What to know about Pulmonary Arterial Hypertension (PAH)

STATEPOINT – Each year, an estimated 500-1,000 people nationwide are diagnosed with pulmonary arterial hypertension (PAH). While there’s currently no cure, treatment can help control symptoms and improve quality of life.

The American Lung Association, with support from Janssen, launched a new campaign to ensure patients get an earlier, accurate PAH diagnosis and feel empowered to participate in discussions regarding treatment options. As part of the campaign, they’re also sharing insights from Lindsay T., a patient living with PAH.

PAH DEFINED: PAH is a progressive disorder and one form of a broader condition known as pulmonary hypertension, which is high blood pressure in the lungs. In PAH, this increased pressure in the vessels is caused by obstruction in the small arteries in the lung, and can occur for a variety of reasons.

CAUSES: While the cause of PAH is usually unknown, about 15-20% of patients have inherited the condition. Most common in women ages 30-60, PAH disproportionately impacts African American and Hispanic women.

SYMPTOMS: Individuals are initially asymptomatic and then begin to experience symptoms similar to other common lung diseases, such as shortness of breath and fatigue. For this reason, PAH can be difficult to diagnose – 74% of patients are diagnosed in advanced stages of the disease. Eventually, symptoms can include swelling of the feet, legs, abdomen and neck, dizziness and fainting spells, chest pain, heart palpitations, and lips and fingers turning blue.

“When I was 23, I started noticing that when I would walk, I would get out of breath really easily. At first, I thought I was just out of shape. It took about a year for my doctors to say it was pulmonary arterial hypertension,” says Lindsay.

DIAGNOSIS: To confirm diagnosis, a right heart catheterization is needed to measure pressure in the heart and lungs. Providers will typically rule out other common diseases with less invasive tests first. An accurate diagnosis of the correct type of pulmonary hypertension helps ensure prompt, optimal treatment. When PAH is suspected, patients should be referred to a PAH specialist at an accredited center. Left untreated, PAH compromises heart function, which can lead to heart failure and even death.

TREATMENT: Patients should see their specialist regularly for structured evaluations. This allows for tailored treatment plans.

Treatment may include supportive therapies, such as supplemental oxygen, pulmonary rehabilitation and diuretic medications, as well as specific treatment, including medications that dilate blood vessels and affect the vasculature to reduce the increased resistance of blood flow that contributes to right-sided heart failure.

There are many clinical trials occurring right now. Those interested should speak with their healthcare provider or visit Clinicaltrails.gov.

TRACKING: Tracking symptoms is a vital part of self-management of the condition.

“One of the most important things to do while living with PAH is to learn to listen to your body because it’ll tell you if you need something, and that’s information that you can talk about with your healthcare team,” says Lindsay, who after noticing worsening symptoms, spoke to her specialist who then started her on a new medication.

Lindsay also advises patients to become their own advocates. “Research empowered me to take control of my diagnosis and actually make decisions for my healthcare,” she says.

SUPPORT: Support groups give patients a chance to connect with others living with their condition. Patients can also learn more at Lung.org/PAH.

While PAH is a serious, potentially life-threatening condition, proper treatment can make the disease manageable. After finding the right treatment plan, Lindsay is thriving.

“This life I lead today, I’m physically active. I teach high school algebra. I’m successfully managing my PAH. It’s not easy, but it’s possible,” says Lindsay.