Effectiveness and safety of elexacaftor/tezacaftor/ivacaftor therapy: An Italian center experience

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

Pulmonary sarcoidosis

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.

Anxiety and depression in adolescents with cystic fibrosis

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.

Lung clearance index using a novel washout device is elevated in premature infants

Introduction: Lung clearance index (LCI) is a measure of lung gas mixing, and a sensitive indicator of peripheral airway obstruction. In children born prematurely there have been previous reports of elevated LCI at birth and childhood. LCI in infants is highly challenging, requiring either 100% O2 to washout, or use of 4% SF6. Here we report on a novel method using a portable LCI device in non-sedated premature infants. Method(s): LCI measurements were undertaken using 0.1% SF6 and washout on air. Premature infants no longer requiring oxygen were recruited just prior to discharge. Follow up measurements were planned 1 year later. Healthy controls were recruited opportunistically when attending non-thoracic MRI scan under sedation. Result(s): 13 premature babies (mean 30+6 weeks at birth, 37+6 corrected at measurement) were recruited and successful measurements were achieved on 9 (69%). LCI data are available for 36 healthy controls (mean 62weeks old, range 12-154 weeks). Mean LCI of the premature children (8.4, SD 1.6). was significantly higher than that of the healthy cohort (mean LCI 7.0, SD 0.6), p=<0.001. Median coefficient of variation of ex-premature LCI was 6.6%. Mean(SD) FRC was 68.95(47.96)ml (21 ml/kg (SD=3.19)). Only one child attended for a follow- up measurement (study interrupted by COVID pandemic);initial LCI of 7.3 at 38+6 (7 weeks of age) which increased to 8.0 at 61 corrected weeks of age. Conclusion(s): LCI is elevated in premature infants at time of discharge. This can be measured successfully using a portable device without high concentrations of SF6 or O2, and without sedation. The longitudinal change is still to be determined.

Post-COVID pulmonary rehabilitation at primary hospital in Nepal: Preliminary experience from LMIC

Introduction: Nepal is low and middle income country (LMIC) in South Asia with over 11 million SARS-CoV-2 infections reported till February 2022. Decline in lung function with poor functional status have been observed in substantial proportion among post-COVID patients in Nepal. Aims and objectives: The aim of this study was to assess benefits of pulmonary rehabilitation in post-COVID patients at Methinkot hospital, a primary level hospital in Nepal where specialized health services and rehabilitation facilities are not available. Method(s): A cross-sectional study was conducted in December 2021 at Methinkot hospital. Post-COVID patients with persistent symptoms were included. A structured pulmonary rehabilitation program comprising of breathing exercise, airway clearance technique, flexibility and endurance exercise was designed and was delivered by a visiting pulmonary rehabilitation specialist from tertiary hospital. Patients were instructed for thrice weekly home based rehabilitation for four weeks and were assessed at the end of four weeks. Result(s): Forty-two patients were included. Mean age of patients was 38.33+/-10.7 years. There were 17 (40.5 %) male. Shortness of breath on exertion (71.4%), fatigue (52.4%) and chest discomfort (35.7%) were the most common reported symptoms. Upon completion of four-week pulmonary rehabilitation, 34(81%) patients reported symptomatic improvement while 30(71.4%) reported rehabilitation measure to be beneficial. Conclusion(s): Early pulmonary rehabilitation service could be highly beneficial in reducing post-COVID morbidities. Establishment and continuation of sustainable and affordable rehabilitation service is challenging in resource limited setting.

Why Technegas is Fast Replacing 99mTc-Based Aerosols in Lung Scintigraphy

Aim/Introduction: Technegas is fast becoming the gold standard for lung ventilation scintigraphy in Canada. Technegas is a carbonbased nanoparticle bound to 99mTc which is small enough to behave more like a gas than an aerosol. Due to its small size, Technegas can easily perfuse the peripheral airways and does not aggregate in the central airways like larger aerosol particles often do. Furthermore, since the Covid-19 pandemic began, there has been a significant drop in ventilation studies performed due to the risk of exposure to the virus when using traditional aerosols. Improved image quality, fast and easy administration, enhanced patient comfort, and ability to safely administer Technegas with Covid-19 positive patients makes Technegas far superior to traditional Tc-based aerosols. Material(s) and Method(s): A review of the literature comparing Technegas to other commonly used 99mTcbased lung ventilation imaging agents was conducted. Recent purchasers of Technegas units were interviewed to determine their motivation for switching to Technegas and their overall impression with using it after changing over. Result(s): The small particle size, reduced central airways deposition, and lack of lung clearance leads to improved count-statistics and better target-to-non-target ratio, which allows for SPECT imaging, which is not feasible when using traditional aerosols. Technologists report that administering Technegas is far easier and faster than administering traditional aerosols. Often patients only require 1 or 2 breaths of Technegas to achieve the desired count rate, as opposed to 5 or more minutes of breathing an aerosol. Technegas is a 'dry' aerosol, which means that it is considered a non-aerosol generating procedure, so it is safe to use on Covid-19 positive patients. Justifying the small start-up cost to purchase a Technegas generator was the limiting factor for departments delaying their switch to Technegas. Canada's health care system is publicly-funded, which often delays widespread access to technological advancements. The Covid-19 pandemic has increased demand for Technegas across Canada, which has resulted in increased funding to purchase more Generators. Conclusion(s): Technegas is far superior to other 99mTc-based aerosols used in ventilation imaging and is fast replacing Tc-based aerosols as the agent of choice in nuclear medicine departments across Canada.

Why Technegas is Fast Replacing 99mTc-Based Aerosols in Lung Scintigraphy

Aim/Introduction: Technegas is fast becoming the gold standard for lung ventilation scintigraphy in Canada. Technegas is a carbonbased nanoparticle bound to 99mTc which is small enough to behave more like a gas than an aerosol. Due to its small size, Technegas can easily perfuse the peripheral airways and does not aggregate in the central airways like larger aerosol particles often do. Furthermore, since the Covid-19 pandemic began, there has been a significant drop in ventilation studies performed due to the risk of exposure to the virus when using traditional aerosols. Improved image quality, fast and easy administration, enhanced patient comfort, and ability to safely administer Technegas with Covid-19 positive patients makes Technegas far superior to traditional Tc-based aerosols. Material(s) and Method(s): A review of the literature comparing Technegas to other commonly used 99mTcbased lung ventilation imaging agents was conducted. Recent purchasers of Technegas units were interviewed to determine their motivation for switching to Technegas and their overall impression with using it after changing over. Result(s): The small particle size, reduced central airways deposition, and lack of lung clearance leads to improved count-statistics and better target-to-non-target ratio, which allows for SPECT imaging, which is not feasible when using traditional aerosols. Technologists report that administering Technegas is far easier and faster than administering traditional aerosols. Often patients only require 1 or 2 breaths of Technegas to achieve the desired count rate, as opposed to 5 or more minutes of breathing an aerosol. Technegas is a 'dry' aerosol, which means that it is considered a non-aerosol generating procedure, so it is safe to use on Covid-19 positive patients. Justifying the small start-up cost to purchase a Technegas generator was the limiting factor for departments delaying their switch to Technegas. Canada's health care system is publicly-funded, which often delays widespread access to technological advancements. The Covid-19 pandemic has increased demand for Technegas across Canada, which has resulted in increased funding to purchase more Generators. Conclusion(s): Technegas is far superior to other 99mTc-based aerosols used in ventilation imaging and is fast replacing Tc-based aerosols as the agent of choice in nuclear medicine departments across Canada.

43 Jahrestagung der Gesellschaft fur Padiatrische Pneumologie

The proceedings contain 85 papers. The topics discussed include: T2-high asthma across all ages - comparative analysis in children and adults from the ALLIANCE cohort;targeting IL-6 to prevent vascular and bronchial remodeling in an experimental model of bronchopulmonary dysplasia;diagnostic testing in people with primary ciliary dyskinesia around the world: where do we stand?;effects of elexacaftor/tezacaftor/ivacaftor in children with cystic fibrosis: a comprehensive assessment using spirometry, lung-clearance-index, functional and structural lung MRI;cough phenotypes in children: findings from the Swiss Pediatric Airway Cohort;pleuropneumonia caused byNocardia cyriacigeorgica in a 14-year-old girl with PCD;in severe juvenile-onset recurrent respiratory papillomatosis of a 10-year-old, systemic bevacizumab is highly effective and well tolerated;and impact of COVID-19 related alterations in circulating respiratory viruses on children and adult patients with recurrent wheeze and asthma.

Pathomorphological changes in some organs and tissues of COVID-19 convalescent patients

New coronavirus infection (COVID-19) is highly contagious viral disease caused by SARS-CoV-2 leading to the pandemic. The autopsy of COVID-19 patients often showed features of previous brain diseases including neurodegeneration, previous strokes, demyelinating diseases and atherosclerosis. Patients with acute cerebrovascular accidents and severe COVID-19 had higher numbers of lethality in comparison to non-severe course of infection without cerebrovascular accidents. A comparative analysis of morphological changes in lungs of deceased patients who died in different periods of first clinical symptoms is to be conducted. Objective. Description of pathomorphological changes in deceased patients during the period of reconvalescence. Patients and methods. The analysis of 15 fatal cases which took place in Botkin Hospital with the diagnosis of ischemic stroke and new coronavirus infection in the previous 2-4 months has been held. Macro and microscope examination of brain, lungs, brachiocephalic arteries, kidneys and liver has been carried out. Results. All patients had morphological features of ischemic damage of grey matter in the brain. Beside necrosis of neurocytes with diffuse infiltration in the grey matter, hematoxylin cycles were found, in some cases they were placed in a perivascular way in choroid plexus. Also 5 patients suffered a myocardial infarction up to 3 days. 10 patients had structures disorganisation in areas of lung parenchyma with hystoacrchitectonic changes because of the fibrosis. Alveoli in some places collaborated mostly with single airway clearance. The fact that most patients had lung hemosiderosis can prove coronavirus infection suffered earlier with microcirculatory bed damage. Conclusion. Thus, morphological changes seen in the period of reconvalescence of COVID-19 is a result of pathomorphosis of changes described earlier for acute period of coronavirus infection and affect not only lungs, but also other organs and tissues. This proves systematic characteristic of the infection.

International collaboration and development of a cystic fibrosis physiotherapy-specific telehealth toolkit

Objective: The COVID-19 pandemic triggered a worldwide need for telehealth services. Previously providing services in remote communities, virtual consultations were implemented to accommodate the needs of people with CF (pwCF) who were shielding and unable to attend faceto- face consultations. Method: This group has compiled a CF Physiotherapist’s toolkit of essential elements necessary to enable delivery and promotion of safe, equitable virtual sessions, specific to pwCF. Through international collaboration and shared experience, the toolkitwas developed to support physiotherapists working across paediatric and adults with CF. Results: Centres implementing telehealth into routine care need rigorous methods to evaluate safety and effectiveness ensuring optimal care. Using telehealth for joint sessions with shared care centres, or during transitional periods, should be considered ensuring equitable access and specialised care for all. The health benefits of telehealth, the coincident enhanced control of cross infection and resource savings, to the hospital and the person/family with CF in time and travel costs, are invaluable. A blend of virtual and faceto- face consultations could be the mainstay of future CF care. CFTR modulator therapy, improved specialised care, and improving life expectancy is compelling clinicians to review current services, improve efficiencies and continually optimise care and health outcomes. Telehealth, may provide an option for alternative models of care which may not be suitable for all but should be considered as an option in future CF services. Conclusions: Using this toolkit, the CF Physiotherapist is provided with relevant guidance and support for delivery of online/virtual respiratory review, spirometry assessment, evaluation of inhalation therapy, airway clearance and exercise opportunities. The toolkit promotes an equitable translation from face-to-face care to virtual care and includes strategies for risk mitigation in the virtual setting.

Extubated VV-ECMO for COVID-19 ARDS in an Immunosuppressed Pediatric Renal Transplant Patient

Extracorporeal membrane oxygenation (ECMO) for coronavirus disease 2019 (COVID-19) associated acute respiratory distress syndrome (ARDS) in adults is common, and outcomes are similar to those for non-COVID-19 ARDS on ECMO1. However, children are much less likely to have severe COVID-19 disease, and thus the need for extracorporeal support is rare2,3. Even pediatric solid organ transplant recipients with active COVID-19 infections typically do not require ECMO.4 We present the case of an immunosuppressed pediatric patient with COVID-19-related ARDS who had an excellent outcome on VV-ECMO. An 11-year-old female with a history of polycystic kidney disease status post renal transplant three years earlier was admitted to the pediatric intensive care unit (PICU) with COVID-19 pneumonia. She developed severe ARDS and required intubation on hospital day (HD) 19. The patient's condition deteriorated despite trials of prone positioning, nitric oxide, and neuromuscular blockade. Due to her refractory hypoxemia, she was cannulated to venovenous (VV) ECMO on HD 28 utilizing a 27 french Avalon bi-caval dual lumen cannula. Laboratory testing demonstrated severely impaired immune function due to her baseline immunosuppressive regimen of mycophenolate, prednisone, and tacrolimus. In addition, she had received B cell depleting therapy with Rituximab several months prior to admission for management of transplant rejection. During her ECMO run, she developed pulmonary aspergillus in addition to her COVID-19 infection. Thus, the overall treatment goal was to minimize immunosuppression sufficiently to manage the COVID-19 and aspergillus infections while not triggering rejection of her transplanted kidney. On admission, her mycophenolate dose and tacrolimus trough goal were reduced. When her status worsened shortly after intubation, mycophenolate was discontinued, and tacrolimus trough goal was further decreased. For treatment of acute COVID-19 pneumonia she received remdesivir, dexamethasone, tocilizumab, and convalescent plasma. Farther into her course, she was also treated for suspected MIS-C with anakinra, IVIG, and methylprednisolone. COVID-19 polymerase chain reaction (PCR) and cycle threshold times were monitored weekly. Gradually her cycle threshold times increased, and her COVID-19 PCR became negative on HD 61. With this laboratory and clinical improvement, her mycophenolate was restarted at 50% of the previous dose, and the tacrolimus trough goal was increased to its previous level. There were no concerns for graft rejection during her hospitalization. However, continuous renal replacement therapy (CRRT) was required in tandem for the first four days of extracorporeal support. Following this, she maintained good renal function with her transplanted kidney for the remainder of her hospital course. Five days after VV-ECMO cannulation she was extubated to a high-flow nasal cannula. With weaning of neurosedative infusions, she was able to participate in pulmonary clearance and physical rehabilitation while on ECMO. With aggressive rehab she could sit on the edge of her bed and even transfer to a chair with assistance. After 33 days of ECMO support, she was decannulated on high-flow nasal cannula. She left the PICU on HD 83 and was discharged home without supplemental oxygen on HD 104. Our case experience suggests that extubated VV-ECMO can be a safe and effective rescue therapy for COVID-19-related ARDS in children, even in the setting of immunosuppression. Modification of immunosuppressive regimens in the setting of acute COVID-19 disease is not well described in the pediatric population.3 Our case study demonstrates it is possible to balance the risk of transplant rejection with infection control and ECMO therapy. Immunocompromised pediatric patients with COVID-19 can be considered candidates for ECMO support.

Pulmonary function tests in the follow-up of children with COVID-19.

The SARS-CoV-2 virus has infected more than 235 million people since it was accepted as a pandemic in March 2020. Although a milder disease is seen in the pediatric age group, the extent of lung damage and its long-term effects are still unknown. In this study, persistent respiratory symptoms and pulmonary function tests were investigated in children with COVID-19. Fifty children with a confirmed diagnosis of COVID-19 were included in the study. Patients were evaluated for ongoing respiratory symptoms and pulmonary function tests 3 months after infection. Patients with and without persistent symptoms were compared in terms of demographic, clinical, laboratory, and radiological characteristics and also disease severity. Three months after infection, persistent respiratory symptoms were found to be present in 28% of patients; cough, chest pain and tightness, dyspnea, and exertional dyspnea were the most common symptoms. Three patients had an obstructive deficit, and one had a restrictive deficit. Four patients had impaired diffusing capacity of the lungs for carbon monoxide (DLCO). A significant decrease in FEV1/FVC and an increase in lung clearance index were found in the patients with persistent respiratory symptoms. Persistent respiratory symptoms were present in 50% of patients who had severe disease and 12.5% with non-severe disease. DLCO was also significantly lower in the severe disease group.   Conclusions: Our study suggests that the persistence of respiratory symptoms is not related to the severity of acute COVID-19 in children. The inflammatory process due to COVID-19 may continue regardless of its severity, and consequently, peripheral airways may be affected. What is Known: • As compared with adults, children with COVID-19 exhibit a milder disease course and lower mortality rates. However, due to the lack of follow-up studies on children, the long-term effects of their contracting the disease are unknown. What is New: • Although COVID-19 has been thought to have a milder course in children, respiratory system symptoms persist in approximately 30% of patients 3 months after infection. The persistent respiratory symptoms suggest that the inflammatory process due to COVID-19 may continue in some children, even if the clinical findings at admission are not severe, and that the peripheral airways may be affected accordingly.

RESPIRATORY DISTRESS IN A NEONATE - WHEN BOARDS MEET REAL LIFE

Case Report A male infant is born at 37w to a 34-year-old G3P2 mother by vaginal delivery after an uncomplicated pregnancy. Prenatal screens are negative. The patient had a birth weight of 2,620 g, with Apgar scores of 9 and 9. On day 2 after birth, had increased work of breathing which prompted transfer to a level II NICU for further management. On arrival to the unit, the infant is tachypneic with mild chest wall retractions and thick nasal secretions. A CBC and blood culture were collected and empiric antibiotic therapy was started. Respiratory viral panel and COVID test are negative. A chest radiograph shows a middle lobe opacity concerning for pneumonia (figure 1). His clinical status failed to improve and on day 4 after birth, supplemental oxygen was provided. The primary team consulted ENT and Pulmonology services. Flexible laryngoscopy showed a normal anatomy. Pulmonology recommended transferring to our NICU for a chest CT with bronchoscopy. Our differential diagnosis for this neonate with respiratory distress that fails to improve over time or with antibiotics was broad, but further testing revealed this infant's condition. A CBC, CRP and a blood gas were collected on admission and were normal. ID service was consulted. A Chest CT showed bilateral atelectasis. Bronchoscopy showed a normal anatomy. Bronchoalveolar lavage was sent. Umbilicus swab was positive for MRSA, nasal wash/sputum culture/bronchoalveolar fluid also grew moderate S. aureus. Nasal ciliary biopsy sent for electron microscopy. Positive umbilicus and nasal swab, and subsequently BAL for MRSA led to a diagnosis of MRSA neonatal rhinitis. Therapy with IV vancomycin was initiated and later changed to oral clindamycin to complete a total of 14 days of therapy. The neonate was weaned off oxygen support on day 11. His clinical symptoms improved. He was discharged on oral clindamycin with follow up appointments with pulmonology and ID clinics. His ciliary biopsy showed absence of outer and inner dynein arms, compatible with the diagnosis of primary ciliary dyskinesia (PCD) (figure 2). Genetic testing for PCD showed mutations in the DNAAF1 and CCDC40 genes. This neonate was diagnosed with primary ciliary dyskinesia (PCD) but his presentation at birth was nonspecific and the differential diagnosis was broad. There is no gold standard diagnostic test for PCD and high clinical suspicion is important. Since it is most likely an AR inheritance, screening of family members is essential. Initial management of neonates may include measures that manage the respiratory distress, airway clearance to prevent respiratory infections and treat bacterial infections. Chest physiotherapy may help if recurrent atelectasis. Flexible bronchoscopy and bronchoalveolar lavage may help both to diagnose and treat the underlying infection. Antibiotic therapy based on organism growth for exacerbations may prevent development of bronchiectasis. (Figure Presented).

Chronic lung disease following covid-19 in an infant

Case Report Chronic respiratory sequelae are well documented in adults after COVID-19 infection, however, in young children and infants, evidence is still evolving. Here we report an infant with significant chronic respiratory complications after COVID-19. Case Report A 10 month old female with no significant past medical history was admitted to the PICU secondary to hypoxemia, respiratory distress, and respiratory failure following COVID-19 infection in January 2021. She was also positive for Rhinovirus and Enterovirus. CXR displayed worsening bilateral alveolar infiltrates, and she developed subsequent pneumothorax requiring a chest tube. Apart from mechanical ventilation, she received supportive treatment and broad spectrum antibiotics. Cardiac echocardiogram revealed pulmonary hypertension, PFO, and PDA. Due to worsening respiratory status and hypoxemia, she received bronchodilators, inhaled nitric oxide, sildenafil, steroids, and magnesium. After 3 weeks, her respiratory status improved and she was discharged. The patient required another hospitalization in March and an ER visit in April for persistent cough and shortness of breath. After evaluation by pulmonology, she began inhaled steroids and airway clearance treatments including chest physical therapy, hypertonic saline, and bronchodilators. Further workup ruled out cystic fibrosis, primary ciliary dyskinesia, and immunodeficiency. Chest CT showed diffuse bilateral patchy airspace opacities representing atelectasis and scarring. Despite a short period of improvement, the patient was hospitalized for respiratory distress in June, where she was hypoxemic and diagnosed with pneumonia. She required repeated outpatient visits to the PCP for persistent respiratory symptoms. PDA closure was performed in September. The patient continues to have persistent respiratory symptoms addressed with outpatient respiratory treatment regimen. Conclusion As we have ruled out other underlying causes, the patient's chronic lung disease and persistent respiratory symptoms occurred most probable secondary to COVID-19. This case report highlights the importance of monitoring respiratory symptoms in pediatric patients with severe COVID-19 infection for early identification of chronic respiratory sequelae.

A retrospective service evaluation of a virtual respiratory physiotherapy outpatient clinical service

Keywords: Virtual;Airway clearance;Outpatients Purpose: The COVID 19 pandemic at the start of 2020 resulted in non-urgent outpatient services being temporarily cancelled. Following national guidance our respiratory outpatient service was challenged on how it could be provided virtually. Telephone clinics emerged as a solution however this would not allow for effective assessment, teaching and modifying a patient's airway clearance technique (ACT). The use of virtual clinics within other specialities, i.e. CF is already being used successfully. Aim: To review the effectiveness of a virtual service delivery for Airway Clearance in a chronic respiratory population and to guide future use of technology in outpatient services. Methods: We conducted a retrospective audit of virtual provision of Airway Clearance to a respiratory outpatient population. Patients’ attendance and outcomes were reviews. Results: Forty-seven 47 patients (36% male, mean (SD) age 57 (16)) were referred to the respiratory outpatient physiotherapy service to establish an ACT between March 2020 and 2021. 70% were diagnosed with bronchiectasis. 87% attended appointments virtually with 74% via video call. To establish an effective ACT, it took 2 appointments of 30 min. 58% (24) were taught acapella, 40% (16) ACBT and 2% (1) another ACT. 5% (2) of patients did not have English as their first language, nor a family member present, and required advocates to translate during the appointment. Of those who attended virtually, 1 patient was admitted to hospital within 3 months of being discharged from the service with an infective exacerbation of bronchiectasis. 6% (3/47) declined a virtual consultation with their main reason being access to technology (60% male, mean (SD) age 46 (3)). Our DNA rate was 18%. Conclusion(s): Our findings indicate that virtual clinics are feasible for respiratory assessments and teaching an ACT. Patients could be taught different types of airway clearance within a few appointments, with no adverse events. The impact of the COVID pandemic resulted in changes to outpatient services occurring with immediate effect therefore it is difficult to compare these outcomes to face to face clinics from previous years. As face to face appointments were unavailable, patients had limited choice on their appointment type during this time period However this change in practice helps overcome other historical barriers for face to face appointments that were being faced prior to the pandemic, i.e. Travel distance and cost, infection control, time off work. Admission rates may have also been lower due to a variety of reasons, including shielding resulting in reduced exposure, reluctance from patients to be admitted to hospital. They do not reflect exacerbations managed at home via GP's. Barriers identified were around access to technology. Further work is required to identify if there is long term carry over and effectiveness of these treatments. Qualitative data on patient's perceptions of a virtual clinic would be valuable to guide any long term changes to the service. Impact: The project has enabled a service design change that may improve better access to our respiratory outpatient service. It also indicates a potential path for digital transformation within respiratory outpatient physiotherapy. Funding acknowledgements: The work was not funded.

Taking cystic fibrosis physiotherapy homecare into the virtual era

Keywords: Virtual;Community;Cystic fibrosis Purpose: The requirement for people with cystic fibrosis (PwCF) to shield during COVID19 led to face-to-face contact with the Physiotherapy service being limited. Historically, the Physiotherapy Homecare team completed most of their work through home visits to PwCF during home IVs, pregnancy, exercise support, oxygen assessment, and to optimise airway clearance and nebulised therapies within their own environment. The service covers a wide geographical area ranging from Cumbria to Wales, therefore the Homecare team were often required to drive long distances to deliver the service. The aim was to rapidly transform the service, embracing all the technological advances available to maintain the delivery of care to PwCF in their own home during COVID19. Methods: The service facilitated a rapid distribution of home spirometry devices (Nuvoair) supported by an online portal and app, which was supported by funding from NHS England for all cystic fibrosis centres during the pandemic. Video calls were implemented via Attend Anywhere for treatment sessions and during home IV ward rounds. The service continued to utilise phone call and text message monitoring already established. Results: Over 12 months (April 2020 to March 2021) 70 PwCF were supported by the team. An average of 116 direct contacts per month were delivered. Of these contacts, three were home visits resulting in the team driving 42.8 miles. This compares to a 12-month period prior to COVID19 (January to December 2018) when 194 home visits were carried out resulting in driving in excess of 6000 miles. Patient feedback was gathered from those who had experienced support from both the original home visits and then virtual support. Comments have included: “flexibility of virtual follow up has meant I’ve had more contact allowing quicker changes to my airway clearance and improved adherence rather than 1 visit a month”;“awkward doing airway clearance, like hands on physio”;“can do virtual sessions anywhere”;“no difference between virtual and home visits” and “mixture of virtual and home visits would my preference.” Conclusion(s): It was possible to transform the service into a fully virtual service and maintain patient contact at a satisfactory level. However, this would benefit from a further review against the CF standards of care in the future. Despite the positive feedback and success of maintaining the service there were also limitations such as reduced objective markers, poor internet streams, dislike of technology and inability to evaluate the home environment that would prevent the service being fully virtual in the future. There was a huge reduction in mileage over the year which translates to a cost saving. However, this could be offset by cost associated with telehealth devices when no longer funded by NHS England. Impact: Implementing telehealth and virtual contacts has the potential to increase productivity and efficiency by reducing travelling for patient contacts, though there were some limitations found. In the future a combination of virtual follow ups and home visits will support a robust, bespoke homecare service that is cost effective, meets CF standards and offers flexibility for patients. Funding acknowledgements: This work did not receive any funding.