Thursday, April 30, 2009

Still's Disease? What's That?

What is This Noise All About Anyway?: JRA, Still's Disease, and Other Fun Stuff

The type of Juvenile 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 four. 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 the 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. Now, I can't truthfully explain the pains that I go through everyday, 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 four 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 iritis 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 pop 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 can actually pop my jaw out of place, which can be a cool party trick or a 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, which I have found only Burt's Bees to cure. 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.

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.

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 four and a half year old banned from eating PB&J! Imagine the sheer torture! But I digress, as usual...

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 nitrogenhigh 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. Now, I know pizza isn't the best thing for me, but I love most meat lovers zza's. I usually try to have some sort of nuts around, like cashews. Veggies are good to have around too. I love Caesar salads and wraps. And, thanks to Chartwells' amazing new options, there's an organic place to eat on campus where I can get tasty soy and tofu products too.

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.

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 control it with Aleve, diet, and meditative pain management. Why? Because I have no insurance. I also do not like some of the (possible) side effects of the medicines out there - anal leakage? C'mon, really? I'd rather limp and have my dignity.

In all seriousness, this is really a disease that needs medical attention. I don't personally think that all medicine, all the time is the way to go, but for some people that works. I took liquid 
Naprosyn as a child and it reeked havoc on my digestive system. As I mentioned before, I do take Aleve - which is Naproxen Sodium and not that much better - occasionally. Any over-the-counter pain reliever is good for me really. Since I really don't have any experience with other forms of medication, I went a-searching the interweb to bring you information on this front:
First-line medication. Nonsteroidal 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.10 Ibuprofen is an effective alternative. But in general, less than one-third of children will have significant relief from NSAIDs.5

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.105

Second-line medication. If symptoms are not well-controlled with NSAIDs or corticosteroids, stronger medications such as methotrexate are often used successfully.105 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.5

Biological therapy is a newer option to treat JRA, particularly polyarticular JRA, that does not respond to other treatments. The biological agent etanercept, 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.11

Medications used to treat JRA

First-line

Nonsteroidal anti-inflammatory drugs (NSAIDs)
Injected corticosteroids

Second-line

Methotrexate
Etanercept (Enbrel)
Infliximab (Remicade)
Oral corticosteroids

Other second-line medications used less often

Sulfasalazine
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.10

In one of my first posts on my other blog, I detailed how I meditate to relieve pain. 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 focus on what I am doing, but am concentrated enough that I don't really feel the minor pains. I won't lie and say that I am pain-free - far from it - but I feel less pain on a consistent basis than other people I know with JRA or RA.

I have a feeling that a lot of it has to do with attitude. There is an older woman who works in the shoe department at work with RA. Her hands are deformed terribly and yet she still works. She still tries her hardest to not let the disease get to her. I almost 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. Who needs a better excuse to get a hot tub huh? Range 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. I've been debating whether or not I should set aside some money to get multivitamins to take everyday. Seeing as I don't always eat right, it's probably a good idea, but I'm too lazy.

And if all else fails, wrap the most affected joint with an ace bandage. Seriously, it helps a lot.

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?

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.

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