Pirfenidone - NORTH WEST INTERSTITIAL LUNG DISEASE …



NORTH WEST INTERSTITIAL LUNG DISEASE NETWORK

PATIENT INFORMATION REGARDING PIRFENIDONE TREATMENT

What is pirfenidone and what is Idiopathic Pulmonary Fibrosis (IPF)?

Pirfenidone is a drug that is used for some patients with idiopathic pulmonary fibrosis. Your specialist has diagnosed IPF in your case and will have given you either the British Lung Foundation leaflet or a local leaflet about this condition. You can access more information about IPF from the British Lung Foundation Website. .uk

The term Usual Interstitial Pneumonia or Usual Interstitial Pneumonitis (UIP) may also be used to describe your condition. However, pulmonary fibrosis (hardening or stiffening of the lung tissue) is very different to ordinary pneumonia which is an acute infection of the lung tissue. IPF is a long-term condition that usually progresses slowly over time. Pirfenidone is an anti-fibrotic agent. The aim of treatment with this drug is to reduce the rate of progression of lung fibrosis.

How well does pirfenidone work for patients with Idiopathic Pulmonary Fibrosis?

Unfortunately, no medication has yet been found which can improve lung function or symptoms for patients with IPF but pirfenidone and other new drugs act by slowing the disease process.

Details

Your lung capacity is measured by a test called Forced Vital Capacity (FVC). This test measures the total amount of air that you can blow quickly into a spirometer (lung function equipment). People with IPF who received placebo (dummy) treatment in clinical trials had an average fall in lung capacity of about 8% over one year whereas the decline averaged 5% amongst patients taking Pirfenidone.

In individual patients, a decline in FVC of more than 10% is considered to be clinically significant.

In one study of pirfenidone, 17.7% of people taking the placebo tablet had at least 10% reduction of FVC over a 12 month period compared with 6.5% of people taking pirfenidone.

Does it work for all IPF patients?

This is not yet known because experience with the drug is limited.

Details

Outcomes are better in clinical research trials than in unselected cases because people with significant additional conditions such as COPD, asthma, heart disease, liver disease or kidney disease and people aged over 80 and cigarette smokers and people with severe IPF were excluded from clinical trials. Therefore, we do not yet know if the drug has benefit for these groups of people (which may include you).

Does pirfenidone improve life expectancy?

There appears to be a small improvement in life expectancy on Pirfenidone treatment compared with no treatment in clinical trials involving highly selected patients.

Details

By combining the results of several studies, researchers have reported a mortality rate of 4.0% at one year on pirfenidone treatment (96% of patients alive at 12 months) compared with 7.2% mortality amongst patients taking the placebo tablets (93% alive at 12 months). The trials did not continue beyond one year. The mortality rate is lower in clinical trials compared with ordinary patients with IPF because people with severe IPF and people with significant additional medical problems are excluded from trials.

Will I feel better on pirfenidone treatment and will I be able to exercise more?

Patients who took pirfenidone in published trials did not report any significant improvement in breathlessness or in their quality of life compared with patients taking placebo (dummy tablets).

Details

The drug has a number of side effects, the most common of which are listed below. Not everyone will get these side effects but it is important that you are aware of them before you decide if you want to be considered for this treatment.

The distance that IPF patients could walk in six minutes at entry to one study of pirfenidone averaged about 420 metres (about 460 yards). The six minute exercise distance fell by about 33 metres (8% fall) during 12 months amongst patients taking pirfenidone and it fell by about 60 metres (14% fall) amongst patients taking placebo (dummy) tablets.

Where can I get the treatment from?

Pirfenidone treatment can be initiated at specialist centres following discussion of each case by a multi-disciplinary team of specialists (MDT). The MDT must agree that the patient meets the national criteria for pirfenidone treatment.

Details

It is recommended by the National Institute for health and Care Excellence (NICE) that IPF patients should have access to this therapy if their lung capacity is between 50 and 80% of the predicted value. Limited numbers of specialist centres around the country have been identified to ensure safe assessment and monitoring of patients that meet the criteria and chose to try it. If you meet the criteria and are interested in this medication and want to be discussed by the MDT and considered for referral to the Interstitial Lung Disease centre at Wythenshawe Hospital or Aintree University Hospital, we would be happy to arrange this. A visit to meet the team will provide the opportunity to discuss this treatment in more detail and you will be considered for funding of this drug if you meet the NICE criteria. Please do not hesitate to ask your specialist if you wish us to discuss referring you for this assessment.

Who should NOT take pirfenidone?

• Severe lung fibrosis with lung function (FVC) below 50% of the predicted value

• Mild lung fibrosis with lung function (FVC) above 80% of the predicted value

• Severe liver problems (CAUTION in mild to moderate liver impairment)

• Significant kidney problems

• Taking medication known to interact with this drug

• Pregnant or breast feeding

• Lung fibrosis that is not due to IPF

What are the side effects of pirfenidone?

Pirfenidone has a number of side effects as listed below. The majority of these side effects can be improved by simple measures such as how and when you take the drug, avoiding fatty meals and sometimes reducing the dose or adding in other medication or by the use of sun-block. This is something you will be able to discuss with your IPF specialist nurse and local hospital team.

Details

Common side effects (affecting more than one in ten people taking the medication): Fatigue, skin rashes (especially with exposure to the sun), feeling sick (nausea) diarrhoea, indigestion or heartburn

Rarer side effects: Headache, dizziness, difficulty sleeping (insomnia), drowsiness, severe itching (pruritus), hot flushes, weight loss and loss of appetite or vomiting, stomach discomfort, wind (flatulence), infections. (Your specialists can supply you with a fuller list if required):

Are there any warnings/precautions I should be aware of?

• Pirfenidone may cause dizziness and/or fatigue which may affect your physical or mental abilities; you must be cautious about doing tasks which need mental alertness such as operating machinery or driving a car.

• Avoid direct sunlight or sun lamps if you are taking this medication because between 12% and 51% of people in trials became sensitive to sunlight (photosensitivity). If unavoidable then wear sun screen above factor 50 or total block and a hat and protective clothing. Avoid using pirfenidone at the same time as other medications that cause sensitivity to the sun.

• Weight loss and loss of appetite (anorexia) have been reported with use; weight must be monitored throughout the period of drug administration.

• You should promptly report any new symptoms, reaction, or rash; the drug may be stopped or the dose reduced.

• The drug can cause liver problems.

How do I recognise the signs of liver disorder? (For patients taking pirfenidone)

Look out for non-specific symptoms such as bruising, itching, vague abdominal pain, loss of appetite, dark urine, weakness or fatigue. More specific symptoms related to liver disease are yellowing of the skin and eyes, itching and bruising easily. If you experience any of these symptoms seek prompt medical attention either with your GP where possible or Out of Hours service or at the hospital emergency department or contact your IPF nurse specialist (if available) for advice. If the symptoms are severe then attend the emergency department as a matter of urgency.

How will I take the drug if it is prescribed for me?

• Take the capsule with food as this may reduce the chance of feeling sick (nausea), vomiting, diarrhoea, indigestion and heart burn (dyspepsia).

• Swallow whole with plenty of water.

• Avoid grapefruit whilst taking this drug

What doses are recommended? (Your specialist will advise on the best dose for you)

Days 1-7: One capsule three times daily

Days 8-14: Two capsules three times daily

Day 15 and thereafter: Three capsules three times daily

Restarting therapy following interruption: If interruption less than14 consecutive days, may restart therapy at previous daily dose without gradually increasing; if interruption 14 days of more, restart therapy with the lowest dose for 2 weeks then gradually build up to recommended daily dose.

What monitoring will be needed if I am started on Pirfenidone treatment?

You will be assessed for the treatment in the first instance at the specialist centre. If you are suitable you will be able to decide if you are happy to try the treatment once you are clear about all the details of possible benefit and risks involved. You will visit the specialist centre regularly for blood tests when the treatment first starts. If there are no problems you will then return to the care of your local hospital and have regular monitoring and blood tests locally.

Pirfenidone treatment will be stopped if it is ineffective in your case (if your lung function deteriorates by more than 10% over a twelve month period). About one in 20 patients in clinical trials had a fall of 10% or more in Vital Capacity despite pirfenidone treatment.

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