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Synopsis: This report documents the outcomes of 136 medical ICU admissions of adult patients with severe cystic fibrosis, managed in a large CF center with a lung transplantation program. The results support the conclusion that such care is as appropriate and effective for this group of patients as for many others commonly managed in the ICU.
Source: Sood N, et al. Am J Respir Crit Care Med. 2001;163:335-338.
Sood and colleagues at the University of North Carolina reviewed all admissions of patients with cystic fibrosis (CF) to their medical ICU from 1990 through 1998, representing the first 9 years since establishment of a lung transplant program at that center. Patients admitted after lung transplantation were excluded. In addition to patient demographics, diagnoses, and severity of underlying disease, Sood et al examined the interventions used, complications, and survival data.
During the study period, 76 adults with CF (41 females, ages 16-42 years; mean 26 years, and 35 males, ages 18-45 years; mean 30 years) were admitted to the medical and respiratory ICUs a total of 136 times. Primary admitting diagnoses were exacerbations of CF with respiratory failure (48% of admissions), massive hemoptysis (24%), antibiotic desensitization (22%), pneumothorax (2%), and miscellaneous (4%, including 2 suicide attempts). In the course of 65 admissions for CF exacerbation with acute respiratory failure, ventilatory support was used in 50. Tracheal intubation was carried out in 32 episodes in 30 patients on 5 occasions after failure of noninvasive ventilation; in 13 other episodes, intubation was avoided by using mask ventilation.
Among the patients requiring intubation, 12 (40%) died. Mean duration of ventilatory support for these patients was approximately 10 days (range, 1-45 days) for both survivors and nonsurvivors. Of the patients admitted to the ICU with respiratory failure, 17 (40%) received lung transplants, and 14 of these (82%) were alive 1 year later; without transplant, 3 patients (7%) were alive and 3 (7%) were dead after 1 year. Sex, body mass index, baseline pulmonary function, and respiratory bacteria did not correlate with survival. Sood et al conclude that ICU care for adult patients with CF who have potentially reversible complications is appropriate and effective. They also conclude that ventilatory support is appropriate for at least some of these individuals who are candidates for lung transplantation.
A survey in the late 1970s documented very poor outcomes among CF patients who required mechanical ventilation.1 In those days, the recommendation of some authorities for CF patients with severe lung disease and respiratory failure was palliative care only. Things have definitely changed. CF is no longer a disease managed just by pediatricians. Median survival for individuals with CF is currently somewhere in the fourth decade, with increasing numbers in their 40s and beyond. This means that patients with CF are now managed by clinicians caring for adults, and that hemoptysis, acute respiratory failure, and other complications are increasingly encountered in the medical ICU. It is important that old notions of the futility of aggressive care in these circumstances be discarded, as emphasized by the results of this study.
In this series, 60% of the CF patients with acute respiratory failure who required intubation and ventilatory support survived through to hospital discharge. This is comparable to current reported survival rates in the acute respiratory distress syndrome, and definitely better than for patients with respiratory failure complicating solid-tumor or hematologic malignancy. With the prospect of lung transplantation for at least some of these patients, it is reasonable to expect not only a return to the previous state of severe functional impairment but—for some anyway—achievement of dramatically improved health status, if not a completely normal life.
It is important to note that some factors that might have been expected to predict a poor outcome in these patients, such as severity of airflow obstruction and low body mass index, were not reliable predictors of a poor outcome. The same was true for respiratory tract colonization with Staphylococcus aureus, Burkholderia cepacia, and antibiotic-resistant Pseudomonas aeruginosa.
This paper reports findings on a highly selected population of CF patients, managed at a large referral center with specialized expertise in this disease and a major lung transplant program. The fact that 60% of these patients with respiratory failure made it out of the hospital despite the need for intubation and mechanical ventilation does not mean that the same would necessarily happen at my hospital, or yours. The results reported by Sood et al show what can be achieved at a highly specialized center. Nonetheless, it is encouraging to note that the outlook for CF patients with critical illness is far better than it was just a few years ago. This is reflected in the title of the editorial accompanying this paper: "Cystic fibrosis critical care: No longer an oxymoron." 2
1. Davis PB, di Sant’Aguese PA. JAMA. 1978;239:1851-1854.
2. Wallick K, et al. Am J Respir Crit Care Med. 2001;163:310-312.