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 are varied: EGPA can manifest differently based on what organs are affected. However, nearly everyone living with EGPA will experience these signs and symptoms:
- Feeling frequently ill and tired
- Loss of appetite, resulting in weight loss
- 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 are key to 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 your 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 that persistence is needed to get proper care.
“My first EGPA symptoms 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 2.5 years. Eventually I paid out of pocket to see an EGPA expert and enrolled in an EGPA clinical trial. After various medication adjustments, I am now 10 years out from diagnosis, and have had no active symptoms for 5 years,” says Dr. Anne Tournay, an EGPA patient.
Treatment can help: There is no cure, but treatment can reduce symptoms, decrease the risk of organ damage, and improve your quality of life. Because EGPA can affect multiple organs, you may need to work with more than one healthcare provider. They’ll review your 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 your immune system’s activity, and biologics, which target inflammation caused by eosinophils.
Lifestyle changes support management: Good health practices, like keeping vaccinations up to date, eating a well-balanced diet, staying active and getting adequate sleep can help you feel your best. It is also important to quit smoking, to avoid secondhand smoke and to protect yourself from fumes, dust and chemicals. Other strategies include tracking any new, worsening or changing EGPA side effects or symptoms, managing your asthma and seeing your healthcare provider regularly.
Support is available: EGPA is rare, but you’re not alone. Get your questions answered and find support:
- Contact 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 on Inspire.com.
- The Patient & Caregiver Network, which provides free, direct access to education and support.
Not sure if your symptoms could be EGPA? The American Lung Association will be hosting 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 you can take to ensure you have the correct diagnosis. Be sure to sign up online to attend this virtual event.
By better understanding Eosinophilic Granulomatosis with Polyangiitis, you can take control of your symptoms, get needed support, and better manage the disease.
(StatePoint)
PHOTO SOURCE: Dr. Anne Tournay, a patient with managed EGPA
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