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Cystic fibrosis

Mucoviscidosis · Cystická fibróza

What it is

A CFTR chloride-channel fault makes every secretion thick and sticky.

Symptoms & signs

  • Recurrent lung infections
  • salty sweat
  • poor weight gain
  • greasy stools
  • infertility in men
  • clubbing

🚩 Get help if…

  • call 112 / 911 now Coughing up more than a spoonful of blood
  • call 112 / 911 now Sudden chest pain with breathlessness
  • call 112 / 911 now Swollen tummy, green vomit and no stools
  • see a doctor soon More phlegm, fever or weight loss

Where it strikes

Medical treatment

  • CFTR modulators (elexacaftor-tezacaftor-ivacaftor) transformed the disease
  • airway clearance
  • dornase alfa
  • pancreatic enzymes
  • nebulised antibiotics

Natural & lifestyle

  • High-calorie high-salt diet
  • daily airway clearance and exercise
  • fat-soluble vitamins
  • infection segregation

Emerging & experimental

  • Gene therapy and mRNA CFTR delivery
  • CRISPR correction
  • therapies for non-modulator mutations

Alternative, off-label & anecdotal

Approaches used outside the guidelines, with what their supporters report and what testing has found so far.

  • Curcumin — corrected the defect in mice; human trials failed not confirmed in trials
  • Probiotics — small trials show fewer flare-ups and less gut inflammation early / small studies
  • Inhaled glutathione — a controlled trial found no benefit not confirmed in trials
  • Salt-water and sea-air holidays — patients' experience; untested anecdotal / traditional

ICD codes