Happy 30th Anniversary????

Happy(???) 30^th 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.

Appointment at pain management

I had my bi-monthly pain appt. It went as it always does. Spent a bit letting Stacy know life is as usual, she asks the standard "How have things been?" question that I know answering with "fine" will get me the look. I don't know exactly how to describe that look either. It's almost one of those Mom looks that we use when we know there's more to the story than what we're being told. But it's not quite as maternal as the Mom look. But it's a look that says she KNOWS there's more to it than what I am telling her. I told her that there's nothing more than the normal ups and downs of life with chronic illness. That I couldn't complain....to which she replied, "Yes you can." I had to laugh at that because we've discussed in the past how complaining about the normal stuff that can't be changed does me no good. I told her about my new meds. We discussed my request to change how I take my Neurontin and Robaxin. If I took meds as they are supposed to be scheduled it'd go a bit like this:

• 6 am: Neurontin, Robaxin, Prednisone, Vesi-Care, Provigil, Plaquenil, Omeprazole, Calcium, a Multivitamin and then 2 ea. Methadone and Guaifenesin.
• 9 am: Oxycodone Technically it's PRN and I don't take it if I don't need it, but when I am up early, I am usually getting myself cleaned up and dressed around that time of day and need it then. At the nursing home, most of my nurses didn't ask or make me ask if I wanted it since they knew I always wanted it.
• 12pm: Neurontin, Robaxin, Metoprolol ER
• 2pm: 2 Methadone
• 6pm: Neurontin, Robaxin, Acyclovir, Oxycodone (PRN)
• 10pm: 2 Methadone
• 12am: Neurontin, Zanaflex, Plaquenil, Folic Acid, Arava
• 3am: Oxycodone (PRN if I am awake and need it, which is many nights)

That's a bit much. 4hrs at most between doses. And that list doesn't include any Salagen, which I can have twice a day or anything for headaches. It's a lot better than one of the nursing home med schedules they had me on. It was wacky. I didn't have but 2 hrs between a doses a couple times a day. I took meds at 6am, 8am, 9am, 12pm, 2pm, 3pm, 6pm, 8pm, 9pm, 12am & 3am. That didn't last long and thankfully Dr Wilson was great when I told him it was driving me nuts. He sent the unit mgr to work out the schedule I wanted. I don't know if that happens with too many people. But he was also the doc that if I asked for a med and a nurse backed me, I definitely got it. It didn't always take a nurse to back me either. Many times I asked and he said sure. But then again, I am not the type to just ask for meds just to get them. If it were up to me, I'd not need meds. But unfortunately, I do. So I have no choice. I've learned my lesson about leaving RA untreated. It causes more problems than it solves.

Stacy said that I could adjust the timing within limits. If I wanted to go longer than 6hrs, I could. Not shorter of course but that's not what I needed. She changed both the Neurontin and Robaxin scripts to read PRN, that way if I didn't need them a month after my fill date, the pharmacy didn't hassle me about not properly taking meds. I appreciated that. She said I didn't HAVE to take all 3 Robaxin and all 4 Neurontin if I didn't need them. But I know I need them most of the time. Now when I am in one of those major fibro flares where I am doing nothing but sleep 12-18 days then I may not need them. But I usually do because I definitely notice when I miss em. Especially the Neurontin. The neuropathy gets so bad that I just feel as if my hands are burning and tingling. And anything touching my feet, including socks, has me in that amped up pain state. So because she said I could make changes, I am gonna. What I am doing is dropping the Zanaflex and taking 3 Robaxin a day. Or if I get to where it's not letting me sleep, I have 2 other options. I can double up on Neurontin at bedtime if the neuropathy becomes an issue. The other thing I can do is drop to 2 Robaxin and take Zanaflex at bedtime. That may be what I end up doing. IDK yet. Now with the changes, my med schedule looks like this:

• 6am: Neurontin, Robaxin, Prednisone, Plaquenil, Provigil, Vesi-Care, Metoprolol ER, Prilosec, and 2 each of Methadone and Guaifenesin
• 10am: Oxycodone PRN
• 2pm:Acyclovir, Neurontin, Robaxin, and 2 Methadone
• 6pm: Oxycodone PRN I only take this if I really need it, although many times, I don't need it. 
• 10pm: Neurontin*, Robaxin, Folic Acid, Arava, Plaquenil, and 2 Methadone. *I am allowed a second Neurontin if I need it. 

She was glad I had success with Candyce. We were discussing the addition of acyclovir to my med list because of shingles. That got us discussing the shingles vaccine and whether people who have had shingles can get the vaccine. I thought I'd read that they could but didn't know for sure. (I looked again when I got home.  And that is what I'd read. So I left her a FB msg about that.) Then as she walked me out to the desk I told her again how glad I was to be back in her care. She hugged me and thanked me for saying that. Then I just had to wait on Gar to finish up with his 2 runs. He had 2 ppl at appts and needed to run them home, then he switched vehicles and got his paperwork for today so he could just come home when he picked me up. I kinda crashed for a bit, then looked up the info I wanted to find for Stacy and sent it to her and called Mom and that was it. I was wiped out.

Now I have NOTHING appt wise until 9/25 when I see Stacy at 2 and then labs at 3:30 and I see Candyce at 3:45! That will be interesting!

Follow-Up with New Nurse Practitioner

Follow-Up with New Nurse Practitioner

Another good visit all in all. The nurse Joyce is great. Shocked her by telling her a bit of my past. Especially about the not walking part and docs at the hospital saying how it was unlikely I'd do so or even live at home. Her jaw dropped and she said, "So where are you living now.?" When I told her that I've been home since Nov. 2011, she was happy to hear that. I've somehow managed to drop 2lbs. It took no time for Candyce to come in. I like her. She's not the type to sugar coat things. She did acknowledge that she had to tell me things she knew I was likely aware of just to make sure I knew. It was nice to have it acknowledged that I am a pretty-well informed patient. There's a lot of good news/bad news. Good news on x-rays: my cartilage in my knees is not bad. Which leads to the bad news (that was no shock to me as I had a guess what showed. The pain and swelling is RA, not OA. There's erosions, which to be honest is not shocking as out of the 28 years I've had JRA/RA, I wasn't on DMARDs until 2002. I was on Plaquenil/MTX in varying doses for a little over a year in 2002-03, then off meds until 2006. And then I was on MTX, Arava, Imuran, Enbrel, Humira, and Remicade in various combos until Aug 2008 when I went off Remicade because I lost insurance. I did have Arava, Neurontin and some other meds until Dec 2008 when I got sick on Dec 12 with what I had thought was the stomach flu at first, but after 3 days realized was likely not the flu and was possibly another UTI. Then no DMARD until I restarted Arava in 2010. But that's another story for another day...or week. So the fact there are erosions in my knees, hands, and wrists wasn't too shocking. She doesn't think Arava is enough which is why she added Plaquenil, it's as much for the Sjogren's as it is the RA as she did say Arava doesn't really help Sjogren's. She is planning adding Rituxan but NOT until I can deal with the dental issues that the Sjogren's has caused, because she said to do so is basically sepsis waiting to happen! Even if she felt the risks outweighed the benefits, which I don't see any sane, responsible doc doing in my case, I'm not too willing to risk it, given what all happened the last time I had sepsis. Missing 3 full months and large chunks of another 6-9mo of my life  is NOT something I want to risk if I can help it.

Again she addressed issues before I could ask my questions! On my list was med refills and we started with that list. I also wanted to ask about her mention of a shingles preventative at my first visit. I wondered if, since the treatment for shingles is the same med as for other forms of herpes (antivirals like acyclovir, valacyclovir, and famciclovir) and the maintenance dose to help lessen the chance of  outbreaks of the other forms is taking the med that treats it in a lower dose daily. I don't recall what I was on dose wise for treatment but she verified that I was right in thinking that the treatment and preventative meds are the same ones and she put me on acyclovir 200 mg, once a day. (I later recalled that the 3 times I do remember being treated for shingles, I took meds something like 5 times a day, and the dose of acyclovir for treating shingles is 800mg 5x/day.) She also decided that since my BP was up (for me not as much as it has been in the past) around 140/110 we're treating it. She chose a beta-blocker, metoprolol er 50mg/day. I was on metoprolol in the past. So that's good because I don't recall any side effects. Except good ones, such as less headaches, because it lowers my BP. When my BP is high, I tend to get really bad headaches.

My DEXA scan showed severe (although she didn't give me the numbers) osteoporosis (OPO). Again, just part of corticosteroids, although I forgot to mention that I'd had the OPO dx long before ever taking any form of corticosteroids. And of course she is putting me on something for that. I don't have details yet; she said I'll probably get a call within a week or two. She is referring me to a place called Palmetto Infusion (where I went for my Remicade) for Reclast. She likes that it's once a year and I'm done. Little chance of me being non-compliant! I am good with that. But she said it will do little good to start Reclast unless she also addresses the issue with my Vit D being very low (again no mention of level). I can ask for copies of labs and the results of the DEXA and x-ray reports. I just had so much on my mind  that getting that was the last thing on my mind! So she prescribed Vit. D3 50,000 IU once a week for 12 weeks. I did forget to ask if she wanted me to continue calcium w/vit D or switch to straight calcium and what dose she prefers me on so I called and left a msg with Joyce about that.

Her plan for AFTER I deal with my teeth is to have me get Rituxan infusions. And then after awhile on that and hopefully seeing the improvements many people see, she will send me to a DO who has a good success rate with helping contractures as well as starting pool therapy. I need to let her know of the issue I have with even heated pools causing me to have serious muscle problems for days after being in the pool and doing nothing (since I can't swim) but stand in shoulder deep water. But, who knows, I may not have that reaction any longer since I am on meds and when I had that reaction I wasn't. That was right around my fibro dx. She did say IF I wanted PT we could discuss it then. So basically neither she nor Dr K wants me to have PT right now! Which is good and bad. So I get to look for options for dental ins. and possibly have to wait out the pre-existing issues clauses. But I'm not dealing with ANYTHING else health related today except a stop at the pharmacy. Tomorrow is soon enough. But today I have other plans!

I have the remainder of the eye testing Thurs. and then NOTHING ELSE until the end of July! YAY!!!! I don't go back to Candyce until 9/25. She gave me 3 mo of everything with 1 refill on everything except the Arava and Vit D. So, can't complain there! My biggest concern was not knowing how she was on pred use and then what happens when it started causing cataracts. But she seemed to feel as I do about it. She acknowledged that she was sure I knew a few things about pred but that it is a band-aid approach that controls the symptoms not the disease. I am aware of that but given I cannot take NSAIDs daily, pred is my best option (although somewhere I read that it has DMARD-like properties in addition to the anti-inflammatory ones but that's not many who feel that's true). She also said that cataracts are common with pred use, especially in higher doses or with long-term use, both of which fit me. Although that high dose no longer applies and I am trying hard to keep it that way! And so aside from forgetting I needed that script, which she asked me to double check and make sure she got em all and when I looked, I missed that she skipped 2 meds I needed. I caught one before either of us left the office, just as she was walking out the door, but the pred I didn't catch until late last night when I was telling Mom about my new meds. But I have enough to last until sometime in July so I can call and ask them to either call it in or have Gar pick it up sometime between now and when I run out. 

My meds are now:

Acyclovir (shingles prevention), Arava (RA), Folic Acid (side effects of Arava), Guaifenesin (chronic sinus congestion), Methadone (pain), Metoprolol (hypertension), Neurontin (neuropathy), Oxycodone (breakthrough pain), Plaquenil (RA and Sjogren's), Prednisone (RA), Prilosec (GERD),  Provigil (sleepiness), Reclast (osteoporosis), Robaxin (muscle spasms), Salagen (dry mouth), Vesi-Care (bladder problems), Vit. D (Vit D Deficiency).