CF weekCF week

Anatomy of the LRTAnatomy of the LRT Airway branchings & LMB vs RMBAirway branchings & LMB vs RMB

Name levels from trachea to alveoliName levels from trachea to alveoli Changes as we descend resp tract?Changes as we descend resp tract?

Pleural cavityPleural cavity Lungs – lobes and fissuresLungs – lobes and fissures CirculationCirculation InnervationInnervation

Respiratory epitheliumRespiratory epithelium

BronchusBronchus

BronchioleBronchiole

Terminal bronchioleTerminal bronchiole

Respiratory bronchioleRespiratory bronchiole

AlveoliAlveoli

Physiology - mechanicsPhysiology - mechanics Pleural pressurePleural pressure Muscles and movements in inspirationMuscles and movements in inspiration Muscles and movements in expirationMuscles and movements in expiration

Passive vs active expirationPassive vs active expiration Forces keeping alveoli open vs closedForces keeping alveoli open vs closed

PhysiologyPhysiology VentilationVentilation

Pulmonary ventilation vs alveolarPulmonary ventilation vs alveolar What causes dead space?What causes dead space?

PhysiologyPhysiology Diffusion Diffusion

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

PhysiologyPhysiology PerfusionPerfusion

Normal V/Q ratioNormal V/Q ratio Hypoxic pulmonary vasoconstrictionHypoxic pulmonary vasoconstriction

Pathology of obstructionPathology of obstruction

Tubes – muscular vs non-muscularTubes – muscular vs non-muscular Non-mechanicalNon-mechanical Mechanical - extramural, intramural and Mechanical - extramural, intramural and

intraluminalintraluminal ExamplesExamples ConsequencesConsequences

CF CF Type of inheritance?Type of inheritance? IncidenceIncidence

SequelaeSequelae

LungLung Abnormal mucous, less, thickerAbnormal mucous, less, thicker Infection/ inflammationInfection/ inflammation BronchiectasisBronchiectasis

PancreasPancreas Pancreatic obstruction leads to…Pancreatic obstruction leads to…

Sweat glandSweat gland Decreased reabsorption of NaCl.Decreased reabsorption of NaCl.

Pancreatic pathologyPancreatic pathology

Sweat glandSweat gland

Other abnormalitiesOther abnormalities

Bilateral absence of ductus deferensBilateral absence of ductus deferens Meconium ileusMeconium ileus Liver pathologyLiver pathology Males generally infertile, woman are not.Males generally infertile, woman are not.

DiagnosisDiagnosis

Sweat test and DNA test for CFTR Sweat test and DNA test for CFTR mutationmutation

6 classes of mutation6 classes of mutation

ManagementManagement

Can you list 5 management options?Can you list 5 management options?

ManagementManagement• • Chest physiotherapy Chest physiotherapy

Postural drainage and manual techniquesPostural drainage and manual techniquesBreathing & airway clearance +/- PEP devicesBreathing & airway clearance +/- PEP devicesExercise therapyExercise therapy

• • MedicationsMedicationsBronchodilators (preventers and relievers)Bronchodilators (preventers and relievers)Anti-inflammatory therapyAnti-inflammatory therapyMucolytics & pulmozyme (rhDNase)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 therapyPotential gene therapy- aim to restore CTFR function- aim to restore CTFR function

Psychosocial/burden of Psychosocial/burden of CFCF

PrognosisPrognosis

Time consuming, life altering illness.Time consuming, life altering illness. CostlyCostly Good opportunity to learn about support Good opportunity to learn about support

and respite groupsand respite groups

Surface anatomySurface anatomy

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