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Existing drug therapies

Table 2: Treatment for gastrointestinal complications of CF Organ

Manifestation Pancreas

Exocrine insufficiency: malabsorption, steatorrhoea

Management High fat diet

PERT and fat-soluble vitamins Fat absorption may be aided by alkaline environment (H2-blockers or proton pump inhibitors) Gastrostomy feeds

Acute pancreatitis (pancreatic sufficient patients)

Oesophagus Gastro-oesophageal reflux

Small bowel Distal Intestinal obstruction

syndrome (DIOS)

Coeliac disease (increased incidence in CF)


Fatty liver (usually asymptomatic) Cirrhosis

Hepatocellular failure a late manifestation

Oral pancreatin powder (anecdotal evidence only)

Proton pump inhibitors, prokinetic agents Surgery if refractory symptoms

Oral Gastrograffin or kleanprep

Review dose of, and adherence to, pancreatic enzyme replacement therapy, perform 3 day faecal fat collection Consider pro-kinetic agents

Severe acute cases, relieve with colonoscopy; laparotomy a last resort Gluten free diet, as for isolated celiac disease

Liver ultrasound at least every two years Ursodeoxycholic acid, taurine (seek specialist advice) Severe cases may need transplantation

Ibuprofen, which is commonly used in the US, is prescribed very infrequently in Europe because of concerns over side-effects and the requirement for strict monitoring and dose adjustment.

(iv) Pulmonary exacerbations: periods of increased symptoms, often accompanied by a fall in lung function, weight loss +/- systemic upset, are common.8

In early childhood, many of 16

with paediatric trials ongoing. This non-absorbable sugar alcohol creates an osmotic force in the airway lumen to

detected should be treated promptly; in particular, the success rates for pseudomonal eradication therapy are high. The optimal regimen for eradication is currently being explored in the UK TORPEDO trial (http://www.controlled- (iii) Established lung disease: once airway damage has become established, treatment is aimed at reducing the rate of progression. Mucolytics will be almost universally recommended as adjuncts to airway clearance. In addition to hypertonic saline and DNase, inhaled mannitol is now available for adult patients,4

rehydrate the airway surface liquid. Some patients respond to inhaled bronchodilators, although trials have shown that inhaled corticosteroids are not widely beneficial;5

they may be useful

in patients with an asthmatic phenotype. Chronic infection, most commonly with P. aeruginosa, will no longer be eradicable but suppressive therapies in the form of topical antibiotics will be used to try and limit bacterial numbers and thus the host inflammatory response.6 Nebulised colomycin has long been used in much of Europe, although, as it was introduced before the current rigor of randomised, controlled clinical trials, it does not have a clear evidence base. More recently, trials have confirmed efficacy of

these are likely triggered by viral infections, although the exact cause is unknown. It is thought that, in patients with chronic infections, PEx are not usually due to acquisition of new organisms and an increase in numbers of the chronic bacteria is rarely seen; they may be caused by changes in bacterial behaviour. Despite this, patients do respond to high-dose antibiotics administered either orally or intravenously; there is little correlation between clinical response and in vitro sensitivities, so the latter should be used as a guide only. Treatment will usually be for periods of at least two weeks, although the optimal duration is unknown. The frequency and severity of PEx is linked to general rate of lung function decline, so their early detection and treatment are important. (v) Allergic bronchopulmonary aspergillosis: infection with Aspergillus fumigatus is common in CF and a number of patients will go on to develop a syndrome of increased symptoms (breathlessness and wheeze), a drop in lung function and evidence of IgE- mediated sensitivity to the fungus (raised total and specific IgE +/or positive skin prick tests). Treatment is with high-dose systemic corticosteroids (oral or IV) usually together with antifungal agents and may need to be of a prolonged duration. (vi) Advanced and end-stage lung disease: once lung damage advances, patients are at increased risk of pulmonary complications such as massive

nebulised tobramycin, aztreonam and dry powder formulations (tobramycin and colomycin). Infection with atypical mycobacteria is increasing in frequency and often requires treatment with multiple agents for prolonged periods. Mycobacterium abscessus, in particular, has been associated with a poorer prognosis. With regard to inflammation, azithromycin, a macrolide antibiotic with anti-inflammatory effects, has been shown to improve lung function in this group of patients when given once daily, although the exact mechanism of action is unclear.7

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