Do more for your PAH Treatment
When you have PAH, being "stable" or "feeling okay" may seem good enough. After a while, some people may accept the idea that they'll never feel better or get closer to the life they had before PAH. But you don't have to accept "good enough." Prostacyclin-class medicines have been shown to be an effective PAH treatment option to reduce symptoms, increase 6-minute walk distance, reduce NT-proBNP (an indicator of the strain on your heart), slow disease progression and improve your functional class, which means you may be able to feel better and do more.
What is Prostacyclin (PCY)?
Prostacyclin (pros-tuh-sahy-klin)—or "PCY"—is a natural substance in your body that works in different ways within the blood vessels. PCY is essential to help your lungs function properly. Normal PCY levels help maintain blood vessel health, keeping them open and allowing normal blood flow. Prostacyclin also helps prevent clotting, and slows down rapid cell growth (called proliferation), which prevents blood vessels from becoming too thick.
People with PAH may not produce enough natural prostacyclin. When you have too little prostacyclin, the vessels of the lungs become narrow and restrict blood flow. That's why your doctor may consider prescribing a Prostacyclin-class medicine.
See how PAH affects blood vessels
Prostacyclin-class medicines target the prostacyclin pathway and mimic some of the effects of natural prostacyclin that your body lacks, to help the blood vessels in your lungs stay open. They may help prevent blood platelets from clumping together (forming clots), which can also narrow blood vessels in the lungs.
Prostacyclin-class medicines may work by:
- Widening or opening the blood vessels (known as vasodilation)
- Slowing down the vessel thickening process
- Preventing clotting (clotting can narrow blood vessels)
- Smoothing the vessel lining, which can help prevent the vessel from becoming too thick.
Benefits of Prostacyclin-
In clinical studies, Prostacyclin-class medicines have been shown to
- Improve 6-minute walk distance
- Improve symptoms associated with exercise, like shortness of breath and fatigue
- Improve functional class
- Slow disease progression (keep PAH from getting worse)
- Reduce blood pressure in the lungs
- Improve NT-proBNP numbers
Because these are key elements in determining your risk score, these benefits indicate that Prostacyclin-class medicines may help improve your risk status.
Side effects of Prostacyclin-
You may experience side effects with prostacyclins. The most common side effects of this class of medicine include:
- Flu-like symptoms
- Jaw pain
- Muscle spasm
There may be different side effects depending upon how you take the medicine. PAH specialists are familiar with these expected side effects and know how to help manage them.
Talk to your doctor about the side effects before you start taking prostacyclin therapy. You will be prepared and know what to do if they do occur. Tell your healthcare team if you have any side effect that bothers you or that does not go away.
Getting started on Prostacyclin-class treatment
If your doctor prescribes a Prostacyclin-class medicine, it will be delivered by a Specialty Pharmacy.
Specialty Pharmacies are different from your local pharmacy. They offer personalized care and teams of trained, experienced nurses and pharmacists who provide the customized support you may need, including:
- 24/7 phone support
- In-home nurse visits to explain more about the medicine
- Home delivery of medicines
- Scheduled follow-up calls from Specialty Pharmacy nurses and pharmacists