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NICE Guidance

Management of idiopathic pulmonary fibrosis

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Idiopathic pulmonary fibrosis is a life-limiting, incurable condition. Recent guidance from NICE identifies priorities of care for this group of patients

Abstract

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.

Introduction

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.

Clinical features

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.

Pulmonary rehabilitation

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.

Supportive care

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.

Pharmacological interventions

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.

Lung transplantation

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.

Conclusion

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.

Key points

  • 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
  • 4 Comments

Readers' comments (4)

  • My mother died from IPF recently, as a recently retired Community Matron I had perhaps high expectations on how my mother should be treated by Community Services in the last weeks of her life as her preferred place of death was at home. Unfortunately the District Nursing team that supported my mother had little understanding of IPF and without constant input from myself I feel that her last few days would of been unbearable and she may have ended up in hospital which is the place she did not want to be. I feel that if my mother had been diagnosed with lung cancer the end of life care she received would of been good , but dealing with a disease they did not understand made their decisions on her care poor. There is a need for better education , and advice re palliative care of patients with IPF as being given this diagnosis is worse than being told you have lung cancer.

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  • My mother passed away from IPF 3 weeks ago in the North-East. Her care for the last 2 years was coordinated by a community respiratory nurse with close input from her GP and for the last 15 months weekly attendance at the day hospice. Her care was fantastic and patient focused. She deteriorated rapidly and kept at home as her wishes for end of life care with Marie-Curie input. The value of a good relationship with a specialist nurse as part of a MDT with palliative care input can not be understated, the support had gave Mum (and my Dad who became a full-time carer) confidence, knowledge and as far as possible control over a very challenging and often distressing condition.

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  • This is a great overview of the disease. I have a close family member with IPF. It has been really sad to see the effects. The biggest issue has been the rapid weight loss. At first, it was just shortness of breath, but now the weight loss has gotten severe. There is another blog that has a lot of good info about pulmonary fibrosis written by an md. They also have a pretty good page on treatment options that was written recently and includes some of the newer treatments. Here's the link if anyone is interested: http://pulmonaryfibrosismd.com/idiopathic-pulmonary-fibrosis-treatment-options/

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  • Here is a better link:

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