21 September 2014

Happy 30th Anniversary????

Happy(???) 30th Anniversary!

On 21 September 1984, after spending a  week in the hospital at Riley Hospital for Children in Indianapolis, IN,  we heard the words that would forever change my life. I had been in the hospital all week. But unlike the rooms where most hospital stays occur, this room was more like an efficiency room in a hotel, a private room with a small refrigerator (I think), of course a private restroom. There was less a feeling of being hospitalized and more a feeling of just staying there for tests because it was more convenient. I vaguely remember being seen by quite a few doctors and nurses as well as having lab-work, X-rays, eye tests, and other exams. We had went into the situation with a fairly good idea of the outcome. The problems I'd been having for awhile were getting less scary and more of a hassle than a major worry. i don't know if I got more used to the pain and so woke up less in enough pain to scream as had happened at first. Or if I just got used to it enough that it no longer took me by such surprise and was a shock to me. In the beginning, Mom, like most parents would have, assumed I was having nightmares. And in a way, she was right, I was having a major nightmare, but not in the way most parents consider the use of the word nightmare. I was so wracked with leg pain that i was unable to move my legs. The stiffness and pain was unexplainable really beyond the tears and saying I hurt. So, like most parents, she took me to the doctor. He was one of those old country docs who practiced in a tiny rural town in the early 1980s. I later heard that he did not have a good reputation amongst older family members, which I don't believe Mom was aware of. We left there with no clues as to what was going on. All that doctor would tell her was that I had "growing pains". But something about that just didn't sit right with Mom. I wonder if her own pain led her to see that my pain was very real and went beyond what is typically described as "growing pains". In addition to waking up at night, screaming and complaining about leg pain as well as being unable to move them, I also had a problem with my right ring finger that we later found out was called a "trigger finger" as well as a ganglion cyst on my left hand.  Over the last 30 years my hands have changed to what is now noticeable damage and misshapen fingers but not to the extent in the drawing below. I do have each of the items mentioned: boutonnière deformity in my thumbs, ulnar deviation of my hands as well as swan-necking of the first joint of some of my fingers and hypertension of the middle joints on some fingers. The  drawing is much worse than my hands are thankfully.

Needless to say, she took me to a different doctor in Danville, IN. This doctor, after examining me, declared that he was not about to treat me and that I needed to be seen by a specialist at Riley Hosp. for Children in Indianapolis. His concern was that I had either a muscular dystrophy or a form of juvenile arthritis. The next I remember was the week-long stay at Riley, which culminated in the official diagnosis: seronegative poly-articular juvenile rheumatoid arthritis, quite a mouthful for a 7 yr old! The seronegative part simply meant that there were none of the markers of JRA in my lab work, a not uncommon occurrence. In adults, at the time, at least 20%
of patients with RA were seronegative. My erythrocyte sedimentation rate, or SED rate for short (aka ESR) was elevated but it can be elevated with any kind of inflammation in the body, including infections. I had a lot of problems with upper respiratory and ear infections so having an elevated ESR was not too out of the ordinary. But I was never positive for Rheumatoid Factor (RF) at any point in my life. According to Lab Tests Online, RF is: "Rheumatoid factor (RF) is an autoantibody, an immunoglobulin M (IgM) protein that is produced by the body's immune system. Autoantibodies attack a person's own tissues, mistakenly identifying the tissue as "foreign." While the biologic role of RF is not well understood, its presence is useful as an indicator of inflammatory and autoimmune activity." I have NEVER tested positive for ESR in the past 30 years. There are more tests now that they can use to lead to diagnosing RA or distinguishing between the various types of arthritis but the last I recalled, i am either completely negative for those tests that the results are either negative or positive, or for those that have a cut off of the normal levels of what is being tested for in the blood, I am under that normal level. I have been tested for the Sjogren's antibodies, anti-CCP, CRP etc. and even now everything is either in normal ranges for that particular test or is negative. My labs still show that I am sero-negative 30 years later. Again, my SED rate is often elevated but that could be infection related as much as anything else. 
I recall the doctor thinking when we first got there that I had some form of juvenile arthritis most likely.  As the week wore on and tests were done and results were in,  I think he was more and more sure of his original thought. I remember that Mom was certain that since it was my body and my life affected by the diagnosis, that she wanted me to be told everything. I had to know what I was dealing. I don't know if my doctor, who at the time was Dr. Murray Passo, agreed with her that it was the best choice or not, but, he went along with it.  And for me, it worked. By the time I was 10, I would go to the reference section of the library and read medical books for fun. Not that I understood the greater portion of it but I understood enough. I was used as a teaching student. I was at the clinic enough that it made sense to have me as one of the patients that student doctors visited on their trips through the clinic. After awhile, because I tended to pay attention to what the doctor said, I began to be able to answer his questions if the students would or could not. It was not any great feat of intelligence I can be sure of. It was quite simply repetition. No matter a child's age, if they hear something repeated over and over, especially about themselves, they are likely to remember it. I was 10 when I asked about how RA shortened people's lives, which I think was surprising to many people. I know at one point, Dr Passo had told me the worst case scenario —that by the time I was 16, I'd not walk again, I'd be on medications for the rest of my life, I'd not work part time even let alone full-time and I'd not marry and have a family. I was determined to prove them wrong. At 10, was my hand surgery. Because of the concern of giving a child pain medications for more than a few days post-op, I did not have adequate pain control when it came time to begin PT. It was then I began considering PT to be patient torture vs physical therapy. But, I also had a hard time explaining how I hurt beyond say "I hurt," which admittedly makes it hard on a doctor to know if the child truly is experiencing pain and is used to it enough that the typical reactions to pain are not there (I can be in a lot of pain now and no one but those who know me well can tell IF I am not wanting it to show; after this long of my being filled with some amount of pain everyday for as long as I can remember, you tend to learn to mask it) OR if there is something else bothering them.  I know many of my doctors in the past had a hard time believing I was really in the pain I said I was in. Another reason I think I have such difficulty in explaining how I hurt id the fact that I truly do not remember what it is like to be pain free. I know at some point in my childhood I was pain free but it was so long ago that I do no remember it. It's as if between the time that has passed and all that my body has experienced, that those pain-free days have been pushed out of my memory.

 I do know that Dr Passo was a great doctor. Looking back, it might be wondered why I was never put on any of the disease modifying anti-rheumatic drugs available at the time. Or why I'd never been given ______. The biggest reason is simply that I was not considered to be a severe case of JRA.  This was during a time when ibuprofen and naproxen were beginning to be studied as treatments for kids with JRA. There was hope that they would work well enough that the stronger drugs with the worst side effects could be avoided. This was before the practice of "early aggressive treatment" was given the emphasis that it is given today. We know now how important it is to slow the progress of JRA in children and RA in adults as early as possible. We know now that it is in the first 10 years that the most damage occurs in those patients who are disabled by RA or JRA. As Dr Passo explained it to me, the difference between the three forms of JRA, pauci-articular, poly-articular and systemic are: those with pauci-articular JRA tend to have five joints or less affected; those with more than five joints affected have poly-articular JRA and those with salmon colored rashes, daily fevers, etc in addition to the five or more joints being affected are those with systemic JRA. As it was explained to me, polyarticular JRA is much like adult RA while systemic JRA is also known as Still's disease, which generally occurs in children but can wait until adulthood to begin, in which case it is known as "adult onset Still's disease" (AOSD). That said, poly-articular JRA, (which I will hereafter refer to simply as JRA and the other forms by their longer names) as well as adult RA both have components of the disease that are systemic. The low grade fevers common to many patients with both JRA and RA, especially during periods of active inflammation is common as is fatigue, feeling flu-like, etc. For many patients of either, slow healing cut and sores are common as is increased bruising. The bruising can be related to certain medications, primarily prednisone or other glucocorticosteroids but in some people can be part of RA/JRA. I was not on any glucocorticosteroids of ANY kind until after I was 2 and had my son, yet I bruised VERY easy as a pre-teen and teen. Even into adulthood, I bruised fairly easy. At one point in my life, I was seeing a physical therapist who, during the course of the treatment ordered by my doctor was leaving rather large bruises on me. They were not overly painful, but the PT was concerned at first as to what my husband would say when he saw them. I assured him that my hubby would know about them before seeing them so that he was not upset over them. He knows my easy bruising nature anyway and so is unlikely to become angry before giving me a chance to explain. I can understand the PT's concern  as well since I was bruised from neck to the back of my thighs, because he was doing myofascial release massage therapy, which released the knots formed by muscular waste chemicals that would build up in the muscles. It was hard work in a way to have that type of massage therapy. The waste that was broken up and released into the system caused nausea and sleepiness on the days I had therapy. So while it felt good in a
way to have those knots broken up, the sleepiness, fatigue and nausea were too much. 

After my first pediatric rheumatologist transferred to a hospital in Cincinnati, OH, I got a new
doctor that I don't recall much about. I don't recall caring much for her but then again, I am not sure if that is the case, or if she just doesn't compare one bit to Dr Passo.  I do remember him a being a great doctor. And he is the one who started to encourage me to learn all that I could about my JRA. He taught me quite a bit as well. And of all the doctors I've met over the last 30 years, and there have been quite a few, he's been one of those I have had the best memories of. I still to this day, even though it's been probably 20 years since I've seen him, would trust him implicitly. Although, in many ways, he made it hard for me to find other doctors to live up to the standards he set. I have been blessed to have a few who do.  And for those I am thankful. One of my biggest ways of knowing if a doctor will work out is how much they value a patient's knowledge. If they have a problem with patients who are well-informed about their diagnosis, then they will not work for me. I will not be treated as if I don't know my own body just to make a doctor like me better. And the doctors, as well as other health-care providers that I see currently all seem to value that in a patient. i am blessed with a great health-care team right now. I am thankful for that to be honest.  Because while I know my own body, and what is and is not normal for me, that's where my knowledge stops. Yes, I know quite a bit about rheumatic diseases but not as much as a doctor who specializes in them. But the best thing is that I do know what is and isn't normal for me and after 30yrs can usually recognize the abnormal. I say usually because as was proven in 2008, there are times a med can mask what would be normal symptoms of a problem that I was well-aware of what the symptoms felt like. Yet, when you have no symptoms of a problem, you can't exactly know it is there!  Thankfully, the medications that caused that issue are no longer ones I take or are ones I still take but at a MUCH lower dose.

Will I ever live a "normal" life again? Of course not. I have way too much joint damage as well as too much deformity etc to have what would be close to a "normal" life. But, while I know that JRA may have taken its toll on me, and it may have won some of the battles, it has NOT won the war. It can only do that if I let it knock me down mentally as well as physically.  And I will NOT let that happen if I can help it. I may not be able to do as much as I was able to do physically before the "long vacation" but it hasn't totally won yet. For that I owe quite a lot to the influence of many people over the last 30 years. But I would not have had as many tools to fight with had it  not been for Dr Passo as my diagnosing doctor. Don't get me wrong, Mom insisting i be told the truth had a big influence on me as did the influence of a number of family members.

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