What Is PAH?

Pulmonary arterial hypertension (PAH) is a rare but serious condition that causes the blood pressure in the arteries of the lungs to become too high. Blood pressure rises due to the constriction of the pulmonary arteries, which are the blood vessels that carry blood to the lungs (where the blood picks up oxygen). Narrowed arteries also mean the heart must work harder to pump enough blood to the lungs. Over time, the heart muscle can weaken, leading to heart failure.

Symptoms of PAH

The symptoms of PAH are similar to those of other heart and lung conditions, and often include:

  • shortness of breath
  • dizziness/fainting
  • weakness
  • fatigue
  • racing pulse or heart palpitations 

Symptoms usually get continually worse as the disease progresses, though many are treatable.

Treatments

Treatment for PAH generally involves managing and treating its symptoms, as there is no known cure for the disorder. Treatment options include both medical interventions and lifestyle changes. The most suitable forms of treatment usually depend on the type and severity of your PAH.

Medical Treatments

Certain medications can be helpful in the treatment of PAH, depending on the cause of the condition (if the cause is even known):

  • Blood thinners like warfarin can help to prevent clotting, keeping blood flowing as smoothly as possible.
  • Diuretics work by eliminating excess fluid from your body, including any fluid accumulating in the lungs and lower extremities.
  • Blood pressure medications, like calcium channel blockers, may be appropriate for some types of PAH.
  • Endothelin receptor agonists (ERAs) work to prevent further narrowing of the arteries.
  • Inhalers and prostacyclins, help treat and prevent shortness of breath.

In addition to medications, there are a few other medical options for treating the symptoms of PAH. Oxygen treatments help to increase the amount of oxygen taken in by lungs when the blood flow to the lungs is restricted. In certain situations, a lung transplant may even be an option.

Lifestyle

There a number of lifestyle changes than can positively affect the symptoms of PAH. The biggest obstacle for many PAH patients is the resulting lack of energy. This is difficult to deal with, especially if you were previously used to a more active lifestyle. Fortunately, a few minor adjustments can help you to conserve your energy, and save it for things you love to do:

  • Make a list. The simple truth is that you probably don’t have enough energy to do everything you used to do. But that’s okay. Make a list of the things you think should be done, and then prioritize that list. You may find that you can focus on the top priorities, and either let the chores at the bottom go or allow someone else to do them for you.
  • Work smarter, not harder. When performing a task at the top of your list, pay attention to ways that you can streamline the process. Don’t walk back and forth across your home gathering dirty laundry, when you could carry a basket and make only one trip. When you visit the grocery store, make sure your list is written in order according to where items are stocked.
  • Don’t bend over backwards. Lots of tasks become easier if you raise them by about six inches. Store items you use frequently on shelves or countertops that are easy to reach. Install a raised toilet seat. Have a friend raise floor-level appliances or low tables using bricks or cinder blocks. This is especially helpful for your washer and dryer to keep you from having to bend so much.

In addition to taking care of yourself physically, it’s crucial to take care of your emotional health. It’s easy to give in to feelings of fatigue, loneliness, and sadness. Combat these ways of thinking by making time (and saving energy) for activities you love, particularly social activities. And if you don’t feel like going anywhere, invite friends and family to come to you.

Potential Emergencies

Emergencies can happen to anyone, but being prepared is your first line of defense. Talk to your doctor about any potential emergencies that are unique to your particular situation, and what you should do if they occur. Don’t be afraid to call an emergency number if your symptoms suddenly worsen, or if you have any of these symptoms:

  • shortness of breath that is worse than usual
  • black or tarry stool
  • chest pain
  • fainting
  • high fever
  • coughing up blood or mucus

PAH is a serious disease. The good news is that even though there is no cure, as long as you manage your symptoms properly, and make a few lifestyle adjustments, you can continue to do what you love and live a fulfilling life.