Living with Churg-Strauss Syndrome (CSS), or Eosinophilic Granulomatosis with Polyangiitis (EGPA), is a journey that often begins with confusion, misdiagnosis, and frustration. Since CSS is a rare condition, patients may spend years trying to pinpoint the root cause of their symptoms. For many, their lives are disrupted by recurring health issues like asthma, allergies, and unexplained fatigue long before they receive the proper diagnosis.
The following is a hypothetical patient's story. This narrative seeks to explore the emotional and physical challenges faced by a person living with CSS, while also shedding light on the impact of the disease, the path to diagnosis, and the day-to-day realities of managing this chronic illness.
I was 30 when I started noticing that something wasn't quite right with my health. I had always been active - jogging in the mornings, working full-time, and keeping up with my social life - but I began experiencing asthma-like symptoms. At first, it was just mild shortness of breath during exercise, but over the months, it progressed to wheezing and coughing, especially at night.
When I visited my doctor, I was diagnosed with asthma. I left the office with a prescription for an inhaler and some medication to manage my symptoms. It seemed like an open-and-shut case. I thought, “Okay, I have asthma. Millions of people live with asthma every day, and with the right medication, so can I.”
But as the weeks turned into months, my symptoms didn't improve. In fact, they got worse. The inhaler barely made a difference, and now I was getting headaches, sinus congestion, and feeling utterly exhausted all the time. I returned to the doctor, who adjusted my asthma treatment. We tried different combinations of medication - steroids, bronchodilators, antihistamines - but nothing really worked.
At this point, I began feeling more frustrated. The doctors reassured me that it was “just asthma” and that I should be patient with the treatments. But deep down, I knew something else was wrong. Allergies were thrown into the mix. I went through allergy testing and was advised to avoid certain triggers. Still, the symptoms persisted.
By the second year of dealing with these symptoms, my life had changed drastically. I was constantly tired, my muscles ached, and I began losing weight without trying. More alarming, though, were the strange, migratory pains that began affecting my hands and feet. I would wake up in the morning to find my fingers stiff and swollen. My feet hurt so much that walking became a challenge.
It was around this time that I started experiencing occasional numbness and tingling in my hands. As the months went on, I noticed patches of red skin appearing on my legs and arms. I went back to the doctor, and after a battery of tests, I was referred to a rheumatologist, who finally began to suspect that this wasn't just asthma or allergies.
The rheumatologist ordered more tests, including blood work that showed elevated eosinophils - a type of white blood cell involved in allergic reactions and inflammation. I underwent a lung biopsy and imaging tests, and after what felt like an eternity of waiting, I was diagnosed with Churg-Strauss Syndrome (CSS).
The diagnosis came as a shock. I had never even heard of Churg-Strauss Syndrome before. The doctor explained that it was a rare autoimmune condition where the immune system attacks the blood vessels, causing vasculitis and inflammation. The elevated eosinophils in my blood had been infiltrating my lungs, skin, and nerves, leading to the worsening asthma, skin lesions, and the numbness in my hands and feet.
There was relief in knowing that I finally had a name for what I was experiencing, but at the same time, fear set in. How would this condition affect my life moving forward?
Following the diagnosis, I was started on high-dose corticosteroids to reduce the inflammation. Initially, the steroids worked wonders. My breathing improved dramatically, and I had more energy than I had in months. But the side effects of long-term steroid use soon became apparent: weight gain, mood swings, and trouble sleeping.
The doctor then added immunosuppressive medication, including azathioprine and methotrexate, to help manage the disease while reducing the reliance on steroids. Over time, the symptoms became more manageable, though not completely gone. I had to learn to adjust to my new normal, understanding that Churg-Strauss Syndrome was a chronic condition that would require ongoing treatment and monitoring.
I kept working full-time, but there were days when the fatigue was overwhelming, and I had to call in sick. I had to slow down my lifestyle, being careful not to overexert myself or expose myself to illness. Even a simple cold could trigger a flare-up, setting me back weeks in recovery.
Living with CSS is an emotional rollercoaster. In the beginning, it was hard to explain to friends and family what I was going through. People would see me on good days - when I was out and about, laughing and socializing - and think I was perfectly fine. They didn't see the nights I spent lying awake, too tired to move but unable to sleep because of the pain in my legs.
It's an invisible disease in many ways. The people around me don't see the moments when I'm gasping for air, or when I can't button my shirt because my fingers are too swollen. They don't know about the fear that comes with every new symptom. The weight of wondering, “Is this a sign of a flare-up?” or “What organ will this disease affect next?”
Finding a support group was a game-changer. I found a community of people living with Churg-Strauss Syndrome, both online and in-person. Being able to share my experiences with others who understood the struggles - without needing to explain the disease - gave me the comfort and encouragement I needed. It's easy to feel isolated when you're living with a rare disease, but the knowledge that others are going through the same thing brought me some peace of mind.
Over the years, I've learned to listen to my body in ways I never did before. Self-care became not just a buzzword, but a necessity. I learned that pushing through the fatigue or pain wasn't heroic - it was harmful. I started keeping a symptom journal, documenting flare-ups, triggers, and how my body responded to different treatments.
I also began to prioritize rest, even when I felt guilty for not being as active as I used to be. I cut back on social obligations, limited my working hours, and focused more on activities that promoted my physical and mental health, like gentle exercise, meditation, and spending time with close family and friends.
Living with Churg-Strauss Syndrome requires constant balancing. Every day is a little different - some days are better, some are worse. I have to carefully balance my medication regimen, watching out for side effects while making sure that I'm not allowing the disease to flare.
Diet also plays a significant role in managing my symptoms. While there's no specific “CSS diet,” I've found that eating an anti-inflammatory diet - rich in vegetables, whole grains, lean proteins, and healthy fats - helps keep my energy levels more stable. I avoid processed foods and limit sugar, as I've noticed they tend to trigger inflammation for me.
Staying on top of doctor's appointments and regular testing is essential. I have a team of specialists, including my rheumatologist, pulmonologist, and sometimes a neurologist, depending on how my symptoms are evolving. Monitoring my eosinophil count, lung function, and any signs of vasculitis is crucial to catching flare-ups early and adjusting my treatment plan.
CSS affects not only my health but also my relationships. There are times when I feel guilty about needing extra help from my spouse or family, especially on days when the fatigue is so intense that I can barely get out of bed. I've had to become more open about asking for support and leaning on the people around me when I need it.
At the same time, I've found that maintaining relationships is a delicate balance. Some people are incredibly supportive, but others don't fully understand the unpredictability of chronic illness. Over time, I've learned to communicate more clearly about my boundaries and my needs, even if it's difficult to explain why I have to cancel plans or why I can't always be as present as I'd like to be.
Living with Churg-Strauss Syndrome is a journey of ups and downs, but I've learned to take things one day at a time. While there are no guarantees in terms of how the disease will progress, I know that advances in biologic therapies offer hope for better, more targeted treatments. Already, drugs like mepolizumab and benralizumab have shown promise in controlling eosinophilic inflammation, offering a glimpse of what the future may hold for people like me.
I don't know what tomorrow will bring, but I'm learning to be kinder to myself, to celebrate the small victories, and to keep moving forward.