CF week

Anatomy of the LRT  Airway branchings & LMB vs RMB  Name levels from trachea to alveoli  Changes as we descend resp tract?  Pleural cavity  Lungs – lobes and fissures  Circulation  Innervation

Respiratory epithelium

Bronchus

Bronchiole

Terminal bronchiole

Respiratory bronchiole

Alveoli

Physiology - mechanics  Pleural pressure  Muscles and movements in inspiration  Muscles and movements in expiration  Passive vs active expiration  Forces keeping alveoli open vs closed

Physiology  Ventilation  Pulmonary ventilation vs alveolar  What causes dead space?

Physiology  Diffusion  What are the layers of the blood-air-barrier?  What effects the rate of diffusion?

Physiology  Perfusion  Normal V/Q ratio  Hypoxic pulmonary vasoconstriction

Pathology of obstruction  Tubes – muscular vs non-muscular  Non-mechanical  Mechanical - extramural, intramural and intraluminal  Examples  Consequences

CF  Type of inheritance?  Incidence

Sequelae Lung  Abnormal mucous, less, thicker  Infection/ inflammation  Bronchiectasis Pancreas  Pancreatic obstruction leads to… Sweat gland  Decreased reabsorption of NaCl.

Pancreatic pathology

Sweat gland

Other abnormalities  Bilateral absence of ductus deferens  Meconium ileus  Liver pathology  Males generally infertile, woman are not.

Diagnosis  Sweat test and DNA test for CFTR mutation  6 classes of mutation

Management Can you list 5 management options?

Management Chest physiotherapy Chest physiotherapy Postural drainage and manual techniques Breathing & airway clearance +/- PEP devices Exercise therapy Medications Medications Bronchodilators (preventers and relievers) Anti-inflammatory therapy Mucolytics & pulmozyme (rhDNase) IV antibiotics (infective exacerbations) IV antibiotics (infective exacerbations) Oxygen therapy (infective exacerbations, or end stage) Oxygen therapy (infective exacerbations, or end stage) Nutritional support (+/- enzymes) Nutritional support (+/- enzymes) Transplantation (end stage) Transplantation (end stage) Potential gene therapy Potential gene therapy - aim to restore CTFR function

Psychosocial/burden of CF  Prognosis  Time consuming, life altering illness.  Costly  Good opportunity to learn about support and respite groups

Surface anatomy 6,8 and 10 for lung.8, 10 and 12 for pleurae.