What is it?
Systemic JIA is also known as Still’s Disease. It is one of the least common types of JIA, only affecting as small a portion at ten percent. Like other forms of JIA, Still’s causes arthritis or swelling in the joints. However, it does not just stop there. Because it is systemic, this disease affects organs more commonly than polyarticular or oglioarticular. The most commonly affected organs are those that deal with the lymph system, which is what this disease moves through. The lymph system helps to clean out toxins while delivering nutrients to specific spots like lymph nodes. The spleen, liver, and kidneys all play a part in the system and your tonsils even do too.
One of the most commons signs of Still’s is a salmon-colored rash that presents itself on the skin. This rash usually shows up more in the nighttime, so it can be hard to get Still’s properly diagnosed because by the time a doctor’s visit can be made the rash is gone. The rash can be itchy and often looks like welts from oil burns. Usually, if you scratch it, it grows. Along with the rash come very high fevers. Temperatures of up to 104 degrees are somewhat common and can go even higher. This can be a problem, as fevers with temperatures higher than 107 degrees can actually cause brain damage. Children with Still’s are also more susceptible to deal with stunted growth due to the higher percentages for disfigurement and disability. It is considered the most severe type of juvenile rheumatoid arthritis.
Just like other forms of autoimmune arthritis in children and adults, testing for the disease is more like a ruling out process than finding one test that gives you a diagnosis. For children with Still’s, tests that normally show things like rheumatoid arthritis often come back negative – these include the rheumatoid factor and the c reactive protein tests.
The only drug that has been specifically approved to treat children with Still’s is actemra (tocilizumab). This drug is given through an infusion. However, children with Still’s can be treated with a variety of drugs that are used to treat other forms of autoimmune arthritis, like methotrexate (a chemotherapy drug), Enbrel (which alters your DNA), and many others. Prednisone, a corticosteroid, is often used for a very long time and in high doses for children with Still’s as it seems to be even harder for children with the disease to achieve remission. The quicker children are diagnosed and treated aggressively though, the better chance they have at remission.
It is really important for children with any type of juvenile arthritis to stay somewhat active. Many of our joints, like the knee joints, are what are called self-lubricating. This means that as you move the joint, lubrication is actually made within the joint itself which helps to reduce pain and damage. Swimming and other water exercises are really good for children with Still’s because they help to reduce weight and pressure on the joints while still providing resistance. Physical therapists are great people to help figure out what exercises we need to do, because they can help pinpoint which joints are weaker and can give you specific exercises just for you to help strengthen those. As children with Still’s grow up, they sometimes use the aid of a personal trainer at the gym too. They can do similar things to physical therapists, but focus more on keeping weight down and working on fitness instead of just joints and range of motion.
Children with Still’s often miss more school than normal children. This can be because of doctors’ visits or fatigue and even illness-related stay home days. It makes it harder sometimes for kids to go to school, between keeping up with school work and being picked on by other kids for missing school or even because of the use of assistive devices like wheelchairs or wrist braces. It is really important for parents to work with doctors and teachers to help other kids understand what a child with Still’s goes through. This can help cut down on bullying and teasing, and help teachers be more flexible with school work too. But some kids find it easier to do school online and even more, some parents are able to teach their kids at home. I ended up being taught at home for many years and it helped a lot with my disease – I was able to sleep when I needed and rest a lot. But that also meant I didn’t get to interact well with other people and as a result I do not necessarily have great social skills or a lot of friends.
What is it like to live with it?
I am 24 and I have had Still’s since I was 5 years old – that’s 19 years! At first, we didn’t know it was Still’s though. Doctors could not figure out what it was, partially because they couldn’t see my rash and it was really hard for me to tell them how I was feeling. I didn’t know hurting wasn’t normal because I wasn’t old enough yet. I don’t even remember what normal feels like. It took them close to eight months to figure out what was wrong with me, but for some people it takes years.
Sometimes, it feels like someone has a sword and is trying to pop off my kneecap. That makes it really hard to walk. Other times, I feel so good I can even run. It makes it really hard to plan things with my friends though, because I never know when I will feel okay and when I will feel bad. It also can make it hard for us to stay in shape. Prednisone causes weight gain, which doesn’t help too. One of the tough things with Still’s is that it doesn’t just pick a few joints like some other forms of Juvenile Arthritis. Because if affects the whole body, I might feel too tired to do something because of fatigue or I might be super sick to my stomach or I might be hurting so bad all over that I can’t even get out of bed.
I take ten pills every morning just to help me function. I have to take a couple of those pills another few times throughout the day. Once a week, I do an injection of Enbrel and it is really helping me to feel more normal than I have ever felt. But sometimes I feel pretty sick after the shot and my pills. I have been on a lot of different medications but some of them just make me too sick to do anything but sleep and play video games.
Between the medications and being at the doctor often, I tend to get sick a lot more than normal people do. It can be scary though, because even a little case of the sniffles could turn into something worse like pneumonia. That is part of why it is so important to have a good relationship with the doctor and the office he or she works in. It also makes it so much more important to share how you are feeling with people because you never know if your stomach is mad you ate spicy food or if you are reacting to medications or are getting really sick.