Cystic Fibrosis

Question 1

Describe the nature of CF

Answer 1

GENETIC CONDITION

LIFE-LIMITING

Question 2

Relate the CFTR channel to CF

Answer 2

MUTATION of ΔF 5O8 gene encoding for CFTR channel (Cl- movement)
> faulty channel
> reduced Cl- absorption
> reduced Na+ & water absorption
DEHYDRATION

Question 3

Aims of treatment/management

Answer 3

Prevent long term complications
DIABETES
CHRONIC INFECTION
Maintain BMI, support growth & development

Question 4

Clinical features of CF

Answer 4

THICK, STICKY MUCUS in lungs & pancreas
Salty skin (high [Cl-] in sweat)

Outcomes:

PANCREATIC INSUFFICIENCY
Reduced lung function (FEV1)

COMPLICATIONS

Question 5

Explain how CF causes pancreatic insufficiency

Answer 5

Pancreatic duct blocked by thick mucus
Cannot release digestive enzymes into SI
Reduced food digestion

Question 6

Therapeutic treatment for babies with CF

Answer 6

Nutritional therapy (high cal drinks)
Multivits (fat soluble)
Pancreatic enzyme supplements (Creon)
Antibiotics

Question 7

Monitoring in babies includes:

Answer 7

Head circumference, height, weight
Pulmonary status
Oropharyngeal cultures (bacteria)
Glucose tolerance tests
Bone density

Question 8

Creon capsules:

Answer 8

Used for pancreatic insufficiency

PANCREATIC ENZYMES

Question 9

DORNASE ALFA:

• Effects
• Mechanism
• Administration

Answer 9

• reduces mucus viscosity, expectoration, improves FEV1 (lung function)
• deoxyribonuclease I breaks down DNA fragments from neutrophil degradation
• INHALATION - jet nebuliser

Question 10

CF babies: G+ bacteria eradication

SPECIFICALLY:

Answer 10

S. aureus

Question 11

CF patients (older): G- bacteria eradication
SPECIFICALLY:

Answer 11

P .aeruginosa

Question 12

AZITHROMYCIN

• Dose
• Actions

Answer 12

500mg 3x weekly
Treat INFLAMMATION due to CHRONIC infection
improve FEV1

Question 13

OGTT

• abnormal levels
• indication
• abbreviation

Answer 13

> 11mmol/L
DIABETES
Oral Glucose Tolerance Test

Question 14

HbA1c

• abnormal levels
• sign of:
• pharmacological indication

Answer 14

> 6.5%
DIABETES
Glycosylated Hb due to XS sugar