in case the murmur didn't resolve".

Our cardiologist was the kind of guy that gives that vein of medicine a bad name.  He was arrogant.  He didn't listen.  He disrespected us at almost every turn.  I was still barely keeping my head above water with her diagnosis at 3 weeks out and didn't have the mental fortitude to slap him down or get another doctor.

After a sonogram of her heart and an EKG, we were graduated to a diagnosis of a small hole or two in her heart in the middle dividing muscle.  They still believed that they would resolve and she was sent home.

She was still a minuscule baby.  She hadn't hit 6 pounds yet and she hadn't gained back the weight she lost right after birth. 

By January, she still hadn't wrestled her way to 7 pounds, and she had a second routine cardiology appointment, and she was pronounced in Cardiac Failure.  Suddenly the medical world started literally buzzing around us.  We had to "buy" as much time as possible.  I was to pump and mix into the breast milk both preemie formula and corn oil to get her enough calories to "live until surgery".  We were praying for not only survival, but we were praying desperately for a few extra ounces of fat to help her withstand the cracking of her chest and the toll the surgery would take on her tiny body.  The pressure was so high, and she was so exhausted keeping her heart pumping, that I was literally pouring her high calorie potion down her throat with a syringe as she had no energy to nurse nor to suck on a bottle.

Because of her size, she needed to go to a hospital that specialized in heart repair surgery.  We were given the option of Vanderbilt and Cincinnati Children's.  We jumped at Cincinnati, as it was exceedingly reputable, and I had family there.  And not only was it family, but my uncle was a doctor, had the inside track on everyone's reputation.  Based on his research and advice we chose our cardiac surgeon.  And I cannot say enough good things about Dr. Peter Manning.  Not only was he stellar technically, but he was an amazing man with a warm and encouraging bedside manner.

At the EKG and sonogram there at Cincinnati, we discovered that the holes were bigger than originally thought, and that there would be a need for patching, not just stitching.

And I noticed for the first time that a heart ultra sound looks like a butterfly.  And that as it pumps, it looks like it is flying.

They expected that her surgery would last 3-4 hours. It appeared to be a fairly easy patch job, with the only expected difficulty being that she was so tiny.  We had successfully clawed our way to 7 lbs, 3 4 months.

The surgery lasted just over 6 hours.  They got in and discovered that it wasn't a simple patch job.  That both the EKG and the sonogram had missed just how many and large the holes were.  That the holes were responsible for her having to work so hard to live...and why growing was simply a bonus miracle.  She was patched up for both an ASD (atrial septal defect) and a VSD (ventricular septal defect), and her mitral valve was also flapping loosely allowing the blood to wash back into the heart, and so he stitched that up as well, but he was very cautious in not allowing that to become too tight and cause any other problems that would lead to another surgery.  She still has a tiny amount of cardiac reflux to this day...but it's not slowing her down.

The real miracle is this:  Open Heart Surgery to correct malformations has only been performed since the mid 1950's.  Even into the 60s-70s, a child with Down Syndrome was recommended not to over-exert themselves as a primary form of treatment for heart malformations.  Into the 80s, doctors could and would routinely refuse to correct heart defects on the grounds that it was a wasteful surgery that was unwarranted.  There is even an article in the US National Library of Medicine which addresses the fact that parents were being steered away from surgery based on Down Syndrome as recently as 2001.

The year of her birth was 2002.  Her surgery was February 2003.

The surgery was visibly a success from the moment we saw her, we knew it to be so.  Before the surgery, she was white as porcelain, and had no energy.  She slept all the time.  She never attempted to roll over or do any milestones babies her age did.

When we saw her after the surgery, moments after being moved to the CICU, we saw pink cheeks for the very first time in her life.  We saw her fighting her restraints to yank off the breathing tube under her nose.  The nurse greeted us with "I swear, she is acting like she'd like to jump off the bed and run down the hall!"

And as soon as she got home, she a made up for lost time.  She couldn't get her eyes wide enough.  She couldn't try hard enough.  She couldn't LIVE enough.

And that was the first chapter of the butterfly that is Elise.  Since then, she has fought bigger and bigger battles, but thanks to that heart of courage of hers...she is always the victor.


  1. Elise has been a fighter since day 1. Love the last picture of her! I am not a big fan of all the first docs that you had. Seriously? They should have had her lined up with a cardiologist before she was discharged from the hospital at birth! I am ever so grateful that Elise had such a good surgeon. That can make all the difference. I will tell you this. I worked in a PICU/CICU from 2002-2004 and the majority of our cardiac babies had Ds--mostly AV canals. It scare me to think that just prior to that advice was given to docs to just not perform these lifesaving surgeries!

  2. Beautiful, beautiful post. Happy belated Heart Day to Elise! I'm so sorry you had such a bad time with arrogant doctors, but I feel that we are so fortunate to have had our beautiful children at a time when these kinds of repairs are routine for children with Down syndrome. I met an amazing young woman with Down syndrome last weekend who had a heart *transplant* two years ago, after numerous other cardiac surgeries. She said that when her heart began to truly fail, her parents did some research and found that there were 2 other people with Ds who had had transplants, so they, and her cardiologist, pushed for it and got it, and now she's thriving, able to carry on her message as a self advocate and a beautiful example of what medical intervention can accomplish.

  3. I can't believe doctors used to be able to refuse to perform surgery on kids with Down syndrome! I mean, I can believe it, but I can't BELIEVE it. I am happy that Elise got her surgery and that it helped her to become the vibrant kid she is now!