Saturday, October 29, 2016

HPTP Training + 4th Open Heart Surgery

"Little Warrior" Shirt c/o Jacob .Grace Designs

As medical parents, I legitimately feel like “we’ve done it all”. Okay, so I know that isn’t quite true, but going home with a GTube, trach, home ventilator, oxygen and IV therapy…. you can’t fault me for feeling that way. I didn’t think we could get anymore attached or tethered then we already are while at home – but guess I’ve learned that it’s still possible (however, I would like to boldly clarify that the trach/vent will remain as the most critical, intense and complicated of it all…it’s an airway after all). After cardiac conference this past Tuesday (which I’ll get to in a moment), we were officially given the green light to initiate the Home Parenteral Therapy Program (HPTP) and work towards discharge. A nurse from the Infectious Disease (ID) clinic came and met with me on Wednesday (October 26) to go over the basics and set up the timing and training of it all, but I’ll admit that I was a little surprised by a few small logistics. I guess the biggest surprise is the fact that though Evanna only gets ceftazidime three times a day, she is hooked up to the home IV pump 24/7 (we only disconnect her to change out IV bags once every 24 hours). I plan to post the mechanics of it sometime later next week, but basically even though Evanna essentially gets 3 doses of ceftazidime every 8 hours in the form of a “bolus” through her PICC line, to help avoid unnecessary disconnects while at home, the pump continues to deliver a very small amount (I believe it’s 2mLs/hr) in between each bolus. Talk about a small “pain in butt” -- though I’m still incredibly grateful that this is an option that we are able to utilize and allow us to take Evanna home – even if it’s just for a little while.  
On Thursday (October 27), Nick and I met with the ID nurse down in clinic where we spent almost 2 hours going over how to work the IV home pump and change the bag IV bag once a day. I had to chuckle because even though the nurse was showing us “best practice” that is taught to parents – Nick obviously did a few things “his way” due to his profession as a Paramedic (which the nurse didn’t mind at all).
Yesterday afternoon Evanna was hooked up to the home IV pump, and we were finally allowed to take her home. The homecare team came out this afternoon to walk us through our first bag change around 3pm, and will come again tomorrow. They will come again once more on Monday as they have to come every THREE DAYS while we’re home to prime and attach a new line regardless, but we should be good to go from there on out. I’m most nervous about Tuesday, as we have a follow-up appointment with Cardiology around the same time we have a “bag change” ….so that’ll be interesting to try and handle in the middle of an appointment (thankfully I’ll have one of our night nurses with me to help if needed).

OKAY…onto cardiac conference. Though it’s already been 4 days since Dr. Fruitman (our primary Cardiologist) came and explained the situation, I’m still trying to wrap my head around it all. It was decided by the Stollery surgical team that we do in fact need to transfer Evanna back up to Edmonton in the very near future and replace her hardware/prosthetic material (BOTH the conduit + valve). This is both because of the type of infection we are dealing with, and how long it took to clear from her blood stream. From an infectious disease perspective, Pseudomonas is an incredibly scary bug to have within your bloodstream. It’s not one easily cleared from the blood, and while it’s hard to see from diagnostic imaging, it more often then not likes to create an impenetrable “biofilm” around foreign material (ie. heart prosthetics). While every open heart surgery comes with an enormous amount of risks – the fact that have 4+ negative blood cultures makes them all breathe a little easier and not as hesitant to proceed with this plan of another OPEN HEART SURGERY -- Evanna’s 4th to be exact. We however have to wait until the end of November due to the rhinovirus/enterovirus (cold) that we experienced last week (the sole reason why we ended up in PICU) before the surgical team can proceed going back into Evanna’s chest.
This means that yes, though we are HOME from hospital – it’s only temporary (*cue tears*) for just a few weeks. We don’t quite have a date, but I’m assuming it will be sometime after November 17th, as that’s about 1 month from the positive viral swab we took in October -- but we will hopefully know more after Tuesday when we see our Cardiologist for follow-up. While it hasn’t been confirmed, I’m also 99% sure that we initially will have to admit Evanna down here at the Alberta Children’s Hospital so she can be medically transferred up to the Stollery Children’s Hospital due to all of her medical equipment (trach, ventilator, oxygen, etc), and I anticipate that we will be in Edmonton/at the Stollery for 1-2 weeks before transferring back down to ACH to continue Evanna’s recovery (this timeline is complete guess based off our own personal experience).

------------------that’s not all------------------

Our HPTP journey won’t quite end after this next open heart surgery either – Evanna will have to complete another 4-week antibiotic course post surgery, and so if we experience a decently quick recovery, we will once again come home with HPTP.
While the details are all a little fuzzy, the cardiology team has also decided that it’s time to do another cardiac cath, as Evanna hasn’t had one since May 2015. I doubt they will do it before Evanna’s open heart surgery, but I’m not sure if it’s something they’ll do right after or have us come back up to Edmonton at a later date to complete. Cardiac caths on Evanna are a bit more complicated, as the routes they normally take are clotted, and so they have to go through her liver instead (which is a bit more risky). One of the main reasons they want to do another cardiac cath is to accurately measure the pressures in Evanna’s heart and send those results to Dr. Hanley in California….. which brings me to here: we have ALL of this going on over the next 4-8 weeks, and we still potently have California on the table. Oi

>> you may be wondering that if we're still waiting to hear from Dr. Hanley, why not just wait and possibly hold off on going back up to Edmonton/Stollery and avoid an "extra" open heart surgery, when Dr. Hanley could potentiality replace her prosthetic material and work on her collaterals at the same time? Well, now that Evanna is much more stable compared to 1.5 weeks ago, the surgical team believes the risks of going back into Evanna's chest have come down greatly and if and when we do hear from Dr. Hanley, we could be looking at several months before we could secure a surgery date anyways (he tends to book months and months in advance). It's better that we completely take care of this infection now and get Evanna in "tip top" shape before we potentially send her down to California --hope that makes sense to you. 

By now Nick and I are somewhat use to going with the flow and the odd curveball when it comes to Evanna…. but to say we’re currently a little overwhelmed at the moment would be a slight understatement to say the least. I’m trying to just focus on the current steps directly in front of us (home on HPTP and transfer back up to Edmonton), but I’m a little in disbelief when I think about all what has recently transpired, and everything that’s coming our way within the next 2-6 months.

Just a current glance of “our plate” right now:
– Trached child who is ventilated and on 4L of oxygen 24/7
– GTube feed every 4 hours (0200, 0600, 1000, 1400, 1800 + 2200)
– Manage IV therapy which involves changing and hanging a new bag every 24 hours (and know that this will be something we will have to deal with for at least the next 8-12 wks depending on the timing of everything…)
– Facilitating a HPTP homecare visit every 3 days so they can prime and attach a new line
– Continued juggling of our unique work schedules (full time shift rotation for Nick and casual/part time for me)
– Prepare for another hospital transfer and admission up to Edmonton for open heart surgery (though, at least we have a few weeks to prepare vs a few hours that we’re somewhat use too lol). This includes packing, arranging hotel/Ronald McDonald House, booking off work, informing and canceling any ongoing therapy appointments, etc
– Evanna’s very first dentist appointment (at the hospital) on Monday
– Check-up & ECHO with Cardiology on Tuesday
– Complex Airway Clinic & Infectious Disease blood work appointments within the next few weeks
– Quick personal trip/vacation to Seattle in less than 2 weeks with a friend (not that I’m at all complaining about this) that I hope to still attend and still have to make plans for (welcoming all ideas!)
– RSV shots every 28 days starting at the end of November (so happy that Evanna was approved for yet another year of RSV protection!)
– Resume physical therapy as much as possible before having to press “pause” once again for 4-6wks after Evanna’s OHS (ugh…this one hurts a little as I can’t emphasize just how far behind Evanna already is, and she’s soo darn stubborn when it comes to PT activities to begin with)
– Continue to stay on top of our night nurses schedules (my job), their paychecks (Nick’s job), and make appropriate arrangements with them once we get an Edmonton date.
– Squeeze in an immunology follow-up appointment when we find time (they’ve already called to schedule an appointment, but I had to ask them if I could call them back once we had a better idea of how our December/January will look like).
– Schedule a swallowing test with ENT asap
– Advocate for and book a new bronchoscopy with Respirology + ENT after the holidays

So this is where we currently stand, and we still are missing a few pieces of key information that I hope to gain over the next few weeks. We're so glad that Evanna is completely back to her baseline and at home with us -- even if it's just for a short while. Nick and I could definitely tell she was getting a little bored the past few days before discharge, but as you can see -- she is very happy to be home. Continued prayers + good thoughts would be greatly apprecitated at this time, and I'll update one I know more on both Edmonton and California. 

xo The Irvine Home

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