This article defines palliative care for swallowing disorders as treatment for severe and chronic dysphagia or intractable aspiration when the recovery of normal swallowing is not anticipated and attempts to restore normal swallowing have been unsuccessful. Palliative treatment for dysphagia is not only for the dying patient because patients with difficulty swallowing can live for a long time. Palliative care for dysphagia is aimed at maximizing swallowing function, maintaining pulmonary health, and supporting healthy nutrition despite the impaired ability to swallow. When despite all attempts at intervention a patient becomes totally unable to swallow, the goal of therapy changes toward finding ways to provide adequate nutrition for the patient.
Dysphagia, or difficulty swallowing, occurs commonly, especially in elderly and debilitated patients. The exact prevalence of dysphagia is unknown, but some reports suggest that the prevalence could be as high as 22% in persons aged more than 50 years. Approximately 10 million people are evaluated annually in the United States for swallowing difficulties. Several studies conclude that between 300,000 and 600,000 individuals in the United States are affected by neurogenic causes of dysphagia each year. Within the hospital setting, many persons experience dysphagia due to general weakness, debilitation, severe pulmonary disease, intubation, or a reduced level of alertness. These numbers clearly indicate a significant burden for treatment teams and patients alike.
This article defines palliative care for swallowing disorders as treatment for severe and chronic dysphagia or intractable aspiration when the recovery of normal swallowing is not anticipated and attempts to restore normal swallowing have been unsuccessful. Palliative treatment for dysphagia is not only for the dying patient because patients with difficulty swallowing can live for a long time. The focus herein is on a variety of common causes for stable or progressive swallowing dysfunction. Palliative care for dysphagia is aimed at maximizing swallowing function, maintaining pulmonary health, and supporting healthy nutrition despite the impaired ability to swallow. When despite all attempts at intervention a patient becomes totally unable to swallow, the goal of therapy changes toward finding ways to provide adequate nutrition for the patient.
Normal swallowing mechanism
The act of swallowing can be divided into oral, pharyngeal, and esophageal phases. The initial oral phase is mainly voluntary and includes putting food or liquid in the mouth, preparing it, and then transporting it to the pharynx. In addition to motor ability, food consistency, taste, hunger, and motivation affect the efficiency of the oral phase. The pharyngeal phase consists of several coordinated actions which transport the food from the oropharynx to the esophagus. This phase can occur involuntarily but may also be modified volitionally via cortical and subcortical input. The esophageal phase is strictly involuntary and partly under autonomic control. This article concentrates solely on oral and pharyngeal, or oropharyngeal, dysphagia.
Standard dysphagia therapy
Before discussing palliative care for dysphagia, the standard approach to helping all patients who are experiencing difficulty swallowing is reviewed. In most cases of oropharyngeal dysphagia, there is an underlying medical or functional cause for the problem which must be determined before appropriate treatment can be pursued. If the underlying medical condition is permanent or progressive, a chronic type of dysphagia may be present. Surgery and medications may lessen the swallowing problem, but behavioral and dietary treatments are often the most effective intervention techniques. Speech-language pathologists are the specialists who are trained to evaluate and treat dysphagia with behavioral techniques.
Compensatory
Interventions that are intended to compensate for the problem are compensatory in nature. Examples of compensatory techniques are diet modifications (eg, eliminate hard, chewy food or thin liquids), postural changes (eg, tuck in the chin when swallowing), alterations in swallowing behavior (eg, swallowing three times to clear the residue before taking the next bite), or external manipulations (eg, taking liquids from a spoon). Most of these techniques are aimed at reducing the bolus size or redirecting the bolus path and protecting the airway to prevent aspiration and improve bolus clearance; however, they are all “immediate fixes” that need to be employed consistently to work. Another type of compensatory treatment is the use of dental appliances in the mouth. These devices are useful to occlude nasopharyngeal defects and assist the tongue with bolus propulsion. Any of these compensatory interventions may be useful in a patient who has chronic severe dysphagia.
Rehabilitative
Rehabilitative interventions are meant to improve the underlying ability of the person to swallow faster, stronger, or in a timelier manner. The long-term goal is to improve the swallow without the need for any intervention. Prime examples of these interventions are exercises for the tongue, larynx, or pharynx. Research has proven the benefit of several of these exercises; however, it takes a compliant cooperative patient with the endurance to undertake the exercises for them to be practical. These interventions also require that the underlying medical condition will not make exercise counterproductive (eg, will lead to fatigue without improvement in strength).
Compensatory/Rehabilitative
Some interventions have immediate positive effects on swallowing efficiency or safety but may also change the long-term ability of the person to swallow better. These interventions are compensatory when first introduced but may be rehabilitative in the long run. Examples are the use of increased sensory stimulation (ice, sour taste, thermal stimulation, electrical stimulation) or the use of swallow maneuvers that require effort on the part of the patient (Mendelsohn maneuver, effortful swallow, or suprasuperglottic swallow). These interventions have been shown to have immediate positive effects on the swallow, but their permanent effect has yet to be tested.
Standard dysphagia therapy
Before discussing palliative care for dysphagia, the standard approach to helping all patients who are experiencing difficulty swallowing is reviewed. In most cases of oropharyngeal dysphagia, there is an underlying medical or functional cause for the problem which must be determined before appropriate treatment can be pursued. If the underlying medical condition is permanent or progressive, a chronic type of dysphagia may be present. Surgery and medications may lessen the swallowing problem, but behavioral and dietary treatments are often the most effective intervention techniques. Speech-language pathologists are the specialists who are trained to evaluate and treat dysphagia with behavioral techniques.
Compensatory
Interventions that are intended to compensate for the problem are compensatory in nature. Examples of compensatory techniques are diet modifications (eg, eliminate hard, chewy food or thin liquids), postural changes (eg, tuck in the chin when swallowing), alterations in swallowing behavior (eg, swallowing three times to clear the residue before taking the next bite), or external manipulations (eg, taking liquids from a spoon). Most of these techniques are aimed at reducing the bolus size or redirecting the bolus path and protecting the airway to prevent aspiration and improve bolus clearance; however, they are all “immediate fixes” that need to be employed consistently to work. Another type of compensatory treatment is the use of dental appliances in the mouth. These devices are useful to occlude nasopharyngeal defects and assist the tongue with bolus propulsion. Any of these compensatory interventions may be useful in a patient who has chronic severe dysphagia.
Rehabilitative
Rehabilitative interventions are meant to improve the underlying ability of the person to swallow faster, stronger, or in a timelier manner. The long-term goal is to improve the swallow without the need for any intervention. Prime examples of these interventions are exercises for the tongue, larynx, or pharynx. Research has proven the benefit of several of these exercises; however, it takes a compliant cooperative patient with the endurance to undertake the exercises for them to be practical. These interventions also require that the underlying medical condition will not make exercise counterproductive (eg, will lead to fatigue without improvement in strength).
Compensatory/Rehabilitative
Some interventions have immediate positive effects on swallowing efficiency or safety but may also change the long-term ability of the person to swallow better. These interventions are compensatory when first introduced but may be rehabilitative in the long run. Examples are the use of increased sensory stimulation (ice, sour taste, thermal stimulation, electrical stimulation) or the use of swallow maneuvers that require effort on the part of the patient (Mendelsohn maneuver, effortful swallow, or suprasuperglottic swallow). These interventions have been shown to have immediate positive effects on the swallow, but their permanent effect has yet to be tested.
Chronic dysphagia after head and neck cancer
A patient with cancer of the head and neck who undergoes extirpative surgery is likely to have impairment of function of nerves and muscles that control swallowing. After the immediate effects of the chemotherapy, radiotherapy, and surgery subside, a chronic dysphagia may become apparent. Patients may be free of cancer, but their quality of life can be significantly reduced. Before these long-term issues are addressed, a brief review of the most common problems is in order.
Oral Cancer
Severe swallowing disorders of the oral tongue may persist following surgical resection of the mobile tongue if grafts or free flaps are adynamic, the anterior tongue is tethered to the floor of the mouth, or the tongue is immobile secondary to hypoglossal nerve resection. Obviously, the person will not be able to masticate adequately or move the bolus out of the mouth. Pauloski and colleagues found that aspiration was not a common occurrence after surgery of the oral tongue unless removal extended deep into the floor of the mouth including the geniohyoid and mylohyoid muscles, affecting the pharyngeal stage of swallowing.
Oropharyngeal Cancer
Resection of tumors of the tonsil and base of tongue affect the pharyngeal phase of swallowing and increase the risk of aspiration. Logemann and colleagues found that patients with resection of the tongue base had difficulty initiating the swallow and slowed pharyngeal transit time. Zuydam and colleagues reported that patients with more than 25% of the tongue base resected experienced great swallowing difficulty, with postoperative aspiration a particular concern.
Laryngeal Cancer
Surgical resection of stage III and IV cancers of the larynx often adversely affects swallowing and airway protection. Tumors of the epiglottis, aryepiglottic folds, and false vocal folds may require a supraglottic laryngectomy. Because these structures are involved in the protection of the airway, their removal increases the risk of aspiration. When the resection needs to be extended into the tongue base or to the arytenoids, aspiration during the swallow is the likely consequence. Supracricoid laryngectomy predisposes patients to swallowing disorders and aspiration.
Radio Therapy
Radiotherapy to the oral, pharyngeal, and laryngeal cavities often causes early difficulty in swallowing due to mucositis, odynophagia, trismus, loss of taste, and alteration in saliva. Although these effects generally subside within a few months after radiotherapy, the late effects of therapy may persist for months or years, leaving a chronic severe dysphagia that may grow worse over time. The toxicity will be worsened with higher doses of radiotherapy or with the addition of chemotherapy. Postradiation edema and radiation-induced fibrosis of the oropharyngeal musculature lead to noncompliance of tissue and immobility of underlying muscles. These changes result in significant difficulty moving the bolus through the pharynx and closing off of the airway, leaving residue within the pharynx and increasing aspiration. Logemann and colleagues reported similar findings in patients treated with various chemoradiation protocols.
Patients with dysphagia following head and neck cancer may be able to execute swallowing maneuvers that keep the bolus out of the airway (eg, the suprasuperglottic swallow), and they may be able to maintain an oral diet if they can clear residue of food with liquids without aspirating. Nevertheless, these maneuvers may be impossible for older, more severe, or more fragile patients. When weight loss and recurrent pneumonias become evident, it may be time to consider alternate treatments.
Chronic dysphagia in progressive neurologic disease
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease of undetermined etiology. Presentation normally occurs during the fifth to eighth decade. Male patients outnumber females 2 to 1. The prevalence is estimated at 2 to 7 cases per 100,000 persons. The disease process involves degeneration of upper (UMN) and lower (LMN) motor neurons within the cortex, brainstem, and spinal cord. Presentation varies depending on the initial site of involvement. Patients with spinal onset (approximately 70%) present with limb symptoms such as muscle atrophy, weakness, spasticity, or stumbling. Approximately 30% of patients present with bulbar onset which initially affects speech, swallowing, and voice. If UMN symptoms predominate, slowed slurred speech is the initial symptom, whereas if LMN involvement is dominant, swallowing difficulties may emerge before speech is noticeably worse. As the disease progresses, both UMN and LMN damage are present.
In general, the incidence of dysphagia in this population tends to be high. Virtually all patients experience swallowing problems at some point in the disease if they live long enough. There is evidence that patients may benefit from swallowing therapy early on in the course of the disease. As the dysphagia becomes severe, aspiration pneumonia becomes a greater threat; however, the most frequent cause of death is ventilatory failure due to palsy of the respiratory muscles; 50% of patients with bulbar onset die within 3 years of diagnosis.
Standard swallowing assessments for ALS patients rely on a clinical, videofluoroscopic, or laryngoscopic procedure to assess the severity of the problem. In the authors’ experience, corroborated by Leder, fiberoptic endoscopic evaluation of swallowing is a patient-friendly assessment procedure that can be done conveniently in the clinic and provides immediate visual biofeedback for the patient and family. Standard therapy for dysphagia in ALS emphasizes compensatory interventions such as postural changes and diet modifications. Swallowing exercises are contraindicated due to excess fatigue and the lack of evidence for any benefit. Compensatory techniques are the mainstay until the patient cannot take anything by mouth without choking and aspirating.
Both assessment and therapy need to take the cognitive status of the ALS patient into consideration, because approximately one third of patients and nearly one half of bulbar onset patients have some degree of frontotemporal lobar dementia (FTLD). These patients may not recognize or self-report their swallowing problems, nor will they monitor their ability to swallow safely. It is important to work closely with the caretakers of ALS patients with dementia to guide them in helping the patient compensate for the specific deficits.
Parkinson’s Disease
Parkinson’s disease is an idiopathic, progressive, neuromuscular disorder that affects about 2 million people in America. The cardinal symptoms of tremor, rigidity, and bradykinesia are due to alterations in neural circuits within the basal ganglia that regulate movement. These changes are correlated with loss of pigmented dopaminergic cells in the pars compacta region of the substantia nigra. The average life expectancy of a patient who has Parkinson’s disease is generally lower than for people who do not have the disease, but the progression of symptoms may take 20 years or more. The reported incidence of swallowing dysfunction in Parkinson’s disease ranges from 30% to 52%, with symptoms correlating with disease severity and duration.
The cardinal symptoms of akinesia, bradykinesia, and rigidity are responsible for the dysphagia in Parkinson’s disease. Some of the salient symptoms are inefficient vertical chewing movements, an open mouth posture with anterior leakage of the bolus, delayed and uncoordinated lingual posterior thrust of the bolus into the pharynx, and delayed triggering of the swallow reflex. Pharyngeal clearance and laryngeal valving may appear weak, and bolus clearance is reduced. Potulska and colleagues quantified several of these problems in a study comparing Parkinson’s disease patients with controls. Interestingly, they reported that dysphagia was observed in all 18 patients studied, even though only 13 complained about it. Subclinical dysphagia may be one of the early symptoms of Parkinson’s disease.
Behavioral management of the dysphagia can prove useful in the early stages of Parkinson’s disease. In addition to compensatory strategies, exercise is recommended and may even retard progression of the swallowing disorder; however, this has not been proven in a controlled study. Later in the course of the disease, exercise is no longer effective, and compensatory diet and postural changes may be of some benefit. In some patients, dysphagia becomes so severe that feeding tubes are the only option to weight loss and aspiration-related complications. Because the disease progresses slowly, many patients and families opt to continue to eat orally until the end. With careful feeding, they may succeed in this strategy. Others opt for supplemental tube feeding but retaining some oral feeding for pleasure. Quality of life is the preeminent concern at this stage.
Most studies enforce the idea that levodopa and deep brain stimulation are usually beneficial for treating limb motor signs associated with this disease; however, there is no equivalent improvement in dysarthria and dysphagia with these same interventions. One exception to this finding is the study by Bushman and colleagues who demonstrated improved swallowing with the use of levodopa in 7 of 20 patients.
The authors’ clinical experience with Parkinson’s disease patients suggests that they do not generally seek out treatment for dysphagia until it is severe. Voice problems are usually more troublesome for the patient. If the voice therapy can include some general tongue, laryngeal, and pharyngeal strengthening exercises, this may benefit swallowing, but this impression needs to be investigated carefully in a controlled study.
The Dementias
Dementia is a pathologic disorder defined by an acquired and progressive deficit in one or more major cognitive functions. The most common of these is Alzheimer’s disease, which according to the Centers for Disease Control affects about 4 million Americans, with 370,000 new cases a year. Other types include vascular, Lewy body, and FTLD. The dysphagia seen across these disorders is similar in many respects; therefore, it is addressed herein with regard to the most common etiology of Alzheimer’s disease.
Aspiration pneumonia has been reported to be the most common cause of mortality in various forms of dementia. In addition to dysphagia, other conditions that predispose to aspiration pneumonia include a reduced level of consciousness, a bed-bound state, dependency for activities of daily living, advanced age, periodontal disease, and the effects of various tubes in the respiratory and gastrointestinal tracts leaving patients vulnerable to indigenous and nosocomial flora. The presence of dysphagia in late-stage dementia was well described by Feinberg and colleagues who evaluated oral and pharyngeal function in 131 institutionalized elderly patients with advanced dementia with videofluoroscopic swallow studies. In fact, objective findings were normal in only nine (7%) patients. Priefer and colleagues discovered that dysphagia may manifest early on in the disease process in patients with dementia. Similarly, patients with early FTLD may manifest swallowing deficits that generally go unrecognized, which may be due to the more obvious aberrant eating behaviors that these patients often display, such as rapid compulsive eating. As the dementia progresses, dysphagia is more apparent, especially when motor problems are a part of the diagnosis, such as in progressive supranuclear palsy.
As is true for other disorders, management of dysphagia in dementia is complicated. Little evidence suggests that aspiration can be prevented by standard management techniques, and the beneficial role of enteral feeding in patients with advanced dementia has not been shown. There is much debate regarding the benefits and risks of long-term enteral feeding in these patients. Patients on enteral nutrition are still at increased risk of pneumonia from aspiration of saliva or gastroesophageal reflux.
Cerebrovascular Disease
Cerebrovascular accidents are devastating occurrences that can have life-altering consequences. There were projected to be 750,000 strokes in 2007, and approximately 4.6 million Americans are currently living post stroke. The morbidity and mortality of cerebrovascular accidents are significant, with a 1-month fatality rate of about 23%. For patients with dysphagia, the mortality rate is even higher, that is, 37% to 42%. After acute stroke, 27% to 50% of patients have dysphagia, including up to 50% of the stroke neurorehabilitation population. Swallowing abnormalities in stroke are variable and may include oral food retention, delayed oral transfer, delayed elicitation of a pharyngeal swallow, decreased hyolaryngeal elevation, and aspiration.
Dysphagia manifestations, severity, and prognosis are greatly affected by the locus of neurologic damage. Several investigators have disputed the notion that only bilateral strokes can lead to dysphagia, although the nature of the problem varies with the site of lesion. In general, anterior and subcortical strokes result in a high rate of dysphagia, whereas brainstem stroke typically causes the most severe dysphagia, especially if the medulla is affected. Patients with the right hemisphere affected may manifest behavioral deficits that reduce their ability to use compensatory strategies taught to them.
Recovery of dysphagia from a cerebrovascular accident varies depending on the area affected. Meng and coworkers found that although 81% of the patients had dysphagia at the time of initial clinical swallowing evaluation, 88% of the patients resumed full oral intake 4 months after the onset of stroke. Generally, if swallowing function is going to recover it will do so within 6 or 7 weeks after a stroke. Brainstem stroke, bilateral hemispheric involvement, or multiple cortical strokes may take longer to recover and may result in a permanent dysphagia. These patients need palliative care when standard treatment fails. Because they have a stable dysphagia and may live many years after the stroke, care is particularly frustrating. Finding food or liquid that they can take orally enhances quality of life immensely. Exercise may help at later stages, although this has not been proven effective in a controlled trial. When a patient who is several years post stroke seeks treatment, the authors tend to put them through an aggressive exercise program for swallowing muscles and then move to compensatory techniques if that fails. Crary and Bryant have reported success in the treatment of chronic dysphagia in brainstem stroke patients using electromyographic biofeedback to help guide swallow exercises.
Multiple Sclerosis
Multiple sclerosis is a chronic, unpredictable, potentially debilitating inflammatory disease of varying severity. The affected sites are the myelin sheaths and the nerves they surround, potentially resulting in deficits in muscle coordination, strength, sensation, and vision. Multiple sclerosis affects approximately 300,000 persons in the United States and over 1 million worldwide, affecting women twice as often as men. Initial presentation is usually between the third and fifth decade, but the relapsing and remitting nature of the process can pose a challenge for diagnosis.
As would be expected in a disease that affects muscle coordination, sensation, and strength, there is a high prevalence of oropharyngeal dysphagia. Over 30% of individuals with multiple sclerosis experience swallowing problems, a higher rate than previously assumed. In addition, Terre-Boliart and colleagues found through videofluoroscopic studies that up to 40% of these patients are silent aspirators.
Standard therapy has emphasized compensatory techniques in the past, but recent evidence suggests that exercise may have beneficial effects in this population. As with other progressive diseases, this intervention is not a permanent remedy, and, eventually, dietary changes and careful feeding techniques are the mainstay of treatment. Complications from pneumonia are a common cause of morbidity and mortality in advanced multiple sclerosis; therefore, conservative and surgical therapies are aimed at preventing aspiration and resultant pneumonia.
As complications develop: surgical options
Recurrent Aspiration
When conservative measures to treat dysphagia and resultant aspiration have failed, surgical management becomes the final option to prevent life-threatening consequences of pneumonia and respiratory compromise. Tracheotomy is the universal treatment for patients with life-threatening aspiration; however, tracheotomy does not necessarily prevent aspiration. On the contrary, there can be an increased aspiration risk due to limitation of laryngeal elevation and anterosuperior excursion during swallowing. Schonhofer and colleagues demonstrated a 30% rate of aspiration in patients who underwent tracheotomy. This concern has come under criticism lately in a series of articles by Leder who showed that, in many patients, there was no difference in their swallow with or without a tracheotomy. Rather, the underlying medical reason for needing the tracheostomy was the major cause of their aspiration. The main advantage of tracheotomy is that it permits more effective pulmonary toilet. Aggressive pulmonary toilet including suctioning must be maintained if tracheotomy is to be effective in preventing the consequences of aspiration.
When considering the multitude of permanent surgical options for aspiration, many factors must be taken into account, including comorbidities, the reason for aspiration, and the overall prognosis. Table 1 lists the most common surgical options for patients with dysphagia with a brief discussion of each procedure.
| Procedure | Benefits | Indications | Contraindications |
|---|---|---|---|
| Tracheoesophageal diversion | Eliminates the larynx as a route of alimentation | When there is good prognosis for recovery of swallowing function | Disease of the upper trachea |
| Laryngotracheal separation | Preferred if tracheotomy is already present | Same as diversion | Same as diversion |
| Laryngeal suspension | Widens hypopharynx and keeps glottis closed during swallowing | Aspiration following supraglottic/hypopharyngectomy | Life-threatening aspiration |
| Glottic closure | Compensates for inadequate glottic closure | Aspiration secondary to vocal cord paralysis/atrophy | Patients without adequate pulmonary reserve |
| Vocal fold medialization | Preserves voice | Aspiration due to unilateral vocal cord paralysis | Laryngeal cancer, bilateral vocal cord paralysis, poor pulmonary status |
| Laryngeal closure | Glottis blocked off, forcing food down lateral pharynx | Inadequate glottic closure | Poor prognosis, as this is a reversible procedure |
| Total/partial cricoid resection | Larger opening into the hypopharynx aids with swallowing and smaller opening to the larynx reduces the risk of aspiration | When recovery of swallowing function is poor | Good prognosis for swallow recovery |
| Laryngectomy | Definitive treatment for aspiration | Reserved for severe neurologic impairment in nonverbal individuals or following resection of head and neck cancers, last resort | Anticipated neurologic/swallowing recovery or T1 and T2 laryngeal tumors |
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