NAVIGATING PF
Medications for PF include:
Corticosteroids (Prednisone): This drug suppresses the immune system and decreases
inflammation. Corticosteroids may prevent lung scarring for some lung conditions. However,
they are not effective for treating IPF. They may cause unwanted and possibly serious side
effects if used as a chronic therapy for IPF.
Cyclophosphamide (Cytoxan): This is a chemotherapy drug (usually used to treat
certain types of cancer) that can also suppress inflammation. Used with a corticosteroid, it
may allow for a reduction of the corticosteroid dose so that fewer side effects occur.
Mycophenolate mofetil (CellCept ® ): This medicine suppresses the immune system. It
can decrease inflammation and help control the disease while allowing corticosteroid doses
to be reduced (and possibly discontinued).
Azathioprine (Imuran ® ): This is an anti-inflammatory drug used in treating autoimmune
diseases, such as rheumatoid arthritis or other autoimmune conditions that can involve the
lungs and may cause lung scarring. Azathioprine may be used in place of corticosteroids or
allow corticosteroid doses to be reduced.
Nintedanib (Ofev ® ): Nintedanib, a relatively new, FDA-approved antifibrotic drug, can
slow down lung scarring in patients with IPF. Although clinical testing showed that it can
slow down the decline in lung function, it is not a cure for IPF and has not been approved
for the treatment of other types of PF.
Pirfenidone (Esbriet ® , Pirfenex ® , Pirespa ® ): Pirfenidone is another relatively new, FDA-
approved antifibrotic medicine. Clinical trials have shown that it slows down the progression
of lung scarring and loss of lung function in patients with IPF. It is not a cure for IPF and is
not approved for the treatment of other types of PF.
Traveling with Oxygen
Air travel has restrictions
related to oxygen. You
cannot travel on an airplane
with an oxygen canister but
must use either an airline-
supplied oxygen canister or
an FAA-certified portable
oxygen concentrator. Other
requirements and paperwork
may apply, so check with your
airline and/or oxygen supplier
at least 2 weeks before your
planned travel.
Your medical team may also suggest the following
treatments for PF:
Supplemental oxygen therapy: Supplemental oxygen is often prescribed for PF patients
to prevent shortness of breath, keep oxygen saturation levels at the correct ratios, and
provide some relief when the oxygen in the bloodstream is too low. Oxygen may help
prevent shortness of breath, improve quality of life, maintain activity, and prevent pulmonary
hypertension (when the pressure needed to pump oxygen through the lungs is too high).
Oxygen is not addictive, meaning increased use won’t make you need it more. Oxygen
therapy may also help improve sleep, mood, and mental alertness; better allow the body to
conduct its everyday functions; and help prevent heart failure.
There are three types of oxygen:
• Compressed gas oxygen, which comes in steel or aluminum containers in different
sizes for home use and traveling.
•
• Liquid oxygen, which changes liquid into a breathable gas.
If your pulmonologist prescribes oxygen, be sure to ask about:
•
•
•
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Oxygen concentrators, electrical devices that remove nitrogen from the air, thus
concentrating the oxygen.
The oxygen flow rate or setting, so you receive the right amount of oxygen per minute of use
When you should wear your oxygen, such as during activity, while sleeping, or continuously.
Which type of oxygen equipment best suits your lifestyle needs.