Oxford Handbook of Palliative Care (2 edn)
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While this chapter focuses on the common oncological emergencies in palliative practice, other emergencies include a wider range of issues such as:
An emergency discharge so a patient’s wish to die at home can be met
Emotional emergencies, with high levels of expressed anxiety
Spiritual/existential/social emergencies with pressure to ‘sort things out’ before it is too late
It is important to have a clear understanding of the management of emergencies in palliative care as clear thinking is crucial in handling such situations. Providing transparent decisiveness to the patient, their family and staff can transform a crisis situation filled with anxiety.
Faced with an emergent, acute problem in the palliative care setting, clinicians would do well to establish quickly the answer to three simple questions:
Where is the patient on their disease trajectory?
What is causing this particular problem at this particular time?
What ideas, concerns and expectations does the patient/family have?
The time frame for normal hospice and palliative care interventions are modulated by the need for calmness and patient comfort. There are, however, several emergency situations which can occur for hospice patients requiring urgent and prompt diagnosis and management. This may create some dissonance among staff and other patients. It is vital that all staff appreciate the nature of these emergencies and the appropriateness of an urgent response.
Sepsis in the neutropenic patient
All clinical staff should be acutely aware of the serious risk and precarious condition of patients who have become neutropenic and febrile following oncological treatments. If not managed rapidly and effectively, these patients will die from overwhelming infection.
An absolute neutrophil count of less than 0.5 × 109/L is defined as severe neutropenia, although the risk of infection increases as the count falls below 1.0 × 109/L
Initially, patients may develop general influenza-type symptoms, highlighting the need for vigilance and a high index of suspicion in patients who have recently received either cytotoxic chemotherapy or immunosuppressant therapy.
Patients should be admitted to a unit where essential investigations can be carried out rapidly and where intravenous antibiotics can be given and suitable monitoring maintained. Appropriate facilities are not usually available within a hospice unit. Occasionally, patients may develop neutropenic sepsis while in a palliative care setting, which can create a dilemma. After full discussion, emphasizing the gravity of the situation and the high chance of a good response to optimal management in an acute unit, patients may still refuse transfer, preferring to stay in the hospice and receive suboptimal care. This should be discussed with their oncologist if possible.
Empirical treatment of a febrile neutropenic patient should be instigated and an appropriate antibiotic regime started, with advice from the local bacteriologist since antibiotic regimes vary across the country.
A regime currently being advocated at The Royal Marsden Hospital is outlined below as an example
Spinal cord compression
Back pain (present in 90% of cases)
Weak legs
Increased reflexes
Sensory level
Urinary hesitancy (late feature)
Spinal cord compression occurs in 3–5% of patients with cancer, and 10% of patients with spinal metastases develop cord compression,2 the frequency being highest in multiple myeloma and cancers of the prostate, breast and bronchus.
Malignant causes:
intramedullary metastases
intradural metastases
extradural compression (80%)
vertebral body metastasis
vertebral collapse
tumour spread
interruption of vascular supply
It is important to have a high index of suspicion for possible cord compression, because of the consequences of paraplegia and urinary and faecal incontinence with a delay in diagnosis.
Symptoms and signs
Spinal cord compression must be considered in all cancer patients with back pain. Pain often pre-dates neurological changes by some considerable time
A sensation of weakness in the legs and often vague sensory symptoms in the legs may be early manifestations. Patients may complain of a band-like pain, particularly on coughing or sneezing
For those presenting with profound weakness, a sensory ‘level’ and sphincter disturbance, which are relatively late features, the outcome is poor and the compression is much less likely to be reversible
Tenderness along the bony spine may be present
thoracic in 70%
lumbosacral 20%
cervical 10%
Lesions above L1 (lower end of spinal cord) will produce upper motor neurone signs and often a sensory level, whereas lesions below L1 will produce lower motor neurone signs and perianal numbness (cauda equina syndrome). Multiple sites of compression may produce different and confusing neurological signs (Table 15.1).
| Upper motor neurone lesion | Lower motor neurone lesion | |
|---|---|---|
Power | Reduced/absent | Reduced/absent |
Tone | Increased | Reduced |
Sensation | Sensory loss | Sensory loss |
Reflexes (plantars) | Increased (upgoing) | Absent/reduced (downgoing) |
| Upper motor neurone lesion | Lower motor neurone lesion | |
|---|---|---|
Power | Reduced/absent | Reduced/absent |
Tone | Increased | Reduced |
Sensation | Sensory loss | Sensory loss |
Reflexes (plantars) | Increased (upgoing) | Absent/reduced (downgoing) |
Management
High dose corticosteroids (dexamethasone 16mg/day) to relieve peritumoural oedema (with proton pump inhibitor cover); urgent referral to oncology centre.
Spinal cord compression is an emergency and two questions need to be answered urgently:
Does this patient have a reasonable likelihood of having spinal cord compression?
Would this patient benefit from instituting emergency investigation and treatment?
Does this patient have a reasonable likelihood of having spinal cord compression?
Even the most skilled clinician is unable to diagnose spinal cord compression with absolute certainty. Often by the time clinical signs are ‘classic’, it is too late for patients to benefit from treatment, as it has a limited role in reversing symptoms which are already established. Thus if intervention to prevent paraplegia is to be successful, potential compression needs to be diagnosed early.
Having a high index of suspicion in patients with spinal metastases, particularly in patients with breast, lung and prostate cancer and with pain and tenderness on palpation or percussion of the vertebra at the level of the suspected lesion
Taking patients’ complaints about back pain, odd sensations in the legs and difficulties in passing urine seriously
The patient will need to be transferred to a specialized unit where an MRI scan can be carried out and treatment given. In the context of metastatic cancer, radiotherapy is often the most appropriate treatment, but surgery may need to be considered in specific circumstances.
Deciding whether the particular course of treatment is appropriate for a particular patient involves an overall assessment.
Does the patient want emergency management?
Is the patient still walking?
Is the patient suffering from severe back pain?
Does the patient already have established cord compression?
Does the patient have a short prognosis (e.g. week by week deterioration)?
Where suspicion of spinal cord compression is high, it is quickest to telephone the oncological team in the cancer centre where the patient has been managed, who can then coordinate the necessary scan and appropriate emergency treatment (Table 15.2).
| Indications for surgical decompression | Indications for radiotherapy |
|---|---|
• Uncertain cause—to obtain histology • Radiotherapy has not been effective or symptoms persist despite maximum radiotherapy • Radioresistant tumour, e.g. melanoma, sarcoma • Unstable spine • Major structural compression • Cervical cord lesion • Solitary vertebral metastasis | • Radiosensitive tumour • Multiple levels of compression • Unfit for major surgery • Patient choice |
| Indications for surgical decompression | Indications for radiotherapy |
|---|---|
• Uncertain cause—to obtain histology • Radiotherapy has not been effective or symptoms persist despite maximum radiotherapy • Radioresistant tumour, e.g. melanoma, sarcoma • Unstable spine • Major structural compression • Cervical cord lesion • Solitary vertebral metastasis | • Radiosensitive tumour • Multiple levels of compression • Unfit for major surgery • Patient choice |
Patients with spinal cord compression provide great challenges to the multidisciplinary team. These challenges include:
Mobility management (risk of venous thrombosis)
Skin management in a patient confined to bed (risk of pressure sores)
Bowel management
Urinary system management
Psychological management.
There is no consensus on the optimum time to start mobilizing patients diagnosed with cord compression. In general, if the spine is stable and the pain is relatively well controlled it would seem wise to introduce physiotherapy as soon as possible to maintain muscle tone and motor function. The occupational therapist will be crucial in helping with goal-setting and rehabilitative techniques.
Steroids are usually continued at high dose to start with, and then tailed off gradually and completely discontinued after some time (4–6 weeks), or to the lowest dose that maintains stability. Radiation-induced oedema may exacerbate symptoms and the dose of steroids may need to increase temporarily during treatment.
Prognosis
Overall, 30% of patients may survive for one year. A patient who is ambulant after treatment may survive for 8–9 months, but the life expectancy for a patient who remains paraplegic is a few weeks only. Function will be retained in 70% of patients who were ambulant prior to treatment, but will return in only 5% of those who were paraplegic at the outset. Return of motor function is better in those with incomplete spinal compression and particularly so with partial lesions of the cauda equina. Loss of sphincter function is a bad prognostic sign.
In practice, most patients with an established diagnosis are relatively unwell and have multiple metastases, and will be referred for radiotherapy, achieving similar results to those of surgery.
Superior vena cava obstruction
Superior vena cava obstruction (SVCO) may be due to external compression, thrombus formation inside the SVC, or direct invasion of the vessel by malignancy. It is caused most commonly by carcinoma of the bronchus (75%)—particularly small or squamous cell—and lymphomas (15%). Cancers of the breast, colon, oesophagus and testis account for the remaining 10%.
Symptoms and signs
Symptoms are those of venous hypertension, including breathlessness (laryngeal oedema, tracheal or bronchial obstruction/compression), headache or fullness particularly on bending or lying down (cerebral oedema), visual changes, dizziness and swelling of the face, neck and arms.
Signs include engorged conjunctivae, periorbital oedema, non-pulsatile dilated neck veins and dilated collateral veins (anterior chest, forehead and arms). If the latter sign evolves, the symptoms of SVCO may stabilize. Papilloedema is a late feature.
Management
In the palliative care setting, the diagnosis (usually of carcinoma of the bronchus) is already established. SVCO can present acutely, resulting in very distressing symptoms. The patient should be referred to the oncology centre for urgent management.
Dexamethasone 8–16mg p.o. or IV (with proton pump inhibitor cover). There is no good evidence for the efficacy of steroids, but they may be helpful in reducing oedema (if there is associated stridor), or as an anti-tumour agent for lymphoma
Furosemide 40mg p.o. or IV
Consider a prophylactic anticonvulsant as these patients are at risk of seizures
Appropriate analgesia may be necessary for the relief of headache
Supplemental oxygen may be indicated where breathlessness is associated with hypoxia
Treatment is the standard oncology treatment for the particular underlying cancer, e.g.: chemotherapy for small-cell lung cancer (SCLC) and lymphoma; radiotherapy for non-small cell carcinoma of the bronchus
An intraluminal stent can be inserted via the femoral vein and is the treatment of choice for patients with severe symptoms
Thrombolysis may be considered prior to stenting
Prognosis
Without treatment, SVCO can progress over several days leading to death. Prognosis is poor in a patient presenting with advanced SVCO unless the primary cancer is responsive to radiotherapy or chemotherapy. Generally, the prognosis is that of the underlying tumour.
Haemorrhage
Haemorrhage may be directly related to the underlying tumour, or caused by treatments, e.g. steroids or non-steroidal anti-inflammatory drugs (NSAIDs) resulting in gastric/duodenal erosion. A generalized clotting deficiency, seen in thrombocytopenia, hepatic insufficiency or anti-coagulation therapy are also contributory factors in patients with cancer.
Non-acute haemorrhage
Treatments for non-acute haemorrhage include oncological, systemic and local measures. Palliative radiotherapy is very useful for treating superficial tumours and those of the bronchus and genitourinary tract. If radiotherapy is not appropriate, coagulation should be enhanced with oral tranexamic acid 1g t.d.s., but caution is necessary with haematuria since clots may form resulting in further problems such as urinary retention. Local measures for superficial tumours, such as topical tranexamic acid or adrenaline (1:1000) soaks, may be useful. Sucralfate may act as a local astringent to stop stomach mucosal bleeding in addition to a proton pump inhibitor such as lansoprazole.
Acute haemorrhage
Erosion of a major artery can cause acute haemorrhage, which may be a rapidly terminal event. However, it may be possible to anticipate such an occurrence, and appropriate medication and a red blanket to reduce the visual impact should be readily available. Relatives or others who witness such an event will need a great deal of support. If the haemorrhage is not immediately fatal, such as with a haematemesis or bleeding from the rectum, vagina or superficially ulcerated wound, the aim of treatment is local control (if possible) and sedation of a shocked, frightened patient. Rectal or sublingual diazepam (Stesolid)10mg or midazolam 10mg SC or buccally act quickly.
Members of the palliative care team need to balance the anxiety of alerting and preparing the family for such an event against the likelihood of it occurring. If the patient chooses to be looked after at home, the issues of managing acute haemorrhage need to be discussed with the family and the home care team and a clear plan worked out.
It may be appropriate to have emergency medication in the home to sedate the acutely bleeding patient. Such a strategy needs lengthy discussion with the family, carers and the patient’s GP, along with clearly documented plans.
Convulsions (
see Chapter 6J)
Hypercalcaemia ( see Chapter 6i Endocrine and metabolic complications of advanced cancer)
Hypercalcaemia occurs in 10% of patients with cancer. The pathogenesis of hypercalcaemia in malignancy may be related to lytic bone lesions, the production of parathyroid hormone-related peptide (PTHrP) or uncontrolled vitamin D conversion. Calcium is released from bone, and there may also be a decrease in the excretion of urinary calcium. Malignancies most commonly associated with hypercalcaemia include squamous cell carcinoma of the bronchus (and other squamous cell tumours), carcinoma of the breast, prostate and renal tract, multiple myeloma and lymphoma.
A corrected plasma calcium concentration above 2.6mmol/L defines hypercalcaemia. It is often mild and asymptomatic and significant symptoms usually only develop with levels above 3.0mmol/L, but the rate of rise of blood calcium dictates the symptoms and signs. Levels of 4.0mmol/L and above will cause death in a few days if left untreated.
Symptoms include drowsiness, confusion, nausea, vomiting, thirst, polyuria, weakness and constipation.
Management
Treatment is only necessary if there are symptoms of hypercalcaemia and may be unnecessary if the patient is very near to death. Patients should be encouraged to mobilize if appropriate. The absorption of calcium from the gut is generally reduced, so patients may eat what they wish regardless of the calcium content of food.
Hypercalcaemia usually responds to specific antitumour therapy if appropriate.
Fluid replacement
Patients are usually dehydrated and need adequate fluid replacement. A high oral intake of fluid should be encouraged as appropriate for the individual clinical situation. Alternatively, extra fluid should be given intravenously. Fluid replacement alone improves symptoms but rarely achieves total control.
Bisphosphonates
Bisphosphonates inhibit osteoclast activity and thereby inhibit bone resorption. Because of poor alimentary absorption, they are usually given intravenously initially. Both disodium pamidronate or sodium clodronate are effective in 70–80% of patients for an average of two to three weeks. Zoledronic acid can be given over a shorter period and the effect lasts for longer. However, patients receiving bisphosphonates may develop transient fever and bone pains; they may also become hypocalcaemic and need to be monitored.
e.g. zoledronic acid 4mg in 50mL sodium chloride 0.9% over 15 minutes
e.g. disodium pamidronate 60–90mg in sodium chloride 0.9%, 500mL over 2–4h
e.g. sodium clodronate 1.5g in sodium chloride 0.9%, 500mL over 4h
Plasma calcium levels start to decline after 48h and fall progressively for the next six days. Oral bisphosphonates have been reported to delay the recurrence of hypercalcaemia and may be important for maintenance.
The introduction of once- or twice-daily oral bisphosphonates have made compliance easier.
Prognosis
Some 80% of cancer patients with hypercalcaemia survive less than one year.
