1. CF week

2. 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

3. Respiratory epithelium

4. Bronchus

5. Bronchiole

6. Terminal bronchiole

7. Respiratory bronchiole

8. Alveoli

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

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

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

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

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

14. CF  Type of inheritance?  Incidence

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

16. Pancreatic pathology

17. Sweat gland

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

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

20. Management Can you list 5 management options?

21. 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

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

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