Monday, May 3, 2010

What is JRA?

When my daughter Emily was first diagnosed with JRA at age 6 we were devastated. Thanks to her pediatrician starting off by saying "Well, it's not leukemia" we were more prepared than some, but still bewildered. Where to begin? I did a ton of research from that point, but still felt quite alone at many points after diagnosis. I didn't really know where to turn. What might her future hold? I wanted to hear other people's stories, but where to begin? I've had two years to read, listen, analyze and contemplate now, and it occurred to me that maybe I should share our stories. Our story could help another family!

There were signs and symptoms early on, like large red hive-like spots on her knees that would come and go. From around 18 months on she cried almost all of the time, and if she hurt herself just the slightest little bit she would carry on like it was the end of the world. As a toddler she had chronic constipation... like the kind that had her screaming when she had to go potty, and clogged up the toilets once finally out. (No tummy problems have currently been diagnosed but she does still have tummy issues. I'm not sure if they're related or not.) She started to develop numerous anxieties and phobias. In 2007 Emily developed pnemonia. And a bad limp. In a race with her great-grandmother Emily probably would have lost. She started to develop the ganglion cysts, or rheumatoid nodules. Of course, we had no idea what they were at the time. Her pediatrician ordered x-rays of the first one, and they came back clear. By week's end she had a new one next to the first, and then 2 on her other hand. They started popping up on all of her joints. She could barely hold a pencil. When she started school her kindergarten teacher used to carry her around to her specials and lunch. She started making accomodations for Emily early on. (Mrs. Pacimeo was the best kindergarten teacher EVER.) It took many more x-rays, lab draws and months of patience waiting to get in to see the rheumatologist. It was a long wait. (There is a shortage of pediatric rheumatologists. Some STATES don't even have one.) Of course Dr. Nickeson knew right away that she affected all over, and that she just wasn't happy. He prescribed Naproxen, an NSAID, to control inflammation. She was on Naproxen only for about 6 weeks when he decided to add more meds, and start her on Methotrexate. We were terrified to find that this med was also a chemo medicine and an immune suppressor. Even more terrifying was the prospect of injecting this sweet, pain riddled little baby of ours once a week. The first few injections were horrible, having to hold her down while she screamed and cried like we were trying to kill her, with me crying right with her. I cannot describe how awful that was. It was just heart-wrenching. After about a month she realized that she was starting to feel better. From that point on she still had a hard time with her shots, but she tried very hard to be brave. By September of 2008 it was decided that the MTX wasn't helping enough. Dr. N decided that it was time to try a more aggressive medicine. One of the newer biologics, Enbrel was his choice for her. This, too, was terrifying even though I had been hoping that he would suggest the Enbrel because we thought that the MTX had stopped making progress. Besides that, her liver enzymes and white cell counts were going up and down, and we had to back down the dose. There was a whole year period that we had to stop the MTX. We could no longer use the Naproxen anymore due to blood in her stool. Naproxen also gave her this rash that morphed around. It would last about 3 weeks per round, starting as a pink patch and eventually turning purple and very painful. The patches would pop up anywhere, and looked like we were beating her, I'm sure. We were really looking forward to Enbrel. Enbrel began as not just a medicine but a process. The pharmacy had to be special, I couldn't just send them the scrip... it had to be done by the doctor's office. We had to get a TB test, spend hours on the phone setting everything up, and finally getting the medicine. Thank God for Norma, Dr. N's teaching nurse who had taught us all about Enbrel and how to set it up, because the medicine had to be set up using a complicated process, and then it can not be shaken! Also, we went from one shot per week to 2 injections of Enbrel per week. Everything about it was scary. But ya know what? I am truly convinced that Enbrel was an answer to our prayers. It has been our miracle. It doesn't work for everyone, but for us this medicine has transformed our child from a sad mess to a happy, active girl. No, she isn't perfect, and it isn't just going to go away. But now she can tackle her brother, and run and play. She will still flare, as we recently found out. Since Enbrel is also an immune suppressor she gets sick more often than other kids. She's more prone to skin infections from cuts, and due to tight skin. Yes, there are other issues. Her physical therapist noted that she has no curve to her back. She surmised that Em tried to change the way that she walked to compensate for the pain, and that over time made the curve disappear. Her feet are losing their arches as well.

Emily's had all sorts of labwork run on her. One thing that we learned right away was that she is positive for the Rheumatoid Factor in her bloodstream. Most children are not. Her disease is closer to the adult form with that factor. Her doctor had decided at one point that she had scleroderma. We ran the tests with me expecting them to be positive. I had many reasons for that. Her ANA (auto nuclear antibody) came back high-titer positive speckled. That is a marker. The pattern that an ANA forms is different for different diseases. The lab takes the pattern and dilutes it. The more times it is diluted with the pattern still showing tells the titer. The speckled is related to scleroderma, but none of the other tests pointed towards that. This gave us enough to worry about, as usually kids that are pauci (less than 5 joints involved) usually have the +ANA, which also shows that they are more at risk for various arthritis of the eye. Emily is poly (more than 5 joints involved) but now still more at risk for the eye issues due to the ANA. After Dr. N said that it didn't really point to scleroderma I was shocked but very relieved. One of the main symptoms of scleroderma is Raynaud's Phenomenon. Raynaud's is actually also arthritis related ( ) but is more often associated with scleroderma. Emily definitely has Raynaud's. I have had phone calls home from the school like this: "Mrs. Tipton, we have Emily down here at the nurse's office. Um, we're not really sure what to do. Um, she's purple. Both of her hands are just purple. Do you have any idea what to do?" This is our world. This is our reality. I went to pick her up one day just a couple of weeks ago, on a warm April day. Both hands purple. She came home over the January winter break with mild frostbite. She didn't go outside to play that day. They kept her inside with her mittens and heavy winter jacket. She wears 2 undershirts when it's chilly out to keep the core warm. She keeps mittens with her even in the summer because often just having the air conditioning on will trigger it. At this point my biggest fear and worry is the Raynaud's. I'm terrified that one day she will come home with severe frostbite. People with Raynaud's are at risk for losing their fingers and toes. All this said, I was shocked when he said she didn't have scleroderma. What did I learn from this? Just because something is ruled out doesn't mean it will stay that way. He eventually decided that labs be damned, she does have scleroderma. Not the systemic form like I worried so much about, but she has the skin tightening form. Her fingers, affected enough already from the JRA and Raynaud's, often look like little sausages from the scleroderma. Her hands are greatly affected by it. It does affect her arms... it seems to come and go there to me, but I don't really know enough about how to tell to judge well. Her feet are affected. I have come to realize that she cannot wear shoes without socks. I don't care what kind of shoes she wears... she has to have something between the shoes and her feet. Otherwise she gets sores along the sides of her feet. The sores have led to an infection that required 20 days of topical and oral antibiotics. And that was despite our using Neosporin on it as soon as it started to inflame. I thought it was MRSA.

We have learned so many things through our journey so far. We have learned to trust God more. We thought before diagnosis that Emily was unstable, couldn't handle much. That child is my hero. She feels crappy every day, so much so that when she's sick she can't tell me for sure because she's used to it. She handles pain like I can't even believe, and trudges on when most of the rest of us would have quit. She's so smart, and so sweet. She can tell you what meds she's on. Kids are resiliant in ways that I hope most people don't have to see. We have learned that no matter how bad it seems, there are worse cases out there. We have learned that nothing is for sure. Just because a diagnosis is ruled out doesn't mean it will stay that way. Expect nothing and anything. You have to find a way to accept what you cannot change, but you can still keep fighting! You have to find a way to let go and let your kids have a life... even if they are worried about getting hurt. And just because you have a disease doesn't mean it should define you and run your life. My goal is to help other people going through what we have. Being alone sucks, and having no one to turn to does to. Please, if you need a shoulder, an ear, advice, whatever, please know that I am here.

No comments:

Post a Comment