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Mr Binley Case Study

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Module Two
Question 2
Mr Bingley is a 13-year-old boy with cystic fibrosis. He presents to his GP complaining of cough, which is productive of green sputum. He appears to be short of breath, and can be heard wheezing on physical examination. He also appears to be underweight. Describe the pathophysiology of cystic fibrous. In you answer explain why Mr Bingley appears underweight and has problems with his airways.

Cystic fibrosis is a genetic disorder that affects the endocrine system, renal system, gastro-intestinal system, and reproductive system, but it’s effects are felt mostly in the lungs where chronic infection leads to respiratory failure and eventually death (Craft, Gordon, and Tiziani, 2010). The disease is characterized by it’s viscous mucus, and cycle of …show more content…

The airways of patients with cystic fibrosis are eventually obstructed by mucus and hyper-inflated; lung function becomes progressively worse as the airway walls are repeatedly damaged in a cycle of inflammation and infection (Cohen and Prince, 2012). For approximately 90% of patients, respiratory failure will eventually become fatal unless they receive a lung transplant (Davies, Ebdon, and Orchard, 2014). Common symptoms of cystic fibrosis are coughing, diarrhoea, and malnutrition (Haack, Arago, and Novaes, 2013). Patients often experience tachypneoa, wheezing, chronic rhinitis (inflammation of the nose), nasal polyps, haemoptysis (coughing blood) and problems associated with lung inflation such as atelectasis, hyperinflation, or pneumothorax (Lumbamba et Al., 2012). Cystic fibrosis sufferers may also have a barrel chest, club fingers and toes, pulmonary hypertension and low tolerance for physical exercise due to their damaged respiratory system (Lumbamba et Al., 2012). The lack of normal CFTR creates hyper viscous secretions with

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