Wednesday, July 12, 2017

|| cardiac cath + first half of summer ||

....I don't know why, but I had a lot of trouble finding the time and motivation to formally write/summarize our trip up to Edmonton for Evanna's cath that happened back in the middle of June and just little surprised its taken me almost a month to get it up here on the blog. While I still kept everyone updated through my Instagram and our Facebook page, I guess due to the fact that the whole situation/referral is still very up in the air at the moment (ie. we are still waiting for an answer... the referral status is discussed further below), the lack of additional information made it feel pointless and a bit mundane to blog about it. However, documenting this journey remains a huge priority for me, so here goes ---

Despite how much planning went our Cardiac Cath procedure, I still feel like that week was a bit of a whirlwind/roller-coaster, but I'm just so happy to have this step behind us now. Not everything went exactly as planned (though, I'm very happy with how it all played out), and due to contracting a little GI bug from the hospital.....recovery took a little longer than expected. I feel like we're still trying to process all the information we got from Evanna's cath, and at the same time disappointed that things still remain a huge question mark until Dr. Hanley reviews the cardiac cath and sends us his official opinion. Though we knew what he was looking for, things weren't exactly as black + white as I hoped they would have been, and both our medical team in Edmonton and Calgary are very interested and eager to see what Dr. Hanley says. While things don't look too great (especially on Evanna's left side), Dr. Hanley is known for saying yes when everyone else says no, and we're somewhat in this weird grey area and basically left wondering how this is all going to play out. The cath doctor (Dr. S) stated that based on what he saw, "most cardiovascular surgeons in the world wouldn't touch her", and asked that he be kept in the loop in regards to what Dr. Hanley's says, as it would be very intriguing if he can do something based on what he saw during the Cath.



[INSTAGRAM UPDATES]

June 13, 2017

||| stollery, day 1 ||| we got confirmation at 10 this morning that we indeed had a bed, and were on the road towards Edmonton just 30 minutes later. The drive went really well, and Evanna only got a little antsy/grouchy towards the end (normal toddler behaviour in my opinion lol). We are now admitted and settled on the ICE unit ("intermediate care environment"), as her trach requires higher observation that the regular rooms can't provide. 

We met with the doctor this afternoon who will be performing the cardiac cath tomorrow (Dr. S), and confirmed that they will indeed be going through her liver and the overall goals of this procedure (imaging for Dr. Hanley, check lung pressures and do ballooning of any collaterals if they can). He also made a comment that he has heard a LOT about Miss Evanna (he's somewhat "newer" to the Stollery, and so this is our first time meeting him), and apparently is well aware of her reputation.... 😳My only disappointment is that we're currently slated for 3rd case tomorrow, which means there is a small possibility we could be bumped (however, our nurse reassured me they've taken cases as late as 5pm), and she'll have to be NPO (no food) during the day vs while sleeping. 

PCICU has been made aware of our admission (actually ran into a favourite intensivist of ours while grabbing a grilled cheese sandwich -- my favourite thing to eat here), but have high hopes that she won't need to go to the ICU after and can just return to the ICE room postop — where we'll stay overnight for monitoring (followed by blood work the next morning to check some liver numbers). 

June 14, 2017

||| stollery, day 2 ||| well, at 4pm we were informed that Evanna's cath was indeed being cancelled/postponed, as the first 2 cases took too long. We're completely bummed.... but knew this was also a possibility, and are just waiting to hear what the new plan will be.


This is by far the healthiest and strongest Evanna has ever been while inpatient at the Stollery, which means we got to be a little adventurous and try some new things like ride a tricycle this morning while trying to pass the time (stuff that I could only dream of just 2 years ago). It's been a super long day of waiting, and despite being NPO (no food or fluids) since 7am, Evanna was such a champ about it. Seriously, this girl has been through so much in her little life, yet remains so happy, calm and content with everything, and I couldn't be more proud to call her mine. 

UPDATE: we're now scheduled to be FIRST case tomorrow πŸŽ‰πŸŽ‰ (which if I'm going to be honest, would prefer over being last case today)

June 15, 2017
||| stollery, day 3 ||| well, the moment we've been waiting for since October/November is FINALLY here! We know Evanna is in great hands (with the same AWESOME anesthesiologist she had back in December for that tough PICC line insertion), but remain anxious and nervous about what kind of results we're going to receive after. Staff were taken back by her easygoing nature while transferring her over to the procedure bed and laughed when she fought the sleeping gas and sat up for as long as she could to watch Tangled on the tablet (which obviously didn't last long).


||| stollery, day 3 part 2 ||| Evanna was in the Cath Lab for roughly 5 hours – and considering this was just a "diagnostic cath" (ie. no ballooning or stents placed), it was definitely a tad on the longer side, as they encountered some trouble trying to access the left side. Unfortunately Evanna's history and extremely high tolerance of sedation medication caused a few minor issues towards the end, which meant we had to make a little pit stop to the PCICU for about 5 hours before we could go back upstairs to our room. While Evanna handles anesthetic beautifully – she metabolises through it insanely quick, and so they had to throw a LOT at her, including a paralytic towards the end, which meant it was safer to recover under the close eye of the ICU.


So cath results - overall, there weren't any major changes and everything essentially remains the same as her last cath from 2 years ago. Good news is her lung pressures remain favourable, which means there are no contradictions for a potential future surgery. However, there is still a huge question mark in whether there is anything to rehabilitate in regards to her MAPCA's/collaterals, and Dr. S remarked that most cardiac surgeons in the world wouldn't touch her. He's aware of Dr. Hanley and his reputation, and basically reiterated what our cardiologists have said -- that if anyone can help Evanna, it'll be him. So now it's just another waiting game of sending down these cath results to California and seeing what he says. 

The plan is to rest easy tonight (we just got back up to ICE), do some blood work in the morning, go over the cath results again with Dr. S in the morning, discharge, and drive back to Calgary. Thanks for all the love + prayers!

June 16, 2017

||| stollery, day 4 ||| and we're DISCHARGED πŸŽ‰πŸŽ‰ After an xray to check the "plugs" they inserted into her liver after the cath, some blood work, and a final chat with Dr. S, – we were told we could take our little girl home with instructions to follow up with a cardiologist next week.

For the most part Evanna is doing well, and we're just dealing with some post-anesthesic nausea right now.

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So why is everything still so unknown? Well, from how it was explained to us (and will ensure that Nick has everything clarified at Evanna's cardiology appointment on the 20th), though it was straight forward that Evanna's right side is in need of some ballooning and possible stenting... it's the left side that remains the big question mark (which we kind of already knew...and the sole purpose of this referral). Evanna essentially has no pulmonary blood flow to her left side and relies completely on her right lung to oxygenate her blood. The left side does receive what's called "back flow" and is fed blood through little collaterals that run along her ribcage -- which is basically what keeps the left lung tissue alive and well.... but it just means the left lung doesn't really do what a lung is suppose to do. Dr. S (the cath doctor) believes there may be some pulmonary fed collaterals just past her hilum (the point at which the bronchi, pulmonary arteries and veins enter the lung), which is what everything will comes down to. Most cardiothoracic surgeons won't go into/touch lung tissue, but it's kind of what Dr. Hanley is KNOWN for (particularly around the hilum). So it's seriously anyone's guess as to what Dr. Hanley thinks he can do (IF anything at all!) once he reviews these cath images.


[REFERRAL STATUS]

This is kind of where things are getting a little frustrating (again), and the somewhat small consequence of our universal healthcare system when things are stable and the situation isn't necessarily considered urgent. For some reason (that was blamed on technology issues), our cardiologist/cardiology clinic didn't even receive the cath package from the Stollery/Edmonton until just last Wednesday (July 5th)....... and to my knowledge, it was hopefully forwarded to Dr. Hanley within the last few days.
So though I was hoping we'd have an answer by the time we saw our Cardiologist on July 20th, we definitely won't have an answer until at least the very end of July, if not the beginning of August. Trust me, if that news frustrates you, you can just imagine how Nick and I currently feel.

I'm trying my best to remind myself that good things come to those who wait and that everything will work out the way it's suppose to. Evanna is continuing to do well, and I would much rather deal with all this waiting because she's doing so well, then have the opposite problem right now. We feel beyond blessed and so thankful that we do have time on our side, and are focusing on that fact instead. Between my pregnancy and weaning Evanna off the ventilator (which continues to go well), we remain quite busy and distracted, and so we aren't really dwelling on this delay... except calling the cardiology clinic every week to ask for an update lol (always a tough balance of not being "that annoying mom" and properly advocating for things to happen in a timely manner). 

[JUNE + JULY HAPPENINGS]




Not only have we been kept distracted by my pregnancy and weaning Evanna off the ventilator (which currently involves monthly appointments to the Respirology clinic), our social calendar has somewhat remained quite full too! The weekend before Evanna's cath, Nick and I actually went away for two nights with our friends to a cabin in Golden, BC, and we left Evanna in the care of our homecare nursing team. We enjoyed a nice relaxing weekend surrounded by the beautiful rocky mountains, and definitely hope to make it an annual trip (and crossing our fingers that Evanna will be able to come with us next year). The weekend following the cath, Nick and I attended a wedding at the Calgary Zoo (so BEAUTIFUL btw) of his old EMS partner, and had lots of fun with tons of Nick's colleges (it was a very EMS filled wedding due to the fact the bride is an EMT and the groom is a Paramedic that both work on the same shift), and we agreed it was hands down one of the best weddings we've ever been too.


Just after Father's Day, we unexpectedly got a phone called from our ENT's office, and were asked if we wouldn't mind doing our yearly "combined clinic" (an appointment with both our ENT and one of the trach respirologist) at the end of June vs September. Apparently they had a ton of cancellations, and were trying to fill some spots (why are some specialists easier to see than others?!). Well, the appointment was night and day compared to last years, and the mood was definitely much happier to say the least. Last September, we were at this huge plateau in regards to Evanna's respiratory status, and I was having a really hard time with the fact that we had made absolutely no improvement nor gains towards weaning Evanna's very high ventilator settings or sprinting since bringing her home. I was met with "well it is what it is" type attitude from the ENT, and told we just had to be more patient. Yeah.... was definitely not happy with that answer and even got a little emotional during the appointment.
This time, it was a much more positive visit with the ENT, who was extremely happy to see that her bronchoscopy results were showing her tracheomalacia is essentially gone, and we're making awesome progress with sprinting off the ventilator. I didn't really have anything to discuss, as we're on a pretty steady path with weans and sprints, and there's not much more to talk about (ie. decannulation) until Evanna is completely weaned off the ventilator. Overall, the whole team was happy to see the major improvements Evanna has made since last Fall, and will continue to keep tabs on her (especially if we end up going to California).


We enjoyed a busy, but fun Canada Day long weekend with the additional bonus that Nick was home for almost all of it. We first attended an event at the Foothills Stake Centre, as they put on a huge annual Canada Day event every year (apparently the mayor attended last year), and were able to catch up with quite a few old acquaintances. After that (and a tiny cat nap for this preggo), we headed out to Okotoks and had a picnic at the splash park. Evanna wasn't really a fan of the splash pad part, but that's not going to stop me from trying to continue to introduce Evanna to new experiences. We were able to do both of these activities (event and splash pad) with Evanna sprinting and left the ventilator in the back of our car -- something I can't emphasize enough just how liberating that was. The evening was concluded with a quick BBQ at my parents house and one tired little girl.
We entertained Nick's family at our house the following day, and ended the weekend with a family photo session with my friend Alissa of Lissables Photography.

Nick's birthday was this past weekend, but due to insisting that we keep everything extremely low key, just had one of the homecare nurses come over early to watch Evanna so we could go for a simple and quick dinner (next year is his 30th....which means that same request will most likely be disregarded).


Next week we have our detailed ultrasound where we hopefully find out baby #2's gender, which means we can finally start discussing names (Nick refuses to discuss baby names until then, as why have two arguments, when it just requires one? lol) and have invited some friends and family over later that night so we can reveal the news together.

xo The Irvine Family

PS. if you haven't already listened to it, I did a 15 minute podcast interview with the Orange Socks Organization that was released the day we publicly announced we were pregnant with baby #2! I briefly talk about when we received the news of Evanna's diagnosis, the impact it's had on our lives, how she's been doing, and what the future currently holds for our family. It basically sums up our journey thus far and shows that despite the last few years being incredibly hard and full of trials, things are looking up and we actually have a lot to be thankful for!


Link to the interview HERE (it's about 15 minutes long).


Wednesday, July 5, 2017

|| due with number 2 ||



Nick and I are excited to officially announce that come this December, we are expecting baby #2 and we're just completely over the moon with this news!

A few months ago, I wrote how "April is off to a fantastic start" (you can read that blogpost here), and although I was indeed mostly referring to our awesome bronchoscopy results and  finally being able to move forward with weaning Evanna off the ventilator (!!!)....I was somewhat secretly referring to the fact that Nick and I had also found out that I was pregnant and due at the end of the year and things were finally looking on the "up and up" for our little family (well... it's about time!).

I've been very open to the fact that Nick and I want more kids, and how we've been a little surprised that it's taken longer than expected for it to actually happen (I seriously thought we'd be working towards baby #3 by now.... but obviously this type of thing is somewhat outside my control). We took last summer off from trying, and gave my body a break after my miscarriage in July -- but were a little taken back when it didn't happen right away like it did the first two times. In February I got some blood work done to check my hormone levels, and despite slightly lower progesterone levels (that my GP said was most likely due to stress... which I completely blame on our very chaotic hospital admissions this past Fall) my doctor stated that he was confident I would get pregnant by the end of the year given our history. Thankfully he was right, and once our life resumed to "our normal" and my stress level came down a few notches, we finally got that positive pregnancy test we had been waiting months for.

I'm already 17wks pregnant (and will actually be 18wks tomorrow), and can't believe we're almost at the halfway mark! While it's been no secret to anyone who's physically around us such as our friends, family, and my coworkers -- as hiding this bump would be completely impossible (I actually started showing at 9/10wks), finding the time to publicly announce the news kind of fell to the bottom of our priority list with our trip up to Edmonton for Evanna's cath and so much of our focus on our California referral (update coming soon - I promise!) and weaning Evanna off the ventilator. Then I found out my dear friend Alissa who's an absolutely amazing photographer (and have been wanting to do a photography session with her for years) was coming up to Canada for the summer (both her and her husbands family live up here), and so I decided to hold off another few weeks til she got here to do the photoshoot with her.

As for documenting this pregnancy, I'm curious to see what happens. Evanna has been the "star of the show" for quite sometime, and as everyone knows, subsequent children are documented slightly less than the oldest. My personal goal is to include "bump updates" at least once a month, and do a good summary of each trimester (already working on one for the first!). For example, I want to document what it's been like breaking the news to our medical teams in both Calgary and Edmonton, as the response has been incredibly encouraging and extremely heartwarming to say the least. We have built some wonderful relationships with many of the specialists + intensivists involved with Evanna's care, and I think because they know and truly understand everything we've been through -- there have been a number of doctors (like our cardiologist!) who have been extra excited about my pregnancy.
On the flip side, there has also been some anxiety and fears with this pregnancy (can you blame me?) -- and while some of my fears have been put at ease by our genetics team and the positive nuchal ultrasound we had several weeks ago, I'm definitely nervous for our detailed ultrasound that's booked in exactly two weeks time!


---------------------------------------------

A few months ago I was approached by the Orange Socks Organization, and asked if I would be interested in a doing a little podcast interview talking about the day we received the news of Evanna's diagnosis, the impact it's had on our lives, how she's doing now, and what the future holds for our family. In anticipation of this pregnancy announcement, I thought it would be a great opportunity to pair the two together -- as it would help sum up our journey thus far and show that despite the last few years being incredibly hard and full of trials, things are looking up and we actually have a lot to be thankful for.

Link to the interview HERE (it's about 15 minutes long).

xo The Irvine Family


Photography by Lissables Photography || Located in Las Vegas, Nevada
Dress c/o Pink Blush Maternity || Dress Style - here



Monday, June 12, 2017

|| sweet Avelyn + respiratory update ||

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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 ----> https://www.youcaring.com/somer-sean-and-avie-stahlman-pickel-531248

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


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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.

[CATH UPDATE]

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.

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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 naΓ―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

Sunday, May 28, 2017

|| strawberry shortcake + SPRINTING! ||


Man oh man, I was doing so good in April! I got a number of posts up and was on top of my updates… but for some reason I’ve been having a hard time over the past few weeks trying to find the motivation (and time) to sit down and get stuff written up (even my Instagram has been severely neglected). Believe it or not, blogging is a lot harder than it looks, and there’s a huge amount of time that goes into every post – such as the actual writing, grammar checks (which is actually not my forte, but I actually feel like this “hobby” has somewhat helped me improve in this particular area over time), photo editing, and post formatting. The end result always makes it so worth it, as I now have this beautiful journal/scrapbook documenting our life, and I love going back and reading through old posts of mine all the time!
I definitely need to take the time and get it printed into hardcopy, and I would love to have the motivation to blog about other things I’m actually passionate about (lifestyle, little bit of fashion, motherhood, thoughts on having a medically fragile child, etc)…. but then my horrible time management issues create this barrier – and something I need to really get better at (… I’m serious – I waste so much of my day just “doing nothing”, and something Nick and I discuss often because of how bad it is).

At the end of April we held a little birthday party for Evanna (already posted a few photos here), and just invited family and a few close friends. I didn’t manage to send out the “official Facebook invite” until just two weeks prior (…cause of said time management skills), but most of our family knew about it way ahead of time, and almost everyone was able to show up. I wanted to play off Evanna’s gorgeous red hair that everyone always obsesses over (myself included), and decided to do a “Strawberry Shortcake” themed birthday. It was a super long day for Evanna, but she handled herself really well and feel like she somewhat enjoyed herself.

We finally put an ad up online for our basement suite the same weekend as Evanna’s birthday party, and ended up receiving an awesome response and showed it to several prospects during the first few weeks of May. The rental market is a tad saturated right now (currently a “renters market” here in Calgary)… but we priced it really well and garnered the right attention. After selecting a tenant, we had her sign the lease and pay the damage deposit early last week, and handed over the keys to allow her to slowly move her things over. We’re so excited to have this huge project behind us and are now tackling our yard (well.... Nick is). We were one of the last people to move onto our street last summer, but our first priority was developing our legal basement suite and getting it rented out. This meant that our yard had been severely neglected, and was completely overgrown with weeds…. and we had somewhat become one of “those neighbors” to say the least. It wasn’t a huge deal during the winter when the snow covered everything, but now that it’s all melted, I somewhat cringed at the state of our yard. Thankfully that all changed this past week!

We have made tentative plans to finish the fence (our neighbor built the one side last summer) in August… but will wait a few more months before we officially decide what we’re going to do, and have talked about maybe holding off just one more year before we complete this particular project -- as Nick pointed out that we really don't have animals or kids we're trying to "keep in", so it's not a super vital project we need to complete this year.



[EVANNA]  

We’re currently averaging a medical appointment about once (maybe twice) a month these days – and after a year of hitting this major “plateau” with her health, we’re finally making some really nice gains this Spring. At the beginning of April we had huge bronchoscopy that gave us the news we’ve been desperately waiting for, and began making significant weans OFF the ventilator. We went back for a clinic visit on May 10th for “more weaning”, and were a tad surprised when the Respirolgist gave us the approval to take Evanna completely OFF the ventilator! While I knew it was a slight possibility from the conversation we had with him when we were discharged in April, I wasn’t about to get my hopes up, and would have been happy to just wean ventilator pressures – so I was definitely happier with this decision. Here’s what I wrote in my social media update on that day –



[INSTRAGRAM UPDATE]


May 10, 2017
Add caption


WE'RE SPRINTING πŸŽ‰πŸŽ‰πŸŽ‰ || we had an airway appointment today, and we trialed Evanna OFF of the ventilator!!! I repeat -- Evanna is now SPRINTING OFF THE VENTILATOR!!! Pictured is Evanna with just an HME (which provides humidity) and oxygen.

The Respirologist felt she could handle it based off her bronchoscopy that he did back in April, and so we gave it a go (and our trach clinic RT and nurse were just as excited and surprised as we were when given the orders lol). Evanna did extremely well for about 1.5 hrs, and all her "numbers" remained within normal limits (CO2, Heart Rate, Respiratory Rate) – and in fact, her oxygen saturations consistently stayed in the high 80s and low 90s (she normally sits between 75-85 due to her heart defect). However, as we reached the 2 hr mark, Evanna's "work of breathing" was noticeably worsening and was sounding more "wheezy".... which mean some of her malacia showing and parts of her lungs were beginning to collapse, so we called it and put her back on the vent.

So, based on today, we've been given permission to sprint Evanna for 1 hour, TWICE a day! We will then reassess at our next appointment​ on June 7th and go from there (ie. potentially increase the amount of time Evanna can be off the ventilator). Considering where we were just over a MONTH ago (PEEP of 12) – this is a huge win for us, and I'm extremely excited for a #ventfreesummer.
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Since then, we have been successful at sprinting Evanna twice a day for an hour each time – and it seems to be going real well. Due to the fact it’s obviously more work, she is napping a bit more consistently (but still have days where Evanna is a “no-napping girl”), or goes to bed earlier and falls asleep even quicker (not that this was an issue before). The true test will be if there is any marked improvement at our next appointment on June 7th and we’re approved for longer sprints. We have noticed that Evanna doesn’t like being on her back when sprinting, so she’s usually standing or sitting up – and we even use the opportunity to put her in her walker and make her walk across the house (using the tablet/movies as good ol’ bait!). Speaking of her tablet – I have to make note of this cute little thing she did a few weeks ago. Evanna has a very small selection of movies that she likes and it’s almost impossible to introduce a new movie to her (girl knows what she likes!). She was standing up against the couch, and I was sitting on the floor beside her. Nick recently added Ice Age to her movie playlist and I decided to see if she was willing to watch it. Within a minute of pressing play, she literally grabbed my arm and moved it towards the tablet in an effort to get me to change the movie to something else. Speech is still something we have made little progress on (though, the team is getting a speaking valve to try at our June 7th appointment) – but this was a new form of communication we’ve never seen come from her, and thought it was kind of neat.


We’ve also been given a Cath date (finally…..) for June 14th (at one point it was originally scheduled for June 12th… but were bumped just a few days later). For those who don’t recall – this Cath is for our California Referral, as it was requested by Dr. Hanley to assess if there is anything he can do for Evanna. We are beyond nervous for what results will be produced by this procedure, but I’ll go into further details as to why (Evanna’s case is considered extremely severe on the “MAPCA scale”, and so we have legitimate fears that the Cath will show that her case is just too complicated – even for Dr. Hanley and she’ll be deemed “inoperable”) in a completely different post closer to the Cath. I’m currently trying to concentrate on the positives of this Cath – which of course is the fact that we get to go up to the Stollery and have a chance to see some of our favorite people and “show Evanna off” to the medical team up there lol. Despite all the progress we’ve made from a respiratory standpoint, it’s been decided that it would still be best to medically transport Evanna to Edmonton, as it would be a bit too much to go up as “out-patient” (which is what's normally done), and sit in a hotel with no access to oxygen and such (Evanna requires a LOT of oxygen…. and so we would have to luge our concentrator and tons of oxygen tanks with us up to Edmonton… yeah no bueno). Details are a tad fuzzy, but we’ll most likely get transferred up to the ICE unit on 4C on June 13th, in preparation for the Cath on the 14th.
* I would also like to point out that Cath’s are extra risky for Evanna (and the reason it’s been over two years since our last one… and waited for Dr. Hanley to specifically ask for it). Cardiac Caths are generally done through the femoral arteries. However, Evanna’s are both occluded… so they actually have to go through her liver (which carries much higher risks of this already risky procedure). I’m sure they’ll do an ultrasound to double-check they’re still occluded… but it’s more than likely they’ll have to go through her liver again. Just another thing that makes Evanna extra complicated and special….oi.

The week before her Cath, Nick and I are actually going away with a group of friends to a cabin just a few hours away from Calgary for TWO WHOLE NIGHTS! Everything has been arranged with our homecare nurses to stay here with Evanna, and our primary medical team is thrilled that Nick and I are making the effort to go away for some good ol’ respite. I’m also trying to work on another mini getaway with Nick at the end of summer (and pushing for three nights…. but will be happy if we do two again)… but as usual – Nick is making it difficult, as he doesn’t like going away (he loves spending all his free moments just here at home with Evanna), and spending money (he’s the definition of “cheap”). But it’s the only other mini vacation I want to do this year, so I’ll most likely get my way in the end :)

I promise June will be very post heavy with everything we’ve got going on…. so stay tuned!


xo The Irvine Family

Sunday, April 30, 2017

Evanna is [T H R E E]



I’m generally good at keeping my emotions in check and remain level headed.... but then something will trigger and I become this big blubbering mess unable to stop the tears from flowing. Sometimes it tears of happiness… but other times it tears of sadness too. Despite the small level of fear Nick and I live with in regards to Evanna and her health, I'm happy to say that -- 


Evanna – you are officially T H R E E years old!


Another absolutely amazing milestone… and I’m almost having a hard time wrapping my head around it! You have such a personality right now, and it I could sum it up in just a few words -- they would be: calm… yet feisty, content… yet strong-willed, and easygoing… yet determined. It’s crazy that you can be one thing and then another, but allow me to explain:

CALM – Despite hospital admissions becoming shorter and further in-between (such an amazing accomplishment in its own accord), we continue to get tons of compliments on your overall “calm demeanor” and medical staff are always so surprised at well-behaved you remain while getting accessed or going through a procedure. Blood draws are starting to get a bit harder as you get older, but you still take it like a champ!

FEISTY – let’s just say that you would not be here today if it weren’t for this quality. You are one fierce little fighter, who continues to knock one curve ball after the next out of the park and almost make it look a little easy. There's a reason I often use the phrase #EvannaStrong in my updates. 

CONTENT – whether it’s playing for hours and hours by yourself or simply satisfied to sit and watch movies on your tablet… you my dear, are just a dream. While you've started to realize there's a world outside our 4 walls, you're the happiest at home playing with your favorite familiar toys with just mommy or daddy (or one of the nurses) within arms reach.

STRONG-WILLED – while this is a great quality, it sometimes makes certain tasks harder than it needs to be. We definitely see this come out the most during occupational therapy (but if we're going to be honest -- feeding therpay, physical and speech). We are trying so hard to expand your world, but no matter how hard we try, you almost shut down and not entertain new exercises and toys. You know what you like, and nothing else matters! While I hope you never loose this quality, I do hope that as you start to understand the benefit of these activities and be a little more open-minded to try new things. xo

EASYGOING – it’s amazing that despite everything you go through, how practically unphased by it all you remain. I do sometimes wonder if this will be something that will change as you get older, but I still feel like as you begin to comprehend more and more, you’ll still remain somewhat easygoing and continue to go with the flow as we’ve always done so in the past. You've been handed a rough set of cards, but continue to take it all in stride -- I couldn't be more prouder to be your mom than I already am!

DETERMINED – when there's something you want, you won't stop til you get it. Whether it's trying to gain our attention, play with a certain toy, watch a movie, or dictate how a medical task is completed. While you're very good at getting your nightly trach care done, lying perfectly still for ECHO's or receive breathing treatments (ie. that we did to treat that horrible cold that landed us in PICU last October) -- it's still done on your terms. You'll behave wonderfully for not so pleasant medical procedures, but only when it's done under favorable conditions... ie. you get to watch a move on the tablet. But hey, we'll take what we can get, and are happy to do whatever it takes to keep you happy. You endure more than most 3 year olds do, but take all those expectations that have thrusted upon you with so much grace and dignity -- and couldn't be more proud and honored to call you ours.  

 ////// things I want to remember ---
˔ you always wake up so incredibly happy and excited, it's infectious - wish I was that excited to wake up in the morning lol.
˔ despite your current lack of speech, you definitely know how to tell us what you want or express when you're either happy or upset.
˔ you do this cute little happy bounce when we've figured out what you want and we comply.
˔ brushing our hands aside is generally how you tell us no - but it's so adorable nonetheless.
˔ you get so excited when we pack up and are getting ready to take you out, and when we pull into the garage arriving home.
˔ your favorite movies currently consist of Frozen (still!), Monsters Inc (probably your #1 right now), Lilo & Stitch, Finding Nemo, Tangled, and Lion King if you're in the mood. It's really hard to "get you onto new movies", but I keep trying in an effort to save our sanity.
˔ while you're becoming less reliant on soothers, you always have "a favorite", and it always seems to change monthly lol.
˔ the "if you're happy and you know it" game is your favorite thing to do with your dad.
˔ I always gets asked if you tug or pull on your vent tubing, but the most you ever do is pick up and move the tubing if it gets in the way of your playing. It's kind of funny to witness.
˔ still doesn't happen very often, but once in awhile, you'll finally give in and give me a quick cuddle. Just a few days ago, your dad figured out that if he uses a blanket as a pillow (as he doesn't have boobs), you'll cuddle with him for a few minutes too.
˔ you absolutely love getting up on my lap and insist we play "peek-a-boo". you've even begun to dictate when I should cover my eyes, or uncover them to say "peek-a-boo".



Right now, things are GOOD. Close to perfect that we haven’t experienced in quite some time (which I would pin point to the summer after you were born). We’re essentially able to live “season to season” right now, and it’s a marvelous feeling to say the least! We’re very busy, but enjoying a lot of normalcy lately – and the future is starting to look very bright. We’re gaining some real momentum, and the flow of good news about your health right now has us feeling “cautiously optimistic”. I still have moments where I grow afraid of when this "period" will end, but I try my best to quickly shake those feelings away and enjoy the here and now while we can.

You continue to be our greatest blessing and life lesson… all rolled into one really cute package, and we love you so very much! 

[More pictures of Evanna's "Strawberry Shortcake" themed birthday party will be posted when I do my next update in a week or two]