Sunday, October 23, 2016


I get a little anxious when I get behind on my blog, and though I’ve certainly gone longer between updates, so much has happened that past two (almost three) weeks…. with no signs of slowing down. Despite my Instagram or our Facebook page for the intermediate updates, this platform continues to be my singular place to document and journal everything. This continues to be a huge personal outlet for me, and it honestly does provide a bit of relief when I’m able to organize my thoughts and write them out.

At the beginning of October, Evanna began to exhibit some symptoms that she was coming down with something. We held out for a day or two in hopes to avoid a hospital admission -- as we have been given the tools to deal with minor illnesses at home – unfortunately, Evanna continued to deteriorate and we decided to pack her up and take her into emergency (October 5). Cardiology had alerted the ER of a potential visit, as we kept in contact with them from the moment Evanna became ill at home – and so along with her long list of diagnoses, we were immediately taken to the back and seen by the emergency physician without having to wait.
After being assessed by the pediatrician, we were then assessed by one of the ICU intensivists for a possible ICU admission (protocol with most trach/vent kids). We of course knew the intensivist very well from last year, but we all agreed that clinically Evanna was “well”, and an ICU admission was unnecessary at this point of time, and we could proceed with going up to Unit 2. However, the “trach+ vent hallway” on Unit 2 was full…. so we ended up being admitted to ICU anyways (such is life).

Within 12 hours of coming into hospital, Evanna’s clinical picture actually improved dramatically, and we began talking about discharge from the ICU and continue to support Evanna at home. However, the night before we intended to leave, her labs came back from the blood draw that we did in emergency, and they were positive for an infection (pseudomonas). They immediately started her on some antibiotics and we knew right away that we weren’t going anywhere fast.
The following morning (October 7) a bed up on Unit 2 opened up, and so because Evanna was stable, we transferred up to the floor and settled right in, knowing that without a doubt we’d be in for Thanksgiving (marking the only holiday to date that Evanna has spent each year consecutively in hospital… though we’re cutting it close and will most likely see that same milestone for Halloween too).
The first week or so was spent hanging out, talking to our regular specialists of the clinics we’re associated with (cardiology & respirology), and waiting for the infection to clear so we could proceed with a PICC line (a more stable IV line to administer the antibiotics through) that would allow us to go home. At one point another bumpbegan to grow at the top of Evanna’s sternotomy and directly beneath her tracheostomy site, which had Cardiology and Infectious Disease freaking out and when we starting talking and communicating with Edmonton to keep them in the loop in case we had to transfer back up for another debridement surgery.

One of the biggest highlights from our first week in hospital was having a few good in-depth conversations with one of our trach respirologists about some major concerns I have about our “trach journey” and what I would like to see happen over the next year. Last month we had a “combined clinic” appointment with our respiratory and ENT teams, and I walked out quite disappointed and defeated with how it all went. Essentially I felt we got a “it is what it is” type answer for working towards weaning Evanna off the vent over the next year and the severity of our situation is not being taking seriously. After talking to the trach Respirologist, I was able to better convey some issues and concerns we had, and come up with a more concrete plan to book another bronchoscopy soon and to keep some additional options on the table should things remain the same by next summer. We also saw cardiology lots throughout the week as we are dealing with an Endocarditis diagnosis, and so they’re keeping a close eye on everything and keeping the communication lines open with Edmonton, but for the most part we wanted to see if we can clear things with antibiotics alone, as a debridement or even another open heart surgery would be a very big deal and pretty invasive.

About halfway through the second week, we noticed Evanna was getting a runny nose and concluded she had indeed caught a cold while in hospital. For the first several days it didn’t seem to cause any additional problems, but by day 5 (October 17) we began to experience issues with trying to maintain appropriate oxygen saturations. After hours of consistently bumping up her oxygen (to 10-12L) and administrating Ventilon every 2 hours (this helps treat wheezing and open the airways up), the unit called on the ICU to come assess Evanna and decide if we should transfer back down the ICU. Well, due to the fact that Evanna was surpassing the floor’s threshold and capabilities, they ended up just sending their transport team up to help pack us up and bring her back down to the ICU, as it was better to deal with this in a calmer state, than for things to progressively get worse and be rushed.

Down in the ICU they kept Evanna on 12L of oxygen, bumped her ventilator PEEP from 12 up to 14 and started her on a continuous Ventolin drip (it was hooked into the vent’s humidifier so that it could be pumped in through her vent tubing directly into her airway). By the next morning Evanna started to improve and we were able to decrease the amount of oxygen she was on. Unfortunately we learned that we got yet another positive blood culture that started to really worry the whole medical team, as it had been over 2 weeks since starting Evanna on IV antibiotics. On Wednesday (October 19) one of the cardiologists that is helping us send down a referral to California (there are two cardiologists helping us with our referral – Dr. Fruitman, our primary cardiologist and Dr. Rutledge because of our history and relationship with her up in Edmonton) to Dr. Hanley for other open heart surgery, came in and explained the current situation and how this infection is starting to become extremely worrisome and could potentially interfere with our entire California plan. Infectious Disease feels it’s time we transfer up to Edmonton for surgical intervention to switch out her conduit that they are certain is now “seeded” with Pseudomonas. While the IV antibiotics are somewhat clearing her blood, they think the conduit continues to “reinfect” everything. The problem with that, is it’s “easier said than done”, especially when we’re pursuing a different/another open heart surgery down in California by Dr. Hanley. So Dr. Rutledge informed us that they rushed the referral down to California that evening and have asked for an expedited answer so we can better plan on how to proceed over the next few weeks.

On Thursday (October 20), Infectious Disease and Cardiology decided to have a meeting, as they continue to somewhat disagree on what needs to happen. I was working, so Nick attended it by himself -- as we are continuing to try and work around each other’s work schedules so that one of us is always at the hospital with Evanna. Basically, Infectious Disease reiterated that Evanna needs surgical intervention, however Cardiology is saying that going back to Edmonton to change out the conduit is more complicated than it sounds. We are hoping to hear from California in the next week or so and essentially wait to transfer Evanna down there to have the open heart surgery we want to do and change out the conduit at the same time. However, in the same breath, the cardiologist made it known that she honestly thinks Evanna’s case is somewhat beyond Dr. Hanley’s capabilities and believes he will tell us that there’s nothing more he can do. If so, while our next option would be to go to Edmonton to change out the conduit, the cardiologist asked that we consider our options very carefully and weigh out how much we want to continue to put Evanna through, as again, another open heart surgery to change out her conduit is much more complicated than it sounds, and they really worry about how she’d do from a recovery standpoint.  


Nick and I have been through this before, and if anything, it just continues to get harder to talk about the older Evanna gets. We’re absolutely stunned that we are having to talk about this again so quickly, and it truly leaves you breathless.

So where are we now? Well they did a CT on Thursday to both assess the bump at the top of her sternotomy and have a fresher angiogram to send down to Dr. Hanley (which was immediately sent by FedEx). The good news is the bump continues to improve and I guess it’s a good thing to have fresh imaging for Dr. Hanley to base his decision on.
We finally got a PICC line yesterday morning as we’re running out of IV sites (which went well) and we’re somewhat in a very nerve-wracking “holding pattern”, as we wait to hear from both California and Edmonton with their opinions. As of today, we have finally gotten THREE NEGATIVE BLOOD CULTURES in a row, which is allowing us to all breath a little sigh of relief and feel a little less rushed with our decision making. Evanna has also back to her “baseline” from a respiratory standpoint (down to 4L of oxygen, etch) since catching this cold and so we transferred out of the PICU and back up to Unit 2 this afternoon.
Tomorrow is Monday, which means we’re in for a very busy day with hopefully trying to come up with a discharge plan. Yes, we have a PICC line, but Evanna is still on TWO antibiotics, and they generally don’t let you do Home Parenteral Therapy Program (HPTP) on more than one IV drug. I’m honestly hoping we can come up with some sort of compromise as the longer we’re in hospital, the chances of Evanna catching another cold increases – especially this time of year. Our most acute issue right now is Evanna has gotten a little “backed up” with poop this past week and is incredibly uncomfortable. We’ve been working on clearing her out the past few days (yeah laxatives), but we still have a little ways to go based on her grumpiness level and continued periods of retching (especially when we sit her up).

This hospital admission has been probably one of our most brutal to date emotionally (especially the past 5 days). We were admitted due to an infection (that is finally showing signs of clearing), but we’ve been forced to see the reality of our situation. At home, all we see is a happy and thriving Evanna, but the truth of the matter is, on the inside, Evanna is slowly fading. We no longer have pulmonary blood flow to her left lung (and there is NO way to surgically fix that…not even Dr. Hanley), and the fact we have not been able to make any progress in decreasing the amount of ventilator and oxygen support Evanna is on since last year, only confirms our greatest fears – we are eventually going to lose Evanna, and it’s only a matter of time (to clarify, it could certainly be several years, and a transplant is NOT an option due to how her heart and lungs are uniquely intertwined). We are still waiting to hear what Dr. Hanley has to say, but our medical team has stated that even IF Hanley comes back saying he might be able to help improve Evanna’s quality of life, it will come with many risks attached to it. Nick and I are generally on the same page in regards to the “bigger decisions” and have known for quite sometime that Evanna is terminal, but the team has asked that we start to discuss the smaller details in regards to how aggressive we want to be and evaluate what’s important to us (which at this point I will keep private, as according to our medical team “there is no wrong answer”, and so those details will remain personal and not be posted for public opinion).

Thank you to everyone who continues to pray for Evanna and support us. I DO read each and every comment, message and text sent to me (sorry for my terrible responding skills), as they truly help lighten and uplift us. Our situation continues to remains incredibly hard, and so our hearts ache under the weight of this trial that Nick and I are having to endure and the decisions that we have to continuously make as parents.

xo The Irvine Family

Monday, October 3, 2016

[summer diary]

As a blogger, I had every intention of posting our summer happenings as they occurred…but alas, it wasn’t meant to be. Instead, I’ll just do a little synopsis for journaling purposes, get some pictures up, and call it a day.

The first week of July consisted of some fun, and then not so fun moments. We enjoyed a really nice Canada Day at my parents’ house, where we kept it light + casual, and reflected where we were this time last year, and how far we had come since that day. Then just a few days later, I ended up in Emergency as I was finally miscarrying from my chemical pregnancy, and was rolled into the operating room at 3am for a D&C. Thankfully, it happened on a night that Nick was home, we already had our night nurse at the house watching Evanna and coincidentally already had a nurse booked the following morning, who was able to continue to take care of Evanna in our absence. Though I lost a fair amount of blood, I was able to avoid a transfusion and was discharged in a timely manner at noon the that day.

A few days later we completed our 2nd last walk through of our new house, celebrated Nick’s birthday down at Prince’s Island Park –followed by playing a few games at a board game cafĂ© here in Calgary before calling it a night. Later that weekend, I left for Edmonton to a enjoy some time away with a few heart mamas friends at a lake house located at Jack Fish Lake and thoroughly enjoyed myself. The weekend consisted of lots of laughs, singing, campfires and boating fun. We actually repeated it again over the September long weekend, but unfortunately the weather hasn’t been super cooperative here in Alberta this past summer…. so I hope that when we do it again next summer, we’ll finally have the weather on our side.
The day after I got back from the lake, we had a photoshoot booked for the upcoming issue of the Stollery HEROES magazine that came out at the end of August that featured our family’s story. It was an incredibly huge honor and definitely wasn’t an easy secret to keep to say the least (#proudmommymoment for sure).
Other July moments included a hilariously fun night out during the Calgary Stampede with some friends, and of course the physical move into our new home.

Other than trying to get settled into the new house (a little easier said than done when it comes to Evanna), the biggest highlight of August was my “staycation” with some friends at the Country Thunder Music Festival here in Calgary. There was a LOT of coordination that went into it, as it landed on a weekend that Nick was working, but we were approved for additional respite hours back in January (every year, we can ask for a “respite weekend” up to 4 times and gain some additional funding to pay our homecare staff), and our nurses graciously agreed to work some extra shifts so I could enjoy this little treat. It was such a blast, and depending on what happens over the next year, I hope to do it again next summer (but that’s a little too far in advance to plan right now).

OH SEPTEMBER. Probably our busiest month socially. Like I previously mentioned, I went back up to Edmonton/ Jack Fish Lake the first weekend to see some heart mamas again. We only spent the first night at the lake, as the weather left much to be desired, but figured out some alternative plans in Edmonton instead.

The next weekend I went to Canmore for a “Spa Weekend Getaway” for a friend’s bachelorette/bridal shower, which included a fabulous private dinner, some major spa/relaxation treatments for this mama, a crazy night out on the town, followed by a beautiful brunch and soaking in the Upper Hot Springs in Banff before heading back home. The following weekend, Nick attended the bachelor party (we were both in the wedding party, so the wedding events somewhat felted doubled for us lol) here in Calgary, and the wedding took place last weekend. We were blessed with beautiful weather (which unfortunately was a rarity this summer), and we had an absolute blast at the reception (even met a new friend or two!).

Plans for Fall/Christmas are already getting underway, and my planner continues to fill up like always. I’m trying to not plan too far in advanced right now, as we don’t exactly know what's going to happen with Evanna medically, but my gut is telling me that we are somewhat safe until after Christmas. First we plan on hosting two separate family Thanksgivings this upcoming weekend, as it will be easier for our families to come to us –and now that we’re in the new house, our dining space will make it a lot more feasible this year (unlike the tiny little nook we had in the townhouse). In November I hope to throw a casual housewarming party and invite both close friends, as well as some people we haven’t seen in a while. My 28th birthday is also lands in November, and my best friend and I have booked a trip to Vancouver and Seattle to see her family and have some fun site-seeing (Seattle tips would be greatly appreciated!). While I have an idea of what Christmas will entail (again, we’ll probably host our families at our house for convenience), we’ll wait to see what comes of our consult/second opinion in California before solidifying plans at the moment. 

Overall, we kept this summer both light and fun. We needed a break to just enjoy quality time as a family and be happy -- so that's exactly what we did. It’s still incredibly hard to take Evanna out of the house right now (I actually have a post on this particular topic currently saved as a draft -- which will be uploaded later this month) – plus if the situation involves Evanna having to stay in the stroller, it’s just not quite worth it, as she doesn’t enjoy nor benefit from an outing. We have been able to take her to church almost every Sunday with help from my parents on the Sunday’s that Nick works, and have even worked our way up to the full THREE hours (which is a lot of work entertaining a immobile toddle without the use of noisy toys)! While taking her to nursery at church is on our radar, Nick and I have both agreed that we’ll examine that option sometime after Christmas. Obviously one of us would have to stay with her the whole time (not about to put the care of a trach baby on the nursery leaders lol), but Evanna is just starting to get to a place where she actually interacts with her surroundings, so she isn't quite ready to make that transition. Though kids start attending nursery at church at 18 months old, Evanna is not at that stage neurologically, and wouldn't benefit from it at this moment in time. However, now that we’re better moved into our new house, I’ve been inviting some people over like my sister and her kids for play dates, and hopefully we’ll see continued development in that particular area. It’s definitely a challenge to do things outside the house with Evanna that are both logistically and developmentally possible, and right now Evanna does the best at home in her familiar surroundings, interacting either with us or her homecare nurses. My hope is that we can make some gains this winter/spring with the amount of life support that Evanna is on, and enjoy a more active summer next year. When I do get a little disheartened about how much we had to somewhat leave Evanna at home and inside this summer, I try to think about where we were last year and how much strides we have already made and hope that we will continue to make those same type of gains and encounter less logistical issues next year.

xo The Irvine Family

Sunday, September 25, 2016

Spotlight Q&A

////// Earlier this year, I was asked  if I could answer some questions for another bloggers Q& A. I spent a fair amount of time answering them, and feel it's only appropriate to repost it on my own personal blog for our readers to see. xo //////

To start things off, tell me what the past two years have looked like for you.
A roller-coaster. Our beautiful daughter Evanna was born with a rare and extremely complex congenital heart defect. After enjoying a blissful 5 months at home, we handed her over into the arms of our surgical team for her first open heart surgery on October 6, 2014. She went into the surgery relatively “healthy”, and overall we were expecting a 6-8 week hospital stay. While the surgery itself went well, we unfortunately experienced setback after setback and complication after complication that would keep us in the ICU for 10.5 months for a total of 400 days in hospital. I ended up having to take an extended maternity leave so I could be at our daughter’s bedside at all times, while my husband commuted between the city of Edmonton and Calgary to both fulfill his responsibilities as an employee and husband/father. To date, our daughter has now had 9 surgeries – 2 additional open heart surgeries, a major left thoracotomy (ie. it was a heart surgery done from the side), 2 diaphragm plications, a tracheostomy, Gtube placement and a sternal debridement, among countless other procedures and tests. She coded 3 weeks after her first open heart surgery, experienced a very scary mechanical valve failure (it clotted in an almost closed position), an acute liver failure episode, and complete Septic Shock episode last July that almost (and should have) took her life. We were finally discharged on November 9, 2015, and have been enjoying having our whole family sleeping under the same roof once again.

How is Evanna doing now?
Evanna is doing quite well despite circumstances. Our admission was extensive, and so the “recovery” will essentially take years; however, she is blowing our medical team out of the water and already surpassing their expectations. We were told that a child of her complexities tend to bounce in and out of hospital, but other than a little “bump” this past April, we have remained admission free (“planned admissions” don’t count, because they generally mean progress in my book).
We are also moving on with our life – I’ve returned to work part time (in the capacity we had always planned regardless of Evanna’s medical situation), built a new house, and plans to expand our little family are in the works (while this was all caused by a chromosomal defect, it was not hereditary, so we have been cleared by our genetic team to have more children). As for her future, we unfortunately will never be given a guarantee (but I try to think that no one in life has a guarantee). We believe decannulation (weaning of the home vent and taking the trach out) will happen sometime in the next few years – but it’s Evanna’s “special heart” that will unfortunately be her life time battle (this disease will eventually kill her to say the least). At the moment, a heart transplant is not an option for due to unique anatomy issues (though I must clearly state that a transplant is in no way a cure), and there is only so much our medical team can do – so it’s mostly up to Evanna and her body to keep fighting. My husband and I have decided that we will do whatever we can to advocate for Evanna, and as long as she willing to fight – then so will we. However, if there comes a time that she is done and nothing more that our medical team can do, then we will ensure it’s done in the gentlest way possible, knowing in our hearts that we have done everything we could.

What would you say has been the hardest part of everything your family has been through in the past two years?
“The Talks”. Probably every parent’s worst nightmare is being told that your child is terminal or be prepared to “let them go”. From the moment Evanna was born and received her rare diagnosis, we have had “the talk” on numerous occasions with different doctors and specialties. Some have been extremely serious – like last summer when she went into Septic Shock and we were told that “this may be it”, to a more casual (for lack of a better word) conversation with our primary cardiologist about Evanna’s potential prognoses. We’ve even had one ICU physician somewhat question why we would pursue medical care in the form of additional surgeries and procedures down the road, if she’s going to be terminal anyways (we weigh each surgery’s risks and benefits, and absolutely take her quality of life into account each and every time).
Whenever we have a new admission, we always have to reconfirm her “care goals” (think DNR/comfort care questions), but thankfully our primary medical team has recognized our current goals. While we don’t talk about the issue too often anymore, our primary medical team do know that my husband and I have a good understanding of the situation, and despite our “cheery disposition”, for our own sanity, we only now discuss it when absolutely necessary.

Did you find that your personal health and well-being were affected by all of this?
I think the biggest issue has been my diet. While up in Edmonton, my husband and I were given a certain daily allowance to spend on food. It was actually quite the generous amount, and so we lived off a lot of fast food, items from the hospital cafeteria, and the comfort food cooked for us at the Ronald McDonald House by volunteers. Unfortunately, making our own meals fell to the bottom of our “to-do list”, and it wasn’t a priority while our daughter was in the PICU, and so the scale has somewhat reflected this over the past year or so. We have finally somewhat reached a level of stability and new normalcy, so I’m currently trying to figure out the best way to take charge of my personal health and make it a priority.

How did you balance self-care?
Creating balance and self-care kind of evolved over time, and changed depending on where we were (we bounced multiple times between our local children’s hospital PICU in Calgary and the Stollery PCICU in Edmonton). In the beginning, we spent incredibly long days in the PCICU; however, with no choice in sleeping there (it’s an old and open ICU, which means there are no private rooms – unless you’re on isolation), you have to leave in order to get any sleep. I know many parents find this particular issue really hard (guilt being the biggest culprit), but we felt our child was in the best hands, and so this was sort of a nonissue for us. We knew we could call any time of day, the unit was open to us at all hours (ie. visiting hours do not apply to us), and they of course would call if something came up (which unfortunately happened a handful of times, often requiring our immediate presence). While the option to sleep at the hospital became available to us whenever we were transferred back to our “home” children’s hospital, or when Evanna finally came out of ICU and onto the regular floor – we still continued to go home and sleep in our own bed. It was the routine that we were all use too (and Evanna never seemed to mind), and so for our own sanity, we continued this practice. Yes there may come a day, when Evanna gets older and may require one of us to stay the night with her during future hospital stays…but we’ll cross that bridge when we get there.
While one of us was always at the hospital physically, we made sure to always take breaks whenever possible. The days my husband was not working and up in Edmonton with us, we would both go to the hospital – however he somewhat took over in paying attention to Evanna, so I could just sit back, and read either a book or be my phone (giving me a mental break). Towards the end of our main admission (I'm talking month 10/11 here), and back in Calgary, we decided to each take turns on a weekly basis to take a whole day off. Usually my husband would take the first day after his work rotation off to sleep in (as generally he was getting off his last night shift), run errands and have some leisure time. In return, I usually took the following day off from going to the hospital to also run any personal errands and essentially do whatever I pleased. As parents, we are both equally involved with our daughter’s care, so our medical team loved that we did this, as it ensured we wouldn’t experience “caregiver burnout”. When Evanna was finally transferred out of the ICU, the hospital also assigned a special volunteer to us that strictly came in two mornings a week to be with Evanna, so we didn’t feel rushed to come in those days. The volunteer would work on physical therapy and entertain Evanna with books and toys when she woke up, and overall ensuring that her care remained consistent even though we weren’t present. Overall, every family needs to figure out what works best for them while attending and balancing individuals needs too. "Hospital Mode" (as I like to call it) is unique and different for each family depending on social, family and financial dynamics, but as long as the end goal is the same  then there's no really right or wrong way to go about it. 

Taking breaks and prioritizing self-care has continued to remain in practice since we’ve been discharged from the hospital – with just a few tweaks here and there. My husband loves playing board games (it’s a huge hobby for him), and so almost once a week, he will get together with his friends, and they will play for hours and hours. In return, I plan lots of social get-togethers and quite frequently am able to get out of the house to see friends. While I know this isn’t feasible for many “medical families” (heck, I’m sure as more kids enter the picture, the frequency of this may decrease slightly for us), I can’t emphasize taking time out for yourself. My advice is to work together as a team and build a support system, as “caregiver burnout” can occur when the responsibilities fall on just one person.

Who or what has been the biggest source of support for you and your family?
For me personally – my husband. He has been my rock and partner through all of this, and continues to be to this day. He continued to work almost full time through the entire admission (only taking days off when it was absolutely necessary), and took over for me at the hospital when present, to allow me a mental break. We have gotten through this as a team, and will continue to moving forward.
As for our family – well it’s been the whole community. From hospital volunteers and strangers (whom I now consider friends) at the Ronald McDonald House, to people from our church and local neighbourhood. I also have to mention the entire medical team (at both hospitals); as when they aren’t busy treating our daughter, they also have been so caring and compassionate towards us, ensuring we are well taken care of!
Then of course our awesome friends and immediate family members, who have been there to help where they can, and support us every step of the way.

For other mothers with hospitalized children who may be reading this, what message would you like to share with them?
Take it one day at a time – this is a MARATHON, not a sprint, and so be careful not to burn out (I feel like this has been a theme for this particular questionnaire – oops!). When your child is in the hospital for a prolonged amount of time, your role as a parent changes. You have a medical team essentially “kissing the booboos” and making them feel better (ie. pain meds), and your job is to now to advocate for your child’s medical care, be their voice, ensure that things remain constant and that everyone is on the same page (sometimes things can become muddled when you have lots of “cooks in the kitchen”). Being well nourished and rested means that you will remain strong and of “sound mind” – which is what your child needs the most right now.

Is there anything I didn’t ask that you would like to share?

I guess I would like to touch on what readers can do to help. From simply educating yourself on congenital heart defects and their prevalence (each year more children die from congenital heart defects than from all childhood cancers combined), and the fact that there is no cure (heart surgery is considered a “palliative measure”, and is in no way a complete and total “fix”). Learn about and become an organ donor. While I don’t wish trauma on any family, knowing what you would do ahead of time may help make the decision much less difficult should the situation ever arise. If you are healthy individual, looking into becoming a regular blood donor (to date, our daughter has had over 30 blood product transfusions), as it’s a relatively simple and easy thing to do. None of the above involves money, but obviously if you are in a position to donate towards CHD, do your research, and ensure you are giving to an organization that actually puts money towards congenital heart research, and helping families that are affected by CHD’s.

Tuesday, September 20, 2016

Shooting for the || MOON + STARS ||

Photo by Guenard Photography

For the most part, I’m generally an open book (you sort of have to be if when you decide to dive into the blogging world), but I’ve recently realized that I’ve actually become a little closed off in what I actually share publicly (which you may not necessarily believe, but it’s true). I think it started when we surpassed the 4-5 month mark in the ICU and a tracheostomy became our new reality. I am the ultimate social butterfly (to which the ICU staff graciously put up with), and suddenly I no longer wanted to interact with other heart families within the ICU and even became somewhat resentful towards the ones that came and left so quickly. I then became aware of the mean-spirited and judgmental trolls that live online who just love tearing people down – no matter their situation, and while I’ve only been a victim to it once or twice over the past 2.5 years, I began to put some major filters on what I shared online to avoid such unnecessary situations. I’m also still in a place of anger and even a little jaded when it comes to the “cards we’ve been dealt”, and so I’ve sort of withdrawn myself from the online medical community – except the select few that are more specific to Evanna’s diagnoses. And finally, I just hate talking about something that isn’t a guarantee – only for nothing to come of it…. probably because it was a little heartbreaking anytime I wrote/blogged about something (Evanna related), only for something else to come up and the plan to completely change – so I began to not post something until it was a for sure thing.

However, there are some MAJOR things happening, and while my instincts are telling me to not talk about it until things are official, truth is we need the support, prayers and same love that we had last year to get through this continued roller-coaster. We might experience a miracle, but we also might experience some major heartache, and regardless of what happens, I owe it to those who continue to love Evanna and our family the same openness that they’ve come to know. So here it goes:

Last week I posted that I was going to put my “advocating mama” pants back on, and somewhat “rock the boat”. Obviously this was my way of letting everyone know that stuff was happening, but remain vague in case nothing came of it. WELL, basically we are perusing not just one, but TWO more surgeries for Evanna – and BOTH would take us to the USA. That’s right, there is a chance that we could be headed stateside and seek medical care South of the border, and the reason I’ve been reluctant to share any of this publicly, is that there is a huge possibility that nothing will come of any of it either. Then there’s a possibility that we will only be able to proceed with one of the surgeries…. and then there’s also a small possibility that both could be on our horizon and it all could actually happen.

First (the “stars”) would be another open heart surgery down at the Lucile Packard Children's Hospital in California. There is a pediatric cardiovascular surgeon named Dr. Frank Hanley there who happens to specialize in Evanna’s specific congenital heart defect and very well known within the CHD world. We’ve somewhat come to this plateau with Evanna (specifically the large amount of oxygen she’s on), and so we’ve asked if we can obtain a second opinion + consult with him. What’s extremely scary and so incredibly nerve-wracking is that if he comes back and says there is nothing he can do, then we somewhat know we are that we are officially out of options with Evanna and her special little heart. I can’t tell you how hard it is to say those words – heck, write them out. He will either answer all of our prayers, or give us an answer that we’ve been so trying so hard to avoid.
Our cardiologist Dr. Fruitman completely agrees with this step and has also agreed (at my request) to involve another interventional cardiologist who knows Evanna very well (who coincidently use to work at the Stollery just last year) and is behind on previous CHD cases that were sent to Dr. Hanley. Our cardiologist will first email our primary surgeon Dr. Rebeyka (as we do need them to also support this decision if Dr. Hanley agrees to performing surgery on Evanna for funding purposes), and will then proceed sending Evanna’s records down to Dr. Hanley’s office for him to review. It will then be a little bit of a waiting game to receive an opinion and whether he thinks he can help Evanna. After that, it will be serious of steps to arrange the funding, surgery date, and transport down to LPCH – however I don’t even want to think about that until we hear back from Dr. Hanley first (and because Evanna is currently very stable, we probably won’t see a surgery date until after the New Year due to his very large case load).
My brain hurts, I’m already mentally exhausted – and I’m only halfway through.

The second surgery (the “moon”) is an airway surgery that’s currently in clinical trial down in Michigan. This one is slightly less likely to happen, but we are still shooting for it nonetheless. From what we know – Evanna does seem like a great candidate for this procedure and our primary respirologist is interested in willing to try and go for it. From a cardiology perspective, we have their support, because they believe anything that will improve her respiratory status will only help and improve her cardiac status too.

There is a lot more hoops to jump through for this particular surgery – the biggest being the fact that it’s still in a clinical trial phase. Then take into account that we live in Canada, and all the red tape that brings – overall, I’m trying my best to remain level headed about the whole thing and know it’s going to take a lot of work and advocating to see this thing through.

The surgery entails inserting a biodegradable 4D custom stent around Evanna’s trachea/upper bronchi to help make it stronger and keep it stented open. The fascinating thing about it, is it grows with the patient and then slowly dissolves into the body over a 3 year period (so there’s no having to go in and switch it out as she grows – which was a HUGE problem they encountered in the past). The custom stent is printed from a 3D printer (how cool is that!?), and actually costs very little produce. It’s the other parts to this that make it easier said than done (getting accepted as a candidate, persuading the out-of-country department to get funding approved, getting Evanna down there, etc), and so I’m trying my best to take it just one step at a time.

First step will be to officially get our whole respiratory team on board – including our ENT. As mentioned in previous posts, we have a “combined clinic” at the end of the month with both a respirologist (unfortunately it will not be our primary one, but she’s making sure it won’t cause any issues) and our ENT. Earlier this summer I emailed them a video (click HERE to watch for yourself – but be warned that it’s very long [skip the first 10 minutes] and filled with lots of medical jargon…to which I mostly understand) that is a recording of a recent of a conference where the physicians who created this device and procedure talk in detail about the whole thing – the surgeries they have done, and the absolutely amazing results they have already seen to date.

coincidentally ran into our primary respirologist a few weeks ago while working at ACH, and after letting her know how Evanna was doing, asked if she got my email. She informed me that not only did she get my email, but the week before our appointment, the whole team has scheduled a meeting and they will watch it together as a group. That actually shocked me a little, and was somewhat taken back at how serious they are taking this whole thing/my request.

Essentially they are calling this surgery the “cure” for tracheomalacia. It will take children with mild to moderate tracheomalacia and potentially avoid a trach and vent all together, and then take severe cases like Evanna and dramatically decrease the amount of time they will require a trach/ventilation for. That’s just mind blowing to say the least! They’ve already done a fair amount of these surgeries, and the results are just astounding, and so they have more or less moved into a period of clinical trials so they can make this a routine/standard procedure in the near future. I’m trying to emphasize this to our team here, as I think it would be a great opportunity for them to send Evanna for this procedure and somewhat be at the forefront as this technology/technique becomes more and more widely used. The amount of money spent keeping trached + vented children alive and healthy is insane (think time spent in hospital, medical supplies, homecare, etc); and so if a single surgery can either help avoid or decrease that time significantly, then I think our medical team/health region/Canada needs to pay very close attention – not to mention the remarkable increase of quality of life it will provide to these families too.

So that’s where we are -- shooting for the moon + stars, and essentially seeing where we will land (and I’m finally laying it all out there….as talks for these surgeries have been happening since May). It’s so incredibly nerve-wracking because nothing could come from any of this, or we could get just one of the surgeries, OR we could be in for a whirlwind and see ourselves through two more major surgeries – both of which are very far away from home.

That being said, I would like to state which one is more important to us and why. Both of these surgeries could provide huge benefits for Evanna and her overall continued health, however, one is slightly more important than the other. We (and our medical team) feel that the heart surgery by Dr. Hanley in California is far more important and crucial for Evanna than the airway one. At the end of the day, everything comes back to Evanna’s special heart, and it’s what will determine her long term prognosis. If he can help her MAPCA’s/collateral's and even do a “full repair” (and close her VSD), that would be HUGE. The airway one is definitely important and will improve her quality of life exponentially and could even benefit her cardiac status, but it’s slightly a little less crucial at this very moment in time, as a “no” will just mean a longer trach/vent journey. Nick is also slightly more apprehensive when it comes to pursuing the airway surgery.....but he’s generally more reluctant than I am when it comes to agreeing to more surgeries anyways (he somewhat fought the PEG tube and then fought it again when we switched to the button, though in the end he is very happy we did it lol).

Alright, I’m done. As you can see, we’ve had a nice little break and time at home with Evanna to basically regroup, but clearly we still have many more mountains to climb, and so it’s been a little overwhelming situation lately. We’re trying to just take it one step at a time and not get ahead of ourselves… but that isn’t always easy. I’ve lately been asking myself what the heck we’re getting ourselves into, but I’m trying to stay focused on the task at hand and know it’s for the best. Wish us luck, and your sincere prayers, love + good thoughts would be greatly appreciated once again.

If you read this far, and was able to somewhat keep up – then kudos to you! To say that I’m experiencing a little anxiety would be a huge understatement right now. I even told my SIL Megan who was helping me take Evanna to our appointment, that I felt a little shaky as we left the clinic, and I’ve even been experiencing a few moments of nausea from being so nervous that I could almost puke (not pregnant fyi) since then. I just absolutely the hate these “limbo/crossroads” moments, and the overall feeling of “not knowing”. Certain things are completely out of my control, and if there’s one thing I hate – it’s not being in control….especially when it comes to my child’s life.

All of this isn’t going to be easy, and I’m trying to prepare myself in the situation we are told “no”; however, I’m going to do whatever I can and continue advocating my heart out for Evanna’s heart regardless of what happens over the next few months.

xo The Irvine Home


Other tidbits:

- Though I already posted on our Facebook page at the beginning of September, we finally heard back from the immunology clinic and just like we figured – Evanna does indeed have a great + normal immune system, and was given the go ahead to proceed with getting Evanna caught up on the rest of her vaccinations. Like I wrote a longtime ago, immunodeficiency is actually very common among the 22q11/DiGeorge community (the chromosomal defect that caused Evanna’s heart defect and airway issues), which is why we had to wait to do some blood work and get immunology’s approval before proceeding with any live vaccine. It’s just been a small relief that we can keep this one major thing off our problem list right now.

- As of last week, Evanna is officially caught up on all her immunizations! Other than needing a little Advil/Tylenol the first 24 hrs after the MMR shot – she handled it all very well.

- We actually received some good news during last week’s cardiology appointment. Last year, Evanna’s right ventricle (the bottom right chamber of the heart), took a bit of a hit and were told there was some “decreased function”. Generally speaking, this is one of the first areas that will take a “hit” when things aren’t going well cardiac wise (if the heart is struggling, the right ventricle will essentially try to help and alleviate things), which unfortunately can begin to lead to many other problems (such as high pressures and heart failure). Last week’s ECHO showed lots of improvement to which our cardiologist is very happy about (and so are we!).

- We are followed by the cardiac dietician, and so we also quickly met with her during our cardiology appointment too. She plotted Evanna’s height and weight on both the global and 22q11 growth chart, and is also very happy with how things are going. While she’s roughly hits the 3% - 12% on the regular chart (don’t quote me on those exacts numbers), on the 22q11 growth chart, Evanna is 25% for height and 75% for weight and “leaning out quite nicely” lol

Sunday, September 18, 2016

HEROES Cover Shoot

Alright, it’s about time I stop withholding these photos (not intentional -- I promise). I was originally going to share these photos in the HEROES Magazine Cover blog post, but then when I got my hands on them and saw how many there were (and good ones at that), I decided they needed a post of their own.

Just want to again thank the Stollery Foundation for this absolutely amazing honour and Brian Bushsdruecker of Bookstrucker Photography for being so flexible and capturing some beautiful photos of our little girl. 


– just one week left to enter to win a $75 voucher for a stunning JORD watch [Click HERE to enter]
– I will be posting a very important post on Tuesday (it’s all written up and just waiting for me to hit the “upload” button). There are some very important and serious things happening right now, and I’ve decided to “lay it all on the table”, and fill everyone in on what’s going on regardless of what happens.