(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


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
Apps E et al (1998) Sputum rheology changes in CF lung disease following two different types of physiotherapy. Flutter versus autogenic drainage. Chest, 114, 171-7
www.chestjournal.org/cgi/reprint/114/1/171

Balfour-Lynn IM, Prasad SA, Laverty A et al (1998) A step in the right direction: assessing exercise tolerance in CF. Ped Pulmonol, 25, 278-84.
• 3-minute step test for assessing exercise tolerance

Bargon J (1999) Prophylactic antibiotic therapy is associated with increased prevalence of Aspergillus colonization in CF. Resp Med. 93,835-8
• long-term antibiotics predispose lungs to Aspergillus colonisation

Bell S (2004) Abnormal glycaemic control is common and easily detectable in children and adults with cystic fibrosis. Eur Respir J, 24: Suppl. 48, 616s
www.ersnet.org/ers/lr/browse/media.aspx?id_dossier=18215&id_fiche=123909
• glucose intolerance is very common in CF and early identification is advisable

Bech B (2004) Long-term outcome of lung transplantation for cystic fibrosis. Eur J Cardio-Thoraco Surg, 26, 1180-6
www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6T35-4DDRBJM-5&_coverDate=12%2F01%2F2004&_alid=296557256&_rdoc=1&_fmt=&_orig=search&_qd=1&_cdi=4937&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=42f47532cc5e59b2f745b0e6d382282e

Boucher GP (1997) Activity levels and the relationship to lung function and nutritional status in children with CF. Am Phys Med Rehab, 76, 311-15.

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

Bye PTP, Alison JA, Regnis JA (1997) Exercise performance and rehabilitation in cystic fibrosis. Crit Rev Phys Rehab Med. 9, 1, 1-33

Chetta A (2001) Six-minute walking test in CF adults. Resp Med, 95, 986-91
• six-minute test can be used to identify patients who might desaturate during exercise

Coates A (1997) Chest physiotherapy in CF. J Pediatrics, 131, 506-7
• condensed and balanced overview

Conway SP, Morton AM, Oldroyd B et al (2000) Osteoporosis and osteopenia in adults and adolescents with CF. Thorax, 55, 798-804

Dakin CJ, Numa AH, Wang HE et al (2002) Inflammation, infection and pulmonary function in infants and young children with CF. Am J Respir Crit Care Med, 165, 904-10
interaction between infection, inflammation and pulmonary function

Dark (1997) Lung: living related transplantation. Br.Med.Bull, 53, 892-903
• surgery in which a lower lobe donated by each parent

Dinwiddie (2000) Pathogenesis of lung disease in CF. Respiration, 67, 3-8
• 50% of patients have respiratory symptoms by age three months; bronchodilators show objective benefit in a minority but can increase bronchospasm in as many

Dufresne V, Knoop C, Van Muylem A (2009) Effect of systemic inflammation on inspiratory and limb muscle strength and bulk in cystic fibrosis. Am J Respir Crit Care Med, 180, 2, 153-158
ajrccm.atsjournals.org/cgi/content/abstract/180/2/153
• diaphragm thickness is increased in CF, but shows a marked variability between patients, this difference not being related to systemic inflammation; diaphragm training can occur despite inflammation.

Elborn JS, Prescott RJ, Stack BHR (2000) Elective versus symptomatic antibiotic treatment in cystic fibrosis patients. Thorax, 55, 355-8
• antibiotics best given on symptomatic basis

Elkins MR et al (2006) A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. New Eng J Med, 354, 229-40
www.nejm.org/doi/full/10.1056/NEJMoa043900
• hypertonic saline preceded by a bronchodilator is effective for patients with CF

Eng PA, Morton J, Douglass JA (1996) Short-term efficacy of ultrasonically nebulized hypertonic saline in CF. Pediatr.Pulmonol, 21, 77-83
• hypertonic saline improves lung function, precede by bronchodilator

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

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

Granton JT et al (2002) Noninvasive nocturnal ventilatory support in advanced lung disease from cystic fibrosis. Respir Care, 47, 675-81
• NIV well-tolerated and improves symptoms

Gumery L, Edenborough F, Stableforth D, Strachan A (1998) Physiotherapy and nebuliser use in a Birmingham adult cystic fibrosis unit. Physiotherapy, 84, 3, 127-132
• patients need an individually tailored airway clearance and exercise programme to improve adherence, knowledge and practice.

Heijerman HGM (1992) Long-term effects of exercise training and hyperalimentation in adult CF. Int.J.Rehab.Res, 15, 252-7.
• exercise training improves lung function, respiratory muscle endurance, VO2max

Haworth CS (2002) A prospective study of change in bone mineral density over one year in adults with CF. Thorax, 57, 719-23
• to inhibit the development of osteoporosis, vitamin D supplements required, HRT if puberty is delayed

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

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

Jennings P (1992) Coping mechanisms. Ped Nurs, 4, 8, 13-15.
• families of CF children show double the normal divorce rate

de Jong W, van Aalderen WMC, Kraan J et al (2001) Skeletal muscle strength in patients with cystic fibrosis. Physiother Theory Pract, 17, 23-8
• muscle strength reduced in patients with airflow obstruction; nutritional supplements required

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
onlinelibrary.wiley.com.ezproxy.brighton.ac.uk/doi/10.1111/j.1748-1716.2010.02156.x/full

Kettler LJ et al (2002) Determinants of adherence in adults with CF. Thorax, 57, 459-64

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
• evidence for physiotherapy reducing airflow obstruction and increasing oxygenation

Kluft J, Beker L, Castagnino M, Gaiser J, Chaney H (1996) A comparison of bronchial drainage treatments in cystic fibrosis. Pediatr Pulmonol , 22, 271-4
www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=retrieve&db=pubmed&list_uids=8905888&dopt=Abstract
• high-frequency chest wall oscillation at least as effective as postural drainage, vibrations & percussion

Luff V (1996) An evaluation of the effectiveness of PD in CF adults. ACPRC Journal, 9, 22-25.
• postural drainage increases mucus transport even in small-sputum producers

Madden BP (2002) Noninvasive ventilation in CF patients with acute or chronic respiratory failure. Eur Resp J, 19, 310-13
• NIV useful for acute patients and may prolong life in chronic patients

McIlwaine PM (2001) Long-term comparative trial of positive expiratory pressure versus oscillating positive expiratory pressure (flutter) physiotherapy in the treatment of cystic fibrosis. J.Pediatrics, 138, 845-50
www.jpeds.com/article/PIIS0022347601343494/abstract
• Flutter not as effective in maintaining pulmonary function in CF patients compared to PEP, and is more costly due to increased hospitalization and antibiotics.

McPhail GL (2010) Improving evidence-based care in cystic fibrosis through quality improvement. Arch Pediatr Adolesc Med, 164, 10, 957-60

Oermann CM (2000) Validation of an instrument measuring patient satisfaction with chest physiotherapy in CF. Chest, 118, 92-7

Orr A, McVean RJ, Webb AK, Dodd ME (2001) Questionnaire survey of urinary incontinence in women with CF. Br Med J, 322,1521
• high incidence of incontinence in all age groups, which affects ability to perform airway clearance

Prasad SA, Main E (1998) Finding evidence to support airway clearance techniques in CF. Disability & Rehabil, 20, 235-46
• there is no evidence base for chest clearance in children who have no respiratory symptoms

Riedler J (1996) Inhaled hypertonic saline increases sputum expectoration in CF. J.Pediatr.Child Health. 32, 48-50
• hypertonic saline clears more secretions than isotonic saline

Robinson PJ (2002) Dornase alfa in early CF. Paediatr Pulmonol, 34, 237-41
• reduction in exacerbations with DNase in some patients

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
Schneiderman J (2000) A randomized controlled trial of a 3-year home exercise program in CF. J.Pediatr. 136, 304-10

Selvadurai HC et al (2002) Randomized controlled study of in-hospital exercise training programs in children with CF. Pediatr Pulmonol, 33, 194-200
• both aerobic and resistance training benefit people with CF

Serra A (2002) Non-invasive proportional assist and pressure support ventilation in patients with CF and chronic respiratory failure. Thorax, 57, 50-54
• NIV can be used for acute decompensation or, nocturnally, for chronic respiratory failure

Shale DJ, Ionescu AA (2004) Mucus hypersecretion: a common symptom, a common mechanism? Eur Respir J, 23, 797-798
www.erj.ersjournals.com/content/23/6/797.full
• mechanism of mucus hypersecretion with particular reference to CF

Sharma R, Florea VG, Bolger AP et al (2001) Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax, 56, 746-50
• good nutrition improves survival

Smalldone GC, Palmer LB (2000) Aerosolized antibiotics current and future. Respir Care, 45, 67– 675
www.aarc.org/marketplace/reference_articles/06.00.0667.pdf

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

Steinkamp G, Wiedemann B (2002) Relationship between nutritional status and lung function in CF. Thorax, 57, 596-601

Wahl AK (2005) Living with cystic fibrosis: Impact on global quality of life. Heart Lung, 34, 5, 324-31

Wark PAB, McDonald V (2004) Nebulised hypertonic saline for cystic fibrosis (Cochrane Review)
www.cochrane.org/cochrane/revabstr/ab001506.htm
• nebulised hypertonic saline improves mucociliary clearance in CF

White D, Stiller K, Roney F (2000) The incidence and severity of symptoms of incontinence in adult CF patients. Physiother Theory Pract, 16, 35-42.
• excess coughing has led to over a third of females, and some males, developing stress incontinence

White D, Stiller K, Haensel N (2006) Adherence of adult cystic fibrosis patients with airway clearance and exercise regimens.
J Cyst Fibros, Aug 9
www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=16904388&query_hl=1&itool=pubmed_DocSum
• time-related factors are the most commonly reported reasons for low adherence

Wills PJ, Hall RL, Chan W (1997) Sodium chloride increases the ciliary transportability of CF and bronchiectasis sputum. J.Clin.Invest, 99, 9-13
• hypertonic saline can double mucus clearance in bronchiectasis and CF; manual techniques have shown improved pulmonary function


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