Diagnosing and managing cystic fibrosis

31st January 2018 by Louise Hudman

Diagnosing and managing cystic fibrosis

This is a new guideline from NICE on diagnosing and managing cystic fibrosis. Most of this guideline will be much more relevant to secondary care, so I’m just covering the list of possible presentations (as I’d forgotten quite a lot of these) and also some of the different mucolytic treatments (as I was not aware of them).

Presentations that should increase the suspicion of cystic fibrosis.

Positive infant blood spot test. It is worth noting that some patients can get to adulthood with atypical presentations of cystic fibrosis. Some patients will not be picked up with the infant blood spot test and some will not have been tested at all.

I haven’t listed all the possible presentations here, just the ones that we may be seeing…

  • Family history is positive.
  • Distal intestinal obstruction (beware this as a complication in patients too – this presents as central abdo pain, or RIF pain, often with a RIF mass).
  • Faltering growth or undernutrition (intestinal complications cause various issues including poor absorption of fat soluble vitamins like A, D, E and K).
  • Recurrent and chronic pulmonary disease (eg recurrent LRTI, persistent CXR changes or chronic productive cough).
  • Chronic sinus disease.
  • Obstructive azoospermia.

Mucoactive agents – things I didn’t know…

  • rhDNase – patients with CF get increased levels of DNA in their secretions, which is released after infections by dying inflammatory cells like neutrophils. This DNA increases the thickness of their secretions, which makes further infections more likely. DNase breaks up the DNA.
  • Mannitol dry powder for inhalation – the mannitol basically causes more water to be released into the secretions, so making them less sticky, which makes it easier for them to be expectorated.

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