National Survey on Pediatric Respiratory Physiotherapy Units: Primary Ciliary Dyskinesia and Non-CF Bronchiectasis

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Methods: A national descriptive study based on a survey questionnaire was conducted. The questionnaire consisted of three different sections: distribution and characteristics of the centres, services provided by respiratory therapists, physiotherapists’ perception of the unit. The survey was distributed to all healthcare providers via an online platform, and a descriptive data analysis was performed. Results: The survey had a response rate of 97.5% with twenty-nine responses collected. The centers are heterogeneously distributed: thirteen in the northern regions, eight in the central regions and eight in the southern regions. Of the 29 centers with a physiotherapy unit, 19 had a specialized respiratory therapy unit. Respiratory therapy was provided in different care settings: regular wards (28/29 centers, 97%), outpatient service (29/29 centers, 100%), and intensive or semi-intensive care units (17/29 centers, 59%). The interventions provided by respiratory therapists involved more than just airway clearance (29/29). More specific interventions, such as pulmonary function tests (23/29), functional tests (27/29), educational training (26/29), management of workout exercise programs (25/29) and interventions developed in collaboration with physicians such as non-invasive ventilation (NIV) (23/29) and oxygen titration (21/29) are performed. It is interesting to note that therapists are also involved in various activities, such as telemedicine, physiotherapists’ research projects, and supporting alongside physicians, for the prescription at home of medical devices. Perception of the unit was also evaluated. Conclusions: The involved centers are heterogeneous in terms of the distribution and treatments offered. The role of respiratory physiotherapists still seems to be fragmented. This first descriptive analysis of the physiotherapy units and the main differences between centers opens query on the clinical approaches used for pediatric patients with PCD in terms of respiratory physiotherapy. However, in response to evolving treatment needs, a more specialized and standardized approach to patient care is required. Primary Ciliary Dyskinesia non-Cystic Fibrosis bronchiectasis pediatrics physiotherapy unit respiratory physiotherapy survey physiotherapy procedures airway clearance Figures Figure 1 Figure 2 Figure 3 Figure 4 1 BACKGROUND PCD and NCFB are two rare conditions affecting the respiratory system. PCD is a genetic disorder of motile cilia, primarily transmitted as an autosomal recessive trait ( 1 ). Motile cilia are located on the apical surface of the upper and lower respiratory tract, on the ependymal cells lining the ventricles of the central nervous system, as well as on the oviducts of the female reproductive system and in the flagellum of male spermatozoa ( 2 ). The clinical phenotype of PCD is broad but respiratory manifestations are part of the typical presentation of the disease and are central in the management and treatment of patients ( 3 ). On the other hand, NCFB has been recognized as a clinical problem in children for approximately two centuries ( 4 ) and is characterized by progressive and often irreversible bronchial dilatation due to structural changes in the bronchial wall and chronic airway inflammation ( 5 ). NCFB is a heterogeneous disorder and recognizes several underlying etiologies, including PCD, which has been suggested in various studies to be the primary cause of the onset of this disease ( 6 ) ( 7 ) ( 8 ). Both conditions are characterized by daily productive chronic cough ( 5 ) and recurrent upper and lower respiratory tract infections ( 9 ) ( 3 ). To date, chest physiotherapy is one of the cornerstones of treatment and prevention of exacerbations and management of bronchial secretions ( 10 ) ( 11 ). Treatment should be initiated in early childhood ( 12 ). Currently, therapeutic strategies for PCD and NCFB do not base on validated disease-specific recommendations, but often refer to the available evidence for Cystic Fibrosis (CF) ( 3 ) ( 13 ). Physiotherapy treatments should follow common protocols and the approaches offered should not differ between centers. To the best of our knowledge, no data have been published on the organization of physiotherapy units for pediatric patients with PCD and NCFB in Italy. The primary objective of this survey was to identify Italian pediatric centers with a respiratory therapy team and to determine their characteristics and the services offered, with the aim of obtaining a primary overview of the Italian context and set up of a professional network. 2 MATERIALS AND METHODS A multidisciplinary team, consisting of respiratory therapists and pediatric respiratory physicians, designed a survey questionnaire to be distributed to Italian pediatric centers treating PCD and NCFB. Given that the Italian National Healthcare system does not currently centralize care for PCD and NCFB ( 14 ), we reached out the major number of CF centers, children's hospitals, and general hospitals for our study. The questionnaire was designed to assess the services provided in Italian healthcare providers in relation to respiratory therapy. In order to involve as many centres as possible, we contacted all the members of the SIMRI (Italian society of pediatric respiratory diseases) study group "PCD and NCFB" and the physiotherapy group of the SIFC (Cystic Fibrosis Italian Society). Centers that did not treat pediatric patients with PCD or NCFB were excluded from the survey. Clinicians who declared not to have a physiotherapy service at their centers, were excluded from the survey and were subsequently contacted to inquire about the physiotherapy management of the patients with a shorter set of questions sent by email. The questionnaire was sent via an online survey platform to the physiotherapist head of department (or delegate) of each unit. One week before the survey was distributed, an email was sent to each participant explaining the purpose of the survey. The survey was sent out in May 2023, and responses were collected until August 2023. Centers were prompted through a weekly email reminder. 2.1 Questionnaire We investigated various aspects related to the organization of physiotherapy units. Specifically, the questionnaire was divided in three sections: Distribution and characteristics of the centers Services provided by respiratory therapists Physiotherapists’ perception of the unit 2.1.1. Distribution and characteristics of the centers To obtain a national overview of the centers, we first identified the location of each hospital and the corresponding Italian region. Next, we asked in detail total number and age of the patients with NCFB and PCD followed in each unit. 2.1.2. Services provided by respiratory therapists The second section of the questionnaire investigated the physiotherapy unit. In particular, the team composition, the existence of a specific respiratory physiotherapy unit, the tasks of the physiotherapists and the settings of intervention. Finally, we inquired the instrumental and functional tests performed by the team. 2.1.3 Physiotherapists’ perception of the unit Physiotherapists’ perception was investigated in two different areas. First, team's skill in airway clearance was assessed. Five different airway clearance techniques were analyzed and a Likert scale (No skills – Highly specialized) was used to determine the perceived mastery. Secondly, the availability of instruments and devices was assessed. 2.2 Analysis This descriptive study was based on data obtained from the participating centers' questionnaires. Once all available responses were collected, data were entered into a database for analysis. All data analysis was performed by the principal investigator and shared with other researchers. Data were collected in a database and statistical analysis and graphs processing were performed using Excel (16.0 Software). Response rate was calculated taking into account responders and missing in relation to the involved centers. Absolute frequency and percentage were calculated for categorical variables, and the mean value was calculated for continuous variables. For open-ended questions, responses were categorized and analyzed narratively. To measure liking of the procedure a 5-point Likert scale was used. Since the survey included only general information and did not disclose any sensitive patient data, formal approval from the Ethical Committee was not sought. 3 RESULTS As shown in Fig. 1 , out of 40 centers contacted, 39 replied (97.5%). Ten centers were excluded from the survey: seven reported not to treat pediatric patients with PCD or NCFB and three centers reported not have physiotherapy unit/physiotherapists for respiratory care. To these centers was provided a shorter set of questions about the physiotherapy management for their patients, with responses provided by the contacted physician. For the three centers, patient care was delegated to other facilities outside the region of residence (n = 1), to nurses (n = 1) and to physicians only for techniques review (n = 1). 3.1 Distribution on the territory and centers’ characteristics The distribution of centers with a physiotherapy unit for respiratory care that completed the survey, and the relative number of PCD and NCFB patients managed is represented in Fig. 2 . We managed to reach centers from the north to the south of Italy and showed a heterogeneous distribution of centers throughout the country. Four Italian regions were not represented (3 did not retrieved, 1 reported not having a center with the physiotherapy unit). Thirteen centers are located in the north Italian area, 8 in the center and 8 in the south. Among the included centers, 25/29 (86,2%) took in charge also CF patients. 3.2 Services provided by respiratory therapists Regarding the organization of physiotherapy team, many differences were noted. Of the 29 centers with a physiotherapy unit, only 19 had a specialized respiratory therapy unit, whereas 10 offered a general physiotherapy service. Of these 10 units, 4 reported spending < 50% of their daily time on respiratory care, whereas 6 reported spending ≥ 50% of their daily time on the respiratory care. The centers had an average of 4.1 professionals per unit in the north, 3.4 in the center, 1.8 in south. Respiratory therapy intervention was administered in different care settings: regular ward (28/29 center, 97%), outpatients (29/29 center, 100%), intensive or semi-intensive care setting (17/29 center, 59%). The interventions performed by respiratory therapists are shown in Fig. 3 . These interventions include more than just airway clearance, which is the common element for the centers (29/29, 100%). Physiotherapists performed more specific interventions such as pulmonary function tests (such as spirometry, DLCO, etc.) (23/29, 79,3%), functional tests (such as 6MWT, SPPB test, etc.) (27/29, 93,1%), educational training (26/29, 89,6%), management of workout programs (25/29, 86,2%) and intervention developed in collaboration with physicians, as NIV (23/29, 79,3%) and oxygen titration (21/29, 72,4%). According to tests and procedures performed by physiotherapists, major geographical differences were found in the hospital ward activities. In addition to airway clearance techniques (100% north, center, south), units are usual to perform mostly pulmonary function tests (84.6% north, 87,5% center, 100% south) and functional tests (92,3% north, 100% center, 100% south) in outpatient regimen, conversely oxygen titration (69,2% north, 62,5% center, 12,5% south), NIV titration (92,3% north, 62,5% center, 12,5% south) and the set-up of workout programs (69,2% north, 87,5% center, 50% south) are performed mainly in hospital wards. Table 1 summarizes the tests and procedures more frequently performed by therapists both outpatient setting and hospitalization care. Table 1 Tests and procedure performed by physiotherapists Moreover, we also asked to describe additional services provided by therapists in each center. Therapists were also involved in different activities such as telemedicine, research projects, and in the support, alongside physicians, for prescription at home of medical devices. Additionally, the collection of biological material for microbiological examination is often entrusted to respiratory therapists. 3.3 Perception of physiotherapists of the unit Figure 4 represent the self-assessment Likert scale of team's skill in five different airway clearance techniques. Secondly, concerning physiotherapists’ perception of the units’ devices availability, 17% of the therapists consider it inadequate. 4 DISCUSSION This study provides, for the first time, an overall view of the national distribution, organization, and structure of physiotherapy departments following pediatric patients affected by PCD and NCFB. Previous surveys on physiotherapy have mainly focused on techniques and clinical management of patients ( 15 ) ( 10 ), and more specific data on the Italian physiotherapy units is lacking. The importance of services provided by hospital-based physiotherapy departments is increasingly recognized, particularly in the multidisciplinary treatment of respiratory diseases. This need is even greater for rare conditions as PCD and NCFB, which, as has been reported in the literature over the last decade ( 16 ) ( 10 ) require the establishment of daily airway clearance treatment and individualized exercise programs to reduce the decline of lung function over time. For this reason, it seemed appropriate to study the distribution of physiotherapy units on the Italian territory that follow pediatric PCD and NCFB patients, and then try to identify the organization and services offered by each center. In our survey we achieved an excellent response rate of 97.5%, confirming the positive attitude and increasing interest of Italian physiotherapists to be engaged in research projects. Unfortunately, we are missing data from three regions (Valle D’Aosta, Molise, Basilicata) in our survey. As hypothesized in our previous national research project ( 14 ), this can be related to the absence of specialized centers in those regions or to our difficulties in reaching all the territorial centers. Moreover, the three centers lacking a physiotherapy unit for respiratory care, declared to refer patients to facilities outside the region or to medical/nurse care. Our data reveals the challenge in finding professional physiotherapists trained in respiratory care. We all agree that a well-planned chest therapy program is essential, and the competencies that physiotherapists are acquiring over time are of paramount importance, as suggested by the literature ( 17 ) ( 3 ). These data, highlighting the potential absence of professional respiratory therapists in some Italian regions should be seriously taken into account. Analyzing the distribution and features of the centers, the primary distinction lies is in terms of territorial distribution, particularly between the northern (13 centers) versus center/southern Italian area (8 centers/8 centers). The heterogeneity of units is evident, and this diversity extends to the organization of logistic teams. Respiratory therapy predominantly takes place in outpatient and regular ward settings as observed in 100% and 96,5% of centers respectively. In contrast, intensive and semi-intensive care units play a partial role in the work of physiotherapists, with interventions being conducted in only 17 out of 29 centers. Furthermore, concerning team composition, among the 29 centers, 19 (65%) have a dedicated respiratory team. Nevertheless, therapists still spend ≥ 50% of their time on respiratory care in 6 out of 10 units. This leads us to hypothesize that the need for respiratory physiotherapy is definitively growing due to a progressively greater number of diagnosed rare respiratory diseases or an increasing importance given to respiratory physiotherapy treatments. In our survey we retrieved a decreasing average number of respiratory therapists per center, going from the north to the south region of our country. We could hypotize that the northern centers have a greater availability of respiratory therapists. However, we cannot show if this data is related to a major workload of patients treated from the unit. Considering the interventions made by respiratory therapists, our survey showed that professionals not only play an irreplaceable role in developing an airway clearance program. In fact, assessment of patient condition through tests and pulmonary function tests, educational training for caregivers, titration of oxygen definition of NIV requirements, and workout programs are usual care activities in which Italian respiratory therapists are involved. This highlights the crucial role of respiratory therapists on multidisciplinary teams dealing with PCD and NCFB ( 18 ). Moreover, we appreciate how physiotherapists are expanding their expertise in respiratory care, and the growing competence of these professionals is aligning with the increasing knowledge and consideration of these rare diseases. Additionally, our survey revealed heterogeneity between healthcare providers in the interventions provided by physiotherapists, possibly due to the unique needs and specificities of each center. In the majority of centers, therapists perform walking tests and spirometry. However, it is important to note that in almost 20% of centers, tests such as bronchial provocation and dilation tests and Multi-Breath Washout tests, are also conducted by physiotherapy units. Definitively fewer centers perform more specialized procedures such as DLCO, FOT and IOS. These results show that according to the specific needs of centers, physiotherapists are professional figures that can cover various respiratory tests and functional evaluations. Related to the professional perception of the offered service, our study emphasized that although airway clearance is a core element of the treatment of PCD and non NCFB, there is still a varying perception of the confidence of the different airway clearance techniques. In many cases, therapists do not feel fully confident when using more complex techniques, such as airway clearance using instrumentation (as IPV, cough machine, EFA and HFCWO) and with NIV. Moreover, the adequacy of devices available was considered inadequate in 17% of units. This result strongly highlights the importance of addressing the training needs of professionals involved in the respiratory field and further implementing the devices needed in all centers. This could ensure professionals and patients to have the same clinical possibilities for high quality treatment. Finally, our results raise new questions about the evolution of respiratory therapists’ skills. These include telemedicine, chest ultrasounds and collaboration with physicians in the titration and training process for NIV and oxygen therapy. This evolution could be explained by the need for a more specialized approach to patient care. Forming a network of professionals throughout the national territory is crucial to address the new challenge that the future presents, namely the increase in complex cases and, consequently, in healthcare needs. 5 CONCLUSIONS In conclusion, this national survey showed for the first time discrepancies in the national distribution of the physiotherapy units involved in respiratory management. Moreover, we revealed that respiratory physiotherapists in Italy perform various specialized techniques not only related to airway clearance, depending on the needs of each center. Finally, our work allowed us to define the characteristics and the increasing expertise of the therapists included in respiratory teams and marks the first step in fostering cooperation among units and in the standardization on the approach to patient care. Abbreviations PCD: Primary ciliary dyskinesia NCFB: Non-Cystic fibrosis Bronchiectasis NIV: Non-Invasive Ventilation DLCO: Diffusing Capacity of the Lungs Test FOT: Forced oscillation technique IOS: Impulse oscillometry IPV: Intrapulmonary Percussive Ventilation EFA: Expiratory Flow Accelerator HFCWO: High-frequency chest wall oscillation Pts: patients Declarations Ethics approval and consent to participate Not applicable Consent for publication Not applicable Availability of data and materials The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request. Competing interests The authors declare that they have no competing interests. Funding Not applicable Authors' contributions BT, NU, PL, BF, EB, MDA conceived the study. BT, NU, AB wrote the manuscript. RC conceived and directed the work thanks to his knowledge of the subject matter. BT, EB, MDA, BG, SDD, SM, IP, CP collected data. BT, NU, PL analyzed and interpreted data. All authors read and approved the final manuscript. Acknowledgements This work was supported also by the Italian Ministry of Health with “Current Research funds”. The project was conducted in collaboration with the "Primary Ciliary Dyskinesia and Non-CF Bronchiectasis" SIMRI Study Group and the Physiotherapy Group of the Italian Cystic Fibrosis Society (SIFC). We extend our gratitude to the units that participated in the survey: Servizio FT esclusivo del Reparto di Fibrosi Cistica nel PO “G. Tatarella “ di Cerignola ASL FG; Centro Regionale Fibrosi Cistica Calabria - Lamezia Terme; A.O.U.Policlinico "G.Rodolico - San Marco" – Catania; Azienda universitaria policlinico Federico II – Napoli; AOU policlinico Bari – Ospedale pediatrico Giovanni XXIII; Azienda universitaria ospedaliera consorziale – Centro regionale di riferimento per la Fibrosi Cistica – Bari; Azienda Ospedaliera "G. Brotzu" – Cagliari; Policlinico universitario "G. Martino" – Messina; A.R.N.A.S. Ospedali Civico Di Cristina Benfratelli – Centro di riferimento regionale Fibrosi Cistica – Palermo; Azienda ospedaliera universitaria Careggi di Firenze; Ospedale Pediatrico Bambino Gesù – Roma; A.O.U. Sant'Andrea – Roma; Azienda Ospedaliera Universitaria Meyer IRCCS – Unità Professionale di Riabilitazione – Firenze; Azienda Ospedaliera Universitaria Pisana; A.O.U. delle Marche – Presidio Salesi – Ancona; Riabilitazione respiratoria presso il Centro Regionale Fibrosi Cistica Marche – Ancona; Azienda Usl toscana sud est – Grosseto; Policlinico Umberto I – Centro Regionale Fibrosi Cistica – Roma; Centro Regionale Fibrosi Cistica Umbria – USL 1 Gubbio; Asl Teramo – C.R.R. Fibrosi Cistica Atri; Usl Toscana nordovest – Servizio di supporto Fibrosi Cistica Livorno; Servizio di supporto regionale Fibrosi Cistica Nord Ovest Sardegna – Alghero; IRCCS Burlo Garofolo – Centro regionale di Fibrosi Cistica – Trieste; ASP IMMeS e PAT Pio Albergo Trivulzio – Milano; Centro Fibrosi Cistica – Azienda Ospedaliero Universitaria Integrata di Verona; Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico – Servizio Fisioterapia Pediatrica – Milano; APSS Trento – Ospedale di Rovereto; Azienda ULSS 2 Marca Trevigiana - U.O.S.D. Fibrosi Cistica – distretto di Treviso; Villa Pineta – Modena; Ospedale Bufalini – Centro di riferimento Fibrosi Cistica – Cesena; Azienda Ospedaliera Universitaria di Parma; Azienda Provinciale Servizi Sanitari Provincia Autonoma Trento – Servizio fisioterapia Ospedaliera Distretto Sud- annesso UO Pediatria-Ospedale di Rovereto; ASST Fatebenefratelli Sacco – P.O. BUZZI – Ambulatorio di Fisioterapia – Milano; UODS Fibrosi Cistica – Istituto Giannina Gaslini di Genova; Pediatria istituti ospedalieri Bergamaschi – U.O Riabilitazione; Ospedale dei bambini Asst Spedali Civili di Brescia – Centro regionale supporto Fibrosi Cistica; Azienda Ospedaliera San Luigi Gonzaga – Orbassano; A.S.O. Città della Salute e Scienza di Torino - P.O. O.I.R.M. Pneumologia Pediatrica; UOSD Pneumologia e allergologia pediatrica – Azienda Ospedaliera Universitaria – Padova; Authors’ information Beatrice Tani (corresponding author): [email protected] ; [email protected] Nicola Ullmann: [email protected] Eugenio Barbieri: [email protected] Alessandra Boni: [email protected] Matteo D’Angelo: [email protected] Sara De Dominicis: [email protected] Beniamino Giacomodonato: [email protected] Paola Leone: [email protected] Stefania Monduzzi: [email protected] Irene Piermarini: [email protected] Chiara Pizziconi: [email protected] Beatrice Ferrari: [email protected] Renato Cutrera: [email protected] References Wallmeier J, Nielsen KG, Kuehni CE, Lucas JS, Leigh MW, Zariwala MA, et al. Motile ciliopathies. Nat Rev Dis Prim. 2020 Dec 1;6(1). Horani A, Ferkol TW, Dutcher SK, Brody SL. Genetics and biology of primary ciliary dyskinesia. Vol. 18, Paediatric Respiratory Reviews. W.B. Saunders Ltd; 2016. p. 18–24. Mirra V, Werner C, Santamaria F. Primary ciliary dyskinesia: An update on clinical aspects, genetics, diagnosis, and future treatment strategies. Vol. 5, Frontiers in Pediatrics. Frontiers Media S.A.; 2017. Laennec RTH (René TH. A treatise on mediate auscultation, and on diseases of the lungs and heart by R.T.H. Laennec ... ; with the notes and additions of M. Mer. Laennec ... and of M. Andral ... ; translated from the latest edition by a member of the College of Physicians ; edi [Internet]. Available from: http://resource.nlm.nih.gov/9308216 [Bronchiectasis not related to cystic fibrosis in children: Guidelines for diagnosis, monitoring and treatment]. Arch Argent Pediatr. 2020 Dec;118(6):S164–82. Eralp EE, Gokdemir Y, Atag E, Ikizoglu NB, Ergenekon P, Yegit CY, et al. Changing clinical characteristics of non-cystic fibrosis bronchiectasis in children. BMC Pulm Med. 2020 Jun 16;20(1). Bahçeci S, Karaman S, Nacaroğlu HT, Yazıcı S, Girit S, Ünsal-Karkıner Ş, et al. Changing epidemiology of non-cystic fibrosis bronchiectasis. Ullmann N, Porcaro F, Petreschi F, Cammerata M, Allegorico A, Negro V, et al. Noncystic fibrosis bronchiectasis in children and adolescents: Follow-up over a decade. Pediatr Pulmonol. 2021 Sep 1;56(9):3026–34. Satırer O, Mete Yesil A, Emiralioglu N, Tugcu GD, Yalcın E, Dogru D, et al. A review of the etiology and clinical presentation of non-cystic fibrosis bronchiectasis: A tertiary care experience. Respir Med. 2018 Apr 1;137:35–9. Lee AL, Button BM, Tannenbaum EL. Airway-clearance techniques in children and adolescents with chronic suppurative lung disease and bronchiectasis. Vol. 5, Frontiers in Pediatrics. Frontiers Media S.A.; 2017. Schofield LM, Duff A, Brennan C. Airway Clearance Techniques for Primary Ciliary Dyskinesia; is the Cystic Fibrosis literature portable? Vol. 25, Paediatric Respiratory Reviews. W.B. Saunders Ltd; 2018. p. 73–7. Audag N, Dubus J-C, Combret Y. [Respiratory physiotherapy in pediatric practice]. Rev Mal Respir. 2022 Jun;39(6):547–60. Metersky M, Chalmers J. Bronchiectasis insanity: Doing the same thing over and over again and expecting different results? [version 1; peer review: 2 approved]. Vol. 8, F1000Research. F1000 Research Ltd; 2019. Ullmann N, Santamaria F, Allegorico A, Fainardi V, Borrelli M, Ferraro VA, et al. Primary ciliary dyskinesia: A multicenter survey on clinical practice and patient management in Italy. Pediatr Pulmonol. 2023 Apr 1; Phillips J, Lee A, Pope R, Hing W. Physiotherapists’ use of airway clearance techniques during an acute exacerbation of bronchiectasis: a survey study. Arch Physiother [Internet]. 2021 Feb 1 [cited 2023 Aug 1];11(1):3. Available from: http://www.ncbi.nlm.nih.gov/pubmed/33517917 Strippoli MPF, Frischer T, Barbato A, Snijders D, Maurer E, Lucas JSA, et al. Management of primary ciliary dyskinesia in European children: Recommendations and clinical practice. Eur Respir J. 2012 Jun 1;39(6):1482–91. O’Neill K, O’Donnell AE, Bradley JM. Airway clearance, mucoactive therapies and pulmonary rehabilitation in bronchiectasis. Vol. 24, Respirology. Blackwell Publishing; 2019. p. 227–37. Cooper BG, Troosters T, Burge G, Field D, Hrafnkelsdottir SS, Pitta F, et al. Allied respiratory professionals. Vol. 36, European Respiratory Journal. European Respiratory Society; 2010. p. 701–3. Table Table 1: Tests and procedures performed by physiotherapists % PROCEDURES PERFORMED 82 Six minute walking test (6MWT) 71 Spirometry 29 Sit to stand test 21 Bronchial provocation test and bronchial dilation test Measurement of maximal inspiratory and maximal expiratory (MIP/MEP) pressures Medical Research Council (MRC) scale Shuttle walking test (MSWT) 18 Multi-breath washout test Sub-Maximal Cycle Ergometer & step Test 14 Body plethysmography Fractional exhaled nitric oxide test (Feno) 11 Diffusing Capacity of the Lungs Test (DLCO) Impulse oscillometry (IOS) Nocturnal pulse oximetry Peak Expiratory /Inspiratory Flow (PEF/PIF), Peak expiratory cough flow (PCEF) MultiBreath Washout test Short Physical Performance Battery (SPPB scale) 7 Chest ultrasound 4 Challenge test Tidal Breathing Pulmonary Function of Children < 2 y.o. Forced oscillation technique (FOT) Indirect calorimetry Cardiopulmonary exercise testing (CPET) Bruce modified test One-repetition maximum test (1RM) Cite Share Download PDF Status: Published Journal Publication published 06 Mar, 2025 Read the published version in Italian Journal of Pediatrics → Version 1 posted Editorial decision: Major revision 18 May, 2024 Reviewers invited by journal 04 Apr, 2024 Reviewers agreed at journal 14 Mar, 2024 Editor assigned by journal 21 Feb, 2024 First submitted to journal 16 Feb, 2024 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-3950515","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":279594198,"identity":"faec4ee2-6979-4da4-a53a-49a7fe8c3c8a","order_by":0,"name":"Beatrice 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Gesu","correspondingAuthor":false,"prefix":"","firstName":"Nicola","middleName":"","lastName":"Ullmann","suffix":""},{"id":279594200,"identity":"4015e6b6-42a6-4a47-be22-1d3954a68163","order_by":2,"name":"Paola Leone","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Paola","middleName":"","lastName":"Leone","suffix":""},{"id":279594201,"identity":"82d44369-9e0a-4bda-9be8-825d674b7cf0","order_by":3,"name":"Alessandra Boni","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Alessandra","middleName":"","lastName":"Boni","suffix":""},{"id":279594202,"identity":"7253f70a-ae87-4b5b-83c9-62ca4e2228a7","order_by":4,"name":"Eugenio Barbieri","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Eugenio","middleName":"","lastName":"Barbieri","suffix":""},{"id":279594203,"identity":"021499b8-361f-4ec6-9840-4f2483ccf54a","order_by":5,"name":"Matteo D'Angelo","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Matteo","middleName":"","lastName":"D'Angelo","suffix":""},{"id":279594204,"identity":"6a122d54-a6c1-4449-8977-f0b76a6815e4","order_by":6,"name":"Sara De Dominicis","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Sara","middleName":"","lastName":"De Dominicis","suffix":""},{"id":279594205,"identity":"0e4ce58a-fd5a-4e44-8725-bcf891409c5d","order_by":7,"name":"Beniamino Giacomodonato","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Beniamino","middleName":"","lastName":"Giacomodonato","suffix":""},{"id":279594206,"identity":"bae0c784-599d-4bac-a536-d23e6b0c215d","order_by":8,"name":"Stefania Monduzzi","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Stefania","middleName":"","lastName":"Monduzzi","suffix":""},{"id":279594207,"identity":"939cdcf9-ea62-4802-9fac-276ff4b79d13","order_by":9,"name":"Irene Piermarini","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Irene","middleName":"","lastName":"Piermarini","suffix":""},{"id":279594208,"identity":"44bae285-1dab-47b8-be56-3ee1e6604ec9","order_by":10,"name":"Chiara Pizziconi","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Chiara","middleName":"","lastName":"Pizziconi","suffix":""},{"id":279594209,"identity":"5564f7ce-a1c7-4dcf-bb5a-fcb635d14b9d","order_by":11,"name":"Beatrice Ferrari","email":"","orcid":"","institution":"Children Hospital Meyer: Azienda Ospedaliero Universitaria Meyer","correspondingAuthor":false,"prefix":"","firstName":"Beatrice","middleName":"","lastName":"Ferrari","suffix":""},{"id":279594210,"identity":"a287d081-a0cd-42a1-b8a6-d28dd6e8fe74","order_by":12,"name":"Renato Cutrera","email":"","orcid":"","institution":"Ospedale Pediatrico Bambino Gesù: Ospedale Pediatrico Bambino Gesu","correspondingAuthor":false,"prefix":"","firstName":"Renato","middleName":"","lastName":"Cutrera","suffix":""}],"badges":[],"createdAt":"2024-02-12 08:12:24","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-3950515/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-3950515/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s13052-025-01904-0","type":"published","date":"2025-03-06T15:57:02+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":52919012,"identity":"d716c396-1ca9-4e1b-8687-1956647c26f0","added_by":"auto","created_at":"2024-03-18 17:12:30","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":45529,"visible":true,"origin":"","legend":"\u003cp\u003eFlowchart of “National Survey on Pediatric Respiratory Physiotherapy Units: Primary Ciliary Dyskinesia and Non-CF Bronchiectasis” sent to Italian Pediatric respiratory units treating PCD and NCFB pts\u003c/p\u003e","description":"","filename":"1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3950515/v1/ec1ce227cd96b4545802c0a3.jpg"},{"id":52919009,"identity":"6f45a311-03d8-4a4e-9f23-93f81ccec4a7","added_by":"auto","created_at":"2024-03-18 17:12:30","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":76501,"visible":true,"origin":"","legend":"\u003cp\u003eGeographical representation of centers with respiratory therapy unit or respiratory therapists treating PCD and NCFB pts\u003c/p\u003e\n\u003cp\u003eNorth: Valle d'Aosta, Piemonte, Liguria, Lombardia, Trentino-Alto Adige, Veneto, Friuli-Venezia Giulia, Emilia Romagna; Center: Toscana, Marche, Umbria, Lazio, Abruzzo, Molise, Sardegna; South: Campania, Puglia, Basilicata, Calabria, Sicilia.\u003c/p\u003e","description":"","filename":"2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3950515/v1/8b5f49a888dea07094d8789a.jpg"},{"id":52919010,"identity":"01575605-b4a4-4f7b-a677-0f85719c185b","added_by":"auto","created_at":"2024-03-18 17:12:30","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":96876,"visible":true,"origin":"","legend":"\u003cp\u003eServices offered\u003c/p\u003e\n\u003cp\u003eService offered from respiratory therapists in general admission patients - % centers\u003c/p\u003e","description":"","filename":"3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3950515/v1/26230476c05af0d3982a700e.jpg"},{"id":52919011,"identity":"d3b0e61d-9e6c-41d7-8ec7-1a9768663013","added_by":"auto","created_at":"2024-03-18 17:12:30","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":77626,"visible":true,"origin":"","legend":"\u003cp\u003ePerceived mastery of airway clearance techniques\u003c/p\u003e","description":"","filename":"4.jpg","url":"https://assets-eu.researchsquare.com/files/rs-3950515/v1/3f71262ae4687c79b382ecb2.jpg"},{"id":78191304,"identity":"793ef2e1-28a2-48f5-999b-083c4c1df733","added_by":"auto","created_at":"2025-03-10 19:55:48","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":1006819,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3950515/v1/89f42193-6329-4486-b31c-7be21138adbd.pdf"}],"financialInterests":"","formattedTitle":"National Survey on Pediatric Respiratory Physiotherapy Units: Primary Ciliary Dyskinesia and Non-CF Bronchiectasis","fulltext":[{"header":"1 BACKGROUND","content":"\u003cp\u003ePCD and NCFB are two rare conditions affecting the respiratory system. PCD is a genetic disorder of motile cilia, primarily transmitted as an autosomal recessive trait (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). Motile cilia are located on the apical surface of the upper and lower respiratory tract, on the ependymal cells lining the ventricles of the central nervous system, as well as on the oviducts of the female reproductive system and in the flagellum of male spermatozoa (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e). The clinical phenotype of PCD is broad but respiratory manifestations are part of the typical presentation of the disease and are central in the management and treatment of patients (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eOn the other hand, NCFB has been recognized as a clinical problem in children for approximately two centuries (\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e) and is characterized by progressive and often irreversible bronchial dilatation due to structural changes in the bronchial wall and chronic airway inflammation (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e). NCFB is a heterogeneous disorder and recognizes several underlying etiologies, including PCD, which has been suggested in various studies to be the primary cause of the onset of this disease (\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e) (\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e) (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eBoth conditions are characterized by daily productive chronic cough (\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e) and recurrent upper and lower respiratory tract infections (\u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e) (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e).\u003c/p\u003e \u003cp\u003eTo date, chest physiotherapy is one of the cornerstones of treatment and prevention of exacerbations and management of bronchial secretions (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e) (\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e). Treatment should be initiated in early childhood (\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e). Currently, therapeutic strategies for PCD and NCFB do not base on validated disease-specific recommendations, but often refer to the available evidence for Cystic Fibrosis (CF) (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e) (\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). Physiotherapy treatments should follow common protocols and the approaches offered should not differ between centers. To the best of our knowledge, no data have been published on the organization of physiotherapy units for pediatric patients with PCD and NCFB in Italy.\u003c/p\u003e \u003cp\u003eThe primary objective of this survey was to identify Italian pediatric centers with a respiratory therapy team and to determine their characteristics and the services offered, with the aim of obtaining a primary overview of the Italian context and set up of a professional network.\u003c/p\u003e"},{"header":"2 MATERIALS AND METHODS","content":"\u003cp\u003eA multidisciplinary team, consisting of respiratory therapists and pediatric respiratory physicians, designed a survey questionnaire to be distributed to Italian pediatric centers treating PCD and NCFB. Given that the Italian National Healthcare system does not currently centralize care for PCD and NCFB (\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e), we reached out the major number of CF centers, children's hospitals, and general hospitals for our study.\u003c/p\u003e \u003cp\u003eThe questionnaire was designed to assess the services provided in Italian healthcare providers in relation to respiratory therapy. In order to involve as many centres as possible, we contacted all the members of the SIMRI (Italian society of pediatric respiratory diseases) study group \"PCD and NCFB\" and the physiotherapy group of the SIFC (Cystic Fibrosis Italian Society).\u003c/p\u003e \u003cp\u003eCenters that did not treat pediatric patients with PCD or NCFB were excluded from the survey.\u003c/p\u003e \u003cp\u003eClinicians who declared not to have a physiotherapy service at their centers, were excluded from the survey and were subsequently contacted to inquire about the physiotherapy management of the patients with a shorter set of questions sent by email.\u003c/p\u003e \u003cp\u003eThe questionnaire was sent via an online survey platform to the physiotherapist head of department (or delegate) of each unit. One week before the survey was distributed, an email was sent to each participant explaining the purpose of the survey. The survey was sent out in May 2023, and responses were collected until August 2023. Centers were prompted through a weekly email reminder.\u003c/p\u003e \u003cdiv id=\"Sec3\" class=\"Section2\"\u003e \u003ch2\u003e2.1 Questionnaire\u003c/h2\u003e \u003cp\u003eWe investigated various aspects related to the organization of physiotherapy units. Specifically, the questionnaire was divided in three sections:\u003c/p\u003e \u003cp\u003e \u003col\u003e \u003cspan\u003e \u003cli\u003e \u003cp\u003eDistribution and characteristics of the centers\u003c/p\u003e \u003c/li\u003e \u003c/span\u003e \u003cspan\u003e \u003cli\u003e \u003cp\u003eServices provided by respiratory therapists\u003c/p\u003e \u003c/li\u003e \u003c/span\u003e \u003cspan\u003e \u003cli\u003e \u003cp\u003ePhysiotherapists\u0026rsquo; perception of the unit\u003c/p\u003e \u003c/li\u003e \u003c/span\u003e \u003c/ol\u003e \u003c/p\u003e \u003cdiv id=\"Sec4\" class=\"Section3\"\u003e \u003ch2\u003e2.1.1. Distribution and characteristics of the centers\u003c/h2\u003e \u003cp\u003eTo obtain a national overview of the centers, we first identified the location of each hospital and the corresponding Italian region.\u003c/p\u003e \u003cp\u003eNext, we asked in detail total number and age of the patients with NCFB and PCD followed in each unit.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec5\" class=\"Section3\"\u003e \u003ch2\u003e2.1.2. Services provided by respiratory therapists\u003c/h2\u003e \u003cp\u003eThe second section of the questionnaire investigated the physiotherapy unit. In particular, the team composition, the existence of a specific respiratory physiotherapy unit, the tasks of the physiotherapists and the settings of intervention. Finally, we inquired the instrumental and functional tests performed by the team.\u003c/p\u003e \u003c/div\u003e \u003cdiv id=\"Sec6\" class=\"Section3\"\u003e \u003ch2\u003e2.1.3 Physiotherapists\u0026rsquo; perception of the unit\u003c/h2\u003e \u003cp\u003ePhysiotherapists\u0026rsquo; perception was investigated in two different areas. First, team's skill in airway clearance was assessed. Five different airway clearance techniques were analyzed and a Likert scale (No skills \u0026ndash; Highly specialized) was used to determine the perceived mastery. Secondly, the availability of instruments and devices was assessed.\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv id=\"Sec7\" class=\"Section2\"\u003e \u003ch2\u003e2.2 Analysis\u003c/h2\u003e \u003cp\u003eThis descriptive study was based on data obtained from the participating centers' questionnaires. Once all available responses were collected, data were entered into a database for analysis. All data analysis was performed by the principal investigator and shared with other researchers. Data were collected in a database and statistical analysis and graphs processing were performed using Excel (16.0 Software). Response rate was calculated taking into account responders and missing in relation to the involved centers. Absolute frequency and percentage were calculated for categorical variables, and the mean value was calculated for continuous variables. For open-ended questions, responses were categorized and analyzed narratively. To measure liking of the procedure a 5-point Likert scale was used.\u003c/p\u003e \u003cp\u003eSince the survey included only general information and did not disclose any sensitive patient data, formal approval from the Ethical Committee was not sought.\u003c/p\u003e \u003c/div\u003e"},{"header":"3 RESULTS","content":"\u003cp\u003eAs shown in Fig. \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e, out of 40 centers contacted, 39 replied (97.5%). Ten centers were excluded from the survey: seven reported not to treat pediatric patients with PCD or NCFB and three centers reported not have physiotherapy unit/physiotherapists for respiratory care. To these centers was provided a shorter set of questions about the physiotherapy management for their patients, with responses provided by the contacted physician. For the three centers, patient care was delegated to other facilities outside the region of residence (n\u0026thinsp;=\u0026thinsp;1), to nurses (n\u0026thinsp;=\u0026thinsp;1) and to physicians only for techniques review (n\u0026thinsp;=\u0026thinsp;1).\u003c/p\u003e\n\u003cdiv id=\"Sec9\" class=\"Section2\"\u003e\n \u003ch2\u003e3.1 Distribution on the territory and centers\u0026rsquo; characteristics\u003c/h2\u003e\n \u003cp\u003eThe distribution of centers with a physiotherapy unit for respiratory care that completed the survey, and the relative number of PCD and NCFB patients managed is represented in Fig. \u003cspan class=\"InternalRef\"\u003e2\u003c/span\u003e. We managed to reach centers from the north to the south of Italy and showed a heterogeneous distribution of centers throughout the country. Four Italian regions were not represented (3 did not retrieved, 1 reported not having a center with the physiotherapy unit). Thirteen centers are located in the north Italian area, 8 in the center and 8 in the south. Among the included centers, 25/29 (86,2%) took in charge also CF patients.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec10\" class=\"Section2\"\u003e\n \u003ch2\u003e3.2 Services provided by respiratory therapists\u003c/h2\u003e\n \u003cp\u003eRegarding the organization of physiotherapy team, many differences were noted. Of the 29 centers with a physiotherapy unit, only 19 had a specialized respiratory therapy unit, whereas 10 offered a general physiotherapy service. Of these 10 units, 4 reported spending\u0026thinsp;\u0026lt;\u0026thinsp;50% of their daily time on respiratory care, whereas 6 reported spending\u0026thinsp;\u0026ge;\u0026thinsp;50% of their daily time on the respiratory care.\u003c/p\u003e\n \u003cp\u003eThe centers had an average of 4.1 professionals per unit in the north, 3.4 in the center, 1.8 in south.\u003c/p\u003e\n \u003cp\u003eRespiratory therapy intervention was administered in different care settings: regular ward (28/29 center, 97%), outpatients (29/29 center, 100%), intensive or semi-intensive care setting (17/29 center, 59%).\u003c/p\u003e\n \u003cp\u003eThe interventions performed by respiratory therapists are shown in Fig. \u003cspan class=\"InternalRef\"\u003e3\u003c/span\u003e. These interventions include more than just airway clearance, which is the common element for the centers (29/29, 100%). Physiotherapists performed more specific interventions such as pulmonary function tests (such as spirometry, DLCO, etc.) (23/29, 79,3%), functional tests (such as 6MWT, SPPB test, etc.) (27/29, 93,1%), educational training (26/29, 89,6%), management of workout programs (25/29, 86,2%) and intervention developed in collaboration with physicians, as NIV (23/29, 79,3%) and oxygen titration (21/29, 72,4%).\u003c/p\u003e\n \u003cp\u003eAccording to tests and procedures performed by physiotherapists, major geographical differences were found in the hospital ward activities. In addition to airway clearance techniques (100% north, center, south), units are usual to perform mostly pulmonary function tests (84.6% north, 87,5% center, 100% south) and functional tests (92,3% north, 100% center, 100% south) in outpatient regimen, conversely oxygen titration (69,2% north, 62,5% center, 12,5% south), NIV titration (92,3% north, 62,5% center, 12,5% south) and the set-up of workout programs (69,2% north, 87,5% center, 50% south) are performed mainly in hospital wards.\u003c/p\u003e\n \u003cp\u003eTable \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e summarizes the tests and procedures more frequently performed by therapists both outpatient setting and hospitalization care.\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003eTable \u003cspan class=\"InternalRef\"\u003e1\u0026nbsp;\u003c/span\u003e\u003c/strong\u003eTests and procedure performed by physiotherapists\u003c/p\u003e\n \u003cp\u003eMoreover, we also asked to describe additional services provided by therapists in each center. Therapists were also involved in different activities such as telemedicine, research projects, and in the support, alongside physicians, for prescription at home of medical devices. Additionally, the collection of biological material for microbiological examination is often entrusted to respiratory therapists.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec11\" class=\"Section2\"\u003e\n \u003ch2\u003e3.3 Perception of physiotherapists of the unit\u003c/h2\u003e\n \u003cp\u003eFigure \u003cspan class=\"InternalRef\"\u003e4\u003c/span\u003e represent the self-assessment Likert scale of team\u0026apos;s skill in five different airway clearance techniques. Secondly, concerning physiotherapists\u0026rsquo; perception of the units\u0026rsquo; devices availability, 17% of the therapists consider it inadequate.\u003c/p\u003e\n\u003c/div\u003e"},{"header":"4 DISCUSSION","content":"\u003cp\u003eThis study provides, for the first time, an overall view of the national distribution, organization, and structure of physiotherapy departments following pediatric patients affected by PCD and NCFB.\u003c/p\u003e \u003cp\u003ePrevious surveys on physiotherapy have mainly focused on techniques and clinical management of patients (\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e) (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e), and more specific data on the Italian physiotherapy units is lacking.\u003c/p\u003e \u003cp\u003eThe importance of services provided by hospital-based physiotherapy departments is increasingly recognized, particularly in the multidisciplinary treatment of respiratory diseases. This need is even greater for rare conditions as PCD and NCFB, which, as has been reported in the literature over the last decade (\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e) (\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e) require the establishment of daily airway clearance treatment and individualized exercise programs to reduce the decline of lung function over time. For this reason, it seemed appropriate to study the distribution of physiotherapy units on the Italian territory that follow pediatric PCD and NCFB patients, and then try to identify the organization and services offered by each center.\u003c/p\u003e \u003cp\u003eIn our survey we achieved an excellent response rate of 97.5%, confirming the positive attitude and increasing interest of Italian physiotherapists to be engaged in research projects. Unfortunately, we are missing data from three regions (Valle D\u0026rsquo;Aosta, Molise, Basilicata) in our survey. As hypothesized in our previous national research project (\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e), this can be related to the absence of specialized centers in those regions or to our difficulties in reaching all the territorial centers. Moreover, the three centers lacking a physiotherapy unit for respiratory care, declared to refer patients to facilities outside the region or to medical/nurse care. Our data reveals the challenge in finding professional physiotherapists trained in respiratory care. We all agree that a well-planned chest therapy program is essential, and the competencies that physiotherapists are acquiring over time are of paramount importance, as suggested by the literature (\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e) (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). These data, highlighting the potential absence of professional respiratory therapists in some Italian regions should be seriously taken into account.\u003c/p\u003e \u003cp\u003eAnalyzing the distribution and features of the centers, the primary distinction lies is in terms of territorial distribution, particularly between the northern (13 centers) versus center/southern Italian area (8 centers/8 centers). The heterogeneity of units is evident, and this diversity extends to the organization of logistic teams. Respiratory therapy predominantly takes place in outpatient and regular ward settings as observed in 100% and 96,5% of centers respectively. In contrast, intensive and semi-intensive care units play a partial role in the work of physiotherapists, with interventions being conducted in only 17 out of 29 centers. Furthermore, concerning team composition, among the 29 centers, 19 (65%) have a dedicated respiratory team. Nevertheless, therapists still spend\u0026thinsp;\u0026ge;\u0026thinsp;50% of their time on respiratory care in 6 out of 10 units. This leads us to hypothesize that the need for respiratory physiotherapy is definitively growing due to a progressively greater number of diagnosed rare respiratory diseases or an increasing importance given to respiratory physiotherapy treatments.\u003c/p\u003e \u003cp\u003eIn our survey we retrieved a decreasing average number of respiratory therapists per center, going from the north to the south region of our country. We could hypotize that the northern centers have a greater availability of respiratory therapists. However, we cannot show if this data is related to a major workload of patients treated from the unit.\u003c/p\u003e \u003cp\u003eConsidering the interventions made by respiratory therapists, our survey showed that professionals not only play an irreplaceable role in developing an airway clearance program. In fact, assessment of patient condition through tests and pulmonary function tests, educational training for caregivers, titration of oxygen definition of NIV requirements, and workout programs are usual care activities in which Italian respiratory therapists are involved. This highlights the crucial role of respiratory therapists on multidisciplinary teams dealing with PCD and NCFB (\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e). Moreover, we appreciate how physiotherapists are expanding their expertise in respiratory care, and the growing competence of these professionals is aligning with the increasing knowledge and consideration of these rare diseases.\u003c/p\u003e \u003cp\u003eAdditionally, our survey revealed heterogeneity between healthcare providers in the interventions provided by physiotherapists, possibly due to the unique needs and specificities of each center. In the majority of centers, therapists perform walking tests and spirometry. However, it is important to note that in almost 20% of centers, tests such as bronchial provocation and dilation tests and Multi-Breath Washout tests, are also conducted by physiotherapy units. Definitively fewer centers perform more specialized procedures such as DLCO, FOT and IOS. These results show that according to the specific needs of centers, physiotherapists are professional figures that can cover various respiratory tests and functional evaluations.\u003c/p\u003e \u003cp\u003eRelated to the professional perception of the offered service, our study emphasized that although airway clearance is a core element of the treatment of PCD and non NCFB, there is still a varying perception of the confidence of the different airway clearance techniques. In many cases, therapists do not feel fully confident when using more complex techniques, such as airway clearance using instrumentation (as IPV, cough machine, EFA and HFCWO) and with NIV. Moreover, the adequacy of devices available was considered inadequate in 17% of units. This result strongly highlights the importance of addressing the training needs of professionals involved in the respiratory field and further implementing the devices needed in all centers. This could ensure professionals and patients to have the same clinical possibilities for high quality treatment.\u003c/p\u003e \u003cp\u003eFinally, our results raise new questions about the evolution of respiratory therapists\u0026rsquo; skills. These include telemedicine, chest ultrasounds and collaboration with physicians in the titration and training process for NIV and oxygen therapy. This evolution could be explained by the need for a more specialized approach to patient care. Forming a network of professionals throughout the national territory is crucial to address the new challenge that the future presents, namely the increase in complex cases and, consequently, in healthcare needs.\u003c/p\u003e"},{"header":"5 CONCLUSIONS","content":"\u003cp\u003eIn conclusion, this national survey showed for the first time discrepancies in the national distribution of the physiotherapy units involved in respiratory management. Moreover, we revealed that respiratory physiotherapists in Italy perform various specialized techniques not only related to airway clearance, depending on the needs of each center. Finally, our work allowed us to define the characteristics and the increasing expertise of the therapists included in respiratory teams and marks the first step in fostering cooperation among units and in the standardization on the approach to patient care.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003ePCD: Primary ciliary dyskinesia\u003c/p\u003e\n\u003cp\u003eNCFB: Non-Cystic fibrosis Bronchiectasis\u003c/p\u003e\n\u003cp\u003eNIV: Non-Invasive Ventilation\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eDLCO: Diffusing Capacity of the Lungs Test\u003c/p\u003e\n\u003cp\u003eFOT: Forced oscillation technique\u003c/p\u003e\n\u003cp\u003eIOS: Impulse oscillometry\u003c/p\u003e\n\u003cp\u003eIPV: Intrapulmonary Percussive Ventilation\u003c/p\u003e\n\u003cp\u003eEFA: Expiratory Flow Accelerator\u003c/p\u003e\n\u003cp\u003eHFCWO: High-frequency chest wall oscillation\u003c/p\u003e\n\u003cp\u003ePts: patients\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003e\u003cem\u003eEthics approval and consent to participate\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eConsent for publication\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAvailability of data and materials\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eCompeting interests\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eFunding\u003c/em\u003e\u003c/strong\u003e\u003cstrong\u003e\u003cem\u003e\u0026nbsp;\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAuthors\u0026apos; contributions\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eBT, NU, PL, BF, EB, MDA conceived the study. BT, NU, AB wrote the manuscript. RC conceived and directed the work thanks to his knowledge of the subject matter. BT, EB, MDA, BG, SDD, SM, IP, CP collected data. BT, NU, PL analyzed and interpreted data.\u0026nbsp;All authors read and approved the final manuscript. \u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAcknowledgements\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis work was supported also by the Italian Ministry of Health with \u0026ldquo;Current Research funds\u0026rdquo;.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe project was conducted in collaboration with the \u0026quot;Primary Ciliary Dyskinesia and Non-CF Bronchiectasis\u0026quot; SIMRI Study Group and the Physiotherapy Group of the Italian Cystic Fibrosis Society (SIFC).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eWe extend our gratitude to the units that participated in the survey:\u0026nbsp;\u003cem\u003eServizio FT esclusivo del Reparto di Fibrosi Cistica nel PO \u0026ldquo;G. Tatarella \u0026ldquo; di Cerignola ASL FG; Centro Regionale Fibrosi Cistica Calabria - Lamezia Terme; A.O.U.Policlinico \u0026quot;G.Rodolico - San Marco\u0026quot; \u0026ndash; Catania; Azienda universitaria policlinico Federico II \u0026ndash; Napoli; AOU policlinico Bari \u0026ndash; Ospedale pediatrico Giovanni XXIII; Azienda universitaria ospedaliera consorziale \u0026ndash; Centro regionale di riferimento per la Fibrosi Cistica \u0026ndash; Bari; Azienda Ospedaliera \u0026quot;G. Brotzu\u0026quot; \u0026ndash; Cagliari; Policlinico universitario \u0026quot;G. Martino\u0026quot; \u0026ndash; Messina; A.R.N.A.S. Ospedali Civico Di Cristina Benfratelli \u0026ndash; Centro di riferimento regionale Fibrosi Cistica \u0026ndash; Palermo; Azienda ospedaliera universitaria Careggi di Firenze; Ospedale Pediatrico Bambino Ges\u0026ugrave; \u0026ndash; Roma; A.O.U. Sant\u0026apos;Andrea \u0026ndash; Roma; Azienda Ospedaliera Universitaria Meyer IRCCS \u0026ndash; Unit\u0026agrave; Professionale di Riabilitazione \u0026ndash; Firenze; Azienda Ospedaliera Universitaria Pisana; A.O.U. delle Marche \u0026ndash; Presidio Salesi \u0026ndash; Ancona; Riabilitazione respiratoria presso il Centro Regionale Fibrosi Cistica Marche \u0026ndash; Ancona; Azienda Usl toscana sud est \u0026ndash; \u0026nbsp; Grosseto; Policlinico Umberto I \u0026ndash; Centro Regionale Fibrosi Cistica \u0026ndash; Roma; Centro Regionale Fibrosi Cistica Umbria \u0026ndash; USL 1 Gubbio; Asl Teramo \u0026ndash; C.R.R. Fibrosi Cistica Atri; Usl Toscana nordovest \u0026ndash; Servizio di supporto Fibrosi Cistica Livorno; Servizio di supporto regionale Fibrosi Cistica Nord Ovest Sardegna \u0026ndash; \u0026nbsp;Alghero; IRCCS Burlo Garofolo \u0026ndash; Centro regionale di Fibrosi Cistica \u0026ndash; Trieste; ASP IMMeS e PAT Pio Albergo Trivulzio \u0026ndash; Milano; Centro Fibrosi Cistica \u0026ndash; Azienda Ospedaliero Universitaria Integrata di Verona; Fondazione IRCCS C\u0026agrave; Granda Ospedale Maggiore Policlinico \u0026ndash; Servizio Fisioterapia Pediatrica \u0026ndash; Milano; APSS Trento \u0026ndash; Ospedale di Rovereto; Azienda ULSS 2 Marca Trevigiana - U.O.S.D. Fibrosi Cistica \u0026ndash; distretto di Treviso; Villa Pineta \u0026ndash; Modena; Ospedale Bufalini \u0026ndash; Centro di riferimento Fibrosi Cistica \u0026ndash; Cesena; Azienda Ospedaliera Universitaria di Parma; Azienda Provinciale Servizi Sanitari Provincia Autonoma Trento \u0026ndash; Servizio fisioterapia Ospedaliera Distretto Sud- annesso UO Pediatria-Ospedale di Rovereto; ASST Fatebenefratelli Sacco \u0026ndash; P.O. BUZZI \u0026ndash; Ambulatorio di Fisioterapia \u0026ndash; Milano; UODS Fibrosi Cistica \u0026ndash; Istituto Giannina Gaslini di Genova; Pediatria istituti ospedalieri Bergamaschi \u0026ndash; U.O Riabilitazione; Ospedale dei bambini Asst Spedali Civili di Brescia \u0026ndash; Centro regionale supporto Fibrosi Cistica; Azienda Ospedaliera San Luigi Gonzaga \u0026ndash; Orbassano; A.S.O. Citt\u0026agrave; della Salute e Scienza di Torino - P.O. O.I.R.M. Pneumologia Pediatrica; UOSD Pneumologia e allergologia pediatrica \u0026ndash; Azienda Ospedaliera Universitaria \u0026ndash; Padova;\u0026nbsp;\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003e\u003cem\u003eAuthors\u0026rsquo; information\u003c/em\u003e\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eBeatrice Tani (corresponding author): [email protected]; \u003ca href=\"mailto:[email protected]\"\[email protected]\u003c/a\u003e\u003c/p\u003e\n\u003cp\u003eNicola Ullmann: [email protected] \u0026nbsp;\u003c/p\u003e\n\u003cp\u003eEugenio Barbieri: [email protected]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAlessandra Boni: [email protected] \u0026nbsp;\u003c/p\u003e\n\u003cp\u003eMatteo D\u0026rsquo;Angelo: [email protected]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eSara De Dominicis: [email protected]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eBeniamino Giacomodonato: [email protected]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003ePaola Leone: [email protected]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eStefania Monduzzi: [email protected]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eIrene Piermarini: [email protected]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eChiara Pizziconi: [email protected]\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eBeatrice Ferrari: [email protected] \u0026nbsp;\u003c/p\u003e\n\u003cp\u003eRenato Cutrera: [email protected] \u0026nbsp;\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eWallmeier J, Nielsen KG, Kuehni CE, Lucas JS, Leigh MW, Zariwala MA, et al. Motile ciliopathies. Nat Rev Dis Prim. 2020 Dec 1;6(1). \u003c/li\u003e\n\u003cli\u003eHorani A, Ferkol TW, Dutcher SK, Brody SL. Genetics and biology of primary ciliary dyskinesia. Vol. 18, Paediatric Respiratory Reviews. W.B. Saunders Ltd; 2016. p. 18\u0026ndash;24. \u003c/li\u003e\n\u003cli\u003eMirra V, Werner C, Santamaria F. Primary ciliary dyskinesia: An update on clinical aspects, genetics, diagnosis, and future treatment strategies. Vol. 5, Frontiers in Pediatrics. Frontiers Media S.A.; 2017. \u003c/li\u003e\n\u003cli\u003eLaennec RTH (René TH. A treatise on mediate auscultation, and on diseases of the lungs and heart by R.T.H. Laennec ... ; with the notes and additions of M. Mer. Laennec ... and of M. Andral ... ; translated from the latest edition by a member of the College of Physicians ; edi [Internet]. Available from: http://resource.nlm.nih.gov/9308216\u003c/li\u003e\n\u003cli\u003e[Bronchiectasis not related to cystic fibrosis in children: Guidelines for diagnosis, monitoring and treatment]. Arch Argent Pediatr. 2020 Dec;118(6):S164\u0026ndash;82. \u003c/li\u003e\n\u003cli\u003eEralp EE, Gokdemir Y, Atag E, Ikizoglu NB, Ergenekon P, Yegit CY, et al. Changing clinical characteristics of non-cystic fibrosis bronchiectasis in children. BMC Pulm Med. 2020 Jun 16;20(1). \u003c/li\u003e\n\u003cli\u003eBah\u0026ccedil;eci S, Karaman S, Nacaroğlu HT, Yazıcı S, Girit S, \u0026Uuml;nsal-Karkıner Ş, et al. Changing epidemiology of non-cystic fibrosis bronchiectasis. \u003c/li\u003e\n\u003cli\u003eUllmann N, Porcaro F, Petreschi F, Cammerata M, Allegorico A, Negro V, et al. Noncystic fibrosis bronchiectasis in children and adolescents: Follow-up over a decade. Pediatr Pulmonol. 2021 Sep 1;56(9):3026\u0026ndash;34. \u003c/li\u003e\n\u003cli\u003eSatırer O, Mete Yesil A, Emiralioglu N, Tugcu GD, Yalcın E, Dogru D, et al. A review of the etiology and clinical presentation of non-cystic fibrosis bronchiectasis: A tertiary care experience. Respir Med. 2018 Apr 1;137:35\u0026ndash;9. \u003c/li\u003e\n\u003cli\u003eLee AL, Button BM, Tannenbaum EL. Airway-clearance techniques in children and adolescents with chronic suppurative lung disease and bronchiectasis. Vol. 5, Frontiers in Pediatrics. Frontiers Media S.A.; 2017. \u003c/li\u003e\n\u003cli\u003eSchofield LM, Duff A, Brennan C. Airway Clearance Techniques for Primary Ciliary Dyskinesia; is the Cystic Fibrosis literature portable? Vol. 25, Paediatric Respiratory Reviews. W.B. Saunders Ltd; 2018. p. 73\u0026ndash;7. \u003c/li\u003e\n\u003cli\u003eAudag N, Dubus J-C, Combret Y. [Respiratory physiotherapy in pediatric practice]. Rev Mal Respir. 2022 Jun;39(6):547\u0026ndash;60. \u003c/li\u003e\n\u003cli\u003eMetersky M, Chalmers J. Bronchiectasis insanity: Doing the same thing over and over again and expecting different results? [version 1; peer review: 2 approved]. Vol. 8, F1000Research. F1000 Research Ltd; 2019. \u003c/li\u003e\n\u003cli\u003eUllmann N, Santamaria F, Allegorico A, Fainardi V, Borrelli M, Ferraro VA, et al. Primary ciliary dyskinesia: A multicenter survey on clinical practice and patient management in Italy. Pediatr Pulmonol. 2023 Apr 1; \u003c/li\u003e\n\u003cli\u003ePhillips J, Lee A, Pope R, Hing W. Physiotherapists\u0026rsquo; use of airway clearance techniques during an acute exacerbation of bronchiectasis: a survey study. Arch Physiother [Internet]. 2021 Feb 1 [cited 2023 Aug 1];11(1):3. Available from: http://www.ncbi.nlm.nih.gov/pubmed/33517917\u003c/li\u003e\n\u003cli\u003eStrippoli MPF, Frischer T, Barbato A, Snijders D, Maurer E, Lucas JSA, et al. Management of primary ciliary dyskinesia in European children: Recommendations and clinical practice. Eur Respir J. 2012 Jun 1;39(6):1482\u0026ndash;91. \u003c/li\u003e\n\u003cli\u003eO\u0026rsquo;Neill K, O\u0026rsquo;Donnell AE, Bradley JM. Airway clearance, mucoactive therapies and pulmonary rehabilitation in bronchiectasis. Vol. 24, Respirology. Blackwell Publishing; 2019. p. 227\u0026ndash;37. \u003c/li\u003e\n\u003cli\u003eCooper BG, Troosters T, Burge G, Field D, Hrafnkelsdottir SS, Pitta F, et al. Allied respiratory professionals. Vol. 36, European Respiratory Journal. European Respiratory Society; 2010. p. 701\u0026ndash;3. \u003c/li\u003e\n\u003c/ol\u003e"},{"header":"Table","content":"\u003cp\u003e\u0026nbsp;Table\u0026nbsp;1: Tests and procedures performed by physiotherapists\u003c/p\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"538\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u003cstrong\u003e%\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u003cstrong\u003ePROCEDURES PERFORMED\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e82\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eSix minute walking test (6MWT)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e71\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eSpirometry\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e29\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eSit to stand test\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e21\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eBronchial provocation test and bronchial dilation test\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eMeasurement of maximal inspiratory and maximal expiratory (MIP/MEP) pressures\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eMedical Research Council (MRC) scale\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eShuttle walking test (MSWT)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e18\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eMulti-breath washout test\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eSub-Maximal Cycle Ergometer \u0026amp; step Test\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e14\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eBody plethysmography\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eFractional exhaled nitric oxide test (Feno)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e11\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eDiffusing Capacity of the Lungs Test (DLCO)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eImpulse oscillometry (IOS)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eNocturnal pulse oximetry\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003ePeak Expiratory /Inspiratory Flow (PEF/PIF), Peak expiratory cough flow (PCEF)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eMultiBreath Washout test\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eShort Physical Performance Battery (SPPB scale)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e7\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eChest ultrasound\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e4\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eChallenge test\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eTidal Breathing Pulmonary Function of Children \u0026lt; 2 y.o.\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eForced oscillation technique (FOT)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eIndirect calorimetry\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eCardiopulmonary exercise testing (CPET)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eBruce modified test\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd width=\"5.947955390334572%\" valign=\"bottom\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd width=\"94.05204460966543%\" valign=\"bottom\"\u003e\n \u003cp\u003eOne-repetition maximum test (1RM)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":true,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"italian-journal-of-pediatrics","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"itjp","sideBox":"Learn more about [Italian Journal of Pediatrics](http://ijponline.biomedcentral.com)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/ITJP/default.aspx","title":"Italian Journal of Pediatrics","twitterHandle":"@BioMedCentral","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"Primary Ciliary Dyskinesia, non-Cystic Fibrosis bronchiectasis, pediatrics, physiotherapy unit, respiratory physiotherapy, survey, physiotherapy procedures, airway clearance","lastPublishedDoi":"10.21203/rs.3.rs-3950515/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3950515/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground:\u003c/strong\u003e Currently, there is a lack of data concerning the organization and characteristics of Italian pediatric physiotherapy units for the treatment of patients with chronic lung diseases, especially those with rare conditions such as primary ciliary dyskinesia (PCD) and non-Cystic Fibrosis bronchiectasis (NCFB).\u003c/p\u003e\n\u003cp\u003eMethods: A national descriptive study based on a survey questionnaire was conducted. The questionnaire consisted of three different sections: distribution and characteristics of the centres, services provided by respiratory therapists, physiotherapists’ perception of the unit.\u003c/p\u003e\n\u003cp\u003eThe survey was distributed to all healthcare providers via an online platform, and a descriptive data analysis was performed.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eResults:\u003c/strong\u003e The survey had a response rate of 97.5% with twenty-nine responses collected. The centers are heterogeneously distributed: thirteen in the northern regions, eight in the central regions and eight in the southern regions. Of the 29 centers with a physiotherapy unit, 19 had a specialized respiratory therapy unit. Respiratory therapy was provided in different care settings: regular wards (28/29 centers, 97%), outpatient service (29/29 centers, 100%), and intensive or semi-intensive care units (17/29 centers, 59%).\u003c/p\u003e\n\u003cp\u003eThe interventions provided by respiratory therapists involved more than just airway clearance (29/29). More specific interventions, such as pulmonary function tests (23/29), functional tests (27/29), educational training (26/29), management of workout exercise programs (25/29) and interventions developed in collaboration with physicians such as non-invasive ventilation (NIV) (23/29) and oxygen titration (21/29) are performed. It is interesting to note that therapists are also involved in various activities, such as telemedicine, physiotherapists’ research projects, and supporting alongside physicians, for the prescription at home of medical devices. Perception of the unit was also evaluated.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusions:\u003c/strong\u003e The involved centers are heterogeneous in terms of the distribution and treatments offered. The role of respiratory physiotherapists still seems to be fragmented. This first descriptive analysis of the physiotherapy units and the main differences between centers opens query on the clinical approaches used for pediatric patients with PCD in terms of respiratory physiotherapy. However, in response to evolving treatment needs, a more specialized and standardized approach to patient care is required.\u003c/p\u003e","manuscriptTitle":"National Survey on Pediatric Respiratory Physiotherapy Units: Primary Ciliary Dyskinesia and Non-CF Bronchiectasis","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-03-18 17:12:25","doi":"10.21203/rs.3.rs-3950515/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Major revision","date":"2024-05-19T02:23:11+00:00","index":"","fulltext":""},{"type":"reviewersInvited","content":"","date":"2024-04-04T09:22:45+00:00","index":"","fulltext":""},{"type":"reviewerAgreed","content":"","date":"2024-03-14T12:16:28+00:00","index":0,"fulltext":""},{"type":"editorAssigned","content":"","date":"2024-02-22T00:39:22+00:00","index":"","fulltext":""},{"type":"submitted","content":"Italian Journal of Pediatrics","date":"2024-02-16T06:40:13+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"italian-journal-of-pediatrics","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"itjp","sideBox":"Learn more about [Italian Journal of Pediatrics](http://ijponline.biomedcentral.com)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/ITJP/default.aspx","title":"Italian Journal of Pediatrics","twitterHandle":"@BioMedCentral","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"em","reportingPortfolio":"BMC/SO AJ","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"9101f162-04d6-4bf3-9860-9e15be618ec6","owner":[],"postedDate":"March 18th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-03-10T19:55:43+00:00","versionOfRecord":{"articleIdentity":"rs-3950515","link":"https://doi.org/10.1186/s13052-025-01904-0","journal":{"identity":"italian-journal-of-pediatrics","isVorOnly":false,"title":"Italian Journal of Pediatrics"},"publishedOn":"2025-03-06 15:57:02","publishedOnDateReadable":"March 6th, 2025"},"versionCreatedAt":"2024-03-18 17:12:25","video":"","vorDoi":"10.1186/s13052-025-01904-0","vorDoiUrl":"https://doi.org/10.1186/s13052-025-01904-0","workflowStages":[]},"version":"v1","identity":"rs-3950515","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3950515","identity":"rs-3950515","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}