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Last updated on 14 Mar 2016 at 5:35 PM (Resp03) Chronic sputum production A 25 year old NZ European woman, who has recently moved into the area, presents to your GP clinic with an increase of her cough and sputum production and recurrent small haemoptyses (about a teaspoon in volume). She has a history of cough and purulent sputum production since early childhood. Applied Science for Medicine Anatomy of respiratory tract; particularly airways Microanatomy of the lung including production of airway lining fluid and mucus layer, muco-ciliary clearance; role of Clara cells, goblet cells and mucus glands Radiology of thorax, including high-resolution CT scan Lung defence mechanisms including cellular and immunologic mechanisms; mucociliary clearance Consequences of impairment of lung defence mechanisms Ion and water transport; relevance to disease and pharmacology Microbiology of chronic lung infection; biofilm formation Pathology of bronchiectasis Pulmonary and non-pulmonary manifestations of cystic fibrosis Inheritance of disease - phenotype-genotype correlations Pharmacology of macrolides, anti-microbial agents (particularly anti-pseudomonal antibiotics); mechanisms of drug action and potential drug interactions Clinical and Communication Skills History from a patient with chronic sputum production Examination of the respiratory system Assess co-morbidities e.g. upper respiratory tract disease Perform and interpret spirometry and arterial blood gas Interpret results of sputum culture (past and present), chest X-ray Indications for: high resolution CT, detailed pulmonary function tests, bronchoscopy, sweat chloride test, serum immunoglobulin quantitation, genotyping Differential diagnosis of chronic sputum production Outline the management of bronchiectasis: physiotherapy, pharmacological and surgical Complications of bronchiectasis and cystic fibrosis Prognosis of bronchiectasis and cystic fibrosis Outline the management of cystic fibrosis including pathophysiologic rationale Managing chronic disease - patient education, support, pharmaceutical management, involving the multidisciplinary team including palliative care Transition from paediatric to adult care Personal and Professional Skills Be aware of issues around multiple therapies (complexity for patient and doctor, potential for error, effect on adherence, adverse effects, increased potential for drug interactions) Managing professional/personal boundary issues with long-term patients Death and dying in young patients Hauora Mĕori Appropriate engagement and consultation with whĕnau Awareness of differing risk profiles for Mĕori compared with non-Mĕori Understanding of inequities in access to health services and quality of medical care for Mĕori, and application of this to clinical decision-making and management Population Health Social and economic determinants of health Adult health consequences of paediatric disease Epidemiology of bronchiectasis and cystic fibrosis National newborn screening for genetic disease Patient advocacy Role of community organisations Conditions to be considered relating to this scenario bronchiectasis, chronic obstructive pulmonary disease, Common cystic fibrosis allergic bronchopulmonary aspergillosis, ciliary Less common but dyskinesia syndrome, immunoglobulin deficiency 'important not to miss'
