INTEGRATED PALLIATIVE CARE

Palliative therapy should be offered at all stages of ALS to promote confidence, encourage independence, reduce the burden of physical handicaps, and sustain relationships with family, friends, and colleagues.

The gradual loss of ambulation is a nearly universal feature of progressive motor disease that leads to consultation with physiatry and physical and occupational therapy. These professionals, as well as other members of the interdisciplinary team, will guide the patient in their increasing reliance on assistive devices to maintain independence. In ALS, leg weakness and spasticity are the causes of gait difficulty. Mild to moderately affected patients derive benefit from a cane or walker. Ankle-foot orthoses and other bracing maneuvers may improve balance, preserve energy, promote safety, and avert fatigue that might otherwise preclude the participation of some patients in social activities. When frequent falls occur, a wheelchair may be necessary. Contemporary lightweight chairs are easy to operate and are portable. Self-propelled larger units offer the potential for continued independence even in advanced disease, but they are more expensive and heavier than manually propelled ones.

Communication impairments resulting from dysarthria, anarthria, and dysphonia are challenges for patients with ALS and their healthcare providers. Speech difficulty leads to a sense of isolation, enhances preexisting dysfunctional communication styles, and may limit the ability to communicate basic needs, such as suctioning or repositioning. Consultation with an experienced speech pathologist is essential early in the diagnosis before problems in communication become overtly apparent. Under normal circumstances, speech is possible through the combined action of the lips, tongue, palate, and larynx. Bulbar weakness and spasticity lead to a mixed pattern of dysarthria. Hyperadduction of the vocal cords leads to elevated laryngeal resistance in exhalation and a raspy voice. Flaccid weakness of one or both vocal cords causes a breathy hypernasal voice due to escape of air into the nasal pharynx; in addition, there may be slow strained vocalization with poor pronunciation of consonants.

Management of bulbar symptoms in patients diagnosed with ALS begins with speech, language, and otolaryngologic assessments. It may be helpful to educate patients with bulbar ALS in oromotor exercises for mild impairments and to encourage early intervention for evaluation of augmentative aids. Verbal communication can be prolonged in tracheostomized patients as long as speech is intelligible, in spite of respiratory dependency, by cuffless tubes or intermittent positive pressure ventilation. Computer-assisted aids and electronic communication systems are useful for maintaining communication to family, friends, and the healthcare team and in allowing the patient to actively participate in the decision-making process even late in the illness.

Optimal management of dysphagia and nutritional requirements is important in the psychosocial and physical well-being of patients with ALS and other progressive neuromuscular disorders. Dysphagia precedes ventilatory difficulty in three fourths of patients with ALS and is present in virtually all others late in the illness (16). Normal swallowing requires the coordinated function of structures of the oral cavity, pharynx, larynx, and esophagus. Chewed food moves posteriorly in the oral cavity through constrictor muscles and other pharyngeal spaces to the esophagus where peristaltic movement carries it past the gastric sphincter and into the stomach. Alterations in smell, taste, and fear of aspiration and respiratory weakness can contribute to the occurrence of weight loss even before overt dysphagia is present. Weakness of lip, cheek, lingual, neck muscles, hyperactive pharyngeal gag and cough reflexes, dyspnea, spinal hyperlordosis, and balance difficulty due to axial weakness can all impair the early phase of swallowing; esophageal weakness and dismotility compromise lower esophageal function. The clinical evaluation includes a review of clinical symptoms and signs of dysphagia and inspection of the nasopharynx, larynx, and esophageal paths by fiberoptic and video fluoroscopic studies. Liquids are generally more difficult to swallow than solids. Pooling of liquids and secretions may be found along the vallecula and pyriform sinuses or in the laryngeal vestibule, increasing the likelihood for aspiration.

Even the treatment of mild dysphagia includes dietary counseling, oromotor exercises, and positioning devices for the head and trunk. With bulbar involvement, aspiration can be improved by the management of secretions, abnormal breathing patterns, assisted coughing or chest physical therapy, oropharyngeal suctioning, and percutaneous endoscopic gastrostomy (PEG) placement. Nasogastric tubes are typically not used due to local irritation and an offensive appearance. The PEG is the most used procedure for dysphagia management due to its appeal. The ease of implementation, low risk for individuals with a forced vital capacity of 50% of predicted or greater (16), and minimal anesthesia are factors that contribute to PEG utilization. PEG may prolong survival in ALS (17), particularly before weight loss becomes too great, but its impact on the quality of life is still unknown (18,19). Its routine use runs counter to the view that death due to starvation or malnutrition in ALS is a painless, final, merciful act and is one of many options to be considered for prolongation of life (20-23).

Respiratory symptoms inevitably occur in all patients with ALS, often in association with an ineffective cough, difficulty in clearing secretions, and in the aspiration of fluids or food. An astute clinician will recognize the signs of impending respiratory insufficiency, including agitation, lethargy, orthopnea, poor cough, increased use of accessory muscles, diminution of the volume of speech, and disturbed sleep. Pulmonary consultation can provide helpful information regarding respiratory muscle function to the ALS clinician. Pulmonary muscle function tests are the most reliable and sensitive measures of respiratory strength capacity and life expectancy and are optimally performed every 3 to 6 months. As vital capacity approaches 50% of predicted capacity, noninvasive intermittent positive-pressure aids should be introduced. Some patients decided early in the course of their illness to pursue tracheostomy and are comfortable considering life assisted with ventilation, knowing that they may be unable to move and, at some point, unable to communicate.

The decision to proceed with endotracheal intubation or indwelling tracheostomy should be discussed as openly and supportively as possible with the patient and family members in advance of impending emergencies to remain in compliance with patient preferences for medical decision making. Similarly, documentation with respect to advance directives, healthcare proxy, or, in the case of some states, durable power of attorney, should be completed and placed in the patient's chart and copies distributed to appropriate team members. The optimal situation for home ventilatory support includes adequate financial resources and psychosocial and medical support systems, including proximity to a clinic or hospital for the treatment of complications or emergencies (24). Drooling is a vexing problem in ALS that is associated with oropharyngeal and lower lip muscle weakness, faulty containment and overflow of secretions, but usually not hypersalivation. Early in the disease, patients report a small pool of saliva on the pillow case upon awakening or excessive secretions from the mouth requiring frequent dabbing of facial tissue. Beyond the embarrassment and social isolation it causes, drooling is associated with a heightened risk of aspiration. Medications such as tricyclic antidepressants and atropine-like drugs, with potent anticholinergic effects, reduce salivation by blocking parasympathetic outflow; however, they also have the potential for urinary retention, confusion, and hallucinations.

Care for the mental health of patients with ALS is a steady challenge (25,26). It includes recognition and treatment of clinically significant depression, anxiety, lability of mood, and dementia. The seemingly healthy adjustment to serious illness often includes the denial of depression and anxiety that may be helped through referrals to psychiatry, psychology, social work, or pastoral care and with pharmacotherapy. Observations suggest that treatment for depression has an impact on denial and facilitates adaptive coping mechanisms.

Anxiety disorders and obsessive thought disorders associated with ALS have been less well studied, but experience suggests that they might also be amenable to counseling and antianxiety drugs. Lability of mood, leading to extreme laughter or tearfulness, is especially common among patients with ALS and is probably related to pseudobulbar palsy and frontal lobe release mechanisms. Experience suggests a role for counseling to improve insight and pharmacotherapy for depression. There is increasing awareness of clinical and pathologic syndromes of dementia in association with ALS (26). Mental disturbances in ALS-associated dementia are often minor compared with those with frank Alzheimer's disease and can be easily overlooked by the busy clinician. Cognitive change is subtle and can include variable impairment in reasoning, abstraction, decision-making, goal-directed planning, and organizational ability. For example, these subtle cognitive changes may be seen in the reluctance to introduce formal mechanisms of healthcare planning, such as institution of advance care directives or a healthcare proxy.

Contact with friends and family and the satisfaction with that contact are two important elements in the psychosocial well-being of patients with ALS. Observations have found that the identification of a spiritual frame of reference or worshipping community also serves of benefit. In fact, social contact is central after ambulation ceases, ability to perform activities of daily living is reduced, and hopefulness and interest in the future are lost (9).

Attitudes about terminal or hospice care have varied over time, and such attitudes differ among individual physicians and patients. The term hospice has evolved from its medieval concept as a place of rest for the sick and weary on a long journey. Today's concept is seen more as when medical science cannot add further days to life (expectancy), at least more life will be added to each day (by hospice care). Hospice care offers medical and social services for terminally ill patients and their families, which includes guidance in coping with physical, emotional, spiritual, and psychological distress. This philosophy of care can take place in the home, hospital, or in other dedicated facilities. There has been increasing acceptance of the patient's right to allow a terminal disease to take its course in a hospice setting without treatment to prolong life, with the physician serving the patient's interest (27). Some have advocated legal and ethical standards to protect this right (20,25). Others have found these rights essential to living and leaving a life with dignity.

ACKNOWLEDGMENTS

This chapter is dedicated to the many patients that have received psychosocial care and palliative care from the Eleanor and Lou Gehrig MDA/ALS Center at Columbia-Presbyterian Medical Center (CPMC), New York; to the Muscular Dystrophy Association (MDA), which supports that care; to Dr. Elisabeth K. J. Koenig, The Rev. Dr. John Koenig, and The Rev. William A. Doubleday of General Theological Seminary, New York, who influenced our thoughts on the psychosocial care of patients; and to Lewis P. Rowland, M.D., for his leadership and support .

REFERENCES

An extensive reference list can be found in Motor Disorders, edited by David S. Younger, MD, Lippincott Williams & Wilkins, 1999, pg. 527.