Managing respiratory symptoms in palliative care

Abstract

With an ageing population, the NHS is now frequently managing patients with at least one long-term health condition. There is also an increasing need for palliative care as these individuals approach their end of life. Management of respiratory symptoms in such patients can be a challenging task. Palliative care encompasses non-malignant conditions as well as cancer, and multiple interventions have been developed to ease respiratory symptoms. This article reviews how to assess and treat these symptoms in patients with a palliative diagnosis, and outlines the progress made in managing such symptoms.

The RCGP curriculum and managing respiratory symptoms in palliative care

Clinical module 3.09: End of life care has various aspects that can be applied to respiratory disease and symptoms.:

Summarise the principles of palliative care and end of life care and how these apply to cancer and non-cancer illnesses such as cardiovascular, neurological, respiratory and infectious diseases

Understand the evidence base for care at the end of life, while also acknowledging that it is less rigorous because there are very few trials available

Apply best practice principles for end of life care in community settings, such as those described in the Gold Standards Framework

Manage distressing symptoms, e.g. nausea, pain, shortness of breath and confusion

Prescribe effective drugs and suitable combinations of drugs, pre-empting likely side effects

Describe palliative care emergencies and their appropriate management: superior vena cava obstruction

Clinical module 3.19: Respiratory health highlights that it is also important to apply the knowledge learnt from respiratory health to palliative care. In particular, it requires GPs to be able to:

Be able to explain to patients and their carers why they are breathless, the progression of their disease, benefits and limitations of treatments and how to recognise and treat exacerbations

Know the boundaries of primary care management and the role of specialist services in supporting the patient

Assessment and diagnosis

When a patient with a life-limiting condition presents with respiratory symptoms, it is important to establish whether symptoms are from a malignant or non-malignant cause. Symptoms may be a direct effect of lung cancer or lung metastases. Cancer can also cause compression to surrounding structures and increase the risk of acute conditions such as pleural effusions or pulmonary emboli. Furthermore, treatments such as radiotherapy or chemotherapy can lead to lung damage. The patient may have other co-morbidities that increase their risk of exacerbation. For example, they may suffer from chronic obstructive pulmonary disease (COPD) or occupational diseases such as pulmonary fibrosis (National Institute for Health and Care Excellence (NICE), 2016). Figure 1 outlines the key causes to consider in this patient group.

Figure 1.

Differential diagnosis for respiratory symptoms observed in palliative care.

When taking a history, ask about the nature, timing and severity of symptoms. On assessment for severity of breathlessness, tools including the Numerical Rating Scale or the Modified Borg Scale are recommended in palliative care (Ekström et al., 2015). The patient may experience symptoms such as cough, haemoptysis and respiratory secretions. Added sounds such as wheeze and stridor can point to an obstruction caused by cancer.

It is essential to explore how symptoms impact the patient’s psychological wellbeing. The American Thoracic Society describes breathlessness as:

a subjective experience of breathing discomfort that consists of qualitatively distinct sensations that vary in intensity. The experience derives from interaction among multiple physiologic, psychological, social, and environmental factors and may induce secondary physiological and behavioral responses (Parshall et al., 2012).

Breathlessness is exacerbated by anxiety and fear, and therefore management of the psychological aspect of disease will increase the success of treatments.

Management in general practice

Palliative care is based on improving the quality at the end of life through symptom control and holistic care, rather than giving treatments that are considered futile. In order to help practitioners identify these patients, the RCGP released Proactive Identification Guidance in 2016. The Gold Standard Framework (GSF) on COPD and cancer are important in the context of respiratory symptoms (Thomas et al., 2016) (see Box 1). This process allows GPs to initiate timely referral to the community palliative care team.

Box 1.

Gold Standard Framework: Proactive identification guidance.

Cancer

• Deteriorating performance status and functional ability due to metastatic cancer, multi-morbidities or not amenable to treatment – if spending more than 50% of time in bed/lying down, prognosis estimated in months

• Persistent symptoms despite optimal palliative oncology. More specific prognostic predictors for cancer are available, e.g. the palliative performance scale

COPD

At least two of these indicators:

• Recurrent hospital admissions (at last three in the last year due to COPD)

• Medical Research Council (MRC) grade 4/5 – shortness of breath after 100 m on a level surface

• Disease assessed to be very severe (e.g. forced expiratory volume in 1 second (FEV1) less than 30% predicted), persistent symptoms despite optimal therapy, too unwell for surgery or pulmonary rehabilitation

• Fulfils long-term oxygen therapy criteria (Pa02 less than 7.3 kPA)

• Required intensive unit/non-invasive ventilation (ITU/NIV) during hospital admission

• Other factors, e.g. right heart failure, anorexia, cachexia, more than 6 weeks of steroids in preceding 6 months, requires palliative medication for breathlessness still smoking

Principles and materials for The Gold Standards Framework © K Thomas, the National GSF Centre 2003 - 2019. Used with permission from the National GSF Centre in End of Life Care.

Once a person has been identified and has chosen palliative treatment, conversations surrounding advanced care planning can be made. This allows the patient to make decisions about their health that can be upheld when they no longer have mental capacity. During this time, healthcare professionals can discuss personal, religious and spiritual needs. Decisions around cardiopulmonary resuscitation, ceiling of care and future treatment should also be explored (General Medical Council, 2018).

It is also important to consider when sending a patient to hospital would not be in their best interests. Often patients can be managed better in the community. Even in life-threatening situations such as superior vena cava obstruction, hospital admission may be inappropriate, particularly when stenting is unlikely to be successful. Having such conversations avoids unnecessary hospital admission, and allows someone to die in their preferred place of care, usually their own home (Skorstengaard et al., 2017).

Symptom management

Rocker et al. (2007) outlined a stepwise approach to the management of breathlessness in COPD. These principles can be applied to other palliative conditions. It explains that if specific pharmacological management for that condition is not relieving symptoms, then non-pharmacological methods should be tested. Higher sedative medication, such as opioids and anxiolytics, should be saved for refractory respiratory symptoms.

Figure 2.

How to prescribe opioids for breathlessness.

Non-pharmacological management

Simple measures can initially be taken to improve respiratory symptoms, for example, keeping the air in the room cool, and adapting the home to relieve shortness of breath. In order to reassure patients about breathlessness, patients need to acknowledge their fears of choking, suffocation and dying during an episode. Furthermore, it is important to talk about how respiratory symptoms have an impact on daily living and quality of life, and how situations can be adapted to ease symptoms. The British Thoracic Society (BTS) recommend that a trained health professional should assess patients with intractable breathlessness for non-pharmacological treatments (Hardinge et al., 2015). There are three principles in managing the symptom of breathlessness and the methods that can be used to achieve them are outlined in Table 1.

Table 1.

The principles of breathlessness.

Principles	Methods
Ensure efficient and effective breathing	• Breathing re-training: Diaphragmatic breathing and pursed lip breathing helps control • Positioning of the patient: Upright and sitting forward helps lung expansion and get rid of secretions • Chest wall vibration • Neuro-electrical muscle stimulation
Reduce anxiety and panic	• Relaxation techniques: Massaging the patients back • Cognitive behavioural therapy • Complementary therapies: Acupuncture • Using a hand-held fan
Prioritise conserving energy and avoid unnecessary activities of daily activity	• Use of walking aids • Physiotherapy • Occupational therapy

Adapted from Watson et al. (2010) and Bausewein et al. (2008).

Bausewein et al. (2008) evaluated different non-pharmacological interventions and demonstrated strong evidence for neuro-electrical muscle stimulation and chest wall vibration in relieving breathlessness. However, the use of chest wall vibration was only tested in a respiratory laboratory, so the practical application of this intervention is unclear. Breathing training and walking aids were also found to be useful. Most of these studies were conducted on end-stage COPD patients, so this is yet to be evaluated extensively in cancer-related dyspnoea (Bausewein et al., 2008).

Breathlessness services have been emerging in the last 10 to 15 years. Addenbrookes Hospital in Cambridge has set-up a Breathlessness Intervention Service with a palliative care consultant, a specialist physiotherapist and specialist occupational therapist. They conduct a 2-week intervention that incorporates home visits and regular telephone consultations. Evidence-based pharmacological and non-pharmacological interventions are used to improve the symptom of breathlessness. They have found that using this approach significantly reduces the distress of symptoms compared with standard care in patients with cancer (Farquhar et al., 2014). These services are essential in allowing earlier access to palliative care when dealing with complex breathlessness.

Pharmacological management

Once non-pharmacological interventions have been exhausted, medications can be offered to ease the burden of respiratory symptoms and help anxiety. However, it is understandable that research at the end of life involves practical, ethical and emotional issues. Recruiting patients into studies may be particularly exhausting and emotional for participants. Thus, although pharmacological management is recommended by expert panels, it is based on limited evidence from studies often with methodological flaws (Dorman et al., 2009).

Oxygen

In palliative situations, oxygen should be considered if a patient’s saturation levels are persistently below 92%. However, the BTS do not recommend oxygen therapy in patients if they have mild levels of hypoxia or are non-hypoxaemic (Hardinge et al., 2015). Several studies, including a meta-analysis by Ben-Aharon et al. (2012), show that giving oxygen in cancer-related dyspnoea confers no beneficial effect. Oxygen therapy can have unwanted effects such as psychological dependence and social stigma, and should be prescribed with caution (Twycross et al., 2014).

If a patient’s blood gas shows a PaO2 of below 7.3 kPa (or 8 kPa in pulmonary hypertension/oedema) and they have persistently low oxygen levels, they may qualify for long-term oxygen therapy. Patients may also become hypoxic while walking, and therefore, require ambulatory oxygen. A full assessment should be conducted by the dedicated home oxygen assessment service (Hardinge et al., 2015).

The GP will need to complete and send a home oxygen order form (HOOF) to the local oxygen provider. In palliative patients who require oxygen in their last few months, a HOOF can be completed for temporary oxygen while awaiting assessment. This form requires details such as the flow rate of the oxygen, the number of hours needed, and the type of equipment (NHS Primary Care Commissoning, 2011). From 1 August 2017, healthcare professionals complete the Initial Home Oxygen Risk Mitigation form, a safety questionnaire around potential hazards such as smoking, that may increase the risk of incidents at home (BOC Clinical Services, 2017). Arrangements for oxygen therapy vary in different regions, but these principles still apply. The prescribing of home oxygen therapy in the community is a good topic for discussion within practices and for tutorials between trainers and trainees.

Inhaled and nebulised therapy

Inhaled therapy is mostly prescribed in respiratory diseases such as asthma and COPD. Use of bronchodilators can help wheeze and breathlessness by reversing bronchoconstriction. Even in the absence of wheeze, bronchodilators may give benefit. Beta-adrenoreceptor agonists such as salbutamol are used first line. Anticholinergic bronchodilators such as ipratropium bromide can be used if Salbutamol is not effective (Twycross et al., 2014).

If patients are struggling with metered-dose inhalers, then nebulisers can be given. Nebulised saline may also help to clear respiratory secretions and alleviate coughing. In order to facilitate this approach, referral to the community respiratory service is necessary to assess whether the use of nebulisers is appropriate. In most cases, nebuliser medication can be prescribed on the NHS, but patients may need to buy their own compressor (NHS Lothian Respiratory Network, 2014).

Corticosteroids

Steroids are commonly used for complications in cancer patients. Steroids reduce tumour-associated oedema, lymphangitis and tumour-associated airway obstruction; these can all be a cause of breathlessness and excess secretions. Steroids may reduce harsh stridor. Dexamethasone is normally used as the steroid of choice. When using dexamethasone, gastric protection should be considered. If there is no benefit within 4 to 7 days, then it should be stopped (Watson et al., 2010). In an exacerbation of COPD, inhaled corticosteroids are recommended with a long-acting bronchodilator if patients are suffering with persistent breathlessness. Prednisolone can also be offered during an exacerbation (Twycross et al., 2014).

Opioids

Even though unlicensed for this purpose, opioids are recommended as first line in managing moderate-to-severe breathlessness at the end of life (NICE, 2015). Opioids reduce the ventilatory response from the brainstem to hypercapnia and hypoxia. Opioids cause a decrease in motor signals sent from the brainstem to the sensory cortex, and results in reduced consciousness of respiratory effort (Booth and Dudgeon, 2006). Morphine is still used cautiously by clinicians, due to the associated risk of respiratory depression. Studies on the benefit of oral opioids in palliative care mostly involve small sample sizes (less than 50), are of short duration and do not have standardised outcome measures (Barnes et al., 2016). Further evidence is required to determine the optimal, safe dose of opioids.

Figure 2 outlines how to prescribe oral morphine in patients who present with refractory breathlessness. Using the principles of Fig. 2 consider how you would prescribe opioids in the scenarios presented in Box 2.

Box 2.

Clinical scenarios in prescribing opioids.

Ross, a 70-year-old man with severe COPD, has been started on 5 mg of oral morphine when required for moderate breathlessness after admission to hospital. When he comes to see you at the GP practice, he tells you that he has needed to take oromorph every 4 hours to relieve his breathlessness. How would you change the medication in this scenario and why?

The patient requires modified-release morphine, as this will control the breathlessness over a longer period of time. His total daily dose of morphine over 24 hours is 30 mg, and therefore prescribing 15 mg of slow-release morphine twice daily is appropriate.

You are called to see Maria, one of your patients in the nursing home as she is acutely confused. She is an 80-year-old woman suffering with severe osteoarthritis, chronic kidney disease stage 3 and has recently been diagnosed with breast cancer with lung metastases. She is not suitable for any further treatment. She has been started on 5 mg of oral morphine 4 hourly for symptomatic relief. How would you change the medication in this scenario and why?

Although this dose and frequency is considered acceptable in frail patients, this patient has moderate renal impairment and therefore there is an increasing risk of opioid toxicity. As she has stage 3 kidney disease it is best to switch her to oxycodone and reduce the frequency: 2.5 mg every 6 hours is the maximum recommended dose in these patients. Her confusion is most likely due to the high doses of opioids she is receiving.

Benzodiazepines

Benzodiazepines have been shown to be effective in reducing anxiety associated with breathlessness. However, they can also be prescribed to tackle sleep disturbance, depression, psychosis and terminal agitation. Lorazepam is most commonly used, due to its short half-life. Also, in patients who have difficulty swallowing, lorazepam can be administered sublingually so that it works within 5 minutes. Diazepam may be preferred in patients who suffer with chronic anxiety, as it has a longer duration of action, and a wider therapeutic index (De Lima, 2013). Subcutaneous midazolam is also recommended for insomnia, but is more commonly used in the last few weeks of life in terminal agitation (Twycross et al., 2014).

Treating cough

If the cough is wet or productive, mucolytics can be used to break up secretions. Nebulised saline with chest physiotherapy is a mucolytic that can be used in hospital. Chemical mucolytics such as carbocisteine, erdosteine or acetylcysteine are also commonly prescribed. These medications are all known as protussives or expectorants, and loosen up sputum, making coughing more effective, requiring less effort to expel cough secretions. If there is no benefit, mucolytics should be stopped after 4 weeks.

If the cough is dry, antitussives can be prescribed to reduce the frequency of coughing. Demulcents, such as Linctus which soothes the pharynx, are recommended. If demulcents are not effective, weak to strong opioids can suppress the central cough reflex. Codeine or phylocodine are recommended, as they have less of a sedative effect compared with strong opioids (Twycross et al., 2014). Once antitussives and protussives are exhausted, nebulised anaesthetics such as lidocaine may be considered. However, the effect of nebulised anaesthetics has only been investigated in small trials (Truesdale and Jurdi, 2013). Haemostatics, such as tranexamic acid, can also be given if there is persistent haemoptysis.

Treating respiratory secretions in the imminently dying

When death is imminent, the increase in respiratory secretions can be very distressing for family members. This requires sensitive discussion and to reassurance that this is a natural part of the dying process. Anticipatory medications can be given to ease distress at the end of life. These are normally given subcutaneously via a syringe driver. Morphine is used to help with pain, breathlessness and cough. Midazolam helps with anxiety-associated breathlessness. Hyoscine hydrobromide is used for excess respiratory secretions in those who are too weak to expectorate.

Conclusion

As a GP, it is important to have a management plan in place when treating respiratory symptoms in patients with a life-limiting condition. The plan should incorporate seeking out treatable causes and exploring patients’ psychological, social and environmental concerns. There are now tools available to identify patients with end of life symptoms earlier, so prompt referral can be made to the palliative care team. Local respiratory services can be accessed to facilitate extra support at home. Pharmacological and non-pharmacological techniques can be used to improve respiratory symptoms, despite the low quality of research evidence available. Better evidence on the efficacy of treatments would give clinicians more confidence in the use of these treatments and techniques.

KEY POINTS

Effective management of respiratory symptoms in palliative care focuses on psychological, social and environmental concerns, as well as physical aspects of the respiratory symptom

The GSF Proactive Identification Guidance is used to identify patients with a palliative diagnosis, so additional supportive care can be offered earlier

A trial of non-pharmacological techniques is recommended before using stronger medication for respiratory symptoms

Oxygen therapy for symptom relief is no longer recommended in patients who are non-hypoxaemic

The use of opioids has been found to be effective in treating breathlessness and cough, but the safety profile is yet to be confirmed with good evidence

It is important to find out about your local community respiratory service, to understand how oxygen and nebuliser therapy can be arranged at home

Footnotes

Acknowledgement

We would like to thank Dr Jonathan Mills for his help with the writing of this article under the InnovAiT ‘buddy’ scheme.

References

Barnes H, Mcdonald J, Smallwood N, et al. (2016) Opioids for the palliation of refractory breathlessness in adults with advanced disease and terminal illness. Cochrane Database of Systematic Reviews 3: art. no.: CD011008. DOI: 10.1002/14651858.CD011008.pub2.

Bausewein C, Booth S, Gysels M, et al. (2008) Non-pharmacological interventions for breathlessness in advanced stages of malignant and non-malignant diseases. Cochrane Database of Systematic Reviews 2: art. no.: CD005623. DOI: 10.1002/14651858.CD005623.pub2.

Ben-Aharon

Gafter-Gvili

Leibovici

et al. (2012) Interventions for alleviating cancer-related dyspnea: A systematic review and meta-analysis. Acta Oncologica 51(8): 996–1008. DOI: 10.3109/0284186X.2012.709638org/10.3109/0284186X.2012.709638.

BOC Clinical Services (2017) How to complete a home oxygen order form (HOOF). Available at: www.bocclinicalservices.co.uk/en/healthcare-professionals/hoof/index.html (accessed 21 November 2018).

Booth

Dudgeon

(2006) Dyspnoea in Advanced Disease: A Guide to Clinical Management, Oxford: Oxford University Press.

De Lima L (2013) World Health Organization: Essential medicines in palliative care. Available at: www.who.int/selection_medicines/committees/expert/19/applications/PalliativeCare_8_A_R.pdf (accessed 20 November 2018).

Dorman

Jolley

Abernethy

et al. (2009) Researching breathlessness in palliative care: Consensus statement of the National Cancer Research Institute Palliative Care Breathlessness Subgroup. Palliative Medicine 23(3): 213–227. DOI: 10.1177/0269216309102520.

Ekström

Currow

Johnson

(2015) Outcome measurement of refractory breathlessness. Current Opinion in Supportive and Palliative Care 9(3): 238–243. DOI: 10.1097/SPC.0000000000000147.

Farquhar

Prevost

McCrone

et al. (2014) Is a specialist breathlessness service more effective and cost-effective for patients with advanced cancer and their carers than standard care? Findings of a mixed-method randomised controlled trial. BMC Medicine 12: 194 DOI: 10.1186/s12916-014-0194-2.

10.

General Medical Council (2018) Working with the principles and decision-making models. Available at: www.gmc-uk.org/ethical-guidance/ethical-guidance-for-doctors/treatment-and-care-towards-the-end-of-life/working-with-the-principles-and-decision-making-models—cont#paragraph-63 (accessed 14 September 2018).

11.

Hardinge

Annandale

Bourne

et al. (2015) British Thoracic Society guidelines for home oxygen use in adults: Accredited by NICE. Thorax 70: i1–i43. DOI: 10.1136/thoraxjnl-2015-206865.

12.

NHS Lothian Respiratory Network (2014) Nebulisers and nebulised medication. Available at: www.lothianrespiratorymcn.scot.nhs.uk/wp-content/uploads/2014/09/nebuliser-professional-advice-v2-0.pdf (accessed 20 November 2018).

13.

NHS Primary Care Commissioning (2011) Home oxygen order form [online]. Available at: www.pcc.nhs.uk/home-oxygen-order-form (accessed 14 April 2019).

14.

NHS Scotland (2015) Scottish palliative care guidelines – breathlessness. Available at: www.palliativecareguidelines.scot.nhs.uk/guidelines/symptom-control/breathlessness.aspx (accessed 26 August 2018).

15.

NICE (2015) Care of dying adults in the last days of life. Available at: www.nice.org.uk/guidance/ng31/chapter/Recommendations-for-research (accessed 27 August 2018).

16.

NICE (2016) Clinical knowledge summaries. Palliative care - dyspnoea. Available at: https://cks.nice.org.uk/palliative-care-dyspnoea#!topicsummary (accessed 3 September 2018).

17.

Parshall

Schwartztein

Adams

et al. (2012) An official American Thoracic Society statement: Update on the mechanisms, assessment, and management of dyspnea. American Journal of Respiratory and Critical Care Medicine 185(4): 435–452. DOI: 10.1164/rccm.201111-2042ST.

18.

RCGP. Clinical module 3.09: End of life care. Available at: www.rcgp.org.uk/training-exams/training/gp-curriculum-overview/online-curriculum/caring-for-the-whole-person/3-09-end-of-life-care.aspx (accessed 25 September 2018).

19.

RCGP. Clinical module 3.19: Respiratory health. Available at: www.rcgp.org.uk/training-exams/training/gp-curriculum-overview/online-curriculum/applying-clinical-knowledge-section-1/3-19-respiratory-health.aspx (accessed 25 September 2018).

20.

Rocker

Sinuff

Horton

et al. (2007) Advanced chronic obstructive pulmonary disease: Innovative approaches to palliation. Journal of Palliative Medicine 10(3): 783–2 DOI: 10.1089/jpm.2007.9951.

21.

Skorstengaard

Neergaard

Andreassen

et al. (2017) Preferred place of care and death in terminally ill patients with lung and heart disease compared to cancer patients. Journal of Palliative Medicine 20(11): 1217–1224. DOI: 10.1089/jpm.2017.0082.

22.

Thomas K, Armstrong Wilson J and GSF Team (2016) The gold standards framework proactive identification guidance (PIG) 6th edition. Available at: www.goldstandardsframework.org.uk/PIG (accessed 2 September 2018).

23.

Truesdale

Jurdi

(2013) Nebulized lidocaine in the treatment of intractable cough. American Journal of Hospice and Palliative Medicine 30(6): 587–589. DOI: 10.1177/1049909112458577.

24.

Twycross RG, Wilcock A and Howard P (2014) PCF5: Palliative Care Formulary.

25.

Watson

Lucas

Andrew

et al. (2010) Oxford Handbook of Palliative Care, Oxford: Oxford University Press.