Being diagnosed with pulmonary arterial hypertension (PAH) may be overwhelming. Working with your doctor to create a care plan can ease your symptoms and give you some peace of mind.
Treatment can’t stop or reverse this aggressive disease, but medications can help slow the progression of PAH and improve your symptoms.
PAH is a type of high blood pressure. It affects your pulmonary arteries and the right side of your heart. Your pulmonary arteries carry blood from your heart to your lungs where fresh oxygen is pumped into your blood.
If you have PAH, it’s tricky for these arteries to carry enough oxygen and blood to your body.
Over time, PAH can get worse. It can lead to death if your organs don’t receive enough oxygen. The goal of PAH drugs is to stop further damage to your pulmonary arteries.
PAH may cause symptoms, which can include:
- shortness of breath
- chest pain
Drugs for PAH can also help relieve these symptoms.
Once you’re diagnosed with PAH, you’ll work closely with your doctor. Together, you’ll make a treatment plan, which includes taking medications. To properly manage your condition, you need to take your PAH medication long term.
There are several types of medications used to treat PAH.
Many people with PAH need to take vasodilators, or blood vessel dilators. These drugs work to open blocked and narrowed blood vessels in your lungs. They can help more blood and oxygen flow through your body.
Common side effects of these drugs can include:
- pain in your arm, leg, or jaw
- leg cramps
Examples of vasodilators include:
Iloprost (Ventavis) and treprostinil (Tyvaso)
Some vasodilator drugs are inhalable. These include iloprost (Ventavis) and treprostinil (Tyvaso). These drugs are inhaled through a nebulizer, which is a breathing device that delivers medication to your lungs.
Epoprostenol (Flolan, Veletri)
Other vasodilators are given through an intravenous (IV) injection, which means the drug is injected into your vein. These drugs include epoprostenol (Flolan, Veletri). You receive this drug continuously.
These vasodilators use a pump that’s often on a belt you wear around your waist. Your doctor attaches the pump to you, but you give the drug to yourself as you need it.
Treprostinil sodium (Remodulin)
Another vasodilator is called treprostinil sodium (Remodulin). Your doctor gives you this drug through an IV, or you may also receive it subcutaneously, or under your skin.
Treprostinil sodium is also available as an extended-release oral tablet that’s marketed under the brand name Orenitram. “Extended release” means the drug is released slowly into your body.
First, you receive treprostinil sodium as an injection from your doctor. Then you start taking part of your dosage in the tablet form.
Your doctor slowly increases your oral dosage and decreases your injection dosage. Eventually, you only take the oral form of this drug.
Selexipag (Uptravi) is another vasodilator for PAH. It comes as an oral tablet. This medication may be better at slowing the progression of PAH than improving the symptoms of PAH.
People with PAH have a higher risk of blood clots in their lungs. Anticoagulants are blood-thinning drugs that prevent blood clots from forming. They also stop clots from blocking the small pulmonary arteries.
Warfarin (Coumadin) is one example of an anticoagulant.
A common side effect of this class of drugs is increased bleeding if you’re injured or cut.
If you take a blood thinner, your doctor will monitor your condition closely. They’ll likely do routine blood tests to check how the drug affects your body.
Your doctor may also make changes to your diet and your medications to prevent dangerous interactions. Be sure to tell your doctor about all medications, vitamins, or herbs you’re taking.
Endothelin receptor antagonists
Endothelin receptor antagonists work by reversing the effect of endothelin. Endothelin is a natural substance in your blood. If you have too much of it, it can slowly build up on the walls of your blood vessels.
As it builds up, your blood vessels become narrower. This can make it even more difficult for blood and oxygen to flow to the rest of your body.
All of the drugs in this group are oral drugs. They include:
- ambrisentan (Letairis)
- bosentan (Tracleer)
- macitentan (Opsumit)
Common side effects of endothelin receptor antagonists can include:
- anemia (low red blood cell levels)
Serious side effects of these drugs can include:
- Low blood hemoglobin levels. This means your blood can’t carry oxygen as well as it should.
- Liver damage. Symptoms can include:
- loss of appetite
- pain on the right side of your stomach
- dark urine
- yellowing of your skin or the whites of your eyes
Soluble guanylate cyclase stimulators
Riociguat (Adempas), an oral tablet, is one example of this type of drug.
It comes with serious risks. Because of these risks, you can only get this drug through certain certified pharmacies.
Common side effects of Riocoguat can include:
- upset stomach
Serious side effects of this drug can include:
- very low blood pressure
- bleeding, including bleeding in your breathing passages
Riociguat interacts with several drugs. These include sildenafil and tadalafil, which are two other drugs used to treat PAH.
Riociguat also interacts with antacids and cigarette smoke. Tell your doctor about all medications you’re taking.
Other medications used to treat PAH
Your doctor may prescribe other drugs to treat your PAH. These can include:
Sildenafil (Revatio) and tadalafil (Adcirca)
These drugs work like vasodilators. They open narrowed blood vessels, which helps blood flow through your lungs more easily.
Calcium channel blockers
Amlodipine and nifedipine are calcium channel blockers.
High doses of calcium channel blockers can help a small number of people with PAH. These drugs relax blood vessel walls, which encourages more blood to flow through your body.
Digoxin helps your heart pump more effectively, which causes more blood to flow. It also causes more blood to reach your lungs.
These drugs remove extra fluid from your body. This makes it easier for your heart to move blood into your lungs and around your body.
PAH is often caused by another illness like heart disease or contracting HIV. Treating your other condition is important if you have PAH.
Keep in mind that you and your doctor must be extra careful when treating other conditions along with PAH. This is because some drugs can be dangerous for people with PAH.
For example, certain medications may affect your pulmonary arteries and increase your risk for dangerous side effects. These include anesthetics and sedatives. Tell your doctor about every drug you’re taking.
The best treatment for your condition depends on several factors, which include:
- your health history
- how advanced your PAH is
- what treatments you’re undergoing for other health problems
Treating the condition that caused your PAH won’t cure it, but it may slow the progression. It may also improve some of your PAH symptoms.
Work with your doctor to create a care plan that’s right for you. Your doctor may change your treatment if you have certain side effects or if your condition gets worse.