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168 Chapter 12
Table 7 Opioids for cough.
Codeine phosphate linctus (15 mg/5 ml strength) 15–30 mg
PO PRN, then regularly TDS-QDS if beneficial
Morphine liquid IR 2–5 mg PO PRN, then regularly QDS if
beneficial (or MR preparation 5–10 mg BD PO)
amplify the perception of breathlessness. It is also worth
noting that the response to opioids can be reduced by anx-
iety and depression [58, 59].
Suitable antidepressants to use in this situation are
selective serotonin reuptake inhibitors for example,
Sertraline 25–50 mg OD or Citalopram 10 mg OD.
Mirtazapine 15 mg nocte is an alternative option which
may also help with insomnia. Doses can be titrated to effect.
Oxygen
Palliative oxygen (O
2
) (e.g. 2–4 L/min) can be considered
for symptom management if the O
2
saturations are less
than 90–92% on air and other measures have not helped.
Benefit is not necessarily related to the degree of hypoxia,
and other interventions for example, opioids are likely to
be more beneficial in most cases [60]. If someone is not
hypoxic then O
2
is no more helpful than air and a cool
breeze may be as beneficial (see fan therapy). Be careful
about introducing oxygen therapy unnecessarily as
psychological dependence can be an issue and home
oxygen can be very limiting in terms of someone’s mobility
and ability to go outdoors. When oxygen is used for the
palliation of breathlessness, it’s effectiveness should be
assessed in terms of an improvement in breathlessness,
rather than an improvement in O
2
sats, and if it is unhelpful
should be discontinued. Oxygen therapy can often be
withdrawn in the last days of life without problems [61].
Long-term oxygen therapy (LTOT), used for 16 h + per
day, plus or minus ambulatory oxygen is used in specific
circumstances and generally improves survival rather than
offering symptomatic benefit. It should be prescribed and
monitored by a respiratory specialist. Some patients who
desaturate only on exertion (e.g. those with ILD) may be
prescribed ambulatory oxygen alone.
Home oxygen should not be prescribed when anyone in
the household still smokes because of the risk of fire. Oil-
based emollients, petroleum jelly, and paraffin-containing
products should be avoided. Oxygen use does not in itself
prevent someone from being able to drive. Nasal cannulae
(prongs) are often better tolerated than a mask.
Humidification can help prevent dryness and advice on
mouth and skin care should be provided.
Managing Cough
As with breathlessness, the first step in managing cough
where possible is to identify and treat the underlying cause.
When assessing and managing cough it is important to dis-
tinguish between a dry cough and one where sputum
management is the main issue.
Productive Cough
It is common for patients with lung cancer and chronic
respiratory disease for example, COPD, bronchiectasis to
experience a chronic productive cough. Patients with tra-
cheostomies and those with MND may experience similar
problems with secretions. Infective exacerbations can be
treated with appropriate antibiotics. Chronic sputum which
is thick and viscous and difficult for patients to expectorate
can be helped by mucolytics such as carbocisteine 750 mg
TDS which can be reduced to a maintenance dose of 375
mg TDS or 750 mg BD in time. Nebulised sodium chloride
0.9% (normal saline) qds + prn can also be effective.
Nebulised hypertonic saline (3–7%) is sometimes recom-
mended in bronchiectasis. Some patients find that simple
measures can help such as keeping well hydrated, drinking
hot drinks, and steam inhalation. For those with more
problematic sputum, physiotherapists can recommend
specific techniques and equipment such as a flutter device
or cough assist machine in certain circumstances.
For those not responding fully to the above protussive
measures, antitussives can be used in addition (see dry
cough). For patients nearing the end-of-life, suctioning may
be appropriate and/or the use of antimuscarinic medication
for example, glycopyrronium or hyoscine butylbromide as
well as careful patient positioning, mouth, and skin care.
Dry Cough
First of all, consider if the cough is unrelated to any lung
condition for example, secondary to medication (e.g. ACE
inhibitor) or due to gastro-oesophageal reflux disease
(GORD) or post-nasal drip (PND). Consider a two-month
trial of a proton pump inhibitor (PPI) BD for GORD [62,
63], or intranasal therapy (steroid, antihistamine, ipratro-
pium bromide) for PND [64].
For some people simple linctus (5 ml TDS-QDS) or
menthol lozenges can help.
Opioids for Dry Cough
A dry cough can be managed by cough suppression.
Opioids are effective antitussives (see Table 7 for options).
For those most bothered by cough overnight, a dose of
opioid can just be given OD at bedtime.
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The Management of Respiratory Symptoms and Advanced Chronic Obstructive Pulmonary Disease 169
Alternative Antitussives for Dry Cough
Some alternative antitussive options, if opioids are unsatis-
factory are listed in Table 8.
Each have neuro-inhibitory effects and may act by inter-
fering with the cough reflex and/or central sensitisation
which leads to cough hypersensitivity present in most
patients with chronic cough.
Thalidomide can be considered for intractable cough
due to Idiopathic Pulmonary Fibrosis (IPF) not helped by
other measures. This should be under the guidance of an
ILD specialist [65].
COPD
In the UK alone there are 1.2 million people living with
COPD and 30,000 deaths per year. Patients are often highly
symptomatic for many years but, due to the invisibility of
chronic breathlessness already discussed, symptoms are
often ignored or poorly managed. Patients with COPD
have a very different illness trajectory to those with cancer.
They experience exacerbations (infective or non-infective)
which often lead to hospitalisation and any one of these
can potentially lead to death. Despite an overall gradual
decline in condition, death can be difficult to predict and
can appear unexpected (see Figure 4).
This can lead to “prognostic paralysis” [67] where clini-
cians avoid talking about the future and people’s wishes
because they cannot predict when a significant deteriora-
tion will happen. Actually, this lack of a crystal ball
shouldn’t stop important conversations taking place. A
helpful mantra is to “hope for the best but prepare for the
worst” [68]. The Gold Standards Framework (GSF)
Proactive Identification Guidance (PIG) is a useful tool for
identifying people with COPD who could benefit from a
palliative care approach, conversations about the future,
and possibly a referral to specialist palliative care services/
hospice (see Table 9) [69].
Usual therapy for patients with COPD includes inhaled/
nebulised beta-2 agonists, muscarinic antagonists, and
inhaled corticosteroids. Long-acting and combination
preparations are available. Spacers can be helpful. For
nebulised medications, some patients find a mouthpiece
less claustrophobic than using a mask. Long-term use of
oral corticosteroids should be avoided. Oral theophylline
is occasionally recommended. Prophylactic antibiotics
(e.g. azithromycin 250 mg 3 times a week) are sometimes
used for those who have frequent/prolonged/severe infec-
tive exacerbations. Diuretics can be helpful for those with
oedema due to cor pulmonale. A small selection of pat-
ents are suitable for lung reduction surgery or transplan-
tation [70].
The stepwise approach to breathlessness management
described above should be used for people with COPD. If
symptoms persist despite optimisation of usual COPD
therapy (step 1) then non-pharmacological (step 2) mea-
sures should be considered using the BTF model to assess
which would be most effective, followed by palliative phar-
macological interventions if needed.
People with COPD often experience frequent exacerba-
tions. Patients should have a clear and regularly reviewed
Function Function
High
Low
Time
High
Low
Time
Death Death
Cancer COPD
Figure 4 Illness trajectories in cancer and COPD. Adapted from Lunney, J.R., Lynn, J. and Hogan, C. (2002) [66].
Table 8 Alternative antitussives.
Gabapentin 100–300 mg OD and titrate dose/frequency every
few days to effect.
Diazepam 5 mg nocte.
Amitriptyline 10 mg nocte.
Baclofen 10 mg TDS or 20 mg OD.
https://t.me/med1917
170 Chapter 12
plan for how to manage these when they occur in order to
avoid unnecessary hospital admissions. Patients are taught
how to recognise signs of infection and often have rescue
packs (antibiotics and steroids) at home to treat these
without delay. Non-infective exacerbations are best
managed with step 2 and 3 measures tailored to the
individual. It can be useful to have a written plan for
patients and their carers to follow in a crisis.
Acute exacerbations sometimes require hospitalisation
for intravenous antibiotics (although sometimes these can
be given at home), closer monitoring, and invasive or non-
invasive ventilation (NIV). Patients with chronic hyper-
capnic respiratory failure can be considered for long-term
home NIV. NIV can be quite a distressing intervention for
patients. Those who have experienced it and those who
are at risk should have the opportunity to discuss their
preferences regarding future treatment escalation. Some
people choose to refuse this treatment in advance. Some
choose to have it withdrawn when they are unwell and
gaining no overall benefit from it. NIV withdrawal in
either an acute or chronic situation is a complex interven-
tion and should be done jointly by respiratory and pallia-
tive care specialists.
Interstitial Lung Disease (ILD)
There are numerous types of ILD. One of the most
common is Idiopathic Pulmonary Fibrosis (IPF). IPF has a
poor prognosis and patients should be offered a palliative
care approach at diagnosis. A few patients will be suitable
for lung transplantation and some will meet the strict cri-
teria for antifibrotics (pirfenidone or nintedanib). Most
patients with ILD are very symptomatic at diagnosis and
will benefit from step 2 and 3 interventions for breathless-
ness management as well as interventions for cough.
Patients are often hypoxic requiring increasing levels of
home oxygen therapy. Exertional desaturation is common
and so patients often have one oxygen prescription for at
rest and a second higher flow rate prescribed for when
mobilising. Although patients with ILD can experience
infective exacerbations, this is not so much a feature as it is
for those with COPD and patients tend to have a slightly
more predictable illness trajectory, more similar to those
with malignancy. Patients with both COPD and ILD have
a particularly poor prognosis.
COVID-19
On 12 March 2020, COVID-19 was declared a pandemic
by the World Health Organisation. In severe cases symp-
toms can escalate rapidly therefore an anticipatory
approach to symptom management is key [71]. Symptoms
can include breathlessness, cough, fatigue, anxiety, agita-
tion, drowsiness, and delirium. As with all causes of
breathlessness, opioids and the non-pharmacological
measures described above can be helpful. However, the
use of a handheld fan is not recommended in COVID-19
because of the theoretical risk of increasing droplet and
aerosol spread. Patients who are able to adopt a prone
position themselves should be encouraged to try it for
relief of breathlessness. Benzodiazepines and/or antipsy-
chotic medication can be used to manage anxiety, agita-
tion, and delirium. Communication with patients and
family can be challenging due to noisy NIV machines, the
use of personal protective equipment (PPE), social dis-
tancing and restricted visiting in hospitals. Technology
can be helpful here, for example, for remote consultations
and to support at the bedside communication. Simple
bedside tools include transparent face masks and hand-
held whiteboards.
Table 9 Gold Standards Framework (GSF) Proactive
Identification Guidance (PIG) for COPD [69].
GSF PIG for COPD – at least two of the following
indicators:
• Severe disease (e.g. FEV1 < 30% predicted),
persistent symptoms for example, breathlessness despite
optimal therapy, causing distress
• Recurrent hospital admissions (at least three in
last year due to COPD)
• Hypoxia/fulfilling long term oxygen therapy
criteria (PaO2 < 7.3kPa)
• Too unwell for surgery or pulmonary
rehabilitation
• MRC grade 4/5 – shortness of breath after 100
metres on level surface
• Required ITU/NIV during admission or
ventilation contraindicated
• Other factors for example, right heart failure,
anorexia, cachexia, >six weeks steroids in preceding six
months, despite specialist review/treatment optimisation,
requires palliative medication for breathlessness.
Reproduced with permission of the National GSF Centre in
End-of-Life Care UK.
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The Management of Respiratory Symptoms and Advanced Chronic Obstructive Pulmonary Disease 171
Hospitalised patients deteriorating despite non-invasive
ventilation (NIV), usually Continuous Positive Airway
Pressure (CPAP), may choose to have this withdrawn, or a
decision to withdraw treatment may be made in best inter-
ests when the patient lacks capacity. Parenteral opioids and
benzodiazepines are usually required to enable a patient to
be comfortable enough to tolerate this. This is a complex
intervention which usually requires both respiratory and
specialist palliative care team input.
A Joint Specialty Multidisciplinary Team
(MDT) Approach
In some localities, there is joint specialty working bet-
ween respiratory and palliative care teams. Patients can
be referred for an MDT discussion and some services
offer joint consultations in outpatient clinics or other set-
tings. These services tend to focus on patients with non-
malignant conditions who experience chronic symptoms,
exacerbations, crises, and frequent hospital admissions.
This approach enables a greater proportion of patients to
live and die in their preferred place while improving their
physical, emotional, and social wellbeing [72].
Summary
In this chapter, we have reviewed the causes of breathless-
ness and other respiratory symptoms in advanced malig-
nant and non-malignant disease and how to best manage
these. Careful assessment and the use of a stepwise approach
to management is important. Non-pharmacological inter-
ventions should always be considered ahead of palliative
pharmacological measures. Understanding illness trajec-
tories in non-malignant disease helps clinicians communi-
cate more effectively about prognosis and treatment
options. As with all areas of palliative care, a holistic
approach and good use of the full multidisciplinary team
is key.
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Handbook of Palliative Care, Fourth Edition. Edited by Richard Kitchen, Christina Faull, Sarah Russell, and Jo Wilson.
© 2024 John Wiley & Sons Ltd. Published 2024 by John Wiley & Sons Ltd.
13
estimated 2.5% will present clinically in their final five
years of life [1]. Approximately a quarter of those present-
ing with MSCC have no known cancer diagnosis [2]. The
risk of MSCC differs widely between cancer diagnoses;
over half of patients have a diagnosis of breast, lung, or
prostate cancer with haematological, gastro-intestinal and
renal cell cancers much less frequent [3].
Early Suspicion and Clinical Diagnosis
Patients with known bone or spinal metastases and those
at high risk of developing spinal metastases, should be
given written information explaining the symptoms to
look out for [4].
In patients with MSCC, 94% experience back pain, with
pain often developing over a median of 3 months before
further symptoms and signs occur, giving a “window of
opportunity” for diagnosis [3]. Localised pain in the
vertebral column may be associated with bony tenderness
and radicular pain, sometimes radiating in a dermatomal
distribution. Pain is severe and unremitting, often prevent-
ing sleep and worsening with activities that raise intra-
abdominal pressure, involve movement, lying supine,
percussion over the vertebrae or neck and hip flexion.
Myelopathy manifests as weakness, variable sensory dis-
turbance, and loss of bowel and bladder function.
Weakness is often symmetrical although unilateral presen-
tation can occur early on. Sphincter dysfunction occurs
late in spinal cord compression but is an earlier feature of
cauda equina compression, resulting in urinary retention,
constipation, faecal incontinence, and loss of anal tone [5].
The clinical picture varies and the frequency of symptoms
is shown in Table 1 [6]. Infrequently, myelopathy due to
MSCC can manifest with neurological impairment in the
absence of pain.
Managing Complications of Cancer
Lesley Charman, Rachael Barton and Katie Spencer
Introduction
Patients with advanced or metastatic cancer often experi-
ence complications caused by their disease or its treatment.
These may have a profound effect on their functional
ability and quality of life. Although such complications are
commonly seen in oncology, they may be rare outside the
specialty. It is important to identify early those who may
benefit from specific treatment, since a prompt referral
allows the rapid palliation of symptoms minimising their
impact on quality of life. It is equally important to identify
those patients who are in the terminal stage of their illness
who may be helped more by symptomatic measures than
by referral for investigation and treatment. Advance care
planning and understanding of patients’ priorities in the
context of their expected disease trajectory can help ensure
that the care delivered aligns with patient preferences. This
chapter outlines the clinical problems most frequently
encountered and suggests management guidelines, so that
non-specialists may have confidence in dealing with what
is often a distressing situation for both patient and carer.
Malignant Spinal Cord Compression
(MSCC)
MSCC is an oncological emergency requiring urgent rec-
ognition, diagnosis, and management. Where function has
been lost at the point of treatment, it is rarely regained. It is
therefore essential to have a high level of suspicion in
patients with known cancer, particularly where bony meta-
static disease has been previously demonstrated.
Post-mortem evidence suggests MSCC may be present
in 5–10% of patients with advanced cancer and an
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176 Chapter 13
cautious mobilisation can begin [7]. In the absence of sur-
gical stabilisation, or brace support, following initial
assessment, the need to maintain neutral alignment
requires careful consideration. In all cases the risks and
benefits of potentially permanent immobilisation versus
the risks of mobilisation as pain allows should be dis-
cussed with the patient and decisions made in line with
their preferences.
Subsequent Treatment Options
Despite the development of MSCC being a poor prog-
nostic sign, active management is indicated in the majority
of patients. Definitive treatment is arranged via the MSCC
coordinator and needs to begin before further neurological
deterioration, ideally within 24 hours of diagnosis.
The potential benefit of treatment is extremely limited
in patients very near the end-of-life or where paraplegia is
established in the absence of pain. For these patients multi-
disciplinary assessment and sensitive discussion can guide
a more appropriate management plan focussing on the
medical management of symptoms.
Surgery
Surgery is considered when there is structural spinal insta-
bility with or without pain, particularly if neurological
compromise can be prevented. Spinal decompression and
stabilisation followed by radiotherapy improves the out-
come in terms of maintenance of mobility and continence
compared with radiotherapy alone [9]. Other relative indi-
cations for surgery include malignancy of unknown pri-
mary (to support histological diagnosis) or disease that is
radio-resistant. Patients most likely to benefit from surgery
are those with a good prognosis and limited sites of meta-
static disease. Patients with a prognosis of less than three
months should not be managed surgically given the time
required for recovery. A recognised prognostic scoring
system such as that described by Tokuhashi is recom-
mended to aid decision making (see Box 2) [7, 10].
Radiotherapy
Radiotherapy is the mainstay of treatment, and pain
improvement is seen in approximately 50–60% of patients
[11]. In general, radiotherapy is more effective when cord
compression occurs without bony collapse and instability
[12]. A single fraction is as effective as two or five fractions
in patients with limited prognosis while for those with a
better prognosis limited evidence supports consideration
of a longer course [7, 13, 14]. Where a patient with poor
prognosis has complete paraplegia and no pain, a holistic
approach to symptom control may be more appropriate
than radiotherapy [11].
Investigations
Patients with new or unremitting spinal pain on a
background of known malignancy, or a suspicious history
in patients not known to have cancer, with or without
clinical signs, require urgent clinical assessment. If clini-
cally suspicious for MSCC, urgent discussion with the
local MSCC co-ordinator is required, and an MRI of the
whole spine should be performed within 24 hours unless
there is a specific contraindication [4]. Occasionally MRI
will be required sooner than 24 hours if there is a pressing
clinical need for emergency surgery.
Treatment
Immediate Management
All patients with suspected or confirmed MSCC should
be prescribed dexamethasone 16 mg immediately. This
should only be given parenterally if there is concern
about oral absorption [7]. This should be followed by
dexamethasone 16 mg daily (in 1–2 divided doses, the
latest given before 2p.m. to avoid night-time wakefulness,
although this should not preclude emergency
administration of the first day’s dose). Patients who
remain on steroids for more than a week should be issued
with a steroid alert card and advice regarding the
necessity of gradual weaning to avoid adrenal crisis [8].
Concurrent prescribing of gastric protection is required.
Strong opioids and nonsteroidal anti-inflammatories
(NSAIDs) (where appropriate) are often required for
pain, and potentially also antispasmodics and neuro-
pathic agents if appropriate.
Patients with severe mechanical pain suggestive of
spinal instability or symptoms/signs suggestive of MSCC
should be nursed flat with neutral spinal alignment until
bony and neurological stability are established and
Table 1 The frequency of symptoms in MSCC. Source:
Levack et al. [6].
Symptom % with symptom
Pain 94
Weakness or difficulty walking 82
Changes in sensation 68
Difficulty passing urine 56
Constipation 66
Faecal incontinence 5
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Managing Complications of Cancer 177
Outcomes
Function
Most patients who are ambulant at the point of treatment will
maintain ambulation subsequently. Where ambulatory
function is lost this is regained in less than a third of patients
undergoing radiotherapy and less than 10% of patients pre-
senting with paraplegia [15]. Of those not requiring a urinary
catheter before treatment, 80% will remain catheter-free com-
pared to 20% of those who require a catheter at diagnosis.
Survival and Prognostic Factors
In patients diagnosed with MSCC, 18–30% survive for one
year. Prognosis, however, may vary from short days to a
number of years. Factors influencing survival are summa-
rized in the Tokuhashi score (see Table 2) [10].
Rehabilitation and Discharge Planning
The physiotherapist and occupational therapist play a key role
in supporting adjustment and, where appropriate, rehabilita-
tion in patients presenting with MSCC. Patients and carers
may need support to adapt to a limited prognosis, loss of
mobility and bladder/bowel function but also to the distress
associated with loss of social status and change in body image.
Immediate needs may include:
•
Special equipment or adaptations at home.
• An alternative place of care such as a care home or palli-
ative care unit.
•
A “package” of social and health care.
• Psychosocial support.
Follow-up
More than 70% of patients will have other vertebral sites
affected by metastatic disease giving a risk of further MSCC at
both the treated site and beyond. Radiotherapy is often given
where other lesions are painful although there is no evidence
to support prophylactic treatment. To continue to optimise
function, pain control, and quality of life, regular follow-up is
vital and this may be community rather than hospital led.
Superior Vena Cava Obstruction (SVCO)
SVCO describes a syndrome characterised by obstruction
to blood flow through the superior vena cava (SVC).
Malignancy is the major cause of SVCO (in excess of 90%
of cases) with a majority of cases being due to lung cancer
(75%) or lymphoma (15%) [16]. Where malignancy is
present SVCO is frequently the initial clinical
presentation.
Chemotherapy
Chemotherapy may be indicated for chemosensitive
tumours such as lymphoma, small-cell lung cancer, and
germ cell tumours, and is usually followed by radiotherapy.
Table 2 Tokuhashi prognostic scoring system in spinal
cord compression [10].
Predictive Factor Score (Points)
Performance status (KPS)
Poor (KPS 10–40%)
Moderate (KPS 50–70%)
Good (KPS 80–100%)
0
1
2
Number of extraspinal bone
metastases
≥3
1–2
0
0
1
2
Number of metastases in vertebral
bodies
≥3
2
1
0
1
2
Metastases to visceral organs
Unresectable
Resectable
No metastases
0
1
2
Primary cancer diagnosis
Lung, Pancreas, Oesophageal, Gastric,
Bladder, Osteosarcoma
Liver, Gallbladder, Unidentified
Others
Uterus, Kidney
Rectum
Thyroid, Prostate, Breast, Carcinoid
0
1
2
3
4
5
Paralysis
Complete
Incomplete
None
0
1
2
Using this system, patients scoring 0–8: Prognosis <6 months;
9–11: Prognosis 6–12 months; ≥12: Prognosis >12 months.
KPS=Karnofsky Performance Status, a measure of a patient’s
functional ability and activity levels which deteriorate near
the end-of-life.
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