Cystic fibrosis telemedicine in the era of COVID-19.

The coronavirus disease 2019 pandemic has resulted in large-scale changes to incorporate telemedicine for the delivery of care. People with cystic fibrosis (CF) have care considerations that pose challenges to telemedicine; they include frequent visits for pulmonary disease progression, medication management, and evaluation by a multidisciplinary team of providers. We share our center's experience with video visits replacing in-person clinic evaluation, using quality improvement strategies to create a replicable workflow. Key considerations include incorporation of the multidisciplinary team into the visit, limitations of remote delivery of care, as well as patient and staff perceptions of this care model. Results revealed that video visits were convenient, efficacious, and comparable to in-person visits, with interest for its continued incorporation into the traditional CF care model.

Cystic fibrosis and COVID-19: Care considerations.

The coronavirus disease 2019 (COVID-19) pandemic has demanded large scale changes in patient care. People with cystic fibrosis have unique considerations, including underlying lung disease and routine aerosolizing therapies, but there is insufficient evidence to create comprehensive practice guidelines. We share a case of a patient with CF and COVID-19 as well as alterations to routine CF care at a large academic center. Key considerations include accessible COVID-19 screening, augmented infection control practices, and rapid integration of telemedicine.

Skin Necrosis Distal to a Rapid Infusion Catheter: Understanding Possible Complications of Large-bore Vascular Access Devices.

Rapid infusion catheters (RICs) allow expedient conversion of peripheral intravenous (PIV) catheters to peripheral sheaths; however, little is known about potential complications. In this case, a 64-year-old male polytrauma patient had a 20-gauge PIV catheter in the right cephalic vein upsized to an 8.5 French (Fr) RIC without incident during an arrest with pulseless electrical activity (PEA). On RIC post-placement day two, the patient developed edema and ecchymosis extending from the right dorsal mid-hand to the antecubital fossa, just distal to the RIC insertion point. Compartments were soft; the volar surface (including an arterial line location), fingers, and upper arm were normal. The RIC flushed and returned blood appropriately. Ultrasound revealed a noncompressible cephalic vein either related to the catheter or thrombosis, and imaging of the hand showed an ulnar styloid fracture and a minimally displaced triquetral fracture. The RIC was removed immediately. Over the next week, the areas of ecchymosis developed bullae and then sloughed, leaving open wounds extending into the dermis. The patient later expired from unrelated causes. The area and timing of the skin necrosis were highly suspicious for a catheter-associated complication, despite the presence of the arterial line and small distal fractures. The necrosis was potentially due to thrombosis of the superficial venous outflow system, leading to congestion and skin compromise, but we found no similar reports. Alternatively, the catheter may have ruptured the vein and caused a gravity-dependent ecchymosis, but the volar surface was not impacted, and the catheter was functioning properly. The RIC may also have encroached on the arterial space, decreasing flow, but we would have expected distal hand changes. The only published reports we could find on RIC complications involved a lost guide wire, fragmentation of a catheter during placement, and a case of compartment syndrome, raising the question of whether skin necrosis is truly a rare event or simply underreported with the RIC. Although the exact causal relationship remains unknown in our case, RICs should be removed as soon as possible after immediate stabilization.

Correlations between cystic fibrosis genotype and sinus disease severity in chronic rhinosinusitis.

OBJECTIVE: Cystic fibrosis (CF) patients commonly develop chronic rhinosinusitis (CRS). The impact of the most common cystic fibrosis transmembrane conductance regulator (CFTR) mutation, F508del, on the severity of sinonasal disease remains inconclusive. The objective of this study is to evaluate the impact of CFTR genotype functional classification on sinonasal disease severity in patients with CRS. METHODS: Retrospective chart review of patients with CF who underwent endoscopic sinus surgery for chronic rhinosinusitis from 1998 to 2015. Patients were divided into high- or low-risk genotypes based on standardized CFTR gene functional classification. The primary outcome was the 22-item Sino-Nasal Outcome Test (SNOT-22) score. Secondary outcomes included endoscopic scores, extent of surgery performed, presence of polyposis, number of revision surgeries, and Lund-MacKay computed tomography scores. RESULTS: Thirty-eight patients harbored a high-risk CFTR genotype, and 11 had a low-risk genotype. On bivariate analysis, there was no association between CFTR genotype risk stratification and measures of preoperative disease severity or postoperative outcomes. There were no associations between genotype risk stratification and outcome variables on multivariate linear regression, adjusted for age and gender. There were significant improvements in several SNOT-22 subdomains before and after endoscopic sinus surgery (P < 0.05), but the magnitude of improvement was not significantly different on the basis of CFTR genotype risk stratification. CONCLUSION: High-risk CFTR genotypes are not associated with worse sinonasal disease severity or postoperative symptom control than low-risk CFTR genotypes after adjusting for confounding factors. LEVEL OF EVIDENCE: Level 4. Laryngoscope, 1752-1758, 2018.

Outpatient endoscopic sinus surgery in cystic fibrosis patients: predictive factors for admission.

BACKGROUND: An increasing number of adult patients with cystic fibrosis (CF) are becoming candidates for elective endoscopic sinus surgery (ESS). We sought to identify perioperative factors in this patient population that were predictive of postoperative admission. METHODS: Retrospective chart review of CF patients who underwent ESS during the years 2005 through 2012. Multiple preoperative, intraoperative, and immediate postoperative variables were analyzed. RESULTS: Thirty-three patients who underwent 37 outpatient ESSs were identified. Successful same-day discharge was observed in 54%. In 46% of cases, postoperative admission was necessary, with a mean postoperative stay of 1.4 days. Pulmonary function, CF-related comorbidities, and history of lung transplant were not predictors of postoperative admission. Univariate analysis demonstrated that patients were more likely to be admitted if they had 1 of the following conditions: history of ≥4 prior ESS; procedure duration >2.5 hours; intraoperative blood loss greater than 150 mL; increased immediate postoperative pain scores; or larger narcotic requirements for pain control. On logistic regression analysis, a maximum pain score ≥ 7 out of 10 in the postanesthesia recovery unit was the only significant predisposing factor for postoperative admission. CONCLUSION: Although over 50% of adult CF patients can successfully undergo ESS on a same-day discharge basis, it is prudent to have contingent plans for potential inpatient observation postoperatively. Multivariate analysis suggests that preoperative demographics and pulmonary status cannot predict the need for postoperative admission, whereas higher pain scores in the postanesthesia care unit are predictive of the necessity for inpatient observation.