When a person has high blood pressure of the blood vessels running from their heart to their lungs, doctors diagnose them with pulmonary arterial hypertension. PAH is one type of pulmonary hypertension.
The underlying causes of PAH are complex, but studies have shown that a molecule called prostacyclin is related to the condition. People with PAH show lower amounts of prostacyclin circulating in their bodies. This has led researchers to conclude that an imbalance between prostacyclin and other molecules may lead to PAH.
Lower amounts of prostacyclin in the body cause the blood vessels in the lungs to narrow, leading to the symptoms of PAH. Many PAH medications approved by the U.S. Food and Drug Administration (FDA) affect the prostacyclin pathway to help alleviate symptoms and slow down the disease.
Despite the health benefits of prostacyclin analogues — medications which act like the body’s own prostacyclin — these medications do come with side effects. This can limit the use of these drugs.
Injury or inflammation triggers the innermost layer of blood vessels (endothelial cells) throughout the body to make and release prostacyclin.
Prostacyclin travels from the inside layer to other parts of blood vessels, activating nearby receptors. This works similarly to putting a key into a door lock. Once prostacyclin links to these receptors, it causes the following actions:
Proliferation of smooth muscle happens when extra cells grow in the walls of the blood vessels. This leads to high blood pressure. Prostacyclin helps counteract this.
Since people with PAH have lower amounts of prostacyclin circulating in their bodies, they do not get these benefits of prostacyclin. Instead, they experience higher levels of inflammation, smooth muscle overgrowth of their blood vessels, blood clotting, and a narrowing of blood vessels.
For the treatment of PAH, doctors often prescribe prostacyclin analogues. These medications help to counter prostacyclin imbalance by acting as stand-ins for the body’s own prostacyclins.
Prostacyclin analogues help to decrease inflammation, block smooth muscle overgrowth, limit blood clotting, and open blood vessels. In that way, they do the same activities as natural prostacyclin in the body.
Doctors usually recommend prostacyclin analogues for people with PAH who are in a higher risk group, have more advanced PAH, or are not responding well to other therapies. This is because getting the dose right with prostacyclins can be difficult. Also, people on prostacyclins for PAH can develop a tolerance to the medication — along with unwanted side effects. Side effects of prostacyclin analogues may include:
Through clinical trials, the FDA has approved one prostacyclin medication (epoprostenol), two prostacyclin analogues (iloprost and treprostinil), and one prostacyclin receptor agonist (selexipag) for the treatment of PAH.
A prostacyclin receptor agonist does not try to mimic the shape of the body’s own prostacyclin — like the prostacyclin analogues do. However, a prostacyclin receptor agonist still acts like the body’s own prostacyclin and has a similar effect on the body as natural prostacyclin.
People need careful dose monitoring and adjustments by their doctor when taking any of these medications:
Other groups of medications for the treatment of PAH include endothelin receptor antagonists and phosphodiesterase-5 (PDE-5) inhibitors.
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In addition, injectable forms of prostacyclin drugs such as subcutaneous treprostinil, can cause pain at the injection site on the body.
Talk to your doctor about what side effects are possible from your PAH medications. Your doctor can help you understand what is normal and what is a cause for concern. They can explain whether side effects will diminish over time or if there are ways to lessen their severity. Tell your doctor if side effects are making it difficult for you to stick with your treatment.
Many treatments for pulmonary arterial hypertension, including prostacyclin drugs, are expensive in the U.S. The Pulmonary Hypertension Association recommends resources that can help people afford their medications. These resources include programs offered through drug manufacturers that help people afford their prescriptions.
You can also ask your health care provider about resources to make medications more affordable.
On myPHteam, the social network for people with pulmonary hypertension and their loved ones, more than 40,000 members come together to ask questions, give advice, and share their stories.
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