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.'
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.|
In addition to these symptoms, those afflicted with Still's also face more organ involvement, particularly those involved in the lymph system like the spleen and tonsils. Children with SD also face a much higher rate of disability and disfigurement due to the severity of the disease. However, unlike the poly or oglio forms of JRA, Still's affefcts all genders equally and actually has a lower chance of causing uveitis (about 1%). Still's makes up about 10% of the 300,000 children with JRA.
What do you mean by autoimmune arthritis?
In all forms of autoimmune arthritis (AA), the primary cause of the illness is a malfunction of the immune system. The body controls this system via the lymphatic system which circulates lymph fluid throughout the body similarly to how the circulatory system pumps blood. Instead of using arteries, the lymph system uses lymph nodes and trunks.
|I don't think the system is green though.|
If you have ever gotten a nasty cut or scrape, you have seen your lymph system work in producing scabs. Mmm scabs.
Essentially, the lymph system cleans up the body. At lymph nodes, lymph fluid is filtered and cleaned before it is returned to the blood stream. Nodes are also responsible for the production of lymphocytes which attack toxins and viruses in the body once they reach the nodes. The thymus, located above the heart, also helps in the production of certain lymphocytes known as T cells (those of you familiar with HIV/AIDS might recognize T cells - we'll come back to this later). The spleen also helps in the function of the lymph system, filtering blood similarly to how nodes handle lymph fluid.
In a regularly functioning body, all these pieces of the lymph system help to protect one from illness, infection, and some tiny foreign objects. However, in a body with a malfunctioning immune system, immune response is - as you might expect - crappy to say the least.
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.
This brings me back to HIV/AIDS. In autoimmune arthritis the body has an overactive immune system, going so far as to fight its own body. In HIV/AIDS the immune system starts shutting down causing the white blood cells and T cells to deplete. A slight cold could turn into pneumonia very quickly. If only we could share immune systems! Many of the medications in the collage above actually do try to cause the same kind of immune system damage on a much smaller scale that HIV/AIDS causes as a way to control disease symptoms. And this is why many can experience complications from these drugs - they open us up so much to infections.
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...
Well, of medical care at least :)
Edit: If you're looking for picture of rash, you can find them here. Onset stories can be found here.