SPONSORED CONTENT -- (StatePoint) Each year, 500 to 1,000 people in the United States are diagnosed with pulmonary arterial hypertension (PAH), a rare lung disease that can be fatal if left untreated.
To encourage earlier diagnosis of PAH, the American Lung Association, with support from Johnson & Johnson, is sharing information about the disease, its symptoms, and the importance of testing and proper treatment. Here’s what the Lung Association wants you to know:
What is PAH? PAH is a rare, progressive lung disease caused when the tiny arteries in your lungs become thickened and narrowed. This blocks blood flow through your lungs, causing the blood pressure in your lungs to rise. The heart has to work harder to pump blood through the narrowed arteries. Over time, your heart has difficulty effectively pumping blood through your body.
What are the symptoms of PAH? In the early stages, you may not notice symptoms. As PAH progresses, common symptoms include shortness of breath, especially with activities like walking up the stairs or long distances, fatigue, edema (swelling of the feet, legs and eventually the abdomen and neck), dizziness and fainting spells, chest pain, heart palpitations, and lips and fingers turning blue. Contact your healthcare provider if you’re experiencing these or any worrisome symptoms.
Who is most at risk? While anyone can get PAH, those most at risk are women ages 30-60, and Black and Hispanic women.
Why is PAH difficult to diagnose? Because symptoms of PAH are similar to other diseases, the average time it takes to get a diagnosis is about 3 years. This delay can be frustrating for patients and their families, and can result in poorer health outcomes. Unfortunately, health disparities exist due to a wide-range of factors, like lack of access to healthcare, health insurance coverage, transportation to specialists, and health literacy, and can exacerbate these delays. Increasing awareness of this rare disease can help patients get a quicker diagnosis so they can get the treatment they need.
Elva V., a mom and nurse, has been living with PAH since she was 23. She shares that getting the correct diagnosis and treatment plan has been a “game changer,” helping her “feel well enough to do the things I enjoy and live a good life with my daughters.”
How can the path to diagnosis be shortened? Your provider may order several tests to help with diagnosis:
• A simple blood test, the BNP test (also called B-type natriuretic peptide test) or NT-proBNP test, can help determine if your heart is working harder than it should, a sign that you may have PAH. Getting this test done early can help speed the path to diagnosis.
• Electrocardiogram to show the electrical activity of your heart.
• Echocardiogram to check the size and condition of your heart.
• Lung function tests.
• A right heart catheterization to confirm PAH, once your provider suspects it.
How is PAH treated? PAH-specific medications come in multiple forms: oral, inhaled and subcutaneous. Some allow blood to flow more easily through the arteries of your lungs. Others improve heart and lung function. PAH medications help slow how quickly your disease worsens. For optimal health outcomes when dealing with a rare disease like PAH, it’s best to see a specialist.
“When seen by a specialist, you’ll receive the latest treatment and resources to help you live your best life,” says Dr. Michael Cuttica, a pulmonary hypertension specialist.
That is certainly the case for Lindsay T., who has been living with PAH since her early twenties and works with her doctor to manage her care. “My BNP has been within a normal range since I alerted my specialist that my symptoms were getting worse and he adjusted my medications,” she says.
If you have unresolved respiratory symptoms that are not improving with your current treatment, learn more about PAH at Lung.org/pah.
A PAH diagnosis doesn’t have to be delayed. With the right tests and care from a specialist, you can get on the path to the treatment you need.
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