Hooray for dragons!
Dragons are illusive creatures, but when you find one you have to really put your all into the fight. It’s no wonder why Still’s Disease is also referred to as ‘the dragon.’
There are two forms of Still’s disease – systemic onset juvenile idiopathic arthritis (SJIA) or adult onset Still’s disease (AOSD). Both are considered fairly rare so it can be pretty hard to get an accurate diagnosis.
I live with SJIA, and in November I will have been doing so for 19 years. I barely remember what it was like to be ‘normal’ but I consider this a gift – why? I have watched so many people be completely blind sided by a diagnosis later on in life, after they’ve established running or weight lifting as their favorite activity. To watch them struggle with learning their new limits just breaks my heart. I didn’t have to go through that falling ill at 5 years old – I learned my limits while I learned how to do normal things. My limitations, for the most part, are about what they have always been. I am very lucky.
So what is Still’s anyway?
SD is a form of autoimmune arthritis that is named after a pediatrician, Sir George Frederic Still. His doctoral thesis was actually about Still’s, and he was the first to describe the phenomenon. He noted that the children afflicted with the disease not only presented with arthritis, but also high grade fevers and a funky rash. Over the years, people starting also noticing these symptoms in adults, thus ‘creating’ AOSD as a classification.
About 95% of children with SD present with the rash but only 85% of adults do. The rash can itch, or it can just be there. It also seems to present more with the fevers, though the rash can be brought about manually – by clothes, rubbing against things, and even hot temperatures and water (i.e., showers).
|This wasn’t post shower, but you get the idea.|
|I don’t think the system is green though.|
Instead of filtering and removing toxins, the system instead can introduce even more crap at any given point into the system. Think of it like a combo garbage and milk man but on crack. Instead of leaving healthy milk and removing garbage, this wasted dude gets all turned around. He misses stops, drops off garbage instead of picking it up, and steals all the milk. Ass. Here’s the best part – since the lymph system involves bone marrow, blood, lymph fluid and more, there is no point of refuge from the crazy garbage man. No spots are left untouched by AA in a general sense.
Your body keeps getting these messages that something is wrong, and sends T cells and white blood cells out to protect and serve. They’re on like super secret protection status, like a super protective parent. Anything that a normal person might do – like running for example – is seen as a huge problem. Your immune system says “Whoaaa, hold up there! We got an invasion on our hands!” and calls for reinforcements to protect your knee… but then they get there with all the troops and the only thing there to attack is the knee itself. Swelling leads to immobility of the knee, which can be painful because then you rest the knee not realizing that the knee is a self-lubricating joint – resting too much then makes it hurt more. Voila, welcome to joint damage!
It all sounds kind of easy to explain, but the problem in finding a cure lies in what causes the immune system to freak out. And this brings us the different classes of drugs we have to treat different kinds of arthritis, from TNF alpha inhibitors to interleukin 6 inhibitors to chemotherapy medications.
Side note: I need to get some mice and a research lab stat.
There is only one medication actually approved by the FDA to treat Still’s right now and that is tocilizumab, or Actemra. It is an IL-6 inhibitor given by infusion usually about once a month. However, because SD is a form of autoimmune arthritis it can be treated with other medications like enbrel, methotrexate, humira, etc.
Some very very lucky people get to experience remission with Still’s. However, the only statistic I have found – for AOSD – isn’t very comforting. Only about 20% are expected to reach remission. Even then, once you get there, you might not keep that for very long.
|Seriously you guys? Seriously.|
There are a number of theories for how to make a cure for autoimmune arthritis diseases, probably the most common one involves the use of stem cell therapy. There has been at least one study done with stem cells – in some other country obviously, thanks to crazy right-wing stem cell hating in the US (every new study on SC keeps getting shut down). A large percentage of people experienced a flare up and worse disease from the process while a slightly smaller number did achieve eased disease activity – for how long is yet to be seen. A very small portion actually… well, they died. The process is very involved and difficult for the body to go through. While I maintain hope that stem cells will hold the answer to a cure, the odds are just not in my favor right now.
I’m not going to sugar coat it and say that living with Still’s is easy or super awesome because it is entirely too far from the truth. With the right mix of medications, physical therapy, and a great support system living with SD can be manageable.
The best advice I could give someone with SD would be to value yourself. It is so so easy with how society treats the disabled – and the invisibly disabled is even worse, holy crap – for us to devalue ourselves. We clearly deserve our ‘fake’ illness. But you are worth so so so much more than you might think.
Next time I’ll be writing about the importance of assembling the…