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1.
J Cyst Fibros ; 22(3): 456-463, 2023 May.
Article in English | MEDLINE | ID: covidwho-20234331

ABSTRACT

BACKGROUND: The COVID-19 pandemic resulted in the use of telehealth to deliver the cystic fibrosis (CF) care model, which recommends routine follow-up for monitoring of nutritional status, bacterial culture surveillance, pulmonary function testing, and screening for CF-related complications such as diabetes or osteoporosis. METHODS: The objective of this study was to use Cystic Fibrosis Foundation Patient Registry (CFFPR) data to quantify the extent to which persons with CF received the recommended components of the care model in 2019 versus 2020. A risk factor analysis was implemented to identify patient characteristics associated with attaining the recommended CF care and use of any telehealth using multivariable logistic regression. RESULTS: A total of 28,132 CFFPR participants were included in the study. The proportion of individuals meeting the recommendations for CF care was lower in 2020 for every indicator, and lower in adults compared to children. In adults, demographic, socioeconomic and CF-related disease covariates were significantly associated with both achieving an aggregate level of care and use of telehealth. In the pediatric population, minority race/ethnicity and markers of lower socioeconomic status were associated with a lower odds of telehealth use. In all analyses, having received the recommended level of care in 2019 was associated with a higher odds of both reported telehealth use and achieving the recommended elements of the CF care model in 2020. CONCLUSION: Fewer participants met recommendations for care in 2020 despite widespread use of telehealth, and use of telehealth did not equate to adherence to all aspects of CF care.


Subject(s)
COVID-19 , Cystic Fibrosis , Telemedicine , Adult , Humans , Child , United States/epidemiology , Cystic Fibrosis/diagnosis , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Pandemics , COVID-19/epidemiology , COVID-19/prevention & control , Cystic Fibrosis Transmembrane Conductance Regulator
2.
Pediatr Pulmonol ; 58(7): 2025-2034, 2023 Jul.
Article in English | MEDLINE | ID: covidwho-2300991

ABSTRACT

BACKGROUND: The COVID-19 pandemic has spread across the world, leading to government measures associated with a negative impact on mental health. The aim of this study was to evaluate the impact of the pandemic on depression, anxiety and resilience in Dutch people with cystic fibrosis (PwCF) or primary ciliary dyskinesia (PwPCD) and their caregivers. METHODS: Adolescents (12-17 years) and caregivers of children (0-17 years) with CF, and adolescents, adults and caregivers of children with PCD completed questionnaires on depression, anxiety and resilience between September 2020 and February 2021. The psychosocial impact of COVID-19 was measured by the Exposure and Family Impact Survey (CEFIS) Part 2. Mixed model analyses compared depression and anxiety results to participants' prepandemic scores. RESULTS: One hundred ten participants (10 PwCF, 31 PwPCD, 52 CF caregivers, 17 PCD caregivers) completed questionnaires during the pandemic. Prepandemic outcomes were available for 87 participants. The prevalence of symptoms of depression and anxiety (PHQ-9 or GAD-7 scores ≥5) in PwCF and PwPCD and their caregivers before and during the pandemic was high, with an increase in depression in PwCF (2.75; 95% confidence interval: 0.82-4.68) and increase in anxiety in CF caregivers (1.03; 0.09-1.96) during the pandemic. Resilience was within the normal range for all groups, CEFIS scores corresponded to a low to normal impact. CONCLUSION: PwCF and PwPCD and their caregivers were at risk of elevated depression and anxiety symptoms both before and during the pandemic, which emphasizes the importance of mental health screening and psychological care in CF and PCD.


Subject(s)
COVID-19 , Ciliary Motility Disorders , Cystic Fibrosis , Adult , Child , Adolescent , Humans , Caregivers/psychology , COVID-19/epidemiology , COVID-19/complications , Depression/epidemiology , Depression/etiology , Cystic Fibrosis/epidemiology , Pandemics , Anxiety/epidemiology , Anxiety/etiology , Anxiety/psychology , Ciliary Motility Disorders/complications
3.
J Med Internet Res ; 25: e45249, 2023 04 20.
Article in English | MEDLINE | ID: covidwho-2306090

ABSTRACT

BACKGROUND: The COVID-19 pandemic disrupted the needs and concerns of the cystic fibrosis community. Patients with cystic fibrosis were particularly vulnerable during the pandemic due to overlapping symptoms in addition to the challenges patients with rare diseases face, such as the need for constant medical aid and limited information regarding their disease or treatments. Even before the pandemic, patients vocalized these concerns on social media platforms like Reddit and formed communities and networks to share insight and information. This data can be used as a quick and efficient source of information about the experiences and concerns of patients with cystic fibrosis in contrast to traditional survey- or clinical-based methods. OBJECTIVE: This study applies topic modeling and time series analysis to identify the disruption caused by the COVID-19 pandemic and its impact on the cystic fibrosis community's experiences and concerns. This study illustrates the utility of social media data in gaining insight into the experiences and concerns of patients with rare diseases. METHODS: We collected comments from the subreddit r/CysticFibrosis to represent the experiences and concerns of the cystic fibrosis community. The comments were preprocessed before being used to train the BERTopic model to assign each comment to a topic. The number of comments and active users for each data set was aggregated monthly per topic and then fitted with an autoregressive integrated moving average (ARIMA) model to study the trends in activity. To verify the disruption in trends during the COVID-19 pandemic, we assigned a dummy variable in the model where a value of "1" was assigned to months in 2020 and "0" otherwise and tested for its statistical significance. RESULTS: A total of 120,738 comments from 5827 users were collected from March 24, 2011, until August 31, 2022. We found 22 topics representing the cystic fibrosis community's experiences and concerns. Our time series analysis showed that for 9 topics, the COVID-19 pandemic was a statistically significant event that disrupted the trends in user activity. Of the 9 topics, only 1 showed significantly increased activity during this period, while the other 8 showed decreased activity. This mixture of increased and decreased activity for these topics indicates a shift in attention or focus on discussion topics during this period. CONCLUSIONS: There was a disruption in the experiences and concerns the cystic fibrosis community faced during the COVID-19 pandemic. By studying social media data, we were able to quickly and efficiently study the impact on the lived experiences and daily struggles of patients with cystic fibrosis. This study shows how social media data can be used as an alternative source of information to gain insight into the needs of patients with rare diseases and how external factors disrupt them.


Subject(s)
COVID-19 , Cystic Fibrosis , Social Media , Humans , COVID-19/epidemiology , Pandemics , Cystic Fibrosis/epidemiology , Rare Diseases , Time Factors
4.
Pediatr Int ; 64(1): e15009, 2022 Jan.
Article in English | MEDLINE | ID: covidwho-2287466

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is a chronic disease causing recurrent respiratory tract infections. Viral respiratory tract infections are more severe in CF. The first case of coronavirus disease 2019 (COVID-19) was seen in Turkey on March 11, 2020, and nationwide school closure and lockdown were implemented. School closure and home confinement might have adverse effects on children's physical and mental health. In this study, we aimed to compare the effect of the COVID-19 pandemic on psychological reactions of CF patients and healthy controls. METHODS: This is a controlled cross-sectional study including 7-18-year-old children with CF. The survey included questions regarding family environment and peer relations, self-care, and psychological reactions to the COVID-19 pandemic. The questionnaire was administered to children via telephone calls under parental supervision. RESULTS: We evaluated 132 CF patients and 135 of their healthy peers. Mean age was 11.5 ± 2.9 years in the CF group and 11.8 ± 3.2 years in the control group (P = 0.98). There were 55 girls (41.7%) in the CF group and 81 girls (60%) in the control group (P = 0.027). The socioeconomic status of their families was similar. The CF patients were found to be less anxious for family members at risk of COVID-19, less upset about school closure, and less anxious about the COVID-19 pandemic (P < 0.001, 0.02, 0.01, respectively). CONCLUSIONS: Cystic fibrosis patients seem to show more resilience in coping with the pandemic. Appropriate psychological support should be provided to them and resilience strategies in coping with the pandemic should be nurtured.


Subject(s)
COVID-19 , Cystic Fibrosis , Adolescent , Anxiety/epidemiology , Anxiety/etiology , COVID-19/epidemiology , Child , Communicable Disease Control , Cross-Sectional Studies , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Female , Humans , Male , Pandemics
5.
Int J Environ Res Public Health ; 19(22)2022 Nov 16.
Article in English | MEDLINE | ID: covidwho-2143128

ABSTRACT

There is an increasing interest in father-child interactions and their effects. Due to the rising number of working mothers, marital interruptions, divorces, and child custody arrangements, paternal duties and the relevance of fathering continue to be re-evaluated. As there are rising expectations for men to undertake more childcare and household responsibilities, it was hypothesized that the presence of a disabled or chronically ill child would have a significant impact on the couple's future family situation, marital conduct due to paternal dissatisfaction, and increased stress levels. Therefore, the purpose of this study was to examine paternal intimacy problems, stress levels, and couple satisfaction inside families that have children with cystic fibrosis. The study followed a cross-sectional design with five questionnaires that were answered by a total of 107 fathers of children with cystic fibrosis from the "cases" group as the reference group, and 124 fathers of healthy children from the "control" group. The statistically significant findings of the current study show that men who were taking care of their child with mucoviscidosis engaged less frequently in sexual activity. A significantly higher number of these respondents were smokers. A higher proportion of them reported marital distress (OR = 2.54) and inhibited sexual desire (OR = 2.02), all in association with a higher number of men taking psychiatric medication (7.5% vs. 1.6%). More than 40% of all respondents declared high levels of general stress and parenting distress, while the most frequently used coping mechanism for stress was avoidance-oriented (45.8% vs. 25.8%). Other important findings were the high levels of dissatisfaction and lower levels of marital quality on the SII scale, equivalent to the intimacy problems on the MIQ scale. It is likely that paternal stress is higher when parenting children with cystic fibrosis, and the lack of intervention in this vulnerable group seem to be associated with intimacy problems, couple dissatisfaction, and maladaptive coping mechanisms. It is recommended that these concerns should not only be raised for the mothers of children with mucoviscidosis, but also for the child's father or the male caretaker partner since they might experience the same problems as the opposite gender.


Subject(s)
Cystic Fibrosis , Personal Satisfaction , Female , Humans , Male , Cross-Sectional Studies , Cystic Fibrosis/epidemiology , Father-Child Relations , Sexual Behavior
6.
Int J Environ Res Public Health ; 19(23)2022 Nov 30.
Article in English | MEDLINE | ID: covidwho-2143161

ABSTRACT

Research shows that people with cystic fibrosis are more prone to suffer from psychological problems than healthy people; thus, the outbreak of the COVID-19 pandemic in Poland could have had an impact on their mental health. To assess this impact, we examined the mental health of patients before and during the pandemic. Survey participants were asked to fill in questionnaires that consisted of Beck Depression Inventory (BDI), 12-Item General Health Questionnaire (GHQ-12) and Cystic Fibrosis Questionnaire-Revised (CFQ-R; for the purpose of the study, an emotional functioning domain was used) during their hospital visits. A total of 81 patients took part in the study: 39 before the COVID-19 pandemic (BP) and 42 during the COVID-19 pandemic (DP). Patients' medians were lower for the BDI, GHQ-12 and higher for the emotional domain of CFQ-R during the pandemic (3, 6, 75 vs. 4, 10, 73.33). Fewer patients felt that their mental health had deteriorated during the pandemic (Δχ2 = 7.723; p = 0.005), and GHQ-12 scores were lower in the DP group (Z = -3.044; p = 0.002). No significant differences were found between groups in terms of experiencing depressive symptoms (Δχ2 = 1.036; p = 0.309). It was found that patients with cystic fibrosis from our study group not only maintained but also improved their mental health state during the COVID-19 pandemic.


Subject(s)
COVID-19 , Cystic Fibrosis , Humans , Mental Health , Pandemics , COVID-19/epidemiology , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Cystic Fibrosis/diagnosis , Anxiety/epidemiology , Poland/epidemiology , Depression/psychology
7.
JBI Evid Synth ; 20(11): 2721-2726, 2022 11 01.
Article in English | MEDLINE | ID: covidwho-2116374

ABSTRACT

OBJECTIVE: The objective of this review is to determine the incidence and prevalence, clinical features, and outcomes of COVID-19 in persons with cystic fibrosis. INTRODUCTION: Cystic fibrosis, predominantly a chronic respiratory illness, has long been known to be fatal with concomitant bacterial or viral infections. Consequently, the effects of COVID-19 on this protracted disease need to be understood, especially since the major manifestations affect the respiratory system. Hence, this review aims to examine the burden, clinical features, and outcomes of COVID-19 on individuals with cystic fibrosis. INCLUSION CRITERIA: This review will consider studies of persons in all age groups with preexisting cystic fibrosis who are diagnosed with COVID-19 using either a polymerase chain reaction, serology, or point-of-care test for SARS-CoV-2. Eligible studies will report the incidence, prevalence, clinical features, or outcomes of COVID-19 in individuals with cystic fibrosis. Studies in community or health care settings from any geographic location will be considered. METHODS: The JBI methodology for systematic reviews of prevalence and incidence will be used for this review. A methodical search for eligible studies in English (as well as available translations) in MEDLINE, Embase, Scopus, and CINAHL, and unpublished literature in Google Scholar, Dissertation Abstracts International, ProQuest Dissertations and Theses, and MedNar will be conducted from the year 2020 onwards. Studies meeting the inclusion criteria will be selected for appraisal and their methodological quality will be assessed by two independent reviewers based on study titles and abstracts, followed by full-text review focusing on sampling and statistical analysis. Data extraction will be accomplished using a standardized tool. If adequate synthesized data are obtained, a meta-analysis will be conducted; otherwise, the findings will be presented in narrative format, including tables and figures to aid in data presentation. SYSTEMATIC REVIEW REGISTRATION NUMBER: PROSPERO CRD42021237792.


Subject(s)
COVID-19 , Cystic Fibrosis , Humans , COVID-19/epidemiology , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Incidence , Prevalence , SARS-CoV-2 , Systematic Reviews as Topic
8.
Pediatr Pulmonol ; 57(11): 2707-2714, 2022 11.
Article in English | MEDLINE | ID: covidwho-1971320

ABSTRACT

BACKGROUND: The spread of COVID-19 was associated with increased stress and new mental health concerns for people with cystic fibrosis (pwCF), already at increased risk for depression and anxiety. This study assessed stress and mental health in adolescents and young adults with CF 1 year from when the pandemic began. METHODS: Sixty-six pwCF (mean age = 24; range 14-36) completed a new measure of the impact of COVID-19 (COVID-19 Exposure and Family Impact Scale-Adolescence and Young Adult; CEFIS-AYA); the Patient Health Questionnaire-9, and the Generalized Anxiety Disorder-7. The Italian translation of the CEFIS-AYA was performed. RESULTS: On the CEFIS-AYA, the mean Exposure score was 5.2 (SD = 2.6) out of 28. The mean Impact score was 1.8 (SD = 0.7; negative valence > 2.5). Individuals were more sedentary and undertaking less exercise. Average stress rating was 5.9 (SD = 2), indicating moderate stress. No significant differences were found between those who did (N = 12) and who did not have a COVID infection (N = 54). A high percentage of participants scored above the clinical cut-off for depression (45%) and anxiety (41%), with a low proportion reporting moderate-severe symptomatology. CONCLUSION: After 1 year, the pandemic was having a less significant impact on patients' daily lives. Sedentary activity and reduced exercise were common. Despite expectations that this group was particularly vulnerable, depression and anxiety scores were similar to the rates described for this population before the pandemic. Overall, these results suggested that pwCF are highly resilient and nearly 1 year after the onset of COVID-19, have returned to similar daily activities and emotional health.


Subject(s)
COVID-19 , Cystic Fibrosis , Adolescent , Adult , Anxiety/epidemiology , Anxiety/psychology , COVID-19/epidemiology , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Depression/epidemiology , Depression/psychology , Humans , Mental Health , Pandemics , SARS-CoV-2 , Young Adult
9.
Front Biosci (Landmark Ed) ; 27(6): 178, 2022 06 02.
Article in English | MEDLINE | ID: covidwho-1912654

ABSTRACT

BACKGROUND: Coronavirus pandemic has influenced our society with social distancing and management of chronic disease such as cystic fibrosis (CF). During the Italian lockdown from March to May 2020, CF patients reduced the number of outpatient visits, limited social interactions and spent more time at home. The aim of this study is to evaluate the impact of the lockdown on body mass index (BMI) and lung function tests on CF patients. METHODS: We retrospectively reviewed clinical data about 111 CF patients followed in our Regional Cystic Fibrosis Reference Centre (Policlinico Umberto I, Rome) according to two periods: pre-lockdown (from October 2019-March 2020) and post-lockdown (from May 2020-October 2020). We collected data on nutritional (BMI and body weight) and lung function status; we chose the best values of the 'pre-lockdown' and 'post-lockdown' period for each patient. Patients were divided into 3 groups according to FEV1 value (Forced Expiratory Volume in the 1st second): group 1 (FEV1 <40%), group 2 (FEV1 40-70%), group 3 (FEV1 >70%). All patients received a telephone interview asking for the number of hours per week devoted to physical activity, number of pulmonary acute exacerbations and subjective evaluation of adherence to medical therapy, respiratory physiotherapy and diet, during the two periods. RESULTS: Comparing weight, BMI and respiratory function between pre and post lockdown periods, we noticed an increase in weight during among overall patients. Male patients improved weight, BMI, FEF 25-75% (Forced Expiratory flow between 25% and 75% of vital capacity) and Tiffenau index more than female patients. The most severely compromised patients (group 1), showed a significant loss of both weight and BMI. Instead, patients with moderate respiratory function (group 2) showed a significant increase of both weight and BMI and a slightly reduced CVF (Forced Vital capacity). We found no differences among patients with good respiratory function (group 3). Comparing each clinical sub-groups, we noticed a significative improvement of weight (p = 0.018) and BMI (p = 0.030) among patients with moderate respiratory function compared to patients with compromised respiratory function. During lockdown, patients reported less physical activity, no variation in food amount and composition, more adherence to therapy (43%) and more consistent daily respiratory physiotherapy (47.6%). CONCLUSIONS: Lockdown period had benefit among CF patients in terms of weight in particular in male patient. The greatest benefit on nutritional state was observed in patients with moderate reduction of respiratory function. In addition, we noted a stabilization and sometimes a slight improvement of lung function, instead of a continuous and steady decline that is normally observed in CF patients. These beneficial effects are slight but significative, bearing in mind the general worsening that CF patients experience annually.


Subject(s)
COVID-19 , Cystic Fibrosis , COVID-19/epidemiology , Communicable Disease Control , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Cystic Fibrosis Transmembrane Conductance Regulator , Female , Humans , Male , Retrospective Studies
10.
Pediatr Pulmonol ; 57(10): 2553-2557, 2022 10.
Article in English | MEDLINE | ID: covidwho-1905503

ABSTRACT

To address the discrepancy in the quality of care and outcomes between cystic fibrosis centers (CFCs) in high-income countries and limited resources countries (LRCs), a collaboration between our team at the University of Michigan CFC (UMCFC) and a CF center in Turkey (Marmara University CFC [MUCFC], Istanbul) was established. The collaboration included evaluation of all aspects of care and initiation of quality improvement (QI) measures. Teaching and implementing QI tools has led to start of improvement in MUCFC care. Close monitoring and sharing resources like UMCFC algorithms, protocols, and QI processes were done.


Subject(s)
COVID-19 , Cystic Fibrosis , COVID-19/epidemiology , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Humans , Michigan/epidemiology , Pandemics , Quality Improvement , Turkey/epidemiology , United States/epidemiology
11.
J Infect ; 85(3): 318-321, 2022 09.
Article in English | MEDLINE | ID: covidwho-1885923

ABSTRACT

COVID-19 has shown a relevant heterogeneity in spread and fatality among countries together with a significant variability in its clinical presentation, indicating that host genetic factors may influence COVID-19 pathogenicity. Indeed, subjects carrying single pathogenic variants of the Cystic Fibrosis (CF) Transmembrane Conductance Regulator (CFTR) gene - i.e. CF carriers - are more susceptible to respiratory tract infections and are more likely to undergo severe COVID-19 with higher risk of 14-day mortality. Given that CF carrier prevalence varies among ethnicities and nations, an ecological study in 37 countries was conducted, in order to determine to what extent the diverse CF carrier geographical distribution may have affected COVID-19 spread and fatality during the first pandemic wave. The CF prevalence in countries, as indicator of the geographical distribution of CF carriers, significantly correlated in a direct manner with both COVID-19 prevalence and its Case Fatality Rate (CFR). In a regression study weighted for the number of tests performed, COVID-19 prevalence positively correlated with CF prevalence, while CFR correlated with population percentage older than 65-year, cancer and CF prevalence. Multivariate regression model also confirmed COVID-19 CFR to be associated with CF prevalence, after adjusting for elderly, cancer prevalence, and weighting for the number of tests performed. This study suggests a putative contribution of population genetics of CFTR in understanding the spatial distribution of COVID-19 spread and fatality.


Subject(s)
COVID-19 , Cystic Fibrosis Transmembrane Conductance Regulator , Cystic Fibrosis , Heterozygote , COVID-19/epidemiology , COVID-19/mortality , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Genetics, Population , Humans , Mutation
12.
J Cyst Fibros ; 21(4): e221-e231, 2022 07.
Article in English | MEDLINE | ID: covidwho-1885892

ABSTRACT

BACKGROUND: This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13th December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen. RESULTS: SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1<40% were hospitalised with oxygen verses 3.2% with ppFEV >70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p<0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007). CONCLUSIONS: This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2.


Subject(s)
COVID-19 , Cystic Fibrosis , COVID-19/epidemiology , COVID-19/therapy , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Cystic Fibrosis Transmembrane Conductance Regulator , Ethnicity , Humans , Minority Groups , Oxygen , SARS-CoV-2
13.
J Cyst Fibros ; 21(3): e184-e187, 2022 05.
Article in English | MEDLINE | ID: covidwho-1873121

ABSTRACT

Data regarding immunogenicity of SARS-CoV-2 BNT162b2 vaccine in cystic fibrosis (CF) patients are limited. We prospectively measured total (TAbs-RBD; U/ml) and neutralizing (NAbs-RBD; %) antibodies of SARS-CoV-2 spike-receptor binding domain (RBD) protein in 33 CF patients and 66 healthy controls with median age (IQR): 19.6 (17.6-24.3) years and 31 (29-36) years, respectively and investigated possible associations with epidemiological and clinical parameters. Compared to healthy controls, CF patients had higher levels of TAbs-RBD and NAbs-RBD after both doses (P-value < 0.001). One month after the second dose, CF patients and controls had TAbs-RBD: median (IQR): 3396 (2443) and 1452 (1231) U/ml, respectively. Similarly, the NAbs-RBD (%) were: 97.30 (1.00) and 95.70 (3.71) %, respectively. CF patients also had fewer local and systemic adverse events (AEs) (P-value < 0.001). Among CF patients, no significant differences in immunogenicity were detected regarding the phenotype, genotype, medications, or severity of the disease. BNT162b2 vaccine was immunogenic with limited reactogenicity in CF patients regardless of the phenotype or severity of disease.


Subject(s)
COVID-19 , Cystic Fibrosis , Vaccines , Adolescent , Antibodies, Viral , BNT162 Vaccine , COVID-19/epidemiology , COVID-19/prevention & control , COVID-19 Vaccines/adverse effects , Cystic Fibrosis/epidemiology , Humans , SARS-CoV-2 , Young Adult
14.
Int J Qual Stud Health Well-being ; 17(1): 2062820, 2022 Dec.
Article in English | MEDLINE | ID: covidwho-1795455

ABSTRACT

PURPOSE: This study explores the concept of social connectedness for adults with Cystic Fibrosis (CF), generally and during the onset of the COVID-19 pandemic, to help inform contemporary CF healthcare. Social connectedness is an essential component of belonging and refers to an individual's sense of closeness with the social world. Unique disease factors make exploration of social connectedness pertinent, added to by COVID-19, with the CF population potentially facing increased risk for severe illness. METHODS: Seventeen adults with CF in Western Australia undertook interviews, with findings categorized as overarching themes. RESULTS: In a general sense, participants described social connectedness challenges caused by CF, despite which they reported meaningful connections that benefits their mental and physical health. Within a COVID-19 specific context, participants demonstrated resilience in the face of adversity, highlighted the importance of empathy in relation to the pandemic, and described how social support is both an outcome and enhancer of social connectedness. CONCLUSIONS: This study contributes to limited social connectedness literature within CF and chronic illness in general, highlighting the importance of social connectedness awareness raising, assessments and interventions in CF healthcare inside and outside the COVID-19 pandemic.


Subject(s)
COVID-19 , Cystic Fibrosis , Adult , Cystic Fibrosis/epidemiology , Humans , Pandemics , Social Support
16.
J Cyst Fibros ; 21(3): e176-e183, 2022 05.
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
17.
Clin Respir J ; 16(4): 335-340, 2022 Apr.
Article in English | MEDLINE | ID: covidwho-1714159

ABSTRACT

OBJECTIVES: The COVID-19 pandemic has resulted in unprecedent changes to clinical practice, and as the impact upon delivery of exercise services for people with cystic fibrosis (CF) in the United Kingdom was unknown, this was characterised via a national survey. METHODS: An electronic survey was distributed to healthcare professionals involved in the exercise management of CF via established professional networks. RESULTS: In total, 31 CF centres participated. Findings included significant reductions in exercise testing and widespread adaptation to deliver exercise training using telehealth methods. Promisingly, 71% stated that they would continue using virtual methods of engaging patients in future practice. CONCLUSION: These findings highlight adaptation to the COVID-19 pandemic and the need to develop sustainable and standardised telehealth services to manage patients moving forwards.


Subject(s)
COVID-19 , Cystic Fibrosis , COVID-19/epidemiology , Cystic Fibrosis/epidemiology , Cystic Fibrosis/therapy , Exercise , Humans , Pandemics , SARS-CoV-2
18.
Am J Respir Crit Care Med ; 205(11): 1271-1280, 2022 06 01.
Article in English | MEDLINE | ID: covidwho-1698208

ABSTRACT

A dramatic global reduction in the incidence of common seasonal respiratory viral infections has resulted from measures to limit the transmission of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) during the pandemic. This has been accompanied by falls reaching 50% internationally in the incidence of acute exacerbations of preexisting chronic respiratory diseases that include asthma, chronic obstructive pulmonary disease, and cystic fibrosis. At the same time, the incidence of acute bacterial pneumonia and sepsis has fallen steeply worldwide. Such findings demonstrate the profound impact of common respiratory viruses on the course of these global illnesses. Reduced transmission of common respiratory bacterial pathogens and their interactions with viruses appear also as central factors. This review summarizes pandemic changes in exacerbation rates of asthma, chronic obstructive pulmonary disease, cystic fibrosis, and pneumonia. We draw attention to the substantial body of knowledge about respiratory virus infections in these conditions, and that it has not yet translated into clinical practice. Now that the large scale of benefits that could be gained by managing these pathogens is unmistakable, we suggest that the field merits substantial academic and industrial investment. We consider how pandemic-inspired measures for prevention and treatment of common infections should become a cornerstone for managing respiratory diseases.


Subject(s)
Asthma , COVID-19 , Cystic Fibrosis , Pneumonia, Bacterial , Pulmonary Disease, Chronic Obstructive , Respiratory Tract Infections , Virus Diseases , Viruses , Asthma/epidemiology , Cystic Fibrosis/complications , Cystic Fibrosis/epidemiology , Humans , Pandemics/prevention & control , Pulmonary Disease, Chronic Obstructive/epidemiology , Pulmonary Disease, Chronic Obstructive/microbiology , Pulmonary Disease, Chronic Obstructive/prevention & control , Respiratory Tract Infections/epidemiology , SARS-CoV-2
19.
Ann Am Thorac Soc ; 19(10): 1697-1703, 2022 10.
Article in English | MEDLINE | ID: covidwho-1686147

ABSTRACT

Rationale: Cystic fibrosis (CF) centers transitioned to telemedicine during the spring 2020 peak of the coronavirus disease (COVID-19) pandemic. Objectives: We hypothesized that people with CF (pwCF) with more severe disease would be more likely to be seen in-person. Methods: We used paired t tests to compare within-subject changes in body mass index (BMI) and percentage predicted forced expiratory volume in one second (FEV1) and calculated relative risk (RR) to compare pulmonary exacerbations (PEx) between pwCF enrolled in the CF Foundation Patient Registry with at least one in-person clinic visit after March 15 in both 2019 and 2020. Results: Overall, the proportion of clinical encounters that were in-person clinic visits decreased from 91% in 2019 to a low of 9% in April 2020. Among pwCF seen after March 15 in both 2019 and 2020, the mean (95% confidence interval [CI]) FEV1 percentage predicted was 1.3% (0.1-2.4) predicted higher in 2020 for children 6 to <12 years of age, and 7.5% (7.1-7.9) predicted higher in 2020 among pwCF ⩾12 years of age eligible for the highly effective CF transmembrane conductance regulator modulator, elexacaftor-tezacaftor-ivacaftor (ETI). There was no difference in FEV1 percentage predicted for pwCF ⩾12 years of age who were not eligible for ETI. Similarly, the mean (95% CI) BMI was 2.4 (2.0-2.8) percentile higher in 2020 for children 6 to <12 years of age and 5.2 (4.8-5.7) percentile higher in 2020 among children 12 to <18 years of age eligible for ETI. Mean (95% CI) BMI was 1.2 (1.2-1.3) (kg/m2) higher for pwCF ⩾18 years of age eligible for ETI, and 0.2 (0.1-0.3) (kg/m2) higher for pwCF ⩾18 years of age not eligible for ETI. The proportion of in-person clinic visits where any PEx was present was lower in 2020 compared with 2019, 25% compared with 38%, RR 0.82 (0.79-0.86). Conclusions: The care of pwCF was substantially changed during the spring 2020 peak of the COVID-19 pandemic. Among pwCF seen in-person in both 2019 and 2020 after the spring peak of the COVID-19 pandemic, lung function and BMI were higher in 2020 for children 6 to <12 years of age and pwCF eligible for ETI.


Subject(s)
COVID-19 , Cystic Fibrosis , Aminophenols/therapeutic use , Child , Child, Preschool , Cystic Fibrosis/epidemiology , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Forced Expiratory Volume , Humans , Mutation , Pandemics
20.
Pediatr Pulmonol ; 57(5): 1238-1244, 2022 05.
Article in English | MEDLINE | ID: covidwho-1669622

ABSTRACT

BACKGROUND: The COVID-19 pandemic impacted many households due to shelter-in-place orders and economic hardship. People with cystic fibrosis (CF) experienced increased food insecurity compared to the general population before the pandemic, even though adequate food access is needed to maintain nutrition goals associated with improved health-related outcomes. Little is known about the impact the pandemic had on the food insecurity of people with CF and their families. OBJECTIVE: To investigate how the COVID-19 pandemic impacted food insecurity, mental health, and self-care in people with CF. METHODS: Adults with CF and parents/guardians of children with CF were recruited via social media to complete online questionnaires from May 2020 to February 2021. Questionnaires in English and Spanish included USDA 2-question food insecurity screening, Patient Health Questionnaire-4 for mental health screening, and directed questions on the impact of the pandemic. RESULTS: Of 372 respondents, 21.8% of the households experienced food insecurity during the pandemic compared to 18.8% prepandemic (p < .001). More food insecure patients with CF reported weight loss (32.1% vs. 13.1%, p < .001), worse airway clearance adherence (13.6% vs. 5.8%, p < .01), and worse medication adherence (12.4% vs. 1.7%, p < .01) compared to food secure patients. Food insecure subjects were more likely to have an abnormal mental health screen compared to food secure subjects (53.1% vs. 16.2%, p < .001). CONCLUSION: Food insecurity increased in the CF population during the COVID-19 pandemic. Food insecure subjects reported worse mental health and self-care during the pandemic compared to food secure subjects.


Subject(s)
COVID-19 , Cystic Fibrosis , Adult , COVID-19/epidemiology , Child , Cross-Sectional Studies , Cystic Fibrosis/epidemiology , Food Insecurity , Food Supply , Humans , Mental Health , Pandemics
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