Fig. 1.1
Trajectories of dying from lung disease. The rate at which lung diseases progress is highly variable. Sudden death is a feature of catastrophic illnesses such as severe pneumonia. Lung cancer often follows a typical cancer trajectory. Chronic lung diseases such as COPD are characterized by gradual progression with intermittent exacerbations. Many patients with COPD are elderly with progressive frailty. A transplant trajectory is a particular feature of some lung diseases such as cystic fibrosis
Predicting the prognosis of lung disease is often difficult and it is sometimes necessary for the patient and the clinical team to accept uncertainty. The rate at which a disease progresses – the disease trajectory – is often highly variable in chronic lung disease and patients follow their own unique clinical course [17–19]. Sudden death is a feature of catastrophic illnesses such as severe pneumonia or acute lung injury. The end of life phase may be short, often over a few hours, and is likely to be in the setting of an intensive care unit (ICU). In contrast, patients with lung cancer often follow the “cancer trajectory” where there is an initial phase of disease-modifying treatments such as chemotherapy and radiotherapy, followed by a phase of progression of the cancer and declining health, evolving into a clearer palliative phase culminating in end of life care which may be in a hospice or in the patient’s home, for example. However, this traditional model of palliative care often does not fit well with chronic lung disease, where the clinical course is more variable. Patients live their lives with the disability and distress of a chronic illness which cannot be cured and in the knowledge that this is a life-limiting condition. However, a patient’s life span after diagnosis can be decades. Patients with diseases such as COPD, cystic fibrosis, or neuromuscular disease show considerable resilience and fortitude under these circumstances. In most cases, palliative care runs in parallel with disease-modifying treatments, supportive care and measures aimed at improving quality of life. Many chronic lung diseases are characterized by acute exacerbations that can be reversed by acute treatments, which often involve admission to hospital. It is important to appreciate that acute exacerbations usually cause severe symptoms such as breathlessness, cough, pain, and distress which require emergency treatment. Such treatments not only reverse the underlying disease process but are the most effective way of relieving symptoms. Thus, a course of intravenous antibiotics, with oxygen and sputum clearance physiotherapy, is the most effective way of relieving symptoms in a patient with an exacerbation of cystic fibrosis lung disease. Patients may suffer acute complications in the course of chronic lung disease, such as pneumothorax, pneumonia, or major hemoptysis, which cause an acute crisis with severe distress, that can be relieved by prompt diagnosis and expert interventions. Such complications are more likely to occur in advanced-stage disease, and can be very difficult to manage well if an artificial model is followed, whereby palliative care is separated from active disease-modifying care. Such models of care provision are usually inappropriate for patients with progressive lung disease. It is usually better to organize care in such a way that disease-modifying treatments, emergency interventions for any acute crisis, and palliative and supportive measures run in parallel.
Patients with COPD typically have a trajectory of gradual decline punctuated by episodes of acute deterioration and recovery with treatment. The end of life phase is not clearly defined. The patient recovers from all acute exacerbations except the final one, and death can then be somewhat unexpected. This is sometimes referred to as “acute crisis/recovery trajectory.” Many patients with chronic lung disease are elderly and frail with comorbid conditions [16]. They may suffer dwindling health with increasing difficulties in coping at home even with intensive support, such that they may decide to live in a care home, which provides a high level of nursing and supportive care. Such patients may be following a chronic lung disease trajectory, but death may occur from other causes such as a myocardial infarction or stroke [6].
A “transplant trajectory” is a particular feature for some lung diseases such as cystic fibrosis, idiopathic pulmonary fibrosis, primary pulmonary hypertension, or emphysema due to alpha-one-antitrypsin deficiency, for example. The patient is seriously ill, has a high level of symptoms, and may die but is hoping for a rescue lung transplant which can transform the trajectory of the disease dramatically.
Other trajectories may apply to specific aspects of a disease. For example, emotional distress tends to peak at the time of diagnosis of a serious disease, such as lung cancer, at times of deterioration or relapse, and as death approaches. At the time of diagnosis the patient has to cope with the communication of bad news and adjust to living with a serious life-limiting disease. There are elements of grief and loss at this time.
There are substantial limitations to predicting the disease trajectory in patients with lung disease but there may be some advantage in predicting that a patient is likely to die within “months rather than years” or within 6 months, as this may avoid “prognostic paralysis” and may allow access to some additional support services. In some healthcare systems, particular services or financial support are made available to patients who are unlikely to survive more than 6 months. For some diseases, such as lung cancer or progressive idiopathic pulmonary fibrosis, the disease trajectory is generally more predictable, and this facilitates discussion of prognosis and end of life planning. For other patients, such as those with COPD or cystic fibrosis, it is necessary to acknowledge uncertainty. The prognosis and future plans can be discussed in terms of what is likely to happen over a period of time, but precise planning is often unhelpful and plans need to be adaptable to deal with events.
Integrating Palliative Care into Respiratory Care
Palliative care of respiratory disease is highly complex and no one model of care will suit all patients. Different countries organize their palliative care services in different ways but good quality care must be achieved in a variety of settings including the patient’s home, care homes, clinics, emergency departments, specialist respiratory wards, and ICUs. Delivery of care will depend on the patient’s needs, the skills of the clinical team, and the availability of specialist palliative services. It is now widely recognized that palliative care principles apply at all stages of disease and that all doctors and clinical teams should be able to recognize the patient’s needs and to deliver general palliative care, including elements of symptom control, support, communication, and discussion of future care planning. Specialists in palliative medicine have a crucial role in providing education and support to these clinical teams, with collaborative working across traditional boundaries.
There are substantial differences in the use of specialist palliative care services between patients with cancer and patients with other chronic lung diseases [6–8]. Surveys have shown that only a minority of patients dying with chronic lung disease have had input from specialist palliative care services [20]. This could be due to many factors including the clinical course followed by the disease, a failure to refer patients to palliative services, a reluctance of palliative services to be involved with these patients, or a reluctance of the patients to consider palliative care. These are complex issues and the best way of providing palliative care to these patients has not yet been established. Many of these patients have longstanding relationships with a multidisciplinary respiratory team which endeavors to provide holistic care. Patients often value this continuity of care and it is not clear that their needs are best met by a transfer of care from respiratory services to palliative services, especially when disease-modifying therapies, emergency treatments, and palliative care need to run in parallel. This is particularly the case in specialist areas such as cystic fibrosis, fibrotic lung disease, and neuromuscular disease for example. It is often more appropriate to integrate palliative care and specialist respiratory care, with members of the palliative care team working within the respiratory multidisciplinary team. As such patients enter advanced stage disease, particular palliative care skills may be needed in relieving complex symptoms and in addressing end of life issues. Specialist palliative care services may be able to facilitate a patient’s wish to be at home when dying or to give access to hospice care if appropriate. Community-based hospice teams are increasingly able to provide high-intensity palliative care in the patient’s home. In this integrated model of collaborative working between respiratory and palliative care teams, it is easier to manage any acute crises or complications which may arise in advanced lung disease. Integrated palliative care also encourages a focus on symptom management, support, communication, and quality of life issues at an earlier stage in the disease process. Respiratory teams may be highly focused on specific disease-modifying treatments and parameters such as lung function, and traditionally have been less focused on balancing the burden of treatment against the burden of disease, although increasingly quality of life is being assessed as a key outcome measure of any treatment. Respiratory teams may be reluctant to discuss prognosis and future planning in detail. Some studies suggest that patients want to make plans for their future care and would like their clinical teams to start such discussions. Palliative care clinicians can facilitate such discussions. Sometimes a question such as “what worries you most about the future?” can be a useful way of approaching the subject. There may be key events which should trigger these discussions such as an admission to hospital with an exacerbation of COPD, an episode of respiratory failure requiring non-invasive ventilation, or a deterioration in lung function in a progressive respiratory disease. Some patients have particular fears which can be addressed and alleviated. For example, patients who have problems with respiratory secretions may fear that they will choke to death and breathless patients may fear that death will be painful with them struggling to breathe [21]. They can be reassured that such symptoms can be controlled. Palliative care clinicians working within respiratory teams can help to identify patients with additional needs for specialist palliative care input and services.
It is particularly important that palliative care has a high profile in certain areas such as ICU, emergency departments, and acute medicine wards. Because acute exacerbations and complications often occur in advanced lung disease, these patients require access to emergency services. Prompt assessment is required to identify the problem and urgent specific treatment is needed to deal with complications such as infection, hemoptysis, and pneumothorax. In many cases, accurate diagnosis and specific treatment will relieve symptoms and lead to recovery from the acute crisis. However, an acute crisis may be the start of the dying process for these patients. If disease-modifying treatments are failing and the patient is progressively deteriorating, it is important to recognize when the patient is dying and when escalation of treatments such as invasive ventilation may be futile and not in the patient’s best interests. It is crucial that patients, their relatives, and the general public have confidence in the ability of emergency services to provide urgent palliation and that the general public do not develop erroneous concepts of death in hospital being an undignified, painful struggle with high-technology intrusive treatments being applied inappropriately. One of the major successes in recent times has been the restoration of the caring role of clinical teams in managing a dying patient, with the progress in palliative care as a specialty. There is sometimes an inappropriate concept that patients in the palliative stages of a disease should not be admitted to an acute hospital. Acute palliative care is a key component of emergency medicine and these patients often need emergency assessment and management during an acute crisis and hospital is often the best place to achieve this, and should not be denied to the patient. This may be the best way of bringing comfort and control to a patient and family in severe distress because of an acute crisis in the course of a progressive lung disease.
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