In April we went to our routine monthly GI Clinic visit. I say monthly, however, it had been a while since Isla let us go a whole month without a hospital visit. They were becoming more and more frequent due to her condition worsening by the week. The GI team had been phenomenal with handling our worries and they were becoming very close to our little lady. Thankfully they had always taken Katie and my concerns seriously probably because only one out of the many calls to the IWK Switchboard had turned out to be a false alarm in the last six months. We had been to the hospital for ascites (fluid buildup in the abdomen), for a fever, and many surprise blood draws. Isla was continually progressing in her yellowy-olive tan (probably should ease off on the Banana Boat tanner). She was vomiting more due to the disgusting medications she was on (for example one tasted like a handful of salt and was to be given three times a day). I was getting to the point of spending the majority of my day administering medications, cleaning up her puke, changing her clothes, and consoling her. I began to derive a strategy to limit the puking:

1. Give medications right BEFORE eating (not during or after or you're just asking for it).
2. Any medications kept refrigerated needed to be brought to room temperature first (little miss Isla wasn’t a fan of the cold liquids).
3. Medications given during naps were more effective. She was actually very good at lapping them back while sleeping (let’s hope this doesn’t develop into a sleep walking feeding frenzy later in life).

We had tried everything and came out with many fine tuned strategies (I’m not going to list them all off so all you fellow BA baby parents don’t hesitate to ever ask me what we have found works. We have had a lot of practice now and never shy away from any challenges).

The Visit

Back to the GI clinic visit. Our lovely, pleasant, laid back baby was beginning to show signs of her illness. Until now, if she wasn’t yellow, you would not have known she was a sick kid. We have always said how fortunate we were to have a calm and happy baby however I strongly believe that how you act as a parent plays a big role in how your child acts as well. Katie and I have handled all of Isla's fussiness with a calm demeanor and we have never shied at bringing her around lots of people. So far so good! However, the shittiest part of Biliary Atresia is that at some point you will begin to see your child degrade in health/personality and the only fix is transplant. All of the medications, blood work, clinic visits, etc are just patchwork for this rare (little understood) disease. We were told when Isla was diagnosed, at a month and a half old, that the surgery she underwent was mainly to tie her over until her body was bigger and more receptive to a transplant. For all of these reasons, we issued a sigh of relief when Dr. R told us that we would be talking more about transplant in the next couple of weeks to months. Katie and I even noticed on that clinic visit that Isla’s chart read:

“failed Kasai”

“splenomegaly (swelling of the spleen)”

“portal hypertension (high pressure in the portal vein)”

“hypocalcemia (calcium deficiency)”

And many other deficiencies in vitamins and minerals, which would otherwise be fine if her body was releasing bile properly into her gut. Katie and I could tell, qualitatively, that her health was deteriorating, but that chart put it into perspective for us. The thing is, even though transplant is a huge surgery and very risky, it was a fix to her incurable disease. We would quickly enter a fairly normal life and more importantly, I would not have to watch my pride and joy lose her sparkling personality. I would not have to watch her in pain nor feel like crap any longer. I am sure we will have our moments of illness throughout her life, but it would no longer be an expected future outcome for her. As soon as Dr. R had mentioned that her health was getting worse, I immediately became anxious for transplant to happen sooner rather than later. The rest of that clinic visit was pretty routine and a blur. We met with the social worker, we met with the nutritionist, the GI Nurses assessed her, and Dr. R came in and poked around her belly. You can pump all the nutritional supplements and medications into her but if her body won't absorb it into her bloodstream then it's just a continuous up hill battle to which you keep rolling back down.

IWK Admission: Round Four

You know how I mentioned that Isla had never let us get through a whole month without visiting the hospital? Well guess what...

It was about three days after the clinic visit, I brought Isla to see the family doctor for her 6 month vaccines (yes we vaccinate and no she doesn’t have autism. Don't be stupid people and vaccinate your children. Something we can no longer benefit from; not by choice). Anyways, this was the first time we had seen our GP since Isla was diagnosed with Biliary Atresia and the poor woman felt horrible. Isla had her shots, the scattered nurse left me in the room for 30 minutes (aka forgot about us), I said “fuck it,” and we went home. However during the visit the GP noticed some concerning soft spots on the back of Isla’s head (fontanels) which should have been closed at 6 months of age. She referred us to the local pediatrician to have a more thorough exam (the same doctor who referred us to IWK for BA). This visit was a week later and during that time her jaundice started to take on an almost brown hue and her lovely ascites returned. We went over everything with the pediatrician, he was also worried about the soft spots as well as the ascites, so he referred us to the IWK. He even stayed well passed his working hours to make sure the doctors at IWK understood the severity of his concerns. The next day we were “on the road again” to get admitted back on the pediatric unit at the IWK.

No one really knew what to do about the soft spots, but they were all fairly certain that it was due to her underdeveloped bones. This was caused by her severe vitamin D deficiency, which would later be diagnosed as Rickets before getting discharged. Their main concern was the worsening jaundice and returned ascites to which they treated with IV diuretics again (make you pee drugs). They worked right away and as soon as Isla started feeling relief from the abdominal pressure, she began to show signs of her silly old self again. It wasn’t long before we were discharged (just under a week), but this time it was on a different note. Before leaving the hospital the doctor on call, did not want to make a decision for our GI doctor, but made it very clear that due to Isla’s returned ascites and malnutrition that she would be moving towards transplant as soon as possible. She was correct. Dr. R agreed and we met back at the GI clinic early the following week. There wasn't much more to learn from the GI team this trip. It was mainly just to confirm our trip to Toronto. The bulk of the visit was to go over all of the financials and technicalities involved with our stay in Toronto. We were planning to leave at the end of the week to start all of Isla's testing that coming Monday (thankfully, things move very quickly once the decision to get assessed in Toronto has been decided). This trip would have us meet with the experts in pediatric liver transplant to determine how urgently Isla would need one.

Bring it On!

Onto another adventure; Isla's first flight. At 7 months old this child had more life experience than some adults I know. I will let you know how that went on the next post... to be continued!