by Cindy Smoot, Churg Strauss Syndrome Board of Directors
VF Education and Awareness Council
Our family has been dealing for 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 again. 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.