Cystic fibrosis

Question 1

what organs are affected

Answer 1

lung, pancreas, liver, intestine and reproductive organs

Question 2

clinical signs

Answer 2

pulmonary disease with recurrent infections
production of copious amounts of viscous sputum
malabsorption due to pancreatic insufficiency

Question 3

aim of treatment

Answer 3

prevent and manage/optimise lung function
loosening and removing thick sticky mucus from the lungs
preventing or treating intestinal obstruction
providing sufficient nutrition and hydration

Question 4

key predictor of life expectancy of CF patients

Answer 4

lung function

Question 5

drug

Answer 5

evidence of lung disease: dornase alfa
dornase alfa and hypertonic sodium chloride or hypertonic sodium chloride alone

mannitol dry powder for inhalation is recommended when dories alfa is unsuitable or lung function is rapidly declining or other osmotic drugs not appropriate

Question 6

pulmonary infection treatment

Answer 6

Patients not taking prophylaxis and have a new Staphyl aureus infection can be given an oral anti-Staph. aureus antibacterial, if they are clinically well.
If they are clinically unwell and have pulmonary disease, oral or IV (depending on infection severity) broad-spectrum antibacterials with activity against Staph. aureus should be given (consult local protocol).

A long-term antibacterial should be considered to suppress chronic Staph. aureus respiratory infections in patients whose pulmonary disease is stable. In patients with chronic Staph. aureus respiratory infections who become clinically unwell with pulmonary disease, oral or intravenous (depending on infection severity) broad-spectrum antibacterials with activity against Staph. aureus should be given. In those patients with new evidence of meticillin-resistant Staphylococcus aureus (MRSA) respiratory infection (with or without pulmonary exacerbation), specialist microbiological advice should be sought.

Antibacterials should not be routinely used to suppress chronic MRSA in patients with stable pulmonary disease.

If a patient with chronic MRSA respiratory infection becomes unwell with a pulmonary exacerbation or decline in pulmonary function, specialist microbiological advice should be sought

If a patient with cystic fibrosis develops a new Pseudomonas aeruginosa infection, eradication therapy with a course of oral antibacterial should be started (by intravenous injection, if they are clinically unwell), in combination with an inhaled antibacterial. An extended course of oral and inhaled antibacterial should follow (consult local protocol).

If eradication therapy is not successful, sustained treatment with an inhaled antibacterial should be offered. Nebulised colistimethate sodium should be considered as first-line treatment .

Question 7

Nutrition and exocrine pancreatic insufficiency

Answer 7

pancreatin for exocrine pancreatic insufficiency

acid suppressing drug such as H2 receptor antagonist or PPI

A short-term trial of an appetite stimulant (for example up to 3 months) [unlicensed indication] can be considered in adult patients if attempts to increase calorie intake are not effective.

Question 8

liver disease

Answer 8

If liver function blood tests are abnormal in patients with cystic fibrosis, ursodeoxycholic acid [unlicensed indication] can be given until liver function is restored.