Intro

(Ratjen et al., 2015)

Prevalence
)Cystic Fibrosis Trust,” 2022“(
Life expectancy
)Lopes-Pacheco, 2016(

Diagnosis and treatment

)National Heart, Lung, and Blood Institute,” 2022 “(

Lung function
Slide one

)Milla and Warwick, 1998(

List S2

)Chen et al., 2018(

)Hommerding et al., 2015(

)Emirza et al., 2021 (

Slide 3
)Schneiderman-Walker et al., 2000 (

)Gupta et al., 2019a(

Slide 4

)Kriemler et al., 2013 (

QUALITY OF LIFE
:S1
)Blanco-Orive et al., 2022; Quittner et al., 2005; Uchmanowicz et al., 2015 (

:S2
 )Gupta et al., 2019; Klijn et al., 2004 (

S3

)Urquhart et al., 2012 (

Cochraance
)Radtke et al., 2022(
Guidelines
)Williams et al., 2010 (

Reference

Blanco-Orive, P., del Corral, T., Martín-Casas, P., Ceniza-Bordallo, G., López-de-Uralde-Villanueva, I.,
2022. Quality of life and exercise tolerance tools in children/adolescents with cystic fibrosis:
Systematic review. Medicina Clínica (English Edition) 158, 519–530.
https://doi.org/10.1016/j.medcle.2022.05.001
Chen, J.J., Cooper, D.M., Haddad, F., Sladkey, A., Nussbaum, E., Radom-Aizik, S., 2018. Tele-Exercise
as a Promising Tool to Promote Exercise in Children With Cystic Fibrosis. Front Public Health 6.
https://doi.org/10.3389/fpubh.2018.00269
Cystic Fibrosis Trust [WWW Document], 2022.
Emirza, C., Aslan, G.K., Kilinc, A.A., Cokugras, H., 2021. Effect of expiratory muscle training on peak
cough flow in children and adolescents with cystic fibrosis: A randomized controlled trial.
Pediatr Pulmonol 56, 939–947. https://doi.org/10.1002/ppul.25259
Gupta, S., Mukherjee, A., Lodha, R., Kabra, M., Deepak, K.K., Khadgawat, R., Talwar, A., Kabra, S.K.,
2019a. Effects of Exercise Intervention Program on Bone Mineral Accretion in Children and
Adolescents with Cystic Fibrosis: A Randomized Controlled Trial. The Indian Journal of Pediatrics
86, 987–994. https://doi.org/10.1007/s12098-019-03019-x
Gupta, S., Mukherjee, A., Lodha, R., Kabra, M., Deepak, K.K., Khadgawat, R., Talwar, A., Kabra, S.K.,
2019b. Effects of Exercise Intervention Program on Bone Mineral Accretion in Children and
Adolescents with Cystic Fibrosis: A Randomized Controlled Trial. The Indian Journal of Pediatrics
86, 987–994. https://doi.org/10.1007/s12098-019-03019-x
Hommerding, P.X., Baptista, R.R., Makarewicz, G.T., Schindel, C.S., Donadio, M.V., Pinto, L.A.,
Marostica, P.J., 2015. Effects of an Educational Intervention of Physical Activity for Children and
Adolescents With Cystic Fibrosis: A Randomized Controlled Trial. Respir Care 60, 81–87.
https://doi.org/10.4187/respcare.02578
Klijn, P.H.C., Oudshoorn, A., van der Ent, C.K., van der Net, J., Kimpen, J.L., Helders, P.J.M., 2004.
Effects of Anaerobic Training in Children With Cystic Fibrosis. Chest 125, 1299–1305.
https://doi.org/10.1378/chest.125.4.1299
Kriemler, S., Kieser, S., Junge, S., Ballmann, M., Hebestreit, A., Schindler, C., Stüssi, C., Hebestreit, H.,
2013. Effect of supervised training on FEV1 in cystic fibrosis: A randomised controlled trial.
Journal of Cystic Fibrosis 12, 714–720. https://doi.org/10.1016/j.jcf.2013.03.003
Lopes-Pacheco, M., 2016. CFTR Modulators: Shedding Light on Precision Medicine for Cystic Fibrosis.
Front Pharmacol 7. https://doi.org/10.3389/fphar.2016.00275
Milla, C.E., Warwick, W.J., 1998. Risk of Death in Cystic Fibrosis Patients With Severely Compromised
Lung Function. Chest 113, 1230–1234. https://doi.org/10.1378/chest.113.5.1230
National Heart, Lung, and Blood Institute, 2022.
Quittner, A.L., Buu, A., Messer, M.A., Modi, A.C., Watrous, M., 2005. Development and Validation of
the Cystic Fibrosis Questionnaire in the United States. Chest 128, 2347–2354.
https://doi.org/10.1378/chest.128.4.2347
Radtke, T., Smith, S., Nevitt, S.J., Hebestreit, H., Kriemler, S., 2022. Physical activity and exercise
training in cystic fibrosis. Cochrane Database of Systematic Reviews 2022.
https://doi.org/10.1002/14651858.CD002768.pub5
Ratjen, F., Bell, S.C., Rowe, S.M., Goss, C.H., Quittner, A.L., Bush, A., 2015. Cystic fibrosis. Nat Rev Dis
Primers 1, 15010. https://doi.org/10.1038/nrdp.2015.10
Schneiderman-Walker, J., Pollock, S.L., Corey, M., Wilkes, D.D., Canny, G.J., Pedder, L., Reisman, J.J.,
2000. A randomized controlled trial of a 3-year home exercise program in cystic fibrosis. J
Pediatr 136, 304–310. https://doi.org/10.1067/mpd.2000.103408
Uchmanowicz, I., Jankowska-Polańska, B., Rosińczuk, J., Wleklik, M., 2015. Health-Related Quality of
Life of Patients Suffering From Cystic Fibrosis. Advances in Clinical and Experimental Medicine
24, 147–152. https://doi.org/10.17219/acem/38147
Urquhart, D., Sell, Z., Dhouieb, E., Bell, G., Oliver, S., Black, R., Tallis, M., 2012. Effects of a
supervised, outpatient exercise and physiotherapy programme in children with cystic fibrosis.
Pediatr Pulmonol 47, 1235–1241. https://doi.org/10.1002/ppul.22587
Williams, C.A., Benden, C., Stevens, D., Radtke, T., 2010. Exercise Training in Children and
Adolescents with Cystic Fibrosis: Theory into Practice. Int J Pediatr 2010, 1–7.
https://doi.org/10.1155/2010/670640
 

•  American College of Sports Medicine, 2013. ACSM's guidelines for exercise testing and
prescription. Lippincott williams & wilkins.
• Blanco-Orive, P., Del Corral, T., Martín-Casas, P., Ceniza-Bordallo, G. and López-de-
Uralde-Villanueva, I., 2022. Quality of life and exercise tolerance tools in
children/adolescents with cystic fibrosis: Systematic review. Medicina Clínica (English
Edition).
• Bradley, J.M. and Moran, F., 2008. Physical training for cystic fibrosis. Cochrane
Database of Systematic Reviews, (1)
• Centers for Disease Control and Prevention (2020). Cystic Fibrosis | CDC. www.cdc.gov.
Available at: https://www.cdc.gov/genomics/disease/cystic_fibrosis.htm (Accessed:
30/03/2023).
• Charman, S., McClenaghan, E., Cosgriff, R., Lee, A. and Carr, S., 2019. UK Cystic Fibrosis
Registry Annual data report 2018. Cystic Fibrosis Trust, London, United Kingdom.
• Chen, J.J., Cooper, D.M., Haddad, F., Sladkey, A., Nussbaum, E. and Radom-Aizik, S.,
2018. Tele-exercise as a promising tool to promote exercise in children with cystic
fibrosis. Frontiers in public health, 6, p.269.
• Cystic fibrosis FAQs (no date) CF Trust. Available at:
https://www.cysticfibrosis.org.uk/what-is-cystic-fibrosis/faqs#:~:text=Around
%2010%2C800%20people%20in%20the,100%2C000%20people%20in%20the%20world.
(Accessed: April 28, 2023).
• Emirza, C., Aslan, G.K., Kilinc, A.A. and Cokugras, H., 2021. Effect of expiratory muscle
training on peak cough flow in children and adolescents with cystic fibrosis: A
randomized controlled trial. Pediatric Pulmonology, 56(5), pp.939-947.
• Gruber, W., Stehling, F., Blosch, C., Dillenhoefer, S., Olivier, M., Koerner-Rettberg, C.,
Sutharsan, S., Mellies, U., Taube, C. and Welsner, M., 2022. Effects of a Long-Term
Monitored Exercise Program on Aerobic Fitness in a Small Group of Children with Cystic
Fibrosis. International Journal of Environmental Research and Public Health, 19(13),
p.7923.
• Gupta, S., Mukherjee, A., Lodha, R., Kabra, M., Deepak, K.K., Khadgawat, R., Talwar, A.
and Kabra, S.K., 2019. Effects of exercise intervention program on bone mineral
accretion in children and adolescents with cystic fibrosis: a randomized controlled
trial. The Indian Journal of Pediatrics, 86, pp.987-994.
• Heinz, K.D., Walsh, A., Southern, K.W., Johnstone, Z. and Regan, K.H., 2022. Exercise
versus airway clearance techniques for people with cystic fibrosis. Cochrane Database of
Systematic Reviews, (6).
• Hommerding, P.X., Baptista, R.R., Makarewicz, G.T., Schindel, C.S., Donadio, M.V., Pinto,
L.A. and Marostica, P.J., 2015. Effects of an educational intervention of physical activity
for children and adolescents with cystic fibrosis: a randomized controlled
trial. Respiratory care, 60(1), pp.81-87.
• Klijn, P.H., Oudshoorn, A., van der Ent, C.K., van der Net, J., Kimpen, J.L. and Helders,
P.J., 2004. Effects of anaerobic training in children with cystic fibrosis: a randomized
controlled study. Chest, 125(4), pp.1299-1305.
• Kriemler, S., Kieser, S., Junge, S., Ballmann, M., Hebestreit, A., Schindler, C., Stüssi, C.
and Hebestreit, H., 2013. Effect of supervised training on FEV1 in cystic fibrosis: a
randomised controlled trial. Journal of Cystic Fibrosis, 12(6), pp.714-720.
• Lopes-Pacheco, M., 2016. CFTR modulators: shedding light on precision medicine for
cystic fibrosis. Frontiers in pharmacology, 7, p.275.
• Milla, C.E. and Warwick, W.J., 1998. Risk of death in cystic fibrosis patients with severely
compromised lung function. Chest, 113(5), pp.1230-1234.
• Narang, I., Pike, S., Rosenthal, M., Balfour‐Lynn, I.M. and Bush, A., 2003. Three‐minute
step test to assess exercise capacity in children with cystic fibrosis with mild lung
disease. Pediatric pulmonology, 35(2), pp.108-113.
• NHS (2021). Overview - Cystic fibrosis. NHS. Available at:
https://www.nhs.uk/conditions/cystic-fibrosis/ (Accessed: 30/03/2023).
• Paranjape, S.M., Barnes, L.A., Carson, K.A., von Berg, K., Loosen, H. and Mogayzel Jr, P.J.,
2012. Exercise improves lung function and habitual activity in children with cystic
fibrosis. Journal of Cystic Fibrosis, 11(1), pp.18-23.
• Quittner, A.L., Buu, A., Messer, M.A., Modi, A.C. and Watrous, M., 2005. Development
and validation of The Cystic Fibrosis Questionnaire in the United States: a health-related
quality-of-life measure for cystic fibrosis. Chest, 128(4), pp.2347-2354.
• Ratjen, F., Bell, S.C., Rowe, S.M., Goss, C.H., Quittner, A.L. and Bush, A., 2015. Cystic
fibrosis. vol. 1. Nat. Rev. Dis. Primers, 15010.
• Schneiderman-Walker, J., Pollock, S.L., Corey, M., Wilkes, D.D., Canny, G.J., Pedder, L.
and Reisman, J.J., 2000. A randomized controlled trial of a 3-year home exercise
program in cystic fibrosis. The Journal of pediatrics, 136(3), pp.304-310.
• Schneiderman-Walker, J., Pollock, S.L., Corey, M., Wilkes, D.D., Canny, G.J., Pedder, L.
and Reisman, J.J., 2000. A randomized controlled trial of a 3-year home exercise
program in cystic fibrosis. The Journal of pediatrics, 136(3), pp.304-310.
• Selvadurai, H.C., Blimkie, C.J., Meyers, N., Mellis, C.M., Cooper, P.J. and Van Asperen,
P.P., 2002. Randomized controlled study of in‐hospital exercise training programs in
children with cystic fibrosis. Pediatric pulmonology, 33(3), pp.194-200.
• Uchmanowicz, I., Jankowska-Polańska, B., Rosińczuk, J. and Wleklik, M., 2015. Health-
related quality of life of patients suffering from cystic fibrosis. Adv Clin Exp Med, 24(1),
pp.147-52.
• UK Cystic Fibrosis Registry 2021 Annual Data Report. (2022). Available at:
https://www.cysticfibrosis.org.uk/sites/default/files/2022-12/CF%20Trust%20Annual
%20Data%20Report%202021%20-%20WEB.pdf (Accessed: 24/04/2023).
• Urquhart, D., Sell, Z., Dhouieb, E., Bell, G., Oliver, S., Black, R. and Tallis, M., 2012. Effects
of a supervised, outpatient exercise and physiotherapy programme in children with
cystic fibrosis. Pediatric pulmonology, 47(12), pp.1235-1241.
• What is cystic fibrosis? (no date) National Heart Lung and Blood Institute. U.S.
Department of Health and Human Services. Available at:
https://www.nhlbi.nih.gov/health/cystic-fibrosis (Accessed: April 28, 2023).
• Williams, C.A., Benden, C., Stevens, D. and Radtke, T., 2010. Exercise training in children
and adolescents with cystic fibrosis: theory into practice. International journal of
pediatrics, 2010.
• Zach, M., Purrer, B. and Oberwaldner, B., 1981. Effect of swimming on forced expiration
and sputum clearance in cystic fibrosis. The Lancet, 318(8257), pp.1201-1203.