Thursday, July 12, 2012

So you have a dragon: living with Still's Disease


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.


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.

Wait, ewww.

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.


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.

Mmm bio-hazards.
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.


15 comments:

  1. Great read! There's always something new to learn about Still's. I know what you mean about it being easier starting with this illness young. Although I did have a period of 'remission' during childhood, where I was actually quite sporty, the Still's was always lurking in the background and I was already used to all the hospitals, tests and so on when it hit again.

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  2. I was watching Mystery Diagnosis today and one woman's case was about adult onset Still's Disease. I thought of you immediately because of the rarity of the disease be it adolescent onset like you, or adult onset.

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    1. Ha! I do believe that I have seen that episode. I swear, if we had more material on my health at that point I could be one of those episodes. So crazy.

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    2. Do you know what episode it was?

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    3. I'm unsure of the Mystery Diagnosis epi but there was a Mystery ER epi that featured AOSD: http://www.imdb.com/title/tt1270379/plotsummary?ref_=tt_ov_pl

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  3. Hey - lovely blog and thanks for doing it. I just got officially diagnosed with AOSD, although my rheumatologist has been pretty sure for 2 years and I've been sick for almost 5 years. I'm hoping to get ins approval for Ilaris, but expect a row. Best to all my fellow special people!

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  4. Kirsten,
    When I read this quote I had the thought that aside from a few details, it was something I could have written. I have said similar things in the past and fully agree with you.
    "I live with SJRA, 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."

    For me, it was polyarticular seronegative JRA and in Sept it'll be 28 yrs. I was 7. But the rest of what you wrote sounds like what I've said. I feel I am lucky because I was diagnosed so young. I'd not had my heart set on a career that I'd end up not doing. Being so young, life is full of change and adapting, so adapting to JRA was just another thing to adapt to. Despite JRA being the more dangerous of my dx'es because of it's systemic nature and the fact that JRA/RA shortens life spans etc., it is the dx that I have an easier time dealing with. I honestly believe it is because I grew up with it, and to me, it's just a normal part of my life. It rarely causes something new to be concerned about. It's my status quo. I don't really remember what it is to be pain free. In fact, the day I recall being one of my least pain days was the day I gave birth to my son. Most women regard the day they gave birth as painful. For me, it was the first time I'd had pain meds in 2 months. To go from horrible body-wide pain to having pain medication in me was a major change and made the labor and delivery one of those things that compared to my daily pain it was tolerable. And for me, from induction to delivery it was only 6 hrs, with only 3 hrs of contractions that really registered and about 45 min of hard labor. Most women give me the "I hate you." comment when they hear my labor experience. But that was some pain in a fairly localized area and I am used to dealing with body-wide pain that can be severe at times despite medications.

    I have had people mistake my comment that in my own experience it is easier to be dx'd as a child than an adult. They mistake that for me wishing a horrible disease on children. That's far from it. I'd not wish any form of autoimmune arthritis on my worst enemy, let alone an innocent child. But, it is easier to deal with as a child vs an adult. Comparing it to a dragon makes perfect sense!

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  5. Hello dear, I just saw your video tonight on u tube and admire your site and the awareness you are sharing with others.
    My nickname is Melly. My onset was in 1977 at the age of 19 with AOSD. I am the artist for the dragon shown here in red that we use on the International Still's Disease Foundation site. I have been in remission for a few years now and am doing very well. I want to let anyone that is able to know that Dr. Cush is speaking at our conference in Las Vegas at the Orleans hotel on September 3rd this coming Monday. Thank you for all that you do to help others. We are fighters and shall stay strong as a family. Write to me any time. My address is at our web site. Night with love and smiles to all, Melly

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  6. Hi,
    My name is Rachel and I am a thirteen year old living with polyarticular juvenile rheumatoid arthritis. I just wanted to say thank you for this post, because it helped me to further understand the differences between systemic JA and polyarticular/oligoarticular. I love your blog!

    - Rachel
    www.thekidwitharthritis.blogspot.com

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  7. Hi,
    I first read your blog, I'm from Portugal and I started today taking Embrel.
    His story seems my.
    Really there is nothing better than to be optimistic.
    Congratulations on the blog
    António Ricarte (45 years, have the disease since 12)

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  8. Thank you for all the great information. My name is Kayla and I am 27 years old. I was diagnosed with stills disease 3 months ago and am having a really hard time with it. I thought I was a strong person who could tolerate pain until I came in contact with this disease. I am currently on prednisone and have been for months with no much relief. The prednisone causes me to be irritable and having a lot of trouble sleeping. My doctor has tried to start me on daily injections but due to the cost of these they are not a possibility at this time. I appreciate reading your blog and knowing that there is hope..

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    1. Hi Kayla,
      Depending on your insurance, you may be able to get some of the injectables for free from the companies. I was on Humira for a while, and got it free from them. Enbrel and some of the other drugs have similar patient assistance programs so definitely look into those! I believe that Kineret (the daily injection) does too, though I know it doesn't lower the cost enough to help many. Things will improve and you will learn how to manage life with this disease I promise <3

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  9. Hello! I've just started reading through your blog, but I already want to tell you that you are awesome. I just got diagnosed a few months ago. I didn't get sick till my early 30s, and I was working in a very physical career (which I loved and can't do anymore) so your comment about the "benefits" of early onset was very interesting to me. But in general, you've done a really great job at explaining the confusing medical stuff with a good dose of humor and humanizing it. Thank you. :)

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    1. Hey Doc!
      I'm sorry that you're unable to work in your past career :( Please feel free to email me if you like at kirsten -at- notstandingstillsdisease -dot- com. I like to hear stories that I can pass on in my activism.
      Thanks so much <3

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  10. Hi, Kirsten,
    You've written the best--and most understandable to us regular folk--description of what autoimmune means I've ever read. I hope you don't mind if I quote you and link back to you in the future as I write on my own blog. I don't have Stills, I have RA (or RD, for Rheumatoid Disease), but I've thought of it as a dragon gnawing, biting, and attacking my joints almost from the first. From what you wrote here, it sounds like Still's is aptly named.

    Thanks for writing such a great description of Stills, autoimmune diseases, and the many ways each of us adjust to living well with them. Your advocacy is shining. :o)

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