The World Health Organization describes palliative care as “an approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illnesses, through the prevention and relief of suffering by means of early identification, and impeccable assessment and treatment of pain and other problems, physical, psychological or spiritual”.¹

Palliation has been conventionally associated with neoplastic conditions. In recent years, it has gradually become more recognised as an essential component of therapeutic strategies to treat other progressive conditions, including movement disorders.² “Limitations in access to palliative care are primarily due to financial barriers, lack of comprehensive guidelines, and referral patterns of different medical specialties.”²

Regulations, reports and guidelines

In 2000, the ‘Gold Standard Framework’ was established in order to indicate specific regulations in end-of-life care. It prioritises continuity and co-ordination of care, symptom relief and a steadfast commitment to support caregivers. It emphasises the importance of distinguishing the unique needs of each patient, allowing an organised approach to their care.^2,3 Above all, it encourages advanced care planning (ACP) to ensure patient-focused medical care.⁴ ACP involves a statement from the patient, while cognitively competent, expressing their wishes on end-of-life treatment.²

In 2005, a report titled Just Invisible by the Parkinson’s Disease Society of the UK highlights the faults in end-of-life care for patients with Parkinson’s disease (PD). These shortcomings included fragmented facilities and decreases in access to vital services with the progression of the disease. The absence of face-to-face individual management and the insufficient information concerning condition, medication and finances were also deemed issues. 

The focus on age, rather than severity of condition, and above all failure to communicate about future planning were also highlighted in the report.⁵ Following the introduction of the Gold Standard Framework in 2000, palliative care has gone from strength to strength to address these issues, especially in movement disorder care. Several guidelines are currently being developed in order to promote a more patient-centred policy in palliation.²

In the UK in 2004, guidelines entitled Neurological Care Pathway, developed by the Neurological Conditions Policy Group (a subset of the National Council for Palliative Care UK), aimed to promote palliation. It was established following the First National Survey of Palliative Care, Neurology and Rehabilitation Services.² The pathway depicts clinical markers for referral and highlights the theory of rehabilitation in neurological disorders. Such guidelines greatly benefit those suffering from movement disorders.

Management of PD and HD

The management of advanced movement disorders is typically palliative. Palliation in movement disorders such as PD and Huntington’s disease (HD) aims to enhance the quality of life of both patients suffering from these progressive illnesses and their families. It encompasses not only the physical manifestations of the disease, but also the social and emotional welfare of the patient, within their individual social and medical context.⁴

Palliation in movement disorders aims to maximise an interdisciplinary team-based approach to care. Treatment aims to sustain maximal autonomy for patients. 

Palliation in movement disorders encompasses many different disciplines of medicine. It strives to accomplish a multidisciplinary therapeutic strategy encompassing:

• Neuropsychology
• Speech and language therapy
• Occupational therapy
• Nutrition and dietetics
• Physiotherapy
• Social worker
• Respiratory care
• Specialist nurses
• Orthotic technicians and wheelchair services
• Geneticists
• Neurologists.⁶

The demand for various disciplines will vary during the disease progression. The NHS recommends that patients and their caregivers are continuously updated and informed on the professionals and services to contact should the need arise.⁶ To achieve a successful integrated palliative approach, active communication between team members is vital.⁶

HD is an autosomal dominant neurodegenerative movement disorder which progressively develops similar issues as PD with dysphagia and pain. PD is a neurodegenerative movement disorder involving the destruction of dopaminergic neurons. In the end stage of PD and HD, life-prolonging therapies are steadily succeeded by a more palliative approach.⁷

As PD and HD progress into the late stages, a variety of debilitating problems can manifest. As the diseases flourish into the end stage, it is advised that the patient and family frequently update and review the advanced directives regarding “pain management, fatigue, nausea, vomiting, bowel management, urinary tract disorders, artificial hydration, artificial nutrition, dementia, agitation and restlessness”.⁷

Patients with advanced PD and HD typically suffer from dysphagia and sialorrhoea, due to progressive loss in muscle control, rigidity and hypokinesia.² This can result in recurrent respiratory tract infections and extreme weight loss.⁸ Speech and language therapy provides treatment for early-stage PD and HD associated dysphagia.^9,10 As dysphagia progresses a more radical approach may be needed. 

Percutaneous endoscopic gastrostomy (PEG) is an endoscopic medical method, which allows enteral feeding of patients with PD and HD-associated dysphagia via a feeding (PEG) tube.¹¹ PEG is typically indicated in end-stage PD and HD due to an escalated decline in patients’ ability to take food by mouth.¹²

If the patient is aspirating saliva due to sialorrhoea, hyoscine patches are most commonly used to decrease excessive or pooling secretions.¹³

Quality of life

It is difficult to judge whether it would benefit the patient’s quality of life by undergoing an invasive operation in advanced-stage Parkinson’s disease. The low mortality of this procedure seems to have led healthcare workers to proceed with this procedure in an almost complacent manner.¹⁴

Would it improve their quality of life, or just be an added suffering? 

In order to comply with palliative regulations, it is necessary for the healthcare giver to convey all outcomes with the patient, if cognitively competent, and any caregiver or family member involved. Rabenek and McCullough’s algorithm for Ethically Justified, Clinical Comprehensive Guidelines for Percutaneous Endoscopic Gastrostomy Tube Placement provides guidelines for physicians to ensure the clinical ramifications of PEG tube insertion are entirely understood.¹⁵

The algorithm focuses on whether health complications other than dysphagia – for example advanced PD – impact adversely on the patient’s quality of life to such an extent that a PEG tube would offer no benefit. In this scenario, it advises discussion of a trial of PEG or perhaps not to insert it at all. 

Unfortunately, there is no clinical evidence supporting a heightened quality of life in advanced PD following PEG tube placement.²

Pain progression

Pain management is a frequent issue associated with advance stage movement disorders. As HD and PD advance into the late stage, severe pain threatens to diminish the patient’s quality of life.  This disease associated pain arises from one of five causes:

• Musculoskeletal pain
• Nerve pain often manifesting in neck or back arthritis
• Dystonic pain
• Pain associated with supreme restlessness
• ‘Primary’ or ‘central’ pain stemming from the brain.¹⁶

Treatment for PD-associated pain is generally achieved through careful alterations in PD medication agents. Gabapentin and pregabalin have also proven efficacious in improving neuroleptic pain.¹⁷ Apomorphine, distributed through subcutaneous pumps, has also proven efficacious in treating PD-related pain.¹⁸ 

Subcutaneous pumps are advantageous in end-stage PD as it is a safer method than intravenous therapy, which can be controlled easily in a home setting. Rehydration through IV fluids is a simple method to decrease pain levels in advanced movement disorders.¹⁹

The progressive dementia linked with advanced stages of movement disorders such as PD, HD, progressive supranuclear palsy (PSP) and may undermine not only effective communication, but also the appraisal and management of pain.²⁰

It is imperative that the palliative care team, especially the speech and language therapists, enhance communication with caregivers and promote the use of ACP. 

The palliative care team and caregivers alike should be implementing the use of behavioural and distress identification tools such as the Disability Distress Assessment Tool (DisDAT). DisDAT aims to identify distress cues through observing differences in the patient when they are content and when they are distressed.²¹

Dementia concern

Dementia is an issue in many advanced movement disorders. Wandering tends to become a major concern. A MedicAlert is a useful way of monitoring patients in order to preserve their safety.²² The palliative care team should encourage awareness in the neighbourhood and community with regards to the patient’s ability to wander. 

For practical reasons and to lessen the fear to a wandering family member the local Garda station and the fire department should also be informed.⁷ This will not only benefit the authorities but also the sufferer.

Advanced PD patients often experience ‘sun downing’. This is a state of evening agitation associated with dementia. The enhancement of environmental stimuli can play an important role in mediating sun downing. Bright rooms are recommended to prevent misconstruing of visual stimuli. Soft background music promotes a peaceful and calm environment for the patient.⁷

From a caregiver’s perspective, taking responsibility of a patient suffering from a movement disorder can be both incredibly distressing and exhausting. Respite care, whether in-home or out-of-home, is imperative. It is important that caregivers are allowed a few hours for themselves in order to ‘recharge and revitalise’.¹⁹ Occasionally caregivers feel guilty with regards to leaving their loved one in a stranger’s hands. However, the palliative care team should encourage the caregiver to take this free time.¹⁹

Care options

Towards the end of advanced-stage, debilitating movement disorders such as PD, HD and PSP, it may no longer be suitable for the patient to continue living at home and a referral to hospice care should be made.⁷ Hospice aims to provide a non-curative approach to care for movement disorder patients.⁷ As movement disorders progress, fear and worry can envelope the patient and their family. The spiritual aspect of palliation may offer some comfort to them in this tragic time.⁶

Spiritual care is an important dimension of palliation in movement disorders. In 2009, a consensus conference sponsored by the Archstone Foundation of Long Beach, California, took place. Its purpose was to define spiritual care as a cardinal aspect of effective palliation.²³ The conference made five recommendations with regards to spiritual care and palliation:

• Spiritual care should be intrinsic to any empathetic and patient-centred palliative model of care
• Spiritual care models should be centred on honoring the dignity of all people and on providing compassionate care
• Spiritual distress or religious struggle should be treated with the same intent and urgency as therapies for pain or any other medical or social issue
• Spirituality should be considered a patient vital sign. Just as pain is screened routinely, so should spiritual issues be considered an integral part of routine care. Institutional policies for spiritual history and screening must be integrated into intake policies and ongoing assessment of care
• Spiritual care models should be multidisciplinary and clinical settings should have a clinical pastoral education-trained, board-certified chaplain as part of the inter-professional team.²³

The end of a patient’s life is often a gruelling and punishing experience, not only for the individual, but also for the caregiver. In many cases, caregivers have invested their time and energy for years into the support of the patient. The caregiving role in movement disorders has been described as a socially isolating experience.⁷

The patient’s death brings a drastic social upheaval. Often caregivers don’t know how to cope in a world without the responsibilities associated with caring for movement disorder patients. Palliation in movement disorders offers bereavement care to caregivers and family members. Bereavement care aims to aid the individual in redefining their role in life and to help them adapt to the return to society. 

The removal of the burden of care allows a window to open for the experience of grief. The palliative care team can refer the individual to bereavement counselling in order for the individual to make a successful conversion to life without the obligation of care.⁷

Summary

In conclusion, movement disorders have a severely detrimental impact on the lives of countless individuals. The management of advanced movement disorders is typically palliative. Its goal is to enhance the quality of life of both patients suffering from these disorders and their families that support them. 

The palliative care approach encourages the identification of the wishes and needs of patients and their families about care throughout a time of declining health and especially during end-of-life care (Kristjanson et al, 2003). Treatment should be tailored with the goal of sustaining maximal autonomy, quality of life and dignity for the patients. 

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