Skip to main navigation menu Skip to main content Skip to site footer

Original article

Vol. 152 No. 3334 (2022)

Physical activity, respiratory physiotherapy practices, and nutrition among people with primary ciliary dyskinesia in Switzerland – a cross-sectional survey

  • Yin Ting Lam
  • Eva S. L. Pedersen
  • Leonie D. Schreck
  • Leonie Hüsler
  • Helena Koppe
  • Fabiën N. Belle
  • Christian Clarenbach
  • Philipp Latzin
  • Claudia E. Kuehni
  • Myrofora Goutaki
Cite this as:
Swiss Med Wkly. 2022;152:w30221


AIMS OF THE STUDY: We know little about the level of physical activity, respiratory physiotherapy practices and nutritional status of people with primary ciliary dyskinesia (PCD), although these are important aspects of patients with chronic respiratory disease. We assessed physical activity, respiratory physiotherapy practices and nutritional status among people with primary ciliary dyskinesia in Switzerland, investigated how these vary by age and identified factors associated with regular physical activity.

METHODS: We sent a postal questionnaire survey to people with primary ciliary dyskinesia enrolled in the Swiss PCD registry (CH-PCD), based on the standardised FOLLOW-PCD patient questionnaire. We collected information about physical activity, physiotherapy, respiratory symptoms and nutritional status. We calculated the metabolic equivalent (MET) to better reflect the intensity of the reported physical activities. To assess nutritional status, we extracted information from CH-PCD and calculated participants’ body mass index (BMI).

RESULTS: Of the 86 questionnaires we sent, 74 (86% response rate) were returned from 24 children and 50 adults. The median age at survey completion was 23 years (IQR [interquartile range] 15–51), and 51% were female. Among all 74 participants, 48 (65%) performed sports regularly. Children were vigorously active (median MET 9.1; IQR 7.9–9.6) and adults were moderately active (median MET 5.5; IQR 4.3—6.9). Fifty-nine participants (80%) reported performing some type of respiratory physiotherapy. However, only 30% of adults saw a professional physiotherapist, compared with 75% of children. Half of the participants had normal BMI; one child (4%) and two adults (4%) were underweight. People who were regularly physically active reported seeing a physiotherapist more often.

CONCLUSIONS: Our study is the first to provide patient-reported data about physical activity, respiratory physiotherapy and nutrition among people with primary ciliary dyskinesia. Our results highlight that professional respiratory physiotherapy, exercise recommendations and nutritional advice are often not implemented in the care of people with primary ciliary dyskinesia in Switzerland. Multidisciplinary care in specialised centres by teams including physiotherapists and nutrition consultants could improve the quality of life of people with primary ciliary dyskinesia.


  1. Fernandes AC, Bezerra OM. Nutrition therapy for chronic obstructive pulmonary disease and related nutritional complications. J Bras Pneumol. 2006 Sep-Oct;32(5):461–71.
  2. Cordova-Rivera L, Gibson PG, Gardiner PA, McDonald VM. Physical activity associates with disease characteristics of severe asthma, bronchiectasis and COPD. Respirology. 2019 Apr;24(4):352–60.
  3. Urquhart DS. Exercise testing in cystic fibrosis: why (and how)? J R Soc Med. 2011 Jul;104(1_suppl Suppl 1):S6–14.
  4. Vogiatzis I, Zakynthinos G, Andrianopoulos V. Mechanisms of physical activity limitation in chronic lung diseases. Pulm Med. 2012;2012:634761.
  5. Barbato A, Frischer T, Kuehni CE, Snijders D, Azevedo I, Baktai G, et al. Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children. Eur Respir J. 2009 Dec;34(6):1264–76.
  6. Goutaki M, Meier AB, Halbeisen FS, Lucas JS, Dell SD, Maurer E, et al. Clinical manifestations in primary ciliary dyskinesia: systematic review and meta-analysis. Eur Respir J. 2016 Oct;48(4):1081–95.
  7. Valerio G, Giallauria F, Montella S, Vaino N, Vigorito C, Mirra V, et al. Cardiopulmonary assessment in primary ciliary dyskinesia. Eur J Clin Invest. 2012 Jun;42(6):617–22.
  8. Schofield LM, Duff A, Brennan C. Airway Clearance Techniques for Primary Ciliary Dyskinesia; is the Cystic Fibrosis literature portable? Paediatr Respir Rev. 2018 Jan;25:73–7.
  9. Kuehni CE, Goutaki M, Rubbo B, Lucas JS. Management of primary ciliary dyskinesia: current practice and future perspectives In: Chalmers JD, Polverino E, Aliberti S, eds. Bronchiectasis (ERS Monograph). 2018.
  10. Kriemler S, Radtke T, Christen G, Kerstan-Huber M, Hebestreit H. Short-Term Effect of Different Physical Exercises and Physiotherapy Combinations on Sputum Expectoration, Oxygen Saturation, and Lung Function in Young Patients with Cystic Fibrosis. Lung. 2016 Aug;194(4):659–64.
  11. Nigro E, Polito R, Elce A, Signoriello G, Iacotucci P, Carnovale V, et al. Physical Activity Regulates TNFα and IL-6 Expression to Counteract Inflammation in Cystic Fibrosis Patients. Int J Environ Res Public Health. 2021;18(9):18.
  12. Simsek S, Inal-Ince D, Cakmak A, Emiralioglu N, Calik-Kutukcu E, Saglam M, et al. Reduced anaerobic and aerobic performance in children with primary ciliary dyskinesia. Eur J Pediatr. 2018 May;177(5):765–73.
  13. Madsen A, Green K, Buchvald F, Hanel B, Nielsen KG. Aerobic fitness in children and young adults with primary ciliary dyskinesia. PLoS One. 2013 Aug;8(8):e71409.
  14. Ring AM, Buchvald FF, Holgersen MG, Green K, Nielsen KG. Fitness and lung function in children with primary ciliary dyskinesia and cystic fibrosis. Respir Med. 2018 Jun;139:79–85.
  15. Firat M, Bosnak-Guclu M, Sismanlar-Eyuboglu T, Tana-Aslan A. Respiratory muscle strength, exercise capacity and physical activity in patients with primary ciliary dyskinesia: A cross-sectional study. Respir Med. 2022 Jan;191:106719.
  16. Goutaki M, Maurer E, Halbeisen FS, Amirav I, Barbato A, Behan L, et al.; PCD Italian Consortium; Swiss PCD Group; French Reference Centre for Rare Lung Diseases; Genetic Disorders of Mucociliary Clearance Consortium. The international primary ciliary dyskinesia cohort (iPCD Cohort): methods and first results. Eur Respir J. 2017 Jan;49(1):49.
  17. Marino LV, Harris A, Johnstone C, Friend A, Newell C, Miles EA, et al. Characterising the nutritional status of children with primary ciliary dyskinesia. Clin Nutr. 2019 Oct;38(5):2127–35.
  18. Goutaki M, Halbeisen FS, Spycher BD, Maurer E, Belle F, Amirav I, et al.; PCD Israeli Consortium; Swiss PCD Group; French Reference Centre for Rare Lung Diseases. Growth and nutritional status, and their association with lung function: a study from the international Primary Ciliary Dyskinesia Cohort. Eur Respir J. 2017 Dec;50(6):50.
  19. Goutaki M, Eich MO, Halbeisen FS, Barben J, Casaulta C, Clarenbach C, et al.; Swiss PCD Registry (CH-PCD) Working Group. The Swiss Primary Ciliary Dyskinesia registry: objectives, methods and first results. Swiss Med Wkly. 2019 Jan;149:149.
  20. Lucas JS, Barbato A, Collins SA, Goutaki M, Behan L, Caudri D, et al. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J. 2017 Jan;49(1):49.
  21. Müller L, Savas ST, Tschanz SA, Stokes A, Escher A, Nussbaumer M, et al. On Behalf Of The Swiss Pcd Research G. A Comprehensive Approach for the Diagnosis of Primary Ciliary Dyskinesia-Experiences from the First 100 Patients of the PCD-UNIBE Diagnostic Center. Diagnostics (Basel). 2021;•••:11.
  22. von Elm E, Altman DG, Egger M, Pocock SJ, Gøtzsche PC, Vandenbroucke JP ; STROBE Initiative. The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement: guidelines for reporting observational studies. J Clin Epidemiol. 2008 Apr;61(4):344–9.
  23. Goutaki M, Hüsler L, Lam YT, Collaud E, Koppe H, Pedersen E, et al. Respiratory symptoms of Swiss people with Primary Ciliary Dyskinesia. Eur Respir J. 2021;58:OA2956.
  24. Goutaki M, Papon JF, Boon M, Casaulta C, Eber E, Escudier E, et al. Standardised clinical data from patients with primary ciliary dyskinesia: FOLLOW-PCD. ERJ Open Res. 2020 Feb;6(1):6.
  25. Physical Activity Guidelines for Americans. 2nd edition. Department of Health and Human Services, 2018. (Accessed 29.03.2022, at
  26. Garber CE, Blissmer B, Deschenes MR, Franklin BA, Lamonte MJ, Lee IM, et al.; American College of Sports Medicine. American College of Sports Medicine position stand. Quantity and quality of exercise for developing and maintaining cardiorespiratory, musculoskeletal, and neuromotor fitness in apparently healthy adults: guidance for prescribing exercise. Med Sci Sports Exerc. 2011 Jul;43(7):1334–59.
  27. Haskell WL, Lee IM, Pate RR, Powell KE, Blair SN, Franklin BA, et al. Physical activity and public health: updated recommendation for adults from the American College of Sports Medicine and the American Heart Association. Med Sci Sports Exerc. 2007 Aug;39(8):1423–34.
  28. Obesity: preventing and managing the global epidemic. Report of a WHO consultation. World Health Organ Tech Rep Ser. 2000;894:i–xii.
  29. WHO Multicentre Growth Reference Study Group. WHO Child Growth Standards based on length/height, weight and age. Acta Paediatr Suppl. 2006 Apr;450:76–85.
  30. Sport Schweiz 2020: Kinder- und Jugendbericht. 2021. (Accessed 29.03.2022, at
  31. Schindera C, Weiss A, Hagenbuch N, Otth M, Diesch T, von der Weid N, et al.; Swiss Pediatric Oncology Group (SPOG). Physical activity and screen time in children who survived cancer: A report from the Swiss Childhood Cancer Survivor Study. Pediatr Blood Cancer. 2020 Feb;67(2):e28046.
  32. Rubbo B, Best S, Hirst RA, Shoemark A, Goggin P, Carr SB, et al.; English National Children’s PCD Management Service. Clinical features and management of children with primary ciliary dyskinesia in England. Arch Dis Child. 2020 Aug;105(8):724–9.
  33. Das Gewicht der Schweiz. Eine quantitative Synthesestudie zum Body Mass Index und Bauchumfang sowie den damit verbundenen Kofaktoren bei erwachsenen Männern und Frauen in der Schweiz. 2020. at
  34. Barak A, Wexler ID, Efrati O, Bentur L, Augarten A, Mussaffi H, et al. Trampoline use as physiotherapy for cystic fibrosis patients. Pediatr Pulmonol. 2005 Jan;39(1):70–3.
  35. Schofield LM, Horobin HE. Growing up with Primary Ciliary Dyskinesia in Bradford, UK: exploring patients experiences as a physiotherapist. Physiother Theory Pract. 2014 Apr;30(3):157–64.

Most read articles by the same author(s)