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.
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.
5 comments:
How heartbreaking!! I am so sorry. You are so true about almost forgetting about CHARGE and then it bites you HARD in the butt. Good luck- I hope everything goes well!!!
Been thinking about you constantly... with love and great empathy, of course.
Continuing to hold you close in my heart and mind as you prepare for yet another surgery.
As for craniofacial clinic... my advice remains the same: Consider whether a yearly laundry list of "assymetries and anomalies" and potential "fixes" are worth it. Does the list change? Are your decisions about moving foward with anything changing within the next handful of years? Are you getting new info that you're not getting from your pediatric dental specialist? Since he doesn't have a cleft, certain things are not going to change unless medical technology changes...
Are they using good discretion and judgment when discussing the "issues" in front of Reuben? (Some clinics don't remove the child from the room... not sure how your's works). Anyway, I guess my point/question is... at almost five, is there anything you intend on doing with this information within the next few years? If something changes in the medical field with regard to procedures, etc., you will likely be aprised via our CHARGE groups and/or Conferences... or if you have questions, can you phone/email consult with panel members of that clinic?
Just a thought... something to remove that painful (maybe, currently not terribly necessary) annual appointment from your calendar until you need it again.
Hugs to your mamaheart... I know it is hurting so much.
Stay strong, Reu! (and mom!)...hoping the bites in the butt like this are nearing their end for him. I have had quite a few reminders from dear friends about the CHARGE bites...
Hugs & Love
I hope everything will be ok with the operation, our boy had open heart surgery when he was nine days old. I remember the moment when they took him away and said he might not survive...it is an awful feeling. I wish you and your sweet boys all the best.
Just wanted to say that I am sorry that Ru has to go through this and that I think you are an inspiration. I read your blog from time to time and you deal with being a Mummy, running an amazing business and dealing with the huge complications of CHARGE better than most Mums I know deal with a common cold!! Hats off to you! I wish I had just a fraction of your energy, determination and positive attitude!!
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