Get the facts on one of two common IPF treatments with the Pulmonary Fibrosis Foundation’s new Fact Sheet Series.

Pulmonary Fibrosis Foundation

In its commitment to serving the PF community, the Pulmonary Fibrosis Foundation has created a series of 13 fact sheets for educating patients on various forms of PF and medications for treating the disease.

Below are highlights of its fact sheet on Esbriet® (pirfenidone).*

What it is and how it works

Esbriet (pirfenidone) is an oral drug for IPF approved by the US Food and Drug Administration in 2014, shown in clinical trials to slow disease progression. Esbriet inhibits cells’ ability to produce scar tissue in the lungs, which slows disease progression (it does not improve lung function).

Who should and should not take it

Professional medical societies advise Esbriet for people with IPF, and that patients be able to choose the medication based on personal preference; for example, some may choose not to take Esbriet based on potential side effects.

Notably, pregnant or breastfeeding women should avoid Esbriet, and the drug may not be suitable for those with liver or kidney issues. People who smoke should first talk to their doctor about ways to quit before starting Esbriet, since smoking can lower blood levels of the drug.

Those with IPF are encouraged to speak with their doctor about whether Esbriet is appropriate for them.

Side effects and drug interactions

Common gastrointestinal side effects of Esbriet are upset stomach, nausea, abdominal pain, diarrhea, vomiting, and acid reflux (heartburn). These can often be kept to a minimum by taking Esbriet with food; strictly adhering to directions; and taking any added medications like antacids, as recommended.

Other symptoms include dizziness, tiredness, headache, loss of appetite, weight loss, joint pain, and rash from sensitivity to sunlight. Risk of rash can be decreased by avoiding sunlight and sunlamps, using sunblock daily (SPF 50 or more), and avoiding exposed skin with long pants, long sleeves, and a brimmed hat.

People are advised to speak with their doctor if they experience any of these side effects.

Additional concerns are increased liver function counts upon blood testing, though no incidence of liver disease has been known to occur; low white blood cells; and angioedema (an allergic reaction causing swelling under the skin).

Drug interactions can occur with the antidepressant fluvoxamine and antibiotic ciprofloxacin increasing Esbriet levels in the body. It is important you inform your doctor and pharmacy if you are taking either of these, or of any changes in medication (prescription and over-the-counter) or dietary supplements (vitamins, herbs, etc.).

Physician monitoring

People taking Esbriet are watched closely by their doctor for side effects and undergo monthly blood testing for liver function during the first six months, and then every three months. If side effects occur, a doctor may add other medications, reduce the dose, or stop Esbriet temporarily or permanently.

How to take it

Esbriet comes in both 267mg capsules and tablets and 801mg tablets. The FDA-approved dose is 801mg taken three times per day. People begin with 267mg (one capsule or tablet) three times daily for one week; if tolerated, they move to 534mg (two capsules or tablets) three times daily and then, if that is tolerated, to 801mg (three capsules or tablets) three times daily. People can eventually switch to one 801mg tablet three times per day. Importantly, Esbriet must be taken with food.

Accessing and paying for it

Esbriet is obtainable by prescription only at a specialty pharmacy. Once required authorization has been approved, the pharmacy mails Esbriet to your home. People are advised to contact their insurance provider regarding payment, since coverage varies by insurer and each person’s policy.

 

There are programs that can help you pay for Esbriet. The manufacturer has set up Esbriet Access Solutions at 866-422-2377 and the Esbriet Co-Pay Program at 844-693-7274. Several other groups are Patient Services Inc. at 1-800-366-7741, the HealthWell Foundation at 1-800-675-8416, and the Patient Advocate Foundation at 1-800-532-5274. You can contact these programs directly to learn about the assistance they provide.

 

The fact sheet can be found at:

https://www.pulmonaryfibrosis.org/docs/default-source/disease-education-brochures/pf-fact-sheet-series—pirfenidone_digital.pdf?sfvrsn=df99918d_2

 

*Pulmonary Fibrosis Foundation. Pirfenidone (Esbriet®) (U.S. only) [Fact Sheet].