New NICE guideline on cystic fibrosis

NICE has published its first guideline on the diagnosis and management of cystic fibrosis (NG78). The guidance provides recommendations on monitoring the condition and managing complications, including treating the most common infections.

An assessment for cystic fibrosis, including a sweat test for children and young people or gene testing in adults, should be carried out in all those with a family history, congenital intestinal atresia, meconium ileus, symptoms of distal intestinal obstruction syndrome, faltering growth, undernutrition or malabsorption, chronic pulmonary disease, chronic sinus disease, obstructive azoospermia, pancreatitis, rectal prolapse or pseudo-Bartter syndrome. Patients should be referred to a specialist cystic fibrosis centre if they have a positive or equivocal sweat test result, one or more cystic fibrosis mutations on gene testing, or if their assessment suggests cystic fibrosis.

Patients with cystic fibrosis should be cared for by a specialist cystic fibrosis multidisciplinary team (which includes specialist paediatricians, adult physicians, nurses, physiotherapists, dietitians, pharmacists and clinical psychologists) based at a specialist cystic fibrosis centre.

Patients may need to travel long distances to these specialist centres, and therefore using phone or video messaging (telemedicine) or home visits for routine monitoring should be considered. Arrangements can also be made for patients to have intravenous antibiotic therapy at home, if appropriate.

NICE points out that people with cystic fibrosis are at risk of many complications, including vitamin deficiencies, distal intestinal obstruction syndrome, reduced fertility, chronic liver disease, arthralgia, airway complications, urinary incontinence, diabetes and osteoporosis. Routine monitoring and annual assessments (including pulmonary monitoring, nutritional status, liver function tests and glucose testing) are therefore crucial to reduce complications of the condition.

Patients who have clinical evidence of lung disease or their carers should be offered training in airway clearance techniques, as well as being prescribed a mucoactive agent, the first choice being dornase alfa (recombinant human deoxyribonuclease).

The NICE guidance offers detailed advice on the prophylaxis and management of pulmonary infection in patients with cystic fibrosis, with individual antimicrobial recommendations for different infective organisms, including Staphylococcus aureus, Pseudomonas aeruginosa, Burkholderia cepacia complex, Haemophilus influenzae, non-tuberculous mycobacteria and Aspergillus fumigatus complex.

NICE emphasises that it is important that people with cystic fibrosis and all involved in their care are informed of the risk of cross-infection and how to avoid it. Specialist clinics should be organised to prevent cross-infection, and people with transmissible or chronic Pseudomonas aeruginosa or Burkholderia cepacia complex infections should be seen in separate clinics.

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