When we asked Dr. Strauss what the first thing he wished people knew about getting a PAH diagnosis, he didn’t hesitate. “I’m always amazed at how many referrals we get for shortness of breath and the patient hasn’t gotten an echocardiogram because nobody thinks about a heart issue when there is breathing difficulty.”
When Dr. Strauss sees a patient for the first time, he must first determine a thorough and accurate diagnosis before beginning treatment. “This is where care can so quickly run off the rails—during that first visit,” he explains, “You can’t prematurely go down a specific disease path because there are different treatment options depending on the class of pulmonary hypertension that a patient is diagnosed with.”
In the early stages of PAH, you likely won’t notice symptoms, but as the disease progresses, symptoms emerge that may mimic other more common chronic lung diseases. Increased shortness of breath, fatigue, swelling of the feet and legs, dizziness and cough can indicate a variety of health conditions and are all reasons to contact your healthcare provider.
Pulmonary Arterial Hypertension (PAH) is a rare type of high blood pressure in the lungs that most commonly affects women between the ages of 30-60 but can occur in any person at any age. There is no cure, but treatment is available to control symptoms and improve quality of life. Learn more at Lung.org/PAH.
Diagnosis of PAH begins with elimination of other diseases that share the same symptoms. Your healthcare provider will first review your medical history and conduct a physician exam. Lung function tests rule out diseases like asthma or COPD. Blood tests help identify HIV, lupus, scleroderma or rheumatoid arthritis. Six-minute walk tests determine if your oxygen levels drop while you are physically active. Electrocardiograms show the electric activity in your heart to help determine abnormalities. An echocardiogram checks the size and condition of the chambers in your heart and estimates blood pressure in your lungs. A right heart catheterization is more invasive and provides blood pressure measurement in the lungs.
The Oregon Clinic, where Dr. Strauss works, is a PAH accredited center. “To become accredited, you must prove you have a guidelines-based process in place to determine an accurate diagnosis and experience developing an appropriate treatment plan in partnership with the patient. Being accredited takes a lot of resource gathering and having access to a ready and knowledgeable healthcare team is a clear patient benefit.”
Healthcare team members like Brittany Watson are another great source of support and care in a coordinated office setting. Brittany is a registered nurse with a caseload of about 100 patients with pulmonary hypertension. She has plenty of experience getting prior authorizations and coverage for treatments, though she says, “My biggest job is being available to patients so they can call and speak with me directly. Prompt conversations about symptoms or medication side effects they are experiencing helps keep their disease manageable. It’s not easy having a lifelong disease like PAH, but we’re here to help make it easier.”
Treatment Pathways for PAH
Medications taken for PAH work together to dilate arterial blood vessels so your blood can flow more easily when pumped from the right side of the heart to your lungs. There are three well-known treatment pathways – the endothelin, nitric oxide and prostacyclin pathways – that must be addressed. Each pathway is named for a natural substance within the small blood vessels of your lungs that has become unbalanced but can be corrected with medication.
“My treatment goal is a normal life span,” explains Dr. Strauss, “We may not get there for everyone, but the medications work and they work well.” Once treated, the right side of your heart doesn’t have to pump so hard to move your blood and your symptoms should improve. Not only will you feel better, but you’ll also be preventing cardio-pulmonary damage that could shorten your life. That’s why it is imperative that you get an early and accurate diagnosis and begin treatment right away.
In addition to medications, Dr. Strauss recommends these tips:
- Use a scale to monitor your weight. Get on a low salt diet and drink lots of water, but not too much. Remember this is a disease of fluid retention.
- Gentle walking is important. No matter which heart or lung disease you have, you will do better by incorporating daily exercise into your life. Speak with your healthcare provider about pulmonary rehabilitation if you aren’t sure how to start.
- Consider joining a research study. Any patient interested can probably get into a clinical trial.
You can visit Lung.org/PAH for more information.
Support for this educational campaign is provided by Janssen.
Blog last updated: August 25, 2023