Losing track of time

I’m at the point now where I can’t remember how long we have been in. Five weeks. All the Christmas holidays. 

I’m supposed to be returning to school this week. 

Chloe is supposed to be out and healthy and returning to daycare. 

We are supposed to be setting up our new house and beginning 2017 on a high. 

Well that ship has well and truly sailed. 

Chloe is not out. She’s not ‘healthy’. We are not sleeping in our new house (although we have moved and thanks to some great friends and family everything is at the new house ready for us). 

I am not returning to work yet. But in some good news leave has been approved and I still have a contract. 

It’s been an emotionally draining weekend. A new rotation of cardiologist came on last Friday. He seemed upset that we were simply ‘waiting’ and no one had taken action. I’m not sure how this was deemed my fault. 

In fact he marched in the room and talked with his colleagues without addressing me. We finally had a short talk and he said he remembered me from my prenatal scans. He then asked me how old Chloe is (shouldn’t he know this from her chart?) and I responded with four. 

He followed that with asking why she had the fontan so early. I was shocked and exasperated at this point. I certainly didn’t turn up at emergency and demand she have a fontan. 

He then refused to give Chloe FFP. FFP is something we’ve been asking about giving Chloe for a while. This is sometimes given to children with SLOS to help with recovery.  It’s frozen fresh plasma. We had had a lot of discussion over this for several days and had given one single dose on Thursday following Chloe diagnostic catheter she had Wednesday. Our cardiologist and the one on rotation that week were happy to try it. Sadly the new rotation of doctor was vehemently opposed. Chloe had gone back in to theatre on Wednesday for another catheter. 

The catheter was to see if there was any reason for the consistency chyle leak she’s experiencing. They couldn’t find a reason. It was an awful day. She woke in excruciating pain from the anaesthetic and the whole day was a write off. 

Frozen fresh plasma! Looks like pee!

Her left drain site was redressed and sutured during the procedure and was sticking out from her side. She was in a lot of pain and spiked a fever. 

Drain site! Sorry if you’re squeamish!

The catheter found that Chloe’s fontan circulation was working beautifully and there were no obvious reasons for the continued leak. This is good news but also bad news. If a reason was found then it could have been addressed. 

So on the Saturday the cardiologist walks in after seeing that Chloe had ‘dumped’ over 400ml of chyle fluid in the first hours since the catheter and then another 200ml following that.  He seemed irritated again. In fact he opened with an accusation of whether I was deviating from the low fat diet she is on. Chloe is only allowed 10g of fat a day. I responded that I do not like to be accused of not following medical advice, so obviously this doesn’t go down well. 

Again he is surprised that her chyle leak is still milky and not improving. He blamed the dose of FFP and is refusing to continue that. Again I try to ask about what we are going to do and he quite directly says to me that he warned us during my pregnancy that these complications happen with fontan and that is why is counselled us with ‘options’. 

I was shocked by this. He is in such a frenzy talking and making his point that he marches over to Chloe and gets into her space. He is now aware that her drain is sitting right there and stands on it. The entire room gasps as he quickly checks he hasn’t dislodged it. Chloe is now hitting and swatting him. 

And I think, “fire away daughter. Hit him good!’

He then disagrees with me about treating Chloe as a whole picture. I try to tell him she has a syndrome that affects her body. Her ability to repair tissue and build cell growth is impaired. He informs me he is only concerned about Chloe’s fontan and that is what he is treating. I reply that you can’t do that. He tells me that SLOS doctors in the USA (who I have emailed prior and our cardiologist is aware of) don’t know anything about fontan. 

To which I bluntly reply, ‘well you don’t know anything about SLOS. Chloe is the only child in the entire world that I know of who has SLOS and a fontan! All other slos patients with her heart defect died very young. So we’re all flying blind don’t you think!’

So as you can imagine this doctors’ rounds did not go well. The rest of the day was pretty much a write off while Mike battled it out with a nurse who didn’t speak too kindly of me and then refused to look after Chloe. Between the nurses and the changes of the doctors there had been much miscommunication and mike and I were being told different stories all the time of what was going to happen with treatment. 

Finally a resolution of all the miscommunication came on Sunday. Our awesome surgeon has stepped in as our ‘point guy’! He understands that we must also treat Chloe’s syndrome and wants her back on her cholesterol asap. He acknowledges that her cholesterol is dropping due to the leak and wants our Metabolic team to start consulting as well. He wants to follow conventional treatment unless we don’t get answers from it. He’s wanting to make sure Chloe is getting better as fast as she can. He spends a long time talking to us and understands where we are coming from. And that of course we want to get out of here too and resume normal life. 

Which brings me to her next treatment. This will involve heading back to theatre tomorrow or Tuesday and placing a PICC line. This is essentially a super long cannula (in laymans terms) that can remain in place for a long time. It is used to give TPN. Total parenteral nutrition. Chloe will no longer be able to ingest solid food removing all fats from her diet. Fats are a part of the chyle leak. If we start this we are all hoping it’s the answer to getting her better. I certainly hope it will as ensuring another general anaesthetic is not fun for her or us. Not to mention her not being to able eat (even Chloe with her feeding struggles will find this hard. Even the other day she kept telling daddy she wanted some cheese. She’s not allowed cheese.  It’s too fatty. Well finally she tapped him quite hard and said forcefully, ‘listen, cheese, yum!’  And rubbed her belly!)

And so if you’ve got this far in our update you can see that our prolonged stay is becoming more complicated. Chloe has to beat this thing. I’m told we’re now dropping into the 20% of fontan patients who drain for more than four weeks and develop complications. I don’t want to be in this statistic!!!! What I want is an amazing miracle where the draining stops before we head into theatre and everyone is amazed!!! How about we pray for that one!

Still other miracles are happening. Chloe’s speech is coming along in leaps and bounds. She certainly can assert herself when she needs to. In fact she’ll often walk the ward telling anyone who looks like a nurse or doctor ‘no!’ Accompanied with a fairly impressive death stare. 

Tonight while eating shepherds pie mince ( who knew she even liked that?) and following that up with a sandwich (all super low fat of course) she wanted more ham.  She very clearly said, ‘I’m hungry!’ Great timing Chloe. Not only do you find these words now and find your hunger, but you do it right before we tackle a stint of TPN! 

So as we head into this week I have all my fingers and toes crossed (as my best friend would have said) that she’ll surprise all the doctors and stop draining before we need that PICC line! And TPN. 

Hey, I can dream can’t I!?

Chloe carries her own drain now!