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

Hooray for dragons!

Dragons are elusive 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.’

As of 2023, Still’s Disease is one label that covers what was formerly known as systemic-onset juvenile idiopathic arthritis (SJIA) and adult-onset Still’s Disease (AOSD).

I live with SJIA and have since mid-November 1993. Honestly, I barely remember what it was like to be ‘normal’ but I consider this a gift. I’ve watched so many people be completely blindsided by a diagnosis later on in life after they’ve established running or weight lifting as their favorite activity. Watching 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 autoinflammatory 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’ Adult-Onset Still’s Disease (AOSD) as a classification.

About 95% of children with this condition 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 other forms of JIA, Still’s affects 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 JIA.
What do you mean by autoinflammatory arthritis?
In most forms of arthritis, 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.
If you have ever gotten a nasty cut or scrape, you have seen your lymph system work in producing scabs. Essentially, the lymph system cleans up the body. At lymph nodes, lymph fluid is filtered and cleaned before it is returned to the bloodstream. 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. 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 combination garbage and milkman but very confused. He should have called into work. Instead of leaving healthy milk and removing garbage, this dude gets all turned around. He misses stops, drops off garbage instead of picking it up, and steals all the milk. Here’s the best part – since the lymph system involves bone marrow, blood, lymph fluid, and more, there is no point of refuge from this guy. No spots are left untouched.

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!

Treating this disease

It all sounds kind of easy to explain, but the problem in finding the best, most effective treatment 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 inhibitors to chemotherapy medications.

Mmm bio-hazards.

As of 2020, there are a few medications approved to treat these conditions. There was only one medication actually approved by the FDA to treat Still’s as of 2012 – tocilizumab or Actemra. It is an IL-6 inhibitor given by infusion usually about once a month. Thankfully, there are a few other options. Canakinumab (or Ilaris) is a newer medication that targets the IL-1 beta protein. It’s usually given by injection once a month or so. Anakinra (or Kineret), another IL-1 inhibitor, is given by daily injection.

Some people respond well to other arthritis medications such as Enbrel, Humira, Methotrexate, and others. That’s especially true in combination with one of the above medications.

Some very very lucky people get to experience remission with Still’s. However, the only statistic I have found – specifically 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.

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 have been a couple of small studies done with stem cells in other countries, obviously, due to odd feelings about these cells in the US. 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… died. The process is very involved and difficult for the body to go through.

While I maintain hope that stem cells may hold the answer to kicking this disease’s butt, the odds are just not in our favor right now.

Living with this

I’m not going to sugar coat it and say that living with Still’s is easy or 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 this condition can be manageable.

In 2015, I started Kineret. As of the end of 2023, I’m still on it. It’s done wonders to help control the symptoms I experience. In fact, it’s done so well that I sometimes miss my SJIA symptoms. That’s a head game I never thought I’d play.

The best advice I could give someone with this condition would be to value yourself. It is so so easy with how society treats disabled people for us to devalue ourselves. Ableism is a vital thing to learn about and unpack.

Learn as much as you can about this condition. Figure out what you need and want out of life. Then try to think about how to make that happen. I play hockey and perform burlesque now, two things I never thought would be possible.

July is Juvenile Arthritis Awareness Month!

Ahh, I have had a lot of craziness going on you guys! I’m so busy this summer. It’s great, but it also means my fatigue monster is super strong so I’ve been letting things slip over here. I’m sorry 🙁

But hey! July, huh? Did you know it’s juvenile arthritis awareness month? Well, you do now!

At 24 years of age, I know I’m not a juvenile anymore… at least physically 😉 That still doesn’t change the fact that I have a form of juvenile arthritis. So understandably, this is something that I really support. The word arthritis entered my life before I even had a chance to learn names of bones or even my colors in Japanese. It is very tough to grow up with one of these illnesses and not know how to explain how it feels to others. I still find myself running into this problem, often making up ridiculous (though accurate) descriptions like how it feels sometimes that my bones are filled with gel they use to make ice packs instead and it can’t decide if it wants to be a hot pack or an ice pack. I know how it is to get teased through school, to be bullied because you are different and physically otherwise capable. And I know what it’s like to transition into the grown up world – finding your own healthcare, navigating employment options and collegiate activities. It sucks. But you know what? The other kids that I know that grow up with these diseases too… well, they’re the toughest bunch of kids I know.

The rest of this month, I have some great things to share with you guys. All that time on hiatus hasn’t been exactly spent not thinking of you 🙂  Coming up, I want to focus on children dealing with juvenile arthritis – posts on dealing with schooling, with bullies, etc. But I mostly want to focus on the awkward transitiony stages – transitioning from child care to adult care, navigating insurance on your own, explaining your illness to jobs and professors, etc. I’ll also be posting tips on how to do every day things easier, like dry shampooing instead of hopping in the shower on high disease activity days. And I’m even working on a video! Or maybe a few. Whoa!

For now, I’d like to share some great books and further sources of information on arthritis in case you are interested. These might not just be juvenile centered, but I have found them to be helpful for me.

  • A Primer on Rheumatic Diseases by the Arthritis Foundation – This book covers essentially every disease in the rheumatology field as you might expect. It has some great information on Still’s diseases as well as other forms of arthritis that I didn’t even know but that made so much sense. Very very helpful, but keep a dictionary handy. Plus, um, did you see you can buy it for like a cent used? Seriously, get it.
  • A book on anatomy – Just find a generic classroom copy, preferably one that focuses on the whole body and not just the skeleton. Did you know that your tonsils are a part of your lymph system? I didn’t until I did my anatomy research and now I know why I get sore throats!
  • Out of Joint – This book is about the journey of a young mother who falls ill with rheumatoid arthritis. Her story is not only comforting for its honesty and truth but also inspiring for her strength and character.
  • Partners in Healing – This book can be very helpful for those who need care as much as care givers. It offers care givers a unique peek into our minds though, and helps them to understand what they can and cannot do in order to help us on our journey with chronic illness.
  • This website has a few documents on making the transition from a child to an adult when it comes to healthcare.
I’m going to try to keep things more generic this month instead of focusing so much on Still’s just in light of JA awareness but I will probably have a post specific on Still’s research I’ve done and a comparison between AOSD and SOJRA.