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Cystic Fibrosis Which cystic fibrosis patients have >80% predicted mortality during or in the 6 to 12 months after critical illness (level 1)? Level 3 criteria were validated in the GAP model, predicting a relatively low probability of 1-year mortality.[7] COPD Which COPD patients have >80% predicted mortality during or in the 6 to 12 months after critical illness (level 1)? Which COPD patients have >30% predicted mortality during or in the 6 to 12 months after critical illness (level 3)? Posted on March 28, 2020 https://www.corhealthontario.ca/Clinical-Triage-Protocol-for-Major-Surge-in-COVID-Pandemic-March-28-2020.pdf 5 S. Gupta, J. Batt, J. Bourbeau, Position statement from the Canadian Thoracic Society (CTS) on clinical triage thresholds in respiratory disease patients in the event of a major surge during the Covid-19 pandemic. [Extracted from the article]

Objective: To define the clinical characteristics and diagnostic parameters of patients with cystic fibrosis (CF) diagnosed in adulthood. Design: Retrospective cohort study. Setting: Tertiary care center. Patients and methods: All patients with a diagnosis of CF made at the Toronto CF Clinics between 1960 and June 2001. Data were collected prospectively and analyzed retrospectively. Results: There were 73 of 1,051 patients (7%) with CF diagnosed in adulthood. Over time, an increasing number and proportion of patients received a diagnosis in adulthood: 27 patients (3%) before 1990, compared to 46 patients (18%) after 1990 (p <0.001). The mean sweat chloride level was lower for those with CF diagnosed as adults, compared to those with a diagnosis as children (75 ± 26 mmol/L and 100 ± 19 mmol/L, respectively; p <0.001) [mean ± SD], and adults were more likely to have pancreatic sufficiency (PS) than children (73% vs 13%, respectively; p < 0.0001). In 46 adults who received a diagnosis since 1990, the reason for the initial sweat test was pancreatitis (2 patients, 4%), pulmonary symptoms (18 patients, 39%), pulmonary and GI symptoms (10 patients, 22%), infertility (12 patients, 26%), and genetic screening (4 patients, 9%). Other manifestations were biliary cirrhosis (one patient) and diabetes mellitus (four patients, 9%). The diagnosis could be confirmed by sweat test alone in 30 of 46 patients (65%), by mutation analysis alone in 15 patients (33%), and by a combination in 31 patients (67%). Nasal potential difference (PD) measurements alone confirmed the diagnosis in the remaining 15 patients (33%). Conclusion: Patients with CF presenting in adulthood often have PS, inconclusive sweat test results, and a high prevalence of mutations that are not commonly seen in CF diagnosed in childhood. Although most patients have lung disease of variable degrees, single-organ manifestations such as congenital bilateral absence of the vas deferens and pancreatitis are seen. Repeated sweat tests and extensive mutation analysis are often required. Nasal PD may aid the diagnosis, but has not been standardized for clinical diagnosis. [ABSTRACT FROM AUTHOR]

Study objective: To determine the repeatability of measurements of FEV[sub 1] in adults with lung disease due to cystic fibrosis (CF). Design: Single cohort study nested within a randomized controlled trial. Setting: Adult CF of a university teaching hospital. Subjects were participants in a randomized trial of an experimental mucolytic drug. Patients: Twenty-one adults (mean age, 27.5 ± 9.2 years [± SD]) with CF and mild-to-moderate airflow obstruction (FEV[sub 1] 70 ± 15% predicted). Patients were in clinically stable condition prior to and during the study. Interventions: Repeated FEV[sub 1] measurements were obtained at specific times of the day for 9 consecutive days, for a total of 31 measurements from each subject. Statistical measures of repeatability were calculated. Variation over the course of 1 day and variation from I day to the next were examined separately. Measurements and results: For day-to-day FEV[sub 1] measurements, the within-subject SD was 0.145 L (4.5% of predicted), indicating greater variation compared to values previously established in normal subjects. The coefficient of repeatability indicated that day-to-day measurements could differ by as much as 13% of predicted in the absence of clinical change. For measurements within a single day, variation was not observed to be greater than normal. Conclusions: In adults with CF, day-to-day variation in FEV[sub 1] measurements is greater than normal and similar to that seen in other obstructive lung diseases. Changes in FEV[sub 1] over time in adults with CF can likely be interpreted using the same criteria that apply to asthma or COPD. Key words: adult; cystic fibrosis; forced expiratory volume; reproducibility of results; respiratory function tests. [ABSTRACT FROM AUTHOR]

Objective: To assess the effect of pregnancy on pulmonary function and survival in women with cystic fibrosis (CF) and to assess the fetal outcome. Design: Cohort study. The data analyzed were collected from the Toronto CF database, chart review, and patient questionnaire. Setting: Tertiary-care center. Patients: All women with CF who, at the time of diagnosis or pregnancy, attended the Toronto Cystic Fibrosis Clinics between 1961 and 1998. Results: From 1963 to 1998, there were 92 pregnancies in 54 women. There were 11 miscarriages and 7 therapeutic abortions. Forty-nine women gave birth to 74 children. The mean follow-up time was 11 ± 8 years. One patient was lost to follow-up shortly after delivery, and one was lost after 12 years. The overall mortality rate was 19% (9 of 48 patients). Absence of Burkholderia cepacia (p < 0.001), pancreatic sufficiency (p = 0.01), and prepregnancy FEV[sub 1] > 50% predicted (p = 0.03) were associated with better survival rates. When adjusted for the same parameters, pregnancy did not affect survival compared to the entire adult female CF population. The decline in FEV[sub 1] was comparable to that in the total CF population. Three women had diabetes mellitus, and seven developed gestational diabetes. There were six preterm infants and one neonatal death. CF was diagnosed in two children. Conclusions: The maternal and fetal outcome is good for most women with CF. Risk factors for mortality are similar to those for the nonpregnant CF population. Pregnancies should be planned so that there is opportunity for counseling and optimization of the medical condition. Good communication between the CF team and the obstetrician is important. [ABSTRACT FROM AUTHOR]