Introduction Cystic fibrosis (CF) is the most common life-limiting genetic disease in the Caucasian population and is also found in other ethnic groups. It is a multisystem disorder, although the main cause of morbidity and mortality is respiratory failure. The median life expectancy has increased significantly over the last two decades and is currently in the mid-thirties. A factor in the improvement of life expectancy is an increase in the range and intensity of treatments including nutritional support, physiotherapy, antibiotics, and other medications (Daniels,2010). Over the last few years, new physiotherapeutic interventions in CF have moved away from stand-alone therapy to a patient-tailored, individualized treatment regimen, consisting of inhalation therapy, airway clearance, exercise and education about the disease and treatment (Van Ginderdeuren et al.,2008). Definition Cystic fibrosis (CF) is a disease of abnormal ion transport. Specifically, abnormalities in the expression and function of the cystic fibrosis transmembrane conductance regulator (CFTR) result in abnormal salt and water transport across epithelial surfaces in the gastrointestinal and hepatobiliary systems, respiratory tract, reproductive system, and sweat glands(Allen et al.,2010). Manifestations CF affects the mucus production of exocrine glands, which become thick and obstruct the channels in which it is secreted. This manifestation can occur in numerous epithelial cells including sweat, pancreatic and bile ducts, airways, intestinal and deferent vessels (Maiworm et al.,2011). Although CF is characterized by disease in many organs, respiratory disease is currently the leading cause of morbidity and mortality. In the CF lung, airway obstruction is a common finding. Both pulmonary function tests and chest X-ray computed tomography (CT) have revealed evidence of air trapping and impaired airway function early in the course of the disease. With disease progression, reduced airflow rates become almost universal. These abnormalities in airway function plus the defects in airway epithelium and submucosal glands have focused attention on airways in investigating CF pathogenesis (Meyerholz et al.,2010). The primary clinical manifestations usually occur in the lungs include cough, excess sputum, shortness of breath and respiratory muscle fatigue, resulting in progressive lung dam-age and eventual death from respiratory failure(Maiworm et al.,2011). Cystic fibrosis-related symptoms appear throughout life, with great overlap and variability of symptoms and timing from patient to patient. Patients with CF have common signs and symptoms like; family history of cystic fibrosis, salty-tasting skin, clubbing of fingers and toes, Cough with sputum production, hypochloraemic metabolic alkalosis, and mucoid Pseudomonas aeruginosa isolated from airway secretions(O’Sullivan & Freedman,2009). Hemoptysis is common in patients with CF; it was reported that 9.1% of patients had hemoptysis in a 5-year period. The bleeding, most commonly, is scant to moderate, but massive, life-threatening bleeding can occur. Approximately 4.1% of all patients with CF will suffer massive hemoptysis during their lifetime, and the average annual incidence is less that 1%. Approximately 3.4% of individuals with CF will experience a pneumothorax during their lifetime. Both of these complications occur more commonly in older patients with advanced disease (Flume et al.,2010). Musculoskeletal and postural disorders, which are common in people with CF are secondary to pulmonary disease, with a complex relationship between posture and respiration. Thoracic kyphosis and vertebral wedging are prevalent in people with CF and are related to worsening lung function and clinical symptoms. There is also evidence of decreased thoracic mobility and muscle weakness. These changes in soft tissue and spinal geometry are likely to be attributed to an increased work of breathing and the hunched posture assumed during excessive coughing (Wedzicha et al.,2009). References 1- Allen J.L., Penitch H.B., Rubenstien R.C. “Cystic Fibrosis” Lung Biology in Health and Disease.Volume 242. Chapter 2. Pages 11-23.2010. 2- Daniels T. “ Physiotherapeutic management strategies for the treatment of cystic fibrosis in adults” Journal of Multidisciplinary Healthcare. Volume 3. Pages 201- 212. 2010. 3- FlumeP.A., Mogayzel P.J.,et al “Cystic Fibrosis Pulmonary Guidelines Pulmonary Complications: Hemoptysis and Pneumothorax” American Journal of Respiratory and Critical Care Medicine.Volume 182. Pages 298-306. 2010. 4- Maiworm A.I., Monteiro M.B. et al. ” Cystic fibrosis and the relevance of the whole-body vibration exercises in oscillating platforms: a short review” Health. Volume 3.Number 3. Pages 656-662. 2011. 5- Meyerholz D.K., Stoltz D.A.,et al“Loss of Cystic Fibrosis Transmembrane Conductance Regulator Function Produces Abnormalities in Tracheal Development in Neonatal Pigs and Young Children” American Journal of Respiratory and Critical Care Medicine. Volume 182. Pages 1251-1261.2010. 6- O’Sullivan B.P. & Freedman S.D.” Cystic Fibrosis” The Lancet. Volume 373. Pages 1891-1904.2009. 7- Van Ginderdeuren F., Verbanck S., et al “Chest Physiotherapy in Cystic Fibrosis: Short-Term Effects of Autogenic Drainage Preceded by Wet Inhalation of Saline versus Autogenic Drainage Preceded by Intrapulmonary Percussive Ventilation with Saline” Respiration. Volume 79. Pages 175-180.2008. 8- Wedzicha J.A., Johnston S.L.,et al “Guidelines for the physiotherapy management of the adult, medical, spontaneously breathing patient” Journal of the British Thoracic Society. Volume 64. Pages 18-25. 2008.
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