Three Foundational Pathways
Small blood vessels in your lungs produce 3 substances: nitric oxide, endothelin, and prostacyclin. The right balance of them enables red blood cells to flow freely through the lungs and carry oxygen to the rest of the body.
When you have PAH, 1 or more of the 3 substances become out of balance. Currently, there is no test available to determine which of the 3 substances needs to be adjusted. Some patients may benefit from treatment addressing more than 1 pathway.
Studies Show Benefits to Treating All Three Foundational PAH Pathways
Endothelin Pathway
Too much ENDOTHELIN can cause blood vessels to narrow and tighten.
An ERA targets the endothelin pathway by blocking the effects of the extra endothelin that contracts blood vessels.
Nitric Oxide Pathway
The body may not have enough NITRIC OXIDE to keep arteries from getting stiff.
A PDE5 inhibitor targets the nitric oxide pathway by helping to keep blood vessels open.
Prostacyclin Pathway
The body may not make enough PROSTACYCLIN to keep arteries open.
A prostacyclin pathway agent targets the prostacyclin pathway by helping to prevent clotting in the arteries and by slowing the thickening of blood vessels.
Want to learn more about the Three Foundational Pathways and treating PAH?
Watch this quick video.
Treatment Goals for PAH
When you’re living with PAH, speaking out means knowing your status and taking an active role in your treatment plan.
Setting realistic goals with your doctor can help you focus on what matters most to you and provide motivation throughout your treatment. An overall treatment goal is to achieve a low-risk status which can include things like reducing symptoms, being able to exercise, and preserving right heart function.
Shared
Decision-Making
It's crucial to engage actively with your care team by discussing the specific ways your illness is affecting you. A collaborative approach enhances the quality of your dialogue with your healthcare providers. It ensures that you're at the center of all treatment decisions, combining your personal needs with the medical expertise of your care team.
