We evaluated the clinical characteristics and outcome of tracheostomy in amyotrophic lateral sclerosis (ALS) using data from the Piemonte and Valle d'Aosta Register for ALS, a prospective epidemiological register collecting all ALS incident cases in two Italian regions. Among the 1260 patients incident in the period 1995–2004, 134 (10.6%) underwent tracheostomy. Young male patients were more likely to be tracheostomised. Site of onset (bulbar vs spinal) and period of diagnosis (1995–1999 vs 2000–2004) did not influence the likelihood of being tracheostomised. The mean duration of hospital stay was 52.0 days (SD 60.5). Overall, 27 patients died while still in hospital (20.1%). Sixty-five patients (48.5%) were discharged to home, whereas 42 (31.3%) were admitted to long-term care facilities. The median survival time after tracheostomy was 253 days. In the Cox multivariable model, the factors independently related to a longer survival were enteral nutrition, age, marital status and ALS centre follow-up. In conclusion, in an epidemiological setting, ALS survival after tracheostomy was <1 year. Sociocultural factors influence the probability of choice to be tracheostomised, even in a highly socialised health system as Italian one.
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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterised by a progressive weakness and atrophy of limb, trunk, bulbar and respiratory muscles. Respiratory failure can occur at different stages, and its management is based on mechanical ventilation with non-invasive ventilation (NIV) or tracheostomy when NIV is no longer effective.1 There are few papers assessing the clinical and demographic characteristics of ALS patients with tracheostomy.2–6
The aim of the present study was to evaluate the clinical characteristics and outcome of tracheostomy in ALS using data from a prospective epidemiological register.
The Piemonte and Valle d'Aosta Register for ALS is a prospective epidemiological register established in 1995 collecting all ALS incident cases in two Italian regions (population 4 332 842). Methods and epidemiological data have been reported in detail elsewhere.7 In brief, the main source of cases was the neurological departments of the two regions. The main secondary source of cases were the Piemonte Central Regional Archive and the Valle d'Aosta Central Regional Archive, two computerised archives reporting all discharges from all Italian public and private hospitals of individuals resident in Piemonte or Valle d'Aosta. The archives also report data about clinical complications and interventions, including tracheostomy.
ALS diagnosis was based on the El Escorial diagnostic criteria (EEC) and the EEC revised criteria. Because patients were prospectively followed up at regular intervals (2–4 months), their EEC diagnosis was regularly verified and, if necessary, modified. A standard form was used for collecting clinical and therapeutical information at each visit. For tracheostomy, it was requested whether it was performed as an elective intervention (ie, after an advanced patient decision) or on emergency basis (ie, with patient's decision taken only when occurred acute respiratory failure). After discharge, the patients were followed at their home or in the nursing home by trained pulmonologists or anaesthesiologists, under the provision of the Italian National Health System Integrated Home Care. No patient was lost to follow-up.
The study has been approved by the Ethical Committee of the coordinating centre.
Survival analysis (last day of follow-up, 30 June 2008) was performed with the Kaplan-Meier method, and the curves were compared with the log-rank test. Multivariable analysis was performed using Cox's proportional hazards method (backward stepwise) (see Supplementary Table 2 for details). A p level <0.05 was considered significant. All calculations were performed with the SAS statistical package, V.8.2 (SAS Institute Inc., Cary, North Carolina, USA).
Among the 1260 patients incident in the period 1995–2004 (mean annual crude incidence rate, 2.90/100 000 population), 134 (10.6%) underwent tracheostomy (75 elective and 59 on emergency). In two cases, ALS diagnosis was made after tracheostomy. The mean duration of ALS before tracheostomy was 2.4 years (SD 1.9). The clinical characteristics of patients who did and did not undergo tracheostomy are reported in the table 1 Tracheostomised patients were younger and more frequently men; in particular, male sex increased the odds ratio to be tracheostomised by 1.50. This effect was completely due to the higher probability of younger men to be tracheostomised (Supplementary Table 1). Site of onset and period of diagnosis did not influence the likelihood of being tracheostomised. Patients followed by ALS multidisciplinary centres, as well as patients who underwent enteral nutrition (EN) or NIV, had significantly higher odds ratios to undergo tracheostomy.
The mean duration of hospital stay was 52.0 days (SD 60.5; median, 36 days; range 5–318); it was shorter for cases followed at a multidisciplinary ALS centre (37.9 (SD 27.2)) than for subjects followed by general neurologists (68.5 (SD 81.4)) (p=0.009); it was also significantly shorter for younger patients, but it was not related to the intervention type (emergency vs elective) or sex.
Twenty-seven patients died while still in hospital (20.1%), 17 of whom within 1 month after tracheostomy; in-hospital mortality was significantly related to age (mean age, deceased vs not deceased patients, 67.9 vs 60.1 years, p=0.0001) and was lower in subjects followed at ALS multidisciplinary centres (13.1% vs 28.8%; p=0.02). No other factor influenced in-hospital mortality.
Sixty-five patients (48.5%) were discharged to home, whereas 42 (31.3%) were admitted to long-term care facilities. The median survival time after tracheostomy was 253 days considering all cases and 339 days excluding patients who died within 30 days after tracheostomy. The overall 1-year, 3-year and 5-year survival rates were 42.6% (SE 4.3%), 22.9% (SE 3.8%), and 12.2% (SE 3.3%), respectively (figure 1). At the last day of follow-up, 20 patients were still alive. The most common causes were respiratory tract infections (48), non-respiratory medical complications (30), acute complications of tracheostomy (15), cardiopulmonary complications (9), sudden death (6) and other causes (6).
At univariate analysis, factors related to survival were age (<45 vs 45–64 vs ≥65 years) (p=0.002), marital status (married vs unmarried, divorced or widow/widower) (p=0.003), follow-up at an ALS centre (yes vs no) (p=0.02), discharge disposition (home vs long-term care facility) (p=0.02) and duration of ALS before tracheostomy (≤365 vs >365 days) (p=0.05) (Supplementary figures 1–5). Factors not related to survival were sex, site of onset, educational level, period of diagnosis and type of intervention. In the Cox multivariable model, the only factors independently related to a longer survival were EN, age, marital status, and ALS centre follow-up (Supplementary table 2).
In this paper we assessed the incidence and prognosis of tracheostomy in a population-based cohort of patients with ALS. Overall, 10.6% of patients in the 10-year study period underwent tracheostomy, a figure similar to that reported in another Italian ALS register.6 On the contrary, in the Irish ALS Register, only 4 (0.8%) of the 465 patients incident in the period 1995–2004 underwent tracheostomy.5 In a recent US clinical trial, 6% of cases underwent tracheostomy.8 These differences could be related to cultural and religious factors, to the different organisation of the healthcare systems or to differences in the attitudes of health professionals.9
Tracheostomy was more frequently performed in young male patients, similarly to what was observed in other ALS series.3 10 The reasons for this inequality are not clear; they do not seem to be related to a different access to health system facilities for males with respect to females. It could be supposed that the choice of tracheostomy by men is more related to sociocultural factors, as indicated also by the observation that the age groups that mostly contribute to the sex “gap” are those <65 years old.
In our series, tracheostomy was more frequently performed in married persons, indicating that the presence of a family care giver (spouse) is one of the main reasons for patients to choose tracheostomy. In fact, because it has been found that caring for a tracheostomised patient carries with it a considerably high burden and low subjective quality of life,11 a spouse is more likely to be disposed to accept this. Moreover, the significant association between EN, NIV and tracheostomy indicates that some patients are more willing than others to accept life-preserving measures.12 Surprisingly enough, there was no difference according to the education level; because education can be considered a proxy of social status, this finding indicates that in our series a lower social level was not a reason for refusing tracheostomy.
The duration of hospital stay in our series was similar to that found in other studies.2 3 In our cohort, 20.1% of patients died while still hospitalised, a figure similar to the proportion of hospital deaths of ALS in a US national survey.13
Median survival after tracheostomy was <1 year; 23% of patients survived 3 years and approximately 10% >5 years. The survival rates observed in our series were similar to those of previous surveys from Sweden and USA,2 10 but lower than those observed in a single-centre Italian study with a particular expertise in ALS ventilation.3 Therefore, in a general population setting, the survival of patients with ALS treated with tracheostomy is lower relatively short, but it can significantly increase when patients are followed up at qualified centres.
The factors independently related to survival after tracheostomy were age, EN, marital status and the fact of being followed up by an ALS centre. Age is a well-known prognostic factor in ALS.14 EN represents the main measure in the treatment of malnourishment and dehydration in ALS, and is often used in conjunction with NIV or tracheostomy. Marital status has been found to carry a better prognosis, indicating that patients who live with their spouses receive better care.15 The fact of being followed up by a multidisciplinary ALS centre is likely to be related to the better general care given to patients in a multidisciplinary setting.14
In conclusion, tracheostomy is performed by about 10% of patients with ALS in an Italian epidemiological setting, and median survival after tracheostomy is <1 year. Sociocultural factors may influence the choice to be tracheostomised, even in a highly socialised health system as Italian one. The information reported in this study may be useful for the decision-making process when discussing tracheostomy with patients and their families, and in better defining health policies regarding extra-hospital care in advanced ALS.
Appendix The Piemonte and Valle d'Aosta Register for ALS group
Project coordinator: A. Chiò, MD. Collaborating centres: Department of Neuroscience, University of Torino, and AOU S. Giovanni Battista, Torino (A. Chiò, MD; A. Calvo, MD, PhD; C. Moglia, MD; P. Ghiglione, MD; D. Cocito, MD; R. Mutani, MD); Department of Neurology, University of Torino, and AOU S. Luigi Gonzaga, Orbassano (L. Durelli, MD; B. Ferrero, MD; A. Bertolotto, MD); University of Torino, and Istituto Auxologico Italiano, IRCCS, Piancavallo (A. Mauro, MD); Department of Neurology, University of Piemonte Orientale Amedeo Avogadro, and AOU Maggiore, Novara (M. Leone, MD; L. Mazzini, MD, N. Nasuelli, MD, F. Monaco, MD); Department of Neurology, AOU S. Giovanni Battista, Torino (R. Comitangelo, MD); Department of Neurology, Ospedale Mauriziano, Torino (L. Sosso, MD; M. Gionco, MD); Department of Neurology, Ospedale Martini, Torino (M. Nobili, MD); Department of Neurology, Ospedale Maria Vittoria, Torino (L. Appendino, MD; C. Buffa, MD); Department of Neurology, Ospedale S. Giovanni Bosco, Torino (R. Cavallo, MD); Department of Neurology, Ospedale Gradenigo, Torino (E. Oddenino, MD); Department of Neurology, Ospedale di Ivrea (G. Ferrari, MD); Department of Neurology, Ospedale di Chivasso (C. Geda, MD); Department of Neurology, Ospedale di Pinerolo (M. Favero, MD; C. Doriguzzi Bozzo, MD); Department of Neurology, Ospedale di Vercelli (P. Santimaria, MD); Department of Neurology, Ospedale di Biella (U. Massazza, MD); Department of Neurology, Ospedale di Domodossola (A. Villani, MD; R. Conti, MD); Fondazione S. Maugeri, Clinica del Lavoro e della Riabilitazione, IRCCS, Scientific Institute of Pavia (G. Mora, MD; F. Pisano, MD); Department of Neurology, AO Santi Antonio e Biagio, Alessandria (M. Palermo, MD); Department of Neurology, Ospedale di Casale Monferrato (F. Vergnano, MD); Department of Neurology, Ospedale di Novi Ligure (M Aguggia, MD); Department of Neurology, Ospedale di Tortona (M.T. Penza, MD); Department of Neurology, Ospedale di Asti (F. Fassio, MD; N. Di Vito); Department of Neurology, AO S. Croce e Carle, Cuneo (P. Meineri, MD); Department of Neurology, Ospedale di Savigliano (D. Seliak, MD); Department of Neurology, Ospedale di Alba (C. Cavestro, MD; G. Astegiano, MD); Department of Neurology, Ospedale Regionale di Aosta (G. Corso, MD; E. Bottacchi, MD).
Funding The work was in part supported by the Regione Piemonte (Ricerca Finalizzata 2002, grant 12944; Ricerca Scientifica Applicata 2004, grant A317) and by the Compagnia di San Paolo (grant 2003.0078).
Competing interests None.
Patient consent Obtained.
Ethics approval This study was conducted with the approval of the Ethical Committee of Piemonte Region.
Provenance and peer review Not commissioned; externally peer reviewed.
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