Survival and quality of life after tracheostomy for acute respiratory failure in patients with amyotrophic lateral sclerosis☆
Introduction
Amyotrophic lateral sclerosis (ALS; also called motor neuron disease) is a devastating disease that involves both upper and lower motor neurons. It is the most common form of motor neuron disease, affecting approximately 1.2 to 1.8/100 000 individuals. Clinically, ALS is characterized by progressive muscular weakness leading to loss of the ability to move and speak and, in almost all patients, causes death between 3 and 5 years from diagnosis. Intellect and awareness remain essentially unchanged [1].
The onset of acute respiratory failure (ARF) due to diaphragm weakness, ineffective cough with airway mucous encumbrance, and inability to handle oropharyngeal secretions is a common event in the advanced stage of ALS and a major cause of death, unless mechanical ventilation (MV) is used [2], [3], [4], [5], [6]. Although noninvasive ventilation (NIV) combined with assisted coughing techniques can be a safer and more effective alternative to endotracheal intubation (ETI) in the treatment of neuromuscular ARF [7], [8], [9], individuals with ALS in most cases have to be excluded from NIV, due to the severe bulbar involvement with inability to adequately protect the airways, and require to be administered ETI. Unfortunately, most ALS patients who undergo invasive MV via ETI fails to wean adequately and may require tracheostomy and long-term MV (LTMV) [4], [6], [10]. The literature evaluating survival of ALS patients receiving LTMV via tracheostomy is disappointingly scarce and contains conflicting reports, with considerable variation among studies in the time between institution of this treatment and death [6], [10], [11], [12]. In addition, previous data suggest that life satisfaction may be poor for individuals with ALS who are administered tracheostomy at the time of a respiratory crisis without discussing this option in advance [12].
In such a situation, although beliefs and practice regarding end-of-life care may vary in different societies, many physicians in the intensive care unit (ICU) may be hesitant to administer invasive MV in the terminal phase of ALS, particularly if the patient has not requested in advance full support in the event of ARF [13], considering that this aggressive ventilatory approach may lead to an uncertain and sometimes unwanted prolongation of life and that the patient's dependence on ventilatory support may determine a dramatic physical and economic burden on family and caregivers after discharge from hospital [14], [15].
This paucity of information prompted us to review our 13-year experience of tracheostomy following ARF in ALS to determine postdischarge survival and identify those patient characteristics predicting long-term outcome. In addition, to gain a better understanding about the impact of tracheostomy on patients' quality of life (QoL) and degree of depression, we prospectively administered the 11-item short-form Life Satisfaction Index (LSI-11) [16] and Beck Depression Inventory (BDI) [17] questionnaires to survivors.
We hypothesized that our findings on the life expectancy and satisfaction after unplanned tracheostomy might help physicians in the ICU to face the challenging ethical dilemma associated with the management of patients with ALS who acutely require MV.
Section snippets
Methods
This study was approved by the institutional review board. Oral consent was obtained from all participants and their families for the interview portion of the study.
Survival
Sixty-one patients with ALS were admitted to our Unit as a result of ARF and were administered ETI and MV as an emergency procedure. None of the patients had expressed any advanced directives pertaining to the desire to undergo tracheotomy. One patient declined tracheostomy and died 35 days after intubation for severe pneumonia and septic shock. Sixty patients who had consented to tracheostomy met the study criteria and participated in the study. Clinical, demographic, and pulmonary function
Discussion
In the present study, postdischarge survival was retrospectively analyzed in a population of 60 patients with ALS who were administered tracheostomy after an episode of ARF, making it one of the largest studies on tracheostomy in ALS; in addition, QoL was prospectively evaluated in survivors. We demonstrated that patients have a high chance of long-term survival (median survival, 21 months; ranging from 0 to 155 months) and that age at tracheostomy is an independent prognostic factor for
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Statement of interests: We all Authors declare that we have no financial and personal relationships with people or organizations that could have inappropriately influenced (bias) our work. No study sponsor has been involved in the study design, in the collection, analysis and interpretation of data; in the writing of the manuscript; and in the decision to submit the manuscript for publication. We have no conflicts of interest to declare.