12. Dalen JE, Alpert JS, Hirsh J. Thrombolytic therapy for pulmonary embolism: is it effective? Is it safe? When is it indicated? Arch Intern Med. 1997;157(22):2550-2556. 13. Carlbom DJ, Davidson BL. Pulmonary embolism in the critically ill. Chest. 2007;132(1):313-324. 14. Konstantinides S, Geibel A, Heusel G, Heinrich F, Kasper W; Management Strategies and Prognosis of Pulmonary Embolism-3 Trial Investigators. Heparin plus alteplase compared with heparin alone in patients with submassive pulmonary embolism. N Engl J Med. 2002;347(15): 1143-1150. 15. Meneveau N, Séronde MF, Blonde MC, et al. Management of unsuccessful thrombolysis in acute massive pulmonary embolism. Chest. 2006;129(4):1043-1050. Pulmonary Rehabilitation for Restrictive Lung Diseases respiratory conditions share a number of C hronic common manifestations, such as dyspnea, cough, fatigue, and inactivity. In addition, patients often experience secondary peripheral muscle, cardiac, nutritional, and psychologic impairments, which individually or in combination with their respiratory condition further limit exercise capacity and healthrelated quality of life (HRQL). The resulting impact on personal and family life, as well as the associated increase in health-care use, is well known. Given the evidence that pulmonary rehabilitation (PR) benefits the large population with COPD, it is a reasonable assumption that those with other respiratory conditions might benefit similarly. Whereas the PR literature has focused on COPD, there is growing interest in evaluating PR for patients with restrictive lung diseases. Two groups reported a total of 87 patients with either idiopathic pulmonary fibrosis1 or a variety of interstitial lung diseases (ILD),2 randomized to usual care or to 8 to 10 weeks of outpatient exercise training. At the end of the training periods, both groups reported significant improvements in 6-minute walk distance (6MWD), with the exercise group improving by 35 to 46 m relative to the control group. Subjects in the exercise group also demonstrated an improvement in HRQL, although not in peak exercise capacity. These results were consistent with those from earlier observational reports of PR in ILD.3-6 Disappointingly, Holland and colleagues found that none of the benefits was sustained when follow-up was extended to 6 months.2 In this issue of CHEST (see page 273), Salhi and colleagues7 present a subset of data from REVALIS, a prospective cohort study examining the effectiveness of outpatient PR in Belgium. They report their observations on 31 subjects entering the study with a diagnosis of restrictive lung disease, 11 of whom had ILD. The program extended over 24 weeks and comwww.chestjournal.org Downloaded From: http://publications.chestnet.org/ on 09/09/2014 bined endurance training, strength training, education, and psychologic support. Overall, patients experienced remarkable improvements in their symptoms and in their exercise capacity. The 11 patients with ILD experienced an impressive improvement in functional exercise, increasing their 6MWD by 79 m at 12 weeks, and 102 m after the full 24 weeks, compared with their baseline values. The accompanying increase in peak workload and oxygen uptake measured by incremental cycle ergometry signified that a physiologic training effect had also been achieved. The Chronic Respiratory Questionnaire8 dyspnea score improved by at least 2.5 points in nearly three-quarters of patients by the end of the program. Although the minimal clinically significant change in scores for 6MWD and for the Chronic Respiratory Questionnaire have not been established for patients with ILD, the improvements noted substantially exceed the value established for patients with COPD. What Lessons Can Be Drawn From This Report? First, the study lends more support to the case that patients with restrictive lung disease can safely participate in PR, given that 26 of 31 subjects, including 10 of 11 with ILD, were able to complete the full 24-week program. Those on oxygen at baseline were equally successful at completing the program, without any serious adverse events. These data are in line with similar observations reported for outpatient PR in COPD.9 Second, severity of illness should not disqualify patients with ILD from participating in PR. On the contrary, those with worse disease may benefit more. Although the clinical trials by Nishiyama’s1 and Holland’s2 groups included patients with mild-tomoderate lung restriction, in whom the 6MWD at baseline was close to 400 m, the current study subjects had more severe restriction and lower baseline 6MWDs. Moreover, they required at least two other measures of impairment in exercise capacity, muscle function, symptoms, or quality of life. The limited gains seen in the previous studies may have been due to a ceiling effect, so that selecting more severely impaired patients enabled the current study to demonstrate greater improvements after PR. Third, the duration of rehabilitation plays an important role. Although the 6MWD and HRQL scores among Holland’s training group returned to baseline on follow-up after a 9-week exercise training program, Salhi and colleagues7 identified greater benefits among their subjects, who completed 6 months of training. These observations are in keeping with reported outcomes in the COPD literature in which longer programs generated greater and more durable benefits.10-12 It is reasonable to assume CHEST / 137 / 2 / FEBRUARY, 2010 247 that longer programs enable patients to reach higher training intensities, which are known to result in physiologic training effects.13 Longer programs may be especially important for PR in ILD, as many of those likely to be enrolled will have early and profound exercise-induced oxygen desaturation in addition to their dyspnea and peripheral muscle weakness, thereby obliging the exercise therapist to start training at a low exercise intensity. An extended program might offset the low initial training loads. As with many observational studies, a note of caution must be applied to the interpretation of these results. The absence of a control group or random treatment allocation weakens the message, as the additional benefit of rehabilitation over usual care could not be reported. Furthermore, the small number of subjects with ILD who enrolled in PR does not exclude the possibility of observed differences arising from the play of chance. In summary, our view of PR for patients with ILD is an optimistic one. We anticipate that the optimal duration, intensity, and program content will be further refined during the next few years. Robert G. Varadi, MDCM Roger S. Goldstein, MB ChB, FCCP Toronto, ON, Canada Affiliations: From the University of Toronto, Department of Respiratory Medicine, West Park Healthcare Centre. Financial/nonfinancial disclosure: The authors have reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article. Correspondence to: Roger Goldstein, MB ChB, FCCP West Park Healthcare Centre, 82 Buttonwood Ave., Toronto, ON, M6M 2J5 Canada; e-mail: [email protected] © 2010 American College of Chest Physicians. Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/ site/misc/reprints.xhtml). DOI: 10.1378/chest.09-1857 References 1. Nishiyama O, Kondoh Y, Kimura T, et al. Effects of pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis. Respirology. 2008;13(3):394-399. 2. Holland AE, Hill CJ, Conron M, Munro P, McDonald CF. Short term improvement in exercise capacity and symptoms following exercise training in interstitial lung disease. Thorax. 2008;63(6):549-554. 3. Holland A, Hill C. Physical training for interstitial lung disease. Cochrane Database Syst Rev. 2008;(4):CD006322. 4. Varadi RG, Goldstein RS, Stanbrook MB. Outcomes of pulmonary rehabilitation (PR) in idiopathic pulmonary fibrosis (IPF) [abstract]. Am J Respir Crit Care Med. 2008;177:A792. 5. Jastrzebski D, Gumola A, Gawlik R, Kozielski J. Dyspnea and quality of life in patients with pulmonary fibrosis after six weeks of respiratory rehabilitation. J Physiol Pharmacol. 2006;57(suppl 4):139-148. 6. Naji NA, Connor MC, Donnelly SC, McDonnell TJ. Effectiveness of pulmonary rehabilitation in restrictive lung disease. J Cardiopulm Rehabil. 2006;26(4):237-243. 248 Downloaded From: http://publications.chestnet.org/ on 09/09/2014 7. Salhi B, Troosters T, Behaeqel M, Joos G, Derom E. Effects of pulmonary rehabilitation in patients with restrictive lung diseases. Chest. 2010;137(2):273-279. 8. Lacasse Y, Wong E, Guyatt G. A systematic overview of the measurement properties of the chronic respiratory questionnaire. Can Respir J. 1997;4(3):131-139. 9. Griffiths TL, Burr ML, Campbell IA, et al. Results at 1 year of outpatient multidisciplinary pulmonary rehabilitation: a randomised controlled trial. Lancet. 2000;355(9201):362-368. 10. Green RH, Singh SJ, Williams J, Morgan MD. A randomised controlled trial of four weeks versus seven weeks of pulmonary rehabilitation in chronic obstructive pulmonary disease. Thorax. 2001;56(2):143-145. 11. Güell R, Casan P, Belda J, et al. Long-term effects of outpatient rehabilitation of COPD: a randomized trial. Chest. 2000;117(4):976-983. 12. Troosters T, Gosselink R, Decramer M. Short- and long-term effects of outpatient rehabilitation in patients with chronic obstructive pulmonary disease: a randomized trial. Am J Med. 2000;109(3):207-212. 13. Casaburi R, Patessio A, Ioli F, Zanaboni S, Donner CF, Wasserman K. Reductions in exercise lactic acidosis and ventilation as a result of exercise training in patients with obstructive lung disease. Am Rev Respir Dis. 1991;143(1):9-18. Life and Death Decisions in the Middle of the Night Teaching the Assessment of Decision-Making Capacity 2:00 am one morning, when I was a much Atyounger attending physician, I received a call from a resident on service in the ICU. He called about a case with “an FYI,” because he had a few minutes and thought I might want to be informed. He told me of a 25-year-old man admitted that night with what appeared to be severe community-acquired pneumonia. The patient carried the diagnosis of developmental delay, but, the resident informed me, the patient had been previously determined to be legally competent to make his own decisions by the courts, and he lived alone. The patient was developing acute respiratory failure, and he refused intubation and mechanical ventilation. The resident informed the patient that he would likely die without this treatment, and the patient told the resident that he understood. The resident tried noninvasive ventilation, and the patient refused it. The resident was calling to tell me that he was planning to transition to comfort measures only. The team had tried unsuccessfully to reach the patient’s parents, although the social worker was still trying. On hearing this story from my warm bed at home, my mind—and heart—began racing. What makes a 25-year-old, previously healthy man want to refuse life-sustaining mechanical ventilation, and how do I Editorials