How Pulmonary Hypertension Guidelines Improve Patient Care

What is Pulmonary Hypertension and Why Were Guidelines Needed?

Pulmonary hypertension means the walls of the lung arteries have become stiff, damaged and narrow. Blood flow becomes more difficult leading to rising blood pressure in the lungs and the right side of the heart must work harder to pump oxygenated blood throughout the body. This added stress to the heart can lead to heart failure. In most cases, pulmonary hypertension cannot be cured, but proper treatment can improve symptoms and slow the disease’s progression.

Oftentimes patients living with pulmonary hypertension were diagnosed long after their symptoms began due to a similarity of symptoms to more common lung diseases like asthma and COPD. The most common symptoms of PH include shortness of breath, fatigue, chest pain, dizziness and fainting, heart palpitations and edema.

What is it Like Living with Pulmonary Hypertension?

Lindsay was 23 years old when diagnosed with pulmonary arterial hypertension (PAH) and shared her story of learning how to read and listen to her body so she could best advocate for her care. PAH is classified by the World Health Organization (WHO) as Group 1 and occurs when the arteries in your lung become thickened and narrow, which blocks blood flow resulting in increased blood pressure in the lungs that puts stress on the right side of the heart. Lindsay is a huge proponent of self-advocating for open and ongoing communication with a specialist, seeking support from others living with the disease and following medication treatments exactly as prescribed.

Delores was diagnosed with another type of pulmonary hypertension, chronic thromboembolic pulmonary hypertension (CTEPH). This form of pulmonary hypertension is classified by the WHO as Group 4 and occurs when blood vessels in the lung have been blocked by a clot for an extended period. Delores shared her experience of going back and forth between lung and heart doctors to figure out what was going on due to her symptoms being like many other cardiopulmonary diseases. Finally, a specialist was able to determine that both her lungs were filled with blood clots and treatment began to ease her symptoms and stop the disease from progressing. Delores wants more people to know about CTEPH, “Even doctors might think you have something else because the symptoms are similar to other health issues. Because CTEPH is a rare disease, many people don’t know about it.”

What Resources are Available for Patients Living with Pulmonary Hypertension?

If you have been newly diagnosed with pulmonary hypertension, no matter what type it may be, learn all you can about the disease, treatment options, support groups and how to manage your PH. Your healthcare provider can help you understand your disease and refer you to a specialist or other resources. They can explain if your treatment follows guidelines-based care, and if it differs, why that makes sense in your situation. If the cost of treatment is a burden for you and your family, speak with your healthcare provider about your concerns and available options.

You can also contact the Lung HelpLine at 1-800-LUNGUSA to speak with medical professionals who can help guide you to available resources.

Learn more at Lung.org.

Support for this program was provided in part by Merck