Clinical update
Idiopathic pulmonary fibrosis Essential facts
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Idiopathic pulmonary fibrosis (IPF) is a disease that causes progressive scarring of the lungs, making it more and more difficult to breathe. It is the most common form of interstitial lung disease and can affect adults of all ages. IPF often kills with devastating speed: barely half of those diagnosed with the disease are still alive three years later and just one in five will survive five years. About 5,000 people are diagnosed with IPF in the UK each year and a similar number die. There is no cure.
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Order the free IPF information pack from the British Lung Foundation shop.blf.org.uk/products/ ipf-patient-pack
What’s new The British Lung Foundation has published the first comprehensive IPF information packs, available to nurses and other healthcare professionals to share with their patients. The packs are intended to help patients, their families and carers better understand IPF and guide them to further help and support.
Signs/symptoms The most common symptom is feeling short of breath, especially when moving about. It can happen during activities that would not usually tire a person, such as getting dressed. A cough that does not go away and feeling tired all the time are two other symptoms associated with IPF. Some patients also have a fever, lose weight, or experience muscle and joint pain. A distinctive sign of IPF is clubbing of fingers and toes, with nails feeling too soft, as if they are coming loose, the shape of nails changing, and the tips of fingers and toes starting to bulge. Clubbing can also be caused by other health conditions.
Expert comment Anne-Marie Russell is a fellow (nursing) at the National Heart and Lung Institute, London
Causes/risk factors The cause of IPF is not known. It is more common in men, people who smoke or have smoked in the past, and those whose work has exposed them to dust from wood, metal, textiles, stone, cattle or farming. Infection might be another cause. In some studies, IPF has been linked to viruses including the Epstein Barr virus, which causes glandular fever. The herpes virus and hepatitis C have also been suggested as possible causes. Some experts believe there is a link between IPF and gastric oesophageal reflux.
How you can help your patient When listening to the chest of a patient who has breathlessness, check the base of the lungs for the distinctive ‘crackling’ sounds that are a classic sign of IPF. Be aware that spirometry readings may look normal but this does not rule out IPF. Carry out a detailed history, including occupation. Refer patients to a specialist respiratory service if they could have IPF.
‘Making an IPF diagnosis can be difficult, as it is a relatively rare condition. It is often missed in the early stages as healthcare staff look for more common lung conditions, such as asthma and chronic obstructive pulmonary disease. ‘Once someone is diagnosed, services can be patchy and patients often feel isolated and abandoned, particularly
National Institute for Health and Care Excellence guidance on the diagnosis and management of suspected IPF (June 2013) www.nice.org.uk/ guidance/cg163 British Thoracic Society guideline on interstitial lung disease (September 2008) www.brit-thoracic.org.uk/ document-library/clinicalinformation/interstitiallung-disease/ild-guidelines/ bts-interstitial-lungdisease-guideline Article from Nursing Standard Palliative care for patients with chronic respiratory disease (2011) rcnpublishing.com/ doi/abs/10.7748/ ns2011.10.26.5.41.c8745
if they have to go into their local hospitals, where staff may not know much about IPF. ‘IPF is a serious condition and patients need good support. Those who have a community respiratory nurse and palliative care support at home report much better care than those who cannot access this service.’
NURSING STANDARD august 27 :: vol 28 no 52 :: 2014 19 Downloaded from RCNi.com by ${individualUser.displayName} on Nov 21, 2015. For personal use only. No other uses without permission. Copyright © 2015 RCNi Ltd. All rights reserved.
Idiopathic pulmonary fibrosis Essential facts
Find out more
Idiopathic pulmonary fibrosis (IPF) is a disease that causes progressive scarring of the lungs, making it more and more difficult to breathe. It is the most common form of interstitial lung disease and can affect adults of all ages. IPF often kills with devastating speed: barely half of those diagnosed with the disease are still alive three years later and just one in five will survive five years. About 5,000 people are diagnosed with IPF in the UK each year and a similar number die. There is no cure.
SCIENCE PHOTO LIBRARY
Order the free IPF information pack from the British Lung Foundation shop.blf.org.uk/products/ ipf-patient-pack
What’s new The British Lung Foundation has published the first comprehensive IPF information packs, available to nurses and other healthcare professionals to share with their patients. The packs are intended to help patients, their families and carers better understand IPF and guide them to further help and support.
Signs/symptoms The most common symptom is feeling short of breath, especially when moving about. It can happen during activities that would not usually tire a person, such as getting dressed. A cough that does not go away and feeling tired all the time are two other symptoms associated with IPF. Some patients also have a fever, lose weight, or experience muscle and joint pain. A distinctive sign of IPF is clubbing of fingers and toes, with nails feeling too soft, as if they are coming loose, the shape of nails changing, and the tips of fingers and toes starting to bulge. Clubbing can also be caused by other health conditions.
Expert comment Anne-Marie Russell is a fellow (nursing) at the National Heart and Lung Institute, London
Causes/risk factors The cause of IPF is not known. It is more common in men, people who smoke or have smoked in the past, and those whose work has exposed them to dust from wood, metal, textiles, stone, cattle or farming. Infection might be another cause. In some studies, IPF has been linked to viruses including the Epstein Barr virus, which causes glandular fever. The herpes virus and hepatitis C have also been suggested as possible causes. Some experts believe there is a link between IPF and gastric oesophageal reflux.
How you can help your patient When listening to the chest of a patient who has breathlessness, check the base of the lungs for the distinctive ‘crackling’ sounds that are a classic sign of IPF. Be aware that spirometry readings may look normal but this does not rule out IPF. Carry out a detailed history, including occupation. Refer patients to a specialist respiratory service if they could have IPF.
‘Making an IPF diagnosis can be difficult, as it is a relatively rare condition. It is often missed in the early stages as healthcare staff look for more common lung conditions, such as asthma and chronic obstructive pulmonary disease. ‘Once someone is diagnosed, services can be patchy and patients often feel isolated and abandoned, particularly
National Institute for Health and Care Excellence guidance on the diagnosis and management of suspected IPF (June 2013) www.nice.org.uk/ guidance/cg163 British Thoracic Society guideline on interstitial lung disease (September 2008) www.brit-thoracic.org.uk/ document-library/clinicalinformation/interstitiallung-disease/ild-guidelines/ bts-interstitial-lungdisease-guideline Article from Nursing Standard Palliative care for patients with chronic respiratory disease (2011) rcnpublishing.com/ doi/abs/10.7748/ ns2011.10.26.5.41.c8745
if they have to go into their local hospitals, where staff may not know much about IPF. ‘IPF is a serious condition and patients need good support. Those who have a community respiratory nurse and palliative care support at home report much better care than those who cannot access this service.’
NURSING STANDARD august 27 :: vol 28 no 52 :: 2014 19 Downloaded from RCNi.com by ${individualUser.displayName} on Nov 21, 2015. For personal use only. No other uses without permission. Copyright © 2015 RCNi Ltd. All rights reserved.
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