FIGURE 22-1 Dementia progression and suggested prioritization of care goals.
Informal carers of people with dementia often suffer from significant levels of distress and burden . They are frequently expected to act as proxies and may have to make difficult and emotionally demanding choices at the end of life, for example, whether or not their relative should be transferred to hospital . These decisions can be a significant source of stress . They may have to act as advocates for their relative or friend, or assist professionals in communicating with the person with dementia, for example, interpreting an individual’s symptoms and signs of pain or other symptoms. Distinguishing between sources of discomfort (e.g., pain or being cold) in severe dementia by integrating the views of a range of caregivers was a key finding and one of the most widely agreed upon recommendations from the European Association for Palliative Care (EAPC) expert consultation (personal communication—van der Steen, 2014). Informal carers who feel competent or who rate themselves as having high levels of self-efficacy tend to cope better and have fewer mental health problems or depressive symptoms . It has also been found that people with dementia die more comfortably when their families accept that this is a life-limiting illness; this association is mediated by better patient–family relationships . Thus, a palliative care approach, which emphasizes the role of families and other friends or unpaid informal carers, may, by providing support and information, improve outcomes for both people with dementia and their carers.
A HOLISTIC APPROACH TO PAIN MANAGEMENT AND PALLIATIVE CARE IN DEMENTIA—PERSONHOOD AND TOTAL PAIN
Person-centered care is a principle philosophy in dementia care. It is a term synonymous with treating people as individuals, respecting their rights as a person, and (from a professional carer’s perspective) building therapeutic relationships . Kitwood  extended the concept of person-centered care to consider personhood and the status of being a person, and developed a definition of personhood: “A standing or status that is bestowed upon one human being by others, in the context of relationship and social being”, which established the concept of person-centered care within relationships with others. People with dementia, particularly those in the advanced stages, are reportedly often denied care that is person centered, and some authors have argued that it is not possible to suffer if we are not sentient . But, the relief of suffering is central to palliative care, and good pain management is an essential part of this. However, as we described earlier in this chapter, in palliative care, we also consider the broader definition—that of “total pain.”
The “total pain” model of suffering highlights how, in all people reaching the end of life, pain is more than a physical and nociceptive experience. First conceptualized by Dame Cicely Saunders, it broadens the definition of pain and includes physical, psychological, social, emotional, and spiritual elements . Psychological distress, including depression and anxiety, is common in people with dementia, even at the advanced stages . Although there has been little research in this area, people with dementia, particularly in the earlier stages, may still experience existential distress—feelings of hopelessness, meaninglessness, disappointment, disrupted personality, or anxiety over dying . Thus, some approaches to palliative care for people with advanced dementia, such as Namaste care (see below), which offers meaningful activities that provide social interaction and physical and spiritual care, may be very therapeutic in addressing aspects of “total pain”.
CAUSES OF PAIN, DISTRESS, AND DISCOMFORT AT THE END OF LIFE OF PEOPLE WITH DEMENTIA
A number of studies from the European Union, the United States, and Israel have reported on the prevalence of physical, psychosocial, and spiritual symptoms at the end of life, enhancing our knowledge on the quality of end-of-life care for people with dementia [18, 28, 44, 50, 55]. From these studies, the most commonly reported clinical care problems at the end of life are eating and swallowing disorders (45.9–85.8% of the studied population), pressure ulcers (14.7–62.3%), urinary incontinence (89.2%), fever due to different types of infection (13.4–52.6%), and the development of pneumonia (10.8–50%).
The commonest reported physical symptoms at the end of life in the population with advanced dementia are pain in 12–76%, shortness of breath in 8–80%, and agitation in about 55%. Physical pain is often due to comorbid diseases such as arthritis, osteoporosis, dental damage, peripheral neuropathy, pressure sores, and shortness of breath (dyspnea) as a result of heart failure, chronic obstructive pulmonary disease, and pneumonia. Psychological symptoms such as behavioral disturbances occur in between 27% and 66%, with fear and anxiety seen in about 33% and 40% of the studied population, respectively. Resistance to care is also frequently reported (up to 42% of patients with advanced dementia) [18, 28, 44, 50, 55]. Spiritual concerns such as the meaning of suffering and life or thoughts such as “I am not yet finished here, I have still things to achieve” are rarely studied in patients with advanced dementia, and spiritual care may be neglected . One recent study found that only 56% of the residents with dementia die peacefully, as perceived by relatives .
Differences in reported symptoms in population-based studies may be due to the use of different measures (dichotomous variables, frequency, intensity) and the use of different time frames (last days, week, or month) to register symptoms. Previous research shows that there is a considerable difference between what is observed by nurses and families on an individual level and what is found at a population-based level where the mean scores on evaluation tools correspond better than the individual scores . In addition, there are regional variations; for example, family reports on comfort in the last week of life in the United States are more favorable compared with Dutch reports  and also indicate a better quality of dying.
CLINICAL ASSESSMENT AND PRACTICAL APPROACH OF PEOPLE DYING WITH DEMENTIA
Undertreatment of pain and other symptoms (shortness of breath and agitation) in patients with advanced dementia during the last week of life is common, as recently shown by Hendriks et al. . However, the recent white paper from the EAPC highlights optimal treatment of symptoms and providing comfort as the top priority for palliative care in dementia, stressing the importance of optimal detection and management of symptoms in patients with dementia .
The first step for adequate symptom management in our population is the detection of discomfort including pain. Moreover, dementia is principally a disease of older people, and holistic care of these patients will have to incorporate challenges such as dealing with disabilities, comorbidities, and age-related problems such as urinary retention, constipation, hearing, and visual impairment. Because of a higher risk of adverse drug reactions and iatrogenic illness in the older population, end-of-life care should always start with a medication review, eliminating those drugs that are no longer necessary and carefully starting those necessary for the comfort of the patient.
No specific evidence-based guidelines are available on the management of pain in patients with dementia at the end of life. Treatment of pain starts with an optimal assessment of pain; this is often complicated by communication problems in patients with advanced dementia. For guidelines concerning assessment of pain in patients with advanced dementia, see Chapter 13.
The basic principles of pain treatment (nonpharmacological and pharmacological) in older patients are also applicable to the older patients with advanced dementia . However, pharmacological treatment is often complicated by swallowing disorders, and so other routes of delivery than oral should be considered. Patches may be used for patients with an expected survival of longer than a few weeks and relatively constant level of pain. However, as it takes a week to obtain a stable plasma level with fentanyl or buprenorphine, treatment with morphine (via a syringe driver) is often the first choice during the last days of life or during a period of unstable pain experience. Because of changes in pharmacokinetics and dynamics, plasma levels of morphine are usually higher in the older patient population, and so the starting dose of morphine should be low; for example, in a naive patient, 2.5 mg every 4–6 hours or 10 mg over 24 hour subcutaneously with 2.5 mg PRN Pro re nata, “as needed” could be considered as a starting dose: We strongly recommend that clinicians follow established guidelines where possible . Pain treatment should be evaluated and adapted at least every 24 hours at the end of life (see also Chapter 18).
Shortness of Breath and Death Rattle
Dyspnea is an objective feeling of difficulty in breathing. It is important to look for possible causes as they can sometimes be actively treated. However, lower respiratory tract infections often occur in patients with advanced dementia and are associated with a high mortality rate (6-month mortality ranging from 36% up to 74%) despite antibiotic treatment , and high levels of discomfort . Therefore, a management focus on symptom relief is vital to ensure quality of life.
Nonpharmacological support is important as severe dyspnea is often frightening and the anxiety induced can exacerbate symptoms. Listening to patients and family, careful explanation, and reassurance can be helpful for the patient. Simple measures such as a fan, a back rest in the bed, and an open window can help. Relaxation, breathing exercises, and help with expectoration of secretions by the physiotherapist are an important aid. The use of oxygen is often controversial; oxygen is only necessary if hypoxia can be demonstrated.
In pharmacological treatment, three classes of drugs may be useful, namely the opioids, corticosteroids, and benzodiazepines. A Cochrane review has shown evidence for the use of oral and parenteral morphine in the relief of dyspnea . Corticosteroids are especially used for their anti-inflammatory effects. Benzodiazepines are important drugs in controlling anxiety aggravating the dyspnea. In the older persons, the use of short-acting benzodiazepines is preferred and may be beneficial. A possible choice is the use of sublingual lorazepam 1–2.5 mg orally or midazolam subcutaneously in low dose (2.5 mg in bolus, followed by 5 or 10 mg over 24 hours) .