Your doctor has talked to you about the use of pirfenidone. This leaflet provides you with information about the effects and use of this medication. It tells you what to do if you get side effects. This leaflet is not a substitute for the patient information in the medication package. If you have any questions after reading this leaflet, please ask your doctor and/or nurse specialist.
Why are you receiving pirfenidone?
Pirfenidone is a medicine used to treat adults with idiopathic pulmonary fibrosis (IPF). IPF is a chronic disease in which scar tissue is constantly being formed in the lungs, causing symptoms such as coughing and shortness of breath. 'Idiopathic' means the cause of the disease is unknown.
How does pirfenidone work?
The mechanism of action of pirfenidone is not precisely known, but it has been shown to reduce the production of cells and substances in the blood involved in the formation of scar tissue during the tissue repair process in the body. This slows the progression of the disease in IPF patients.
What is the dose and how do you take pirfenidone?
Pirfenidone is available in the form of capsules and tablets to be taken three times daily with meals. These are available in doses of 267 mg and 801 mg. The dose is gradually increased: starting with 267 mg three times daily in the first week, followed by 534 mg three times daily in the second week, and finally 801 mg three times daily from the third week and onwards.
Administration: The capsules and tablets should be swallowed as a whole with water. This is best done with food to reduce the chance of nausea and/or dizziness.
Pirfenidone is available by prescription only and treatment should be started by and take place under the supervision of a physician experienced in the diagnosis and treatment of IPF.
What are the side effects of pirfenidone?
It is impossible to predict in advance which side effects will occur in a person and to what extent. The most common side effects of pirfenidone are nausea, rash, fatigue, diarrhea, heartburn, loss of appetite, headache and photosensitivity reactions (sunburn-like reactions after exposure to sunlight).
It is important to prevent skin reactions due to sunlight. You can do this by using high factor sunscreen (factor 50), staying out of the sun and in the shade if possible, and wearing protective clothing.
When should you contact your treating physician?
- Persistent symptoms of gastrointestinal problems, such as nausea, diarrhea with or without loss of appetite.
- Skin reactions after exposure to sunlight or UV light.
If you notice the following (very rare) side effects listed below, you should notify your doctor immediately and stop taking the medication:
- If you experience swelling of the face, lips and/or tongue, difficulty breathing or wheezing, or feel weak. These may be signs of a severe allergic reaction.
- If your eyes or skin turn yellow or your urine turns dark, possibly accompanied by itching. These may be signs of abnormal liver function levels.
What controls are needed when using pirfenidone?
Sometimes the functioning of the liver or the production of blood cells is disturbed. For this reason, the dose of pirfenidone is gradually built up and your doctor will have your blood tested regularly. As a rule, this will be done every 4 weeks for 3 months after starting medication. With stable findings, subsequent blood checks once every 3-4 months will suffice.
When should you not use pirfenidone?
When you:
- Previously had an allergic reaction when using pirfenidone.
- Are known to have severe liver or kidney problems.
- Are pregnant.
Can you use pirfenidone with other medicines?
You can use pirfenidone together with almost all medicines. Always tell your treating specialist which medications you use and what you are using them for.
The effect of pirfenidone has been found to be less effective when using omeprazole, and your treating specialist may recommend replacing it with another antacid.
Pirfenidone should not be used in combination with fluvoxamine (a drug used to treat depression and obsessive-compulsive disorder).
What else do you need to know?
Be careful with alcohol. In combination with the use of pirfenidone, you are at higher risk of developing liver function disorders. Therefore, do not consume more than one alcoholic beverage per day.
Do you need a new prescription?
Pirfenidone should only be prescribed by a doctor experienced with this drug. You can ask for a repeat prescription from your treating specialist during the outpatient visit. Do you need a new prescription earlier? Then you can request it through ‘Mijn Dossier’via 'Medicijnen'. Click on Herhaalrecepten aanvragen'. Alternatively, you can request a repeat prescription by phone through the Pulmonary Diseases outpatient clinic. We ask that you do this at least 2 weeks before you need your new prescription. Otherwise, we cannot guarantee that your prescription will be sent to your pharmacy on time.
Do you have any questions?
If you have any further questions or if you are in doubt, please contact the Pulmonary Diseases outpatient clinic using the contact information known to you.