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By Caroline Downs
The Ron McNeiley family and most of the rest of Kenmare, had never even heard of Churg-Strauss syndrome until Ron’s diagnosis.
According to the Mayo Clinic website, CSS is an autoimmune disorder marked by blood vessel inflammation, also called allergic granulomatosis and allergic angiitis. The inflammation can restrict blood flow to vital organs and tissues, sometimes permanently damaging them.
No cause has been identified for CSS, and no cure can eliminate the disease from a patient’s body or reverse its effects.
However, an aggressive treatment of corticosteroids, chemotherapy and interferon can improve conditions and treat symptoms for patients like Ron. In many cases of CSS, treatment can cause the disease to go into remission, which is what the McNeileys are hoping.
Six criteria for CSS
Asthma is the most common early sign of Churg-Strauss syndrome, but CSS can cause a variety of problems, ranging from hay fever, rash and gastrointestinal bleeding to severe pain and numbness in the hands and feet. Because the symptoms can vary so broadly and appear so similar to other diseases, CSS is often misdiagnosed.
The combination of symptoms and signs, along with the organs affected and the presence of certain abnormal blood tests help doctors make a CSS diagnosis. According to the Johns Hopkins Vasculitis Center website, the American College of Rheumatology has established six criteria used to determine if patient has having CSS.
Generally, doctors will name Churg-Strauss as the disease if four of the six criteria are present in a patient. Some patients are determined to have CSS if only two or three of the criteria are present.
The McNeileys believe Ron has experienced all six conditions to some degree, including:
•An excessive number of one type of white blood cells called eosinophils (known as eosinophilia)
•Damage to nerves or nerve groups
•Spots or lesions on the lungs, seen during chest x-rays, that move from one location to another or disappear without treatment
•Infected, inflamed or polyp-filled sinus cavities
•White blood cells accumulating outside of blood vessels, revealed through a tissue or organ biopsy (known as extravascular eosinophils)
Churg-Strauss syndrome is described as progressing in three phases, although patients may experience symptoms from more than one stage at the same time. The Churg-Strauss Syndrome Association advises that not everyone diagnosed with the disease experiences all three phases, or experiences the phases in this specific order.
The first phase, known as the prodromal phase or allergic phase, almost always involves asthma. Like many other CSS patients, Ron developed asthma as an adult, while long-time asthma patients may experience suddenly worse symptoms at the onset of CSS.
Other characteristics of the allergic phase include facial pain from sinus infections, nasal polyps, allergic rhinitis (sneezing, itching, runny nose) and recurrent bronchitis and/or pneumonia. This phase can last between 4 and 27 months, although some patients stay in the phase for several years.
The second phase involves the overabundance of white blood cells known as eosinophils. The excess white blood cells can occur in the blood or the body tissues, leading to problems in the digestive system such as weight loss, fever, abdominal pain, bloating, vomiting, diarrhea and nausea.
If the white blood cells impact the lungs, the patient may struggle with shortness of breath, a feeling of heaviness in the chest and a constant cough.
A patient may experience the second phase of CSS for months or years and not progress any further with the disease, especially if the disease is detected and treatment has started.
Not every Churg-Strauss patient has symptoms from the third phase, known as systemic vasculitis and characterized by inflammation and damage to blood vessels throughout the body. The situation can lead to further damage in different organs during this phase, and symptoms vary among patients.
The Churg-Strauss Syndrome Association reports general symptoms of fever, weight loss and enlargement of glands, while the organs affected in this phase include the skin, heart, lungs, central and peripheral nervous systems, gastrointestinal tract, kidneys, eyes and musculoskeletal system.
The numbness and pain Ron has in his right leg and hip now may be related to vasculitis, although the symptoms could also be side effects of the medications he takes.
“Looking at these phases is like reading Ron’s life story the last few years,” said his wife, Tami.
Even though a diagnosis of Churg-Strauss is serious and life-changing, the Churg-Strauss Syndrome Association reminds patients that with effective treatment most of the symptoms in any of the three phases can be relieved. With early diagnosis and treatment, many people never experience problems associated with the third phase of the disease.
Researchers are working to develop a greater understanding of CSS to find better treatments and, possibly, a cure. Ron has agreed to be included in those research efforts, with doctors monitoring and documenting his responses to the corticosteroids, immunosuppressive drugs (chemotherapy) and interferon currently available to treat his symptoms.
He has also been accepted in a study for a new drug slated for use against Churg-Strauss, with that medication to be started in the spring of 2013.
Like all CSS patients, Ron and his family are aware the disease cannot be cured and that relapses or flare-ups of symptoms are to be expected. On the other hand, the combination of drugs can send CSS into remission, allowing Ron to return to a more normal lifestyle.
The McNeileys are focused on that goal of remission. Son Jaden, a fourth grade student, has asked the doctors his own questions and come to one conclusion. “This thing can be beat,” he said. “I don’t see why it can’t.”
For more information about Churg-Strauss syndrome, the McNeileys recommend the Churg-Strauss Syndrome Association website at www.cssassociation.org and the Mayo Clinic website at www.mayoclinic.com/health/churg-strauss-syndrome.