The COVID-19 pandemic has brought to the fore an increased awareness of respiratory conditions. Unheard of until a few months ago, SARS-CoV-2, the cause of COVID-19, has become renowned and has prompted a concerted global response to combating the spread of this novel coronavirus, with unprecedented resources being allocated for research and the development of vaccines in an attempt to curb the pandemic. The long-term ill effects of having had a serious episode of COVID-19 are not yet known, but there are early indications that scarring of the lungs is one of the complications. The irony of scarring of the lungs, known as pulmonary fibrosis (PF), is that it is a poorly recognised and misunderstood respiratory condition with variable access to support and resources. This article is timely as a reminder to nurses of the condition and to help consider the specific care and support that this patient group needs to be offered.
The nurse's role is continuously evolving, with nurses becoming more involved in diagnosing, assessing, managing, referring, prescribing and treating patients with complex diseases. As a result, it is essential that nurses have a better understanding of less common respiratory diseases, such as IPF, to enhance the care they provide. IPF is the most common type of pulmonary fibrosis, which is one of more than 200 interstitial lung diseases (American Lung Association (ALA), 2020). IPF has received the most attention among the various interstitial lung diseases because of its uniquely unfortunate prognosis and unresponsiveness to traditional therapies (Wolters et al, 2014).
The British Lung Foundation (BLF) (2016), in The Battle for Breath: The Impact of Lung Disease in the UK, showed that around 32 500 people in the UK live with IPF, with a prevalence rate of about 50 per 100 000, with 6000 new cases diagnosed each year. Around 85% of diagnoses are made in people aged over 70 years and the condition is more common in men than women. Consequently, the disease is likely to increase in prevalence with an ageing population. Every year, there are nearly 9000 hospital admissions and about 5300 people die of the condition (BLF, 2016). Median survival in the UK is about 3 years (Strongman et al, 2018). It is notable that the prognosis and prevalence for IPF is worse than for many major cancers (Action for Pulmonary Fibrosis, 2019). Despite the startling figures, IPF remains inadequately resourced and is misunderstood by many nurses and other health professionals (Action for Pulmonary Fibrosis, 2019).
The condition has been described by many names in the medical literature (Box 1). This history of so many different medical terminologies to describe the disease is likely to contribute toward some of the confusion around it among nurses, other health professionals, patients, carers and the general public.
Box 1.Historical terms used for idiopathic pulmonary fibrosis before consensus was achieved on terminology after the millennium
- Desquamative pneumonia
- Chronic interstitial pneumonitis
- Cirrhosis cystica pulmonum
- Interstitial pulmonary fibrosis
- Lymphangitis reticularis pulmonum
- Idiopathic interstitial pulmonary fibrosis
- Acute diffuse interstitial fibrosis of the lungs
- Chronic interstitial fibrosis of lungs
- Hamman−Rich syndrome
- Diffuse interstitial pulmonary fibrosis
- Subacute diffuse interstitial fibrosis of the lungs
- Chronic diffuse interstitial fibrosis of lungs
- Interstitial nonpurulent pneumonia
- Acute diffuse interstitial fibrosis of lungs
- Fibrosing alveolitis
- Bronchiolar emphysema
- Diffuse fibrosing alveolitis
- Muscular cirrhosis
- Cryptogenic fibrosing alveolitis
- Chronic diffuse fibrosing alveolitis
- Interstitial pneumonia
- Usual interstitial pneumonia
- Organising interstitial pneumonia
- Idiopathic pulmonary fibrosis
- Diffuse interstitial pneumonitis
Source: Wells et al, 2018
The difference between IPF and PF
Pulmonary fibrosis is a form of interstitial lung disease that causes fibrosis (scarring) in the lungs. The most common type of PF is IPF (ALA, 2020). ‘Idiopathic’ is defined in the Cambridge Dictionary (2020) as ‘an idiopathic disease or medical condition [that] has no known cause’. Effectively, with PF it is possible to determine the cause of the disease, but with IPF this is not possible. There are many clinical similarities between PF and IPF, but this article will only use the term IPF.
Definition of IPF
Given the problematic history of identifying IPF due to the changes in medical terminology, it is important for nurses to gain a clear and concise understanding of the definition of the disease. The National Institute for Health and Care Excellence (NICE) (2017) describes the condition as follows:
‘IPF is a chronic, progressive fibrotic interstitial lung disease of unknown origin. It is a difficult disease to diagnose and often requires the collaborative expertise of a consultant respiratory physician, radiologist and histopathologist to reach a consensus diagnosis. Most people with IPF experience symptoms of breathlessness, which may initially be only on exertion. Cough, with or without sputum, is a common symptom. Over time, these symptoms are associated with a decline in lung function, reduced quality of life and ultimately death.’
An international collaborative comprising the American Thoracic Society, European Respiration Society, Japanese Respiratory Society and the Latin American Thoracic Society has also formulated a definition:
‘IPF is a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause. It occurs primarily in older adults, is limited to the lungs, and is defined by the histopathologic and/or radiologic pattern of UIP [usual interstitial pneumonia]. It should be considered in all adult patients with unexplained chronic exertional dyspnea, cough, bibasilar inspiratory crackles, and/or digital clubbing that occur without constitutional or other symptoms that suggest a multisystem disease
The symptoms listed in the above definition are similar to those found with many respiratory diseases and, hence, it is imperative that diagnosis is both timely and correct.
Pathophysiology of IPF
Historically, IPF was considered to be a chronic inflammatory disorder (Richeldi et al, 2017). However, anti-inflammatory therapy did not improve outcome and was shown to increase mortality (Raghu et al, 2012). The condition is now regarded to be a consequence of multiple interacting genetic and environmental risk factors, with repetitive local micro-injuries to ageing alveolar epithelium playing a central role (Richeldi et al, 2017), the consequence of which is irreversible fibrosis.
The appearance of the lungs in IPF shows a characteristic fibrosis distributed along the inferior portions of the lobes. On cross-section, these regions correspond to areas of airspace enlargement and fibrotic retraction (this pattern of fibrosis has been termed gross honeycombing) (Wolters et al, 2014) (Figure 1).
Figure 1. Pathophysiology of idiopathic pulmonary fibrosis: the alveoli become damaged and increasingly scarred
Causes and risk factors
It is now believed that the body creates fibrosis in response to damage in the lung (BLF, 2019). Although idiopathic means of unknown cause, there are still known risk factors for developing IPF (Table 1). The nurse's role frequently involves taking patient histories, so it is essential that nurses are aware of the risk factors for developing the disease.
Table 1. Risk factors and reasons that may lead to the development of idiopathic pulmonary fibrosis
| Risk factors | Reason this may cause idiopathic pulmonary fibrosis (IPF) |
|---|---|
| Gastro-oesophageal reflux (GOR) | Studies have shown that GOR is a risk factor for developing IPF, with one study reporting that as many as 87% of IPF patients have GOR (Raghu et al, 2006). PF can occur after aspiration of gastric contents (Tobin et al, 1998) |
| Obstructive sleep apnoea (OSA) | OSA is common in IPF, with some estimates of prevalence in one study reported as high as 88% (Lancaster et al, 2009). It has been suggested that IPF could be the consequence of large swings in pleural pressure caused by OSA, which may result in tractional microinjury to alveoli (Kim et al, 2017) |
| Viruses | Some studies have shown links between IPF and some viruses, such as the Epstein-Barr virus, cytomegalovirus and hepatitis C (Naik and Moore, 2010). It is possible that chronic inflammation due to infection in an individual with a genetic vulnerability to dysfunctional repair disturbs the normal healing response, causing progressive fibrosis (Naik and Moore, 2010) |
| Environmental factors | A meta-analysis carried out by Taskar and Coultas (2006) showed that working in agriculture/farming, with livestock, and environments with wood dust, metal dust and stone/sand were all risk factors for developing IPF. A constant exposure to particles, microbes, noxious fumes and pollutants creates a constant cycle of lung injury, wound healing and tissue repair (Sack and Rague, 2018). Individuals working in such environments inhale these irritants, putting them at greater risk of developing IPF |
| Family history/genetics | An individual's genetic make-up may also be a risk factor for IPF, but a link has been found only in a minority of cases, or around 10% in the UK (British Lung Foundation (BLF), 2019). Research is trying to determine which genetic variants contribute towards an individual being at risk of developing IPF |
| Gender | Prevalence rates suggest that IPF is 50% more common in men (BLF, 2016). This may be because men are more likely to be exposed to environmental factors, eg through their occupation (Zaman and Lee, 2018). Studies have shown that men with inhalational exposures are more likely to develop IPF than women (Ekström et al, 2014), leading to a hypothesis that sex hormones may contribute towards development of IPF (Zaman and Lee, 2018) |
| Lung microbiome | Micro-organisms are being investigated as possible risk factors for IPF, leading to a theory that IPF microbes might serve as a stimulus for microinjury to the lungs (Zaman and Lee, 2018) |
| Increase in age | The risk of developing IPF is likely to increase dramatically with age: 85% of diagnoses are in over-70s (Snell et al, 2016). Evidence has shown that individuals with a shortened telomere have a greater risk of developing IPF. A telomere is the specific DNA–protein structures found at the end of each chromosome protecting the genome (Shammas, 2011). A small portion of telomeric DNA is lost through every cell division, the telomere may serve as a biological clock to determine life span of a cell. Shortened telomeres are thought to signal a predisposing factor in developing fibrosis on exposure to irritants, perhaps by an impairment of tissue repair (Liu et al, 2013). Beyond abnormal telomere shortening that is seen in some IPF sufferers, the molecular basis underlying the vulnerability of the ageing lung leads to a dysregulated response to lung microinjury (Zaman and Lee, 2018) |
| History of smoking | Smoking is another risk factor for developing IPF. The pathophysiology by which smoking may contribute to IPF is unknown, but many studies suggest a strong association between developing IPF and cigarette smoking (Oh et al, 2012) |
| Diabetes mellitus | A study by Ehrlich et al (2010) indicated that patients with diabetes had a 50% greater risk for developing PF. Tissue fibrosis can be found in diabetic nephropathy and diabetic cardiomyopathy and, as a result, it has been hypothesised that there is a link between diabetes and pulmonary fibrosis (Hu et al, 2014) |
Signs and symptoms
Nurses and other health professionals also need to be aware of the signs and symptoms of the disease in order to help diagnoses or refer to a specialist for expert opinion. Common signs and symptoms are progressive dyspnoea especially on exertion, dry cough and crackles (similar to the sound heard when gently separating the strips of Velcro (Meltzer and Noble, 2008) on a blood pressure cuff or jogging shoes). Finger clubbing (Figure 2) is reported to be present in 50% of IPF sufferers (Meltzer and Noble, 2008). Sarkar et al (2012) highlighted that there are many hypotheses to explain the pathophysiology of digital clubbing, the most promising being that proposed by Dickinson and Martin in 1987. They hypothesised that megakaryocytes emerge from the bone marrow but are trapped by the pulmonary capillary bed, where they fragment into platelets. These large particles bypass the lung capillary network and may reach the fingertips via the peripheral vascular system where they release platelet-derived growth factor. This is known to cause increased capillary permeability and connective tissue hypertrophy, resulting in finger clubbing (Dickinson and Martin, 1987). This can easily be assessed when taking physical observations.
Figure 2. Finger clubbing versus normal finger shapes
Because many of these symptoms can be observed in other common respiratory diseases, such as chronic obstructive pulmonary disease (COPD) and asthma, individuals with IPF are often misdiagnosed (Bonella et al, 2016) or receive a diagnosis when it is in the later stages.
Diagnosis
Frequently, patients with IPF receive a diagnosis of heart failure or COPD (Hewson et al, 2018), suggesting that health professionals often fail to consider the possibility of IPF in patients with dyspnea. In some cases, patients presented with dyspnea and dry cough up to 5 years before IPF was diagnosed (Hewson et al, 2018). Some studies reported that up to 50% of IPF cases could be misdiagnosed as another disease (Collard et al, 2007). Because prognosis is poor, early and accurate diagnosis is essential and will influence care and treatment options (Aiello et al, 2017). However, the fact that diagnosis is often made late into the disease due to misdiagnosis or slow diagnosis—and consequent delayed referral to hospital—may limit the treatment options available (Lindell et al, 2016; Aiello et al, 2017; Action for Pulmonary Fibrosis, 2019).
NICE (2017:10) stresses the importance of being aware of the possibility of an IPF diagnosis when assessing a patient with the clinical features listed below, and when requesting a chest X-ray or referring to a specialist:
- Patient aged over 45 years
- Persistent breathlessness on exertion
- Bilateral inspiratory crackles when auscultating the chest
- Clubbing of the fingers
- Normal spirometry or impaired spirometry usually with a restrictive pattern but sometimes with an obstructive pattern.
The NICE (2017) guidelines highlight the clinical features most commonly found in individuals with IPF. It is essential that nurses and other health professionals consider these when assessing/diagnosing an individual presenting with a respiratory symptom.
Investigations to help confirm diagnosis
As nurses become more involved in requesting and performing investigations, it is critical that they understand the purpose of each test and how it facilitates diagnosis. After a patient's medical history and physical history have been taken, a range of tests can be carried out to help support diagnosis of IPF (Table 2). CT scans are seen as the gold standard in diagnosing IPF.
Table 2. Tests used to diagnose idiopathic pulmonary fibrosis
| Test | Results that may indicate idiopathic pulmonary fibrosis (IPF) |
|---|---|
| Medical history | Positive medical history, namely that the patient has some of the common risk factors for developing IPF |
| Physical examination | Finger clubbing and bilateral inspiratory crackles (that sound like the separation of Velcro when auscultating the chest |
| Chest X-ray See Figure 3 | IPF may be suspected when a chest X-ray shows a reticular pattern, predominantly basal, with or without cystic air spaces, especially if lung volumes are reduced (Aburto et al, 2018:4). However, 5–15% of patients with significant scarring will still have a normal chest X-ray (European Idiopathic Pulmonary Fibrosis and Related Disorders Federation, 2018). Consequently, a chest X-ray alone cannot be used to diagnose IPF alone |
| Pulmonary function test | Often shows a restrictive pattern due to reduced pulmonary compliance due to diffuse fibrosis (Heathcote et al, 2011) |
| Blood tests | To assess inflammatory makers and help in differential diagnosis |
| High-resolution computed tomography (HRCT) See Figure 4 | IPF is ‘defined by the histopathologic and/or radiologic pattern of UIP [usual interstitial pneumonia]’ (Raghu et al, 2018). ‘HRCT features in IPF patients is usual UIP include honeycombing, traction bronchiectasis, and traction bronchiolectasis’ (Raghu et al, 2018:48) |
| Lung biopsy | To gain a histology, used much less nowadays due to the advancement in radiological imaging |
Figure 3. Enhanced chest X-ray with an individual with idiopathic pulmonary fibrosis 
Figure 4. Example of a high-resolution computed tomography (HRCT) scan of an individual with idiopathic pulmonary fibrosis
Prognosis
The progressive nature of IPF is variable and can be punctuated by acute exacerbation (Hajari Case and Johnson, 2017). This can leave the individuals affected in a state of uncertainty and may explain the variability in their experience and level of support they receive. Once diagnosed, median survival for people with IPF in the UK is about 3 years (Strongman et al, 2018), but about 20% of people survive more than 5 years (NICE, 2015:5). The rate of disease progression can vary greatly between individuals, so it is difficult to provide a definitive prognosis at the time of diagnosis. It may only be possible to provide a long-term prognosis after a period of careful monitoring (NICE, 2017).
Supportive care is important in promoting the quality of life in this patient group (Duck et al, 2015). This can take the form of pulmonary rehabilitation (PR), treatment and access to a specialist interstitial lung diseases specialist nurse. NICE (2015:16), in statement 2 of the IPF in adults quality standard, highlights the many benefits of patient having access to a specialist nurse, who has the skills to support patients and provide information about investigations, diagnosis and management, as well as sensitively discuss prognosis, disease severity and progression, and life expectancy. The nurse is able to ensure that patients, families and carers receive the information and support they need throughout the care pathway.
Treatment
Due to poor prognosis, many nurses and health professionals may consider that treatments for IPF are limited, nonetheless, both non-pharmacology and pharmacology treatment options have proven beneficial to many IPF sufferers.
Medication
Drugs that slow the development of scar tissue in the lungs have been offered to those who have a forced vital capacity (FVC) of 50-80% predicted (NICE, 2016; 2018). Such drugs can be prescribed only by a prescribing centre, so patients receiving care from a general hospital may have to be referred to a specialist respiratory centre. The two anti-fibrotic medicines are nintedanib (NICE, 2016) and pirfenidone (NICE, 2018), which can be taken for as long as they have an effect. FVC will be monitored and the drugs stopped if they are no longer working, which will be an absolute decline of 10% or more in predicted FVC within any 12-month period (NICE, 2016; 2018).
These drugs can have adverse effects, for example nintedanib can cause gastrointestinal disturbances such as diarrhoea and elevation of liver enzymes, and blood tests should done to monitor liver function every 3 months. Caution should be taken with those who have a known risk of bleeding (Hajari Case and Johnson, 2017). Loperamide can be used to manage diarrhoea as required.
The most common side-effect of pirfenidone is photosensitivity (Barratt et al, 2018), so precautions should be taken all year around to avoid sun exposure. Patient education is important, and the patient should be advised to cover up and wear clothing with long sleeves, as well as gloves and a hat, and to apply high factor SPF and high UVA sunscreen, even on cloudy days indoors because windows may not protect from UVA rays (Costabel et al, 2014). Liver function tests should be taken prior to treatment, then once a month for 6 months, which should be repeated thereafter every 3 months. If a patient's liver function tests are abnormal, the dose of the pirfenidone may be reduced, or the treatment altered or discontinued (Joint Formulary Committee, 2020a).
Both nintedanib and pirfenidone are expensive, costing over £2000 a month each (Joint Formulary Committee, 2020a, 2020b). Patients should be advised to strictly concord with the prescribed medication regimen to ensure maximum absorption and efficacy.
N-acetylcysteine, a mucolytic, reduces the viscosity of sputum/phlegm, which in turn improves airway clearance and expiratory flow with cough (Tarrant et al, 2019), so this is a treatment option for some individuals.
Oxygen
Supplemental oxygen should be considered if the person has advanced IPF (Action for Pulmonary Fibrosis, 2019) or experiencing breathlessness on exertion, and based on clinical judgement (NICE, 2015: 19–22). Oxygen assessment will need to be undertaken to decide whether to prescribe oxygen and, if so, how much. It could be prescribed by the specialist centre, where the assessment is often undertaken by a health professional with respiratory expertise. Local protocols will inform the decision, as will clinical judgement and investigations such as arterial blood gas (ABG) tests (NICE, 2015:21). Oxygen will be prescribed to ameliorate hypoxaemia, reducing breathlessness and improving quality of life by enabling individuals to be more active and independent (Graney et al, 2017; Action for Pulmonary Fibrosis, 2019). Oxygen will be prescribed for use at home, but also in the form of ambulatory oxygen therapy that can be used during exercise and activities of living (NICE, 2015:22).
Education about the various delivery systems is an important consideration for patients because this will inevitably require the patient to make adjustments in their life (Graney et al, 2017). Duck et al (2015) highlighted how patients on ambulatory oxygen therapy can become anxious about whether the oxygen may run out while they are out of the home environment. As the disease deteriorates the patient may become more dependent on oxygen therapy and therefore will need to be assessed for the suitability of an oxygen concentrator, which will help relieve concerns about running out of oxygen. It is important to ensure that the patient frequently has follow-up appointments with the oxygen specialist nurse team to ensure optimal prescription and method of oxygen therapy. Advice and resources for travelling abroad are available from Action for Pulmonary Fibrosis (https://tinyurl.com/apf-travel).
Lung transplantation
If there are no comorbidities or absolute contraindicators or extreme severity of disease, then a referral to a transplant centre may be considered. Although there is a debate regarding bilateral versus single lung transplantation, so far this procedure has been the only restorative therapy to offer a proven survival benefit (Subramanian and Meyers, 2018). Many people with IPF develop the disease later in life, so they will often have comorbidities, which means that they are unlikely to be suitable candidates for lung transplantation.
Pulmonary rehabilitation
Although PR has been offered extensively to individuals living with COPD, this has not always been the case for those who have IPF. At present, only 53% of this patient group is referred to a PR programme (Taskforce for Lung Health, 2018) and only 14% attend a PR programme specifically run for IPF patients. This is despite the NICE (2017:7) clinical guideline highlighting that at the time of diagnosis the person should be assessed for their suitability for tailored PR. Many people living with IPF find that when they attend PR they are surrounded by people with COPD and therefore their needs become somewhat marginalised (NICE, 2015:23).
The National Institute for Health Research (2018) looked at whether PR improved outcomes in patients with IPF. It found that patients who had had tailored PR performed better in a 6-minute walk test than those who received usual care, walking 44 metres further; this led to improvements in quality of life, reduction in symptoms and improvement in patients' psychological wellbeing. According to a patient survey undertaken by Action for Pulmonary Fibrosis (2019), individuals who attended PR experienced multifaceted benefits: 54% reported improvement in symptoms, 62% felt more in control of their condition, 57% had a better quality of life, 51% said they felt less breathless and 44% felt more independent. The education offered in PR has also been shown to help patients develop greater understanding of their condition (NICE, 2015:24; Graney et al, 2017).
Specialist nurses
Access to a specialist nurse is fundamental to quality care, according to Action for Pulmonary Fibrosis (2019), whose patient survey found that 71% of individuals with IPF had access to a specialist nurse; this is very important because these nurses can provide continuity in the journey of care. There is evidence that patients who have access to such a resource are better informed about treatment options and feel more involved in discussions and decisions about their care (NICE, 2017; Action for Pulmonary Fibrosis, 2019) and it is a requirement of the service specification for having such a service.
Living with IPF
Living life as normally as possible is important. Because IPF is a chronic progressive disease, it is important to advise patients about the benefits of having the annual influenza vaccine. In addition, patients with a long-term condition may need just a single one-off pneumococcal vaccination or to have the vaccination every 5 years (NHS England, 2019). Exacerbations of IPF can lead to a further decline in symptoms. If the patient is still working, then liaison with occupational health may be required to identify what reasonable adjustments can be offered to support the person in the workplace. Adjusting to being breathless can be challenging and requires adaptation, particularly as the disease progresses (Duncan and Ashby, 2018).
The uncertainty of the disease can evoke a range of feelings and emotions in individuals. It may be beneficial for the specialist nurse to apply health psychology techniques and help the person to develop coping strategies by equipping them with knowledge that can be useful such as relaxation techniques, mindfulness and cognitive behavioural therapy (Duck et al, 2015; Barley and Lawson, 2016; Garcia et al, 2019).
Persistent breathlessness on minimal exertion that affects all activities of living will become more evident despite optimal treatment—this is sometimes referred to as refractory breathlessness and is associated with anxiety and heightened emotions (Duck et al, 2015; Speakman and Walthall, 2017; Duncan and Ashby, 2018). Measures such as prescribing benzodiazepines can be taken in relation to refractory breathlessness; these can reduce the sensation of breathlessness through the reducing anxiety, but they can also cause drowsiness (Speakman and Walthall, 2017). Therefore, the titration of low-dose opioids such as Oramorph may be suitable, but they need to be used with caution because this can depress the dyspnoea pathway (Speakman and Walthall, 2017).
Long-term humidified oxygen can be increased as required, and breathing exercises practised when the patient experiences acute breathlessness. Pharmacological interventions may also be considered for both anxiety and depression but, as Glaspole et al (2017) identified, only about one-quarter of patients receive this. A handheld fan aimed at the trigeminal area has been reported to be helpful because it seems to cool the oral and nasal receptors (Speakman and Walthall, 2017; Taskforce for Lung Health, 2018). Deconditioning and fatigue can impair quality of life and, although PR can initially facilitate improvements in quality of life, inevitably the long-term effects of breathlessness will take their toll and can lead to decreasing independence in activities of living. The presence of a cough can have a deleterious effect and can cause incontinence (Duck et al, 2015) and a lack of sleep (van Manen et al, 2016). The effects of cough treatments are variable (van Manen et al, 2016); it can involve some trial and error, but anecdotally codeine linctus can offer some relief.
Support groups
Social isolation is an issue that can affect this patient group (Ahmadi et al, 2014), and there are pulmonary fibrosis support groups for patients and their relatives/carers in many parts of the UK (Action for Pulmonary Fibrosis, 2019). These groups are invaluable in offering up-to-date information, peer support and a social network.
End-of-life care
Although treatment may slow down the disease process, ultimately patients with IPF have a poor prognosis. It is important that advance care planning and the concept of palliative care is introduced to the patient and their loved ones prior to reaching the end stage of the disease. Understandably, in the mild stages it may seem difficult for the patient to come to terms with this and, alongside an unintentional lack of recognition by health professionals, may cause a delay in preparing for end-of-life care (Janssen et al, 2020). At the end of life, symptom control is as important as it is for someone with cancer at the end of life and care should be tailored to the patient (Taskforce for Lung Health, 2018). Lindell et al (2017) identified four needs of patients and family caregivers with IPF at the end of life (Box 2).
Box 2.Palliative care need themes identified by Lindell et al (2017)
- Frustration with the diagnostic process and education received: lack of information about the prognosis progression and management of the disease
- Overwhelming symptom burden: physical and psychological burden for patient and family caregivers, including cough, breathlessness and anxiety
- Hesitance to engage in advanced care planning: perceived as a loss of hope and lacking understanding of what palliative care is
- Comfort in receiving care from pulmonary specialist centre because of resources: access to and availability of a specialist centre or support group
Recommendations
Both authors have had close family members sadly die of IPF and have witnessed first hand the inconsistencies in care. As a result, one author has set up a local charity support group for individuals affected by IPF/PF and witnessed the real benefits such a service can offer. This was the 67th group formed in the UK and establishing the group was challenging, with variable support from health professional teams. Among those who attend, repeated fears and frustrations include: a lack of knowledge and understanding of the condition, the perception that the condition is the same as more common chronic respiratory conditions, a delay in the diagnostic process, and delay in referral to a specialist respiratory centre. One of the authors recalls how one individual in the group was surprised to learn that they were not the only one living with the condition, which reassured the person and enabled them to form an effective social network, which was empowering.
Below is a list of recommendations that both authors consider will help enhance the care of individuals with IPF:
- Raise awareness of IPF through education of health professionals
- Facilitate early diagnosis through the recognition of signs and symptoms
- Promote referral to a specialist centre
- Promote the role of the specialist nurse
- Improve access to PR
- Promote local IPF support groups through Action for Pulmonary Fibrosis (www.actionpf.org)
- Promote awareness of the Pulmonary Fibrosis Trust (www.pulmonaryfibrosistrust.org) for practical, emotional and financial support
- Introduce the concept of palliative care at an optimal time.
Conclusion
The legacy of COVID-19 is, as yet, undetermined and will partly present through the health, wellbeing and survivorship of those who were badly affected by SARS-CoV-2. With an anticipated increase in prevalence of people living with scarred lungs, the needs of people with IPF will hopefully come to the fore and lead to increased investment in providing support, resources and research into the condition. Individuals and their families require care and support specific to them.
IPF remains, arguably, a Cinderella condition and, as such, it is underfunded and misunderstood, with the consequence that care is variable. The holistic impact on the patient and their loved ones is considerable and IPF has a significant impact on a patient's quality of life.
Although treatments and interventions can help slow disease progression and enhance patient wellbeing, there is no cure. Funding for research has been scarce, but in 2019, Action for Pulmonary Fibrosis funded two research projects: one looking at how bacteria interact with cells in the lungs and the effects of antibiotic treatments and the other focusing on identifying genes that cause disease progression and on disease subtypes.
It is the role of every nurse to have empathy for their patients. IPF is a distinctive respiratory disease, so it is essential that nurses are equipped with the knowledge, skills and understanding to deliver the high-quality care that patients deserve.
Key Points
- Currently there is no cure for idiopathic pulmonary fibrosis (IPF), a progressive interstitial lung disease, that impacts on a significant number of individuals and has a poor prognosis
- Diagnosis is often delayed or misdiagnosed, and this can influence the treatment options
- Evidence indicates access to a IPF specialist centres and specialist nurse improves quality of care
CPD reflective questions
- An individual may present with some unique risk factors and signs and symptoms of idiopathic pulmonary fibrosis (IPF). If you encountered a patient with such characteristics, might you suspect that the patient has IPF and consider referral?
- Consider how you would differentiate IPF from other chronic respiratory conditions
- you signpost a person living with IPF or they relative/carer to enable them to access support or information?