Emily’s Juvenile Rheumatoid Arthritis story
Patrick’s Juvenile Rheumatoid Arthritis story
Lyn’s Juvenile Rheumatoid Arthritis story
Here is Emily’s Juvenile Arthritis story, in the words of her mother Dee.
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. Honestly, now I wonder if leukemia would have been better. There’s something like a 98% chance of remission with no recurrence of most types for children, I believe. At least it’s not forever.
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 pneumonia. And a bad limp. In a race with her great-grandmother Emily probably would have lost. She was so slow that it was very hard to keep back with her. 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 accommodations for Emily early on.
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. N knew right away that she was 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. Around this time her personality started changing. The fears, phobias & anxieties were dropping, she started to become more hyper, more active. She was a different child every day.
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 the 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 cannot 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. At her last rheumy appointment he expressed that he isn’t satisfied with her progress right now. He maxed her Enbrel dosage and changed her back to injections of MTX, but he said we will probably have to change her to Orencia, an IV medication after her next appointment. He didn’t have very much hope for the Enbrel dose change to actually work.
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 pauciarticular (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 polyarticular (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 resilient 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. Just because your disease is not flaring, or seems inactive doesn’t mean it’s gone. 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 too.
Every day is a gift. It’s up to you how you use it!
You can read more from Dee on her blog.
Here is Patrick’s Rheumatoid Arthritis story, in his own words.
When I was in my late 20’s and early 30’s I developed a condition known as Reynaud’s Syndrome. When my hands got cold, the tips of my fingers would turn pure white as the blood would leave them. I asked a doctor what this was and she said it is your hands trying to protect themselves from frostbite. I have since learned that it often is the first sign of Rheumatoid Arthritis.
Then when I turned 40 years old my eyesight started to go and eventually I had to wear glasses (trifocals) all the time. At the same time my feet began to hurt when I walked long distances. Then I developed a cataract in each eye and had them removed. Next, the index finger on my right hand became grossly swollen. They could find nothing wrong with it. My knees then started to hurt and eventually I had “bone on bone” in each knee. So, I had both knees replaced at the same time. As soon as I recovered from my knee surgery, my right hip went “bone on bone”. I had it replaced too. Then I developed all these different skin rashes. Now, the bottom of my feet hurt like crazy. I went to a Podiatrist and she said I had severe RA changes in my feet.
I was told that all of these conditions described above are related to RA. My father had RA, I have it and my two sisters have it. I also have hypertension. For the RA I take Humira and Sulfazine. I was on Methotrexate, but it hurt my liver.
It took a full year for the doctors to figure out what I had. I went to every type of doctor known to man. They thought I had Lyme disease and all sorts of other things. Finally, a blood test showed that I had an RA marker in my blood. That’s when they said I had Rheumatoid Arthritis. I have a friend that is an Internist and he says it is normal for it to take awhile to diagnose it.
I use “Icy Hot” on my feet and that helps a little bit. My hands and shoulders are fine. The only things that bother me now are my feet.
This is a really disabling disease. I have been on long term disability for the last 5 years.
Here is Lyn’s Rheumatoid Arthritis story, in her own words.
I always say I got RA in 1956, when I was twenty-three, but reading some of the other stories here of “early warning signs” years before full-fledged RA, I am more than ever convinced that the times when I had to cancel my piano lessons due to sore wrists were omens. At age19, I left the music conservatory at Millikin University to marry my Air Force sweetheart. The only strenuous parts of that happy, fulfilled marriage and life were doing the laundry– khaki uniforms, bedding and all our clothing– in a bathtub, and playing the piano for ten hours a week for a dance school. I don’t remember my hands or wrists complaining, only my back.
I had a healthy baby girl at age twenty-one, and another planned pregnancy a year later. During the three months that it lasted, I had painful ear infections, and near-bronchial pneumonia–that second infection during a long car trip to move from our Air Force home in Mississippi to New York State. I hadn’t even found an OB yet when I began to miscarry, so, in a panic I chose someone from the Yellow Pages, who falsely advertised himself as a specialist. He was not! He “liked to deliver babies,” and was totally inept, as I later learned.
With his full approval, I got pregnant again two months later, and began to have problems almost immediately. The least of my problems was the rapid weight gain, for which I was given amphetamines–“speed.” I kept saying “How can I be getting kicked in the groin and in the belly-button when I’m only 4 1/2 months along? It’s gotta’ be twins!” There were no ultrasounds, sonograms, nor amniotic fluid tests in the mid-fifties. I was right; he was wrong; and at seven months I delivered twin boys who survived just four and eight days. I needn’t tell any of you where the stress level was at that point. Plus, although by then I did have a wringer washer, it hadn’t yet occurred to me that Upstate New Yorkers didn’t hang their clothes outside in the middle of winter.
Normally thin, I had some “baby fat” to work off, so I took a class in volley ball. I came home from the first night’s class with red, swollen, stinging wrists. My husband and I agreed I must have really hit the balls with a vengeance! But the symptoms didn’t go away. By the time I finally got my second beautiful healthy daughter, two years later, I couldn’t lift or diaper her at night. Days weren’t quite as bad, but she had to learn to hold on to me as I lifted her on my forearms instead of hands.
Somewhere during those years, my PCP (an internist) started me on gold salts, and for fifteen years, on and off (off for nine months to have another healthy baby boy), that and aspirin, then Darvon Compound by the handfuls, were all that was available to me. My stress level rose and fell as my husband began to tire of “taking care of a sick wife,” and finally left me and our children. I was lucky to find a loving, caring man to enrich my life, and during his remaining six years before his sudden death from undetected metastatic pancreatic cancer, I went into remission. I was even able to go off the gold salts, dance, play the piano again, and shovel the driveway! People who knew me only during those years were hardly aware of the RA, unless they noticed my bony forearms and fused wrists–it was seldom necessary to mention it unless a contra-dance partner was grabbing me too hard.
Two years later, the remission ended dramatically, and the terrible pain returned along with a sunburn on my wrists, the only exposed part of me as I sat and luxuriated in the sun’s warmth on a cold, damp, day on my beloved Star Island in the Isles of Shoals in 1981.
From there it spread to most of my joints–at one time I counted thirteen that were involved. I had sore feet all my life since a child, but I didn’t connect the dots until my toes began to be deformed and dislocated. My first doctor in 1956 had diagnosed it as fibrositis, but three to four years later he admitted he lied to “keep me from freaking out!” I remember saying “I don’t care what you call it–call it the creeping crud–just make me feel better!” I still had no positive role models. I knew nobody with RA except an elderly woman I heard about who had been in a nursing home all her life, totally curled up into herself and helpless in a bed-wheelchair. Her story scared me to death.
It took me decades to understand that I needed to be seeing a rheumatologist rather than the parade of internists passing through my life. Only then did I get the understanding I needed, but at least the internists had done the right things, even though some thought the sero-negative classification meant that I didn’t really have it–spoken as they looked right at swollen, deformed joints. I have tried just about every medicine that has come along in these fifty-four years. Only when my HMO threw out all the seniors, ten years ago, was I able to get the biologic, Enbrel, through my insurance plan. It has literally given me my life back as part of a cocktail with Plaquinil, prednisone, Methotrexate, folic acid and Mobic.
Along the way, the inability to play the piano after the first flare had led me accidentally into a glorious adaptive career that I loved, and eventually needed to support myself after my marriage crashed and burned. In the nearly forty years as a folk singer, with small lap instruments especially chosen to be easier on my hands, I have had five hand surgeries (two to replace five joints which have subsequently broken and subluxed); four foot and ankle ones, reconstructing and fusing all the toes; three cancer surgeries, one with radiation; two pacemaker implants; about nine other unrelated surgeries; and an eventual diagnosis of another autoimmune condition, Celiac Disease. I now have a severe degenerative spinal condition–only partly due to the RA–and neuropathies in both legs, so I have to use a cane or rolling, seated walker outside of my tiny accessible apartment. I have atrial fibrillation and have had some scary episodes with that— but I’m still here! At seventy-seven, I’m active and productive at writing (three books published since I was sixty-seven), photography (a dozen solo exhibits), blogging on several blogs, teaching memoir writing, attending coffeehouses and concerts (just retired from being the booking agent and publicist for a folk concert series for thirteen years), enjoying my five great-grandchildren, six grands, and their parents, and even occasionally sing a song with my daughter who is also a professional folk singer.
I have far less energy than I used to (and the fatigue was always a serious problem, as you all well know), but I also have far less pain–nothing like it used to be. I know how hard it is–God knows, I know–and my heart aches for all of you who are brave enough to share your journeys with us, and who are just starting. I’ve done my sharing of mourning and I do allow myself to wallow in it when I need to. I get a certain modicum of peace from finally accepting that this is my path–for whatever reason– my only life, and I’m going to enjoy the Hell out of it whenever I can. I write a lot about choosing joy, and get great pleasure from all the people who feel inspired by what I write and what I accomplish under almost impossible odds. My thesis is that one can choose to celebrate or mourn one’s lot in life, and there is always justification for either. And it’s going to hurt whether I sit home or go out and have a good time. Probably less, in fact. If not today, maybe tomorrow or the next day. There were times when I couldn’t stand the pain of playing my instruments at all to rehearse, but on stage I felt no pain (until about an hour later!) Me, I like to sing and dance, even if it’s only virtual these days.
You can find more from Lyn at her own website.
Note: The photo is me enjoying my latest passion– walking our amazing Walkway across the Hudson River– 212 feet above the river, and 1one and 1/4 miles long. I walk part of it very slowly, with my walker, taking lots of sitting breaks and taking photographs. I pay for it the next day, but it’s priceless to me.