Braveheart

Reuben is going to need open heart surgery imminently. When I look at the facts, delaying would only cause the gradual and inevitable demise into heart failure once again.

This is unrelated to the bad incident last week when Reuben was in the ER being sick and his heart rate plummeted to 46 bpm. That was actually an electrical issue in his heart, the sinus node, the heart's natural pacemaker, not firing timely signals, rhythms. When that happened, and I've yet to write on it here, within seconds there appeared a swarm of Drs, the crash team. So here he is wearing his Holter heart for 24 hours to monitor the rhythms.

Today's news which isn't new news at all, just that it's been pushed to the Why wait? agenda, is more of structural heart issue.

Reuben has a flap of tissue left over from his first open heart surgery in August 2007 and it's causing mild-moderate turbulence (66). That turbulence causes his heart to beat faster - I could feel it at the place where his tracheostomy stoma used to be, purring away like a kitten - and if it beats faster, the heart, being a muscle, gets muscles in the form of a thickening of his heart.

In order to avoid any further thickening, that pressure needs to be relieved. It's akin to putting your finger over a hose, that tiny flap of tissue causing the intermittent blockage. And the extra tissue ironically is a remnant, whether scare tissue or a suture left over from his first heart surgery.

Open heart surgery involves stopping the heart, in this case, thankfully for only about 10 minutes. He's previously had his heart stopped for more than an hour aged 3 months, after which it took time for his natural pacemaker to kick start again and allowed him to come off the bypass.

To get access into the heart, you have to break open the ribcage. I remember sitting with my NICU friend Cassandra back then and thinking, about now they'll be breaking through the wall. I also remember the blank expression on the face of the genius surgeon, Dr Starnes, dubbed the hands of God, his fingers perfectly balanced, dancing through the minuscule part of my newborn's heart with the dexterity and grace of a ballerina, as he walked towards us from the OR. I actually fainted at that point fearing the worst. I hadn't before nor since.

Kissing your baby goodbye as they are wheeled away to surgery, that memory is coming back to me now. It's happened 17 times now in addition to the procedures under anesthesia, but nothing can compare to the torturous weight that is felt on the cusp of heart surgery.

I don't want to sound dramatic. I'm actually speaking up for the 100s of families whom I've been connected with in CHARGE and in the world of congenital heart disease. I'm thinking of all the children who have had to suffer so. I know too many.

Sometimes you can be quite remote from CHARGE Syndrome till it comes up again and viscously bites you on the butt, daring you to ever forget the diagnosis. And there's still the congenital spine fusions to think about once again this Spring and reconstructive surgery on his neck, bone grafts, the metal halo in his head and promise of never being able to move his head to the right again. Not a great promise.

When you visit the Craniofacial clinic on a yearly basis as in today, separate from the heart appointment - oh yes did I tell you it was a good day? - the catalyst for which was his recent ER trauma, you walk away with a shopping list of potential surgeries resulting from cranial abnormalities that exist, from individual appointments with the specialities of Plastic surgery, Dentistry, Orthodontics, Ear, Nose & Throat, Speech, Audiology and beyond, everything that relates to the cranium. As complex as this list sounds to the layman, there is also the reminder that these are solely the head issues and in CHARGE you have every other medical facet of the being to consider too. Are his torticollis and neck issues causing his jaw to gradually become more elongated on one side than the other. When these abnormalities are pointed out to you, how do they make you feel about your child's face as you look upon it? Does it feel different now having an aspect of your own child's face pointed out that you've never seen before?

These appointments - are they necessary? I wonder, pull apart the asymmetry of his face, his speech, sight and hearing and push medical science to the limits of what can be achieved to normalise such basic functions as breathing, eating, sleeping comfortably and living as happy and typical a life as change possibly be achieved. I recently watched a programme about Operation Smile whilst in the hospital and was warmed to hear from one of the Drs how she's been involved in this surgeries to repair a child's cleft lip and palette, giving them one of the greatest gifts imaginable, that of being socially accepted.

I'm weepy thinking about how I can start to explain all of this to Reuben, the owies. He's been pointing at his old IV site today from Friday, expecting more of the same with the barrage of Drs who have seen him, yet he still found time to be humorous with the large group of orthodontic specialists all peering into his mouth at the same time, Reuben saying "aaaaaaaaahhhh".

Dentistry came near the end. At least we were commended that his teeth are clean.