Managing Your PAH and Impact on Life Expectancy
Once you have been diagnosed with PAH, your doctor can assess your risk status to set treatment goals, and guide you on how best to manage your condition. Studies show that patients who optimize their therapy early on and lower their risk status may have a better prognosis, or long-term outcomes, including life expectancy.
What is risk status
(and your prognosis)?
You're likely familiar with your functional class and other tests that describe the status of your PAH today. But most people also want to know how their PAH will impact them in the future. PAH experts now know how to use the information about your functional class and 6-minute walk test, along with other test results, to determine how PAH may affect you in the next 5 years – it's called risk status. Risk status goes beyond determining how PAH is affecting you today and helps your doctor assess the chance that your PAH will get worse in the future.
There are 3 levels of risk: low, intermediate and high. If you're low risk today, that means there's a low chance of your PAH getting worse. If you're intermediate or high risk, there is a higher chance that your PAH will get worse. Your doctor may consider adjusting your dose or adding a new medicine to improve your risk status. The goal is to improve your risk status today to lower the chance of your disease getting worse in the future.
How is risk status assessed?
PAH experts now know that by continuing to monitor your condition and assess your risk status, they can work to optimize your treatment plan. Below are 3 of the most common assessments used to help evaluate your risk status. Click on each assessment to learn more about what it measures and how the results can help in assessing your risk status. Slide the bottom arrow from left to right to see what the results may be for low, intermediate and high-risk status.
Keep in mind that your doctor will use these, as well as a combination of other test results and information, to assess your risk status and determine your treatment plan. With the right plan, many patients will continue to see improvements that go beyond their initial therapy.
Set treatment goals to improve your risk status
It's important for you to be clear and honest with your doctor about your symptoms and to discuss treatment goals that will help improve them. For example, you may want to set a specific goal of being able to walk further without feeling short of breath.
To help meet your treatment goals, your doctor will determine the appropriate treatment approach for you. PAH is associated with an imbalance of three natural substances in your body, and studies have shown that treating PAH with only one type of medication might not be enough.
You may want to talk with your doctor or PAH specialist to see if treatment with a combination of medicines can help you reduce symptoms, feel better, and do more.
Be your own best advocate
The key to staying on top of your disease is to find a treatment plan that helps reduce your symptoms, increases your ability to exercise, helps you feel better, and improves your long-term prognosis. Your doctor will monitor your test results, but you're the one who will really know how you're doing day-to-day. It's helpful to use a journal to keep track of your symptoms, along with the activities that seems to trigger them. That way, at your next visit, you can tell your doctor how you've really been feeling so your care team can better understand how you are really doing.
Remember, when it comes to PAH, you may need more than one medicine to help control your symptoms and keep your disease from getting worse. Ask your doctor about a treatment plan that helps you feel better so you can do more.
Find a PAH specialist
Because PAH is a rare disease, not all heart and lung specialists in your community have expertise treating it. For this reason, people living with PAH are encouraged to seek out a doctor and treatment team that specializes in treating PAH. These doctors are committed to treating PAH—they've had specific PAH training, they keep up with the latest research, and they have offices that support the special needs of people with PAH.Find a PAH Specialist
Make the most out of your visit
Download this Doctor Discussion Tool & Visit Checklist to guide your next appointment and help you ask the right questions.Doctor Discussion Tool
Build your personal PAH care network
An important part of living well with PAH is creating a strong network of care professionals, friends, family, and other resources that you can rely on. There are PAH support groups where you can tap into the experience of others who have learned to effectively manage this condition. With a bit of patience, planning, and flexibility, many people affected by PAH find ways to work, travel, exercise, and do some of the things they loved before they were diagnosed. Along with your doctor, your treatment care team may include:
- A care nurse experienced with PAH
- Your Specialty Pharmacy
- Family and friends tasked with specific aspects of caregiving and support
- PAH Support Group
- Other patients you can connect with via Patient-to-Patient Support Lines, email or phone
More things you can do on your own
There may not be a cure for PAH today, but there are ways to manage your condition. Making some small lifestyle changes can go a long way toward helping you feel better. Ask your doctor about:
- Eating a balanced, healthy diet
- Minimizing your salt intake
- Staying active with a doctor-approved exercise program
- Weighing yourself regularly and reporting to your doctor
- Getting a good amount of sleep every day
- Staying up-to-date on PAH treatments