Introduction

This quality standard covers the diagnosis and management of idiopathic pulmonary fibrosis in adults, from the initial suspicion of the disease to referral, supportive care and treatment. For more information see the topic overview.

Why this quality standard is needed

Idiopathic pulmonary fibrosis is a serious and progressive disease in which the alveoli (the tiny air sacs of the lungs) and the lung tissue next to the alveoli become damaged and scarred. The cause of the disease is unknown. Although it is associated with characteristic clinical, radiological and histological features, it is difficult to diagnose. Confirmation of diagnosis often requires the collaborative expertise of a consultant respiratory physician, a thoracic radiologist and a thoracic histopathologist. Most people with idiopathic pulmonary fibrosis have symptoms of breathlessness, which may at first occur only on exertion, and cough, with or without sputum. Over time, these symptoms are associated with a decline in lung function, reduced quality of life and ultimately death.

Idiopathic pulmonary fibrosis is rare in people younger than 45 years and in the UK the median age of presentation is 70 years. The prevalence is around 15 to 25 cases per 100,000 people and increases with age. Most people with idiopathic pulmonary fibrosis smoke or have a history of smoking, and idiopathic pulmonary fibrosis often coexists with chronic obstructive pulmonary disease.

The median survival for people with idiopathic pulmonary fibrosis in the UK is approximately 3 years from the time of diagnosis. However, about 20% of people with the disease survive for more than 5 years. The rate of disease progression varies. A person's prognosis is difficult to estimate at the time of diagnosis and may only become apparent after a period of careful follow‑up.

This quality standard is expected to contribute to improvements in the following outcomes:

  • quality of life of people with idiopathic pulmonary fibrosis

  • premature mortality

  • experience of care

  • emergency admissions or attendances at emergency departments

How this quality standard supports delivery of outcome frameworks

NICE quality standards are a concise set of prioritised statements designed to drive measurable quality improvements within a particular area of health or care. They are derived from high‑quality guidance, such as that from NICE or other sources accredited by NICE. This quality standard, in conjunction with the guidance on which it is based, should contribute to the improvements outlined in the following 3 outcomes frameworks published by the Department of Health:

Tables 1–3 show the outcomes, overarching indicators and improvement areas from the frameworks that the quality standard could contribute to achieving.

Table 1 The Adult Social Care Outcomes Framework 2014–15

Domain

Overarching and outcome measures

1 Enhancing quality of life for people with care and support needs

Overarching measure

1A Social care‑related quality of life*

Outcome measures

People manage their own support as much as they wish, so that are in control of what, how and when support is delivered to match their needs.

1B Proportion of people who use services who have control over their daily life

Carers can balance their caring roles and maintain their desired quality of life.

1D Carer‑reported quality of life*

3 Ensuring that people have a positive experience of care and support

Overarching measure

People who use social care and their carers are satisfied with their experience of care and support services.

3A Overall satisfaction of people who use services with their care and support

3B Overall satisfaction of carers with social services

Aligning across the health and care system

* Indicator complementary with the NHS Outcomes Framework

Table 2 NHS Outcomes Framework 2014–15

Domain

Overarching indicators and improvement areas

1 Preventing people from dying prematurely

Overarching indicator

1a Potential Years of Life Lost (PYLL) from causes considered amenable to healthcare

i Adults

1b Life expectancy at 75

i Males ii Females

Improvement area

1.2 Under 75 mortality rate from respiratory disease*

2 Enhancing quality of life for people with long‑term conditions

Overarching indicator

2 Health‑related quality of life for people with long‑term conditions**

Improvement areas

Ensuring people feel supported to manage their condition

2.1 Proportion of people feeling supported to manage their condition

Reducing time spent in hospital by people with long‑term conditions

2.3 i Unplanned hospitalisation for chronic ambulatory care sensitive conditions

Enhancing quality of life for carers

2.4 Health‑related quality of life for carers**

4 Ensuring that people have a positive experience of care

Overarching indicator

4a Patient experience of primary care

i GP services

ii GP out‑of‑hours services

4b Patient experience of hospital care

Improvement areas

Improving hospitals' responsiveness to personal needs

4.2 Responsiveness to in‑patients' personal needs

Improving people's experience of accident and emergency services

4.3 Patient experience of A&E services

Alignment across the health and social care system

* Indicator shared with Public Health Outcomes Framework (PHOF)

** Indicator complementary with Adult Social Care Outcomes Framework (ASCOF)

Table 3 Public health outcomes framework for England, 2013–2016

Domain

Objectives and indicators

4 Healthcare public health and preventing premature mortality

Objective

Reduced numbers of people living with preventable ill‑health and people dying prematurely, while reducing the gap between communities

Indicators

4.7 Under 75 mortality rate from respiratory diseases*

Aligning across the health and care system

* Indicator shared with the NHS Outcomes Framework

Patient experience and safety issues

Ensuring that care is safe and that people have a positive experience of care is vital in a high‑quality service. It is important to consider these factors when planning and delivering services relevant to idiopathic pulmonary fibrosis.

NICE has developed guidance and an associated quality standard on patient experience in adult NHS services (see the NICE pathway on patient experience in adult NHS services), which should be considered alongside this quality standard. They specify that people receiving care should be treated with dignity, have opportunities to discuss their preferences, and be supported to understand their options and make fully informed decisions. They also cover the provision of information to patients. Quality statements on these aspects of patient experience are not usually included in topic‑specific quality standards. However, recommendations in the development source(s) for quality standards that impact on patient experience and are specific to the topic are considered during quality statement development.

Coordinated services

Between 2000 and 4000 new cases of interstitial lung disease are diagnosed in England each year, and most of these are either sarcoidosis or idiopathic pulmonary fibrosis. Care for people with idiopathic pulmonary fibrosis is subject to specialised commissioning arrangements through NHS England and its area teams. In 2014 there were 11 hospitals providing specialised care. Disease‑specific management plans are drawn up after multidisciplinary team assessment at regional specialist centres.

The quality standard for idiopathic pulmonary fibrosis specifies that services should be commissioned from and coordinated across all relevant agencies encompassing the whole idiopathic pulmonary fibrosis care pathway. A person‑centred, integrated approach to providing services is fundamental to delivering high‑quality care to adults with idiopathic pulmonary fibrosis.

The Health and Social Care Act 2012 sets out a clear expectation that the care system should consider NICE quality standards in planning and delivering services, as part of a general duty to secure continuous improvement in quality. Commissioners and providers of health and social care should refer to the library of NICE quality standards when designing high‑quality services. Other quality standards that should also be considered when choosing, commissioning or providing a high‑quality idiopathic pulmonary fibrosis service are listed in related quality standards.

Training and competencies

The quality standard should be read in the context of national and local guidelines on training and competencies. All healthcare professionals involved in assessing, caring for and treating adults with idiopathic pulmonary fibrosis should have sufficient and appropriate training and competencies to deliver the actions and interventions described in the quality standard. Quality statements on staff training and competency are not usually included in quality standards. However, recommendations in the development source(s) on specific types of training for the topic that exceed standard professional training are considered during quality statement development.

Role of families and carers

Quality standards recognise the important role families and carers have in supporting people with idiopathic pulmonary fibrosis. If appropriate, healthcare professionals should ensure that family members and carers are involved in the decision‑making process about investigations, treatment and care.