Abstract
Objective
To describe how patients cope with the proposal of treatment with intermittent artificial ventilation after acute respiratory failure due to progressive respiratory muscle weakness.
Design
Case series, follow-up study.
Setting
Neurological intensive care unit (ICU).
Patients
7 consecutive patients with metabolic myopathy treated for acute respiratory failure between 1983 and 1992.
Interventions
Intermittent positive pressure ventilation (IPPV) via tracheostomy.
Measurements and results
Symptoms of chronic hypoventilation preceded acute respiratory failure for months. With one exception, patients were mainly disabled from respiratory muscle weakness and sleep-related breathing disorders. IPPV was recommended to prevent recurrent respiratory failure. Two of three patients who accepted home IPPV returned to full-time jobs. One patient, who decided against IPPV, died from CO2 narcosis several months after discharge. All patients adhered to the respiratory regimen once instituted.
Conclusions
Acute respiratory failure in chronic myopathy is heralded by daytime drowsiness. IPPV, or at least regular monitoring of waking and sleeping partial pressure of carbon dioxide, is highly recommended even if weaning is successful. IPPV improved quality of life. The treatment strategy at discharge from the ICU should be optimal, as patients are reluctant to modify regimens.
This is a preview of subscription content, log in via an institution to check access.
References
O'Donohue WJ, Giovannoni RM, Goldberg AI, Keens TG, Make BJ, Plumner AL, Prentice WS (1986) Longterm mechanical ventilation. Guidelines for management in the home and at alternate community sites. Chest 90: 1S-37S
Howard RS, Wiles CM, Hirsch NP, Spencer GT (1993) Respiratory involvement in primary muscle disorders: assessment and management. Q J Med 86: 175–189
Newson Davis J, Goldman M, Loh L, Casson M (1976) Diaphragm function and alveolar hypoventilation. Q J Med 177: 87–100
Keunen RWM, Lambregts PCLA, Opde Coul AAW, Joosten MG (1984) Respiratory failure as initial symptom of acid maltase deficiency. J Neurol Neurosurg Psychiatry 47: 549–552
Guilleminault C, Stoohs R, Quera-Salva MA (1992) Sleep-related obstructive apneas and neurologic disorders. Neurology 42 [Suppl 6]: 53–60
Bertorini T, Yeh YY, Trevisan C, Stadlan E, Sabesin S, DiMauro S (1980) Carnitine palmitoyl transferase deficiency: myoglobinuria and respiratory failure. Neurology 30: 263–271
Heckmatt JZ, Loh L, Dubowitz V (1990) Night-time nasal ventilation in neuromuscular disease. Lancet 335: 579–582
Ellis ER, Bye PTP, Bruderer JW, Sullivan CE (1987) Treatment of respiratory failure during sleep in patients with neuromuscular disease. Am J Respir Crit Care Med 135: 148–152
Carroll JE, Zwillich C, Weil JV, Brooke MH (1976) Depressed ventilatory response in oculocraniosomatic neuromuscular disease. Neurology 26: 140–146
Conway WA, Victor LD, Magilligan DJ, Fujita S, Zorick FJ, Roth T (1981) Adverse effects of tracheostomy for sleep apnea. JAMA 246: 347–350
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Pfeiffer, G., Winkler, G., Neunzig, P. et al. Long-term management of acute respiratory failure in metabolic myopathy. Intensive Care Med 22, 1406–1409 (1996). https://doi.org/10.1007/BF01709559
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01709559