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1.
J Cyst Fibros ; 21(3): e176-e183, 2022 May.
Article in English | MEDLINE | ID: covidwho-1729878

ABSTRACT

BACKGROUND: People with cystic fibrosis (pwCF) may be at risk of complications from COVID-19 but the impact of COVID-19 on pwCF remains unknown. METHODS: We conducted a multicenter retrospective cohort study to assess the impact of the COVID-19 pandemic first wave on pwCF in the New York metropolitan area (NY) from March 1, 2020 to August 31, 2020. Objectives were to determine (1) the prevalence of COVID-19 by PCR and IgG antibody testing, (2) the clinical characteristics of COVID-19, (3) delay in routine outpatient care, and (4) the effect on anxiety and depression in pwCF. RESULTS: There were 26 COVID-19 cases diagnosed by PCR or antibody testing among the study cohort of 810 pwCF. The prevalence of COVID-19 by PCR (1.6%) and IgG antibody (12.2%) testing was low. 58% of cases were asymptomatic and 82% were managed at home. 8% were hospitalized and 1 person died. 89% of pwCF experienced delay in care. The prevalence of anxiety increased from 43% baseline to 58% during the pandemic (P<0.01). In post-hoc analysis, the proportion of patients with diabetes (38% versus 16%, P<0.01) and pancreatic insufficiency (96% versus 66%, P<0.01) were higher while CFTR modulator use was lower (46% versus 65%, P = 0.05) in pwCF who tested positive for COVID-19. CONCLUSIONS: The prevalence of COVID-19 among pwCF in NY during the pandemic first wave was low and most cases were managed at home. CFTR modulators may be protective. PwCF experienced delay in routine care and increased anxiety.


Subject(s)
COVID-19 , Cystic Fibrosis , COVID-19/diagnosis , COVID-19/epidemiology , Cystic Fibrosis/complications , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Humans , Immunoglobulin G , New York/epidemiology , Pandemics , Retrospective Studies
2.
J Immunol ; 208(4): 979-990, 2022 02 15.
Article in English | MEDLINE | ID: covidwho-1631932

ABSTRACT

Calprotectin is released by activated neutrophils along with myeloperoxidase (MPO) and proteases. It plays numerous roles in inflammation and infection, and is used as an inflammatory biomarker. However, calprotectin is readily oxidized by MPO-derived hypohalous acids to form covalent dimers of its S100A8 and S100A9 subunits. The dimers are susceptible to degradation by proteases. We show that detection of human calprotectin by ELISA declines markedly because of its oxidation by hypochlorous acid and subsequent degradation. Also, proteolysis liberates specific peptides from oxidized calprotectin that is present at inflammatory sites. We identified six calprotectin-derived peptides by mass spectrometry and detected them in the bronchoalveolar lavage fluid of children with cystic fibrosis (CF). We assessed the peptides as biomarkers of neutrophilic inflammation and infection. The content of the calprotectin peptide ILVI was related to calprotectin (r = 0.72, p = 0.01, n = 10). Four of the peptides were correlated with the concentration of MPO (r > 0.7, p ≤ 0.01, n = 21), while three were higher (p < 0.05) in neutrophil elastase-positive (n = 14) than -negative samples (n = 7). Also, five of the peptides were higher (p < 0.05) in the bronchoalveolar lavage fluid from children with CF with infections (n = 21) than from non-CF children without infections (n = 6). The specific peptides liberated from calprotectin will signal uncontrolled activity of proteases and MPO during inflammation. They may prove useful in tracking inflammation in respiratory diseases dominated by neutrophils, including coronavirus disease 2019.


Subject(s)
Bronchoalveolar Lavage Fluid/immunology , Cystic Fibrosis/immunology , Inflammation/immunology , Leukocyte L1 Antigen Complex/metabolism , Neutrophils/immunology , Peptides/metabolism , Respiratory System/metabolism , Child , Child, Preschool , Cystic Fibrosis/diagnosis , Female , Humans , Inflammation/diagnosis , Leukocyte L1 Antigen Complex/genetics , Leukocyte L1 Antigen Complex/immunology , Male , Neutrophil Activation , Oxidation-Reduction , Peptides/genetics , Peptides/immunology , Proteolysis
4.
J Cyst Fibros ; 20 Suppl 3: 57-63, 2021 12.
Article in English | MEDLINE | ID: covidwho-1587342

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) programs and people with CF (PwCF) employed various monitoring methods for virtual care during the COVID-19 pandemic. This paper characterizes experiences with remote monitoring across the U.S. CF community. METHODS: The CF Foundation (CFF) sponsored distribution of home spirometers (April 2020 to May 2021), surveys to PwCF and CF programs (July to September 2020), and a second program survey (April to May 2021). We used mixed methods to explore access, use, and perspectives regarding the use of remote monitoring in future care. RESULTS: By October 2020, 13,345 spirometers had been distributed, and 19,271 spirometers by May 2021. Programs (n=286) estimated proportions of PwCF with home devices increased over seven months: spirometers (30% to 70%), scales (50% to 70%), oximeters (5% to 10%) with higher estimates in adult programs for spirometers and oximeters. PwCF (n=378) had access to scales (89%), followed by oximeters (48%) and spirometers (47%), often using scales and oximeters weekly, and spirometers monthly. Over both surveys, some programs had no method to collect respiratory specimens for cultures associated with telehealth visits (47%, n=132; 41%, n=118). Most programs (81%) had a process for phlebotomy associated with a telehealth visit, primarily through off-site labs. Both PwCF and programs felt future care should advance remote monitoring and recommended improvements for access, training, and data collection systems. CONCLUSIONS: PwCF and programs experienced unprecedented access to remote monitoring and raised its importance for future care. Improvements to current systems may leverage these shared experiences to augment future care models.


Subject(s)
COVID-19 , Cystic Fibrosis , Equipment and Supplies/supply & distribution , Home Care Services , Monitoring, Physiologic/methods , Spirometry , Adult , COVID-19/epidemiology , COVID-19/prevention & control , Child , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Delivery of Health Care/organization & administration , Delivery of Health Care/trends , Health Services Accessibility/organization & administration , Health Services Accessibility/standards , Home Care Services/organization & administration , Home Care Services/standards , Humans , Models, Organizational , Needs Assessment , Oximetry/instrumentation , Oximetry/methods , Quality Improvement , SARS-CoV-2 , Spirometry/instrumentation , Spirometry/methods , Telemedicine/methods , Telemedicine/standards , United States/epidemiology
5.
Pediatr Pulmonol ; 57(1): 278-284, 2022 01.
Article in English | MEDLINE | ID: covidwho-1561574

ABSTRACT

INTRODUCTION: The coronavirus disease 2019 (COVID-19) pandemic has accelerated the move towards home spirometry monitoring, including in children. The aim of this study is to determine whether the remote supervision of spirometry by a physiologist improves the technical quality and failure rate of the maneuvers. METHOD: Children with cystic fibrosis who had been provided with NuvoAir home spirometers were randomly allocated to either supervised or unsupervised home spirometry following a detailed training session. Home spirometry was performed every 2 weeks for 12 weeks. Tests were assigned a quality factor (QF) using our laboratory grading system as per American Thoracic Society/European Respiratory Society standards, with tests marked from A to D, or Fail. In our laboratory, we aim for QF A in all spirometry tests, but report results of QF B or C with a cautionary note. QF A was, therefore, the primary outcome, and QF A-C, the secondary outcome. RESULTS: Sixty-one patients were enrolled; 166 measurements were obtained in the supervised group, and 153 in the unsupervised group. Significantly more measurements achieved QF A in the supervised compared to unsupervised group (89% vs. 74%; p = <0.001), while proportions reaching Grade A-C were similar (99% vs. 95%; p = 0.1). All significant declines in spirometry results had a clinical rather than technical reason. Family/patient feedback for both arms was very positive. CONCLUSION: These results suggest that home spirometry in children should ideally be remotely supervised by a physiologist, but acceptable results can be obtained if resources do not allow this, provided that training is delivered and results monitored according to our protocol.


Subject(s)
COVID-19 , Cystic Fibrosis , Child , Cystic Fibrosis/diagnosis , Humans , Monitoring, Physiologic , SARS-CoV-2 , Spirometry
6.
Trials ; 22(1): 578, 2021 Aug 28.
Article in English | MEDLINE | ID: covidwho-1376593

ABSTRACT

The SARS-CoV-2 pandemic has disrupted clinical trials worldwide. The European Cystic Fibrosis Society-Clinical Trials Network (ECFS-CTN) has tracked clinical trial disruption by surveying its 58 trial sites across 17 European countries and collated information on measures to mitigate the impact of the pandemic and ensure trial continuity. Here, we present recommendations on how to reduce the risk of SARS-CoV-2 exposure to patients and trial staff by implementing remote trial visits where possible, using home assessments, video and phone calls, electronic consent, and home delivery of study drugs. We discuss the practicalities of remote source data verification, protocol amendments, changing trial site location, and staff absences and home working. We outline recommendations on how to protect trial outcomes, including home assessments, safety reporting, protocol deviations, and recruitment challenges. Finally, we discuss the importance of continued access to study drugs via extension trials for some patients. This guidance was co-created from the shared knowledge and experience of sites in our network and was re-distributed directly to all ECFS-CTN sites to help mitigate the impact of further waves of the SARS-CoV-2 pandemic. We will also use this guidance to assist companies, academia, and consortia with future protocol design and risk mitigation plans. This guidance can be applied to clinical trials in other diseases and could help sites that are not supported by clinical trial networks.


Subject(s)
COVID-19 , Cystic Fibrosis , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Europe , Humans , Pandemics , SARS-CoV-2
7.
BMJ Open Qual ; 10(3)2021 08.
Article in English | MEDLINE | ID: covidwho-1373969

ABSTRACT

IntroductionThe Cystic Fibrosis (CF) Foundation chronic care guidelines recommend monitoring spirometry during quarterly multidisciplinary visits to identify early lung function decline. During the COVID-19 pandemic, the CF adult clinic at University of Virginia (UVA) transitioned from the classic CF care model to a model that included quarterly multidisciplinary telemedicine visits. While using telemedicine, CF care needed to include spirometry monitoring. Only a fraction of adult CF patients at UVA owned and used home spirometers (HS) in March 2020. AIM: The specific aims of this quality improvement (QI) project were to increase the percentage of eligible adult CF patients who owned an HSs from 37% to 85% and to increase the percentage of adult CF patients seen at UVA with available spirometry in telemedicine from 50% to 95% by 31 December 2020. METHODS: Following the Model for Improvement QI methodology, a standardised process was developed for monitoring forced expiratory volume in 1 s with HS during multidisciplinary telemedicine visits during the COVID-19 pandemic. INTERVENTION: (1) HSs were distributed to eligible patients and (2) Home spirometry was monitored in eligible patients with each telemedicine visit and results were used for clinical care decisions. RESULTS: Both specific aims were achieved ahead of expected date. In March 2020, the beginning of the pandemic, 37% (49/131) of patients owned an HS and 50% (9/18) of patients seen via telemedicine performed spirometry at home. By September 2020, 97% (127/131) of adult patients at UVA owned an HS and by October 2020, 96% (24/25) of patients provided spirometry results during their telemedicine encounters. CONCLUSION: Employing QI tools to standardise the process of monitoring spirometry data with home devices via telemedicine is reliable and sustainable and can be replicated across centres that provide care for patients with CF.


Subject(s)
COVID-19 , Cystic Fibrosis , Telemedicine , Adult , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Humans , Pandemics , Quality Improvement , SARS-CoV-2 , Spirometry
8.
Acta Biomed ; 91(3): e2020035, 2020 09 07.
Article in English | MEDLINE | ID: covidwho-982594

ABSTRACT

The novel coronavirus SARS-CoV-2 was first identified in China in December 2019 and has since spread worldwide. People with Cystic Fibrosis (CF) have reduced survival mainly because of respiratory failure due to chronic pulmonary infections. Therefore, CF patients should be considered to have an increased risk of developing severe manifestations in case of SARS-CoV-2 infection. Surprisingly, the results of recent studies concerning SARS-CoV-2 infection in patients with CF show that in these patients the infection rate was lower than that of the general population. Various factors have been considered to explain a possible protective effect of CF against SARS-CoV-2 infection.


Subject(s)
Betacoronavirus , Coronavirus Infections/epidemiology , Cystic Fibrosis/epidemiology , Lung/diagnostic imaging , Pandemics , Pneumonia, Viral/epidemiology , Tomography, X-Ray Computed/methods , COVID-19 , Comorbidity , Cystic Fibrosis/diagnosis , Humans , SARS-CoV-2
9.
Chest ; 158(6): 2502-2510, 2020 12.
Article in English | MEDLINE | ID: covidwho-956971

ABSTRACT

To reduce the spread of the severe acute respiratory syndrome coronavirus 2, many pulmonary function testing (PFT) laboratories have been closed or have significantly reduced their testing capacity. Because these mitigation strategies may be necessary for the next 6 to 18 months to prevent recurrent peaks in disease prevalence, fewer objective measurements of lung function will alter the diagnosis and care of patients with chronic respiratory diseases. PFT, which includes spirometry, lung volume, and diffusion capacity measurement, is essential to the diagnosis and management of patients with asthma, COPD, and other chronic lung conditions. Both traditional and innovative alternatives to conventional testing must now be explored. These may include peak expiratory flow devices, electronic portable spirometers, portable exhaled nitric oxide measurement, airwave oscillometry devices, and novel digital health tools such as smartphone microphone spirometers and mobile health technologies along with integration of machine learning approaches. The adoption of some novel approaches may not merely replace but could improve existing management strategies and alter common diagnostic paradigms. With these options comes important technical, privacy, ethical, financial, and medicolegal barriers that must be addressed. However, the coronavirus disease 19 pandemic also presents a unique opportunity to augment conventional testing by including innovative and emerging approaches to measuring lung function remotely in patients with respiratory disease. The benefits of such an approach have the potential to enhance respiratory care and empower patient self-management well beyond the current global pandemic.


Subject(s)
COVID-19 , Delivery of Health Care/methods , Lung Diseases/diagnosis , Lung Diseases/therapy , Respiratory Function Tests/instrumentation , Respiratory Function Tests/methods , Asthma/diagnosis , Asthma/physiopathology , Asthma/therapy , Breath Tests/instrumentation , Breath Tests/methods , Chronic Disease , Cystic Fibrosis/diagnosis , Cystic Fibrosis/therapy , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy , Inventions , Lung Diseases/physiopathology , Lung Diseases, Interstitial/diagnosis , Lung Diseases, Interstitial/therapy , Lung Volume Measurements , Machine Learning , Oscillometry/instrumentation , Oscillometry/methods , Peak Expiratory Flow Rate , Pulmonary Diffusing Capacity/instrumentation , Pulmonary Diffusing Capacity/methods , Pulmonary Disease, Chronic Obstructive/diagnosis , Pulmonary Disease, Chronic Obstructive/physiopathology , Pulmonary Disease, Chronic Obstructive/therapy , Self-Management , Smartphone , Spirometry/instrumentation , Spirometry/methods
10.
Eur J Pediatr ; 180(4): 1313-1316, 2021 Apr.
Article in English | MEDLINE | ID: covidwho-871468

ABSTRACT

The way results of cystic fibrosis (CF) newborn screening are communicated to parents is critical yet is done differently across the globe. We surveyed parents of 101 children in our tertiary London paediatric centre with a 48% response rate. Parental responses were as follows: 40/42 (95%) said the information could not have been given over the phone and 39/43 (91%) said they wanted both partners present; 27/42 (64%) said it was helpful having the health visitor also present; and 37/40 (92%) felt it was acceptable to wait until the next day for the sweat test. We have reduced the time from first contact to arriving in the home to 2-3 h.Conclusion: We believe that this survey backs up our approach of a home visit by a CF nurse specialist with the family's health visitor to break the news. This is challenging in the current COVID-19 pandemic. What is Known: • Breaking bad news can have a lasting impact on parents when not done the right way. • Giving results of cystic fibrosis (CF) newborn screening is done differently within the UK and around the world. What is New: • Our parental survey revealed that the majority (92%) believed this should be done face to face and not over the telephone. • There was a mixed response to whether the parents should be told the genotype (assuming the CF centre knew), and thus the CF diagnosis before the confirmatory sweat test was carried out.


Subject(s)
Attitude to Health , Cystic Fibrosis/diagnosis , Neonatal Screening , Parents/psychology , Professional-Family Relations , Truth Disclosure , Health Care Surveys , House Calls , Humans , Infant, Newborn
11.
Ital J Pediatr ; 46(1): 143, 2020 Oct 06.
Article in English | MEDLINE | ID: covidwho-818115

ABSTRACT

The Veneto region is one of the most affected Italian regions by COVID-19. Chronic lung diseases, such as chronic obstructive pulmonary disease (COPD), may constitute a risk factor in COVID-19. Moreover, respiratory viruses were generally associated with severe pulmonary impairment in cystic fibrosis (CF). We would have therefore expected numerous cases of severe COVID-19 among the CF population. Surprisingly, we found that CF patients were significantly protected against infection by SARS-CoV-2. We discussed this aspect formulating some reasonable theories.


Subject(s)
Coronavirus Infections/epidemiology , Cystic Fibrosis/epidemiology , Pandemics/statistics & numerical data , Pneumonia, Viral/epidemiology , Pulmonary Disease, Chronic Obstructive/epidemiology , Adult , COVID-19 , COVID-19 Testing , Clinical Laboratory Techniques/methods , Cohort Studies , Coronavirus Infections/diagnosis , Coronavirus Infections/prevention & control , Cystic Fibrosis/diagnosis , Female , Humans , Incidence , Italy/epidemiology , Male , Middle Aged , Pandemics/prevention & control , Pneumonia, Viral/prevention & control , Pulmonary Disease, Chronic Obstructive/diagnosis , Real-Time Polymerase Chain Reaction/methods , Reference Values , Retrospective Studies , Risk Assessment
12.
Curr Opin Pulm Med ; 26(6): 696-701, 2020 11.
Article in English | MEDLINE | ID: covidwho-780547

ABSTRACT

PURPOSE OF REVIEW: The current review provides an overview of key psychological issues and challenges for the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator era of care. It discusses research from diagnosis and beyond, to patient-team communication with a particular focus on medical trials, adherence and living with CFTR modulators. RECENT FINDINGS: The impact of the diagnosis on parents is immense and the complexity of treatment now and in the future, are a challenge for both parents and teams. Communicating digitally is starting to become daily practice for many in CF care, with coronavirus disease 2019 accelerating this process. Participating in trials has a psychological impact, but most of all the (delayed) access and timing of accessing CFTR modulators is an important theme. Adherence remains of significance, both to 'old' and 'new' treatments. Living with CF in the era of CFTR modulators is beginning to impact on patients' quality of life, including new possibilities, opportunities and challenges. SUMMARY: Psychological care needs to engage and keep pace with the rapid medical changes. Some care priorities remain the same, including psychological screening and assessment, as well as psychoeducation, communication training and psychotherapy. The presence of CF psychologist in the CF clinic remains as important as ever.


Subject(s)
Communication , Coronavirus Infections , Cystic Fibrosis/drug therapy , Cystic Fibrosis/psychology , Medication Adherence , Pandemics , Pneumonia, Viral , Betacoronavirus , COVID-19 , Clinical Trials as Topic/psychology , Cystic Fibrosis/diagnosis , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Humans , Quality of Life , SARS-CoV-2
14.
J Cyst Fibros ; 19(6): 872-874, 2020 11.
Article in English | MEDLINE | ID: covidwho-704534

ABSTRACT

BACKGROUND: In Belgium, COVID-19 epidemy began on February 4, 2020 with a peak on April 10, 2020. Patients with cystic fibrosis (CF) followed in the Cliniques universitaires Saint-Luc were rapidly isolated before the government lockdown. METHODS: After the peak of the epidemy, we measured anti-SARS-CoV-2 IgM and IgG antibodies in 149 patients and collected clinical data. RESULTS: Only 3 asymptomatic patients presented IgG against the virus. In one patient hospitalized for COVID-19 (positive molecular testing), we did not detect any anti-SARS-CoV-2 antibodies, as in thirty-five other symptomatic patients considered as possible cases. CONCLUSIONS: Even if respiratory symptoms linked to CF are frequent and compatible with COVID-19, anti-SARS-CoV-2 IgG antibodies were detected only in 3 asymptomatic patients. This reassuring study concerning the risk of COVID-19 in patients with CF illustrates the difficulty to distinguish COVID-19 symptoms from respiratory exacerbations and the need of generalized molecular testing to make a precise diagnosis.


Subject(s)
Antibodies, Viral/analysis , COVID-19 , Communicable Disease Control/methods , Cystic Fibrosis , SARS-CoV-2 , Adult , Asymptomatic Infections/epidemiology , Belgium/epidemiology , COVID-19/epidemiology , COVID-19/immunology , COVID-19/prevention & control , COVID-19/therapy , COVID-19 Serological Testing/methods , COVID-19 Serological Testing/statistics & numerical data , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Diagnosis, Differential , Female , Hospitalization/statistics & numerical data , Humans , Male , Outcome Assessment, Health Care , Risk Assessment , SARS-CoV-2/immunology , SARS-CoV-2/isolation & purification , Seroepidemiologic Studies
15.
Infect Dis Health ; 25(4): 239-241, 2020 11.
Article in English | MEDLINE | ID: covidwho-665894

ABSTRACT

We report the first case of COVID-19 in a pregnant patient with cystic fibrosis. We describe the diagnosis, clinical course and management of the patient and their family with regards to clinical, social and infection control measures around delivery. This case highlights the importance of the cooperation of multidisciplinary teams to achieve good clinical outcomes in complex patients with COVID-19.


Subject(s)
Coronavirus Infections/complications , Cystic Fibrosis/virology , Pneumonia, Viral/complications , Pregnancy Complications, Infectious/virology , Adult , Betacoronavirus/isolation & purification , COVID-19 , Coronavirus Infections/transmission , Cystic Fibrosis/diagnosis , Delivery, Obstetric , Female , Humans , Infectious Disease Transmission, Vertical , Male , Pandemics , Pneumonia, Viral/transmission , Pregnancy , Pregnancy Complications, Infectious/diagnosis , Pregnancy Complications, Infectious/physiopathology , Pregnancy Outcome , SARS-CoV-2 , Transgender Persons
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