This is the first of a series of personal stories from patients and their families who have been diagnosed with Churg Strauss Syndrome. Our disease is very rare and these stories remind us that we are not alone. If you have a story to share please contact Cindy, who is the "In Our Own Words" editor. Special thanks to Maureen for so eloquently sharing her story!
Churg Strauss Syndrome: I have had that "label" since I was 20 years old. Over the past 30 years it has come to mean different things to me - some good and some not so good.
My history began with an initial diagnosis of Henoch-Schonlein Purpura when I was 15 years old. Not much was known about autoimmune diseases back in 1970. Prednisone was the treatment, and for me, it worked wonders. Being young at the time and having the feeling of omnipotence (as most teens did then), I felt being sick was a nuisance. In hindsight I realize it deserved more respect than I gave it. The diagnosis was made after months of painful joints (mostly knees) and flu-like symptoms. During that time, things progressed to the point where I was in bed most of the day due to the pain and tiredness, and it seemed I threw up most of the things I tried to eat. My hands became swollen to the point that I wondered if the skin would be able to stretch any further. My feet hurt, and walking became an exercise in graceful hobbling.
Eventually I was hospitalized for a month. I remember even the touch of the bed sheets hurt, and so I had a "chuck wagon" over my hospital bed to keep the sheets off my legs. (The chuck wagon proved to be a great hiding spot for my forbidden transistor radio.) I had a few stuffed toys in my room, but because no one was quite sure what was triggering my symptoms, even they sat on a shelf across from my bed in plastic bags. By this time I was developing purpura and I had kidney issues (blood in my urine). Eventually I was put on prednisone, the symptoms subsided, and when I was able to eat again, I went home.
I remember my first days back at school - it felt so foreign. The feeling surprised me. Without even trying or noticing, my life had slipped into being something that was very different from what it had been. I guess the child in me liked the attention, but the older "me" knew it was a difference I really did not want.
They say for every door that closes, another one opens, and it was years before I would realize the gift in the adversity. I learned about medical life pretty quickly, but I was a stubborn student when it came to lessons of life. I fought and pretended to be as I was before getting sick. I went to school, worked part-time, and went out with friends, behaving like other kids my age, but it always came with a price - my joints would hurt or my feet would be bruised the next day and my stomach would bother me. Prednisone had side effects that just seemed to compound the innate insecurity everyone seems to experience in their teen years. Looking back, I felt like I was always strapping on the armour to go to battle with whatever might be causing me frustration: anything that stood in my way as I fought to be "normal." It took me years to realize that when you go head-to-head with Life, that Life usually wins.
Back then I didn't spend too much time wondering about self-blame. I was pretty sure that it was all a big mistake - the doctors were wrong, the tests were wrong, and prednisone was wrong. I felt like a victim most of the time, bouncing between tears and anger for many years.
Thankfully (for everyone's sake) after about four years, the symptoms went away. I was no longer taking prednisone and I thought that I had won the battle and the sickness was all behind me. My life could now finally start. It was the fall of 1974, and I enrolled at a university with great plans for a wonderful future. Everything seemed to be the way I thought it should be.
By the end of my first year at the university I had developed sinus problems, as well as other strange symptoms. My eyelids would swell up, and sometimes I'd wake up to find bruising on the whites of my eyes. My joints were getting a bit stiff, my feet would swell and hurt after standing for periods of time, and I was falling asleep in the school library because I was feeling so tired. I saw a specialist about my sinuses, and had myfirst polypectomy. About two months later I was back in his office - the polyps were back, and I had developed a dull aching pain in the middle of my back, and sometimes it hurt to take a deep breath.
A chest x-ray showed a shadow on my right lung, and I was put in isolation in the nearby hospital. They suspected tuberculosis, as another student at the university had been diagnosed with it, but after almost a month of testing, my test results still came back negative. I had another polypectomy, only this time I was told the polyps looked like black fudge. The questionable area on my lung had started to enlarge, and I was sent to a bigger hospital in May of 1975 and put under the care of a very well-respected haematologist. Still in isolation and still more tests. After about two weeks of new testing, it was decided that I should have an open-lung biopsy done. Again, I was very lucky to have had the best-of-the-best perform the operation. I remember the surgeon coming to my room at about 1 o'clock in the morning to talk to me about my fears of surgery. He had been doing research work all evening and before he headed home from his own day, he came to see me. I couldn't have asked for more caring people. I wish I could turn back the hands of time to show them more appreciation than I did. Still being young and inwardly angry that God was picking on me (but I was too scared of Him to tell Him), I wasn't a very good patient. As far as I was concerned everyone was wrong and I was right -my world at that time was very black and white. I couldn't accept the reality of being ill. Deep down, though, I was very very scared. I guess I thought the only way to deal with my fear was to act like I was the stronger opponent in the battle; to get all big-looking and hissy like a kitten that meets its first puppy. That, of course, was when I wasn't crying about all that I didn't understand.
Surgery was long. I remember waking up in intensive care with all kinds of machinery around me. I still remember seeing my parents when they first walked through the door as I lay there. How I wanted them to just take me away from it all. I have since learned that it is much harder to be "beside the bed" feeling helpless, than to be "in the bed" yet still feeling the will to live inside. I could never thank my family enough for all they have endured and pulled me through. I only hope somewhere in me that I have their strength and patience.
To my knowledge, a piece of lung tissue was sent to Oxford University in England, Harvard University in the United States, and another piece was kept for pathology at the University of Toronto in Canada. The diagnosis came back as Churg Strauss Syndrome. I remember being told they knew of one other lady with something similar and she was in England.
While waiting for pathology reports, things became complicated. I developed a high fever. The wound from surgery had become deeply infected, so the stitches were removed and the area was packed with gauze soaked in saline every day. This was done for almost three months before the wound closed. I had developed deep sores on my ankles from rubbing them on the hospital sheets (a reaction to the anaesthetic) and they had become infected with staph. Then I developed a clot in my leg and was on heparin. The tensor stockings pressed on my sore ankles and made them hurt even more. All this happened during my second month in the hospital, and to this day there are people that visited me in the hospital that I do not remember seeing. My weight dropped from 95 pounds (I am only five-feet, two inches tall) to 69 pounds. I went for chest x-rays on a regular basis during that time. I remember fans were the only air-conditioning, and the summer heat made the hospital uncomfortable. I remember having a styrofoam cup of ice-chips beside me to melt in my mouth when I lay on my gurney in the basement hall of the hospital waiting my turn for radiology.
I was started on a high dose of prednisone and things started to resolve. Years later I was told my only other option at that time was dialysis. I learned to accept prednisone a bit better, but I still hated it. Years later I learned I didn't hate prednisone, I hated that I was sick and had to take it. Prednisone was not the enemy.
I was finally discharged from the hospital after three months. My parents took me home in their car as I slept on top of a sleeping bag in the back seat with (finally) my own pillow. Going up the stairs to my room at home was a new challenge. My legs were not used to climbing and I found I was still pretty weak. A nurse used to visit every day to pack my wound with fresh saline-soaked gauze.
After about a week at home, my right lung collapsed. I was told prednisone can retard the healing of some tissues and I guess that in my hurry, I had pushed too hard trying to gain control of my life once more. Again I was hospitalized. Again I had a chest tube inserted. I only stayed about a week.
How I wanted everything to be the way that it used to be.
During the summer, I thought about whether to return to university or not. I was feeling pretty sorry for myself and it would have been easy to just give up and live under the label of chronic illness. At one point in the hospital I had a roommate that was about the same age as me. She was from another part of the country and had been taking courses to be a physical education teacher. She was hospitalized after she had developed symptoms of cystic fibrosis at the age of 20. Even after I was moved to another part of the hospital we talked to each other. I remember calling her room to see how she was doing. She never survived her surgery. I cried for days. I just didn't know how to comprehend so much illness and death - we were still so young. During those days, I realized that even though she had been so much sicker than I had been, she still hung onto her dreams. I decided to hang onto mine, too. Thirty years later and still I think of her courageousness.
I went through many years of follow-up tests and prednisone. I graduated from university (prednisone made reading and comprehension difficult, and my marks dropped quite a bit) but I became a designer. I love art. It has held me together for as long as I can remember. Sometimes I paint what I wish my life was like, and sometimes I paint what my life is like. Sometimes I paint what makes me happy, sometimes I paint what makes me sad. I guess I am lucky to have a gift that enables me to get my feelings out when they are beyond words. When I don't draw, I write or I just sit quietly and imagine my way through the confusion.
While I cannot change some things in my life - like my diagnosis, I can remember what is still a part of my life.
In 1980, I had one more sinus surgery that has left me with chronic sinus infections. As I learn from reading other Churg Strauss patients' histories, I now wonder if that is just part of the way life is for everyone with Churg Strauss, regardless of surgery.
Ten years later, in 1985, after many years of stable tests, I was able to stop taking prednisone. I still have the letter from the doctor to confirm my test results were good. Again, I was surprised at how I felt. It was like someone had given me my life back. I guess I had become so used to having Churg Strauss and prednisone attached to my existence, that to know anything different was a freedom I could never have imagined. I had started working as a self-employed designer, and was settled in a new home that I had bought on my own after much saving. I felt like I needed to get my life established despite my unknown future with Churg Strauss. Life was going to be on my terms at last.
Two years later, I developed pneumonia. I had just started working full-time at a great job (the self-employment proved to be a bit too stressful for my worrisome ways) and I was home sick for about two months. Things got worse. I used to find that as soon as the sun started to set, all my joints would stiffen up and they become very painful. I was having trouble breathing, and I was losing weight again. I remember having to drag myself up the stairs to bed, sitting on one step at a time because to bend my legs was just too painful.
Again I was hospitalized. Again my eosinophil count was very high. I was bed-ridden for another month. Lying in bed, I had pillows under my arms and legs. It even hurt to cry. I was tested for asthma, but no diagnosis was made. I was tested for many other things but my situation was still considered to be Churg Strauss Syndrome. I was put back on prednisone. I felt defeated . . . and angry at Life. I felt it unfair to have to endure this "punishment" from Life, but I pretended to be positive, because I wasn't quite sure what Life would do to me if I rebelled too much.
Looking back, I should have fought less to stay off prednisone, and fought more for my physical well-being. I lost lung function and a year later I was finally diagnosed with asthma. That diagnosis bothered me more than Churg Strauss. I knew what asthma was from being hospitalized with asthmatic children and Churg Strauss was still an invisible monster that I couldn't fully understand, as there was little literature available to read about it.
I froze. I stopped walking and running, trying to hide from a possible asthma attack. (I have since learned, after being in respiratory rehabilitation, that walking is probably the best thing I could have and can do for my lung function.)
Still, I fought to have my life back. This time I just avoided anything that made me feel "different," and as such I started to isolate myself from the outside world. It was all I knew to do.
I am now 49 years old. My lung function is not very good. I still work, though. I own a beautiful home in the country with a huge flower garden to keep me busy, and lots of wildlife to remind me how wonderful it is to be alive. The last few years have been a struggle - things flared up again and I guess the age-and-time factor makes it harder to pretend everything is okay. In some ways I feel I have run out of time to make things better. I have other medical problems now, and I take more pills than I wish I had to.
Last year was a challenging year for me. I suffered many losses. I lost myself, too. Looking back to that time, I believe it was inevitable. Everything had become too much. It took more energy to ignore reality than to accept it. Thankfully, I have at last come to respect Churg Strauss, and have realized how it has made me the person I am, and how I am still a person despite it. I am not perfect. I am human. Not a day goes by that I am not grateful or in awe of what Life holds. I owe much of that to knowing what it is like to not be able to enjoy it sometimes.
I once read that a person cannot grasp the concept of Light without knowing the concept of Dark as well. I may have lost the dream of what I had hoped my life would be, but I have in its place an appreciation that will always be with me despite illness and age. No blame. I may not be able to walk as far as another person, but I am content to sit in my own company now. I may not have a body that amounts to much anymore, but I believe I have the ability to understand so much more. No shame. In the paradox, the physical weakness I feel from having Churg Strauss has made me stronger in so many other ways.
Churg Strauss Syndrome is only one part of who I am - there is a part of me that it cannot take away. That is what I now fight to keep and to remember each day. It is not easy. I fall down a lot emotionally. My beliefs have been put to the test many times over the years, and I am still not sure what watches over us. I feel "It" is too big for me to comprehend. I like to think that all things live in balance, and when we inevitably go through "bad things" there is always the "good things" to help us through. Control is just an illusion. We can only do what we have to do to survive and hang onto our hope.
My own best friend and my own worst enemy is the same: myself. Only I can decide which to have with me in my life, but I really do think life is better with a friend.