Saturday, June 3, 2017

|| the countdown to cath day ||

Evanna playing with her oxygen tubing

We're officially in countdown mode as we edge closer and closer to Evanna's upcoming cath, which means we're keeping her on isolation precautions here at home and tying up loose ends (hospital transfer details, booking time off work, Edmonton accommodation plans made, etc).

Nick and I have had more than our fair share of very stressful moments throughout our medical journey with Evanna. From the time we were told that her diagnosis was somewhat “worse case scenario” the day after she was born, to handing her over for her very first heart surgery…. then going into cardiac arrest 3 weeks later. The anxiety filled weeks and months we spent in the PCICU so desperately trying to extubate Evanna off the ventilator, and probably our worst to date – almost loosing her when she went into Septic Shockon Canada Day back in 2015.

While we’ve had several additional “curve balls” here and there since that admission, it kind of doesn’t phase us anymore due to our past experiences – and something I like to call “desensitization”. However, in a few weeks time, we are headed back up to Edmonton for our much anticipated Cardiac Cath – and we’re actually quite nervous, anxious and stressed about the whole ordeal. Despite the fact that we’re very aware of Evanna’s long term prognosis, it doesn’t make it any easier when we are forced to actually face certain facts and decisions about Evanna’s medical care. Her prognosis is something we try to keep at the very back of our minds when all is well -- and though it's always there, it's something we generally don't dwell on a day-to-day basis, and try to just live as normally as possible. So it absolutely sucks when those discussions are forced to the forefront, and have to be openly discussed with our medical team about the situation and essentially have "the talk" (most recently was back in October, when Evanna's health began to deteriorate when dealing with endocarditis while fighting off a cold at the same time).

While we have to wait for Dr. Hanley's official decision once we send down these cath results to him, due to the fact that we have an idea of what he's looking for (I've highlighted it in the letter we got from him back in November that I still carry with me everywhere), we'll be able to gage what his response will be based on the cath results and what the doctor tells us postop.

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... so here's exactly why our anxiety and worries about this cath are legit and very, very real (while trying my absolute best to explain everything in lamen terms):

- first and foremost, I must remind everyone that we do not have the backup option of a heart or heart/lung transplant. While I'm very aware it's not the perfect solution either, as it comes with it's own consequences (suppressed immune system, organ rejection, the way-too-long waiting list), it would provide me with just a little bit of comfort if we had this option to fall back on somewhere down the road should Evanna's heart start to fail her (which will happen eventually). This is solely due to the anatomical issues that "collateral dependent MAPCA's" brings -- in that there is no place to physically hookup a "normal heart" to Evanna's unique anatomy. The MAPCA's (collateral's that act in place of Evanna's missing pulmonary artery that takes blood to her lungs to become oxygenated) make her heart and lungs are extremely intertwined. And as previously described in this post, it's also been recently revealed that "collateral dependent patients" are not ideal candidates for a heart/lung transplant, and would actually live much longer with their own heart verses undergoing the double organ transplant that presents just too many postop complications that results in death.

** IMPROVING the state of Evanna's heart is the only option we have left -- and Dr. Hanley at LPCH is kind of the only guy for the job given his particular background and skill with MAPCA's.

- therein exists a unofficial "scale of severity" when it comes to MAPCA's.... allow me to try and explain: there are children who have MAPCA', but also have what's called a "native pulmonary artery"... which either means they don't have to depend on their collaterals for pulmonary blood flow -- even to a point where the collaterals are not needed at all and can even be completely "coiled off" in some cases. Evanna does not have a native pulmonary artery and is what's called "collateral dependent". Within this subcategory, is a "scale" that is based off the size of said collaterals and the severity of them. Some children have "nice big" collaterals that grow well with the odd ballooning throughout their life, and their prognosis is considered good. Evanna is on the severe end -- with small collaterals, that have tons of stenosing (narrowing) throughout. This is not so good.....
Evanna was born with 5 collaterals -- 3 that went to her right lung and 2 that went to her left. Her last cath that was done in May 2015 showed that while her collaterals on the right side were "okay", we were somewhat loosing the 2 on the left. We did a CT back at the beginning of 2016, and while it doesn't give us clear results like a cath does, they essentially couldn't see any pulmonary blood flow through those left collaterals whatsoever. Our hope is that the stenosing of those collaterals stops as it goes into lung tissue, so that Dr. Hanley can go into lung tissue (what he's kind of "known for"), and kind of work backwards rehabilitating those collaterals (make sense...?).

- despite the fact that our primary cardiologist (Dr. Fruitman) and her colleague ("Dr. R") did indeed facilitate our request and sent the referral for a second opinion down to Dr. Hanley on Evanna (as it was the "appropriate next step") back in October, it was done with a lot of caution and discretion from our cardiology team. It's been reiterated by both our primary cardiologist and Dr. R that Evanna's case is extremely severe...even for Dr. Hanley, and they have asked that Nick and I prepare ourselves for the real reality that her case is even beyond his capabilities and he will come back saying there is nothing more he can do. The colleague that helped facilitate our request (Dr. R) happened to be the same doctor who has performed previous caths (2 of them in fact) on Evanna (so she is well aware and has an excellent understanding of Evanna's anatomy), and was also behind a previous referral of another local "MAPCA baby" down to Dr. Hanley that was successful. However, Dr. R has been quite frank with us about our situation, and has expressed her doubts in Dr. Hanley accepting our case due to severity. So yes – we have very legitimate reasons to be stressed and anxious about this cath and it's results. However, that being said, if there is ANYONE who can help us now – it will certainly be Dr. Hanley and his team at LPCH (to which Dr. Fruitman and Dr. R agree).

- the risks of the cath procedure itself. As I mentioned in my post last week, normally caths are done through the femoral arteries, but unfortunately, Evanna's have clotted/occluded (just something that happens sometimes when you've had this procedure done several times in the past and/or multiple IV lines that the body creates clots around -- as it's a foreign object) - and so they have to go through her liver. A normal/routine cath is already considered quite the risky procedure - but now we're adding potential injury/bruising to her liver and increasing Evanna's risk of bleeding. Of course with anything, we weigh out the risks and benefits with every procedure, and know this one is necessary for several reasons. We know Evanna is in very good hands up at the Stollery, and the PCICU will be somewhat put on "standby" should we require their assistance (though, the Stollery medical team may have already have planned to place Evanna in the PCICU after the procedure anyways due to her history).

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.......... if you made it all the way to here and somewhat understood everything that I said -- than bravo! I know on the outside, things look fine, but I wanted to try and convey to the best of my ability that things are not always as they appear -- and the littlest setback could cause things to go in the other direction very quickly. Yes, at the moment, Evanna is healthier than she's ever been, but there will be day that the "other shoe drops", and her body may not be able handle it. While we've been absolutely blessed with how much Evanna has THRIVED over the past year despite all the cards stacked against her, that does not mean that we're na├»ve to our circumstances either. We are doing absolutely everything we can as parents to advocate for Evanna, and doing all that we can to "leave no stone unturned", and though you "never want to put all your eggs into one basket".... we unfortunately have no other baskets hold, and so there is a LOT riding on this cath, and the results that will be sent down to Dr. Hanley. We've been told he rarely says no..... and though the man likes a challenge -- we know he can't create miracles out of thin air and needs something to work with in order for him to say yes.

I would like to add one final thought and explain our overall intentions with this referral: even if we get the results we're hoping for AND Dr. Hanley accepts our case, I need to be clear about a few things. At the end of the day, Evanna will still be considered terminal --

Our goal is for Dr. Hanley to improve her current prognosis and quality of life.

Despite his reputation, we're not going down with a false sense of hope that he will perform this almighty miracle surgery, and all of a sudden all our problems go away, and Evanna will be given a normal/long life expectancy. Even if he can help decrease how much oxygen she's on will be considered a win in our books and we will just be happy with whatever he can do.

So please keep Evanna (and perhaps myself and Nick) in your thoughts and prayers over the next few weeks. I know people like specific things to pray for, so here's a list:
- for a smooth, uncomplicated and safe cardiac cath -- followed by a quick recovery
- that the results will be in our favor and appease Dr. Hanley and his team
- for Nick and I to feel some peace and reassurance during this time regardless of the results and remain strong and united as a family. 


xo The Irvine Family

3 comments:

  1. I love you guys and am rooting for you ❤️

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  2. Wrapping your family in prayers.

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  3. Praying for you all!! I pray tomorrow goes well. ❤️

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