CLINICAL VIGNETTES - 2004 - Journal of General Internal Medicine



crest syndrome and pulmonary hypertension :: Article Creator

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.


    Treating Pulmonary Arterial Hypertension

    Pulmonary arterial hypertension (PAH) is one of five types of pulmonary hypertension. It happens when the blood vessels in your lungs narrow and scar so much that it's hard for blood to move through them. This can trigger high blood pressure in the lungs.

    PAH can be hard to diagnose. That's because the symptoms – like shortness of breath – are common to several other medical conditions. There's no cure. But there are FDA-approved treatments. Here's what you need to know.

    How Is Pulmonary Arterial Hypertension Treated?

    What caused your PAH, the symptoms, and how advanced your condition is all impact what treatment might work best for you. It may take time to figure out, but your doctor is there to help.

    Options include:

  • Medications
  • Surgery
  • Oxygen therapy
  • Calcium channel blockers
  • Blood thinners
  • Diet changes
  • Exercise
  • What Medications Can I Take for Pulmonary Arterial Hypertension?

    Prescription drugs called vasodilators specifically treat PAH. These may be taken by mouth or given through an IVor subcutaneously, meaning under the skin. Some you inhale, or breathe in, from a nebulizer.

    In some cases, you may need more than one type of drug to get your condition under control. You might also need other therapies like extra oxygen along with diet and lifestyle changes to help you feel better.

    Intravenous (IV) treatments

    Epoprostenol is a vasodilator that relaxes your blood vessels so it's easier for blood to move through them. There are two brands: Flolan and Veletri.

    Flolan. In 1995, this was the first drug the FDA approved to treat PAH. It's given as a continuous injection through a vein for short-term use. A doctor can also surgically place a special catheter for long-term use. One study found that it helped make exercise easier and boosted survival rates.

    Flolan has to be mixed every day. You must also:

  • Refrigerate the medicine when you're not using it
  • Use ice packs to keep it cold during infusion
  • Have your blood count checked regularly
  • Your catheter also needs to be checked often for signs of possible infection.

    Side effects may include:

  • Headache
  • Diarrhea
  • Nausea
  • Jaw pain
  • Flushing of the skin
  • Dizziness
  • Swelling
  • Itching
  • Muscle or joint pain
  • Low systemic blood pressure
  • Selexipag IV (Uptravi). It can help slow the progression of PAH and lower your risk of being hospitalized. It's used only in patients who temporarily can't take oral medication. 

    Treprostinil (Remodulin). This is another IV formula of the same class of drugs as Flolan and Veletri.  It's also stable at room temperature and doesn't have to be mixed daily. It can also be given under the skin. 

    Veletri. It's a newer version of epoprostenol approved by the FDA in 2010. It doesn't have to be mixed daily, and you can keep it at room temperature. Your doctor may suggest Veletri if you have severe PAH and other treatments haven't worked.

    Side effects for Veletri and Remodulin are similar to those of Flolan. Catheters used for IVs should be checked regularly to avoid infections.

    Nebulizer treatments

    Iloprost (Ventavis).  You breathe it in through a nebulizer about six to nine times every day. If you have asthma or other conditions that can make it hard to breathe, this may worsen those symptoms.

    Treprostinil (Tyvaso). Your doctor may recommend this drug if you have moderate to severe PAH symptoms. You'll likely take it about four times a day. Research suggests it can help you walk longer distances.

    Side effects of iloprost and treprostinil are similar to those of  IV-based PAH drugs. But you may have less flushing or diarrhea with iloprost. Treprostinil may cause a sore throat and cough when you first start to use it.

    Oral treatments

    Ambrisentan (Letairis). This is a  once-a-day medication, but unlike bosentan, it doesn't affect your liver.

    Bosentan (Tracleer). This medication is taken two times a day. The drug could affect your liver function and hemoglobin levels. It's important to get your blood count and liver function tested regularly when you're taking bosentan.

    Neither bosentan nor ambrisentan is recommended for people who are pregnant. Experts recommend two separate birth control options. Monthly pregnancy tests are required to continue using these drugs.

    Sildenafil (Revatio). Small studies suggest this drug can improve blood flow in your lungs. The recommended dose is a 20-milligram pill taken three times a day, but it can be increased if you have a more severe form of PAH.

    Side effects of sildenafil include:

  • Headache
  • Back pain
  • Flushing
  • Possible drug interactions
  • Tadalafil (Adcirca). This is a once-a-day pill prescribed if you have moderate to severe PAH.

    Treprostinil (Orenitram) is the oral formulation of Remodulin and can substitute for the IV medications after you have stabilized.

    Other Ways to Treat Pulmonary Arterial Hypertension

    These include:

    Calcium channel blockers. This class of drugs, in select patients, can help lower the blood pressure in your lungs.

    Blood thinners. Your risk of forming blood clots is higher if you have PAH. Blood thinners, like warfarin (Coumadin), are given to most people with PAH to cut down on clots and improve overall survival. But warfarin interacts with several foods and drugs. Your doctor can tell you what to avoid.

    Diuretic. Sometimes called water pills, diuretics can help your body get rid of extra salt and water. This happens to most people with PAH. Your doctor will usually recommend one pill a day. But if you have a severe form of PAH, they may up the dosage.

    Digoxin. This is an older medication sometimes prescribed to people with severe PAH. It's designed to help your heart pump blood better.

    Oxygen therapy

    When you have moderate to severe PAH, it can make it hard to breathe properly and affect your day-to-day life. The low levels of oxygen can make the blood vessels constrict further, which can make your PAH worse.

    Getting a little extra oxygen can help you not only breathe better but also be able to get through daily tasks and routines. It can also boost your mood and sleep. While some may need extra oxygen every now and then, others may need it continuously.

    Oxygen therapy comes in three types:

  • Compressed gas oxygen. This is in steel or aluminum cylinders that you can use at home or when you travel.
  • Liquid oxygen
  • Oxygen concentrators. This device removes nitrogen from the air to provide a concentrated form of oxygen to breathe.
  • Before you try oxygen therapy, ask your doctor how much you need and how often you may need it. They can also guide you toward the type that works best for your lifestyle.

    Surgery

    If you have severe PAH and medications aren't working, your doctor may recommend a lung transplant. Lung transplant surgery is challenging and can come with serious complications. These include:

  • Infection
  • Kidney failure
  • Organ rejection
  • Your health care team can help you decide if it's right for you.

    Things You Can Do to Manage Pulmonary Arterial Hypertension

    There are some lifestyle changes you can try to help ease your symptoms.

    You can:

  • Get plenty of rest to ease fatigue.
  • Don't smoke, or try to quit if you do.
  • Avoid travel to high altitudes.
  • Avoid activities that can lower your blood pressure, like long, hot baths or saunas.
  • Eat a balanced diet rich in whole grains, vegetables, and fruits, and avoid too much salt.
  • Stick to your treatment plan. Don't make any changes without running them by your doctor.
  • Get recommended vaccines to avoid seasonal lung infections.
  • Try to exercise and stay as active as you can. But don't lift heavy weight or do intense workouts. Talk to your doctor first if you're not sure what's safe.


    How Lupus Affects The Lungs And Pulmonary System

    Inflammation caused by lupus may affect the lungs in many ways, and can involve the membrane lining of the lungs, the lungs themselves, the blood vessels within the lungs, and the diaphragm.

    Pleuritis

    The most common way that lupus can affect your lungs is through inflammation of the pleura, the lining that covers the outside of the lungs. The symptom of pleuritis that you may experience is severe, often sharp, stabbing pain in a specific area or areas of your chest. The pain, which is called pleurisy, is made worse when you take a deep breath, cough, sneeze, or laugh. You may also experience shortness of breath. Sometimes an abnormal amount of fluid will build up in the space between your lungs and your chest wall; when it leaks out it is called a pleural effusion. Pain from pleurisy, with or without effusions, is found in 40 to 60 percent of people with lupus.

    Pneumonitis

    The term for inflammation within the lung tissue is pneumonitis. The symptoms of pneumonitis that you may experience are fever, chest pain, shortness of breath, and cough. An infection caused by bacteria, virus, or fungi is the most common cause of pneumonitis.

    Chronic diffuse interstitial lung disease

    When inflammation in the lungs is chronic, it can cause scarring. This scar tissue can prevent oxygen from moving easily from your lungs into your blood and may cause diffuse (widespread) interstitial lung disease. The symptoms that you may experience include a chronic dry cough, chest pain, and difficulty breathing during physical activity.

    Pulmonary emboli

    Blood clots that block the arteries leading to the lungs are called pulmonary emboli. These blood clots will cause chest pain and shortness of breath, but can also lead to a decrease in oxygen flow in your lungs. You are at increased risk for pulmonary emboli if you have antiphospholipid antibodies, vascular damage, and/or an inactive lifestyle.






    Comments

    Popular posts from this blog

    Novitium's Generic Sildenafil for PAH Treatment Approved by FDA - Pulmonary Hypertension News

    Epoprostenol Via High-Flow Nasal Cannula Improves Severe Hypoxemia in PH - Pulmonology Advisor

    Analysis: Large pharma companies do little new drug innovation - STAT