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.

Opportunities to Improve Utilization of Palliative Care Among Adults With Cystic Fibrosis: A Systematic Review.

CONTEXT: Individuals with cystic fibrosis (CF) frequently survive into adulthood, and many have multifaceted symptoms that impair quality of life. OBJECTIVE: We conducted a systematic review to investigate opportunities to improve utilization of palliative care among adults with CF. METHODS: We searched PubMed, Embase, Scopus, Web of Science, and CINAHL databases from inception until September 27, 2018, and reviewed references manually. Eligible articles were published in English, involved adults aged 18 years and older with CF, and contained original data regarding patient outcomes related to presence of advance care planning (ACP), symptom experience, and preferred and/or received end-of-life (EOL) care. RESULTS: We screened 652 article abstracts and 32 full-text articles; 12 studies met inclusion criteria. All studies were published between 2000 and 2018. Pertinent findings include that although 43% to 65% of adults with CF had contemplated completing ACP, the majority only completed ACP during their terminal hospital admission. Patients also reported high prevalence of untreated symptoms, with adequate symptom control reported in 45% among those with dyspnea, 22% among those with pain, and 51% among those with anxiety and/or depression. Prevalence of in-hospital death ranged from 62% to 100%, with a third dying in the intensive care unit. The majority received antibiotics and preventative treatments during their terminal hospitalization. Finally, treatment from a palliative care specialist was associated with a higher prevalence of patient completion of advanced directives, decreased likelihood of death in intensive care unit, and decreased use of mechanical ventilation at EOL. CONCLUSION: Adults with CF often have untreated symptoms, and many opportunities exist for palliative care specialists to improve ACP completion and quality of EOL care.

Increased prevalence of tracheal diverticula in cystic fibrosis patients.

OBJECTIVE: This investigation sought to evaluate the prevalence and imaging characteristics of tracheal diverticula (TD) among patients with cystic fibrosis (CF). METHODS: A total of 113 patients with CF at our institution, with a median age of 29 years, had chest CT examinations between 2002 and 2014. These imaging studies were retrospectively reviewed to assess for the presence and characteristics of TD, including quantity, size and location. The severity of the CF disease was assessed using the Bhalla CT scoring system and pulmonary function tests. RESULTS: Of the 113 cases reviewed, 20 (17.7%) patients were found to have 1 or more TD. The presence of TD was associated with more severe disease by imaging criteria, with a Bhalla CT score of 13.9 ± 4.3 in patients with TD and 11.5 ± 4.3 in patients without TD. For the pulmonary function tests, forced expiratory volume in 1 s (FEV1) and FEV1 percent predicted demonstrated a trend towards worsening function in patients with TD, although the difference was not statistically significant. CONCLUSION: The prevalence of TD in our patient population with CF based on chest CT exams was 17.7%. In addition, the presence of TD was associated with more severe CF disease by imaging criteria. ADVANCES IN KNOWLEDGE: TD appear to have a higher prevalence in patients with CF than in the general population, are associated with more severe CF pulmonary disease by CT criteria and are frequently underreported by radiologists.