This section addresses issues arising for individuals with progressive ataxia and their carers as a result of the currently incurable nature of these conditions. Little information is available in the literature on palliative or end-of-life care in the ataxias, so in the absence of this, the guidance in this section is drawn from literature pertaining to other progressive degenerative neurological conditions. Although degenerative neurological conditions vary in their presentation and life expectancy, there are parallels in symptoms and the need for services269,270. It seems reasonable therefore to apply evidence from the wider field of progressive neurological conditions to the ataxia population.

What is Palliative Care?

Palliative care is an approach that improves the quality of life of patients and their families facing the problems associated with a life-threatening illness. The aim of palliative care is the prevention and relief of suffering by means of early identification, impeccable assessment and treatment of distressing symptoms and other problems; physical, psychosocial and spiritual271. Although the term ‘palliative care’ is often used to mean ‘end of life care’, the two are not synonymous. Palliative care is often applicable earlier in the course of an illness, in conjunction with other therapies that are intended to prolong life. In this context, palliative care will enhance the patient’s quality of life and support the patient to live as actively as possible with the condition. End of Life care refers to the care that patients need in the last phase of their illness, usually in the months or weeks before death.

Palliative care is a generic term which describes an approach to care that is the responsibility of all health care professionals. Most palliative care for patients with progressive ataxias is provided in the community by district nurses, GPs and other members of the primary care team. However, some patients with complex needs require help from palliative care specialists. Patients with specialist palliative care needs can thus be supported by doctors and nurses at home, during hospital admissions and within a day hospice or inpatient hospice environment.

The importance of palliative care for patients with progressive< neurological disease was highlighted in the UK National Service Framework for long term neurological conditions25. It is estimated that 300,000 people living with neurological conditions in the UK do not receive the specialist palliative care input that they need272. The UK National Service Framework for long term neurological conditions promotes the need for coordination between neurologists, rehabilitation specialists and palliative care services in order to ensure patients receive the best possible care273.

Advance Care planning for patients with ataxia

Advance Care Planning (ACP) is defined as “A voluntary process of discussion and review to help an individual who has capacity to anticipate how their condition may affect them in the future and, if they wish, set on record: choices about their future care and treatment and/ or an advance decision to refuse treatment in specific circumstances, so that these can be referred to by those responsible for their care or treatment (whether professional staff or family carers) in the event that they lose capacity to decide once their illness progresses274.

ACP may involve an individual with ataxia (who has mental capacity) writing a legally binding document detailing an Advance Decision to Refuse Treatment (ADRT). This decision only comes into force should the person subsequently come to lack capacity. Other outcomes of ACP may be the appointment of a Lasting Power of Attorney, a documented decision not to attempt cardiopulmonary resuscitation (DNAR) or a written Preferred Priorities of Care document. It is worth noting that patients cannot request specific treatment as part of an ACP, only refuse certain treatments (such as artificial feeding or resuscitation).

ACP allows a person with a progressive ataxia to retain control over their future treatment and management. Talking and planning ahead can provide comfort and reassurance. However, not all individuals with ataxia are able to engage with advance care planning. Some patients will want only information that is required to live one day at a time and will not want to be part of discussions that focus on future deterioration and further loss of function. These people may find attempts by professionals to engage them in ACP conversations unwanted and distressing. Effective communication, carried out with compassion and sensitivity, is therefore fundamental to the process of providing good quality person centred care.

Only a person with capacity who chooses to do so can take part in advance care planning. Should an individual with capacity wish to record choices about their care and treatment, or an advance decision to refuse treatment, in advance of losing capacity, they should be guided by a professional with appropriate knowledge and this should be thoroughly documented. Patients should be encouraged to review regularly any care planning documentation, to update this as appropriate, and to ensure that revisions are shared with those they wish to involve in their care. Advance care plans should be recorded on an electronic end of life system (if available) such as ‘Coordinate my care’. Where a person lacks capacity to decide, care planning must focus on determining their best interests and making decisions to protect these275.

End of Life Care

The progressive ataxias often have a disease course spanning several decades. It can be hard to know when the end of life is approaching. In other conditions, identifying the patient’s last year of life is important in order to begin advance care planning. However, with some of the progressive ataxias, it may be necessary to engage in advance care planning some time before the individual is in the last year of life; before poor quality speech and other communication barriers intervene. Whether or not ACP has taken place before, identifying the end stage of the illness is still essential to ensure individuals and their families are provided with the most appropriate support.

Accurately predicting prognosis is virtually impossible in most advanced diseases, so clinicians can use certain triggers to identify a person approaching the end of life. One such trigger is the ‘surprise question’: “Would you be surprised if this patient died in the next 12 months?” 276. In addition to the intuitive surprise question, clinicians should consider general and specific clinical prognostic indicators for patients with ataxia:

General indicators (common across different diseases):

  • progressive physical decline
  • increasing need for support
  • progressive weight loss
  • repeated/ unplanned crisis admissions

Indicators specific to ataxia:

  • complex symptoms which are difficult to control
  • swallowing problems (e.g. choking with meals) leading to recurrent aspiration pneumonia, sepsis, breathlessness or respiratory failure
  • Speech problems; increasing difficulty in communication plus progressive dysphasia, dysarthria and fatigue.
  • Congestive Heart Failure New York Heart Association classification class 3 or 4. (Shortness of breath on minimal exertion or at rest.)

Answering ‘no’ to the surprise question and identifying general or disease specific clinical indicators of decline should alert the multi-professional team that an individual with ataxia is approaching the end of life.

While many patients, families and professionals do not want to talk about death and dying, discussion enables the best possible end of life care tailored to the individual. In recent years the concept of a ‘good death’ has emerged277. A ‘good death’ usually means:

  • Being treated as an individual, with dignity and respect
  • Being without pain and other symptoms
  • Being in familiar surroundings and
  • Being in the company of close family and/or friends.

Provision of high quality end of life care includes attention to the patient’s preferred place of care (home, hospital or hospice) and the provision of spiritual and pastoral support. As the person enters the dying phase, the focus of treatment shifts and symptom control and comfort take precedence over life prolonging treatment such as enteral feeding. The five priorities of care defined by the ‘Leadership Alliance for the care of dying people’ must be followed at this phase, and an individualised plan of care agreed with the patient and/or family in the final days/hours of life278.

Recommendations Grade
1. All healthcare professionals should ensure patients are aware that Advance Care Planning is an option and advise patients consider doing it. GPP
2. All healthcare professionals should facilitate advance care planning and documentation of advance care directives in individuals with ataxia. GPP
3. Documented Advanced Care Planning for individuals with ataxia should be regularly reviewed by the individual in conjunction with their treating clinicians. Review may be instigated by the individual or care provider, can be part of regular review or may be triggered by a change in circumstances. GPP
4. Consider referring to a specialist palliative care team when an individual with ataxia has complex distressing symptoms, psychological, social or spiritual needs, and/or a need for end-of-life planning. GPP
5. Ensure an individual identified as approaching the end-of-life stage and their family have open access to specialist palliative care services. GPP
6. If needed offer specialist input in the last few days of life, and aftercare and bereavement support to their families. GPP

This information is taken from Management of the ataxias - towards best clinical practice third edition, July 2016. This document aims to provide recommendations for healthcare professionals on the diagnosis and management of people with progressive ataxia. To view the full document, including references, click here.