Oxford Handbook of Palliative Care (2 edn)
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Introduction
The course of chronic respiratory disease is often marked by slow, inexorable decline with prolonged periods of disabling dyspnoea, reducing exercise tolerance, recurrent hospital admissions and of premature death
Symptoms in the late stages of disease can often be worse than those in patients with advanced lung cancer
Patients also experience loss of dignity, social isolation and psychological problems, with increasing dependency on family and carers. Thus, a holistic approach to management is crucial
Regardless of diagnosis, the needs of the dying patient should be met by palliative care services
Barriers to the provision of excellent holistic end-of-life care include the highly unpredictable disease trajectories of non-malignant respiratory diseases and the failure to appreciate that these disorders are life-threatening
The potential requirement for palliative care in end-stage pulmonary disease
Respiratory disease accounted for 153 168 of 632 062 (24.2%) deaths in the United Kingdom in 1999 (Table 8a.1):
| Condition | Deaths (%) |
|---|---|
All respiratory disease | 153 168 cases (100) |
Pneumonia and TB | 67 591 cases (44.1) |
Cancer | 35 879 cases (23.4) |
Progressive non-malignant causes | 39 939 cases (25.1) |
COPD + asthma | (21.0) |
Pulmonary circulatory disease | (4.1) |
Pneumoconiosis | (0.8) |
Cystic fibrosis | (0.1) |
Sarcoidosis | (0.07) |
Others (congenital, foreign body, etc.) | 9759 cases (6.4) |
| Condition | Deaths (%) |
|---|---|
All respiratory disease | 153 168 cases (100) |
Pneumonia and TB | 67 591 cases (44.1) |
Cancer | 35 879 cases (23.4) |
Progressive non-malignant causes | 39 939 cases (25.1) |
COPD + asthma | (21.0) |
Pulmonary circulatory disease | (4.1) |
Pneumoconiosis | (0.8) |
Cystic fibrosis | (0.1) |
Sarcoidosis | (0.07) |
Others (congenital, foreign body, etc.) | 9759 cases (6.4) |
It is predicted that chronic obstructive pulmonary disease (COPD) will be the third leading cause of death globally by 2020. Research into symptomatology, survival, appropriate care and utilization of services is needed if the needs of this population are to be met.
Terminal symptoms, quality of life, and survival of patients with end-stage pulmonary disease
Symptoms presenting in the final weeks and months of life include dyspnoea, cough, fever, haemoptysis, stridor and chest wall pain—a similar picture to symptoms experienced by patients with lung cancer.
The inability to predict disease trajectory in patients with non-malignant terminal disease makes end-of-life decisions difficult. Studies indicate that quality of life is at least as poor as those suffering from malignant lung disease.
Less than 5% of patients with non-malignant disease die in hospices compared to at least 20% of lung cancer patients. More palliative care services are available to cancer patients.
Dyspnoea can be defined as difficult, uncomfortable or laboured breathing, or, when an individual feels the need for more air. It is the most frequently experienced symptom in those with end-stage respiratory disease and is multifactorial in origin.
Not clearly understood, the mechanism of dyspnoea has been described as a mismatch between central motor activity and incoming afferent information from chemo- and mechanoreceptors. A person’s emotional state, personality and cognitive function also influence its perception.
A good history and examination is invaluable. (
See Chapter 6e.)
This is often a feature in the development of respiratory failure. There may be a bulbar cause, e.g. MND, CVA, or there may be repeated micro-aspiration leading to bronchiectasis.
The right main bronchus is the most direct path to the lungs, leading more commonly to right lower lobe infections. Diagnosis can be made clinically, on CXR or on barium swallow.
Treatment includes:
Nursing in a semi-recumbent position
Speech and language therapy assessment
Thickened foods and fluid
Nasogastric tube
Treatment of the associated pneumonia with antibiotics and physiotherapy
Management of end-stage respiratory disease
The end-stage is not easy to recognize but usually comprises:
Persistent dyspnoea despite maximal therapy
Poor mobility and loss of independence
Increased frequency of hospital admission
Decreased improvements with repeated admission
Expressions of fear, anxiety
Panic attacks
Concerns expressed about dying
Drugs for dyspnoea
Anxiolytics
Anxiety can exacerbate breathlessness. Clinical experience suggests that low-dose anxiolytics (diazepam) can result in improvements despite a lack of evidence.
Antidepressants
Tricyclic antidepressants and serotonin selective re-uptake inhibitors have been shown to be beneficial.
Oral opioids
Site of action may be central (brainstem) or peripheral lung receptors, or they may help by decreasing anxiety. Opioids can cause serious side-effects such as CO2 retention, nausea, drowsiness and respiratory depression, so care is needed
A trial of opioid in COPD patients without CO2 retention is appropriate with close monitoring
Low doses and small increments should be used, e.g. 2.5mg morphine elixir 4 hourly
Subcutaneous diamorphine can be used in patients unable to swallow
In the terminal phase, opioid therapy is justified for the treatment of dyspnoea even in the presence of CO2 retention
Mucolytics
N-Acetylcysteine can be used, as can steam inhalers and nebulized saline.
Palliative oxygen therapy
A significant proportion of patients will have resting hypoxia, although its degree may not correlate with the level of dyspnoea. Symptoms may be improved by oxygen. Even in the absence of hypoxia, oxygen may relieve dyspnoea in COPD patients.
Non-pharmacological measures
General
Vaccinations—influenza and pneumococcal
General nursing care—fan, open windows, regular repositioning, relief of constipation
Good nutrition
Physiotherapy—forced expiratory technique, controlled coughing, chest percussion
Psychological support—aims to improve communication, recognize the impact of anxiety and depression, reduce delays in end-of-life decision-making, provide strategies to relieve symptoms and maximize quality of life
Pulmonary rehabilitation—participants enrol in a programme designed to optimize functional status and reduce symptoms through self-management education and training, psychosocial support and nutritional counselling
Controlled breathing techniques—e.g. pursed lip/slow expiration
Non-invasive mechanical ventilation—shown to decrease the need for intubation
Lung reduction surgery—initial benefit in FEV1, but lasts only 3–4 years
Lung transplantation—emphysema is the most common indication
COPD
Pharmacological treatments
Bronchodilators
Beta-2 agonists, e.g. salbutamol
Anticholinergic agents (may aggravate prostatism or glaucoma), e.g. ipratropium bromide
Inhaled bronchodilators ± spacers should be used where possible as nebulizers deliver medication less efficiently
Inhaled/oral steroids
These benefit 15–20% of patients with stable COPD. As such, a trial with steroids is indicated, where at least a 20% increase in FEV1 would justify their continued use.
Theophylline
The pharmacokinetics of theophylline are unstable and there is a narrow therapeutic range. However, if used judiciously, it may have a place in COPD management.
Oxygen
Has a definite place in the management of selected hypoxic patients
Usually employed overnight, followed by intermittent daytime use through to continuous use
Care needs to be taken where headaches, drowsiness or confusion appear, indicating potential carbon dioxide retention
Long-term oxygen therapy (LTOT)
This can extend life expectancy if administered for 12–15h per day, although there is a lack of evidence to support an increased quality of life.
PaO2 <7.3kPa when breathing air
PaCO2 may be normal or >6.0kPa
Two measurements separated by 4 weeks when clinically stable
Clinical stability = no exacerbations or peripheral oedema for four weeks
FEV1 <1.5L and FVC <2.0L
Non-smokers
PaO2 between 7.3 and 8.0kPa, together with secondary polycythaemia, peripheral oedema or pulmonary hypertension
Nocturnal hypoxia (SaO2 below 90% for >30% of the night)
Interstitial lung disease or pulmonary hypertension where PaO2 <8kPa
Palliation of terminal disease
Interstitial/fibrotic lung disease
These include:
Idiopathic fibrotic disorders, e.g. idiopathic pulmonary fibrosis, autoimmune pulmonary fibrosis
Connective tissue disorders, e.g. systemic lupus erythematosus (SLE), rheumatoid arthritis, scleroderma
Drug-induced diseases, e.g. nitrofurantoin, amiodarone, gold, radiation
Occupational, e.g. silicosis, asbestosis, farmer’s lung
Primary unclassified, e.g. sarcoidosis, amyloidosis, AIDS, adult respiratory distress syndrome (ARDS)
These conditions are, however, rare.
Treatment includes immunosuppressants such as steroids, cyclophosphamide, azathioprine and penicillamine with variable success.
Neuromuscular, restrictive and chest wall diseases
These cause respiratory muscle weakness or loss of compliance in the respiratory cage. Muscular function can be affected at various sites, from the spinal cord to the muscles themselves.
Features that characterize some of these conditions include:
Increased ventilatory drive with inadequate ventilatory response
Sleep disorders
Unbalanced weakness of spinal and thoracic muscles leading to kyphoscoliosis
Bulbar incoordination
Diaphragmatic paralysis
Pulmonary embolism
Supportive treatments
Oxygen
Antibiotics
Physiotherapy
Techniques to clear secretions
Inspiratory muscle training
Beta-2 agonists
Ventilatory support
This can include:
Rocking beds
Abdominal pneumatic belts
Negative pressure body ventilators
Non-invasive positive pressure ventilation
Nasal continuous positive airways pressure
There have been many advances in this field, but many patients still choose to refuse such invasive treatments.
Bronchiectasis
Survival of patients has improved markedly with the advent of antibiotic therapy. Conditions associated with bronchiectasis include:
Cystic fibrosis
HIV infection
Rheumatoid arthritis
Infection, inflammation
Bronchopulmonary sequestration
Allergic bronchopulmonary aspergillosis
Alpha1-antitrypsin deficiency
Congenital cartilage deficiency
Immunodeficiency
Yellow nail syndrome
Bronchial obstruction
Unilateral hyperlucent lung
Diagnosis is usually made by high-resolution CT scanning.
Treatment involves:
Antimicrobial drugs—directed by sputum microbiology, usually treated for longer periods
Bronchodilator therapy
Chest physiotherapy
Nebulized recombinant human deoxyribonuclease
Anti-inflammatory treatment
Supplemental oxygen
Immunoglobulin administration/enzyme replacement
Surgery
Management of haemoptysis
Management of halitosis—e.g. broad-spectrum antibiotics, mouth and gum care
Cystic fibrosis
Affects 1 in 2500 newborns
Marked by alteration in ion and water transport across epithelial cells resulting in recurrent pulmonary infection, bronchiectasis, lung fibrosis and pancreatic insufficiency
Most care takes place in specialized units with home support teams trained in the principles of palliative care
HIV-associated
Pulmonary complications:
Bacterial, e.g. Streptococcus pneumoniae, Pseudomonas aeruginosa
Mycobacterium, e.g. M. tuberculosis, M. avian complex
Fungi, e.g. Pneumocystis jiroveci (formerly known as Pneumocystis carinii), Cryptococcus neoformans
Viruses, e.g. cytomegalovirus
Parasites e.g. Toxoplasma gondii
Malignancies, e.g. Kaposi’s sarcoma, non-Hodgkin’s lymphoma
Interstitial pneumonitis, e.g. lymphocytic pneumonitis
Other, e.g. COPD, pulmonary hypertension
Tuberculosis
Recurrent reactivation results in severe pulmonary scarring, cavitation and secondary aspergillosis infection. If left unchecked, tuberculosis then results in respiratory failure, recurrent bacterial infection and massive haemoptysis.
Chronic bronchitis and emphysema
These conditions cause 80% of cases of pulmonary hypertension. Treatment usually involves:
Oxygen
Non-invasive ventilation
Beta-2 agonists
Diuretics in the management of fluid retention in the acute phase of cor pulmonale
The use of pulmonary vasodilators is of doubtful significance
Obstructive pulmonary hypertension
This is often caused by repetitive, silent pulmonary embolism. Other causes include vasculitis, sickle-cell anaemia and infective endocarditis
Treatment can involve anticoagulation, and occasionally pulmonary thromboendarterectomy or the insertion of an inferior vena caval filter
Primary pulmonary hypertension
Of unknown aetiology
Symptoms can include progressive dyspnoea, decreased exercise tolerance, central chest pain and syncope
Occasionally it is associated with haemoptysis, fluid accumulation and sudden death
Treatment involves oxygen, anticoagulation and vasodilators such as hydralazine and nifedipine
Pulmonary embolism ( see Chapter 6l)
There is an increased incidence of thromboembolism in dependent, hospitalized patients.
The triad of venous stasis, alteration in coagulation and vascular injury are fundamental in the pathogenesis
Should be suspected with symptoms of dyspnoea, pleuritic pain and haemoptysis
40% of high-risk patients with proximal DVTs are asymptomatic when pulmonary embolism occurs
Investigation includes:
Arterial blood gas (not commonly available in palliative inpatient units)
ECG
CXR
Doppler ultrasonography/contrast venography
V/Q scan
Angiography
Enhanced spiral CT scan
Prevention involves adequate hydration, promotion of mobility, the avoidance of venous obstruction, compression stockings and low molecular weight heparin (LMWH).
Treatment usually involves heparinization with LMWH and consideration of warfarinization, or vena-caval filters.
For patients with metastatic malignancy there is increasing evidence that warfarin is not as effective as LMWH. ( See Chapter 6l.)
Pneumothorax and pleural disease
Pathogenesis includes spontaneous and iatrogenic causes. Treatment usually involves intercostal tube drainage if appropriate, or oxygen, analgesia and opiates in the terminally ill.
Causes of pleural effusion are multiple but include infection, cardiac failure, hypoalbuminaemia and renal impairment
Treatment may consist of intermittent aspiration ± chemical pleurodesis
Localized pleural pain may be secondary to rib fracture, infection or pneumothorax. It may respond to normal analgesia, or may require a local anaesthetic intercostal nerve block
Respiratory terminal care and palliative sedation
During the terminal phase, simple measures are important:
Constant draught from fan or open window
Regular sips of water
Sitting upright
In the terminal stages, the emphasis changes from active interventions to supportive and symptomatic measures:
Non-invasive ventilatory support and active physiotherapy may be withdrawn
Drugs for palliating symptoms are often unavoidable
The oral route should be used where possible, but failing this, drugs may be given by the subcutaneous route
The ‘rattle’ associated with loose respiratory secretions, although probably not distressing to the patient, may be addressed by re-positioning, or by the use of hyoscine hydrobromide or glycopyrronium bromide.
As many patients approaching death with end-stage respiratory disease will have uncontrolled dyspnoea, sedation and opioid use should not be withheld because of an inappropriate fear of respiratory depression
Options include benzodiazepines or opioids. The risks and benefits must be carefully considered and the justification for sedation clearly defined. Such decisions are often made by teams rather than individuals, and it is appropriate that patients and families are fully involved in the decision-making process
