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Best practice: End-stage non-malignant lung disease guidelines

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VOL: 103, ISSUE: 24, PAGE NO: 39

Denise Williams, MSc, BA, RGN, is respiratory nurse consultant, Wirral Hospitals NHS Trust; Linda Johns, BSc, RGN, is respiratory nurse specialist, Cardiothoracic Centre, Liverpool.

Williams, D. (2007) Guidelines for patients with end-stage non-malignant lung disease. Nursing Times; 103: 24, 39-41. Denise Williams and Linda Johns discuss palliative care provision for patients with end-stage non-malignant lung disease.

Williams, D. (2007) Guidelines for patients with end-stage non-malignant lung disease. Nursing Times; 103: 24, 39-41. Denise Williams and Linda Johns discuss palliative care provision for patients with end-stage non-malignant lung disease.

Palliative care is concerned with improving the quality of life for patients with life-threatening illnesses and that of their families. The current configuration of palliative care services was developed to meet the needs of people dying from cancer. Unfortunately similar services have not been developed for patients who are dying from end-stage lung disease (Addington-Hall and Higginson, 2003).

However, there is considerable evidence to demonstrate that these patients suffer symptoms, psychological distress and family anxiety similar to that experienced by patients with cancer (Blackler et al, 2004; Simonds, 2004).

Respiratory disease accounts for 20% of all deaths in the UK (British Thoracic Society, 2006) and a survey of 2,500 patients with COPD conducted by the British Lung Foundation (2000) identified that four out of five patients had substantial difficulty with everyday tasks.

Reduced functional ability can often result in social isolation and anxiety; depression and panic disorders are also common in patients with chronic lung disease. Gore et al (2000) identified that 90% of patients with severe COPD were suffering with clinically relevant depression and anxiety, compared with 52% of patients with advanced cancer, and only small numbers of patients in each group had been assessed and treated.

Brenes (2003) found that anxiety and panic disorders occur at a higher rate in patients with COPD than in the general population and Almagro et al (2002) identified a strong relationship between the presence of depression and 1-3-year mortality. In addition to breathlessness other symptoms include pain, fatigue, difficulty in sleeping and thirst, all of which are poorly controlled in COPD (Skilbeck et al, 1997).

As the disease progresses patients experience deteriorating health status and function and have more frequent hospital admissions. Recent UK figures show that of those admitted with COPD 14% had died and 34% were readmitted within three months (Roberts et al, 2002). Patients with impaired gas exchange and severe hypoxaemia (PaO2<8kpa) have="" the="" worst="" prognosis.="" if="" untreated="" their="" chances="" of="" surviving="" for="" five="" years="" is="" less="" than="" 50%="" (national="" collaborating="" centre="" for="" chronic="" conditions,="" 2004).="">

Other chronic respiratory conditions have symptoms and problems similar to COPD, for example interstitial lung disease.

Potential barriers to provision of palliative care

The Department of Health (1998) has accepted the need for access to palliative care on the basis of need and not diagnosis. Unfortunately while recommendations for palliative care interventions are acknowledged in the current UK COPD guidelines (National Collaborating Centre for Chronic Conditions, 2004) they highlight the limited data available and call for further research. Similarly and disappointingly there is no direct reference to palliative care in the recently revised Global Initiative for Chronic Obstructive Lung Disease (2006) guidelines. .

Predicting the prognosis associated with COPD can be difficult and barriers to discussions about end-of-life care between patients, healthcare staff and physicians can occur. Hansen-Flaschen (2004) refers to the variable rate of progress of COPD and co-existing factors, such as cardiovascular disease, that can influence patients' survival. Patients with advanced COPD may also experience severe illness followed by significant recovery lasting several years, before a fairly rapid final event (Shee, 2003).

  • Research has identified various factors considered relevant in the relationship between COPD and survival or death. These have included:
  • Decline in lung function (Anthonisen et al, 1986);
  • Dyspnoea scores (Nichimura et al, 2002);
  • Quality of life (Domingo-Salvany et al, 2002);
  • Body mass index (Chailleux and Laaban, 2003);
  • Exacerbations (Connors et al, 1996);
  • Admissions to hospital (Roberts et al, 2002).

Further research is needed to identify which of the above factors are the most significant in indicating a poor prognosis. However, health professionals can use changes in these factors to trigger conversations with patients about their thoughts for the future.

In our society, barriers to communication about death still exist. Some of the awkwardness is linked to the way society views death and affects both the patient and the health professional. This is confirmed by various studies that have identified health professionals as being poor at communication (Elkington et al, 2001). The consequences are that many of the conversations regarding end-of-life issues are thrust upon patients or their families during an acute, life-threatening exacerbation when they may be too ill or emotional to participate fully in decision-making.

Research has shown patients' preferences are not predictable and do not necessarily correlate with severity of illness or other measured variables (Gaber et al, 2004), which are the parameters often used by health professionals in emergency situations to determine the appropriateness of treatment. Patients with chronic lung conditions are also more likely than those with lung cancer to die receiving aggressive therapy that may go against their preference for comfort rather than life-prolonging treatment (Connors et al, 1996).

Many patients want to communicate about their future believing that a good death involves communication, clear decision-making and being involved in treatment decisions, which can empower them and reduce feelings of fear (Steinhauser et al, 2000). However, not all patients wish to discuss end-of-life care options (Knauft et al, 2005). This may be overcome if health professionals take the lead by acknowledging that although discussions around dying are difficult, they are important. It is essential that health professionals have time to facilitate discussion about end-of-life treatment and care.

Impetus for developing guidelines

The social and economic burden of chronic respiratory disease has led to a proliferation of research and initiatives directed at reducing the impact of acute exacerbations on emergency services. Consequently admission avoidance and early supported discharge schemes - for COPD, in particular - are now common in the UK.

Treatment options in stable disease are increasingly evidence based but there is still little or no research as to the most effective way to provide palliative care to those with the most severe disease (Seamark et al, 2004). Awareness of the need to incorporate the principles of palliative care into treatment approaches for those patients with terminal non-malignant illness is supported by national initiatives (Gold Standards Framework, 2006; DH, 2000). However, extending specialist palliative care services to include those with a non-cancer diagnosis has potential resource implications, particularly as cancer charities are a crucial source of current funding (Dharmasena and Forbes, 2001).

The role of hospital respiratory teams in palliative care has not been formally researched. Some patients have expressed concern about the purpose of secondary care specialists in stable disease management and, in particular, on the quality of communication and the potential for lack of continuity of care in the event of worsening disease (Seamark et al, 2004). Respiratory nurse specialists are a potential source of support for these patients as they may have known them for many years, although this input depends on local arrangements.

In reality most of the responsibility for providing non-specialist palliative care services in the UK lies with primary healthcare teams (Exley et al, 2005). As with specialist palliative care services, this has not formally included those with end-stage non-malignant respiratory disease.

Potential barriers to provision of palliative care in end-stage respiratory disease (Box 1) are likely to be further compounded by lack of strategy and coordination of services for this group of patients. Directives such as The Cancer Plan (DH, 2000) mean that the journey through health services for patients with a diagnosis of cancer is now structured and they are well supported. This usually ensures that important trigger factors for appropriate community support, even just the diagnosis itself, are more likely to take place and communication between primary and secondary care is enhanced. Similar arrangements rarely exist for non-cancer patients.

The guidelines

The shift in emphasis in chronic disease management, including the development of community matrons to provide support through case management, may bridge the gap and lead to a more coordinated approach between primary and secondary care for the most complex patients. Further empowering district nurses through the provision of additional training in palliative care may also extend this approach beyond the traditional cancer diagnosis (DH, 2003, 2000).

Supporting the generalist to extend provision of palliative care to those with a non-cancer diagnosis involves raising awareness, dispelling myths and sharing knowledge of appropriate management strategies, particularly about the most common and distressing symptoms. Recognition of this provided the impetus for the Cheshire and Merseyside respiratory and cancer clinical networks to form a working party with the aims of:

  • Developing symptom control guidelines for the palliative care management of end-stage lung disease (Box 2);
  • Suggesting criteria for the referral of patients to specialist palliative care services;
  • Providing a service directory of specialist respiratory and palliative care resources that are available to support patient care. The directory of resources aims to promote the importance of collaborative working between different specialities and primary and secondary care.

Symptom control and referral

Treatment options for symptom control include both pharmacological and non-pharmacological approaches based on available evidence and best practice guidelines from respiratory and palliative care. This holistic and multidisciplinary approach is particularly useful when applied to the management of breathlessness. Breathlessness is a notoriously complex symptom to control and is often compounded by a fear among health professionals that the use of opiates and anxiolytics will depress respiratory drive and hasten death. The guidelines support and encourage the inclusion of these treatments, in addition to those that are disease-specific, when it is considered necessary in order to control symptoms.

Referral for specialist advice from palliative teams would normally be suggested if more complex support is needed. Criteria have been refined after considerable debate and based on expert clinical opinion as there is no definitive evidence on which to base this (Box 2). These criteria also reflect the importance of ensuring that the underlying diagnosis and treatment are optimal.

A fundamental principle of the guideline is that good palliative care cannot be achieved without open and sensitive communication. Without this it is difficult - if not impossible - to achieve good symptom control and promote patient choice. This is also reflected in the criteria for specialist palliative care referral, as the responsibility for initiating discussions on sensitive issues should not be transferred from the referer to palliative care teams.

Case history:

Edna Miller was a 72-year-old woman diagnosed with extensive pulmonary fibrosis who had received regular follow up with a respiratory physician for two years. She was on maximal medication including oxygen. Mrs Miller was determined to be independent and was supported by her husband and daughter who declined social services input. Contact with the community nursing and therapy services was limited to occasional provision of equipment.

The hospital-based respiratory nurse had seen her previously on several occasions, essentially to manage her oxygen needs. Mrs Miller was admitted to hospital due to panic associated with an increase in breathlessness. She had been seen at home prior to admission by two out-of-hours GPs who acknowledged that panic was probably the main problem. They suggested that anxiolytics might help but felt unable to prescribe them as they might depress her respiratory function.

During hospital admission there were no acute findings, Mrs Miller's oxygen needs were reviewed and she was commenced on 0.5mg lorazepam every eight hours, which considerably reduced the panic attacks.

The respiratory nurse visited her at home after two weeks and noted a significant general deterioration. During the visit the nurse initiated discussion with Mrs Miller and her family about her deteriorating condition and symptom control. She expressed the wish to remain at home in the event of further deterioration, which was supported by her family.

The GP prescribed oral morphine for relief of breathlessness and the district nursing team was contacted to provide palliative support. Several days later the respiratory nurse contacted Mrs Miller who had deteriorated to the point where she could barely manage sips of fluid and was increasingly drowsy. The GP and district nurses were contacted to prescribe medication via syringe driver and support the patient and family with end of life care. Mrs Miller died at home peacefully with her family at her bedside later that day.

Although it appears that this case was handled well, several issues arise from it:

  • The admission may have been avoided if the guidelines had been available at the time to help the GP to confidently manage Mrs Miller's panic.
  • The district nursing team expressed concern that they were asked to provide support in the last few hours of Mrs Miller's life but hardly knew her. In this case there was no automatic trigger point for referral to the district nursing service. During an earlier referral she had been assessed as having no immediate nursing need so contact was limited.
  • The hospital-based respiratory nurse provided the co-ordination for most of the end-of-life care, however this would not happen in all cases. This raises the issue of who should co-ordinate care in these instances.


There is much that the generalist can do to support the patient in the late stages of non-malignant respiratory disease, however, this requires increased awareness and proactive management. The treatment options often referred to as palliative should not be viewed as separate, but rather as part of a continuum of treatment that is adapted to meet the individual patient's needs.

Box 2. Criteria for referral to specialist palliative care:

  • The patient has a diagnosis confirmed by a specialist respiratory physician
  • There are uncontrolled physical or psychological symptoms despite optimal therapy (including pulmonary rehabilitation if appropriate)
  • Patient understands diagnosis and agrees to referral to a specialist palliative care team
  • Anticipated life expectancy is 12 months or less
  • Patient is making increased use of emergency treatment for infection and/or respiratory failure


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