June 2012 Update

When we start getting multiple "How are things going?" and "How is Gabrielle doing? We haven't heard for a long time," ... we know that's our cue to update the blog.

Easy for you. Easy for us.

Here's the gist:

Diaphragmatic Hernia
We've learned that Gabrielle has a diaphragmatic hernia. The surgeon thinks – at this point – it is largely benign. In speaking with the surgeon today at BCCH Gabrielle's hernia is wrapped in a membrane allowing it to stay somewhat contained in the chest cavity.  The risk of it getting worse and having the bowel twist and strangle itself is unknown.  They believe that it is not affecting her pulmonary function at this point in time and we have decided that we don't want to put her through any risky procedures.  Amy asked the I-Cell Support Group whether or not they had to go through this with their kids, and several said they had. The ones who choose pre-emptive surgery (to prevent badness later on) found that their kids' need for oxygen significantly decreased, if not ceased entirely. So, while benign for now, if we see that it becomes more of a problem giving her pain, and compromising her lung capacity we may have no choice but to go ahead with the surgery, only time will tell.

Line Removal
We are meeting with the oncologists at BC Children's to discuss the possibility of removing Gabrielle's central line. This is the super-tube that is inserted for chemotherapy and all the meds involved during and after transplant. It starts on her right chest, runs underneath her skin and into her superior vena cava leading into her heart, and it's allowed Gabrielle to avoid needles and bruising from IV for over a year now. We can't bathe her without wrapping her chest in glad wrap, and even then it's a cloth-based shallow bath. So the prospect of removing her line is also very exciting especially for the summer.  Because of her oxygen requirements and her possible need for tubes, her line will need to be taken out in the OR with an anesthetist in case things go wrong.  Usually children have their lines removed in the clinic and is surprisingly quite a simple procedure.

Tubes
We've also learned that our little girl is partially deaf and would greatly benefit from tubes. She can hear loud, low, strong sounds, but not higher / whispery sounds. Luckily the procedure is very quick and only requires sedation.  In talking with some I-Cell parent friends, the added sense of sound that comes with tubes is hugely beneficial for development and social interaction, and well worth doing.

Risk of Intubation

The catch, however, is that the first two procedures require intubation, ventilation and anesthetic (with tubes requiring sedation), and the harsh reality is that fatality is a serious concern when I-Cell kids are intubated due to their small airways and weak muscle tone(again, a big thanks to the community for this information).

So .... (big sigh) ... yeah. Our number one priority for Gabrielle has always been quality of life, and I'm 80% sure we've decided to go for the line removal and the ear tubes, but the invasiveness of the hernia procedure is something we're leaning against.

NG-Tube
Some unexpected good news is that Gabrielle pulled out her ng-tube last week (i.e. yellow feeding tube she's had for months and months now) and has been thriving without it since. This was something she would regularly yank out (since January) with her quick hands, and we would have to get a nurse to re-thread it down her nose, throat, and into her stomach, always ph-testing it to make sure it was placed right so the feeds landed in the right place. Imagine having a tube threaded down your nose that way. Oh Gabrielle. So we are super happy she's shown us she doesn't need ... kind of intentionally too.

The Family

We're hanging in there. Michael and James are just awesome, are developing incredible gentleness and senses of empathy, and keep us smiling, laughing, and moving so much that we haven't been able to go anywhere too dark. Our new au pair Kaitlyn has arrived from Nova Scotia – she's wonderful, a huge help, and has allowed us to catch our breath. In fact, I think we're only just coming to realize how traumatic the past year and a half has really been – a string of exhausting crisis situations that have, at times, put us on the brink ... testing and affecting us in ways we never thought possible, in good ways and bad ways. Actually, it's amazing what large doses of stress, exhaustion, grief, and joy can do to your psyche.

Maybe Freud was right about those defence mechanisms ... denial and regression come to mind for me (LOL), and I know better than to speak for Amy.

But we're doing alright. It feels like we're heading in the right direction. It's amazing how resilient we all are. We got a little hero who shows us every day what living through adversity really means, and how to do it with a smile.