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We live, work and enjoy living in Calgary, Alberta, which is located just East of the Rocky Mountains here in Canada.
If you happen to every make your way in our neighbourhood, make sure to hit up the Calgary Stampede in July, or enjoy our stunning mountains with a trip to the nearby towns of Canmore and Banff (and make sure to stop by the stunning Emerald Lake on your way there!).
Some of my favourite places to grab a bite to eat in the city are The Coup, Ten Foot Henry, The Cattle Baron, REGRUB, OEB, The Beltliner, The Bro’kin Yolk, and Alforno!

Evanna was born with a congenital heart defect called Tetralogy of Fallot with Pulmonary Atresia, VSD and MAPCA’s. This was all caused by/due to a chromosomal defect called “22q11”, and still sometimes referred to as “DiGeorge Syndrome”.
Essentially Evanna was born without a pulmonary artery, and instead made a bunch of tiny arteries called “collaterals” (MAPCA’s) that takes blood to her lungs to get oxygenated – but these collaterals aren’t as effective as a regular pulmonary artery.
MAPCA’s/collaterals often stenos and can completely occlude (which is what’s happened to Evanna’s left side), resulting in insufficient PULMONARY BLOODFLOW to her heart, and why she requires so much continuous oxygen.
Based on our own research, myself and other MAPCA families have done a rough calculation (as there is very little information available online) and estimate MAPCA’s occur in about 1/1,000,000 births. Within this diagnosis, there lies a spectrum, and Evanna’s case is considered severe because she has no “native pulmonary artery” and is what’s called “collateral dependant”. Her collaterals are very small and even occluded on the left side (which is the first thing Dr. Hanley wants to fix/rehabilitate this summer).

Evanna underwent her very first open heart surgery in October 2014 at just 5 months old. While the surgery itself went okay, we were plagued with some postop complications (primarily airway related), followed by many hospital acquired infections that resulted in an 11 month ICU stay, and a total of 400 days in hospital (discharging in November 2015).

To date, Evanna has now had 10 surgeries –  4 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.

Yes. In fact, one of her biggest surgeries to date is scheduled to take place later this summer down in the USA at Stanford Children’s Hospital/LPCH. It will be her 5th open heart surgery, and we are also anticipating a 6th open heart surgery shortly after as well.

After Evanna’s first open heart surgery, she developed something called “tracheomalacia”, which basically translates to a “floppy airway”. Due to the severity (as not all kids diagnosed with tracheomalacia require a tracheostomy), we were unsuccessful in extubating her off the hospital ventilator for several months -- despite additional procedures and multiple attempts. In fact, for the first two years she had the trach, Evanna required some of the highest pressures (PEEP of 12) for a “homecare patient” here in our province (this has since been drastically decreased over the past 12 months and is on relatively low ventilator settings now).

While I can’t provide a definitive answer at this time, I believe we still have at least another year with the trach (not to mention the fact that we’ve already been living the “trach life” for over 3 years now). A bronchoscopy done last Spring (2016) showed that the tracheomalacia has officially resolved, but after being supported on a ventilator for almost 3 years has caused some major muscle atrophy of her diaphragm, and are currently working towards building that strength back up. Evanna is already off the ventilator during the day, and once she no longer requires the ventilator at night, can we begin to discuss the possibility of decannulation (taking the trach out). Evanna is “trached to be vented”. So once she longer requires the support of the ventilator, than she will no longer require a trach.   

Unfortunately, not even Evanna’s cardiologist can answer this. Evanna is one complex little girl, and it’s a very grey subject. Due to everything she’s been through, Evanna has already surpassed expectations, and why we all remain in awe of her current health + strength. We could have another year with her, or another 20 -- which is why every day we have with her is considered a blessing, and every milestone is celebrated.
With our upcoming surgery(ies), we intend to improve her pulmonary blood flow, potentially get her off oxygen, improve her quality of life, and just buy us more time with her.
Regardless, her life expectancy remains a huge question mark, but we are always trying to find ways to help improve things. While we’ve come to terms with the situation (the very real reality that we’ll lose Evanna someday), we try not to focus on that fact, and instead choose to enjoy the here + now.

No. The best way to explain it, is Evanna’s anatomy make it impossible to “hook up” a normal heart to her lungs because her heart and lungs are so uniquely intertwined due to not having a pulmonary artery (remember that she’s “collateral dependant”), and instead has that crazy network of pulmonary collaterals/MAPCA’s (which is why I call it #EvannasSpecialHeart).

As for a possible heart/lung transplant -- St. Louis Children’s Hospital in Missouri (which is considered the “center of excellence” in the WORLD when it comes to pediatric heart/lung transplants), did a study several years ago, and determined that it’s not a viable option for the MAPCA population whatsoever and highly discourage this route due to it’s devastating results (unable to control postop bleeding from previous collaterals/MAPCA’s leading to death). Essentially maintaining Evanna’s current heart function is the best thing and why we have such limited options.

updated 01/04/2018

1 comment:

  1. Hi. Howie from Norway It`s heartbraking to hear your story of Evanna, and still inspiring. I`ve looked for a long time for a family with a child having a similiar heart sickness as my boy Olai (8 months).
    Our journey is also a nightmare and is still uncertain, I guess as long as Olai lives.
    He also has MAPCA`s with pulmonary atresia, two collaterals going left lung, and one collateral to right lung. Starting with ccTGA, he got mitral atresia, so he only has one atrium (right one, on his left side), so he also has mixed blood. They did Rashkind surgery to secure the hole(kept it open) in the heart, from his disfunctional right atrium to his functionary left atrium. Hes has gotten several AV blocks, arrythmics, so now he has a pacemaker, puting his pulse on 90 :) Hes spO2 levels are 65-80 all the time. We are at home nursing him and giving all life quality we can. The doctors have no option for surgery at the time. This is because of the complexity of the MAPCA`s mainly, but also rest of the heart. We never give up though. He`s 8 months now, and going strong. May the MAPCA`s live strong for the rest of his life - though the odds are bad.
    Write back if you want :) Thanks for the inspiring blog.