ABSTRACT
Respiratory muscles (RM) are a very important part of the respiratory system that enables pulmonary ventilation. This study aimed to assess the post-COVID-19 strength of RM by estimating maximum static inspiratory (MIP or PImax) and expiratory (MEP or PEmax) pressures and to identify the relationship between MIP and MEP and the parameters of lung function. We analyzed the data of 36 patients (72% male;median age 47 years) who underwent spirometry, and body plethysmography, diffusion test for carbon monoxide (DLCO) and measurement of MIP and MEF. The median time between the examinations and onset of COVID-19 was 142 days. The patients were divided into two subgroups. In subgroup 1, as registered with computed tomography, the median of the maximum lung tissue damage volume in the acute period was 27%, in subgroup 2 it reached 76%. The most common functional impairment was decreased DLCO, detected in 20 (55%) patients. Decreased MIP and MEP were observed in 5 and 11 patients, respectively. The subgroups did not differ significantly in MIP and MEP values, but decreased MIP was registered in the second subgroup more often (18%). There were identified no significant dependencies between MIP/MEP and the parameters of ventilation and pulmonary gas exchange. Thus, in patients after COVID-19, MIP and MEP were reduced in 14 and 31% of cases, respectively. It is reasonable to add RM tests to the COVID-19 patient examination plan in order to check them for dysfunction and carry out medical rehabilitation.Copyright © 2022 Obstetrics, Gynecology and Reproduction. All rights reserved.
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The current outbreak of COVID-19 is caused by the SARS-CoV-2 virus that has affected > 210 countries. Various steps are taken by different countries to tackle the current war-like health situation. In India, the Ministry of AYUSH released a self-care advisory for immunomodulation measures during the COVID-19 and this review article discusses the detailed scientific rationale associated with this advisory. Authors have spotted and presented in-depth insight of advisory in terms of immunomodulatory, antiviral, antibacterial, co-morbidity associated actions, and their probable mechanism of action. Immunomodulatory actions of advised herbs with no significant adverse drug reaction/toxicity strongly support the extension of advisory for COVID-19 prevention, prophylaxis, mitigations, and rehabilitation capacities. This advisory also emphasized Dhyana (meditation) and Yogasanas as a holistic approach in enhancing immunity, mental health, and quality of life. The present review may open-up new meadows for research and can provide better conceptual leads for future researches in immunomodulation, antiviral-development, psychoneuroimmunology, especially for COVID-19.Copyright © 2021, Institute of Korean Medicine, Kyung Hee University.
ABSTRACT
The article provides information about a study conducted to investigate the efficacy of doxophylline in post-COVID respiratory syndrome. The study was conducted on an outpatient basis at the Educational and Therapeutic Clinic of the Azerbaijan Medical University for 3 months in 2021-2022. The study included 47 patients with laboratory-confirmed COVID-19 pneumonia (PCR or rapid antigen test) and X-ray 4-12 weeks before the examination. In addition to general instrumental examinations, the intensity of cough was determined in patients - a questionnaire for assessing the severity of cough (Zhan W.), a modified dyspnea scale of the Medical Research Council and spirometry (FEV1 -ANHT1, EF, Tiffno index) examination. In accordance with the purpose of the work, post-COVID respiratory syndrome was confirmed in patients based on anamnestic-questionnaire scales and a spirometric examination performed at the beginning of treatment. After examination, all patients were prescribed doxofillin (Puroxan) 400 mg 2 times a day for 2 weeks. And for some patients who did not have a positive clinical and/or spirometric result, treatment was extended up to 8 weeks. During the study, all patients taking doxophylline showed a significant decrease in the intensity of cough and improvement in respiratory parameters. In conclusion, it can be considered appropriate to administer doxofylline at a dose of 400 mg to patients to improve respiratory parameters in post-COVID respiratory syndrome.Copyright © 2023 Ministry of Health. All rights reserved.
ABSTRACT
Lymphangitis carcinomatosa refers to pulmonary interstitial involvement by cancer and is a dreaded clinical finding in oncology because it is a late manifestation indicative of metastatic malignancy, from either a lung or a nonlung primary cancer, and is associated with poor prognosis. Its presentation is nonspecific, often with subacute dyspnoea and a nonproductive cough in a person with a known history of malignancy, but in some cases is the first manifestation of cancer. CT imaging can be suggestive, typically demonstrating thickening of the peribronchovascular interstitium, interlobular septa and fissures. However, a biopsy may be required to confirm the pathological diagnosis as these changes can also be due to concurrent disease such as heart failure, ILD, infection, radiation pneumonitis and drug reactions. Diagnosis allows symptomatic treatment, with personalised treatment directed towards the primary cancer most likely to provide a meaningful benefit. Future research should focus on prospective clinical trials to identify new interventions to improve both diagnosis and treatment of lymphangitis carcinomatosa.Copyright © ERS 2021.
ABSTRACT
Autoimmune pulmonary alveolar proteinosis (PAP) is a rare disease, especially in pediatrics, but important to consider, as it may avoid unnecessary and/or invasive investigations and delayed diagnosis. This case report highlights an adolescent girl with rapid onset dyspnea but an unremarkable physical exam and initial testing. However, due to a high index of suspicion, a chest computed tomography (CT) scan was done, revealing a "crazy paving" pattern, which then prompted expedited assessment. This finding, however, is not as specific as often discussed and has a broad differential diagnosis, which will be reviewed in detail as part of this case. Furthermore, this report demonstrates a diagnostic approach for PAP that avoids lung biopsy, previously considered to be required for diagnosis of PAP, but is increasingly becoming unnecessary with more advanced blood tests and understanding of their sensitivity and specificity. Additionally, management strategies for PAP will be briefly discussed.Copyright © 2022 Canadian Thoracic Society.
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Background: The advent of highly effective modulator therapies (HEMTs), including elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA), for treatment of cystic fibrosis (CF) has resulted in remarkable clinical improvement for modulator-naive patients and for those who have been treated with prior modulator therapies. Intranasal micro-optical coherence tomography (muOCT) has detected functional abnormalities in the mucociliary apparatus of people with CF. The objectivewas to characterize the effects of ELX/TEZ/ IVA on nasal mucociliary clearance by muOCT and monitor the clinical changes conferred as a way to understand the effects. Method(s): Of 26 individuals aged 12 and older with at least one F508del mutation recruited, 24 were enrolled and followed over three visits: baseline and 1 (visit 2) and 6 months (visit 4) after initiation of ELX/TEZ/IVA therapy;the COVID-19 pandemic affected visit windows. Intranasal muOCT imaging was conducted at baseline and visit 2 as previously described;additional imaging for 18 months (visit 5) is in progress. Clinical outcomes, including percentage predicted forced expiratory volume in 1 second (FEV1pp) and sweat chloride levels were computed as part of the parent Prospective Study to Evaluate Biological and Clinical Effects of Significantly Corrected CFTR Function (PROMISE study). A blinded investigator team analyzed in vivo muOCT parameters including mucociliary transport (MCT) rate, ciliary beat frequency (CBF), and periciliary liquid depth (PCL) after devising an improved stabilization algorithm. Analysis of airway surface liquid (ASL) depthswas excluded because of the limited number of cases in which the necessary condition for measurement,which is preservation of a clear air layer between the mucus layer and the probe, was satisfied. Result(s): Twenty-three subjects completed visits 1 and 2, and 18 completed visits 1, 2, and 4. Average age at baselinewas 27 +/- 8.7, 69% were female, and 43% were on prior two-drug modulator therapy. No significant change in body mass index was found between the visits. FEV1pp increased significantly (10.9%, 95% CI, 76.1-98.4%) by visit 2 and persisted at visit 4 (10.6%, 95% CI, 87.7-107.0;p < 0.001). Sweat chloride levels decreased significantly at visit 2 (-36.6 mmol/L, 95% CI, 40.9-54.9 mmol/L) and visit 4 (-41.3 mmol/L, 95% CI, 34.9-51.8 mmol/L) at visit 4 ( p < 0.001). Analysis of muOCT images revealed significant improvement in MCT rate (2.8 +/- 1.5 mm/ min at baseline vs 4.0 +/- 1.5 mm/min at visit 2, p = 0.048), although no discernable changes were noted in CBF or PCL. When stratified based on use of prior modulator therapy, no significant differences were found for any muOCT metric. No significant correlations between change in MCT rates and change in FEV1pp or sweat chloride from baseline to visit 2were found. Conclusion(s): Treatment with ELX/TEZ/IVA in people with CF, including those that were treatment naive and those on prior modulator therapy, resulted in significant, sustained improvement in lung function and decreases in sweat chloride levels at ~10 months, consistent with recently published reports. Functional improvements in MCTratewere evident after initiation of ELX/TEZ/IVA therapy, which may partially explain the findings of better whole-lung mucus clearance and reduction in chronic infections reported previously. muOCT imaging in people with CF is sensitive to the treatment effect of HEMT and suggests better mucociliary transport as a mechanism of action underlying the clinical benefits for lung health. Acknowledgements: On behalf of the PROMISE investigatorsCopyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Background: Use of home spirometry to monitor lung function has been increasing in popularity in persons with cystic fibrosis (PwCF) since the start of the COVID-19 pandemic. Although clinic spirometry is interpreted from validated standards, expected test-to-test variation of home spirometry and how variation during baseline health may relate to clinical changes are unknown. The aim of this study was to determine variation in baseline lung function during daily home spirometry and identify associations with clinical outcomes. Method(s): Subjectswere selected based on available spirometry data from a cohort of PwCF enrolled in a long-term airway microbiome study. Subjects were provided with a PiKo-6 hand-held spirometer (nSpire Health, Inc., Longmont, CO) and asked to perform spirometry maneuvers three times per. Validity of home spirometry (percentage predicted forced expiratory volume in 11 second (FEV1pp)) was compared with that clinic spirometry using Bland-Altman plots. Spirometry acceptability across multiple maneuvers in the same day was assessed using the American Thoracic Society (ATS) guidelines, with grade A or B (two or more maneuvers within 150 mL) considered acceptable. Variation in FEV1pp was assessed by calculating a mean FEV1pp and coefficient of variation (CoV). The association between CoV and pulmonary exacerbations (PEx) was tested using Cox proportional hazards regression models. Result(s): Thirteen subjects (62% female) with a mean age of 28.7 +/- 8.3 and mean FEV1pp of 59.9 +/- 8.2%were included. Median study durationwas 377 days (range, 33-730 days). Subjects used the home spirometer on average 51.2% of the study days (range 15-97%). On average, 58.9% of subjects (range 12-100%) used the home spirometer at least twice aweek, and 76.8% (range 65-100%) at least once aweek. To focus on periods of baseline health, days associated with PEx (spirometry performed 2 weeks before and during times of antibiotic therapy) were excluded. A median of 204 days (range 11-728 days) of baseline spirometry readings was available for further analysis. Comparing validity of home spirometry with that of clinic spirometry, Bland-Altman plots demonstrated overall good agreement with a slight bias (+0.042 L) toward higher readings for clinic FEV1pp (95% limits of agreement, -0.11-0.19 L). Spirometry quality was graded as acceptable on most study days (mean 90.6 +/- 4.6%) in which two or more maneuvers were recorded. Intra-individual variation in baseline FEV1pp was high, with a mean variation of 17.6 +/- 5.9% day to day and 15.2 +/- 6.2% week to week. Neither rates of acceptable spirometry grades nor CoV was associated with lung disease severity. Of the 13 subjects, 10 experienced one or more PEx, for a total of 32 PEx during the study. CoV was not associated with time to first PEx (hazard ratio [HR], 0.78;95% confidence interval [CI], 0.51-1.21;p = 0.24) or time to subsequent PEx (HR, 0.91;95% CI, 0.73-1.12;p = 0.28) during the study. Conclusion(s): Although home spirometry has generally good validity and acceptability, variation in lung function during baseline health is present and often exceeds expected variation in clinic spirometry per ATS standards. Variability may represent normal physiological variation or be related to the home spirometer itself or other factors but did not portend upcoming PEx. Recognition of variation during baseline health provides important context for interpretation of home spirometry.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Background: The COVID-19 pandemic has altered how we deliver care to people with cystic fibrosis (CF) across the spectrum of disease severity. Because of lockdowns and avoiding exposure to COVID-19 by limiting inperson clinic visits, clinical care has pivoted from standard practices to virtual care in combination with in-person traditional visits. This approach has allowed patients to be monitored and treated in a timely manner. Such virtual visits have the advantage of reducing the time commitment for clinic visits because the patient does not have to travel to and from the hospital, but virtual care lacks the ability to conduct a physical examination and to obtain objective and standardized testing of key measurements known to be associated with health outcomes in CF. The objective of this study was to evaluate the attitude of patients to virtual delivery of care and their comfort level with such care. Method(s): This is a prospective, cross-sectional survey of adults with CF who are followed at St. Michael's Adult CF Center in Toronto, Canada. An online survey was created using SurveyMonkey to assess attitudes toward and satisfaction with virtual care. The survey was emailed to participants and included the Canadian CF Registry ID;a reminder email sent a week later. Baseline demographic and clinical data were obtained from the Canadian CF Registry and presented as median (range) or proportions as appropriate. Questions using a 3-point Likert scale will be categorized into agree, neutral, and disagree. Result(s): A total of 210 participants (53.0% female) completed the survey (median age 37.8, range 19.2-78.9). Median age of diagnosis was 2.2, 95.7% were Caucasian, 76.0% had completed post-secondary education, 63.0% were employed and 11.0% were students, 75% were pancreatic insufficient, 39.0% had CF-related diabetes, and 12.4% were post lung-transplant. Median percentage predicted forced expiratory volume in 1 second was 65.8% (range 17.9-126.9%), and median body mass index was 23.6 kg/m2 (range 15.5-45.7 kg/m2). Eighty-one percent of respondents had had a virtual visit before completing the survey. Sixty percent of respondents felt that in-person visits were the preferred way of completing a medical assessment, and 27.0% preferred virtual visits. Seventy-three percent felt it was important for the virtual visit to occur at the booked time, 59.0% had concerns that their lung function was not assessed during virtual visits, 46.0% felt they were losing the benefits of allied health team assessments with virtual visits, and 40.0% worried that their health would decline if primarily seen virtually. Just over half of respondents wanted to continue with virtual visits in some capacity after the pandemic. The optimal proportion of in-person visits was felt to be 50.0%. More than 85% of respondents were comfortable with technology (phone or computer) and had reliable access to the Internet to conduct virtual visits. Seventy percent of people would like to have access to a home spirometer, but cost was a barrier. Conclusion(s): From the patient's perspective, in-person visits were still the preferred way to complete a medical assessment, which seemed to be driven by concerns over lack of methods for assessment, particularly lung function, and access to the multidisciplinary team. Home spirometers, if freely available, might increase comfort with virtual appointments.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Background: Increasing availability of highly effective cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator therapy (HEMT) has improved the quality of life and long-term prognosis for many people with CF. Thus, more people with CF are considering parenthood. Almost all menwith CF (MwCF) are infertile because of congenital bilateral absence of the vas deferens (CBAVD). Based on CF animal models, CBAVD occurs early in gestation and is unlikely to be reversible using HEMT, but assisted reproductive techniques (ARTs) can enable MwCF to father children using the sperm in their testes. Animal reproductive models suggest no HEMT teratogenicity, and the amount of exposure of the fetus to HEMT via absorption of seminal fluid through the vaginal wall is predicted to be negligible, although to ensure no sperm exposure to HEMT, the life span of sperm would require MwCF to discontinue CFTR modulators for approximately 3 months before ART. Because abrupt discontinuation of CFTR modulators may result in health decline, MwCF and their providers must consider all potential risks. There are no published data in MwCF regarding use of HEMT during conception and partner pregnancy. Method(s): Beginning in August 2021, CF center staff in the United States, United Kingdom, and Australia completed a two-page anonymous questionnaire regarding MwCF who used CFTR modulators during ART (sperm retrieval and in vitro fertilization) or natural conception with subsequent partner pregnancy. Result(s): Providers have submitted 34 surveys for MwCF on CFTR modulators whose partner became pregnant after use of ART (n = 32) or natural conception (n = 2). The median age of the samplewas 32 (range 24- 43). Fifteen were homozygous for F508del, median percentage predicted forced expiratory volume in 1 second was 76% (range (22-111%), and median body mass index was 24 kg/m2 (range 18.5-32.1). Twenty-three were taking elexacaftor/tezacaftor/ivacaftor. The median time that MwCF were taking CFTR modulators before partner conception was 18 months (range 0-82). One newly diagnosed man initiated HEMT after sperm retrieval. Four MwCF stopped CFTR modulators before sperm retrieval, one of whom experienced pulmonary decline. None of the 19 MwCF whose condom use during pregnancy was known used condoms. Fetal complications in partners of MwCF included three first-trimester miscarriages, two* COVID, two breech presentation, two* vaginal bleeding, and one vasa previa. None of the complications were deemed definitively related to use of CFTR modulators. One MwCF experienced testicular infection after sperm retrieval#. Postpartum complications included three# infants with hypoxemia requiring neonatal intensive care unit stay, three maternal blood loss, one forceps delivery, and one caesarean section. No congenital anomalies were reported for any infant. (*/# overlap). Conclusion(s): Use of CFTR modulator therapy during partner conception and pregnancy in 34 MwCF has not resulted in higher-than-expected miscarriage rates or congenital anomalies. Providers should consider the risk to the health of MwCF combined with the lack of teratogenicity in animal reproductive models and limited safety data in the human fetus before discontinuing CFTR modulators before ART or natural partner conception. Survey collection is ongoing;results will be updated for presentationCopyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Background: Telemedicine has flourished during the COVID-19 pandemic. There is increasing interest in performing spirometry at home as part of a telehealth program, especially in cystic fibrosis (CF), to follow the course of the disease, but it is unclear whether the quality and accuracy of home spirometry are comparable with those of in-clinic spirometry [1-3]. This study aimed to evaluate the feasibility and measurement quality of telehealth spirometry assessments for people with CF. Method(s): Patientswith acceptable hardware at homewere provided with a flow sensor portable spirometer (Spirobank Smart) compatible with ATS/ ERS 2019 standards for volume accuracy. They performed spirometry during "home admissions" or ongoing home monitoring for 1 year during the COVID-19 pandemic (January 2021 to January 2022). At the end of each session, the family forwarded the data to the CF center. Result(s): Twenty-nine people were evaluated (median age 17.4, range 6.7- 34;58% female;mean baseline percentage predicted forced expiratory volume in 1 second 79.8 +/- 21.4%). According to American Thoracic Society/ European Respiratory Society criteria, spirometry was performed successfully in 320 of 430 (74.5%) attempted sessions. The median distance between the subject's home and the hospital was 124 km (range 49- 418 km)-a median travel time saving of 1.5 hours per hospital visit. Conclusion(s): Home-based telehealth spirometry is feasible in people with CF and can support the CF team in ongoing outpatient monitoring.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Background: In the light of post severe acute respiratory syndrome (SARS) and Middle East respiratory syndrome (MERS) Pneumonias playing a role in the long-term respiratory complications in patients subsequently involved in trauma, a study was conducted to assess the post COVID-19 Pneumonias on the prognosis of trauma patients in a Tertiary care Hospital of Telangana. Aim of the Study: To identify the post COVID-19 pneumonia and respiratory complications, their severity, factors affecting the management of trauma patients and the long-term sequelae. Materials: 42 patients categorized on American Association for the Surgery of Trauma (AAST) injury scoring scales were included. Patients aged between 18 and 70 years were included. Patients with previous history of post COVID-19 lung disease for 09 months or above were included. Pulmonary function tests like FEV1, FVC, TLC and DLCO were performed and analyzed. The CT scan signs were based on the involvement of the lung parenchyma as: Normal CT (no lesion), minimal (0-10%), moderate (11-25%), important (26-50%), severe (51-75%), and critical (>75%). Result(s): 42 patients with trauma with either COVID-19 disease affecting the lungs or RTPCR positive were included. There were 29 (69.04%) male patients and 13 (30.95%) female patients with a male to female ratio of 2.23:1. The mean age among the men was 41.55+/-3.25 years and 38.15+/-4.10 years in female patients. There were 33/42 patients with positive RTPCR test and 09/42 were negative for RTPCR test for COVID-19. Conclusion(s): Recovery from COVID-19 disease especially with lung parenchyma changes during the active state has shown to affect adversely the morbidity of post trauma surgeries. Preoperative assessment of Lung function tests such as FEV1, FVC, TLC and DLCO would guide the surgeon and the anesthetist in the surgical management of such patients.Copyright © 2023, Dr Yashwant Research Labs Pvt Ltd. All rights reserved.
ABSTRACT
Background: Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulator triple combination therapy (TCT) is available to approximately 85% of the U.S. CF population. Clinical trials of TCT demonstrate numerous improvements in physical health and healthrelated quality of life (HRQoL), but fewstudies have examined the effects of TCTon mental health and psychosocial outcomes, and little is known about whether gains in HRQoL are sustained over time.We aimed to describe the HRQoL and psychosocial outcomes of people with CF (PwCF) initiating TCT and explored changes in these outcomes up to 1 year after starting TCT. Method(s): This longitudinal study enrolled PwCF aged 14 and older who were followed at a large, combined pediatric and adult CF center. Questionnaires were administered within 6 months of initiating TCT (baseline) and 3, 6, and 12 months later. Study self-report measures evaluated were HRQoL (Cystic Fibrosis Questionnaire-Revised;CFQ-R), optimism, self-efficacy, medication-related beliefs (Medication Beliefs Questionnaire;MBQ), perceived social stigma of illness, and body image. Data were also collected from medical charts on measures of health and mental health screening. Four open-ended questionswere included at each timepoint to elicit qualitative data on experiences starting TCT. Longitudinal data were analyzed using linear mixed-effects models for repeated measures. Result(s): Sixty-three adults and adolescents with CF completed the full set of surveys at baseline. Mean participant age was 30.0 +/- 14.2. Fifty-four percent identified as female, 43% as male, and 2% as nonbinary. Seventyfour percent had private insurance. Mean percentage predicted forced expiratory volume in 1 second (FEV1pp) at baseline was 76.0 +/- 24.1%, and mean body mass index (BMI) was 22.9 +/- 3.1 kg/m2. At 12 months, mean FEV1pp was 80.8 +/- 21.9%, and mean BMI was 24.5 +/- 4.1 kg/m2. On standard measures used in CF mental health screening, mean baseline Patient Health Questionnaire (PHQ-9) score was 3.4 +/- 3.5, and mean General Anxiety Disorder score was 3.4 +/- 3.7. Mean PHQ-9 (3.5 +/- 4.0) and GAD-7 (3.4 +/- 3.7) scores at 12 months were similar to baseline. We found no statistically significant differences between the survey time points in participants' physical, respiratory, or emotional functioning on the CFQ-R, but there was a significant change in social functioning ( p < 0.001). There was no statistically significant change over time in optimism or selfefficacy, but there was a significant difference in CF medication beliefs between the four survey time points ( p = 0.008 for MBQ Importance subscale), with a decrease in perceived importance from baseline to 12 months. Conclusion(s): Whereas lung function and BMI increased in our sample by 12 months, similar improvementswere not seen in standard mental health outcomes. There was no change over time in physical, respiratory, or emotional functioning, optimism, or self-efficacy. Only CFQ-R social functioning had changed by 12 months, perhaps reflecting decreased COVID-related social isolation. There was also a change in medicationrelated beliefs, with a decrease in perceived importance of taking CF medications at 12 months. Future directions include conducting qualitative analyses of open-ended questions and further examining data on social stigma, motivation to take medications, and body image, as well as examining relationships between outcome variables and baseline FEV1 and BMICopyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Background: With the introduction of new cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy (elexacaftor/tezacaftor/ ivacaftor), peoplewith CF experiencing severe lung disease can experience significant improvements in clinical symptoms. Method(s): This single-center institutional review board-approved retrospective chart review identified patients with advanced lung disease who met criteria for a compassionate use or expanded access program because of high risk of death or transplant need within 2 years. Clinical data collection for all patients began at baseline, 2 to 4 weeks after therapy initiation, and continued every 3 months for 2 years. Datawere collected on demographic characteristics, clinic progress notes, clinical labs, forced expiratory volume in 1 minute (FEV1),weight, body mass index, respiratory colonization, and hospitalizations after drug initiation. Patients also completed sinus and chest computed tomography (CT) to track clinical changes. Result(s): Eighteen people with CF (aged 15-49, 56% male) from a large midwestern CF center who initiated drug therapy between July and September 2019 in an inpatient hospital or clinic setting were identified. Clinical markers (Table 1) indicated that modulator therapy was well tolerated and not discontinued by any participant;safety lab values did not indicate medical concern or discontinuation. There were 90 admissions for the group in the 2 years before therapy and 17 admissions during the 2 years after, although seven of the posttherapy admissions were for nonrespiratory indications. Monitoring results indicated the safety of modulator therapy because there were no adverse clinical occurrences or laboratory events, and all patients presented with universal stabilization. There have been no deaths and no transplants. Unlike lumacaftor/ivacaftor, therewere no problems with chest tightness or any difficulty with troublesome increases in expectoration burden or choking during initiation of therapy. Most had significant reduction in or loss of spontaneous cough and sputum production. The impact on microbial colonization is unclear, because even in this severe group, inability to produce sputum on command led to considerable missing data in follow-up, leaving colonization status at follow-up unclear. Conclusion(s): This study focused on people with CF who qualified for modulator therapy based on advanced lung disease. Initiation of modulator therapy was deemed safe and resulted in objective positive changes in nutrition;cough;FEV1);and subjective reports of clinical status, level of activity, and reduction in burden of treatment. No evidence was found of difficulty managing the increased expectoration during initial therapy. Limitations were noted in missing data during the COVID-19 pandemic, small sample size, and delayed follow-up for drug monitoring.(Table Presented) Clinical indicators before and after modulator therapy *Completed post-drug initiation (earlier than 12 months), **24 months before and after therapy initiationCopyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Background: Substance use is an understudied aspect of cystic fibrosis (CF) care. Even casual use of drugs or alcohol may reduce compliance with complicated treatment plans, worsen existing conditions associated with CF, or cause potential drug interactions. To understand the need for mitigation mechanisms for risky substance use in a CF population, we studied the prevalence of substance use in our adult CF clinic population to characterize relationships between substance use and health status. Method(s): In our large academic CF center, we performed a retrospective chart ion of 420 patients over a 6-year period (2015-2021). Clinical staff annually administer the Drug Abuse Screening Test (DAST-10), Alcohol Use Disorders Identification Test (AUDIT), Patient Health Questionnaire (PHQ-9), and General Anxiety Disorder (GAD-7) to assess drug use, alcohol use, depression, and anxiety symptoms, respectively. Demographic characteristics, lung function (percentage predicted forced expiratory volume in 1 second (FEV1pp)), and anxiety and depression symptom screening scores were recorded. Because substance use can change over time, we counted each assessment date as an independent observation (n = 1434). The chi-square test (Table 1) was conducted in R/RStudio [1] to assess for associations between self-reported substance use and symptoms of depression and anxiety. Because of insufficient data, aggregate datawere used to describe the presence (PHQ-9 or GAD-7 >= 10) or absence (PHQ-9 or GAD-7 < 10) of symptoms of depression and anxiety. Result(s): Positive scores for risky use were defined as a DAST score of 1 or higher and an AUDIT score of 8 or hither. Eighty-three of 326 patients (25%) met criteria for risky substance use on at least one observation. Therewas a slight male predominance (54.2%) and wide age distribution (mean age 30 +/- 7, range 20-55);Thirty (36.1%) had a higher AUDIT score, 34 (40.9%) had a high DAST score, and 19 (22.9%) had high scores on both.We selected 2019 to evaluate single-year prevalence of positive screenings to avoid the impact of COVID. In 2019, 29 patients had at least one positive screening result (DAST,15/203, 7.3%;AUDIT, 24/193,12%;both,10/193, 5.2%). In the 6- year dataset, we did not find a statistically significant association between symptoms of depression and anxiety and indication of drug or alcohol use in self-reported users (355 observations). Conclusion(s): According to a 2020 national survey of healthy Americans, 20.8% have used illicit drugs at least once in the past year, and 10.2% meet criteria for alcohol use disorder [2], compared with 7.3% of patients in our 2019 data who have used drugs (n = 15) and 12% (n = 24) who indicated risky alcohol use. In this review, positive screening scores on the DAST and AUDIT were not associated with degree of symptoms of depression and anxiety, suggesting that substance use and symptoms of depression and anxiety were not temporally associated with each other. Future work will include analysis of the relationship between substance use and mental health in the larger University of North Carolina clinic population and linear regression to evaluate possible explanatory variables for substance use in this populationCopyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Introduction/Aim: Medium and long-term impacts of COVID-19 pneumonitis are being increasingly recognised. Our study aimed to evaluate outcomes of hospitalised COVID-19 patients with moderate-to-severe respiratory compromise. Method(s): Patients admitted to a tertiary centre with COVID-19 pneumonitis (March 2020-October 2022) were followed in the Post-COVID Respiratory Clinic at 6-24 weeks. Baseline demographics, admission details, pulmonary function tests (PFTs), and clinic data were collected. Univariable and multivariable logistic regression were performed to investigate for predictors of persisting respiratory symptoms (dyspnoea, cough, chest pain) and functional limitation (self-reported). Result(s): 125 patients (64.8%male, 63.2+/-16.7years, 42.5% former/current smokers, BMI 31.0+/-8.0kg/m2, 49.6% fully vaccinated) with median follow-up time of 85 [interquartile range (IQR) 64-131] days were included. Pre-existing conditions included lung disease (29.6%), immunocompromise (15.2%), diabetes (24.8%) and hypertension (43.6%). 35.2% required ICU care (14.4% mechanical ventilated, 4% ECMO), 44.8% received high flow nasal prong oxygen and/or continuous positive airway pressure (CPAP). At initial clinic follow up, 65.4% had persisting X-ray changes. Mean predicted FEV1, FVC, DLCO were 86.8+/-20.7%, 85.3+/-20.3%, 82.2+/-19.8% respectively. Symptoms included dyspnoea (63.2%), fatigue (24.2%), cognitive dysfunction (12.9%) and musculoskeletal complaints (10.5%). Univariate predictors of continued respiratory and/or functional disability included age [odds ratio (OR) 1.03, 95%confidence interval (CI) 1.01-1.06, p = 0.01), prior lung disease (OR2.98, 95%CI 1.05-8.48, p = 0.04), hypertension OR2.61, 95%CI 1.09-6.22, p = 0.03) and length of hospital stay (LOHS) (OR1.03, 95%CI 1.00-1.07, p = 0.04). On multivariable analysis, only LOHS was independently predictive of continued respiratory and functional limitations (OR1.03, 95%CI 1.00-1.07, p = 0.02). Conclusion(s): Patients recovering from COVID-19 pneumonitis have a large burden of disability at follow-up. Older age, hypertension, lung disease and LOHS are risk factors for delayed recovery.
ABSTRACT
Background: Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA), a triple CFTR modulator combination, has proved to be highly effective in Phe508del homozygous and Phe508del/minimal function compound heterozygous people with cystic fibrosis (PwCF).We report preliminary data on the realworld effectiveness and safety of ELX/TEZ/IVA after 6 months of treatment. Method(s):We collected prospective data on PwCF who started ELX/TEZ/IVA and evaluated changes in pulmonary function (spirometry and lung clearance index [LCI]), nutritional status (body mass index [BMI]), sweat chloride, and rate of hospitalization from baseline to 6 months of treatment. Result(s): Between August 2021 and October 2021, ELX/TEZ/IVAwas started in 24 PwCF (12 female,10 Phe508del-homozygous, median age 20.5 (range 13-37), all with pancreatic insufficiency). After 6 months of treatment, all respiratory function indicators improved (median change: +16% percentage predicted forced expiratory volume in 1 second, +12% percentage predicted forced vital capacity, +23% percentage predicted forced expiratory flow at 25/75%, -2 lung clearance index). Improvement was also observed in BMI (+0.41 z-score) and sweat chloride concentrations (-54 mMol/L, 6 PwCF had Cl concentrations within the limit of normality) (Table 1). Over a 6-month period, only one hospitalization due to pulmonary exacerbations was observed, compared with 22 hospitalizations observed in the 6 months before starting ELX/TEZ/IVA (rate per 100 patient-months 15.3 vs 0.7, rate ratio 0.05, 95% CI, 0.01-0.29). Treatment was well tolerated, with only mild and transient adverse events consisting of headache (n = 4), cutaneous rash (n = 2), and mild hemoptysis (n = 2). One PwCF had intestinal subocclusion and required hospitalization. One patient had liver function test elevation after 6 months of therapy during an Changes in clinical variables and sweat test results from baseline through 6 months in patients treated with elexacaftor, tezacaftor and ivacaftor. Data are medians (interquartile ranges). Baseline vs 6 months compared usingWilcoxon signed-rank test. ppFEV1, percentage predicted forced expiratory volume in 1 second;ppFVC, percentage predicted forced vital capacity;ppFEV25/75, percentage predicted forced expiratory flow at 25/75%;LCI, lung clearance index;BMI, body mass index;Cl, chloride. (Table Presented) episode of SARS-COV2 infection, which required adjustment of the dose administered. Conclusion(s): Our data confirm that ELX/TEZ/IVA treatment is safe, well tolerated, and effective in PwCF. ELX/TEZ/IVA improved pulmonary function and nutritional status and remarkably reduced hospitalization rate. Our data indicate that introduction of ELX/TEZ/IVA in CF care will radically change the natural history of and management approach to the disease.Copyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Purpose of Study: The post-acute sequelae of COVID-19, as a multisystemic disease have been described in adults. Although some studies have described the pulmonary complications up to 3 months post-COVID infection, longitudinal data on pulmonary sequalae are sparse. The objective of this review was to summarize the findings of studies that included a longitudinal follow-up of patients with moderate to severe pulmonary COVID-19 infection. Methods Used: We performed a literature search using Pubmed, Google Scholar and Medline using key words: "pulmonary function test", PFT?, "long-COVID", longitudinal? and sequalae?. We included studies of adult patients (>18 years of age) who had been hospitalized with acute COVID-19 infection and had at least two follow-ups with PFT measurements, including one follow-up at least 6 months post-infection. Studies that did not account for co-morbidities and other lung diseases or those which only included one-time follow-up were excluded. Summary of Results: Five studies satisfied our inclusion criteria (See Table). The studies showed persistent lung injury for at least 3 months after discharge, with decreased forced expiratory volume (FEV1), total lung capacity (TLC), forced vital capacity (FVC), diffusion vital capacity of the lungs for carbon monoxide (DCLO) and carbon monoxide transfer coefficient (KCO). Although these values improved at 6 and 12 months of follow-up, those with more severe disease continued to have decreased DLCO suggestive of restrictive lung damage. Studies that included symptomatic assessment revealed that a minority of patients continued with fatigue and dyspnea uf to 12 months after the infection. The limitations of the studies include availability of data from a single center, small sample size and the variability in controlling for different co-morbidities. In addition, baseline PFT measurement before COVID-19 infection was not available for most patients. Most of the studies were done at the time that the Delta variant was dominant, therefore the data may not be applicable to other variants. Conclusion(s): Our literature review shows that some adult patients hospitalized with acute covid pulmonary infection continue to have abnormal PFTs for up to 12 months after infection. Although PFTs improve overtime, a minority of patients with more severe disease on admission continue with abnormal functional abnormalities, specifically restrictive ventilatory pattern with impaired DLCO at 12 months of follow-up. It is important that patients hospitalized with moderate to severe pulmonary COVID-19 infection be followed up and managed for at least 12 months after the initial infection. Larger prospective studies including different variants of COVID-19 that take into account various co-morbidities and different management strategies are warranted.
ABSTRACT
The lung is the most common organ affected by sarcoidosis. Multiple tools are available to assist clinicians in assessing lung disease activity and in excluding alternative causes of respiratory symptoms. Improving outcomes in pulmonary sarcoidosis should focus on preventing disease progression and disability, and preserving quality of life, in addition to timely identification and management of complications like fibrotic pulmonary sarcoidosis. While steroids continue to be first-line therapy, other therapies with fewer long-term side-effects are available and should be considered in certain circumstances. Knowledge of common clinical features of pulmonary sarcoidosis and specific pulmonary sarcoidosis phenotypes is important for identifying patients who are more likely to benefit from treatment.Copyright © ERS 2022.
ABSTRACT
Background: People with cystic fibrosis (CF) are more likely to have anxiety and depression symptoms than the general population, with psychological distress being associated with negative health outcomes. The Cincinnati Children's Hospital Medical Center CF Center has been screening people with CF aged 12 and older since 2016 for anxiety and depressive symptoms. Little is known about longitudinal mental health trends for youth with CF, especially during the COVID-19 pandemic. Method(s): Chart review was conducted for individuals aged 12 and older seen for routine care at our center with at least one General Anxiety Disorder (GAD-7) or Patient Health Questionnaire (PHQ-9) screening result between January 2016 and December 2021. Data included demographic characteristics;dates and scores of GAD-7 and PHQ-9;mental health encounters 12 months before each screening date;and clinical variables of disease severity, including percentage predicted forced expiratory volume in 1 second (FEV1pp), body mass index, CF-related diabetes (CFRD), antibiotics in the 28 days prior, and exacerbations in the 12 months prior. Descriptive statistics were used to summarize demographic variables, logistic regression and linear mixed modeling were used to identify predictive relationships, and t-testswere used to compare impact of COVID with that of prior years. Result(s): The sample included 150 individuals with at least one screen across the 6 years. An average of 83 people completed at least one GAD-7 or PHQ-9 in each year. Across the 6-year time period, the percentage of individuals with low scores increased, and the percentage of people with moderate to severe scores was stable (Figure 1). Approximately 35% of individuals were rescreened at least once in a given year because of a previously high symptom score. For thosewho screened in the moderate to severe range (>=10) on initial screens per year, an average of 32% (GAD-7) and 37% (PHQ-9) had a lower score (<10) on their second screen per year. Individuals who scored 10 or higher on initial GAD-7 or PHQ-9 screens in any year were statistically more likely to have a CFRD diagnosis ( p = 0.02, GAD-7;p = 0.02, PHQ-9) and more psychology or psychiatry visits 12 months before the screening date ( p < 0.01, GAD-7;p < 0.01, PHQ-9) than those with minimal scores. In addition, PHQ-9 scores of 10 or greater were significantly associated with lower FEV1pp than low scores. Adherence to screening protocols consistently increased over time. Of all eligible individuals, 56% completed a GAD-7 and 55% a PHQ-9 in 2016, increasing to 92% and 94%, respectively, by 2021, despite the impact of the COVID-19 pandemic on CF care visit frequency. GAD-7 scores were not significantly different before COVID and during COVID ( p = 0.06);PHQ-9 scores were higher before than during COVID ( p = 0.02) despite similar numbers of screens conducted per year. (Figure Presented)Figure 1. Percentage of initial Patient Health Questionnaire (PHQ-9) and General Anxiety Disorder (GAD-7) scores per year from 2016 to 2021 of people with cystic fibrosis seen at Cincinnati Children's Hospital Medical Center Conclusion(s): These longitudinal trends in mental health symptom scores over time are reassuring,with increasing frequency of lowscores and stable moderate to severe scores. This may be because of greater awareness of mental health symptoms, more interventions through care teams, or improved access to resources. Similarly, although general population data suggest worsening of anxiety and depressive symptoms during the COVID- 19 pandemic, we hypothesize thatwewere able to buffer the impact of the pandemic on mental health in our center by screening and responding to screens. These results highlight the importance of consistent monitoring and support for mental health symptoms in people with CFCopyright © 2022, European Cystic Fibrosis Society. All rights reserved
ABSTRACT
Introduction/Aim: Anxiety and depression in adolescents with cystic fibrosis (CF) is 2-3 times higher than those in the general community. The COVID-19 pandemic has been shown to increase levels of distress and difficulties in secondary students in Western Australia (WA). We aimed to examine the prevalence of anxiety and depression symptoms in adolescents with CF living in WA during the pandemic and investigate associations with respiratory symptoms, lung function and quality of life. Method(s): Adolescents with CF aged between 12 and 18 years old were followed at Perth Children's Hospital (Western Australia) between October 2020 and October 2022. Adolescents completed the General Anxiety Disorder-7 (GAD-7) for anxiety and Patient Health Questionnaire-9 (PHQ-9) for depression when clinically stable. We assessed associations between depression and anxiety scores with respiratory symptoms from the Cystic Fibrosis Respiratory Symptoms Diary and Cystic Fibrosis Respiratory Infection Symptoms Scale (CFRSD-CRISS), lung function outcomes (forced expiratory volume in first second (FEV 1) and lung clearance index (LCI)), and quality of life outcomes from the health-related quality of life questionnaire (CFQ-R). Result(s): In 28 adolescents with CF, 39% had an elevated anxiety score (mild and above) and 28% had an elevated depression score (mild and above). No difference in scores was observed between males and females. Both the anxiety and depression scores correlated with the severity of respiratory symptoms from the CFRSD-CRISS questionnaire. Anxiety and depressions scores were not associated with either FEV 1 or LCI. Depressions scores were associated with most CFQ-R domains whilst anxiety scores were only associated with the social CFQ-R domain. Conclusion(s): Depression and anxiety were common in adolescents with CF in our centre. The results demonstrate the importance of depression/anxiety screening and psychological support for adolescents with CF and their families.