Cystic fibrosis
(SEE ALSO - Sputum Clearance)


BRITISH PHYSIOTHERAPY GUIDELINES (2002) – CF Trust and ACPCF
https://www.cebp.nl/media/m322.pdf

AMERICAN PHYSIOTHERAPY GUIDELINES (2009) - Clinical Practice Guidelines for Pulmonary Therapies Committee - Respir Care, 54, 4, 522–37
www.rcjournal.com/contents/04.09/04.09.0522.pdf

BRITISH MEDICAL GUIDELINES (1996) Clinical Guidelines for CF Care. Royal College of Physicians.

PHYSIOTHERAPY FOR CYSTIC FIBROSIS (2007)
www.cftrust.org.uk/aboutcf/publications/factsheets/Airways-clearance-june07-for_web.pdf


Aaron SD, Chaparro C (2016) Referral to lung transplantation - too little, too late. J Cyst Fibr; 15(2):143–144
• lung transplantation shows clear survival benefits

Abbas C et al (2013) Treatment response of airway clearance assessed by single-breath washout in children with cystic fibrosis. J Cyst Fibr; 12(6):567-574

Abbott J, Hart A, Morton AM et al (2009) Can health-related quality of life predict survival in adults with cystic fibrosis? Am J Respir Crit Care Med, 173, 1, 54-8
• quality of life is associated with longer survival
• surgery may benefit patients whose cough is caused by reflux

Barry PJ, Flume PA (2017) Bronchodilators in cystic fibrosis: a critical analysis. Expert Rev Respir Med; 11(1):13-20. doi: 10.1080/17476348.2017.1246358
• bronchodilators are often used, but without clear evidence

Berkhout MC, Klerx-Melis F, Fokkens WJ et al (2016) CT-abnormalities, bacteriology and symptoms of sinonasal disease in children with Cystic Fibrosis. J Cyst Fibr;
• sinonasal disease and symptoms are present from shortly after birth

Bilodeau C, Bardou O, Maillé E et al (2016) Deleterious impact of hyperglycemia on cystic fibrosis airway ion transport and epithelial repair. J Cyst Fibr; 15(1):43–51. doi: dx.doi.org/10.1016/j.jcf.2015.04.002

Blackwell LS and Quittner AL (2015) Daily pain in adolescents with CF: Effects on adherence, psychological symptoms, and health-related quality of life. Ped Pulmonol; 50(3):244–251. doi: 10.1002/ppul.23091

Bourke SJ, Booth Z, Doe S et al (2016) A service evaluation of an integrated model of palliative care of cystic fibrosis. Palliat Med; 30(7):698-702. doi: 10.1177/0269216315626658

Bradley JM, Moran FM, Elborn JS (2006) Evidence for physical therapies (airway clearance and physical training) in cystic fibrosis. Respir Med,100, 2, 191-201
www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WWS-4J1B1F4-1&_user=10&_coverDate=02%2F28%2F2006&_alid=467613588&_rdoc=1&_fmt=summary&_orig=search&_cdi=7138&_sort=d&_docanchor=&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=abb5b0b3d56afc9c86e5176daa16e851
• five Cochrane systematic reviews which show the benefit of PT

Burge AT, Holland AE, Sherburn M et al (2015) Prevalence and impact of urinary incontinence in men with cystic fibrosis. Physiotherapy; 101(2):166-170. doi:10.1016/j.physio.2014.11.001

Button BM, Wilson C, Dentice R et al (2016) Physiotherapy for cystic fibrosis in Australia and New Zealand: A clinical practice guideline. Respirology; 21(4):656-67. doi: 10.1111/resp.12764

Chakrabarty B et al (2013) Peripheral neuropathy in cystic fibrosis: A prevalence study. J Cyst Fibr; 12(6):754-760

Cialdella P, Carella F (2012) Hypervitaminosis D: case report of pediatric osteoporosis secondary to cystic fibrosis. Clin Cases Miner Bone Metab, 8, 3, 66–68
www.ncbi.nlm.nih.gov/pmc/articles/PMC3317090/?tool=pmcentrez

Cox NS, Alison JA, Button BM et al (2015) Feasibility and acceptability of an internet-based program to promote physical activity in adults with cystic fibrosis. Respir Care; 60(3):422-429

Cox NS, Alison JA, Button BM et al (2016) Physical activity participation by adults with cystic fibrosis: An observational study. Respirology; 21(3):511-8. doi: 10.1111/resp.12719
• regular exercise reduces airflow obstruction and hospital admissions

Craparo EF, Porsio B, Schillaci D et al (2017) Polyanion-tobramycin nanocomplexes into functional microparticles for the treatment of Pseudomonas aeruginosa infections in cystic fibrosis. Nanomedicine (Lond); 12(1):25-42.

Daniel S, Oades PJ, Williams CA et al (2015) Airflow limitation following cardiopulmonary exercise testing and heavy-intensity intermittent exercise in children with cystic fibrosis. Europ J Pediatrics; 174(2):251-257

Daniels T (2010) Physiotherapeutic management strategies for the treatment of cystic fibrosis in adults. J Multidiscip Healthc, 3, 201–212
www.ncbi.nlm.nih.gov/pmc/articles/PMC3024890/?tool=pmcentrez

Dassios T (2015) Determinants of respiratory pump function in patients with cystic fibrosis. Paed Respir Rev; 16(1):75–79. doi:10.1016/j.prrv.2014.01.001

Davies G, Aurora P (2017) The use of multiple breath washout for assessing cystic fibrosis in infants. Expert Rev Respir Med; 11(1):21-28. doi: 10.1080/17476348.2017.1269604

Dentice RL, Elkins MR, Middleton PG et al (2016) A randomised trial of hypertonic saline during hospitalisation for exacerbation of cystic fibrosis. Thorax; 71(2): 141-147. doi:10.1136/thoraxjnl-2014-206716
thorax.bmj.com/content/71/2/141.full
• hypertonic saline speeds the resolution of exacerbation symptoms

Doeleman WR, Takken T, Bronsveld I et al (2016) Relationship between lung function and Modified Shuttle Test performance in adult patients with cystic fibrosis: a cross-sectional, retrospective study. Physiotherapy; 102(2):184-188

Doumit M and Jaffé A (2015) Use of the lung flute for sputum induction in children with cystic fibrosis: A pilot study. Ped Pulmonol; 50(4):340–343. doi: 10.1002/ppul.23126

Driscoll KA, Modi AC, Filigno SS et al (2015) Quality of life in children with CF: Psychometrics and relations with stress and mealtime behaviors. Ped Pulmonol; 50(6): 560–567. doi: 10.1002/ppul.23149

Duff AJ, Latchford GJ (2013) Motivational interviewing for adherence problems in cystic fibrosis; evaluation of training healthcare professionals. J Clin Med Res; 5(6):475-80. doi: 10.4021/jocmr1603w

Elborn JS (2016) Cystic fibrosis. Lancet; 388(10059):2519-2531. doi: 10.1016/S0140-6736(16)00576-6

Elphick HE, Mallory G (2013) Oxygen therapy for cystic fibrosis. Cochrane Syst Rev; Jul 25;7:CD003884. doi: 10.1002/14651858.CD003884.pub4

Fathi H, Moon T, Donaldson J et al (2009) Cough in adult cystic fibrosis: diagnosis and response to fundoplication. Cough, 5, 1.

Feiten T dos S, Flores JS, Farias BL et al (2016) Respiratory therapy: a problem among children and adolescents with cystic fibrosis. Jornal Brasileiro de Pneumologia; 42(1):29-34. doi: 10.1590/S1806-37562016000000068.
www.ncbi.nlm.nih.gov/pmc/articles/PMC4805384/
• low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in paediatric CF patients

Fielding J, Brantley L, Seigler N et al (2015) Oxygen uptake kinetics and exercise capacity in children with cystic fibrosis. Ped Pulmonol; 50(7):647–654. doi: 10.1002/ppul.23189

Fitch G, Williams K, Freeston JE et al (2016) Ultrasound and magnetic resonance imaging assessment of joint disease in symptomatic patients with cystic fibrosis arthropathy. J Cyst Fibros; 15(4):e35-40. doi: 10.1016/j.jcf.2015.12.022
• inflammatory joint damage can be identified by ultrasound and MRI

Flores JS, Teixeira FÂ, Rovedder PM et al (2013) Adherence to airway clearance therapies by adult cystic fibrosis patients. Respir Care; 58(2):279-85. doi: 10.4187/respcare.01389

Flores JS, Rovedder PME, Ziegler B et al (2016) Clinical outcomes and prognostic factors in a cohort of adults with cystic fibrosis: a 7-year follow-up study. Respir Care; 61(2):192-199

Flume PA, Robinson KA, Sullivan BP et al (2009) Cystic fibrosis pulmonary guidelines: airway clearance therapies. Respir Care, 54, 4, 522–37
www.rcjournal.com/contents/04.09/04.09.0522.pdf

Ford B (2017) CFTR structure: lassoing cystic fibrosis. Nat Struct Mol Biol; 5;24(1):13-14. doi: 10.1038/nsmb.3353

Forte GC, Barni GC, Perin C et al (2015) Relationship between clinical variables and health-related quality of life in young adult subjects with cystic fibrosis. Respir Care; 60(10):1459-1468

Goss CH, MacNeill SJ, Quinton HB et al (2015) Children and young adults with CF in the USA have better lung function compared with the UK. Thorax; 70:229-36. doi:10.1136/thoraxjnl-2014-205718

Goss CH (2003) Airway clearance in CF. Respir Care, 48, 20-21
• physiotherapy should be started immediately diagnosis is made, to minimise cycle of airway damage

Haack A, Aragão GG, Novaes MR (2013) Pathophysiology of cystic fibrosis and drugs used in associated digestive tract diseases. World J Gastroenterol; 19(46):8552-61. doi: 10.3748/wjg.v19.i46.8552
www.ncbi.nlm.nih.gov/pmc/articles/PMC3870500/

Haq IJ, Gray MA, Garnett JP et al (2016) Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets. Thorax; 71(3):284-287. doi: 10.1136/thoraxjnl-2015-207588

Hauser AR, Jain M, Bar-Meir M et al (2011) Clinical significance of microbial infection and adaptation in cystic fibrosis. Clin Microbiol Rev, 24, 1, 29–70
www.ncbi.nlm.nih.gov/pmc/articles/PMC3021203/?tool=pmcentrez

Havermans et al 2013) Pain in CF: Review of the literature. J Cyst Fibr, 12(5):423-430

Haworth CS, Dodd ME, Atkins M et al (2000) Pneumothorax in adults with CF dependent on NIPPV. Thorax, 55, 620-22
• practical info on safe non-invasive ventilation

Hayes Don Jr, Glanville AR, McGiffin D et al (2016) Age-related survival disparity associated with lung transplantation in cystic fibrosis: an analysis of the registry of the International Society for Heart and Lung Transplantation. J Heart Lung Transplantation; 35(9):1108-15
• transplantation shows greater success in adults than in children

Hebestreit A (2001) Exercise inhibits epithelial sodium channels in patients with CF. Am J Respir Crit Care Med, 164, 443-6
• exercise increases the water content of mucus

Heltshe SL, Goss CH (2016) Optimising treatment of CF pulmonary exacerbation: a tough nut to crack. Thorax; 71(2):101-102. doi: 10.1136/thoraxjnl-2015-208057
• exacerbations negatively affect quality of life, morbidity and mortality

Hoffman LR, Ramsey BW (2013) Cystic fibrosis therapeutics: the road ahead. Chest, 143, 1, 207-213. doi:10.1378/chest.12-1639

Hoo ZH, Daniels T, Wildman MJ et al (2015) Airway clearance techniques used by people with cystic fibrosis in the UK. Physiotherapy; 101(4): 340–348. doi: dx.doi.org/10.1016/j.physio.2015.01.008

Hoppe JE, Towler E, Wagner BD et al (2015) Sputum induction improves detection of pathogens in children with cystic fibrosis. Ped Pulmonol; 50(7):638–646. doi: 10.1002/ppul.23150

Hotaling JM (2014) Genetics of male infertility. Urol Clin North Am; 41(1):1-17. doi: 10.1016/j.ucl.2013.08.009

Hurt K, Bilton D (2016) Inhaled interventions in cystic fibrosis: mucoactive and antibiotic therapies.
Respiration; 88(6):441-8. doi: 10.1159/000369533

Ireland C (2003) Adherence to physiotherapy and quality of life for adults and adolescents with cystic fibrosis. Physiother, 89, 7, 397-407

Irons JY, Kuipers K, Petocz P (2013) Exploring the health benefits of singing for young people with cystic fibrosis. Internat J Ther Rehabil, 20, 3, 144-153

Karapanagiotis S (2017) Ventilatory limitation and dynamic hyperinflation during exercise testing in cystic fibrosis. Pediatr Pulmonol; 52(1):29-33. doi: 10.1002/ppul.23572

Kaunitz JD, Akiba Y (2011) Purinergic regulation of duodenal surface pH and ATP concentration: implications for mucosal defence, lipid uptake and cystic fibrosis. Acta Physiologica, 201, 1, 109–116

Kelemen L, Lee AL, Button BM et al (2012), Pain impacts on quality of life and interferes with treatment in adults with cystic fibrosis. Physiother Res Internat, 17, 3, 132–141. doi: 10.1002/pri.524

Kerem E, Cohen-Cymberknoh M (2016) Disparities in cystic fibrosis care and outcome: socioeconomic status and beyond. Chest; 149(2):298-300. doi:10.1016/j.chest.2015.08.021
journal.publications.chestnet.org/article.aspx?articleID=2487102

Kilcoyne A, Lavelle LP, McCarthy CJ et al (2016) Chest CT abnormalities and quality of life: relationship in adult cystic fibrosis. Ann Transl Med; 4(5):87. doi: 10.21037/atm.2016.03.08

Kilpatrick P et al (2002) The effect of physiotherapy in stable CF patients attending an outpatient clinic. J Ass Chartered Physiother Resp Care, 34, 22-4
• physiotherapy reduces airflow obstruction and increasing oxygenation

Kitt H, Lenney W, Gilchrist FJ et al (2016) Two case reports of the successful eradication of new isolates of Burkholderia cepacia complex in children with cystic fibrosis. BMC Pharmacol Toxicol; 17(1):14. doi: 10.1186/s40360-016-0054-0

Kopp BT, Sarzynski L, Khalfoun S et al (2015) Detrimental effects of secondhand smoke exposure on infants with cystic fibrosis. Ped Pulmonol; 50(1):25–34. doi: 10.1002/ppul.23016
• passive smoking can stunt growth and facilitate infection in children with CF

Kriemler S, Kieser S, Junge S et al (2013) Effect of supervised training on FEV1 in cystic fibrosis: a randomised controlled trial. J Cyst Fibr; 12(6):714-720

Kriemler S, Radtke T, Christen G et al (2016) 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; 194(4):659-64. doi: 10.1007/s00408-016-9888-x
• in cystic fibrosis, exercise increases SpO2 as well as clearing extra sputum

Lahiri T, Hempstead SE, Brady C et al (2016) Clinical Practice Guidelines from the Cystic Fibrosis Foundation for preschoolers with cystic fibrosis. Pediatrics; 137(4). pii: e20151784. doi: 10.1542/peds.2015-1784

Laurin LP, Jobin V, Bellemare F et al (2012) Sternum length and rib cage dimensions compared with bodily proportions in adults with cystic fibrosis. Can Respir J, 19, 3, 196-200

Lester MK, Flume PA (2009) Airway-clearance therapy guidelines and implementation. Respir Care, 54, 6, 733-50

Levy H, Farrell PM (2015) New challenges in the diagnosis and management of cystic fibrosis. J Pediatrics; 166(6):1337–1341. doi:10.1016/j.jpeds.2015.03.042

Magnet FS (2017) Impact of pseudomonas aeruginosa infection on respiratory muscle function in adult cystic fibrosis patients. Respiration; 93(1):42-50.
• Pseudomonas aeruginosa impairs respiratory muscle function in adolescents

Massery M (2005) Musculoskeletal and neuromuscular interventions: a physical approach to cystic fibrosis. J R Soc Med;98 Suppl 45:55-66

Mayer-Hamblett N, Boyle M, VanDevanter D (2016) Advancing clinical development pathways for new CFTR modulators in cystic fibrosis. Thorax; 71(5):454-461. doi: 10.1136/thoraxjnl-2015-208123
thorax.bmj.com/content/71/5/454.full

McIlwaine MP, Alarie N, Davidson GF et al (2013) Long-term multicentre randomised controlled study of high frequency chest wall oscillation versus positive expiratory pressure mask in cystic fibrosis. Thorax; 68(8):746-51. doi: 10.1136/thoraxjnl-2012-202915

McIntosh LA (2015) Patients’ experience of portacaths in cystic fibrosis: questionnaire-based study. Arch Dis Child; 100(7):659-661. doi:10.1136/archdischild-2014-308045
• preferred position is lateral chest wall

McKeage K (2013) Tobramycin inhalation powder: a review of its use in the treatment of chronic pseudomonas aeruginosa infection in patients with cystic fibrosis. Drugs; 73(16):1815-1827
link.springer.com/journal/40265/73/16/page/1

Meltzer C, Båth M, Kheddache S et al (2016) Visibility of structures of relevance for patients with cystic fibrosis in chest tomosynthesis: influence of anatomical location and observer experience. Radiat Prot Dosimetry;169(1-4):177-87. doi: 10.1093/rpd/ncv556

Mengistu YA, Willgoss GT, Ade FF et al (2012) Relationship between anxiety, depression, and quality of life in adult patients with cystic fibrosis. Respir Care, 57, 4, 550-6

Miragoli F, Federici S, Ferrari S et al (2017) Impact of cystic fibrosis disease on archaea and bacteria composition of gut microbiota. FEMS Microbiol Ecol; 93(2). pii: fiw230.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5155554/

Mogayzel P, Naureckas ET, Robinson KA et al (2013) Cystic Fibrosis Pulmonary Guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med, 187, 7, 680-9

Montgomery ST, Mall MA, Kicic A et al (2017) Hypoxia and sterile inflammation in cystic fibrosis airways: mechanisms and potential therapies. Eur Respir J; 49(1). pii: 1600903. doi: 10.1183/13993003.00903-2016

Moore JE et al (2016) The importance of the mundane - nebuliser care and hygiene. J Cyst Fibr; 15(1):4–5. doi: dx.doi.org/10.1016/j.jcf.2015.11.006
www.cysticfibrosisjournal.com/article/S1569-1993%2815%2900264-7/fulltext
• dry powder inhalers negate the need for antibiotic nebulizers, over half of which are contaminated when used at home

Nicholson TT, Barry PJ, Waterhouse DF (2017) Relationship between pulmonary hyperinflation and dyspnoea severity during acute exacerbations of cystic fibrosis. Respirology; 22(1):141-148. doi: 10.1111/resp.12885

Nick JA, Nichols DP (2016) Diagnosis of adult patients with cystic fibrosis. Clin Chest Med; 37(1):47-57. doi: 10.1016/j.ccm.2015.11.006doi: 10.1136/bmjresp-2016-000168

O'Neill K, Moran F, Tunney MM et al (2017) Timing of hypertonic saline and airway clearance techniques in adults with cystic fibrosis during pulmonary exacerbation: pilot data from a randomised crossover study. BMJ Open Respir Res; 4(1):e000168
bmjopenrespres.bmj.com/content/4/1/e000168.citation-tools

Packel L, Sood M, Gormley M et al (2013) A pilot study exploring the role of physical therapists and transition in care of pediatric patients with cystic fibrosis to the adult setting. Cardiopulm Phys Ther J; 24(1):24-30

Parrot H et al (2013) Real life timing and cleaning of inhalation therapy. J CF; 12(suppl.1):S105

Pauwels A (2012) Risks of PPI treatment in patients with cystic fibrosis: effect of gastric juice of patients “on” PPI on IL-8 production by CF primary bronchial epithelial cells. J Cyst Fibr, 11, Suppl.1, S1

Peckham D (2013) Drug induced complications; can we do more? J Cyst Fibr: 12(6):547-558

Penafortes JT, Guimarães FS, Moço VJ et al (2013) Relationship between body balance, lung function, nutritional status and functional capacity in adults with cystic fibrosis. Braz J Phys Ther; 17(5):450-7.

Pham TMT, O'Malley L, Mayfield S et al (2015) The effect of high flow nasal cannula therapy on the work of breathing in infants with bronchiolitis. Ped Pulmonol; 50(7):713–720. doi: 10.1002/ppul.23060
• high flow nasal cannula therapy reduces the work of breathing in infants with bronchiolitis

Pryor JA, Tannenbaum E, Cramer D et al (2006) A comparison of five airway clearance techniques in the treatment of people with cystic fibrosis. J Cyst Fibr, 5, Suppl.1, S76
• little difference in outcome with various techniques

Quittner AL, Abbott J, Georgiopoulos AM et al (2016) International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety. Thorax; 71(1):26-34. doi:10.1136/thoraxjnl-2015-207488
thorax.bmj.com/content/71/1/26.full

Radtke T, Puhan MA, Hebestreit H et al (2016) The 1-min sit-to-stand test—A simple functional capacity test in cystic fibrosis? J Cyst Fibr; 15(2): 223–226
• the 1-min sit-to-stand test is a reliable and valid test to measure functional capacity

Raju SV, Lin VY, Liu L et al (2017) The cystic fibrosis transmembrane conductance regulator potentiator ivacaftor augments mucociliary clearance abrogating cystic fibrosis transmembrane conductance regulator inhibition by cigarette smoke. Am J Respir Cell Mol Biol; 56(1):99-108. doi: 10.1165/rcmb.2016-0226OC

Reichman G, De Boe V, Braeckman J et al (2016) Urinary incontinence in patients with cystic fibrosis. Scand J Urol; 50(2):128-31. doi: 10.3109/21681805.2015.1096826

Reix P, Aubert F, Werch-Galois M-C et al (2012) Exercise with incorporated expiratory manoeuvres was as effective as breathing techniques for airway clearance in children with cystic fibrosis: a randomised crossover trial. J Physiother, 58, 4, 241–247

Reix P, Matecki S, Fayon M (2016) Early lung disease in infants with cystic fibrosis. Diagnostic tools and possible therapeutic pathways. Rev Mal Respir; 33(2):102-16. doi: 10.1016/j.rmr.2015.06.017

Rogers D, Doull IJM (2005) Physiological principles of airway clearance techniques used in the physiotherapy management of cystic fibrosis. Curr Paeds, 15, 233-8
www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6WDB-4G4FW47-7&_coverDate=06%2F30%2F2005&_alid=355782247&_rdoc=1&_fmt=&_orig=search&_qd=1&_cdi=6762&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=adcaf8b19ae100c41c0e49657674719b

Rubin BK (2009) Mucus, phlegm, and sputum in cystic fibrosis. Respir Care, 54, 6, 726-32

Rutter WC, Burgess DR, Burgess DS (2017) Increasing incidence of multidrug resistance among cystic fibrosis respiratory bacterial isolates. Microb Drug Resist; 23(1):51-55. doi: 10.1089/mdr.2016.0048

Saint-Criq V, Gray MA (2017) Role of CFTR in epithelial physiology. Cell Mol Life Sci; 74(1):93-115. doi: 10.1007/s00018-016-2391-y

Salonini E, Gambazza S, Meneghelli I et al (2015) Active video game playing in children and adolescents with cystic fibrosis: exercise or just fun? Respir Care; 60(8):1172-1179

Sandsund C, Roughton M, Hodson ME, Pryor JA (2011) Musculoskeletal techniques for clinically stable adults with cystic fibrosis: a preliminary randomised controlled trial. Physiother, 97, 209-217

Schindel CS, Hommerding PX, Melo DAS et al (2015) Physical exercise recommendations improve postural changes found in children and adolescents with cystic fibrosis: a randomized controlled trial. J Pediatrics; 166(3):710–716.e2. doi: 10.1016/j.jpeds.2014.12.001

Schneider EK (2017) Can cystic fibrosis patients finally catch a breath with Lumacaftor/Ivacaftor? Clin Pharmacol Ther; 101(1):130-141. doi: 10.1002/cpt.548

Simoneau T, Sawicki GS, Milliren CE et al (2016) A randomized controlled trial of vitamin D replacement strategies in pediatric CF patients. J Cyst Fibr; 15(2): 234–241
www.cysticfibrosisjournal.com/article/S1569-1993%2815%2900166-6/abstract

Slavici T, Almajan B (2013) Artificial intelligence techniques: An efficient new approach to challenge the assessment of complex clinical fields such as airway clearance techniques in patients with cystic fibrosis? J Rehab Med, 45, 4, 397-402(6). doi: 10.2340/16501977-1124

Sokol G, Vilozni D, Hakimi R et al (2015) The short-term effect of breathing tasks via an incentive spirometer on lung function compared with autogenic drainage in subjects with cystic fibrosis. Respir Care; 60(12):1819-1825
• autogenic drainage can mobilise secretions and improve oxygen saturation

Solé A, Pérez I, Vázquez I et al (2016) Patient-reported symptoms and functioning as indicators of mortality in advanced cystic fibrosis: a new tool for referral and selection for lung transplantation. J Heart Lung Transplant; 35(6):789-94. doi: 10.1016/j.healun.2016.01

Sontag MK (2010) Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosis. Pediatr Pulmonol, 45, 3, 291-300

Stevens D (2017) Clinical value of pulmonary hyperinflation as a treatment outcome in cystic fibrosis. Respirology; 22(1):12-13. doi: 10.1111/resp.12906.
onlinelibrary.wiley.com/doi/10.1111/resp.12906/full

Thomsen K, Christophersen L, Bjarnsholt T et al (2016) Anti-Pseudomonas aeruginosa IgY antibodies augment bacterial clearance in a murine pneumonia model. J Cyst Fibr; 15(2): 171–178
• gargling with egg yolk immunoglobulins may reduce Pseudomonas

VanDevanter DR, Kahle JS, O’Sullivan AK et al (2016) Cystic fibrosis in young children: A review of disease manifestation, progression, and response to early treatment. J Cyst Fibr; 15(2): 147–157
• growth impairment and airway abnormalities are present at birth
• disease progression is reported in infancy and throughout childhood.
• early access to routine CF management is associated with improved subsequent health status.

Vendrusculo FM, Heinzmann-Filho JP, Piva TC et al (2016) Inspiratory muscle strength and endurance in children and adolescents with cystic fibrosis. Respir Care; 61(2):184-191

Vermeulen F, Proesmans M, Boon M et al (2014) Lung clearance index predicts pulmonary exacerbations in young patients with cystic fibrosis. Thorax; 69(1):69:39-45. doi:10.1136/thoraxjnl-2013-203807

Williams CA (2016) Physical activity and health of adults with cystic fibrosis. Respirology; 21(3):404-5. doi: 10.1111/resp.12765
onlinelibrary.wiley.com/doi/10.1111/resp.12765/epdf

Williams HD, Behrends V (2010) Hypertonic saline therapy in cystic fibrosis: do population shifts caused by the osmotic sensitivity of infecting bacteria explain the effectiveness of this treatment? Front Microbiol, 1, 120


TREATMENT DIARY FOR PATIENTS:
www.cfvoice.com


ACPCF = Association of Chartered Physiotherapist in Cystic Fibrosis
NIV = non-invasive ventilation
PD = postural drainage
PEP = positive expiratory pressure
RCP = Royal College of Physicians