(Pictured: Dr. Anne Tournay, seen here with her dog, has EGPA. It took her more than two years for the illness to be diagnosed correctly.)
An estimated 5,000 people in the United States are living with Eosinophilic Granulomatosis with Polyangiitis (EGPA), a rare form of vasculitis that causes inflammation or swelling in the small and medium blood vessels. While it can cause damage to organs throughout the body, it most often affects the lungs.
The American Lung Association’s new EGPA Educational Campaign, provided with support from AstraZeneca and GlaxoSmithKline, strives to educate people living with EGPA, and their families and caregivers about this serious but treatable disease. Here’s what they want you to know:
Symptoms
They’re varied. EGPA can manifest differently based on what organs are affected. However, nearly everyone living with it will experience these signs and symptoms:
• Feeling frequently ill and tired.
• Loss of appetite, resulting in weight loss.
• Fever.
• Asthma and/or sinus polyps.
• A higher-than-normal level of eosinophils, a type of white blood cell.
Patients may also experience shortness of breath, coughing, chest pain, rashes, muscle and joint pain, nasal discharge, facial pain, abdominal pain or bloody stools, numbness or loss of strength, tingling in hands and feet, and kidney disease.
Persistence, patience in diagnosis.
Timely diagnosis and treatment are critical to optimally managing and minimizing symptoms. But getting the right diagnosis may take years and involve a clinical exam, blood tests to check white blood count, a urinalysis, a chest X-ray or CT scan, and a biopsy. Those who have been through the process say it can be frustrating, and persistence is needed to get proper care.
Dr. Anne Tournay started to learn she had EGPA with symptoms that included severe adult-onset asthma, congestion due to nasal and sinus polyps, arm pain due to neuropathy, and urticaria, a condition that causes hives.
“Despite being a physician myself and asking repeatedly if I had EGPA, I didn’t receive the right diagnosis and treatment for two and a half years. Eventually I paid out of pocket to see an EGPA expert and enrolled in an EGPA clinical trial. After various medication adjustments, I’m now 10 years out from diagnosis and have had no active symptoms for five years,” Tournay said.
Treatment can help.
There is no cure, but treatment can reduce symptoms, decrease the risk of organ damage, and improve quality of life. Because EGPA can affect multiple organs, a patient may need to work with more than one healthcare provider. They’ll review disease severity, the organs involved, current medications, and other health conditions. Medications may be prescribed, such as anti-inflammatories, which help reduce swelling in the body; immunosuppressants, which can lower the immune system’s activity; and biologics, which target inflammation caused by eosinophils.
Lifestyle changes.
Good health practices, such as keeping vaccinations up to date, eating a well-balanced diet, staying active, and getting adequate sleep can help a patient feel their best. It’s also important to quit smoking, avoid secondhand smoke and fumes, dust and chemicals. Other strategies include tracking any new, worsening or changing side-effects or symptoms, managing asthma, and seeing a healthcare provider regularly.
Support is available.
EGPA is rare, but questions can be answered and support can be found from:
• The Lung Helpline at 1-800-LUNGUSA, where you can talk to trained lung health professionals and receive a free health journal.
• Living with Lung Disease Support Community and Living with Asthma Support Community, which provide anonymous 24/7 peer-to-peer support at Inspire.com.
The American Lung Association will host a free webinar on March 5, intended for those with rare and complex lung diseases that may be misdiagnosed as asthma or another more common lung disease. Dr. Sanober Kable, a pulmonologist and critical-care specialist, will discuss different examples of rare lung diseases, signs and symptoms to watch for, and the steps to ensure you have the correct diagnosis. Registration for the webinar and more information about EGPA are at Lung.org/EGPA.
Source: StatePoint Media
