My Story

Hi! My name is Siobhan, and this is the story of my experience living with a chronic disease……

DIAGNOSIS PHASE

I am often described as a Type-A personality. Focused, driven, goal-oriented, impatient and as temperamental as you might expect of an Irish-Italian with just enough Danish thrown in to keep me manageable. As of July, 2004 I weighed in at 135lbs, 5’2” tall. While I did not make exercise a part of my regular regimen, I worked in a three-story bookstore that kept me quite active, as did my second favorite hobby of ‘home improvement’ (my first favorite hobby being reading!) I loved to tinker around the house and had installed doors, windows, new countertops, refinished hardwood floors, painted the interior, etc all on my own. My brother was living with me and my four dogs (greyhound, Doberman, 2 retriever mixes) in a single-story house, and I had just been promoted to the position of Store Manager of the third largest store in my company.

I have always been healthy, but was also known to ignore signals my body would send me indicating further attention was needed. One example – I once had a cold that I refused to go to the doctor for, despite how debilitating it got, because I just knew I would be told it was a cold and there would be nothing they could do for me. At one point, I remember coming out of a coughing fit to find my brother and a friend leaning over me debating calling 911. I grabbed my brother by his collar and threatened to strangle him. I seemed to kick the cold a week later, then it returned. I finally went to the doctor, who informed me that what I HAD was walking pneumonia and should have been in the hospital, but what I currently had was a cold and there was nothing he could do for me……what is that Alanis song – Ironic?

So in August when I started to have some minor tingling in my hands, I dismissed it as coming from back spasms. In September I attended a national conference where the tingling progressed to numbness and I was informed by my roommate that I must have been a truck-driver in a former life as the pain at night caused some pretty colorful sleep-talking. When I returned from the conference I saw my doctor and asked for muscle relaxants to relieve the ‘muscle spasms’. During this time I also stopped wearing rings as they had become uncomfortable and was beginning to wear the same two pairs of shoes because the others seemed too tight. At any time did I connect the two? Of course not….. The muscle relaxants obviously didn’t work, so I went online to do some research and decided that I had Carpal Tunnel Syndrome. I informed my doctor of this diagnosis (oh, the arrogance!) who referred me to a specialist. The specialist tested me and determined that my symptoms were so advanced that immediate surgery was required as I had already lost some nerve function. He was bemused by how quickly this came on, particularly as there was nothing in my history to indicate a cause. Once again, it didn’t occur to me to mention the other difficulties I was having – including the swelling feet and stiffening back. Among the many tests they administered as a part of their diagnosis, two came back abnormal. One indicated osteopenia (great news when I had to go on prednisone!), the other was abnormal liver function. My primary care physician would go on to give me three more blood tests trying to figure out where these results were coming from. She commented that they read like I had been drinking heavily that morning. All I could do was raise my eye-brows and shrug….clearly, alcohol was not the cause of these results. She told me to return in January for another test to see if it would return to normal (by what means she was expecting that to occur is beyond me).

I had surgery on both hands in November (mustn’t interfere with the retail holiday season!) The relief of pain was immediate, but the swelling progressed, as did the stiffening of muscles. By late December I was using a stool to allow me to fall into bed, where I would lay in the same position until morning as I couldn’t turn over. Using the toilet became an Olympic event as I would need to rock back and forth until momentum would propel me off the seat. Since my toilet is directly across from the tub, I often wondered what would happen if I just kept going one day…..talk about bathrooms being a common area for accidents in the home! None of this caused a major problem at work as I rarely sat. The only issues became my inability to use the stairs without needing frequent rests (not a problem – isn’t that what the escalators and elevators were for?), having to watch what I wore since my pants were mysteriously becoming looser leaving me in danger of giving those nearby a free peep show, and I became quite creative in my excuses for not being able to retrieve dropped items or to get a book for a customer from lower shelves. I told myself it was stress causing tight muscles and figured I would see a massage therapist as soon as the holidays were over. In the meantime, I used my very patient dobie as a lever to get me in and out of bed. Do I need to repeat – type-A personality?? My brother got a job in another state and moved out at about this time. He later told me he wondered if something serious wasn’t going on because I was spending so much time in bed and was not only not working on the house, but was barely even keeping it clean (tough to do when you can’t bend over). I guess my previous threats of physical harm stopped him from asking too many questions or offering his opinion on seeing a doctor……go figure.

I went to a massage therapist who told me she had never seen anything like it before. She couldn’t get the muscles to budge. At this point, the stiffness had progressed to my neck and jaw – I resorted to only eating cereal because I couldn’t get my jaw to open wide enough for anything else. In late January 2005 (holidays finally being over) I finally went to my doctor and dissolved into tears the minute she entered the exam room. I begged her for the strongest muscle relaxant medicine could provide – even offered to go into the hospital to receive it, and (ah-hem) bravely accepted the fact that it would probably cause incontinence, etc. ANYTHING to make the stiffness go away so I could eat, sleep, dress, etc again. Eyes wide, she asked me to perform a couple of physical maneuvers, and when I couldn’t, grabbed me and RAN down the hallway to x-ray then pulled 4 vials of blood. She informed me that I did not have muscle spasms, that she had 80 year old patients with better mobility (have I mentioned I was 35?) and that she thought I had a problem best handled by a rheumatologist. She told me what she thought it might be (I can’t remember the term, but when I looked it up online it said it usually occurred in those in their 80’s.)

I called around, and based on her referral was lucky enough to get an appointment with one for three days later. In the meantime, I finally mentioned to my family that I was having some physical problems, and would be seeing a specialist. As I have a nephew who was born with an extremely rare genetic condition, this prompted more concern from them than I was feeling. My sense was, well – as long as they know what is causing this, they will simply ‘fix’ me and all would be normal. After all, I had always been perfectly healthy, right?

Now enters the Rheumatologist – and after having read the experiences of others with myositis diseases, I recognize just how lucky I was to have made the appointment with this man. He already had my blood test results for the initial visit and began the assessment with many questions as to what I was and was not able to do physically. He peppered his questions with terms like ‘muscle inflamation’ and ‘muscle damage’. At one point I asked if this could be behind my Carpal Tunnel and swollen feet. After he recovered from his shock that I had had the surgeries (I’m sure it was only his professionalism that stopped him from commenting on my stupidity at not fully informing my doctors of ALL physical symptoms) he commented, and I quote, “What I believe you have is a disease and is very serious”….then went right back to the questions!! I sat there wondering, now what the hell does THAT mean? Am I dying? He finally began explaining Polymyositis, complete with diagrams written on the paper covering the exam table. He informed me that my ‘abnormal liver results’ were actually due to my muscles deteriorating and explained what CPKs were and why having levels over 15,000 were a ‘very bad thing’ (normal ranges 40-200, with 200 equating to someone who just finished a marathon). And thus began my education on an auto-immune disease so rare the majority of doctors are unaware of it.

In astonishment, I exclaimed “but, that’s what my dog died of!”. Instead of reacting like he was dealing with an idiot, he was actually interested in this bizarre factoid and asked if I was sure it was immune related. After $3,000 in vet bills from seeing a variety of specialists, I was VERY familiar with the diagnosis and was able to assure him that my golden retriever had suffered from muscle atrophy caused by his immune system and that he died at the age of 7 due to his allergy to the only treatment available…..steroids. THIS is what finally got through to me that maybe this wasn’t something that they were going to be able to fix and allow me to go right back to my old life.

I was placed on 60mg/day of prednisone and he explained that this dosage meant I would experience all side effects. I was somewhat less than thrilled to discover this meant my already round face was about to get rounder, hairy and I would finally experience acne. Oh, the joy. Then there is the osteoporosis. Even more thrilling was hearing that moodiness and episodes of anger would be expected (I wondered if my staff would resort to a rope, a blade or a bullet if I got any more temperamental). In the meantime, I was scheduled for an MRI and a muscle biopsy to confirm the diagnosis he was 90% sure of. He tore off the paper from the exam table at my request so that I could pass the information on to concerned relatives, and so began my habit of maintaining a personal copy of ALL papers and test results….a habit I highly encourage for others who need to keep track of medications, test results, treatments, bills, etc. I keep this all in a portable file and take it to all appointments. There have been a number of occasions where I was able to provide information that had not reached whatever office I was at.

One tip for anyone going in for an MRI…..TAKE THE VALIUM!!!! I have never been claustrophobic, so I rejected the offer the first time around and came out crying, sweating, swearing and swinging. I think if I hadn’t asked for it at the second session, they would have forced it on me for their own safety. By this time, the prednisone took effect by relieving the muscle inflammation giving me an immediate sense of relief – no more pain, no more stiffness. Yippee! This won’t be so bad! I assured my mother (who was already making plans to fly up from Texas) that this would be a breeze, and not to worry about me. I would handle this fine on my own, and besides, I hadn’t been able to thoroughly clean the house in weeks and was horrified at the idea of her seeing this (I actually called my sister-in-law in tears at one point begging her to convince my mother to not come. She responded with – she’s a mother, do you really think anything or anyone is going to stop her??) Facing the inevitable, I hired a cleaning crew who neglected to wash the walls in the one room I was most concerned about – the one my mother would be staying in.

Now, you must be wondering, why on earth is this woman so obsessed with cleanliness – washing walls, for heaven’s sake?? I had FOUR dogs and live in SEATTLE. Just imagine how much damage a wet, wagging tail can do – then multiply that by four. I decided to take care of the walls myself the easy way – I would simply use a mop to wipe them down, then paint them. After all, the rollers come with poles so I could do this all standing up with minimal effort. Well, that plan was just ducky till I realized that I would need to get to ground level to go along the baseboards. This was the first time I was going to ground level. Up to this point, I had been able to avoid kneeling, and hadn’t experienced any falls so I figured I would just use a stool to get back up. I got down ok, but then discovered I didn’t have the strength to roll to my side, much less sit up. I decided that since I was down there already, I might as well finish painting. Then I lay there contemplating being stuck there for 48 hours and having my mother arrive to find me laying like a turtle on my back. Somehow, that gave me the strength and ingenuity to use the bed, a very patient dog and a stool to finally get myself back on my feet.

One week on prednisone, and the last of the swelling was gone. I could now fit in my shoes and could wear my rings…..but it also took away what had apparently been supporting my muscles. I also dropped a few more pounds – I was now 20lbs lighter. Normally, this would thrill me except for the knowledge that it was muscle weight I lost….To my staff it appeared as though I went from being perfectly healthy, if a bit moody, to being on death’s door. I couldn’t walk without a cane, slurred my words, my head would list to the side and I could only walk a few steps before needing to rest and I had lost enough weight to appear rather frail. One of my department managers had been on vacation for a week – when she saw me, she burst into tears. I remember thinking, jeez – do I look THAT bad? Apparently, I did. Everyone, from my mother, my staff and my boss urged me to take disability. I couldn’t imagine doing so – what on EARTH would I do at home all day….watch TV?? At least at work I could continue to use my brain. This began a major battle with my boss who was understandably concerned about having the third largest bookstore in the company being run by someone who appeared barely functional. I won’t go into the ugly details, but this was one for the books as I am sure that the laws protecting those with disabilities are not often used to stay OFF disability. The tension between my boss and I is regrettably still present, and is a stressor I do not need but can't avoid if I want to stay in a job I love.

By this time it was late February 2005. I was still on prednisone alone while we were waiting for test results before proceeding with treatment. My mother (who is an amazing individual) arrived and went into action.
While I went online to research the disease, she went online and began ferreting out all sorts of tools to help me function around the house. These items can be found online for half the price you would find them in a medical supply store, and most of them came from QVC. For those who might find these items useful, here is a list in no particular order:

1) Gopher – I put one in every room in the house, one in the car and one at work. It allowed me to pick up dropped items, dress by stepping into my panties/pants then using it to lift them to within reach. (QVC or Walmart)
2) Sock-puller-uppers (don’t know the official term – got it through a catalogue)
3) A surprisingly attractive and easy to slide in bedrail (QVC)
4) Bed Wedge (for that lovely reflux I couldn’t control as the disease had also taken it’s toll on my esophagus) (JC Penneys)
5) Toilet bars (online medical supply store)
6) Sliding bins for cabinets. I could use the gophers to pull out the bin, then to pick up the item I needed (Lowes)
7) A scooter (not covered my insurance, unfortunately) that allowed me to get around at work…..and enabled me to NOT go out on disability. (you can get one of these on Overstock.com for under $1000 or on loan from some MDA chapters)
8) Silky ‘granny gowns’ for sleepwear that made sliding in and out of bed easier than my t-shirts had. By the way, this also led to my first ‘prednisone induced’ fit in the middle of Sears. Instead of anger, my side-effect seemed to be tears. I had completely balked at the idea of paying for the silk gowns available at Nordstroms (we had already been there and put me into sticker shock picking out some shoes I could slide into that were professional, but had rubber soles for better traction).There I was, crying over how ugly I thought the choices were surrounded by these shoppers who probably thought they were the height of fashion while my mortified mother urged me to just grab a couple. After all, who was going to see me in them? NOT a comforting comment for a single woman in her mid-30’s. I leave the rest of the scene up to your imagination.
9) “Help! I’ve fallen and I can’t get up!” service. I hated this one, and it proved to be a pain as I inadvertently set the button off twice causing needless excitement in the neighborhood when the fire department came to call. We finally got around the problem by placing the button inside a small ring box with the cord hanging out. For those who don’t live alone, I recommend a wireless doorbell, available at Radio Shack. You can wear the button around your neck, and if you fall or run into difficulty push the button to ring the bell and alert whoever else is in the house.
10) 4-prong cane (not as likely to drop it....after all, it's not much use if you can't pick it up!). Great for short distances. (online medical supply)
11) a wheeled walker with a seat and a basket - invaluable for shopping or longer walking distances. (Used my grandfathers...but I would go to an online supplier for this)

She also helped reorganize the house so that critical items were within reach. Unfortunately, it was at this time that I had to find a new home for my greyhound. He was the only dog too large to use the dog door and he had the regrettable habit of assuming every time I woke up it was time to eat and would make a ruckus. Anyone on prednisone knows that sleep is a real issue, and the issue became critical when my 2am restlessness meant not being able to go back to sleep. This was heartbreaking for me – I was used to rescuing dogs, not giving them up. My poor mother had to suffer the sleeplessness along with me as the prednisone was giving me energy that led to me doing odd things at 2am. It drove her crazy - she knew I was supposed to be conserving energy, and there I would be in my glamorous polyester gowns spray-painting trim in the garage ... trim that I couldn’t even install in my current condition. Oh, well. It’s not like the paint splatter could make those gowns any less attractive…. :-)

While my mother surfed the web for useful tools, my web-surfing led me to a site run by a woman in my area who also had PM. I emailed her, and she became an invaluable resource. She referred me to The Myositis Association and recommended contacting the Muscular Dystrophy Association, as well as offering her own experience to help prepare me. It was her very warm emotional support that truly made a difference.

TREATMENT PHASE

So – I now know what is wrong with me ------what next??

I went to the MDA whose neurologist confirmed the diagnosis and set me up with another doctor who was in charge of setting up the occupational and physical therapy. I would give you his title, but I never did figure it out. This guy was something else. I left my first appointment in shock and told my mother it was the first time I had ever left a meeting completely unsure of what exactly had taken place. All I knew was that I had appointments set up with people who were supposed to do something about teaching me to drive (where did THAT come from???), how to use my cane (again -?? was there a secret trick to it?) and (I hoped) one to help improve my flexibility, which he felt was at a critical stage – if not dealt with the damage would be irreversible. He was a lovely man – just the image of a brilliant, if absent-minded professor. He was so eager to ‘help’ that he actually acted offended that much of what he was ready to offer on behalf of the MDA my mother had already found. The one item we didn’t have that he felt was essential was a lounge chair with a lift. I was horrified – where on earth would I fit this huge chair that I would never sit in? Fortunately, his assistant told him there was a waiting list and when one came available my mother caught the call and was able to tell them to move on to the next name on the list.

The occupational therapist saw me once and told me there was nothing she could offer that my mother hadn’t already thought of. I was still able to drive, albeit very carefully as my hands couldn’t reach past the bottom of the steering wheel (I guess there are alternatives if regular driving is a problem?? Still don’t know what that whole ‘driving lessons’ thing was about). I began seeing a physical therapist who worked wonders on getting my jaw working again. She said if I had waited any longer it probably would have been stuck that way.

In the meantime, I was not responding as expected to the prednisone treatment (my CPK levels were remaining above 10,000) so my rheumatologist decided to go for broke and throw everything he could at me. He started by sending me to three days of Solumedrol treatment, which really knocked me on my butt. I started on Methatrexate injections and the first of 4 IVIg treatments. I was at my lowest point from a physical perspective during February/March. It was during these months that I had to rely on aids and struggled to even wash my face. I had one fever incident in the middle of the night that left me so achy the next morning that I decided to try a bath. An experience I will never repeat. At one point, as my 60-odd year old mother struggled to help me out of the tub as I slipped and slid about knocking my head on several occasions, she commented that she thought it would be me doing this for her – not the other way around. You learn quickly to lose your modesty when dealing with a chronic illness…….

I was fortunate that I had very few incidences of falling, but each were rather remarkable – and I don’t use that term lightly.

The first came at a time when my uncle and his girlfriend had come down to help with some of the house rearranging. The two of them drove 3 hours on several occasions to help- talk about family love! On this visit they brought their beagle with them. As I am a dog-lover, this would not normally be a problem. However, one of my dogs is EXTREMELY dog-aggressive. Example -I was walking him one day and a neighbor’s rottie got loose and came over to visit. Teddy kicked the crap out of him. I have never walked him again. This little fart will sit there and wag his tail looking as friendly as can be until the other dog gets within reach, at which point he will launch an all-out assault with no warning. I blame this on his months living on the street, probably fighting for food. When I first brought him home he was incredibly thin and he actually chewed his way into the plastic bin holding the dogfood eating so much he had to go to the vet…..he couldn’t even walk! He is the sweetest thing with my dogs, kids and adults…..but NOT with strange dogs.

So, the poor beagle got tied up out in front of the house all day, while Teddy salivated at the gate. My mother left to get pizza for everyone, and somehow Teddy slipped out and immediately went for the beagle. My brain fired “RUN” and my body responded “Yeah, right!” and I wound up flat on my face on the garage floor. My poor uncle was torn between helping me and saving his dog. I kept yelling “Get Teddy!” so he went to help his girlfriend pull Teddy off their dog, upon which time Teddy made a dash for the street where some poor woman was walking HER dog. A neighbor took a shovel and began hitting Teddy (which I fortunately couldn’t see, still being face down on the concrete). While all this was going on, my mother pulls up in my car, spots Teddy and somehow gets him back in the house (I have no idea how that happened). Next thing I know, I have a crowd standing around me debating how and whether they should get me back on my feet. While they are debating, the same neighbor with the shovel walks up, grabs me under my arms and just plops me on my feet then leaves. I’m in hysterics worried that Teddy had harmed one of the other dogs (he hadn’t , and neither did he suffer any injuries from the shovel – I swear I should have named him Terminator) while my family is completely freaked out over my broken glasses, black eye and bruised ….well, bruised everything. With this disease, when you fall its like a cut tree – you just go straight down, being unable to brace yourself in any way. They were also alarmed because this was the first time they were able to see just how limited my movements were. I wasn’t even able to turn my head, much less my body. All in all – not my best moment.

A few days later, I was leaving for work at 4am, Mom was still in bed and I tripped over some yard waste a neighbor had left after helping clean some of the winter debris. This time I fell on my back. After calling for my mom several times with no result, I thought to push the alarm button on my keyless car remote. I did this several times. My mother didn’t respond – she thought I was doing it accidentally. However, my poor neighbors did. The same neighbor had to come out and get me back on my feet. I thanked them with as much dignity as I could muster, and took off for work. I planned to arrive at work and hour before the rest of the staff to catch up on some paperwork. When I got to the doors, I dropped my keys…this led to the debate of whether to wait an hour for more people to show up or to try and retrieve the keys using the door handles as a support. Would it surprise anyone to know that I chose to try and retrieve the keys? The next thing I know, I am on my knees clinging to the door handles unable to get up OR get the keys. I began calling for help hoping SOMEONE might be at the center at that early hour. Finally, a man approaches. After spending a moment contemplating the picture I made – a strange woman apparently worshipping at the portals of a major bookstore, he asked what the problem was. I explained that I had a condition that led to the “Help! I’ve fallen and I can’t get up” syndrome. He got me to my feet and asked me if I thought it was a good idea to be going into the store alone with my condition. Embarrassing, but true… Perfect strangers have better sense than I do when it comes to this disease.

My final fall was actually an exciting experience for me. It happened after my mother had returned home (much to my father’s relief) and I had been on all three treatments with promising results. I got up at 2 in the morning to answer the call of nature and promptly fell over poor, blind Heidi who had inexplicably decided to go to sleep stretched across the hallway.

I landed squarely on my forehead, which left me with a fairly spectacular knot that started between my eyebrows and extended to my hairline at each temple. But the exciting part was that I was able to get to my knees and crawl to a chair and get to my feet. THIS was an accomplishment! Forget the bruise – I got to my feet all by myself!! Time to celebrate! Believe me, this was something to hold onto when I went to work and had to watch employees struggle between hysterical laughter and sympathy. The knot remained and the blood pooled around my eyes. I looked like a Klingon who met up with the three stooges and got the double-eye poke. Between the black-eyes, the Klingon forehead, and the moon face I had to stay away from customers (particularly the children) for a few days. I was quite a sight.

It is now June of 2005. I am currently on 30mg/day of prednisone, methatrexate injections, have had 2 IVIgs and my last CPK came back at just under 2000. I take vitamins C, D and calcium; prevacid for the reflux; actonel for the osteopenia/prednisone side effects; folic acid and a couple of other supplements as well as something to help me sleep. I no longer need aids for dressing, walking, getting up or down from the floor, etc. I still tire if I push too far (can’t seem to avoid those 10-12 hour days some weeks) but am hugely thankful for how quickly the treatments took effect. Long-term treatment is still in question. Perhaps I will update later……..

Long post, but I know how valuable it was for me to hear of others' experiences with this rare disease and hope that this might provide some insight for anyone newly diagnosed.