Advanced Parkinson’s disease, stage 4 or 5 of the Hoehn and Yahr Scale, is characterized by very limited mobility without assistance, severe motor deficits, risk of falls, and cognitive and psychotic problems. With the advent of L-dopa and other dopaminergic treatments, the progression of PD has become markedly slower; however, over the years treatment loses its efficacy, while a number of complications—such as motor fluctuations and dyskinesia—develop, probably due to the progressive loss of dopaminergic neurons and their striatal and cortical connections. These complications are observed in 50% of patients after 5 years of disease and in 80% of patients after 10 years of treatment (Varanese et al., 2010).
Treatment of the advanced stages of PD is entirely different from earlier stages. Early treatment is geared towards symptom relief and prevention of motor symptoms. During the later stages, the palliative care model is introduced to provide the patient with comfort and support. In the advanced stages, the focus of treatment shifts to treating nonmotor symptoms using a more supportive and palliative approach (Lokk & Delbari, 2012).
Complications in Advanced PD
While worsening of motor function and drug-induced motor complications represents a major challenge in patients with mid-stage to advanced disease, in the advanced stage of PD the most troublesome and distressful complications are usually nonmotor symptoms, including psychiatric and cognitive disorders, autonomic disturbances, and sleep disorders that significantly increase the need for supportive care. Unfortunately, these symptoms are frequently neglected in clinical practice due to limited consultation time, perception of the patient and caregivers that their symptoms are unrelated to the disease, or insufficient awareness of the clinicians, who generally focus on motor symptoms (Varanese et al., 2010).
Proper supporting care becomes increasingly important in advanced PD. Rehabilitative and support services for patients and family become key interventions as the disease reaches its more debilitating stages and pharmacologic or surgical treatment becomes less relevant. Management of motor and nonmotor complications in advanced PD requires careful and ongoing assessment of whether symptoms are a side effect of medication or related to the progression of the disease (Varanese et al., 2010).
The progressive degeneration of the nigrostriatal dopaminergic transmission means fewer and fewer receptors are capable of taking up L-dopa and converting it to dopamine for subsequent storage and release. Unlike early and mid-stage PD, patients with advanced and end-stage PD experience an enhanced sensitivity to small changes in plasma L-dopa levels that narrow the therapeutic window and negatively impact motor function (Varanese et al., 2010).
As the dose requirements of levodopa increase, patients’ functioning is increasingly inhibited in the period before their next dose of medication. This usually functioning deficit takes place 2 to 4 hours after a levodopa dose and may appear as sensory, psychiatric, or autonomic symptoms, or progression of motor symptoms or dystonia. This is called “end of dose wearing off” (Lokk & Delbari, 2012).
“End of Dose Wears Off” Symptoms in Advanced PD
Progression of motor symptoms
On-off fluctuations are sudden unpredictable shifts between an over-treated state (on) and an under-treated state (off). Wearing-off and on-off fluctuations overlap in advanced PD. Wearing-off is generally predictable following the L-dopa administration, with the therapeutic window progressively narrowing over the years (Varanese et al., 2010).
Fragmentation of Dosing
Strategies used to address wearing off and on-off fluctuations include fragmentation of dosing—more frequent administration of lower doses—and use of a COMT inhibitor (entacapone and tolcapone), MAO inhibitor (selegiline and rasagiline), or dopamine agonists. Adjunctive therapy with a COMT inhibitor extends the duration of the L-dopa effect by blocking the COMT enzyme in the peripheral catabolism of L-dopa. Potential adverse events, however, may arise from the COMT inhibitors. Increasing synaptic dopamine levels may also be associated with dyskinesia and increased L-dopa toxicity, leading to worsening of dementia and psychosis (Varanese et al., 2010).
Fragmentation of oral therapy, with L-dopa administered up to 6-7 times a day at about 3-hour intervals is a commonly used and effective strategy. However, lowering individual doses of L-dopa may increase the risk of occasional drug failure or delayed response. Substitution of regular L-dopa with controlled-release L-dopa preparations may be particularly reasonable in end-stage patients, but the available extended-release formulations are not always effective and reliable (Varanese et al., 2010).
Dopamine Agonists Contraindicated
The use of dopamine agonists, although theoretically useful in regulating fluctuations by direct stimulation of the postsynaptic receptors, is generally contraindicated in late-stage disease in order to avoid hallucinations and psychosis, as well as worsening of autonomic dysfunction. The main challenge in controlling the on-off response is to improve the “on” time without increasing the dyskinesia. In very late-stage PD this may be achieved using liquid formulations of L-dopa, which can be prepared by dissolving ten 25/100mg standard-release carbidopa/levodopa tablets and 2g of ascorbic acid in 1 L of tap water (Varanese et al., 2010).
L-dopa and Dietary Proteins
The neutral aromatic amino acids contained in dietary proteins may compete with L-dopa for intestinal absorption and transport across the blood–brain barrier. This limits the efficacy of L-dopa and is responsible for the occurrence of motor fluctuations. Low-protein dietary regimens with protein redistribution by shifting protein intake to the evening are an effective strategy to ameliorate the response to L-dopa. Low-protein products designed for chronic renal failure patients are also a safe, well-tolerated, and useful option for end-stage patients (Varanese et al., 2010).
Maintenance of independent motor function is the primary goal of treatment during the early and later stages of PD. Such a strategy allows the patient to remain independent and mobile for as long as possible and greatly improves quality of life. In end-stage PD the focus of treatment is to make the patient as independent as possible for as long as possible by increasing the time with no dyskinesias and decreasing occurrence of motor and nonmotor “off” times (Lokk & Delbar, 2012).
Dyskinesias in end-stage PD are more frequent and are likely to be a consequence of long-term levodopa therapy. A recent study showed that PD patients treated with levodopa for 4 to 6 years had a 40% likelihood of experiencing dyskinesia. Painful and debilitating dyskinesias are less common today than ten years ago due to more cautious, careful, and individualized anti-PD therapy. Lower doses of levodopa and earlier introduction of other anti-PD agents have contributed to this improvement. However, once dyskinesias occur, lowering of dopaminergic therapy, adding inhibitors of MAO-B or COMT, and adding amantadine may have some effect (Lokk & Delbar, 2012).
Debilitating hypokinesia is one of the most common signs of end-stage PD. Episodes of hypokinesia can occur many times a day and are typically associated with either a failure to respond or to the “off” phase of dopaminergic treatment. Frequent dosing of short-acting levodopa/carbidopa every 3 to 4 hours coupled with COMT inhibitors is currently the best therapy to minimize episodes of hypokinesia. This regimen causes the least variation of levodopa in blood levels, with less off-time, more on-time, and better quality of life (Lokk & Delbar, 2012).
The COMT-inhibitor tolcapone has both central and peripheral effects on the dopaminergic metabolism, in contrast to the COMT-inhibitor entacapone, which only acts peripherally. Tolcapone is particularly indicated in advanced patients where the otherwise most commonly used entacapone is no longer effective (Lokk & Delbar, 2012).
In end-stage patients, dyskinesia may appear in the “off” state as dystonic posture, especially in the lower limbs. Because of the narrow therapeutic window at this stage of the disease, it is not uncommon for patients to experience diphasic dyskinesia—repetitive alternating movements occurring at the beginning as well as at the end of the interval between two L-dopa doses (Varanese et al., 2010).
Controlled-release levodopa may worsen dyskinesias, especially later in the day, due to cumulative effect. Amantadine in doses between 100 mg and 400 mg can be effective, but side effects are frequent in more advanced patients and should be carefully monitored. These include edema, livedo reticularis, and confusional state or hallucinations and psychosis (Varanese et al., 2010).
Clozapine, an atypical dopamine receptor antagonist, has been found to be effective in reducing dyskinesia in advanced patients, and it may be particularly useful when hallucinations are also present. Advanced patients, however, are particularly prone to develop agranulocitosis, with high risks of infections, and thus the white cell count should be regularly monitored. Recent evidence suggests that memantine is also effective in reducing dyskinesia when other options are contraindicated (Varanese et al., 2010).
Despite limited evidence, high-frequency subthalamic DBS (DBS-HFS) has been shown by several reports to be surgically safe and able to produce improvements in dopaminergic drug-sensitive symptoms, and reductions in subsequent drug dose and dyskinesias are well documented. However, the procedure is associated with adverse effects, mainly neurocognitive, with side effects created by spread of stimulation to surrounding structures, depending on the precise location of electrodes.
The occurrence of cognitive complications limits the motor improvements induced by STN-HFS to a short period of time, because patients’ quality of life is greatly impaired by the progressing cognitive disorder. In the late stage of the disease, the number of patients eligible for surgical treatment of PD is extremely low, due to age and general debilitation that significantly increase the risks of short- and long-term complications (Varanese et al., 2010).
The treatment of dystonia varies based upon clinical presentation. Off-state dystonia is generally most troublesome upon awakening in the morning but in advanced disease patients may develop complex twisting dystonic movements during the day. Early morning dystonia, a symptom of overnight wearing off, may respond to nocturnal long-acting dopaminergic agents. In contrast, peak-dose dystonia, which occurs during the day, may respond to reduced dose of dopaminergic medications, given more frequently in smaller doses. Electromyography (EMG)-guided injections of botulinum toxins can be used to treat focal dystonia of a single muscle (Lokk & Delbar, 2012).
Anticholinergics, baclofen, and benzodiazepines are regularly used with caution due to possible cognitive side effects in the end-stage PD patient. The use of botulinum toxin (BT) is increasing in PD patients when treating dystonia, spasms, urinary bladder dysfunction, and drooling. Targeted injections of BT, often guided by EMG, can be tried in these conditions. Botulism toxin only starts having an effect after 3 to 4 days. This effect will gradually increase till about 3 weeks after treatment. There is no permanent effect and the treatment needs to be repeated after 3 to 4 months (Lokk & Delbar, 2012).
Freezing can be the result of either too much or too little dopaminergic effect. “Off freezing” may react to changes in certain medications, while “on freezing” is often associated with end-stage disease and is typically difficult to handle. Nonpharmacologic treatments and tricks can be used in freezing conditions—auditory cueing by counting figures loudly or clapping hands can be tried, as well as visual cues like drawing lines on the floor and using a cane or the light of a laser pointer. These procedures might eliminate or diminish freezing episodes. However, these techniques may be associated with an increased risk of falling, for which PD patients are already at risk, and fall prevention is essential to avoid serious fractures or injuries to the head Lokk & Delbar, 2012).
At the end-stage of PD, nonmotor symptoms can become the most prominent medical problem, leading to increasing decline in quality of life for patient and increasing caregiver burden. Nonmotor symptoms occur in up to 50% of PD patients—especially in association with the medication “off” state—and may be made worse by anti-PD medications. Almost one-third of patients report their nonmotor symptoms to be at least as debilitating as their motor symptoms (Lokk & Delbar, 2012).
All patients with motor fluctuations face at least one nonmotor problem during the “off” phase. In the end-stage of PD, dementia, psychosis, and falls become more complex to manage than the motor complications; as a result, managing nonmotor aspects is important to increase quality of life and decrease the burden of illness. Some studies have noted noticeably high scores among PD patients for impaired taste and smell, impaired swallowing, weight loss, constipation, urinary urgency, forgetfulness, dribbling, sadness, hallucinations, anxiety, sexual dysfunction, falling, reduced concentration, daytime sleepiness, vivid dreams, and sweating (Lokk & Delbar, 2012).
Dopaminergic replacement does not improve cognition and may even worsen it, but cholinergic inhibitors can be helpful. They may not be well tolerated due to peripheral cholinergic adverse effects, and in some cases cholinergic inhibitors can worsen motor functions. Rivastigmine may be the most useful agent, while more controversial is the benefit produced by donepezil. Avoiding the medications that can possibly worsen dementia, like anticholinergics and dopamine agonists, as well as maintaining L-dopa at the lowest effective doses, is a key strategy to contain confusion, hallucinations, and psychosis in advanced patients (Varanese et al., 2010).
Hallucinations, Delusions, Psychosis
Behavioral disorders—especially hallucinations, delusions, and other psychotic symptoms—are frequent in advanced PD, with frequency rates ranging from 25% to 30%. Visual hallucinations, simple or complex in form, are the most common psychotic symptom in advanced PD patients, typically occurring in dim surroundings but often occurring through the entire day in late-stage patients (Varanese et al., 2010).
A range of factors contributes to the development of hallucinations and psychosis in PD, including intrinsic pathology and the effects of dopamine replacement therapy. In the treatment of these complications, the first step should always be to evaluate the role of drugs that can potentially induce or worsen psychosis, such as amantadine, anticholinergics, COMT-inhibitors, and dopamine agonists. These drugs should be tapered off, balancing the effect on psychosis with worsening of motor function (Varanese et al., 2010).
Precipitating events, such as urinary and pulmonary infections, cerebrovascular events, and metabolic dysfunctions, should also be carefully investigated and treated. Even mild metabolic imbalance or infection can profoundly affect the development of psychotic symptoms in those with advanced PD. Decreasing the dose of L-dopa should also be considered when severe psychosis persists, even though this action could worsen parkinsonism (Varanese et al., 2010).
All traditional antipsychotic drugs, such as haloperidol, aripriprazole, and chlorpromazine, should be avoided because of the high sensitivity of PD patients to the adverse motor effects induced through potent antagonisms of dopamine D2 receptors (Varanese et al., 2010).
Clozapine and quetiapine are the only two of the newest antipsychotics that should be considered atypical and thus safe in PD. There is a wealth of evidence demonstrating the efficacy and tolerability of clozapine in PD, but its use is limited by the need for weekly blood testing for the initial 6 months of treatment. Quetiapine is a more practical alternative. Unlike clozapine, quetiapine does not require monitoring of blood cell counts and it is effective in suppressing hallucinations and psychosis in the majority of patients at relatively low doses, ranging from 12.5 mg to 100 mg. Main side effects of quetiapine and clozapine are sedation and postural hypotension (Varanese et al., 2010).
In most cases, psychosis develops late in PD, often due to underlying dementia and as a result of anti-PD medication use. Around 40% of PD patients develop dementia in the late stages of the disease and, in these, psychosis is common. Patients suffering from PD dementia and psychosis are more likely to be placed in a nursing home and are also at an increased mortality risk (Lokk & Delbar, 2012).
The first step for treatment of psychosis is to discontinue or decrease likely offending agents in the hierarchical order of anticholinergics, MAO-B inhibitors, amantadine, dopamine agonists, and, eventually, levodopa. However, there is then a risk of the patient’s having more motor problems. Healthcare providers should resort to atypical antipsychotics as the only remedy in the event psychosis persists despite best efforts to eliminate or decrease anti-PD drugs as being important contributors (Lokk & Delbar, 2012).
Depression and Anxiety
Depression and anxiety occur in up to 40% of all PD patients, possibly higher among end-stage patients with increasing motor complications. Anxiety also tends to be more frequent during “off” periods and often improves when dopaminergic treatment is optimized (Lokk & Delbar, 2012).
In late-stage PD it is essential to identify depression with the assistance of family and other caregivers. In addition to nonpharmacologic treatment, antidepressants are widely used—especially serotonin reuptake inhibitors (SSRIs). Other drugs are less advisable because they carry more risk of cognitive side effects; this is especially true for tricyclic antidepressants. Anxiety is closely related to depression, and it is found that 66% of PD patients with motor fluctuations experience anxiety, often associated with irritability (Lokk & Delbar, 2012).
More activating antidepressants such as selective serotonin reuptake inhibitors (SSRIs) and serotonin-norepinephrine reuptake inhibitors (SNRIs), are useful but significantly limited in advanced patients by the anticholinergic and orthostatic negative effects. SSRIs are also contraindicated in patients receiving selegiline, because of the potential drug-drug interaction leading to serotonin syndrome. S-adenosyl-methionine (SAMe) is reported to have an effective antidepressant effect without worsening of parkinsonism (Varanese et al., 2010).
Anxiety often occurs during “off” periods; it improves with better control of motor symptoms but can be a major source of distress for patients even during the “on” state. Low doses of benzodiazepines are effective when anxiety is persistent and debilitating, but may cause amnesia and confusion in advanced patients and are a risk factor for falls.
Other Medical Issues
Many other issues can arise in the advanced stages of PD. Some are the inevitable exacerbation of problems encountered earlier in the disease process while others emerge in advanced PD as a result of general decline, the long-terms side effects of medications, or medications that no longer work.
Sleep disorders can occur to one degree or another at all stages of PD but by the time the disease progresses to the advanced stage they plague almost all patients. Sleep disorders in advanced PD consist of sleep fragmentation, REM sleep behavior disorders (RBDs), excessive daytime sleepiness, and altered sleep–wake cycle. Sleep fragmentation can be caused by difficulty turning in bed or nocturnal dystonia and can be ameliorated with controlled-release levodopa. Increased nocturnal urinary frequency can also affect sleep and can be controlled by reducing the amount of liquids in the evening when anticholinergic drugs are contraindicated (Valarese et al., 2010).
As mentioned earlier in this course, RBD is a disruption of the normal REM sleep cycle, in which the suppression of movement that normally occurs during REM sleep is incomplete or absent, causing patients to “act out” their dreams, which can be unusually vivid, intense, and violent. Dream behaviors may be complex, including talking, yelling, punching, kicking, jumping from bed, and grabbing, causing great distress for patients and their partners. RBD also prevents physiologic nocturnal restoration of dopamine reserve in cells, with worsening of parkinsonian symptoms. RBD improves when dopaminergic medications are reduced at bedtime. When RBD persists, low doses of clonazepam are effective and should be considered. Modafinil, a wake-promoting agent approved for narcolepsy, is effective without significant side effects in ameliorating daytime sleepiness induced by dopamine agonists and can be helpful in promoting alertness in advanced PD (Varanese et al., 2010).
Orthostatic hypotension is a fall of systolic blood pressure of at least 20 mm Hg or diastolic pressure of at least 10 mm Hg within 3 minutes of standing. Orthostatic intolerance related to orthostatic hypotension results from a reduction of cerebral perfusion when upright and presents in severe cases with lightheadedness or syncope, exposing the patient to high risk of fall. Careful education of patients and caregivers on factors that can trigger the orthostatic symptoms, like avoiding rapid changes of position or straining during micturition or defecation, is essential in the management of orthostatic hypotension (Varanese et al., 2010).
Fluid intake, particularly in the morning, should be maintained at around 2 L of water daily, and at least 8 g of sodium chloride is recommended to ensure adequate hydration. Antihypertensive therapy, when present, should be reconsidered and eventually discontinued. Thromboembolic elastic stocking and abdominal binders can be helpful and should be encouraged. When orthostatic hypotension becomes more severe, it is necessary to start pharmacologic agents such as a plasma volume expander like fludrocortisone, and vasoactive agents such as midodrine (Varanese et al., 2010).
Severe dysphagia occurs frequently at late stage of PD, causing weight loss, malnutrition, dehydration, and significantly increasing the risk of aspiration pneumonia and death. To make swallowing more effective, swallowing maneuvers such as the supraglottic swallow maneuver, the Mendelsohn maneuver, and the effortful swallow maneuver should be taught to patients (Varanese et al., 2010).
Dysphagia for fluid can be controlled by adding thickeners to liquids, which increases their viscosity without substantially modifying taste. Thickeners provide body, increase stability, and improve suspension of added ingredients. Some thickening agents form a gel that can be swallowed by patients, thus significantly reducing the risk of choking. When dysphagia becomes more severe, percutaneous endoscopic gastrostomy (PEG) should be considered. In the advanced phase, PEG can be a useful way to ensure adequate food and fluid intake as well as to provide a mechanism for giving dopaminergic therapy through infusion (Varanese et al., 2010).
Gastrointestinal dysfunction, with erratic gastric emptying worsening over the years, is a common cause of poor absorption of L-dopa in PD. A liquid effervescent levodopa formulation called melevodopa (methyl-ester levodopa) plus carbidopa is a prodrug* with high solubility (about 250 times more than L-dopa) in a small amount of water. It is able to reach the small intestine quickly, where it is absorbed in a more regular and rapid way compared to solid formulations. The drug is approved in certain European countries and currently under phase 2 investigation in the United States (Varanese et al., 2010).
*A prodrug is a medication that is initially administered to the body in an inactive (or less than fully active) form and is converted to its active form through the normal metabolic processes of the body.
Continuous infusion of levodopa/carbidopa gel through portable duodenal systems (duodopa) using PEG can be a practical alternative. The infusion provides constant plasma levodopa concentration and continuous dopamine availability and receptor stimulation. This solution may be particularly reasonable in very advanced patients with severe dysphagia because the PEG may also be used for nutrition (Varanese et al., 2010).
Intrajejunal L-dopa/carbidopa gel infusion is effective in reducing “off” time and severity and duration of dyskinesia in advanced PD. Most important, a recent multicenter study demonstrated that intrajejunal L-dopa/carbidopa infusion provides a beneficial effect on several nonmotor complications, including cardiovascular, gastrointestinal, and urinary symptoms, sleep/fatigue, attention/memory, and pain. An adverse event can occur, however, from the procedure or from the dislocation or occlusion of the intestinal tube. Advanced patients may also experience local complications, particularly inflammation and infections at the site of entry (Varanese et al., 2010).
Apomorphine subcutaneous infusion is also an effective option for patients with severe fluctuations poorly controlled by oral treatment. Apomorphine infusion is often limited by the development of skin reaction at the site of injections after a few years of treatment (Varanese et al., 2010).
Constipation and Urinary Problems
Constipation is a common and early manifestation of PD but in late-stage can become particularly severe due to the combination of anti-PD medications, slowed intestinal motility, immobility, and dehydration. Constipation should be well managed in order to avoid bowel occlusion and to ensure proper absorption of L-dopa and other medications. Dietary supplementation of fibers that stimulate intestinal motility should be encouraged, as well as increased fluid intake. A conservative therapeutic option is administration of macrogol (polyethylene glycol), which can lead to marked improvement (Varanese et al., 2010).
Many late-stage PD patients face urinary problems such as urgency or frequency or stress incontinence, which can cause anxiety and feelings of social isolation. Overactive bladder is the result of loss of normal inhibition by the basal ganglia and the frontal cortex to the sacral spinal cord. Anticholinergics are commonly used to inhibit the overactive bladder, although their use should be discouraged in late-stage patients due to cognitive and other central anticholinergic adverse effects. Newer generation peripheral anticholinergics, like trospium, is better tolerated and can be used sometimes even in advanced patients (Varanese et al., 2010).
Recently, botulinum toxin injections in the detrusor muscle* have demonstrated marked efficacy in reducing urinary frequency with no side effects. Reduced mobility and difficulty toileting often lead to the use of urinary pads or catheters at the end stage of disease, exposing the patients to high risk of dangerous urinary infections when hygienic measures are not appropriate (Varanese et al., 2010).
*The detrusor muscle of the urinary bladder is smooth muscle found in the wall of the bladder. It remains relaxed to allow the bladder to store urine and contracts during urination to release urine.
The incidence of falls in advanced PD is high (40%–70%), even when patients are optimally medicated. Falls occur because of very unstable gait, loss of center of gravity, poor balance, orthostatic hypotension, side effects of medications like antidepressants and benzodiazepines, and disturbances of posture. Falls lead to injuries and fracture that further reduce patient independence and increase the risk of nursing home admission. Patients with previous falls often develop fear of falling, which further limits their mobility, contributing to increased weakness and deterioration (Varanese et al., 2010).
Because of the devastating consequences, an assessment of falls risk should be completed in all advanced PD patients. A combination of both disease-specific and balance- and mobility-related measures is necessary to accurately predict falls in patients with PD (Varanese et al., 2010).
In those with advanced PD, full mobility should be encouraged and maintained as long as possible. Individual rehabilitative therapy sessions should be encouraged 2 to 3 times weekly for 30- to 40-minute durations, even at late-stage, when the patient is able to ambulate safely. Falls are perhaps the greatest concern for late-stage PD patients who are still mobile, and patients should be discouraged from standing or walking without assistance at very late stage of the disease. If patients are bedridden, residual mobility should be maintained through active and passive movement exercises, frequent position changes, and breathing exercises to prevent complications associated with being bedridden, such as decubitus, contracture, pain, and pneumonia (Varanese et al., 2010).
Malnutrition and Dehydration
Malnutrition is a common problem in advanced PD patients. It is caused by difficulty feeding, altered satiety mechanism, diminished gastric and intestinal motility, inactivity, lack of appetite, dysphagia, and metabolic syndrome. In patients still able to eat independently, meal and portion sizes should be monitored in order to provide sufficient nutrition. Any effort, including compensatory strategies, should be considered to delay PEG placement (Varanese et al., 2010).
Adequate hydration is another concern for late-stage PD patients, since even mild temperature change can lead to relative dehydration, exacerbate confusion and orthostatic hypotension, and cause syncope. Many patients become embarrassed when eating or drinking, and nursing assistance can ensure adequate nutrition and hydration through nonjudgmental caregivers who assist patients with the administration of meals (Varanese et al., 2010).
Difficulties with speech (severe dysarthria, hypophonia, tachylalia, freezing of speech) are associated with late-stage PD and can be a significant source of frustration for patients and families. Speech therapy should be encouraged whenever possible. The Lee Silverman Voice treatment has been shown, clinically and scientifically, to be a powerful method of improving speech and related functions such as swallowing and facial expression in PD, with documented improvement in vocal loudness, voice quality, prosody, and speech articulation, sustained at 1-year and 2-year followups. Simplified and codified communications—asking yes/no questions, using alphabet boards or speaking dictionaries—can become the only way of effective communication and should be considered (Varanese et al., 2010).
Palliative care provides comfort and support for people who are facing life-threatening illnesses. In order to maximize quality of life, the palliative care should include a team of medical providers as well as additional caregivers. Such an approach provides traditional medical therapies and emotional and spiritual support while preserving patient autonomy and dignity (Lokk & Delbari, 2012).
As the patient approaches the end-stage of the illness, the main goal of both the patient and the healthcare provider becomes management of motor and nonmotor symptoms according to the principles of palliative care. It is important to take into account that symptomatic control includes the preserving of autonomy as well as stress relief. A holistic approach must be applied from the moment of diagnosis until the end of a patient’s life (Lokk & Delbari, 2012).
Nursing home placement should be delayed as long as possible, because of the well-known risk of reduced survival. As death approaches for late-stage PD patients, it is important to provide them with the best care possible in a passionate environment. Many patients choose to do this through hospice care. Support to families, through social work and psychological counseling, should be offered at this time (Varanese et al., 2010).