What is This Noise All About Anyway?: JRA, Still’s Disease, and Other Fun Stuff
The type of Rheumatoid Arthritis that I have is called Systemic Onset JRA, or Still’s Disease after Brit George Still. Systemic means that the arthritis not only affects my joints but the rest of my body as well. There isn’t a known cause of the disease, but there are a few ideas. The “onset” part of the disease means that an event occurs that triggers a hidden autoimmune disease. This is the most commonly accepted explanation. For example, I had Strep Throat shortly before the symptoms of the disease starting appearing when I was about five. Nowadays, they’ve come up with yet another term for JRA - Juvenile Idiopathic Arthritis (JIA). This is meant to differentiate JRA from Rheumatoid Arthritis.
There are three major types of JRA:
1. Oligoarticular JRA, which affects four or fewer joints. Symptoms include pain, stiffness, or swelling in those specific joints. The knee and wrist joints are the most commonly affected. An inflammation of the iris (the colored area of the eye) may occur with or without active joint symptoms. This inflammation, called iridocyclitis, iritis, or uveitis, can be detected early by an ophthalmologist.
2. Polyarticular arthritis, which affects more girls than boys. Symptoms include swelling or pain in five or more joints. The small joints of the hands are affected as well as the weight-bearing joints such as the knees, hips, ankles, feet, and neck. In addition, a low-grade fever may appear, as well as bumps or nodules on the body on areas subjected to pressure from sitting or leaning.
3. Systemic JRA, which affects the whole body. Symptoms include high fevers that often increase in the evenings and then may suddenly drop to normal. During the onset of fever, the child may feel very ill, appear pale, or develop a rash. The rash may suddenly disappear and then quickly appear again. The spleen and lymph nodes may also become enlarged. Eventually many of the body’s joints are affected by swelling, pain, and stiffness.
About one in 1000 children develops one of the above types of arthritis. Out of those, only about 10% are affected by the systemic variety.
How D’ya Feel?: Symptoms of Still’s
I know that there must be diseases out there where the pain is worse, even more constant. It saddens me to think about that. I can’t truthfully explain the pains that I go through every day, because this pain is really all I know. I don’t know what it is like to be a ‘normal’ person, someone who can be overly active and not suffer greatly for it.
The first major symptom that I had was fatigue. I was exhausted, lacked energy - for a five year old, that’s generally a pretty bad sign. I then developed a salmon-colored rash all over my body that really only occurred during the nighttime and disappearing by the time any doctor’s appointment could be made. Sometimes it itches, sometimes it doesn’t. Scratching it can lead to odd bumps all over the skin. Another big symptom is high fever. I once had a fever of 106 degrees, which sent me into convulsions and shakes. I also had the uveitis that was mentioned in the tidbit on Oglioarticular Arthritis. They thought that I had pink eye for a while.
Other than that, there is excessive bone popping. We all know that terrible feeling when your ankle or elbow pops at the wrong time. On a normal basis, I no longer feel it because the amount of pain I’m normally in is far greater. Another problem that plagues many suffering from JRA are TMJDs, or Temporomandibular Joint and Muscle Disorders. Confused? Basically, I have terrible jaw pains. I actually used to be able to pop my jaw out of place, which can be a cool party trick - or a really painful accident.
Other than the rashes, there are several other skin conditions that are prevalent to those with JRA. Dry skin is a big problem. I also suffer from psoriasis, which seems to only respond to steroid lotion. Occasionally, I will get what we call “alligator skin” - small bumps, similar to goose bumps, which bring about terrible dry skin. This condition can last for up to a month and longer. Nodules can also appear. These can resemble small pimples or giant lumps under the skin. I generally get them on my knees in pimply form. You really aren’t supposed to get rid of it through your own means. However, the pressure from them can be terrible. Sometimes they can be picked like a pimple, others they must be popped with a needle. The best solution, if possible, is just to cover the nodule with a band-aid and wait. I have not had to deal with them for a while now, thankfully.
The biggest thing that makes Still’s Disease different from any other form of JRA is how it affects other parts of the body. My organs are affected on a daily basis. I have problems with my digestive system, which results in those annoying gurgly noises mid-class that end up distracting others. More than that, foods that I may be fine eating one day can turn my day into a nightmare the next. In the past, my stomach has suffered terrible aches for up to eight months at a time. Also, because the disease attacks my liver, I have to be careful of the medicines that I take.
I have also recently found out that both gluten and dairy affect my disease, gluten being the worst offender. A gluten intolerance can also affect the way the body handles high fructose corn syrup. Tell that to my soda addiction L
And let’s not forget that teeth are also bones.
Poked and Prodded: My Life Story
Blood tests for Rheumatoid factor and lupus, which both show the adult stage of Rheumatoid Arthritis, usually fail to show any signs of JRA. Back in the day, doctors really didn’t know about JRA. When I was first sick, they thought I just had allergies. I had a whole booklet of foods that I wasn’t supposed to eat so that we could find out exactly what I had a problem with. I was a five year old banned from eating PB&J! Imagine the sheer torture!
For months on end, they drew blood every week hoping to find what was wrong. I was anemic with a high white blood cell count. I had high levels of phosphorus, alkalines, and LDH. On occasion, I had: high AST (SGOT); low CO2 (poor lung capacity); low glucose; low urea nitrogen; high globulin; high MCH; low MVP; very high sedimentation rate; and, as I have already mentioned, a low red blood cell count.
And I was losing proteins quickly. In fact, any time I sweat or use the bathroom I lose proteins. I always eat as much meat as I can in order to get proteins back in my system.
We got hit by a Mack truck when we got a misdiagnosis. When I was six years old, we were told that I had just six precious weeks to live, that I was dying from Leukemia. The doctors told us that, if I was started on chemotherapy right away, I could prolong my life and maybe even beat this cancer. Luckily, my mother had great medical knowledge and knew that the symptoms didn’t really fit. Through tireless days and weeks and months of research, we were able to come up with Still’s Disease. After the searching, there was a name to the monster that was banning me from school, friends, birthday parties, and normal child activities. Finally, we could start finding ways to treat and cope with this disease… right?
Trick or Treat-ment: Limiting Foods, Gross Medicine, and Ace Bandages
With all these problems, it can be pretty overwhelming to try and live a semi-normal life.
Unlike most people confronted with this disease, I used to control it with Aleve, diet, and meditative pain management. Why? Because I had no insurance. Now that I have some, I am on a few meds but always wonder if the increase in my pain came with an increase in meds as a coincidence…
I’ve compiled a list of the meds out there and some background info on them for y’all.
First-line medication. Non-steroidal anti-inflammatory drugs (NSAIDs) are usually the first medications tried to control JRA inflammation and symptoms. Naproxen sodium is the most frequently used NSAID treatment for JRA. Doctors choose naproxen based on its low incidence of side effects compared to its effectiveness. Ibuprofen is an effective alternative. But in general, less than one-third of children will have significant relief from NSAIDs. NSAIDs and corticosteroids are most often used to control the initial stages of systemic JRA and may be used in children who have pauciarticular (oligoarthritis) with shortening of the muscles around the joints (contractures) or polyarticular disease with joint pain and swelling.
Second-line medication. If symptoms are not well-controlled with NSAIDs or corticosteroids, stronger medications such as methotrexate are often used successfully. Methotrexate, sulfasalazine, and other second-line medications are sometimes referred to as disease-modifying antirheumatic drugs (DMARDs). Some experts prefer to call them slow-acting antirheumatic drugs (SAARDs).
Some children with JRA gain significant benefit from early methotrexate treatment. Although there is no definitive way of knowing which children are the best candidates for early methotrexate treatment, this practice is becoming more common in an effort to prevent joint and eye damage. Early treatment with methotrexate is often used for polyarticular JRA.
Biological therapy is a newer option to treat JRA, particularly polyarticular JRA, that does not respond to other treatments. The biological agent etanercept (Enbrel), which is a tumor necrosis factor (TNF) inhibitor, has had some success in relieving symptoms and decreasing the number of flare-ups. Other TNF inhibitors, such as infliximab, are still under study to treat JRA.
Medications used to treat JRA
Other TNF inhibitors
Other second-line medications used less often
Antimalarials (such as hydroxychloroquine sulfate [Plaquenil]
Adult therapies, such as cytotoxic (cell-destroying) drugs and intravenous human immunoglobulin, that may be used for rheumatoid arthritis in adults but are not yet proven to be safe and effective for children with JRA
Gold salts were one of the first treatments used for joint inflammation, and you may still hear about them. However, injected gold salts have been replaced by methotrexate for the treatment of JRA. Gold salts taken by mouth (oral) have not been shown to be effective for JRA.
I began my step into “real” medication with Plaquenil in Sept 2010. I thought it was helping, but in October, I had a horrible flare. The Plaquenil wasn’t working on its own anymore. I added Prednisone, first to control the swelling of the flare and then permanently… In January 2011, I began MTX and folic acid supplements. I stopped taking both the MTX and Plaquenil in May/June of 2011 because of side effects cause by both, namely the fatigue and illness-prone issue associated with MTX. As I stopped those, I began taking Humira shots, 40mg every two weeks.
I’ve detailed in some posts how I like to meditate for pain relief. That is, when I focus enough in order to do that. On a more normal basis, I actually meditate without really even knowing. I don’t even space out anymore. I wish it helped control pain better though.
I have a feeling that a lot of it has to do with attitude. I try to always have a positive attitude. Even when I’m in pain, I know that things could always be worse. I still have pretty good control of my motor skills and I’m not in a wheelchair (which, by the way, was a prediction for around age eight when I was diagnosed).
Physical therapy is also an important thing. Water activities are better than anything else really, because it’s low-impact exercise. The warmer the water, the better – as long as it’s not too hot. Extra heat can cause extra pain. Who needs a better excuse to get a hot tub huh? Ranges of motion (ROM) exercises are also very important in keeping joints as healthy as can be. Regular exercise should be done when possible. By looking at me now, you’d never guess that I used to run upwards of twenty miles a day, but I did. Surprising huh?
Diet is another important part of controlling the disease. Since I am anemic and hypoglycemic, I have to make sure that I eat enough iron, protein, and sugar. In reality, the hardest part is getting the nutrients that I need daily. Sadly, multivitamins make me really sick to my stomach, so I can only really handle the vitamin D I’m on now.
And if all else fails, wrap the most affected joint with an ace bandage. Seriously, it helps a lot… sometimes.
The Moral of the Story: What Can You Learn About Living With JRA?
The biggest thing to remember is not to feel weird. There are plenty of people out there living with different ailments and there’s no need to feel alienated because you’re not “normal.” After all, what is normal these days? One of my favorite middle school teachers was proud to be called weird, and got upset when called normal. He taught me to embrace being weird, because that means I’m unique.
While there isn’t a cure for JRA, remember that there are many tools available to fight the disease. With a positive attitude and a supportive group of family and friends, you can be the best you possible. Research as much as possible. Ask questions of your doctors. Be as active as you can be. And remember that I am always one email or comment away if you have questions or need advice.
Compare yourself to a hurdle runner - you have many hurdles to jump over, but if you stretch first and pay attention to the track ahead of you, you will be able to clear every hurdle in your way.