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still in a place of anger and even a little jaded when it comes to the “cards we’ve been dealt”, and so I’ve sort of withdrawn myself from the online medical community – except the select few that are more specific to Evanna’s diagnoses.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


  1. The fever shows up 7-10 days after the MMR. I am a nurse and forgot that with all 3 of my boys and wondered why they had a fever but had no other sx of illness.

    1. You're completely right. I wrote this post over the weekend (basically about 4-5 days after the MMR shot), but we have definitely noticed a change the last few days. Nothing too major, but definitely a small spike in temperature, respiratory rate and heart rate --- but minus being a tad on the grumpy side, she is still tolerating it like a champ. Thanks for the tidbit of information and making my mama heart relax a little. Body is busy building antibodies!

  2. Prayers for you all..as the journey continues...she has so much to teach you..and the world...Shoot for those Supa stars...I respect hearing from your Momma heart.. :)Prayers, prayers, prayers!!

  3. We are so happy to see how wonderful Evanna has been doing!! Our whole family will be praying for your upcoming journey and that God directs all the doctors down the paths that are his ways to take for Evanna. Praying that he comforts and gives you strength if you do head down some of these uncertain road and that it all goes well.

    We are from Michigan and have used the pulmonary department at DeVos Children's hospital and at the Motts Children hospital very frequently. I'm assuming it would be one of these 2 hospitals since they are the best in the state. Both of them will leave you in very good hands if you do get approved for the Michigan surgery.

    1. The surgery we're hoping to be eligible for is done out of Mott's Children's Hospital at the U of M. Dr. Green & Dr. Hollister are the men who developed and created this surgery, and would love to see this wish become a reality! xo
      Thanks for the continued prayers!

  4. I can sympathize with many of the feelings you described about blogging/sharing and being connected with the medical family community. We are also following the 3D printed stent research to see if it'll be a possibility for us in a few years. I'll be praying for y'all as you seek these two procedures.

    1. Not sure if you'll read back here, but if you do -- have you guys tried pursuing it at all? Just curious about other families who have looked into it to gage the chance we have of being accepted as a candidate.

      Thank you!