1. Managing end stage COPD in primary care
Clinical Knowledge Summaries CKS Chronic obstructive pulmonary disease (COPD)
Managing end stage COPD in primary care
Educational slides based on the CKS topic Chronic obstructive pulmonary disease (November 2010), and NICE guidance; Chronic obstructive pulmonary disease: management of chronic obstructive pulmonary disease in adults in primary and secondary care (2010).

2. Key learning points and objectives
To be able to:
Identify people who have end stage COPD.
Describe when and how to initiate end of life discussions.
Outline simple measures to help breathlessness.
Describe drug treatments that may be used to help breathlessness.

3. Evidence for managing end stage COPD
Where possible the recommendations for managing end stage COPD are based on NICE guidance, however
There is limited evidence in this area and the best evidence available has been used.
This includes in some places expert opinion from review articles.

4. Recognising end stage COPD
End stage COPD may be defined as COPD that is:
Very severe (forced expiratory volume in 1 second [FEV1] less than 30% predicted).
Unresponsive to usual medical treatment for COPD, and
Associated with a probable life expectancy of less than 6–12 months.
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010), and NICE guidance; Chronic obstructive pulmonary disease: management of chronic obstructive pulmonary disease in adults in primary and secondary care (2010).

5. Recognising end stage COPD
For end-stage COPD, the focus is on palliative care to relieve distressing symptoms. 
This is distinct from but similar to terminal care (care given during the last days of life).
A trigger for palliative care may be if the answer to the 'surprise' question is 'no':
'Would you be surprised if this person were to die in the next 6–12 months?'
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010), RCGP (2008) Prognostic indicator guidance. The Gold Standard Framework National Gold Standards Framework Centre.

6. Overview of management
Optimize medical treatment.
Establish a clear management plan based on shared decision making.
Determine whether future hospital admissions for severe exacerbations are appropriate.
Coordinate care with a respiratory nurse specialist, district nurse, palliative care specialist nurse, and social services.
Consider admission to a hospice (e.g. if symptoms are not controlled or if this is the preferred place of death).
Discuss any advance decisions.
Advise simple measures and offer drug treatments (including oxygen) for breathlessness. 
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010), and NICE guidance; Chronic obstructive pulmonary disease: management of chronic obstructive pulmonary disease in adults in primary and secondary care (2010).

7. Discussing end of life issues
Deciding when and how to initiate discussions about end-of-life issues is difficult, however:
Most people with COPD find this discussion helpful.
Ideally the discussions should occur when the person is stable.
Presenter notes
When to discuss end of life issues.
This recommendation is based on In the National Institute for Health and Care Excellence (NICE) guideline Management of chronic obstructive pulmonary disease in adults in primary and secondary care (partial update) [National Clinical Guideline Centre, 2010] and review articles [Seamark et al, 2007; Curtis, 2008; Halpin et al, 2008; Spathis and Booth, 2008], there is agreement that deciding when and how to initiate discussions about end-of-life issues is difficult.
Acceptability and when to initiate
In one qualitative study identified in the NICE guideline [National Clinical Guideline Centre, 2010], a descriptive questionnaire was used to assess the attitudes of 105 people on a pulmonary rehabilitation programme to end-of-life decision making. Most people wanted to learn more about advance directives, mechanical ventilation, and intubation. They said they would find discussions with physicians about these issues acceptable, but such discussions should take place when the person was in a stable condition. Although only half thought physicians should initiate such discussions, only 20 people had had such discussions, almost all of which had been initiated by the patient and not the physician.
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010).

8. Discussing end of life issues
No criteria have been shown to effectively predict survival of 6 months or less accurately, but
Several features may indicate the need to discuss end-of-life issues:
Forced expiratory volume in 1 second (FEV1) less than 30% predicted.
Frequent exacerbations and hospital admissions.
Low body mass index or weight loss.
Co-morbidities (especially left heart failure).
Presenter notes
Criteria indicating the need to discuss end-of-life issues
In a systematic review of tools and predictor variables to help clinicians estimate survival and appropriate timing of palliative care for older adults with non-malignant life-threatening disease, low forced expiratory volume in 1 second (FEV1) was the only variable that can be measured in primary care that was found to be effective in estimating survival [Coventry et al, 2005]. A review article also considered the evidence for commonly used prognostic criteria, finding them to be unreliable [Spathis and Booth, 2008]. However, given that decisions about the timing of end-of-life discussions still need to be made, the criteria presented are based on prognostic factors each recommended in more than one review article [Seamark et al, 2007; Barnett, 2008; Curtis, 2008;Halpin et al, 2008; Spathis and Booth, 2008].
The Prognostic Indicators Guidance paper from the Gold Standards Framework and the Royal College of General Practitioners suggests indicators for survival of 12 months or less in people with COPD [RCGP, 2008]. The paper references the full 2004 NICE guideline [National Collaborating Centre for Chronic Conditions, 2004]; however, CKS could not locate in the NICE guideline the source of these proposed indicators.
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010).

9. Discussing end of life issues
Evidence indicates that people with advanced COPD may wish to discuss:
Treatments (including explanations of both long-term treatments, such as inhalers and oxygen, and short-term crisis treatments, such as intubation and mechanical ventilation).
Prognosis.
What dying might be like and how distressing symptoms might be alleviated.
Advance decisions, for example:
Whether or not to treat or hospitalize for an exacerbation, or
Whether to have life-support measures.
Presenter notes
What end-of-life issues to discuss
These issues are based on the findings of a focus group qualitative study of perspectives of people with COPD [Curtis et al, 2002], a qualitative study involving semi-structured interviews with people with moderate or severe COPD [Barnett, 2008], and qualitative studies reviewed in the NICE full guideline [National Clinical Guideline Centre, 2010].
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010).

10. Advance decisions (living wills)
The usefulness of advance decisions in COPD may be limited because:
It is rarely feasible to give precise instructions for all potential eventualities.
The person's views and values may change over time in response to the increasing severity of disease or during an exacerbation.
Clinicians are responsible for finding out if a valid advance decision exists.
Presenter notes
Advanced decisions
This information on advanced decisions is based on guidance from the General Medical Council, Treatment and care towards the end of life: good practice in decision making [GMC, 2010]; guidance on the Mental Capacity Act from government departments and the British Medical Association [Office of the Public Guardian, 2005; BMA, 2007; Department for Constitutional Affairs, 2007; BMA, 2008; BMA, 2009]; and review articles [Curtis, 2008; Halpin et al, 2008; Nicholson et al, 2008; Spathis and Booth, 2008].
The statements in relation to the usefulness of advance decisions in chronic obstructive pulmonary disease may be limited are based on expert opinion in review articles [Halpin et al, 2008; Spathis and Booth, 2008] and from CKS reviewers.
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010).

11. Advance decisions An advanced decision:
Allows the person to specify (before they have lost the capacity to decide) what treatments they would not want and would not consent to (e.g. mechanical ventilation).
Cannot demand treatments.
Must be respected by clinicians.
Can be withdrawn if the person retains (or regains) capacity.
Presenter notes
This information is based on guidance from the General Medical Council, Treatment and care towards the end of life: good practice in decision making [GMC, 2010]; guidance on the Mental Capacity Act from government departments and the British Medical Association [Office of the Public Guardian, 2005; BMA, 2007; Department for Constitutional Affairs, 2007; BMA, 2008; BMA, 2009]; and review articles [Curtis, 2008; Halpin et al, 2008; Nicholson et al, 2008; Spathis and Booth, 2008].
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010.

12. Advance decisions An advanced decision: Can be made verbally:
Except for decisions that refuse life-sustaining treatment (such as artificial ventilation), which must be written, signed, and witnessed.
Cannot refuse basic care, such as the provision of warmth, shelter, hygiene, food for eating, and water for drinking, but
Clinically assisted nutrition and hydration can be refused, for example:
Nutrition and hydration that is given intravenously, subcutaneously, or via a gastrostomy (considered in law to be medical treatments).
Presenter notes
This information is based on guidance from the General Medical Council, Treatment and care towards the end of life: good practice in decision making [GMC, 2010]; guidance on the Mental Capacity Act from government departments and the British Medical Association [Office of the Public Guardian, 2005; BMA, 2007; Department for Constitutional Affairs, 2007; BMA, 2008; BMA, 2009]; and review articles [Curtis, 2008; Halpin et al, 2008; Nicholson et al, 2008; Spathis and Booth, 2008].
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010).

13. When is an advanced refusal of treatment binding?
An advanced refusal of treatment is binding if:
The person making the advance decision is over 18 years of age, and has the necessary mental capacity.
It specifies treatment to be refused, and the applicable circumstances.
It has not been withdrawn.
Nobody has subsequently been given power of attorney to make treatment decisions on the person's behalf.
The person making the advance decision has not subsequently given reason to believe that they have changed their mind.
Presenter notes
Advanced refusals
This information on advanced refusals is based on guidance from the General Medical Council, Treatment and care towards the end of life: good practice in decision making [GMC, 2010]; guidance on the Mental Capacity Act from government departments and the British Medical Association [Office of the Public Guardian, 2005; BMA, 2007; Department for Constitutional Affairs, 2007; BMA, 2008; BMA, 2009]; and review articles [Curtis, 2008; Halpin et al, 2008; Nicholson et al, 2008; Spathis and Booth, 2008].
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010).

14. Simple measures for breathlessness
Advise the person on the following:
Sitting in front of a fan or open window (or using a hand-held fan).
Positioning:
To sit or stand leaning forward (e.g. onto a table or the back of a chair), and
Support their weight with their arms and upper body.
Pursed-lip breathing:
Inhale through the nose and then exhale slowly, for 4–6 seconds, through pursed lips.
Presenter notes
These recommendations are based on expert opinion in review articles [Seamark et al, 2007;Barnett, 2008; Spathis and Booth, 2008; Abernethy et al, 2009] and a specialist textbook [Hanks et al, 2010].
Bending forward is thought to improve diaphragmatic function [Hanks et al, 2010].
Pursed-lip breathing is thought to decrease air trapping by stenting the airways and preventing airway collapse [Abernethy et al, 2009].
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010).

15. Drug treatment for breathlessness
Offer an opioid as first-line treatment to palliate breathlessness, if breathlessness is unresponsive to other medical treatments.
If an opioid is insufficient offer a trial of:
A benzodiazepine, and or
Oxygen (if the person is not already on long term oxygen).
Benzodiazepines - useful if there is an anxiety component.
Oxygen - Short-burst oxygen therapy may be helpful (intermittent use of supplemental oxygen for periods of 10–20 minutes).
Use a 24% or 28% Venturi mask at a flow rate of 2–4 L/min.
Presenter notes
When to treat
The recommendation to offer opioids, benzodiazepines, and oxygen to people with end-stage chronic obstructive pulmonary disease (COPD) that is unresponsive to other medical therapy is based on the National Institute for Health and Care Excellence (NICE) clinical guidelineManagement of chronic obstructive pulmonary disease in adults in primary and secondary care (partial update) [National Clinical Guideline Centre, 2010; NICE, 2010].
Opioids
NICE [National Clinical Guideline Centre, 2010] identified one systematic review and meta-analysis of opioids for the palliation of breathlessness in terminal illness [Jennings et al, 2002]. Not all studies were of people with COPD, and most had methodological limitations, including small sample size and the potential for carry-over effects (in crossover trials). A statistically significant effect of opioids was demonstrated for breathlessness using non-nebulized opioids; however, when a subgroup analysis of nine COPD studies was done, no statistically significant difference between the treatment and control groups was found for breathlessness. In spite of this, the NICE guideline development group concluded that opioids are useful for palliating breathlessness in people in the end stages of COPD.
Most review articles and textbooks recommend the use of morphine, but there was considerable variation in the starting dosage between trials and that recommended in review articles and textbooks (from 1 mg daily to 30 mg daily) [Jennings et al, 2002; Seamark et al, 2007; Abernethy et al, 2009; Rocker et al, 2009; Regnard and Dean, 2010]. Consequently, firm recommendations cannot be made; CKS recommends that specialist advice be sought or that regimens recommended in the CKS topic on Palliative cancer care - dyspnoea be used.
Benzodiazepines
A recent Cochrane systematic review included three randomized controlled trials with 47 people with COPD in its meta-analysis [Simon et al, 2010]. No statistically significant effect was observed for benzodiazepines compared with control. The authors recommend a trial of benzodiazepines only in people who have not responded to opioids and non-pharmacological measures.
Benzodiazepines were recommended by NICE on the basis of the expert opinion of the guideline development group [National Clinical Guideline Centre, 2010].
Because few review articles or textbooks make suggestions on the choice and dosage of benzodiazepine specifically for people with end-stage COPD, CKS recommends that specialist advice should be sought or that regimens recommended in the CKS topic on Palliative cancer care - dyspnoea are used.
Oxygen
Oxygen is recommended by NICE for the palliation of breathlessness not relieved by other therapies; this recommendation is based on the expert opinion of the guideline development group [National Clinical Guideline Centre, 2010].
The recommendation to use short-burst oxygen is based on a report of the expert working group of the Scientific Committee of the Association of Palliative Medicine [Booth et al, 2004].
The recommendation on the dose of oxygen is based on recommendations by the British Thoracic Society [British Thoracic Society, 2006] and a report of the expert working group of the Scientific Committee of the Association of Palliative Medicine [Booth et al, 2004].
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010), and NICE guidance; Chronic obstructive pulmonary disease: management of chronic obstructive pulmonary disease in adults in primary and secondary care (2010).

16. Drug treatment for breathlessness
Seek specialist advice if:
These measures fail to palliate breathlessness sufficiently, or
Considering a trial of tricyclic antidepressants or antipsychotics.
Tricyclic antidepressants and major tranquilizers
Although NICE also recommends (on the basis of the expert opinion of the guideline development group) the use of tricyclic antidepressants and major tranquilizers (antipsychotics), CKS could find no other expert opinion in favour of these drugs, or on recommended regimens. Consequently, CKS recommends that specialist advice should be sought if these drugs are being considered.
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010), and NICE guidance; Chronic obstructive pulmonary disease: management of chronic obstructive pulmonary disease in adults in primary and secondary care (2010).

17. Summary End stage COPD may be defined as COPD that is:
Very severe (FEV1 less than 30% predicted).
Unresponsive to usual medical treatment for COPD, and
Associated with a probable life expectancy of less than 6–12 months.
Other features that indicate end stage COPD include:
Frequent exacerbations and hospital admissions.
Low body mass index or weight loss.
Comorbidities (especially left heart failure).
Discuss end of life issues when the patient is stable.
Clinicians are responsible for finding out if a valid advance decision exists.
Discuss advance decisions, for example:
Whether or not to treat or hospitalize for an exacerbation, or
Whether to have life-support measures.
Simple measures such as positioning and pursed lip breathing may help breathlessness.
Drug treatment with opioids, benzodiazepines, or oxygen may help breathlessness.
Based on the CKS topic Chronic obstructive pulmonary disease (November 2010), and NICE guidance; Chronic obstructive pulmonary disease: management of chronic obstructive pulmonary disease in adults in primary and secondary care (2010).