Monday, June 12, 2017

|| sweet Avelyn + respiratory update ||


Before I get into our update on Evanna, I want to take a moment and talk about my dear friend Somer, her husband Sean and their daughter Avelyn (if you follow our family's Facebook page, then you'll know who I'm referring too). Along this journey, I have met many other families in somewhat similar circumstances as us.... whether it be they are a "heart family", "trach family" (sometimes a combination of both), or another "medical family" (ie. SMA, Immune Compromised, "Short Gut", Pediatric Cancer.... this list goes on and on) -- that causes them to go through that same medical roller-coaster of long/multiple hospital admissions, surgery after surgery, endless procedures, tests and the overall emotional, exhausting, financial and physical toll it puts on families like ours who have a very "sick child". There are handful of mom's that I have become really close too over the last few years, as we bond, rant, cry, cheer, and bounce ideas off one another on a consistent basis -- because at the end of they day, they just get it, and physical distance doesn't impact the relationship that's been built whatsoever.
Somer is one of the few mom's that I count within my "inner medical mama circle", because of how closely our journey's have mirrored  one another. Of course Evanna and Avelyn had their differences (ie. Evanna's airway vs Avelyn's kidney failure), but there aren't many others who can really understand, sympathize and comprehend the really long hospital admission(s), curve ball after curve ball, fragility of our child, the nonstop medical appointments, endless therapy sessions, and the constant care they require (that tends to include home care). Somer has been one of our families biggest cheerleaders and closest confidants over the past year or so, and my heart is just broken for her and her husband Sean right now (who I feel is very similar to my own husband in the high caliber they are as husbands and fathers... which unfortunately is somewhat still considered a rarity). Though they got to take their sweet girl home after some 450 days in hospital a few months ago, their daughter Avelyn "Avie" became very sick again (with Septic Shock no less), and passed away a few days ago. She had been through just so much in her short little life (most of it has been documented here [last updated in December 2016] or Somer's IG here if you are interested in reading), and I've somewhat been at a loss of words at what has transpired over the past week or so, and my heart is absolutely shattered at their devastating loss right now. Though we've been busy getting ready for Evanna's upcoming Cardiac Cath on Wednesday (and the major stress and anxiety that we have in regards to the the potential "bad news" we may receive) , I've definitely been in a place of private mourning and can't help but ugly cry real hard when I have a moment to pause and think about Somer and Sean and all they've been through over the past year and a half and this past week in particular. On the other side, there's also a been a huge mix of "survivor's guilt" and "when will the shoe drop for us?" too – as it just comes with the territory of having a extremely medically complex child yourself who's future is still quite unknown.With all that being said – I feel a responsibility to share their story and crowd-funding information that is being used to help cover the mounting medical bills that have accumulated and now added funeral costs on this platform of mine. So if you would like to visit their YouCaring page, please (please) click here for more information ---->

Again, with our deepest sympathies  all our continued love and prayers to Somer and Sean + their extended families during this time. xxo


Last Wednesday, Nick and our homecare nurse Rebecca took Evanna to her airway (CAC) appointment. After a month of sprinting Evanna off the ventilator twice a day for an hour, we wanted to see if she's built up her stamina and could handle even longer sprints. Well, we've been officially approved to increase her morning sprint from 1 hour to 2 hours, and later this week can begin to work her afternoon sprint from 1 hour to 2 hours also (so very good progress!). They also tried out a speaking valve in clinic, but it looks like we have our work cutout for us. Just like we were warned (well from what I read in the global trach mama Facebook group I'm in) it definitely takes a LOT of getting use too, and at the moment Evanna can only handle about 15-20 minutes before her "work of breathing" starts to get labored.... so we'll have to take it slow and steady. Because she doesn't have it on for very long - she hasn't really really experienced the difference it'll make to the amount of noise she'll be able to make with it - but I'm sure that will just come with time.
At this time, we haven't scheduled our next CAC appointment yet (it's usually done at the end of the appointment)  as it was Nick who took her to the appointment and leaves all the appointment scheduling to me. It's quite the work of art in my opinion to keep our life organized with both of our work schedules that vary week to week, staying on top of our homecare staffs schedules (that again can vary week to week), all the therapy and medical appointments Evanna has, and our busy social life -- so in order to avoid any potential mix-ups/double booking  Nick just leaves all of it to me (and in return, I leave the homecare payroll and banking to him). I hope to call our hospital trach nurse on Wednesday while Evanna is having her Cath done and book our next appointment for sometime in July. However, apparently there's a bit of debate on what to work on at our next appointment and whether we increase sprints or wean ventilator settings. The Respirologist (Dr. D) that Nick saw with Evanna on Wednesday (there are three "trach doctors" that we rotate through depending on "who's on") and Nick want to decrease the PEEP from 8 to 6, while I would rather see continued increases of sprinting off the vent. There isn't exactly a wrong answer, and while the "end goal" is the same (ie. decannulation), it's just logistics on how we get there. I posed the question to global trach mama group I'm in, and for the most part, many agree with me that we should continue to focus on increasing the length of time off the ventilator, but I also plan on asking our hospital trach nurse to consult with the other trach doctor (Dr. M) whom we've been primarily dealing with over the past several months for his opinion on the subject, as he was the one who actually performed the bronchoscopy that Evanna had back in April.


Ohhh boy. So things are still currently "a go" for Wednesday's cardiac cath.... but the logistics of it all has definitely been both interesting and slightly exhausting to say the least (and there's been a lot of "telephone tag" between myself, our cardiologist Dr. Fruitman and the Stollery since Thursday). It was originally planned to transport Evanna up to Edmonton via the ICU transport team (which would have required an admission down here), but with how much respiratory improvements/gains Evanna has made over the past few months, it kind of made that plan slightly ridiculous and somewhat "overkill" (definitely a good problem to have lol). However, with how much oxygen Evanna requires (4L), going up as a "true outpatient" and wait at a hotel with her still isn't feasible at the moment (though, I may have figured out a work around for the next time we have to go up to Edmonton as an outpatient), so a final alternative plan was officially made this morning that satisfies all parties involved. Basically we will call Admitting at the Stollery tomorrow morning at 10am to ensure that we still have a bed on the "ICE unit" (intermediate care environment), and will drive Evanna up to Edmonton ourselves (it's a 3.5 hr drive fyi) once we have confirmation of everything. We will head directly to the Stollery (my hope is we'll arrive sometime between 1-2pm) and will have the ECHO and blood work/cross matching for the procedure done as an inpatient instead once we're admitted on the unit. To my knowledge, Evanna's cardiac cath will take place first thing Wednesday morning, and will most likely be kept overnight for monitoring, as the cath she's having carries more risks due to the fact that it's being done through her liver vs the femoral veins (they're occluded as far as we know). 

Immediate updates over the next 48-72 hrs will be made via our Facebook page and my Instagram account, but will do a full update sometime later this week/early next week with our cath results and what it'll mean for our California referral. 
Thanks for all the love, prayers and continued support  – it's greatly appreciated and means so much to us. 

ox The Irvine Family

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.


... 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).


.......... 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