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1.
BMC Pulm Med ; 22(1): 101, 2022 Mar 24.
Article in English | MEDLINE | ID: covidwho-1759734

ABSTRACT

BACKGROUND: In Germany, the first case of coronavirus disease 2019 (COVID-19) was registered on 28 January 2020. By February 2021, the third wave of the pandemic began. So far, only few data are available on the SARS-CoV-2 prevalence and the clinical impact of an infection in patients with cystic fibrosis (CF). METHODS: From February 2020 until March 2021, we screened 156 CF patients for anti-SARS-CoV-2 IgG antibodies (serology) and the presence of SARS-CoV-2 in deep throat saliva or nasopharyngeal swabs (molecular testing). From patients with confirmed SARS-CoV-2 infection, we recorded symptoms and collected clinical data. RESULTS: In total, 13 patients (8.3%) were tested positive for SARS-CoV-2 infection, most of them during the second and the beginning third wave of the pandemic. Ten positive tested patients described symptoms linked to COVID-19. The most common symptom was cough followed by fatigue and headache. SARS-CoV-2 infection did not impair lung function. No positive tested patient needed to be hospitalized. CONCLUSIONS: SARS-CoV-2 infections in patients with CF are not as rare as initially anticipated, as frequent testing revealed. However, infected patients did not experience more severe clinical courses or worse clinical outcome. Our observation is in line with published reports indicating that individuals with CF are not at higher risk for severe COVID-19.


Subject(s)
COVID-19/epidemiology , Cystic Fibrosis/complications , Adolescent , Adult , Antibodies, Viral/blood , COVID-19/complications , Cystic Fibrosis/physiopathology , Female , Germany/epidemiology , Humans , Incidence , Lung/physiopathology , Male , Pulmonary Ventilation , SARS-CoV-2/immunology
2.
Int J Environ Res Public Health ; 19(6)2022 03 11.
Article in English | MEDLINE | ID: covidwho-1753481

ABSTRACT

The aims of this study were to (1) determine the feasibility of a home-based resistance exercise training (RET) program in patients with cystic fibrosis and impaired glucose tolerance using virtual personal training and (2) observe the effects completion of the RET program had on glucose metabolism, pulmonary function, body composition, and physical fitness. The feasibility of the program was defined as 80% compliance. Ten participants (15.80 ± 2.20 yr, 25.1 ± 7.4 kg/m2) began a home-based resistance training program consisting of 36 sessions supervised via online videoconferencing. Compliance scores of 78.9% (all participants) and 81.8% (without one outlier) were observed. A significant increase was observed in 2-h C-peptide levels (2.1 ng/mL; p = 0.04), with a moderate decrease in fasting glucose (-5.2 mg/dL; p = 0.11) and a moderate increase in 2-h insulin (35.0 U/mL; p = 0.10). A small decrease in the fat percentage (-1.3%; p = 0.03) was observed in addition to increases in fat-free mass (1.5 kg; p = 0.01) and the fat-free mass index (0.4; p = 0.01). Small, yet statistically significant increases were observed in V̇O2peak (0.1 L/min p = 0.01), V̇CO2peak (0.1 L/min; p = 0.01), and ventilation (5.3 L/min; p = 0.04). Telehealth-based RET is feasible in adolescents with CF and impaired glucose tolerance and elicits small yet favorable changes in insulin secretion, body composition, and exercise capacity.


Subject(s)
Cystic Fibrosis , Glucose Intolerance , Resistance Training , Telemedicine , Adolescent , Cystic Fibrosis/therapy , Feasibility Studies , Glucose Intolerance/therapy , Humans
3.
Cells ; 11(6)2022 03 12.
Article in English | MEDLINE | ID: covidwho-1742342

ABSTRACT

Over the past decades, a better understanding of the genetic and molecular alterations underlying several respiratory diseases has encouraged the development of new therapeutic strategies. Gene therapy offers new therapeutic alternatives for inherited and acquired diseases by delivering exogenous genetic materials into cells or tissues to restore physiological protein expression and/or activity. In this review, we review (1) different types of viral and non-viral vectors as well as gene-editing techniques; and (2) the application of gene therapy for the treatment of respiratory diseases and disorders, including pulmonary arterial hypertension, idiopathic pulmonary fibrosis, cystic fibrosis, asthma, alpha-1 antitrypsin deficiency, chronic obstructive pulmonary disease, non-small-cell lung cancer, and COVID-19. Further, we also provide specific examples of lung-targeted therapies and discuss the major limitations of gene therapy.


Subject(s)
COVID-19 , Carcinoma, Non-Small-Cell Lung , Cystic Fibrosis , Lung Neoplasms , COVID-19/genetics , COVID-19/therapy , Cystic Fibrosis/metabolism , Humans , Lung/metabolism
5.
Clin Transplant ; 36(4): e14634, 2022 Apr.
Article in English | MEDLINE | ID: covidwho-1731123

ABSTRACT

There has been a shift over decades in the diagnostic indications for lung transplantation in children; in particular, there has been a reduction in the proportion of pediatric cystic fibrosis (CF) patients undergoing lung transplantation early in life, and more transplants occurring in other diagnostic groups. Here, we examine trends in pediatric lung transplantation with regards to indications by analyzing data from the United Network of Organ Sharing, the International Society for Heart and Lung Transplantation Thoracic Transplant Registry, and other sources. Over the past two years, there has been a precipitous decline in both the number of transplants due to CF and the proportion of CF cases relative to the total number of transplants, likely not solely due to the COVID-19 pandemic. In 2020, primary pulmonary arterial hypertension for the first-time surpassed CF as main indication for pediatric lung transplantation in the United States, a finding that is also reflected in international data. We discuss the effect of novel CFTR modulator therapies as a major factor leading to this shifting landscape. Based on our trending, pulmonary hypertension-related diagnoses and pediatric interstitial lung diseases are rising indications, for which we suggest adjustments of consensus guidelines around candidate selection criteria.


Subject(s)
COVID-19 , Cystic Fibrosis , Heart Transplantation , Heart-Lung Transplantation , Lung Transplantation , COVID-19/epidemiology , Child , Cystic Fibrosis/surgery , Humans , Lung Transplantation/adverse effects , Pandemics , Survival Rate , Tissue Donors , United States
6.
Indian J Pediatr ; 89(4): 404-406, 2022 04.
Article in English | MEDLINE | ID: covidwho-1653777

ABSTRACT

Children with cystic fibrosis (CF) constitute a high-risk group for COVID-19 with underlying chronic lung disease. COVID-19 severity varying from mild infection to need of intensive care has been described in children with CF. Two children with significant underlying pulmonary morbidity are described here, who developed severe disease following SARS-CoV-2 infection. Case 1 (a 9-y-old boy) had pneumonia with respiratory failure requiring noninvasive ventilation support. He had delayed clearance of SARS-CoV-2, with recurrence of symptomatic disease with short asymptomatic period in between. He was also diagnosed with CF-related diabetes and allergic bronchopulmonary aspergillosis during the second episode. Case 2 (an 18-mo-old boy) had two episodes of SARS-CoV-2-related severe lower respiratory infection within a period of 2 mo, requiring high-flow nasal oxygen support. Both children had 3rd pulmonary exacerbation but SARS-CoV-2 was not detected in respiratory secretions. To conclude, children with CF with underlying pulmonary morbidity, can develop severe COVID-19 and prolonged SARS-CoV-2 shedding.


Subject(s)
COVID-19 , Cystic Fibrosis , COVID-19/complications , Child , Cystic Fibrosis/complications , Humans , Lung , Male , Respiration, Artificial , SARS-CoV-2
7.
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
8.
Pediatr Pulmonol ; 57(4): 1072-1075, 2022 04.
Article in English | MEDLINE | ID: covidwho-1630763

ABSTRACT

Primary ciliary dyskinesis (PCD) is an autosomal recessive disorder associated with impaired mucociliary clearance caused by defects in ciliary structure and function. The major clinical feature of PCD is recurring or persistent respiratory tract infection. Respiratory tract colonization with drug-resistant organisms impacts the frequency of infections and lung function decline. Protective gear has been employed by caregivers in an attempt to control respiratory tract bacterial spread between patients with cystic fibrosis, but use in PCD is not known. We conducted a web-based survey to investigate infection control and prevention practices of PCD centers in North America, and how practices have been influenced by the COVID-19 pandemic. The response rate was 87.0%. Before the COVID-19 pandemic, glove, gown, and mask use were variable, and only 3.7% of centers used masks during encounters with PCD outpatients. After COVID-19 mandates are lifted, 48.1% of centers plan to continue to use masks during outpatient care, while the practice regarding the use of gloves and gowns was not influenced by the current pandemic. There is no uniform practice for infection control in PCD care indicating the need for practice guidelines. Mitigation of respiratory virus transmission learned during the COVID-19 pandemic may impact future infection control approaches used for patients with PCD and other lung diseases.


Subject(s)
COVID-19 , Ciliary Motility Disorders , Cystic Fibrosis , Kartagener Syndrome , COVID-19/prevention & control , Ciliary Motility Disorders/complications , Cystic Fibrosis/complications , Humans , Infection Control , Kartagener Syndrome/complications , Kartagener Syndrome/therapy , Pandemics/prevention & control , Patient Care
11.
J Cyst Fibros ; 20 Suppl 3: 47-48, 2021 12.
Article in English | MEDLINE | ID: covidwho-1587348

ABSTRACT

The COVID-19 pandemic has accelerated the use of telehealth within the cystic fibrosis (CF) community to deliver CF care. The article by Solomon and colleagues exploring the patient and family experiences of telehealth care delivery, as part of the CF chronic care model in the US, is therefore timely. In this commentary, we discuss how the US experience of telehealth care compares with reports from CF centres in other parts of the world. We highlight the potential challenges, including whether the inverse care law will apply in this new era of CF telehealth.


Subject(s)
COVID-19 , Cystic Fibrosis , Telemedicine , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Humans , Pandemics , SARS-CoV-2
15.
J Cyst Fibros ; 20 Suppl 3: 31-38, 2021 Dec.
Article in English | MEDLINE | ID: covidwho-1587346

ABSTRACT

BACKGROUND: Depression and anxiety are two to four times more prevalent in people with CF (pwCF) than the general population. COVID-19 may exacerbate mental health challenges, increasing demand for psychological services, while decreasing their availability. We assessed the impact of the pandemic on depression and anxiety in pwCF, including how COVID-19 affected the frequency of mental health screening and the types of services provided. METHODS: A 38-item internet survey, completed in June 2020, assessed how COVID-19 affected: 1) the mental health clinician's role and screening processes; 2) barriers to screening and resource needs; 3) impact of COVID-19 on depression and anxiety, and 4) positive outcomes and confidence in sustaining mental health screening and treatment, including telehealth services, after the pandemic. RESULTS: Responses were obtained from 131 of the 289 US CF programs. Overall, 60% of programs (n=79) continued mental health screening and treatment, although less frequently; 50% provided individual tele-mental health interventions, and 9% provided telehealth group therapy. Clinically elevated depression symptoms (PHQ-9≥10; moderate to severe), were found in 12% of 785 pwCF, with 3.1% endorsing suicidal ideation. Similarly, elevated anxiety (moderate to severe; GAD-7≥10) was found in 13% of pwCF (n=779). CONCLUSIONS: The COVID-19 pandemic created an opportunity to implement innovative solutions to disruptions in mental health screening and treatment in CF programs. We found that pwCF had increased access to psychological interventions during the pandemic via telehealth, supporting the continued integration of tele-mental health screening and treatment into CF care.


Subject(s)
Anxiety , COVID-19 , Cystic Fibrosis , Depression , Mental Health , Psychosocial Intervention , Telemedicine , Anxiety/diagnosis , Anxiety/physiopathology , Anxiety/therapy , COVID-19/epidemiology , COVID-19/prevention & control , COVID-19/psychology , Cystic Fibrosis/epidemiology , Cystic Fibrosis/psychology , Cystic Fibrosis/therapy , Delivery of Health Care/methods , Delivery of Health Care/trends , Depression/diagnosis , Depression/physiopathology , Depression/therapy , Health Services Accessibility/standards , Health Services Accessibility/statistics & numerical data , Humans , Mass Screening/methods , Needs Assessment , Psychosocial Intervention/methods , Psychosocial Intervention/trends , Psychosocial Support Systems , SARS-CoV-2 , Surveys and Questionnaires , Telemedicine/methods , Telemedicine/organization & administration , United States/epidemiology
16.
J Cyst Fibros ; 20 Suppl 3: 3-8, 2021 12.
Article in English | MEDLINE | ID: covidwho-1587345

ABSTRACT

BACKGROUND: Novel therapies have dramatically changed cystic fibrosis (CF) and innovative care delivery systems are needed to meet future patient needs. Telehealth has been shown to be an efficient and desirable form of care delivery. The COVID-19 pandemic caused a rapid shift to telehealth, and this presented a unique opportunity to study facilitators, barriers, and satisfaction with this mode of care delivery. We aim to report survey methods, demographics and telehealth use among CF care programs, patients, and families during the pandemic. METHODS: CF programs completed two surveys between July 29 and September 18, 2020, and between April 19 and May 19, 2021. Patients and families completed a similar survey between August 31 and October 30, 2020. The surveys addressed topics assessing the pandemic's financial impact, telehealth modes and experiences, licensure and reimbursement issues, health screening, and remote monitoring. Quantitative data were analyzed with descriptive statistics and were compared to the CF Foundation Patient Registry. RESULTS: Most programs (278 at timepoint one and 274 at timepoint two) provided telehealth during the pandemic. The percent of visits containing either telephone or video components changed from 45% to 25% over the time periods. Additionally, 424 patients and families from various ages and backgrounds responded to the survey and 81% reported having a telehealth visit. CONCLUSIONS: The pandemic accelerated telehealth adoption and these datasets are a valuable source for exploring telehealth barriers and facilitators, the quality-of-care experience, financial and workforce implications, the impact on underrepresented populations, and implications for coverage and reimbursement.


Subject(s)
COVID-19 , Cystic Fibrosis , Health Services Accessibility , Telemedicine , Adult , COVID-19/epidemiology , COVID-19/prevention & control , Child , Communicable Disease Control/methods , Communication Barriers , Continuity of Patient Care , Costs and Cost Analysis , Cystic Fibrosis/epidemiology , Cystic Fibrosis/psychology , Cystic Fibrosis/therapy , Female , Health Services Accessibility/organization & administration , Health Services Accessibility/trends , Humans , Male , Organizational Innovation , Patient Satisfaction/statistics & numerical data , Quality of Health Care , SARS-CoV-2 , Telemedicine/organization & administration , Telemedicine/standards , United States/epidemiology
18.
J Cyst Fibros ; 20 Suppl 3: 9-13, 2021 12.
Article in English | MEDLINE | ID: covidwho-1587343

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) care programs in the United States rapidly adopted telehealth during the COVID-19 pandemic. Understanding factors that promote or impede telehealth will inform planning for future telehealth-enabled care models. METHODS: Adult, pediatric, and affiliate CF care programs in the United States (n = 287) were surveyed twice eight months apart in 2020-2021 about telehealth use. Programs were asked to describe barriers to and promoters of telehealth. RESULTS: Ninety-seven percent of programs provided telehealth services. In the first CF Care Program State of Care Survey (SoC1), programs estimated that 57% of patients exclusively received in-person care, 36% of patients received telehealth by phone/computer with video, and 8% of patients received telephone-only care. In the second CF Care Program State of Care Survey (SoC2), programs estimated that 80% of visits were in-person and 15% were via audio and video telehealth. Pediatric programs (21%) were less likely than adult (37%) or affiliate (41%) programs to recommend telehealth (p = 0.007). All programs ranked lack of internet access as the highest barrier to patient engagement with telehealth. Promoters of telehealth were increased accessibility and avoidance of infection transmission. Top ranked changes to improve telehealth were expanded provision of remote monitoring devices and technology access. Similar proportions of program types anticipated institutional telehealth expansion. CONCLUSION: During the COVID-19 pandemic, CF programs in the United States identified factors to improve future care delivery via telehealth. Targeting specific barriers and promoters will improve the use and quality of telehealth throughout the care center network.


Subject(s)
COVID-19 , Communication Barriers , Cystic Fibrosis , Disease Transmission, Infectious/prevention & control , Health Services Accessibility , Patient Participation , Telemedicine , Adult , COVID-19/epidemiology , COVID-19/prevention & control , Child , Cystic Fibrosis/epidemiology , Cystic Fibrosis/psychology , Cystic Fibrosis/therapy , Female , Health Services Accessibility/organization & administration , Health Services Accessibility/trends , Humans , Internet Access , Male , Needs Assessment , Patient Participation/methods , Patient Participation/psychology , Patient Satisfaction/statistics & numerical data , Quality Improvement , SARS-CoV-2 , Telemedicine/methods , Telemedicine/organization & administration , Telemedicine/standards , United States/epidemiology
19.
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
20.
J Cyst Fibros ; 20 Suppl 3: 1-2, 2021 12.
Article in English | MEDLINE | ID: covidwho-1587341

ABSTRACT

The findings of this body of work are presented in the eight articles included in this supplement. The impact and perspectives of adult and pediatric care teams and patient/families are covered with special attention to mental health care, the financial and personnel impacts within care programs, the experiences of vulnerable and underrepresented patient populations, and implementation of remoting monitoring. Commentaries from colleagues provide a broader perspective, offering reflections on the findings and their implications regarding the future CF care model.


Subject(s)
COVID-19 , Cystic Fibrosis , Delivery of Health Care, Integrated/organization & administration , Delivery of Health Care/trends , Telemedicine , COVID-19/epidemiology , COVID-19/prevention & control , Communicable Disease Control/methods , Continuity of Patient Care , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Health Services Accessibility/organization & administration , Health Services Accessibility/trends , Humans , Organizational Innovation , SARS-CoV-2 , Telemedicine/organization & administration , Telemedicine/standards , United States/epidemiology
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