Clinical Diagnosis of Pulmonary Hypertension



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PAH: The Value Of Good Communication With Your Doctor

Pulmonary arterial hypertension (PAH) is a rare but serious and potentially life-threatening condition that worsens over time. If you or a loved one was recently diagnosed with PAH, you might have a lot of questions on your mind such as: What causes it? Will it affect your day-to-day life? Is there a cure?

There isn't a cure for PAH. But you can manage it with medications, regular care, and, if necessary, certain procedures like a lung transplant. For the best outcome, it's important to work closely with your doctors and other members of your health care team.

In fact, research shows that if you maintain an open line of communication with your doctor, it can improve your odds overall. With routine care, your health care team can help customize a treatment plan that better serves your needs.

Communicating With Your Doctor

Good communication with your doctor is a two-way street. Clarity about what medications you need to take, how many times you need to take them, and possible side effects are all important things you should openly discuss with your doctor.

You'll need to trust, share, and inform your doctor about your health care problems. In return, your doctor should listen to your needs and concerns, present options, and work with you and your family's specific needs.

For your first appointment with a PAH specialist, try to come prepared. Soon after your diagnosis, the flood of PAH-related information might overwhelm or confuse you. It might be helpful to take a trusted friend or family member along for support.

You should:

Stay organized. Being on top of things can help reduce your anxious feelings or stress. Plus, it will help you maximize the short amount of facetime you get with your doctor and focus on the most pressing issues.

For example, before the appointment, write down any questions you may have. Your questions can look like:

  • What is PAH?
  • What's the cause of my PAH?
  • Can other conditions cause PAH-like symptoms?
  • What tests or exams do I need?
  • How advanced is my disease?
  • What's the standard treatment?
  • During the appointment, you should:

  • Listen carefully to the recommendations.
  • Take notes if it helps.
  • Ask for clarifications, especially if your doctor uses medical terms you don't understand.
  • You can also ask if you can record your appointment. This way, if you miss anything, you can come back to it.

    Know your medical history. Your doctor will collect all relevant information to customize a treatment plan for you. This includes your medical history. List all medications and supplements you're taking. Also, talk about all the health conditions you or your close family members have had in the past.

    Advocate for yourself. Currently, there are over a dozen FDA-approved medications for PAH.Your treatment plan will depend on what caused your PAH and how advanced it is. If your doctor recommends certain medications, feel free to ask why they chose those particular ones.

    You should ask questions like: 

  • What side effects can they have on you?
  • Are there generic, cheaper options?
  • Could they interact with any of your current medications?
  • Are there new experimental drugs you qualify for?
  • Monitor your symptoms. Keep a close eye on your PAH symptoms. If you notice changes or new symptoms, let your doctor know as soon as possible. Don't wait until your next appointment. If necessary, they'll adjust your treatment plan accordingly.

    Make a plan to stay in touch. Ask your doctor the best way to reach them between appointments for emergency and non-emergency concerns. 

    Follow Your Treatment Plan to Prevent Complications

    Besides medications and routine tests, your doctor may recommend certain dos and don'ts such as exercise and lifestyle changes to manage your PAH symptoms.

    Dos can include a heart-healthy diet and regular exercise, especially if PAH has caused you to have heart failure or high blood pressure. Before starting a new exercise routine, check with your doctor to see if it's the right amount of physical activity for you.

    Don't plan a pregnancy without running it by your doctor. If you get pregnant, let your doctor know right away. That's because PAH increases your odds of pregnancy complications. In some cases, it can be life-threatening.

    If you're planning to travel by air or to a mountainous area, check with your doctor. For some people with certain types of PAH, traveling higher than about 8,000 feet could make it harder to breathe and cause complications. At that height, oxygen levels are low and could make your symptoms worse.

    Avoid saunas and hot tubs, as they could put a strain on your heart and lungs.

    Follow-Up Appointments and Supportive Care

    Make sure to go to your in-person follow-up appointments to keep your treatment plan up to date. Besides this, your doctor may recommend other forms of supportive care to improve your quality of life as you learn to live with PAH.

    For additional care, you can:

  • Join a support group.
  • Get counseling or therapy from a mental health professional.
  • Get your recommended vaccines to protect yourself from bacteria or viruses that could put your health at risk.
  • Know Your Health Care Team

    Your health care team will include more than just your doctor. This can range from specialists to other health care workers, such as:

  • Cardiologists (heart specialists)
  • Pulmonologists (lung specialists)
  • Pharmacists
  • Physician assistants (PAs)
  • Nurses and nurse practitioners (NPs)
  • Respiratory therapists (RTs)
  • Medical assistants (MAs)
  • Registered dietitians (RDNs)
  • Social workers
  • Case managers
  • In some cases, your care team might include a palliative doctor. They are medical doctors who specialize in pain management. They can help control your PAH-related symptoms. Palliative doctors will also listen to your fears and concerns, whether they're emotional, spiritual, or both.

    If you're seeing multiple specialists outside of your health care team, tell your doctor. This will help them coordinate your care and make sure your treatment plan doesn't overlap or cause problems.


    Exercising When You Have Pulmonary Hypertension

    Because pulmonary arterial hypertension (PAH) often causes shortness of breath, swelling, and fatigue, exercise may seem like the last thing you should do. But the right kind -- with your doctor's OK -- can help symptoms and boost quality of life.

    How Exercise Helps

    Almost any way you slice it, exercise is good medicine.

    "We call sitting the new smoking," says Eugene Chung, MD, chair of the American College of Cardiology Sports and Exercise Cardiology Leadership Council and professor of internal medicine at the University of Michigan. "If you live a predominantly sedentary lifestyle, the biggest bang for your buck, health-wise, is to get up and start moving around more often."

    Regular exercise helps the heart and body work smarter, not harder. It keeps your blood vessels in good shape and eases inflammation. All these have a positive impact on PAH.

    Here's how: PAH stresses the right ventricle of your heart. This stress starts a cascade of changes in your body. One of those is a bump in adrenaline, the hormone that triggers your "fight or flight" response.

    "Exercise has been shown in multiple studies to help with decreasing inflammation and train the heart to respond to the adrenaline surge," says Chung.

    This improves your peak exercise heart rate. That's how many beats per minute your heart can safely pump when exercising. It also lowers your blood pressure, which helps keep the cascade of changes from happening in the first place.

    In short, exercise helps break the cycle of worsening PAH symptoms.

    Best Ways to Move

    "Any exercise program should be started in consultation with your doctors, and you should be followed regularly," says Chung.

    Your doctor understands your limitations and can tell you what's OK. Ideally, you'll exercise under the supervision of a cardiopulmonary rehabilitation program. As for the type of exercise that's best, Chung says they focus on aerobic activity. That's the kind that gets your heart pumping, not isometric moves that make you hold your muscle in a contracted position or high resistance exercise like weightlifting.

    To get your blood pumping, you can try:

    Walking. Get your steps in on a treadmill or by taking a brisk stroll around your neighborhood. Aim for 1-hour sessions three times a week.

    Swimming. Working out in water tones your muscles without straining your joints. You can do water aerobics or simply swim laps.

    Cycling. Elliptical bikes and recumbent bikes are a safe way to cycle without risking a fall.

    Other types of exercise include:

    Yoga. Although there isn't much research about the direct benefits of yoga on PAH, its slow and mindful stretching reduces stress and lowers inflammation in your body.

    Light resistance training. You can keep your muscles flexible and strong by using light weights (soup cans can work well) or just body weight. A rehabilitation specialist can teach you moves such as chair squats, wall pushups, calf raises, bicep curls, and more.

    It's important not to lift heavy weights because it can worsen symptoms.

    "If you were to push it and do more high-intensity weightlifting, there's a chance that, depending on the cause of pulmonary hypertension, you could put increased strain on the right side of the heart," says  Chung.

    You're more likely to hold your breath as you lift as well, which raises the pressure in your chest cavity.

    What to Watch For

    Like with any exercise routine, be on the lookout for signs you've done too much. Keep these safety tips in mind:

  • Exercise at a time of day when you feel best and have the most energy.
  • Don't work out solo: Try to sweat it out with a buddy.
  • Never hold your breath while working out.
  • Always warm up before you work out and cool down afterward.
  • Start small and do more once your body is ready.
  • Tell your doctor about any concerning side effects, like swelling or more shortness of breath than usual.


    Decade Is A Tech-noir Adventure Game Where You Send Children Back In Time To Prevent Metal Rain From Pulping The Planet

    Create multiple futures from a doomed past

    One of my biggest challenges as a writer has been tempering my love of vague gestures at metaphysical concepts with the revelation that the people who read my articles also, apparently, can't read my mind. Pah. A skill issue if I ever saw one, honestly. Decade is a fascinating adventure game that drew me in with its apparent vagueness but then, like some sort of considerate, sensible coward, went on to explain itself well in on its Steam page.

    It's the end of the world, and you're not too happy about it, so you'll be shoving children in a time machine with little more than a rotting Lunchly and some instructions to help you figure out exactly what went wrong.

    What are you dressing as for Halloween? Me, I'm dressing as someone trying to bring back "tray-tray" in an effort to give Edwin a seizure. Here's the tray-tray:

    Watch on YouTube

    "Decade is a narrative point-and-click adventure game where you will send children back into the past - ten years at a time - to learn how the world ended, and what you can do to change it," reads the Steam page. "Investigate documents, technologies, and artefacts to understand history, manage the children and their abilities as they age dramatically, and try to make the right decisions as you create multiple futures that you can venture into."

    "The world is dead. A metal rain has battered the earth into a pulp, making life unsustainable, " it continues. "You were twelve when you left the crumbling facilities designed to protect the last of humanity. Thirteen when you, and three younger children you managed to protect, the last people alive, found safety in a sophisticated bunker. Now you're seventeen. This bunker won't protect you forever, but it does contain a time machine that you now know how to use."

    There's no release date on this one yet, but if you're up for a weighty, poetic adventure game in the meantime, I don't think I've had the chance to shout about Norco nearly enough on this website yet. Would "it's tied with Disco Elysium as the best written game I've ever played" tempt you?






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