As respiratory function starts to deteriorate in those with amyotrophic lateral sclerosis, one of the principal questions that has to be answered is whether it is appropriate to provide ventilatory support. Although expensive, it is perfectly feasible to provide this at home, and this article examines many of the issues surrounding home mechanical ventilation.
Braun SRRespiratory system in amyotrophic lateral sclerosis. Neurol Clinics1987: 5: 9-31.
2.
Munsat TLAdult motor neuron diseases. In: Rowland LP ed. Merritt's textbook of neurology, eighth edition. Philadelphia: Lea and Febiger, 1989 : 682-7.
3.
Munsat TL, Andres PL, Finison L., Conlon T., Thibodeau L.The natural history of motor neuron loss in amyotrophic lateral sclerosis . Neurology1988: 38: 409-13.
4.
Rosen MJRespiratory Failure in Amyotrophic Lateral Sclerosis. In: Caroscio JT ed. Amyotrophic lateral sclerosis: a guide to patient care. New York: Thieme, 1986: 45-59.
5.
Cohen CA, Zagelbaum G., Gross D., Roussos C., Macklem PTClinical manifestations of inspiratory muscle fatigue. Am J Med1982: 73: 308-16. 6 Fallat RJ, Jewitt B., Bass M. et al. Spirometry in amyotrophic lateral sclerosis. Arch Neurol1979: 36: 74-80.
6.
Fallat RJ, Norris FH, Holden D., Kandal K., Roggero PCRespiratory monitoring and treatment: objective treatments using non-invasive measurements. Adv Exp Med Biol1987; 209: 191-200.
7.
Griggs RC, Donohue KM, Utell MJ, Goldblatt D., Moxley R.Evaluation of pulmonary function in neuromuscular disease. Arch Neurol1981; 38: 9-12.
8.
Hyatt RE, Black LFMaximal static respiratory pressures in generalized neuromuscular disease . Am Rev Respir Dis1971; 103: 641-50.
9.
Miller A.Pulmonary function and respiratory failure in neuromuscular disorders with reference to amyotrophic lateral sclerosis. In: Caroscio JT ed. Amyotrophic lateral sclerosis: a guide to patient care. New York: Thieme, 1986; 61-77.
10.
Mulder DW, Howard Jr Fmj.Patient resistance and prognosis in amyotrophic lateral sclerosis. Mayo Clin Proc1976; 51: 537-41.
11.
Caroscio JTAmyotrophic lateral sclerosis: the disease. In: Caroscio JT ed. Amyotrophic lateral sclerosis: a guide to patient care. New York: Thieme, 1986:2-15.
12.
Moss AH, Casey P., Stocking CB, Roos RP, Brooks BR, Siegler M.Home Ventilation for amyotrophic lateral sclerosis patients: outcomes, costs, and patient, family and physician attitudes. Neurology1993; 43: 438-43.
13.
Bach JRMechanical exsufflation, noninvasive ventilation, and new strategies for pulmonary rehabilitation and sleep disordered breathing. Bull NYAcad Med1992; 68: 321-40.
14.
O'Donohue WJ Jr. Prescribing home oxygen therapy. Arch Intern Med1992; 152: 746-8.
15.
Smith RAOn behalf of the patient. Adv Exp Med Biol1987; 209: 319-22.
16.
Smith RA, Norris FH Jr.Symptomatic care of patients with amyotrophic lateral sclerosis . JAMA1975; 234: 715-17.
17.
Preston W., Donohue KM, Eyck Lgt, Goldblatt D.Managing amyotrophic lateral sclerosis manual III: managing breathing problems . Sherman Oaks, Ca: The ALS Association , 1986.
18.
Norris FH, Smith RA, Denys EHMotor neurone disease: towards better care. Br Med J1985; 291:259-62.
19.
Norris FH, Holden D., Kandal K., Stanley E.Home nursing care by families for severely paralyzed ALS patients. Adv Exp Med Biol1987; 209: 231-8.
20.
Prehospital do-not-resuscitate (DNR) form. HS005/EMS. Los Angeles, Ca: Department of Health Services, Emergency Medical Services Agency, 1992.
21.
Wilson WC, Smedira NG, Fink C., McDowel JA, Luce JMOrdering and administration of sedatives and analgesics during the withholding and withdrawal of life support from critically ill patients. JAMA1992; 267: 949-53.
22.
Steffen GE, Franklin C.Who speaks for the patient with the locked-in syndrome? Hastings Cent Rep1985; 15: 13-15.
23.
Hayashi H., Kato S.Total manifestations of amyotrophic lateral sclerosis. ALS in the totally locked-in state. J Neurol Sci1989; 93: 19-35.
24.
Tobin MJWeaning from mechanical Ventilation. In: Simmons DH ed. Current Pulmonology. Chicago: Year Book, 1990; 47-105.
25.
Caplan AL, Callahan D., Haas J.Ethics and policy: issues in rehabilitation medicine. Hastings Cent Rep1987; 17: 1-20.
26.
Braun SR, Sufit RL, Giovannoni R., O'Connor M., Peters H.Intermittent negative pressure ventilation in the treatment of respiratory failure in progressive neuromuscular disease. Neurology1987; 37: 1874-5.
27.
Alba A., Pilkington LA, Kaplan E. et al. Long term pulmonary care in amyotrophic lateral sclerosis. Respir Ther1976; 11/12: 49-105.
28.
Bach JR, Alba A., Mosher R., Delaubier A.Intermittent positive pressure ventilation via nasal access in the management of respiratory insufficiency. Chest1987; 92: 168-170.
29.
Bach JR, Alba AS, Saporito LRIntermittent positive pressure ventilation via mouth as an alternative to tracheostomy for 257 ventilator users. Chest1993; 103: 174-82.
30.
Ellis ER, Bye Ptp, Bruderer JW, Sullivan CETreatment of respiratory failure during sleep in patients with neuromuscular disease, positive pressure ventilation through a nose mask. Am Rev Respir Dis1987; 135: 148-152.
31.
Leger P., Bedicam JM, Cornette A. et al. Long term follow up of severe chronic respiratory insufficiency patients (n = 373) treated by home nocturnal non invasive nasal IPPV. Amer Rev Respir Dis1991; 143: A73.
32.
Sawicka EH, Loh L., Branthwaite MADomiciliary ventilatory support: an analysis of outcome. Thorax1988; 43: 31-5.
Bach JR, Alba ASTracheostomy ventilation: a study of efficacy with deflated cuffs and cuffless tubes. Chest1990;97:679-83.
35.
Stauffer JL, Silverstri RCUpdate: Complications of endotracheal intubation, tracheostomy, and artificial airways. In: Pearson DJ ed. Respiratory intensive care. Dallas: Daedalus Enterprises, 1986: 167-68.
36.
Smillie JGCan physicians manage the quality and costs of health care? The story of the Permanente Medical Group. New York: McGraw-Hill, 1991.
37.
Fischer DALong-term management of the ventilator-dependent patient: levels of disability and resocialization. Eur Respir J1989; 2: 651s-54s.
38.
Make BJ, Gilmartin MEMechanical ventilation in the home. Crit Care Clin1990; 6: 785-96.
39.
O'Donohue Jr WJ, Giovannoni RM, Goldberg AI et al. Long-term mechanical ventilation: guidelines for management in the home and at alternate community sites. Chest1986; 90: 1S-37S.
40.
Robert D., Gerard M., Leger P. et al. Long-term IPPV at home of patients with end stage chronic respiratory insufficiency. Chest1982; 82: 258-9.
41.
Plummer AL, O'Donohue WJ, Petty TL. Consensus conference on problems in home mechanical ventilation. Am Rev Respir Dis1989; 140: 555-60.
42.
Splaingard ML, Frates RJ, Jefferson LS, Rosen CL, Harrison GMHome negative pressure ventilation: report of 20 years of experience in patients with neuromuscular disease. Arch Phys Med Rehabil1985; 66: 239-42.
Goldberg AIMechanical ventilation and respiratory care in the home in the 1990's: some personal observations. Respir Care1990; 35: 247-59.
46.
Salamand J., Robert D., Leger P., Langevin B., Barraud J.Definitive mechanical ventilation via tracheostomy in end stage amyotrophic lateral sclerosis. 3rd International Conference on Pulmonary Rehabilitation and Home Ventilation. Denver, Colorado: National Jewish Center for Immunology and Respiratory Medicine, 1991: 50.
47.
Goldberg AI , Alba AS, Oppenheimer EA, Roberts E.Caring for mechanically ventilated patients at home. Chest1990; 98: 1543.
48.
Silverstein MD, CB Stocking, JP Antel , J Beckwith, RP Roos, M. Siegler.Amyotrophic lateral sclerosis and life-sustaining therapy: Patient's desires for information, participation in decision making, and life-sustaining therapy. Mayo Clin Proc1991; 66: 906-13.
49.
Carey JSMotor neurone disease: a challenge to medical ethics: discussion paper. JR Soc Med1986; 79: 216-20.
50.
Carus R.Motor neurone disease: a demeaning illness. BMJ1980; 280: 455-6.
51.
Malcolm AHThis far and no more, a true story. New York: Times Books, Random House, 1987.
52.
Herr SS, Bostrom BA, Barton RSNo place to go: refusal of life-sustaining treatment by competent persons with physical disabilities. Issues Law Med1992; 8: 3-36.
53.
Mulder DWThe diagnosis and treatment of amyotrophic lateral sclerosis. Boston: Houghton Mifflin, 1980.
54.
Tandan R., Bradley WGAmyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management. Ann Neurol1985; 18: 271-80.
55.
White M., Gribbin J.Stephen Hawking: a life in science. New York: Dutton, 1992.
56.
Rabin R.Six parts love: a family's battle with Lou Gehrig's disease (ALS). New York: Charles Scribner's Sons, 1985.
57.
Chenevert BJTaking hopelessness out of helplessness, second edition. Southfield, Mi: MetroStaff Home Health Care, 1989.
58.
Hamilton L.Why didn't somebody tell me about these things?Shawnee Mission, KS: Inter-Collegiate Press, 1984.
59.
Tsieprati I.It's Your Choice. (videotape). Van Nuys, Ca: Valona Productions, 1992.
60.
Tsieprati I.Caring for the caregiver. (videotape)Van Nuys, Ca: Valona Productions, 1993.
Colbert AP, Schock NCRespirator use in progressive neuromuscular diseases. Arch Phys Med Rehabil.1985 ; 66: 760-2.
63.
Collopy B., Dubler N., Zuckerman C.The ethics of home care: autonomy and accommodation. Hastings Cent Rep1990; 20: 1-16.
64.
Gilgoff IS, Prentice WS, Baydur A.Patient and family participation in the management of respiratory failure in duchenne's muscular dystrophy. Chest1989 ; 95: 519-24.
65.
Nelson JLTaking families seriously. Hastings Cent Rep1992; 22: 6-12.
66.
Callahan D.Families as caregivers: the limits of morality . Arch Phys Med Rehab1988; 69: 323-8.
67.
American Thoracic Society Bioethics Task Force.Withholding and withdrawing life sustaining therapy . Ann Int Med1991; 115: 478-85.
68.
Bone RC, Rackow EC, Weg JG, Panel ACEthical and moral guidelines for the initiation, continuation, and withdrawal of intensive care. Chest1990; 97: 949-58.
69.
Jecker NSThe role of intimate others in medical decision making. Gerontologist1990; 30: 65-71.
70.
Sharp JW, Sivak EDThe role of social work in contemporary home care planning. Cleve Clin Q1985; 52: 355-8.
71.
Thomas VM, Ellison K., Howell EV, Winters K.Caring for the person receiving ventilatory support at home: care givers' needs and involvement. Heart Lung1992; 21: 180-6.
72.
Clark K.Psychosocial aspects of prolonged ventilator dependency. Respir Care1986; 31: 329-33.
73.
Gale J., O'Shanick GJ. Psychiatric aspects of respirator treatment and pulmonary intensive care. Adv Psychosom Med1985; 14: 93-108.
74.
Frye B., Hilton T.Preparing the caregiver to manage the ventilator-dependent patient at home . Rehabil Nursing1988; 13: 38-9.
75.
Goldstein RS , Avendano MALong-term mechanical ventilation as elective therapy: clinical status and future prospects. Respir Care1991; 36: 297-304.
76.
Bach J., Gombas G.Quality of life perceptions of ventilator-assisted individuals. IVUN News1990; 4: 1.
77.
Oppenheimer EA , Baldwin-Myers A., Tanquary P.Ventilator use by patients with amyotrophic lateral sclerosis. 3rd International Conference on Pulmonary Rehabilitation and Home Ventilation. Denver, Colorado: National JewishCenter for Immunology and Respiratory Medicine, 1991: 49.
78.
Bach JR, Intinola P., Alba AS, Holland IEThe ventilator-assisted individual: cost analysis of institutionalized vs rehabilitation and in-home management. Chest1992; 101: 26-30.
79.
Wagner DREconomics of prolonged mechanical ventilation. Am Rev Respir Dis1989; 140: S14-S18.
80.
Indihar FJCost comparison of care for chronic ventilator patients [letter]. Chest1991; 99: 260.
81.
Motwani JK, Herring GMHome care for ventilator-dependent persons: a cost-effective, humane public policy. Health and Social Work1988; 13: 20-4.
82.
Rosen RL, Bone RCEconomics of mechanical ventilation. Clin Chest Med1988; 9: 163-9.
83.
Eddy DMClinical decision making: from theory to practice. Connecting value and costs. Whom do we ask, and what do we ask them?JAMA1990; 264: 1737-9.
84.
Eddy DMWhat care is 'essential'? What services are 'basic'?JAMA1991; 265: 786-88.
85.
Eddy DMClinical decision making: from theory to practice. The individual vs society. Is there a conflict?JAMA1991; 265: 1449-50.
86.
Eddy DMClinical decision making: from theory to practice. The individual vs society. Resolving the conflict. JAMA1991; 265: 2399-401.
87.
Marino WD, Kvetan V.Home positive pressure ventilatory support. In: Rothkopf MM, Askanazi J eds. Intensive Homecare. Baltimore: Williams and Wilkins, 1992: 159-171.
88.
Bach JR, Beltrame F.Alternative approaches to home mechanical ventilation. In: Rothkopf MM, Askanazi J eds. Intensive Homecare. Baltimore: Williams and Wilkins, 1992: 173-97.
89.
Strumpf DA, Millman RP, Hill NSThe management of chronic hypoventilation. Chest1990; 98: 474-80.
90.
McPherson SPRespiratory home care equipment. Dubuque: Kendall Hunt, 1988.