What treatments are available?
There is no cure for IPF. Drug treatment is available that may help to slow down the rate of progression of the lung fibrosis.
Nintedanib is a new anti-fibrotic drug that slows down the progression of idiopathic pulmonary fibrosis. In January 2016 the National Institute for Health and Care Excellence (NICE) approved nintedanib for the treatment of certain patients with IPF in England and Wales. NICE stipulated that Nintedanib could be used for people with IPF whose FVC is 50% – 80% or less of predicted. In Scotland, the SMC approved this treatment for people with IPF whose FVC is 80% or less of predicted.
Pirfendone was the first licenced treatment for IPF. In clinical trials, it has been shown to reduce the rate of decline in lung function in people with IPF. It was approved by NICE in 2013 for people with IPF whose FVC is between 50% and 80% of predicted. In Scotland, the SMC approved this treatment for people with IPF whose FVC is 50% or more of predicted.
Whilst nintedanib and pirfenidone have never been compared head-to-head, these drugs seem to have similar beneficial effects, namely slowing progression of disease by about 50% on average over a year or more. It is hoped that this will translate into people living longer. There are some differences in the side effect profiles of nintedanib and pirfenidone.
N-acetylcysteine is an antioxidant therapy that was thought to be helpful for some people with IPF. However, clinical trials have not shown any effect of NAC on lung function other outcomes in people with IPF.
Acid reflux therapy
Gastro-oesophageal or acid reflux is common in people with IPF. There are a number of medical and surgical treatments available to control acid reflux.
Over the last 10 years there has been an enormous growth in research into understanding the cause and mechanisms that are responsible for IPF.
This is a very exciting time as it has identified new molecules that may be involved in the disease process and could lead to new drug therapies in the future. As a result of this research, there has been an increase in the number of clinical trials of new treatments in IPF.
In order to determine if a drug is going to be effective, it has to be compared with a dummy pill (placebo). The trial has to be conducted in a double blind manner to ensure that the measured outcomes are not biased. This means that neither the person taking part nor their doctor or nurse know what treatment is being given.
People taking part in clinical trials are closely monitored for any side effects.
In the UK, there are a number of centres which take part in clinical trials for IPF. If you would be interested in taking part in a clinical trial, please discuss this with your specialist who will be able to advise.
Further details about current IPF clinical trials can be found on the website clinicaltrials.gov
If your oxygen levels are low, your specialist may discuss oxygen therapy. If your oxygen levels are normal, you will not need oxygen therapy. Ambulatory oxygen is given to people whose oxygen levels are low during or after exercise.
Long-term oxygen therapy (LTOT)
This is given to people whose oxygen levels are low at rest and during exercise.
Pulmonary rehabilitation was initially set up for people with chronic obstructive pulmonary disease (COPD), but many patients with lung fibrosis benefit from this program and it is recommended by NICE.
It typically consists of a six week course which provides advice on:
- How to manage breathlessness
- Exercises to improve muscle strength and fitness
- Self-management training
- Psychosocial support
Lung transplantation is an option for a small number of people with IPF, typically people aged 65 or under who are otherwise in good physical health. It is a decision that is not to be taken lightly and needs careful consideration and assessment.
Management of breathlessness
Breathlessness can be frightening and there are many ways to help relieve this. Certain medicines can help relieve some of the discomfort of breathlessness. These include benzodiazepines, such as lorazepam, which help to reduce the anxiety associated with breathlessness. Small doses of morphine, such as Oramorph, can also be helpful for managing breathlessness.
You may be referred to the supportive and palliative care doctors and nurses for their expertise in helping with your symptoms of breathlessness.