WHERE ARE YOU FROM/DO YOU LIVE?
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!
CAN YOU EXPLAIN EVANNA'S CONDITION AND PROVIDE SOME HISTORY?
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).
HOW MANY SURGERIES HAS EVANNA HAD?
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.
WILL EVANNA NEED MORE SURGERIES?
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.
WHY DOES EVANNA HAVE A TRACH?
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).
HOW LONG WILL EVANNA HAVE THE TRACH FOR?
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.
WHAT IS EVANNA'S PROGNOSIS, AND WILL THESE SURGERIES CHANGE THAT?
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.
CAN EVANNA HAVE A TRANSPLANT?
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
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).
ReplyDeleteOur 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.