Cystic Fibrosis
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Clinically Relevant Anatomy
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Mechanism of Injury / Pathological Process
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Cystic fibrosis (CF) is the most frequent cause of suppurative lung disease in the younger Caucasian population. A depleted volume of the airway surface liquid (ASL) layer in the respiratory system leads to abnormal mucociliary clearance[1]. A chronic cycle of infection and inflammation results in progressive suppurative bronchiectasis and lung damage.
Clinical Presentation[edit | edit source]
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Diagnostic Procedures[edit | edit source]
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Outcome Measures[edit | edit source]
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Management / Interventions
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Although airway clearance continues to be an integral part of care, physical exercise, postural care and the need to address the unique complications which emerge as a result of improved longevity have significantly changed the nature of physiotherapy in CF.
Airway Clearance[edit | edit source]
Despite several comparative studies of airway clearance techniques in CF, there continues to be a lack of robust evidence to support the long term benefits of intervention[2]. A lack of evidence should however not necessarily be associated with a lack of benefit. Clinicians generally agree that airway clearance is likely to be beneficial in those with established respiratory disease[3].
The physiological principles of airway clearance techniques have begun to be investigated in greater detail[4]. Systematic reviews comparing the various airway clearance modalities have not identified a single technique as being superior to others[5][6]. Some studies report a trend for patients to prefer therapies which are self administered,ref>Morrison L, Agnew J. Oscillating devices for airway clearance in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2009, Issue 1. Art. No.: CD006842</ref>[7]. The performance of routine daily airway clearance require considerable time and can impose a significant burden on patients and families. Adherence to routine airway clearance continues to be reported as poor[8][9]. The use of non-invasive ventilation as an adjunct to airway clearance may be useful in improving gas exchange during sleep in those with moderate to severe disease but its impact on disease progression remains unclear[10].
Differential Diagnosis
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Key Evidence[edit | edit source]
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Resources
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Annual Evidence Update on Cystic Fibrosis – Physiotherapy, S Ammani Prasad, Cystic Fibrosis Unit, Great Ormond Street Hospital for Children NHS Trust, for NHS Evidence, 2009.
Physiotherapy National Standards of Care for people with Cystic Fibrosis, Association of Chartered Physiotherapists in Cystic Fibrosis, UK, 2009.
Case Studies[edit | edit source]
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Recent Related Research (from Pubmed)[edit | edit source]
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References[edit | edit source]
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- ↑ Mall M, Boucher RC Pathogenesis of pulmonary disease in cystic fibrosis. In: Bush A, Alton EWF, Davies JC, Griesenback U, Jaffe A (Eds) Cystic Fibrosis in the 21st century Progress in Respiratory Research Karger, Basel 2006; 34:116-121
- ↑ van der Schans CP, Prasad A, Main E. Chest physiotherapy compared to no chest physiotherapy for cystic fibrosis. Cochrane Database of Systematic Reviews 2000, Issue 2. Art. No.: CD001401. DOI: 10.1002/14651858.CD001401
- ↑ Flume PA, Robinson K, O’Sullivan B, Finder JD< Vender FL, Willey-Courand D, White TB, Marshall BC & The Clinical Practice Guidelines Committee. Cystic Fibrosis Pulmonary Guidelines: Airway Clearance therapies. Resp Care 2009;54:522-537
- ↑ McCarren B, Alison JA. Physiological effects of vibration in subjects with cystic fibrosis. European Respiratory Journal 2006; 27:1204-1209
- ↑ Main E, Prasad SA, van der Schans C. Conventional chest physiotherapy compared to other airway clearance techniques for cystic fibrosis. The Cochrane Database of Systematic Reviews 2005, Issue 1. Art. No.: CD002011
- ↑ Elkins M, Jones A, van der Schans CP. Positive expiratory pressure physiotherapy for airway clearance in people with cystic fibrosis. Cochrane Database of Systematic Reviews 2006, Issue 2. Art. No.: CD003147
- ↑ Pryor JA. A comparison of five airway clearance techniques in the treatment of people with cystic fibrosis. Thesis submitted for the degree of Doctor of Philosophy, Imperial College London 2005
- ↑ Bucks RS, Hawkins K, Skinner TC, Horn S, Seddon P, Horne R. Adherence to Treatment in Adolescents with Cystic Fibrosis: The Role of Illness Perceptions and Treatment Beliefs. J Pediatr Psychol 2009 [Epub ahead of print]
- ↑ Arias Llorente RP, Bousoño García C, Díaz Martín JJ. Treatment compliance in children and adults with cystic fibrosis. J Cyst Fibros 2008;7:359-67
- ↑ Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev 2007;4:CD002769
