Cystic Fibrosis

Question 1

What are the common examination findings?

Answer 1

Inspection - Young patient with a short stature and decreased muscle bulk. o2, creon around the bed, indwelling venous catheter.

May have a cough

Hands - Clubbing

Chest:

Inspiratory crackles

Question 2

What are the investigations?

Answer 2

By the bed - History, obs, peak flow, spirometry and sputum. Also want to do an abdominal exam, cardiovascular exam,

Bloods - FBC, CRP, U/E for salt loss, LFTS, fecal elastase for malabsorption

Other - CXR, ECG,ABG

Question 3

What are the key investigation findings?

Answer 3

CXR - Signs of chronic lung disease so increased lung markings

May also show signs of bronchiectasis - thickend bronchi

Question 4

What is the treatment?

Answer 4

Conservative - Explain what it is and the need for MDT management - Physios, OT, GP, Consultant, genetic counselling

Exercise, good diet, postural drainage

Discuss the other effects so pancreas (diarrhoea, F)TT infertility

Medical - Chloride sweat test, Abx for infections and yearly immunisations, pancreatic replacement, bronchodilators, mucolytics

Surgical - Lung transplant

Question 5

What are the causes

Answer 5

Chromosome 7 recessive gene

1 in 25 are carriers

Average life expectancy of 38