Living with Dexter

I wanted to share a little more in depth what happened following our stay in Toronto, so as I previously mentioned Daniel (and Will and I) were discharged from Sick Kids on February 14th. We returned to work, daycare etc. and Daniel continued on the steroid treatment as well as his regular dose of anakinra to prevent the re occurrence of MAS and whatever the auto inflammatory disorder is that he has.

Things were tough, Daniel would sleep at most… increments of 45 minutes at night due to the fact that steroids cause restlessness and he was constantly hungry, which is another side effect of steroids, there is nothing you can do to satiate your hunger as the medication gives you the illusion you are always starving. He was also incredibly moody, we used to try and make light of it by saying things like “he’s turning into a little hulk” the doctors nicknamed his alter ego Dexter (the angry roid rage version of Daniel) Dexter would fight, and hit and kick and bite, when he didn’t get his way and grit his teeth so hard and try not to scream in frustration that we thought eventually he might just turn green (like the hulk).

Steroids are awful!! and even worse for children! Because you can’t explain to them what side effects are and why they feel as awful as they do.

And if the alternative wasn’t organ failure or death from a blood disorder we would never ever want him on them again. But they were keeping him safe and so we needed to tolerate Dexter – I remember I cried and still sometimes do that “this is not my sweet, beautiful, smiling, happy little boy.” and man I missed him. But he was still in there, We would have moments in the day when Daniel would reappear and I would cling to him in those brief moments and soak up as many ’tisses’ and hugs as I could.

A few weeks went by and things began to progress well…we had regular follow up visits to Toronto and were given a fever card by Victoria Hospital Pediatric Oncology department and follow ups in London with our new doctor; Dr. Paul Gibson.

So you may be wondering why are we visiting Oncology? Oncology isn’t that Cancer? and yes…yes it is, however something you may not know about Oncologists is that not only do they specialize in Cancer but they also specialize in Hematology: definition – the study of the physiology of the blood.

Dr. Gibson is one of the only doctors in London qualified and knowledgeable enough about MAS to handle Daniel’s case, our method of emergency was that if Daniel developed a fever we would need to contact the on call pediatric oncologist and let them know, and they would refer us to seek immediate attention at Emergency – they would call ahead let Emergency know we were coming and Daniel was to be put on immediate antibiotics, steroids and blood tests were to be done right away.

Ok, so I was side tracked, things were going well. Follow up blood work was looking well, and they slowly began to ween Daniel off the steroids, we were beginning to have a few more days with Daniel and time with Dexter was becoming a lot less frequent. We were feeling like we could breathe a little bit.

Until March…I received a phone call at work from Daniel’s daycare – he had a fever. We monitored him for one hour the fever didn’t budge, I immediately started the process. I went to pick him up right away, called Will, who called Dr. Gibson, Dr. Gibson called Emergency, Daniel and I went straight to emergency and Will met us there.

Thankfully with Dr. Gibson’s referral and our fever card we were immediately whisked to an isolation room where blood work was drawn and steroids were amped up again, (Keep in mind he was still on steroids) Daniel was put back on IV’s at Vic and we were sent upstairs to bunker down on pediatrics Victoria Hospital Sick Kids for a few more days. Doctors who saw us had there hands tied, as everything that was done for Daniel needed to be consulted by Toronto and Dr. Schneider, we were carefully monitored and another million tests were done. The final verdict! Daniel had…viral pneumonia. Daniel was still on steroids so he didn’t seem to have MAS reoccur as a result of the pneumonia but they couldn’t be sure, so they needed to take precautions. We went back on higher doses of steroids and after a few good weeks we were back to living with Dexter, we were discharged from hospital after 48 hours and the sleepless nights continued…

Thankfully though other than this little hiccup in March things eventually slowed a bit and we were able to move Daniel off the steroids in April, we had a lot of follow up visits in Toronto and frequent blood work but things were starting to look better.

In one of our visits in Toronto, Dr. Schneider shared the results of some of our blood work, Daniel was officially confirmed to have auto inflammatory disorder, and the blood work testing for CAPS disease came back.

Reminder~ CAPS disease or (Cryopyrin-Associated Periodic Syndromes) is the most common of auto inflammatory disorders.

The results were negative…

So what does that mean? it means our sons blood work showed he has an genetically produced auto inflammatory disorder that is treatable by the medication that he is on that is not CAPS, (something that is quite rare but still the most common form of auto inflammatory that doctors are familiar with) Dr. Schneider was not completely shocked since Daniel had also been suffering with MAS as a complication and Rheumatologists have NEVER seen someone with CAPS also have MAS.

Dr. Schneider explained that he had a colleague in the US who was recently researching a brand new strand of auto inflammatory disorder that was in fact associated with MAS, he wanted to be able to send a blood sample from Daniel to find out if maybe this was the blood his friend was looking for, the test they were doing was to find a genetic mutation of a gene NLRC4 (NLRC4 gene is a gene everyone has! they just wanted to see if something was wrong with his)

So…if you aren’t completely confused by all these letters and numbers and talk about genetics and DNA and everything else than awesome! I know I was, Will was able to understand alot of this faster than I was so don’t worry. Basically just understand this, Daniel’s blood was being sent to the states to see if his particular genetic mutation was a brand new strand of a disease a researcher was working on, that hasn’t been discovered yet. Ground breaking medical research here folks, haha…

I mean we always knew Daniel was one in a million – now we have genetic testing to prove it!

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