These are personal accounts from people affected by CSS.
By Cindy Smoot, VF Education and Awareness Council
January, 2007
Our family has been dealing with Churg Strauss (CSS) for many years now. My 13 year old daughter, Lauren, has CSS. Although CSS has been a part of our life most likely since Lauren was just a baby, it took many, many years to unravel the mystery of what was really wrong with Lauren and to finally get a firm diagnosis.
Lauren's problems mostly started with bad asthma, or what seemed "asthma". Actually, if I go all the way back her problems really started at birth when she had a systemic bacterial infection with group B strep, and also developed pneumonia as a neonatal. Lauren was given a 50 percent chance of surviving the night she was born. When she finally recovered from these life threatening infections, she was very weak and left with fragile lungs prone to infection. This turned into chronic infections and horrible asthma into her toddler years. We took the nebulizer everywhere we went, because her asthma was so severe and unstable. The asthma became markedly worse when Lauren was around 3 years old, and she would have severe attacks several times daily. Looking back, I cringe to think how bad she was, when I should have had her in the hospital. We would do treatments non stop for hours every day just to keep her breathing. It just became our way of life for everything to revolve around asthma treatments.
By the time she was five she had constant sinus infections that would not clear, and then had her adenoids out and her first sinus surgery at seven. That was also the year we started seeing her current asthma/allergy/immunology specialist, who still treats her. He knew how severe her asthma was the first day he saw her, and that day is when she started daily prednisone (steroids), and has been on it ever since then. That began my love/hate relationship with steroids, I loved how they helped her, but hated what they did to her with side effects. Even on steroids her asthma did not get better as it should have. She kept getting pneumonia, by that time she had had pneumonia over 10 times. Her stomach killed her all the time, she vomited all the time, and things just seemed to be getting worse. Her GI problems finally put her in the hospital for 2 weeks in Feb of 2002 when she was 8 years old. We found she had multiple bleeding ulcers (from steroids), and that is when they did a surgery called a nissen on her to tie off her stomach, to keep reflux and vomit down. She was better for a month, and then got pneumonia again.
I was so tired of her being sick, and tired of all the specialists, so I just went to her good old pediatrician, (who had guided me so well all these years), and asked him what in the world was wrong with Lauren. He told me to start over, and go to a pulmonologist and just focus on the lungs, as all her other problems seemed to stem from that. So that is what we did, and our first visit with that pulmonologist changed our lives. He immediately told me "I don't know what she has, but it is not asthma". He sent us for many tests, including her first CT scan, then in June 2002, and that finally showed the white patches all over her lungs, called ground glass infiltrates. This appearance is usually what is seen with a fatal lung disease called pulmonary fibrosis, and he was very worried. I got "the" phone call that the tests had come back and it did not look good. She was immediately scheduled for an open lung biopsy, and while she was in ICU we heard for the first time the word eosinophilic. The pathology showed these eosinophils were infiltrating her lung tissue and jam packed in her airways; she was diagnosed with eosinophilic pneumonia. Her blood counts with these eosinophils were also very high. We thought we finally had the answers. Little did we know we were just beginning this long walk that to this day is still unclear. She began massive doses of IV steroids, 750mg a day. She did horrible with these huge doses of steroids. She went into psychosis, and developed fluid on the brain, and her doctor was concerned because her CT's of her lungs were not improving.
At this point I started researching and reading everything I could get my hands on about eosinophilic lung disease. It did not take very long in that research to come across articles about Churg Strauss. I knew when I read the first article I saw that mentioned CSS, that was what Lauren had as it matched her symptoms perfectly.
Her doctor wanted us to see a more experienced physician who had dealt with these diseases before. So we began taking trips to experts in eosinophilic diseases. We went first to a specialist at Texas Children's in Houston. This doctor disagreed with the numerous pathologists and radiologists who had studied Lauren's results, and even with physicians he asked for opinions at his hospital. That left us all very confused, so the very next month her doctor at home sent us to National Jewish in Denver. We stayed there for over 2 weeks. It was there that we learned there was more to this than just lung disease. They discovered her auto antibodies were high, and she still, after all the months of huge doses of steroids, had very high amounts of eosinophils in her lungs. They found them in her sinuses too when they did her second sinus surgery, and they also were the first to realize she had nerve problems, with neuropathy in her legs. While we were at National Jewish we were told it looked like Lauren could have Churg Strauss, but that they did not know of any cases ever reported in a child. Lauren turned 9 the week after we returned from National Jewish.
A few months after going to National Jewish, the pulmonologist was unsure of what to do as Lauren began to decline in her lung functions. So I went back to that same immunologist we originally saw before her lung disease was found, then in the spring of 2003. During that time she began having more GI issues, and was found to have gallbladder problems, so it was removed and also a nerve biopsy was done to confirm if her strange feelings and pain in her legs was neuropathy. The biopsy showed she had active and chronic nerve degeneration of neuropathy. She then had three organs involved; lungs, sinus, and nerves, and it was suspected that her GI problems were linked to eosinophils too.
Her immunologist then consulted with her rheumatologist, and they both thought she had Churg Strauss (CSS), but wanted to confirm it with the doctors in Boston who had actually seen another case in a child. So yet another trip, that summer of 2003. In Boston we met a CSS expert, who turned out to be a great help over the years. We also met with several other pediatric doctors, but they never did quite get it because Lauren looked normal. I have learned when you are really sick, and looking good, it is not always a good thing. They literally said they could not believe that she was the child that the records were about. They showed us her CT's, and talked about her pathology and said it seemed to show severe disease, but they could not think a child could look that good and still be as sick as her records showed. So we came home with no real answers or help from Boston.
During this same summer Lauren stopped her steroids for the first time in 2 years for a couple of months and her blood counts shot up very high, she got serious infections in her lungs, and her lung disease worsened. We tried her first chemo drug. She also began having terrible stomach pain, nausea, and vomiting again. Her doctors worried she had bleeding ulcers again, but when they would scope her and take biopsies not much would show up but some nonspecific chronic inflammation. After a short while she went back on steroids, and has not stopped them since. Her lung functions slowly improved, but her GI problems persisted.
It was that next spring of 04, that her immunologist went to a conference and heard a doctor from Cincinnati Children's Hospital speak about research they were doing into eosinophilic diseases, especially eosinophilic GI disease, and he thought we should go see the group of doctors involved in this research in Cincinnati. That year Lauren's GI problems became horrible and unexpectedly her lung disease began to get somewhat better. So that summer Lauren and I went to Cincinnati, and we finally found a place that really understood these eosinophilic diseases. They told us her pathology looked like it was from an autopsy, and Lauren had an amazing ability to go on and function despite overwhelming disease. They found all the years of stomach problems, that had been looked into five times with biopsies were eosinophilic inflammation in her GI, it was all the way through her GI tract. She now had four organs involved. In Cincinnati, they also thought Lauren most likely had Churg Strauss, but again they said it was almost unheard of in children, so they could not be 100% certain without a specific finding of vasculitis on biopsy, which is the final stage of CSS. We continued going to Cincinnati Children's every three months for two years, to help guide her doctors at home treat Lauren's rare condition.
The GI disease has been very hard to control, and in January 2005 Lauren had a severe flare again in her stomach, despite treatment with steroids and Methotrexate, another immunosuppressant drug. At that time Lauren did daily IV steroids in a high dose for several months. She can no longer tolerate high dose steroids by mouth; they are too hard on her stomach. Unfortunately, the constant use of her central line led to Lauren getting sepsis, which is a very serious, systemic blood infection. This infection was a huge setback for Lauren that took months for her to recover from. When she finally completely recovered from the bout with sepsis, she began feeling much better after all the months on high dose steroids, but was having terrible side effects. She had developed cataracts, osteoporosis, Cushing's syndrome, and looked nothing like herself anymore. Her doctor began to try and lower her steroid dose. After a few months she began having symptoms again in her stomach. In December of 2005, she spent 3 weeks in the hospital unable to eat or drink without vomiting. At the same time she began to leak urine, and lost the reflexes in her legs. Her neurologist thought she was having progressive nerve disease from CSS, and had developed autonomic neuropathy, which affects the way your organs function, and can cause a paralyzed state in your GI tract called gastroparesis. The next month at the end of January 2006, Lauren could not be woken up for 24 hours, she was incoherent, and lost control of her bodily functions without being aware of it. She would fall asleep mid sentence while talking. Again her neurologist felt she was having serious progression of CSS vasculitis into her central nervous system. Several tests were run, a MRI of her brain and spinal cord, but they were normal. With CSS affecting small vessels it is usually not found on MRIs.
This past summer of 2006, the doctors we had been seeing for 2 years in Cincinnati felt too much was going on with Lauren, and she needed more immediate care close to home. Again her team of doctors at home felt she needed an expert to guide them, as none of them had ever had a CSS patient. So it was decided to then send us to an adult expert that had actually seen and treated CSS. All the doctors we had seen before were pediatric specialists, and although they were experts in eosinophilic disease, none of them had a case of CSS in a child. So we finally went to the Cleveland Clinic, and saw Dr. Hoffman, one of the leading vasculitis experts in the world, and he confidently diagnosed Lauren with CSS. We will continue seeing a team of doctors in Cleveland every 3 months until Lauren is well into remission.
Lauren continues to have a very difficult case of CSS, particularly with her GI tract involvement. She continues to flare frequently, and it is very hard to keep her disease controlled. She has one of the more severe cases her doctors tell us, and we have been told by most doctors she will most likely need life long treatment. She has a good outlook on life though, and is anxious to move on past CSS. We are hopeful that in much of the new drugs that are being researched for CSS, a cure for Lauren will be found.
In the process of dealing with this disease over the years, I found help from a wonderful group of people around the world, who also suffered with CSS, on the CSSISG support group. I continued my research of CSS and the mechanisms behind it, as well as the drugs that can potentially treat it.
When the CSS Association was formed I was asked to help by serving on the Board of Directors. With a great deal of help from Jane Dion, who started the CSS Association, and several others, a website and organization was put into operation to help serve other patients with CSS, and to bring awareness of this disease.
A year ago, the Vasculitis Foundation, formerly the Wegener's Granulomatosis Foundation, made the decision to add all the other vasculitic diseases to their umbrella organization. Fortunately, for those with CSS, we are now also represented by the Vasculitis Foundation (VF). Recently I was asked to serve on the Education and Awareness Council for the VF, as a representative for Churg Strauss. I would love nothing more than for CSS to be a common name known by all doctors and the general population. The long, painful journey we have been on with Lauren could have been very different if doctors all those years ago had known about CSS, and if we had known about CSS. Like any disease, the earlier it is caught, the better off you are.
We are hopeful for Lauren, and feel great breakthroughs are around the corner for CSS.
By Ken Abbott, February, 2007
I'm better now, but I spent 12 months battling Churg Strauss Syndrome. I'm telling my story below in the hope that it will be helpful to others going through the same thing. Of course, these are just my personal recollections, they are not intended in any way as medical advice. So, with that caveat, here's the scoop on Churg Strauss from my perspective..
How it began
I had some dental implant work done and the procedure was a bit messy because the implant head fractured and my implant dentist had a tough time. Anyway, a few hours after I got home I developed a sudden rash around my ankles. My dentist thought it was an allergic reaction to the antibiotic. I took Benedryl for a day or so and it went away. I thought no more about it. About a week later I spent a day painting a room in our house, and that evening as I showered I noticed the rash had reappeared. It had developed during the day. It was a Sunday, so I stopped by my medical practice and saw the doctor on duty. He immediately mentioned vasculitis. It was the first time I had ever heard the word, and he explained that it was an autoimmune reaction that attacks blood vessels. On the Monday I saw a dermatologist, who immediately pronounced the rash "probably produced by something internal, it's out of my area". He started me on prednisone and sent me to see a rheumatologist.
My Rheumatologist
I belong to a large medical practice, so all the specialists are in-house and it was easy to see a Rheumatologist. He was new to the practice. (By now of course I had been Googling vasculitis like crazy. I discovered there were many different forms, but the one that most closely matched my situation was a form called Churg Strauss.) I explained my full story to my Rheumatologist. I explained the rash, how it had occured. I also mentioned that a few years earlier I had surgery for nasal polyps. There was an history of asthma in my family, and although I had never had full asthma I had been suffering from light bronchial asthma for several years . He ran blood tests, which showed I had a very high eosinophil level. By now I was also having occasional blurry vision episodes, and also numbness in my palms and ankles. He told me that taken together with my medical history, my symptoms could be a form of vasculitis called Churg Strauss (thanks Google!) He said it was very rare, that he had never actually seen a case so was out of his depth, but he referred me to a Churg Strauss specialist in New York City. He said I must continue to take prednisone, a standard first line treatment for autoimmune problems. I did not realize it at the time, but I would be on prednisone for 12 months. I made an appointment to see the New York City specialist.
The official diagnosis
It was a few weeks before I got to see the New York City specialist, and by then my symptoms were much worse. I had difficulty breathing and walking, with numbness and discoloration of the feet. Also, I had swelling of the face. The specialist reviewed by medical history and blood tests, gave me a physical exam, and then officially confirmed I had Churg Strauss. He said he had seen about 40 cases in his entire career. He also said I had an aggressive case of the disease and so wanted to take strong action by putting me on Cytoxan. This is a drug first used for cancer patients, but later found to be very effective for autoimmune conditions. He explained there were "milder" drugs that could be used, but he felt we should treat the condition agressively from the beginning.
Cytoxan
I took Cytoxan for about 2 months and continued to take the prednisone which was now up to 60mg per day. After I phased off the Cytoxan my blood tests showed a significantly reduced eosinophil level. So progress was good. My feet and lower legs were still numb, which made if difficult to walk, and as a side effect of Cytoxan I lost a lot of my hair. A few weeks after stopping the Cytoxan I began a very slow reduction of the prednisone - a process called "tapering" and during this time my specialist put me on Imuran. I had a few attacks of breathing problems, and even an apparent return of the ankle rash, but my specialist explained that this was probably a reaction to the Imuran and so I stopped taking it. He was right. However, my numb feet caused a totally unnecessary complication.
Unnecessary and annoying
One day, walking down some steps in my house with my numb feet, I tripped and tore my Achilles tendon. Long use of prednisone has several side effects such as an increased appetite and mood swings and even depression, but one that is not mentioned much is tendon weakness, and especially the Achilles tendon. This makes it prone to injury. I went through surgery to fix the tendon, followed by about 4 months of recovery and physical therapy. During this time I continued to slowly taper the prednisone. But the tendon injury was unecessary, annoying and an upsetting complication.
Nutrition
While I was at home in a cast recovering from the tendon rupture I had a telephone consultation with a nutritionist. I explained that I was specifically interested in any diet that would help reduce inflammation. Churg Strauss is an inflammatory autoimmune condition, so I was interested in anything that could help reduce inflammation. She said that such a diet exists and gave me the basic rules. Here's what she said (and what I did). Don't eat anything that's made with white flour - no bread, no cakes, no pastries, no pasta. But whole wheat flour is ok, so whole wheat bread is ok. And whole wheat pasta is ok. No potatoes. Cut way back on red meat, try to eliminate it if possible and eat fish and chicken instead. Eat lots of fruit, especially fruit rich in anti-oxidants such as berries. No cheese. Eat cereals rich in flaxseed. I also discovered a special very dark chocolate that's 60% or more cocoa content. It's advertised as rich in anti-oxidants. I checked this one with my Churg Strauss specialist in New York City and he told me, "go for it." I did.
Recovery
At about the same time I recovered from the tendon rupture I finally got myself off the prednisone. In the end I was tapering very slowly with a reduction rate of just 0.5mg per week. The whole process had taken over 12 months. Of course, I had frequent visits to my rheumatologist during the whole time. He was the primary doctor who managed my case, and he carefully monitored my condition with blood tests and chest X-rays to check lung conditions. As required, he also sent me to other specialist - a cardiologist to check heart condition, a dermatologist for a skin biopsy of the rash, a neurologist to check for nerve damage, and a nephrologist to check kidney function.
Hair
Not only did my hair grow back, but it grew back thicker, richer and slightly darker than before. My friends joked about asking their doctors for a Cytoxan prescription!
Maintenance
This consists of blood tests and urine tests. The main blood tests are sed rate, eosinophil level and ANCA. My rheumatologist also prescribed Bactrim - an antibiotic taken twice a week to help avoid nasal infections. I also see my ENT frequently to check my nasal passages and sinuses for any recurrence of polyps. I use Nasonex to help keep nasal passages clear, and I irrigate twice a day with a ph balanced saline solution. The basic message here is that nasal polyps are not a good sign! During my entire treatment I took Ambien every night to help me sleep and I still take it occasionally as needed. I still can't do real exercise, but I can do my usual lunchtime walks exactly as before this whole thing began.
Flare-Ups
It's not uncommon for some or all of the symptoms to re-appear, but usually in milder form and for short periods. These are called "flare-ups". So far I've had two. Both have been "mild" and nowhere near as bad as the original condition. Neither flare-up included the rash or nasal polyps, but there was some upper respiratory problems. The first time my doctor simply put me on prednisone (Medrol) for a couple of weeks. The second time, with my sed rate ok but with my eosinophil and ANCA levels showing signs of creeping up, my doctor put me on prednisone and Methotrexate.
I'm still not back to normal, but I feel I'm well on the way. And I'm very lucky to have had wonderful doctors.
By Jane Dion, Co-Director Churg Strauss Syndrome Association
Spring 2006
http://www.cssassociation.org/
My name is Jane Dion and I was diagnosed with Churg Strauss vasculitis in May 2001. I live in a small town of 800 people in the foothills of western Massachusetts. I've been married 36(!) years and have a 25 year old son, who lives in Barcelona and a 23 year old daughter. My passions are sea kayaking, travel to faraway places, photography, and going to the movies.
Like many people with vasculitis, I was sick for quite a while before I was diagnosed. Diagnosis was difficult because vasculitis can present in so many ways. I saw many different specialists, but none of the doctors put it all together. I saw a pulmonologist for late onset asthma, an allergist and a dermatologist for skin rashes, and an ENT for sinus problems. I lost a lot of weight, which made me very happy, but I often felt weak and lacking in energy. I had a constant dry cough - my husband said he could never lose me in a store because he could just follow the sound of my cough to find me. I had Raynaud’s syndrome and I sometimes experienced pins and needles in my hands and feet.
My primary care doctor was wonderful in referring me to appropriate specialists, but I did not improve. I lived a busy, full life and I didn't have the time to be sick, so I carried on the best I could. I ended up in the emergency room late one night after feeling that I just couldn't breathe - I felt as though there was a five-ton elephant sitting on my chest. Imaging studies showed infiltrates in both lungs. At first I was put in an isolation room at the hospital while TB was ruled out. After two weeks in the hospital and many, many tests, I was finally diagnosed with CSS. I met five of the six American College of Rheumatology’s criteria needed to be classified as having CSS (only four are needed) which are: 1) asthma; 2) eosinophilia (eosinophils are a kind of white blood cell) greater than 10% on differential WBC count; 3) mononeuropathy; 4) transient pulmonary infiltrates on chest X-rays; 5) paranasal sinus abnormalities; and 6) biopsy containing a blood vessel with extravascular eosinophil. I started on high IV doses of prednisone. Improvement was dramatic. I soon felt better than I had in years! I am very fortunate my disease is responsive to medications and that they also control my infrequent flares. I am currently on 200 mgs of Imuran and 10 mgs of prednisone. I would like to further reduce the prednisone, but it seems I flare whenever I go below 10 mgs
When I was first diagnosed, my husband and friends scoured the Internet for information on CSS. Because the disease is so rare, getting easy-to-understand information was difficult and finding an active patient advocacy group was impossible. The disease is so rare - with fewer than 900 to 3,000 patients in the USA - and there were just not the resources available for newly diagnosed patients. I was, however, fortunate to eventually find a Yahoo message board which proved to be a great support (cssisg@yahoo.com).
I was very lucky that Dr. Peter Merkel from Boston University Medical Center, one of the nation's top vasculitis doctors, became my specialist. When I first met Dr. Merkel he was involved in establishing the Vasculitis Clinical Research Consortium (VCRC). He urged me to find someone willing to act in an advocacy position for CSS patients within the VCRC. After there were no takers among the people on the message board, I, along with Dr. Carol Kavanaugh, a CSS patient from California and Cindy Smoot, mother of a young child from Texas, formed the Churg Strauss Syndrome Association in 2004. Its original purpose was to gain representation on the VCRC, which had recently received more than $6 million in funding for vasculitis research. At a meeting of the Rare Disease Clinical Research Network in Rockville, MD we were further encouraged and supported in our efforts to set up a CSS resource to maintain a presence within the rare disease patient advocacy community.
Because Churg Strauss Syndrome is so rare and because it manifests itself in many ways, diagnosis and treatment can be problematic and confusing. Many of us search the web for pertinent information on the disease and its treatment. This task can feel overwhelming to the newly diagnosed. In addition, there has not been much research on CSS and therefore it remains somewhat of a mystery. Because it affects everyone differently, treatment is not standard. The drugs used to treat the disease themselves need to be understood as they can have adverse effects. Some of the articles are written in “doctor speak” which is hard for the lay person to interpret. We hoped to make the CSSA website a place where patients and their families can easily find the information and support they need to better understand CSS. We also hoped that by establishing a strong presence we might stimulate and support research into CSS.
Helping to create the CSSA is one of the most difficult things I've ever done and one of the things I'm most proud of. I had no relevant experience at all before I undertook this endeavor. In two years we have created a very informative and much accessed website with readers from over 70 countries around the world. We often learn that patients are referred to our site by their diagnosing physicians who themselves have referenced it to learn more about the disease. We have created an international presence with board members from France and the Netherlands. Our Medical Advisory Board consists of the worlds experts in our disease. Many people have contributed patient stories to the news page of the website. Ben Watt, DJ, songwriter, singer and author of the compelling book "Patient - the True Story of a Rare Illness" took the time to write a letter of encouragement to CSSers and people with vasculitis (http://www.cssassociation.org/oldnews/watt.asp). Irina Slutskaya, an Olympic figure skater who has CSS, graciously met with me and offered words of hope to all vasculitis patients. (http://www.cssassociation.org/oldnews/Slutskaya.asp). On the news section we also publicize and promote research and share new information relevant to CSS.
Although the CSSA has accomplished so much in a short period of time, we recognize the need to band together with other vasculitis groups to better raise awareness of vasculitis. There is strength in numbers and together we can create a higher profile and stronger voice within the medical community and among the general population. Because CSS and other forms of vasculitis are so rare, many people are diagnosed late, or not at all, causing permanent damage or even death. We believe that by banding together, we will be more successful in raising awareness of all the vasculitides, resulting in earlier diagnosis and more effective treatment for each of the diseases. Someday, we hope that "vasculitis" will be a household name much as lupus, MS or Parkinsons is now. The CSSA is happy to have a close association with the VF as the WGA/VF was very helpful to our fledgling organization and provided advice, support and shared resources. We have a history of working well together and share many of the same goals.
By Nita A., January, 2007
I am now 51, have had three distinct diagnoses of CSS and since hindsight is 20/20, I now believe that my Churg Strauss actually started in my 20s. I have been reading the other stories on this site, and I am amazed at the similarities with my own experience. I will try to keep my story succinct, and I hope that it is helpful to others.
As a college student I suffered from severe sinus congestion, lost my sense of smell and eventually had surgery for nasal polyps. Shortly thereafter I developed asthma, and needed to be hospitalized multiple times to get it under control. Throughout my 20s I continued to have severe asthma, nasal polyps and recurrent ear infections. Looking back, I also remember that I had itchy red bumps on the palms of my hands (which were later diagnosed as eosiniphiliac).
In 1986, when I was 31, I developed bizarre symptoms about a week after I gave birth to my first child: a purple rash on my chest, night sweats, low grade fevers, joint pain, itchy red bumps on the palms of my hands (which were biopsied and showed eosiniphils), and loss of appetite. I was unable to nurse my baby. I thought I was going crazy! After a couple weeks of many physicians and tests, I was given the news: Churg Strauss vasculitis. I was started on prednisone and felt better almost immediately. I was on the steroids for about 2 1/2 years, and was relatively well (except for asthma, polyps and loss of smell) until the next episode 10 years later.
The second episode occurred when I was on vacation with my husband and two children at Disney World! I experienced a tingling in my hands and a weird sensation in my butt such that I could not sit comfortably. There was nothing at all similar about this to make think of CSS. We returned home, and I woke up the next morning with a foot drop!! Unfortunately I had switched physicians because my health insurance had changed, so I was being evaluated by docs who did not remember what I had experienced previously. This was a huge error in retrospect. I almost ended up with back surgery for a disc problem, before we realized there was something else going on. While a neurologist (who had not read my medical record) was performing an EMG she exclamed, almost to herself, "this almost looks like Churg Strauss"! My mother was in the room with me and we looked at each other in disbelief!! Who would think that such seemingly unrelated symptoms could be the same disease!!
So, since doctors are so concerned about having definitive evidence before they treat, I was asked to endure a biopsy of my leg to confirm the presence of eosiniphils. Although I had multiple incisions, and my seral nerve was cut (leaving me with loss of sensation in part of my foot), they happened to not find a spot that would give them the info they wanted. I was told that my sciatic nerve was involved and that it was as though my sciatic nerve had had a stroke because the inflammation of the blood vessels had cut off the blood supply to the nerve. I had to be fitted with a foot brace, and had to use a cane. I developed severe nerve pain that kept me lying in bed in agony, and was finally started on Elavil which was the only thing that helped the pain. Until I was diagnosed I thought I was dying! The diagnosis was confirmed on the basis of lab results and past history. My symptoms were quickly controlled with prednisone and, later, Imuran. Most of my neurological damage healed over the next year. After six weeks out of work on medical leave, I returned to a job that I love as a social worker in a facility for the aged, though I was barely recognizable to some because of the Cushing's Syndrome and the cane! I joined Weight Watchers a year later, started to exercise, and lost all of the weight that I had put on. Life was good.
The next 10 years were more of the same: asthma and nasal polyps, and now I started to have recurrent ear infections again. The past couple of years have been characterized by an increase in all of these problems. I had tubes put in both ears last March (2006). The ear infections continued despite the tubes. Every time I had an ear infection the asthma also was triggered, so I would end up on a short course of prednisone. This has been going on for quite some time. I also experienced a tingling in my fingers. In retrospect, I think the CSS was a factor, but my lab results have not been indicating that there was an inflammatory process at work. Perhaps they were suppressed from the frequent steroid use.
Soooo, this week as I was recovering from yet another ear infection, I developed symptoms of a UTI and pain in my left mid back. At the same time my sinus congestion became unbearable. My PCP thought that I had developed a fungal UTI from the frequent antibiotic use. Ultrasound of the kidneys showed inflammaton in the ureters. I do not know if this was unrelated or if it is symptomatic of the CSS. I also saw my ENT who (sorry in advance for the graphic description) said that my sinus was red and inflamed, the stuff coming out of my ears was the same stuff in my sinus, and he extracted a thick, white globule that he identified as consisting of eosiniphil cells that had come from the sinus. He was sure that this was Churg Strauss!! I have been so sick for the past week with urinary problems and kidney pain, sinus problems, fever, ear congestion and loss of hearing, but it never occurred to me that this was the CSS again! My lab results now showed an increase in eosiniphils to 17%. I started taking prednisone and immediately began to feel so much better. I have been out of work for the past week. My rheumotologist is probably going to wean me from the steroids onto Methotrexate. She and I had speculated last summer about the Churg Strauss being a factor in my recurrent ear problems, but did not have the lab results to back it up. I am now convinced.
I am looking forward to getting back to my life. I now understand that CSS is a chronic illness with flares, rather than distinct episodes, I can avoid the frequent and recurrent low grade chronic illness that has been making me sick for the past two years.
I hope that others find my story helpful. My thanks to everyone else out there who has shared theirs!