Diagnosis: CCTGA, PAPVD, DIOLV, tricuspid atresia, hypoplastic right ventricle, hypoplastic & interrupted aortic arch type A, total pulmonary stenosis of left lung
Originally Millie, or “Mills” as we call her, was born weighing 4lb 15, and because of that the surgical team in Bristol were unable to pursue a surgical route as they just plain didn’t have the experience carrying out such a complex procedure on such a small baby.
Evelina’s in London was approached as they DID have the experience, and at 5 days old, still on her Prostin, she was moved to a lovely PICU just across Westminster Bridge from Parliament. There they fought over the weekend to stabilise her as she’d taken a turn for the worse early Sunday morning, and at one point there were serious concerns on her even making it through the night. But she did, and two days later she was taken for a CT scan. It was then that the true nature of her heart defects became apparent, and they discovered no less than 6 defects;
congenitally corrected transposition of the great arteries,
partial anomolous pulmonary venous drainage,
double inlet outlet left ventricle,
hypoplastic and torturous aortic arch,
hypoplastic right ventricle
tricuspid valve atresia and
Since her surgeries she has also developed total pulmonary stenosis of the left lung. Although each of her CHD’s are classed as “critical” in their own right, ie life ending, this one is the one that makes or breaks things. This is the one that rules out her Fontan Completion, and turns everything on it’s head more than any other. But there are always options. A great man once said “I don’t believe in a no-win scenario.” Like Millie, he was a true warrior.
We’d put a DNR order in place after her first bounce back because she’d already stated to us that she was a fighter and so, if she went, it would be because she’d had enough and was telling us she wanted to call it a day. But it came with conditions; if she was deteriorating and it could be sorted out with a little tweak of her current infusions then go ahead and do it. If however she had a full blown crash then they were not to attempt resuscitation. We didn’t want someone banging away and shocking her etc when she’d already clearly made her mind up. We carried her DNR on until her first surgery in Birmingham where we lifted it simply for the reason that no self respecting surgical team in the world is going to go into open heart surgery with a DNR in place. The DNR is a very personal thing, but for us, seeing our little girl hooked up to all those machines keeping her alive, it was perhaps the one thing we felt we could do for her that perhaps gave her back some control over her own destiny, whether it be real or perceived.
There came that dreadful moment when we were taken to a private room off of PICU and as soon as that happens you know its bad news. For Mills, combined with her low birth weight it was the worst possible news. After a lot of explanation of her problems, normal procedures for corrective surgery, possible outcomes if surgery were pursued, it came down to the one simple fact that they didn’t feel there was anything they could realistically do for Mills and that they felt the kindest course of action for her would be to make her comfortable and offer palliative care. One of the phrases used by her consultant in that meeting was “Sometimes the kindest thing to do is the hardest.” It takes a moment for your brain to catch up when you’re told something like that and when it does it’s the worst possible feeling in the world. We became lost in the depths of despair and grief examining her options from every conceivable angle, and at one point we even instructed them to “withdraw treatment”. That was when we were reminded that we were entitled to a second opinion and we leapt on that out of sheer desperation.
Mills’ notes were couriered to Birmingham Children’s Hospital and apparently, off the record, their first response upon viewing her CT scan was “Oh.” My wife and I joked with gallows humour familiar to most “heart parents” as to whether that was an “Oh shit”, or an “Oh, we can do something with that.” Thankfully it was the latter.
BCH felt that they had a small window of opportunity and so Mills was blue lighted there from the centre of London in rush hour traffic by an incredible retrieval team and she had her first surgery the following evening and hasn’t looked back.
Another phrase that’s stuck with us through everything so far, and it’s been used independently by both London and Birmingham, is that “we’ve seen everything Millie has…we’ve just never seen it all in one baby.”
First surgery on 10th June was an atrial balloon septostomy and bi lateral pulmonary artery banding, which were done in an effort to stabilise her circulation and improve hemodynamics enough to give her chance to gain enough weight for her Norwood-type op. Up to the point where she had her Norwood+ (I call it a Norwood plus because of the amount carried out during the procedure), she remained on her Prostin, in all for a total of almost 2 months, that as despite understanding that they generally didn’t like to keep babies on it for much more than a week. It wasn’t until she hit 2.5kg that she started showing the first signs of destabilising; steadily lowering sats and bp, increased heart rate, visibly working harder to take breaths. Blood gases were being done and her lactate was showing elevation, meaning that she was starting to work harder. So the decision was made to proceed with her Norwood+.
From our point of view on her surgeries we just resigned ourselves to the fact that they would either work or they wouldn’t. That may sound a bit cold but it was how we dealt with it, our defense mechanism if you will. Of course we were terrified, and both times they were the longest hours of our lives to date, and that was despite sleeping through them. We didn’t have the money to go galavanting around Birmingham, and there wasn’t a great deal else we really could do, plus we were dog tired from the late nights and early mornings with Mills. I guess our bodies knew what we needed better than we did ourselves.
And the joy at receiving the phone call to say Mills had pulled through surgery and was now in recovery is beyond description.
One of the other ways I personally dealt with it was by, in a way, finding God. I’m not a born again Christian by any means, but we did a LOT of praying, and in the end I don’t know if there was any divine intervention, but if there was then my hat is off to “the Man”.
Mills still requires more surgery in the future but for now we’re just all enjoying finally having her home after literally bouncing back from death’s door.
In order to help others try and make some sense out of things when they receive a diagnosis as devastating as being told your child has a congenital heart defect, or series of them, I’ve started work on both an online blog and also a video diary on You Tube of her journey, and if they help even one parent to understand and keep strong for their child, then they will have served their purpose.
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