The management of patients with advanced motor neuron disease

Abstract

Background: There is no specific treatment for motor neuron disease (MND) except hospice or palliative care to improve patients' quality of life and decrease complications. This topic is seldom discussed in Taiwan.

Methods: A retrospective study was conducted of patients with terminal MND who were treated and died at the Veterans General Hospital-Taipei from March 1986 through April 1996. Patients' characteristics, management, length of survival and cause of death were analyzed.

Results: Twenty-three patients (M/F, 17/6) were included. The median age of onset was 59 years (range, 24-69). The median interval from onset of symptoms to diagnosis was nine months (range, 2-36). Seventeen patients received mechanical ventilation for an average median of six months. Nineteen patients had dysphagia, 17 received long-term nasogastric tube feeding, one had gastrostomy and one was treated with cricopharyngeal myotomy. Pain over the neck, trunk or limbs was reported by 18 patients; none received narcotics. Only two patients received respiratory exercise training and two had a cervical collar for stabilization. Electronic communication aids were not available. The median survival from onset of symptoms was 36 months (range, 7-99). The causes of death included sepsis (n = 13), respiratory failure (n = 7), heart disease (n = 2) and MND-related cachexia (n = 1). Cardiopulmonary resuscitation was performed for 12 patients.

Conclusions: In Taiwan, management of patients with advanced MND is mainly hospital-based and most of the effort is focused on life-sustaining. More attention needs to be paid to improvement of the quality of life and dignity of the patient.