I have to be completely clear about something, because for many according to my last post all you heard was the negative side of our Canadian health care system but let me be perfectly clear, the doctors and nurses and staff at Toronto Sick Kids are exceptional! They have dedicated countless hours not just to make sure Daniel was safe and well looked after but to make sure we had the answers we needed and the understanding required to make us feel safe.
We were admitted and had quite a stay – Daniel was placed on IV’s and in our first day must have seen a team of at least 50 doctors, rheumatologists, hematologists, dermatologists, pediatricians, and neurologist was of the first to see Daniel.
Daniel underwent a skin biopsy, multiple MRI’s, bone marrow aspirate, and daily blood work (so much blood work that we were concerned he may need a blood transfusion) Daniel received something called bravery beads for each procedure he underwent, and man was he a trooper!
The worst part of each day was watching him struggle through each procedure. He was still facing fever everyday, and the doctors confirmed that the pain in his limbs was a result of inflammation causing juvenile rheumatoid arthritis.
Here we met Dr. Rayfel Schneider, who has been the most amazing doctor both for us and Daniel, he is so caring and considerate of Daniel, and has been a wonderful teacher for both Will and I as we try and get a handle on all this. He suggested that Daniel did in fact have what looked like an auto inflammatory disorder and that to him the symptoms pointed towards something called CAPS disease or Cryopyrin-Associated Periodic Syndromes. The rash, fever, juvenile arthritis, all fit the symptoms of this disease. He explained that if this continued to go untreated next occurring symptoms may include hearing loss and eventually neurological damage. So they performed a hearing test and MRI to make sure Daniel’s condition had not spread that far. Daniel did appear to have some hearing loss in his left ear, but that may have been a result of inflammation because he had a fever for creeping up to 30 days.
The scariest moment for us, and what appeared to be the most confusing for Doctor Schneider and his team, was when Daniel’s blood work came back with signs of Macrophage Activation Syndrome (MAS) now I know your probably all frantically googling trying to figure out what the heck that means…I say that because that’s exactly what we did. Especially because when Dr, Scheider told us about this he was very concerned, and used phrases such as “we need to act quickly to get this under control” and “we have to have a healthy respect for this syndrome” and “we have to get ahead of this”
So after this conversation with the doctor, Will and I did what every good parent does and goes to the internet to research!
We began reading things like “Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic disease that, for unknown reasons, occurs much more frequently in individuals with systemic juvenile idiopathic arthritis (SJIA) and in those with adult-onset Still disease.” http://emedicine.medscape.com/
Macrophage activation syndrome (MAS) is a severe, frequently fatal complication of systemic juvenile idiopathic arthritis (sJIA) with features of hemophagocytosis leading to coagulopathy, pancytopenia, and liver and central nervous system dysfunction. http://adc.bmj.com/content/85/5/421.full
It was time to talk more in depth with the doctors and find out what our future looked like, we were/are terrified and this MAS is serious threat to our son’s life. Basically, the first thing you should know is – stop googling, it will only do for you what it did for us and that’s scare us senseless. For us we turned to prayer, and many many others prayed with us. It was and is our only source of strength. Have hope, everything we had faced up till now had led us here, at this place (Sick Kids) with Daniel – he only began to have this complication while he was in the care of Toronto Sick Kids. The doctors explained that although his numbers were bad they felt they could get ahead of it and treat him with steroids, they just couldn’t wait. They explained in most cases by the time the child with MAS is seen by them they give them the steroids and can only cross there fingers it would work where as with Daniel they felt confident that his was a case they may be able to get in front of it. The way they explained it is like this, we all have little garbage men in our blood, they clean out the bad stuff that keeps us from getting ill. Well, when MAS occurs those little garbage men turn into repo men and start destroying everything, if not treated in time can cause damage to major organs and potentially organ failure resulting in death.
Right away the team at Sick Kids started Daniel on steroids and a daily injection for his auto inflammatory disorder (whatever that may be). The frustrating thing for the doctors was this MAS through a monkey wrench in there potential diagnosis because they haven’t seen MAS associated with CAPS disease before. It wasn’t a complication that fit the bill.
So now what? This is why Daniel had a bone-marrow aspirate: to confirm that he did in fact have MAS, to rule out that this MAS was not on-going and that it was only a complication. And they also needed to rule out that he did not have leukemia. When the bone-marrow aspirate came back the doctors confirmed, that he DOES NOT have leukemia. I am sure you can only imagine our relief – but that they would have to send away for tests regarding ongoing MAS, they explained that IF it came back positive Daniel may potentially need to have a bone marrow transplant in the future.
We started asking questions about what that would look like and how would we find a match? would we need to be tested? this sort of thing. So many questions and not a lot of answers.