Pulmonary arterial hypertension (PAH) is a form of pulmonary hypertension that causes the small arteries in the lungs to thicken and narrow. This causes high blood pressure in the lungs.
Although there is no cure for PAH, there are many treatment options that can help you manage your symptoms. It is important to work closely with your doctor to ensure your PAH treatment continues to work.
“Treatment ranges from drugs all the way to transplant,” says Richard N. Channick, MD, a pulmonologist at UCLA. Your treatment plan may include:
Vasodilators. Vasodilators, called vasodilators, help relax and open narrowed blood vessels to help blood flow. Your doctor may give you treatment by intravenous (IV) infusion, subcutaneous injection, pill, or inhalation. With inhalation, you will inhale the medicine through a machine called a nebulizer.
Anticoagulant drugs. These medicines can help prevent blood clots. The most common form is warfarin (Coumadin, Jantoven).
diuretics. These are “water pills” that help flush out excess fluid from the body.
Digoxin. This drug can help relieve your symptoms, strengthen your heart muscle to contract, and slow your heart rate.
Oxygen treatment. With this therapy, you breathe in air that has a higher concentration of oxygen than normal air.
Surgery. In some cases, you may need surgery. There are several different types, including pulmonary endarterectomy, balloon pulmonary angioplasty, atrial septostomy, and grafting.
There are other treatments that are less commonly used for PAH.
“We have a whole bunch of potential drugs from which to choose. Which drugs we choose and how we use them is also a very important topic,” Channick said.
The main goals of treatment are to relieve symptoms and slow the progression of the disease. If your PAH seems to be getting worse, you may need to explore new treatment options.
How can you tell if your PAH treatment is working?
“It doesn’t matter how you go on the first day; it’s really how you respond to treatment, which will determine how you perform long-term,” Channick said. There are several different ways to measure the success of someone’s PAH treatment:
functional class. Your doctor may simply ask how you feel about your current treatment. They’ll ask you to rate your symptoms on a scale that experts call a functional rating.
“Functional grades range from one to four. One is [person] No restriction on activity, four means they will show symptoms even at rest or minimal exercise, two or three in between,” Channick said. “Their functional class can help us determine if they will how and whether they need additional treatment. “
Exercise ability. “We can measure this using what we call a ‘6-minute walk’ test, or how far a patient can walk up and down a hallway in six minutes. It’s a very powerful measure of how well a patient is doing,” he said. Say.
other tests. “We then do more direct measurements like blood tests, echocardiograms to see how the right ventricle is functioning, or in some cases, even repeated cardiac catheterizations,” Channick said.
No matter which method your care team uses, it is important to reach out to your doctor and let them know how you are. Every 3 to 4 months is ideal. Don’t wait until you think your situation has gotten worse. With regular appointments and tests, they can more easily determine your risk level.
“It’s important to see your doctor regularly, with or without symptoms,” Channick said. “We have many examples [people] They feel like they’re doing great, but maybe they’re not doing as well as they think they are. “
Symptoms don’t always tell the whole story, but it’s still important to pay attention to how you’re feeling.
“Have you noticed a decrease in your exercise tolerance? For example, things you could do a month ago, you can’t do now,” says Channick.
Weight changes are another potential warning sign.
“One of the problems with PAH that doesn’t respond to treatment is fluid retention. It might not always be obvious. People hide fluid where they can’t see it,” says Channick. “Checking weight regularly may help us prevent real problems without even requiring hospitalization.”
If you notice other symptoms such as:
- Shortness of breath during normal activities (such as climbing stairs)
- swelling in the ankles, abdomen, or legs
- chest pain
- bluish skin or lips
- irregular heartbeat
- Difficulty breathing even when doing nothing
What happens if your PAH treatment doesn’t work?
If one treatment doesn’t control your symptoms, there may be others that can.
“most [people] Started taking two different medications. Then, we do a risk analysis, and if they’re not low risk, and they still have limitations, then we usually add a third drug to the regimen,” Channick said. “So they could end up with three different pulmonary arterial hypertension treat. “
How you respond helps determine if you are a good candidate for an infusion.
“There’s a general consensus that even if pills or other medications don’t work, infusions can help. Ultimately, if those don’t work, then we look at lung transplantation,” Channick said.
But that doesn’t happen very often.
“Most patients benefit only from current therapies and don’t need extreme approaches,” Channick said. “Before these drugs, the median survival for this condition was less than 3 years. Now, we have long-term survivors. We can still do better, but we’ve come a long way.”