Abstract
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Illness Background
PD is a degenerative central nervous system disorder that disproportionately affects the motor system leading to asymmetric muscle rigidity, bradykinesia, and a resting “pill-rolling” tremor. 1 It involves a section of the midbrain called the substantia nigra that leads to a depletion of the neurotransmitter dopamine. Dopamine derivatives (levo-dopa) and agonists are pharmacologic mainstays for the motor symptoms. The illness trajectory varies by Parkinsonian syndrome. Typically PD has a longer prognosis and progresses slower with less nonmotor symptoms and cognitive impairment early in the disease course compared with atypical Parkinsonism that includes a variety of disorders such as multisystems atrophy, corticobasal degeneration, and Lewy body dementia. 2
Impact of Illness
Although the progression of functional impairment and disability is quite variable, typically PD progresses over many years, not months. In general, patients begin to have levo-dopa-related treatment complications such as dyskinesia (irregular jerky movements), psychosis, and dystonia, within five years of diagnosis.3–5 Within 12 years of disease onset, most PD patients have issues with falls, gait disturbance, and balance; within 15–20 years of disease onset, issues with either hallucinations or dementia are common. 6 The symptom burden from the motor and nonmotor symptoms of PD has been shown to be comparable with metastatic cancer.3,4 The slow erosion of functional capacity and the increased dependence on caregivers lead many PD patients to suffer from a diminished sense of personhood and identity. 1 This can lead to significant caregiver distress, financial hardship, and consequently a high utilization of nursing home placement in the last years of life. 7 These factors plus a variable and long prognosis can lead many PD patients to make requests for a hastened death to their clinicians and caregivers. See Fast Facts 156 and 159. Involvement of an interdisciplinary team is often necessary to address the unmet spiritual, psychological, and social needs of PD patients.3,4
Prognosis
With the improvement in disease-modifying therapies such as deep brain stimulators, life expectancy is only modestly decreased compared with age-matched controls and is roughly 6 to 22 years at disease onset.8,9 The long illness prodrome should allow for early advance care planning and appropriate palliative care interventions before late complications. Late-term PD complications are listed hereunder. When encountered, they should prompt clinicians to help patients and families prepare for a peaceful death and should also prompt clinicians to consider hospice care 10
Dementia: approximately 40% of PD patients develop dementia. 11 It is a significant risk factor for nursing home placement and one-year mortality.7,9
Delirium: visual hallucinations and delirium are common in the last years of life. They are also a predictor of nursing home admission. 7
Extrapyramidal symptoms: the presence of muscle rigidity and dyskinesia despite best medical management is associated with an elevated one-year mortality. 9
Dysphagia: this may occur from progression of motor symptoms or dementia. Recurrent hospitalizations and/or aspiration events are common. There is no evidence that feeding tubes improve survival at this stage nor quality of life. Honey-thickened liquids and encouraging a “chin-down” feeding posture are more effective strategies for managing dysphagia in advanced PD. 12
Hospice Considerations
There are no specific hospice criteria for PD nor are there reliable indicators to help clinicians predict a <6 month survival. Medicare claims data suggest that only 54% of PD patients utilize hospice before death, although nursing home residents and patients seen by an outpatient neurologist may be more likely to utilize hospice care. 13 To better identify hospice-eligible PD patients, clinicians should look for a pattern of recurring hospitalizations, dysphagia, and/or progressive dementia. See Fast Facts #125 and #150 for hospice admission guidelines for general neurologic illnesses.
End-of-Life Pharmacologic Considerations
There are no current PD-specific guidelines regarding end-of-life care medication management; however, many experts recommend continuing levo-dopa derivatives and agonists as long as the patient is able to swallow pills. Abrupt discontinuation of these medications can be associated with intense and uncomfortable muscle rigidity. Although levo-dopa-associated symptoms such as dyskinesia may fluctuate dramatically throughout the day and be difficult to observe, patients often prefer these symptoms to the underlying muscle rigidity associated with PD.