Assessing Infertility Literacy and Knowledge Gaps Among Patients with Cystic Fibrosis.

OBJECTIVE: As patients with cystic fibrosis live longer into their reproductive years, fertility concerns are rising. We hypothesized that while patients with cystic fibrosis may be informed of the impact of their disease on their reproductive potential, they remain unaware of the promising role of assisted reproductive technology in helping them conceive biological children. METHODS: We distributed a voluntary and anonymous survey to cystic fibrosis patients and organizations to assess patient understanding of cystic fibrosis-related infertility. The survey questions aimed to capture demographic information, their reproductive education regarding cystic fibrosis, and their preferences for future fertility. RESULTS: Forty respondents completed the survey (median age of 36 ± 14 years). The median age reported for learning about cystic fibrosis-associated infertility was 18 years. Respondents preferred that reproductive and infertility education be provided early; 43% reported the optimal age of education was younger than 18 years while 50% reported between 18 and 24 years. Of the respondents trying to conceive, 43% of patients have been trying to conceive for 1-3 years qualifying for infertility. Yet, the majority of those patients (69%) have not been offered a semen analysis and 90% have not had previous fertility treatments. CONCLUSION: Our findings highlight that cystic fibrosis patients are knowledgeable about cystic fibrosis-related impacts on their fertility, with high-rated self-confidence. A fraction of patients still desire to conceive but have not been provided with assisted reproductive services. We recommend the establishment of active partnerships between cystic fibrosis care teams and fertility specialists to maximize their chances of conception.

SpO2 /FIO2 on Presentation as a Predictor for Early Hemodynamic Deterioration in Intermediate Risk Acute Pulmonary Embolism.

BACKGROUND: Patients with intermediate-risk acute pulmonary embolism are at risk of hemodynamic deterioration, and identification of risk factors for decompensation could guide the administration of thrombolytics. We aimed to assess whether SpO2 /FIO2 on presentation is associated with early hemodynamic deterioration in this population. METHODS: A retrospective chart review of subjects admitted between 2006 and 2018 with intermediate-risk pulmonary embolism (hemodynamically stable with right ventricle to left ventricle ratio > 0.9 or tricuspid annular plane systolic excursion < 18 mm). Early hemodynamic deterioration was defined as requirements for vasopressors or rescue thrombolytics within 48 h. Results are presented as median (interquartile range). RESULTS: A total of 178 subjects were included. Early hemodynamic deterioration occurred in 13% of the subjects and was associated with a median (interquartile range) lower SpO2 /FIO2 on presentation in univariate analysis (243 [123-275] versus 438 [335-457], P < .001) and in a multivariate analysis, including heart rate and right ventricle to left ventricle ratio as covariates (odds ratio 0.992, 95% CI 0.987-0.996; P < .001). The initial SpO2 /FIO2 predicted hemodynamic deterioration with an area under the receiver operating characteristic curve of 0.81 and a threshold of 260 was associated with a sensitivity of 74% and specificity of 88%. Sensitivity analyses restricted to subjects with hypoxemia on presentation and subjects with an elevated troponin level led to similar results. CONCLUSIONS: In intermediate-risk pulmonary embolism, SpO2 /FIO2 on presentation can help predict the risk of early hemodynamic deterioration.

Is venous blood gas performed in the Emergency Department predictive of outcome during acute on chronic hypercarbic respiratory failure?

BACKGROUND: During acute on chronic hypercarbic respiratory failure (AHRF), arterial pH is associated with non-invasive ventilation (NIV) failure and mortality. Venous blood gas (VBG) has been proposed as a substitute for arterial blood gas, based on a good agreement between venous and arterial values. We assessed the predictive value of admission VBG on intubation rate, NIV failure and mortality during AHRF. METHODS: Retrospective chart review of inpatients admitted between 2009 and 2015 with AHRF who had VBG performed on admission. Demographic, clinical and biological data were collected throughout the hospital course. RESULTS: 196 patients were included and hospital survival was not significantly associated with initial venous pH, PCO2 or HCO3-. Patients requiring intubation had significantly lower venous pH [7.29 (7.24-7.33) vs 7.31 (7.28-7.36), P = .04] while venous PCO2 and HCO3- did not differ as compared to non-intubated patients. Intubation within 48 h of admission was associated with significantly lower venous pH [7.28 (7.24-7.30) vs 7.32 (7.28-7.37), P = .002] and higher PCO2 [72 (63-92) mm Hg vs 62 (52-75) mm Hg, P = .04]. Among 69 patients receiving NIV, there were no differences in venous pH [7.29 (7.25-7.31) vs 7.30 (7.27-7.35), P = .3] or PCO2 [68 (44-74) mm Hg vs 70 (55-97) mm Hg, P = .23] associated with subsequent intubation. Using c statistics, we observed poor performances of venous pH, PCO2 or HCO3- for prediction of NIV failure, intubation or hospital mortality. CONCLUSIONS: Our results do not support the use of VBG on admission as a predictor for NIV failure, intubation and mortality during AHRF.