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Treatment

Can I take an inhaler for this? If this is a problem with the lungs, won't that work?

The underlying problem with PAH is not in the airways of the lungs, but in the blood vessels of the lungs. Nevertheless, an inhaled medication that travels through the airways to reach the blood vessels is of potential benefit.


While most of the medications that are used for PAH currently are either pills or medications that are delivered continuously through a vein or the skin, there are two medications that are delivered as an inhaled therapy.


There are two main ways to get medicines into the body through the lungs. One method is to use a metered dose inhaler and the other is through a device called a nebulizer.


Metered dose inhalers (MDIs) are devices that deliver medications into the lungs in the form of a powder or liquid spray. They are most commonly used by patients who have other lung disorders, including asthma and chronic obstructive pulmonary disease (COPD). The most common types of these medications are either bronchodilators (used to open up the airways) or inhaled corticosteroids (designed to reduce inflammation in the lungs). In both asthma and COPD, the airways can become constricted or narrowed. Inhaled bronchodilator medications help re-expand these constricted airways, improving airflow. Currently there are no metered-dose inhalers available for PAH therapy.


A nebulizer is another device which can deliver medications into the lung. With this device, pressurized air is used to "volatize" (turn into a mist) a small amount of liquid medication. A patient breathes this mist in and out of their lungs through a small mask-like device. There are currently two nebulized therapy options approved for PAH called iloprost (Ventavis®) and treprostinil (Tyvaso®). These medications are in the family called prostacyclin medications that acts as a pulmonary vasodilator. These medications are delivered through the lungs instead of through a vein or through the skin.