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[Evidence summary:] What are the outcomes for people with cystic fibrosis who contract Covid-19? [v1.0]

dc.contributor.authorNational Health Library & Knowledge Service (NHLKS)
dc.contributor.authorDelaunois, Isabelle
dc.contributor.authorLeen, Brendan
dc.contributor.authorMcKeown, Declan
dc.date.accessioned2020-09-04T11:42:54Z
dc.date.available2020-09-04T11:42:54Z
dc.date.issued2020-09-04
dc.identifier.urihttp://hdl.handle.net/10147/628222
dc.descriptionBMJ Best Practice3 categorise cystic fibrosis patients as a high-risk population in terms of COVID-19. HSE guidance1 gives clear advice on how to keep well for patients who have contracted COVID-19. BMJ Best Practice3 recommends that for patients with known or suspected COVID-19, airway clearance should be carried out in a well-ventilated room, separate from other people, if possible, as it is a potentially infectious aerosol-generating procedure. Very little reliable research and data on clinical outcomes of cystic fibrosis patients who have contracted COVID-19 has yet been published. A multinational cohort study by Cosgriff et al5 concludes that there is good recovery from SARS-CoV-2 in the heterogeneous CF cohort investigated. The disease course does not seem to differ from that in the general population, but the current numbers are too small to draw firm conclusions, and people with CF should continue to follow public health advice to protect themselves from infection. Colombo et al4 base their recommendations on the 2009 influenza pandemic, H1N1 virus, which caused substantial morbidity in patients with cystic fibrosis and which, in a subgroup with severe lung disease, was associated with respiratory deterioration, mechanical ventilation and even death. As the clinical features of COVID-19 are distinct from the symptoms of cystic fibrosis, COVID-19 in people with cystic fibrosis should be clearly distinguishable, but it is suggested that mild disease might be categorised as being within the normal spectrum of symptoms for some cystic fibrosis patients. The authors recommend a low threshold for testing in the cystic fibrosis population. Several countries are now reporting COVID-19 case data of people with a confirmed diagnosis of CF to the European Cystic Fibrosis Society patient registry. The ECFS present a weekly update6 of centralised, anonymised data on CF admissions throughout Europe. The data is preliminary, incomplete, might change over time, and the number of cases is low; therefore, the information in the ECFS registry should not be used to direct clinical decisions. The data published on 20 May, 2020 shows that 41 patients in Europe were hospitalised (six in the ICU), and 2 patients required ventilation. 11 patients were asymptomatic. The top five symptoms were fever, increased cough, fatigue, increased sputum production and increased breathlessness. Siuba M et al7 looked at mortality in individuals with cystic fibrosis requiring invasive mechanical ventilation (IMV) for respiratory failure. They concluded that mortality per hospitalization in adults with cystic fibrosis who are not bridging to lung transplant and require emergent IMV is 44.5%, suggesting IMV is not futile and that these findings may help providers, families and patients with cystic fibrosis weigh the risks and benefits of IMV for respiratory failure. Farfour et al8 report the case of a SARS-CoV-2 infection in a lung-transplanted patient for cystic fibrosis characterized by several singularities. The case highlights three significant observations: (1) in its early stages, COVID-19 presents in a non-specific manner; (2) swab testing appears to be less sensitive among cystic fibrosis patients who present with primarily respiratory symptoms; (3) the identification of another respiratory pathogen should not exclude a SARS-CoV-2 infection, at least in patients presenting severe pathology. Poli et al9 briefly report a case of COVID-19 in an infant with CF in Italy. The patient never developed fever or any symptoms or signs of infection.en_US
dc.language.isoenen_US
dc.publisherHealth Service Executiveen_US
dc.relation.ispartofseriesEvidence summaries No. 104en_US
dc.subjectCORONAVIRUSen_US
dc.subjectCOVID-19en_US
dc.subjectCYSTIC FIBROSISen_US
dc.subjectRISK ASSESSMENTen_US
dc.title[Evidence summary:] What are the outcomes for people with cystic fibrosis who contract Covid-19? [v1.0]en_US
dc.typeOtheren_US
refterms.dateFOA2020-09-04T11:42:55Z


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