Idiopathic pulmonary fibrosis is a life-limiting, incurable condition. Recent guidance from NICE identifies priorities of care for this group of patients
Idiopathic pulmonary fibrosis is a progressive interstitial lung disease; patients have a mean life expectancy of 2-4 years from diagnosis. This review summarises National Institute for Health and Care Excellence (2013a) guidance and identifies key priorities for patient care.
Citation: Duck A (2014) Management of idiopathic pulmonary fibrosis. Nursing Times; 110, 16, 16-17.
Author: Annette Duck is independent interstitial lung disease specialist nurse, Manchester.
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Idiopathic pulmonary fibrosis (IPF) is a rapidly progressive interstitial lung disease (ILD), characterised by breathlessness and cough; patients have a mean life expectancy of 2-4 years from diagnosis (Wells and Dubois, 1994). There is no cure and, until July 2013, there were no licensed therapies to treat IPF. However, some options are now available and are discussed in a guideline from the National Institute for Health and Care Excellence (2013a).
Navaratnam et al (2008) estimated that 5,000 new cases of IPF are diagnosed annually and approximately 15,000 people have the condition in the UK at a given time. There are very few specialist centres in the UK and patients are often managed by GPs and community nurses.
The NICE (2013a) guideline sets out how patients with IPF should be supported. Given that treatment options are limited and disease progression is rapid, nurses are pivotal in the care of these patients. They have a role in providing information and support during assessment and diagnosis, throughout the treatment pathway, in symptom management and in end-of-life care.
There are over 150 ILDs, including IPF. Patients usually present with shortness of breath and/or cough, sometimes with other symptoms. For example, Raynaud’s disease is seen in ILD associated with connective tissue disorders, while lethargy and tiredness are often experienced in sarcoidosis. Some types of ILD, such as IPF, do not respond to treatment and affected patients will ultimately need palliative care (Duck, 2009).
In IPF, the lungs gradually become scarred, causing them to become smaller and stiffer, making it difficult to inhale. On a high-resolution CT scan, honeycomb scarring in the periphery of the lung can be seen. For this reason, IPF is often referred to as “honeycomb lung disease”.
Many patients describe a protracted time to diagnosis, and those with IPF are often misdiagnosed with asthma or COPD, causing frustration and loss of confidence in health professionals (Schoenheit, 2011). NICE advises clinicians to be aware of the clinical features of IPF when assessing a patient and refer for a chest X-ray or to a respiratory specialist if they have concerns. Box 1 outlines the key signs and symptoms.
Box 1. Signs and symptoms of IPF
- Persistent breathlessness on exertion
- Persistent cough
- Bilateral inspiratory crackles when listening to the chest with a stethoscope
- Clubbing of the fingers
- Normal or impaired spirometry usually with a restrictive but sometimes with an obstructive pattern
The condition occurs typically in people aged over 45 years
Diagnosis of IPF
As IPF is difficult to diagnose and can be confused with common interstitial pneumonias or ILDs, NICE (2013a) recommends that all patients suspected of having IPF are referred to an ILD specialist multidisciplinary team (MDT) for diagnosis. This team should consist of a:
- Consultant respiratory physician with expertise in ILD;
- Consultant radiologist with expertise in ILD;
- ILD specialist nurse;
- MDT coordinator.
A consultant histopathologist with expertise in ILD and a thoracic surgeon should be included in the MDT if patients have had a bronchoalveolar lavage or are likely to need an open lung biopsy to confirm diagnosis.
Information and support
ILD nurses are essential to the MDT as they provide patients with information about investigations, diagnosis and prognosis.
Most patients have never heard of IPF and are unlikely to be prepared for bad news. The five-year survival rate is worse than in all but two cancers - pancreas and lung (Vancheri et al, 2010). Supportive nurses are critical to helping patients adjust and develop coping strategies.
NICE (2013a) recommends offering pulmonary rehabilitation to patients throughout their disease pathway. This should be tailored as the educational needs of IPF patients are different from those with other lung diseases such as COPD.
Nurses are crucial in providing best supportive care, which should be tailored to disease severity and rate of progression, and patient preferences from diagnosis through to end-of-life care.
Since IPF generally affects older people, patients may have comorbidities, take a range of medication and have social issues that also affect quality of life.
Breathlessness on exertion is a characteristic of this disease. Regular assessment for exercise-induced hypoxia and prompt referral for ambulatory oxygen assessment enable patients to engage in normal activities inside and outside the home for as long as possible.
Treatment for cough varies, but it can be eased by mucolytic drugs (which help loosen and clear the mucus from the airways by breaking up the sputum), codeine linctus or oral morphine. Thalidomide improves cough (Horton et al, 2012) but can have unpleasant side-effects and should only be prescribed by specialist doctors.
Palliative care teams can advise on symptom management; referral should be considered for patients who are distressed by symptoms that are difficult to manage.
Monitoring disease progression and withdrawing ineffective therapies that could cause harm or further impair the patient’s quality of life should be considered.
Patients need information about possible treatments. There is no cure for IPF but pirfenidone (Esbriet) has been shown to slow disease progression (Noble et al, 2011). NICE (2013b) has set out criteria for its use in England. It can only be prescribed by registered prescribers and patients have to demonstrate a forced vital capacity (FVC) of 50-80% predicted for age and sex. FVC is the maximum amount of air a person can expel from the lungs after a maximum inhalation. The drug must be stopped if the FVC drops by more than 10% in a year, as this indicates the drug is not working. Patients need support during treatment to manage common side-effects, which can include nausea, loss of appetite, weight loss, lethargy and photosensitivity skin rashes.
There is limited evidence to support other pharmacological interventions for IPF so NICE (2013a) reviewed drugs traditionally used to treat the condition. The guideline group felt that, until more evidence became available, most drugs used to treat IPF should not be offered as some have substantial side-effects. Drugs include ambrisentan, azathioprine, bosentan, co-trimoxazole, mychophenolate mofetil, prednisolone, sildenafil and warfarin.
Some patients have already been prescribed a triple therapy (prednisolone, azathioprine and n-acetylcysteine) and, if they are stable, the patient and the clinical team should discuss the pros and cons of continuing the regimen. The Data Management Committee recently stopped the triple therapy arm in a research study (Panther study) due to an increased incidence in mortality in the triple therapy arm. The N-acetylycisteine and placebo arm continued and the results of this study should be available in May this year.
All physicians should discuss lung transplantation with IPF patients 3-6 months after diagnosis if they have no absolute contraindications (Orens et al, 2006); these include:
- Malignancy in the last two years, except squamous and basal cell tumours;
- Significant chest wall/spinal deformity;
- Untreatable psychiatric/psychological conditions that might affect adherence to follow-up and treatment regimens;
- Substance addiction, including alcohol, tobacco and narcotics, unless substance free for at least six months;
- Advanced dysfunction of major organs (heart, liver or kidney);
- Coronary heart disease not amenable to bypass grafting or percutaneous intervention, or associated left ventricular dysfunction;
- Infection with HIV, hepatitis C and/or hepatitis B;
- Absence of consistent social support.
Clinicians are advised to contact their local lung transplant centre for advice and should expect a response within four weeks.
Review and follow-up
Patients with rapid disease progression should be reviewed every three month, while those who are stable should be reviewed every six months. The review should include:
- Lung function;
- Oxygen assessment;
- Offer of pulmonary rehabilitation;
- Assessment of comorbidities;
- Smoking cessation advice;
- Identifying and assessing any hospital admissions and exacerbations;
- Assessment for palliative care referral.
NICE has gone some way in identifying the needs of patients with IPF and has evaluated the evidence to date to guide clinicians in their management of this fatal disease. There are many research recommendations including the benefit of oxygen, the value of supportive care, symptom control and end-of-life care.
- Idiopathic pulmonary fibrosis is a rapidly progressive interstitial lung disease
- There is no cure and patients have a prognosis of 2-4 years
- Five thousand new cases of IPF are diagnosed annually
- Patients with IPF are often misdiagnosed with asthma or COPD
- All patients suspected of having IPF should be referred to an ILD specialist multidisciplinary team
Duck A (2009) Principles to effectively manage people with interstitial lung disease in the community. Nursing Times; 105: 49-50.
Horton M et al (2012) Thalidomide for the treatment of cough in idiopathic pulmonary fibrosis: a randomised trial. Annals of Internal Medicine; 157: 398-406.
National Institute for Health and Care Excellence (2013a) Diagnosis and Management of Suspected Idiopathic Pulmonary Fibrosis.
National Institute for Health and Care Excellence (2013b) Pirfenidone for Treating Idiopathic Pulmonary Fibrosis.
Navaratnam V et al (2011) The rising incidence of idiopathic pulmonary fibrosis in the UK. Thorax; 66: 462-467.
Noble P et al (2011) Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. The Lancet; 377: 9779prac, 1760-1769.
Orens J et al (2006) International guidelines for the selection of lung transplant candidates 2006 update - a consensus report from the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation. Journal of Heart and Lung Transplantation; 25: 7, 745-755.
Schoenheit G et al (2011) Living with idiopathic pulmonary fibrosis: an in-depth qualitative survey of European patients. Chronic Respiratory Disease; 8: 4, 225-231.
Vancheri C et al (2010) Idiopathic pulmonary fibrosis: a disease with similarities and links to cancer biology. European Respiratory Journal; 35: 3, 496-504.
Wells A, DuBois R (1994) Prediction of disease progression in idiopathic pulmonary fibrosis. European Respiratory Journal; 7: 637-639.