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TOF cough/chronic cough

The cough itself is typically a hoarse brassy ‘seal-like’ cough, due to TM. Some adults have persistent coughing; for others it arises post aspiration or infection and then subsides. Causes of persistent cough include airway reflux, excess or difficult-to-clear secretions, inflamed airways due to TM.

The cough can cause embarrassment, staring and comments in public due to its unusual nature – the TOF charity has produced explanatory badges as this is such a universal experience amongst those with OA/TOF.

It can also affect sleep, damage intercostal muscles, cause airway pain and fatigue, trigger vomiting and facial petechial bleeding due to raised pressure on the small vessels.


Aimed at improving secretion clearance

  • Carbocysteine can thin sputum viscosity, aiding clearance.
  • Hypertonic saline nebuliser/device to thin sputum viscosity. (10)
  • Chest physiotherapy can also help facilitate secretion drainage.

Aimed at treating chronic cough

This should address the underlying cause, usually airway reflux. This may mean investigations such as manometry, endoscopy, pH studies to assess this and look for any undiagnosed anatomical anomalies:

  • Proton pump inhibitors once or twice a day can suppress acid reflux that triggers coughing.
  • Pro-motility antibiotics such as azithromycin can improve oesophageal motility, improving all reflux and oesophageal content stasis. These have been very effective in adults born with OA/TOF treated by Professor Morice, an international (airway reflux) cough expert physician at Hull Teaching Hospitals, UK.
  • Metoclopromide or domperidone can also improve oesophageal motility and reflux, and in turn cough in this specific patient subgroup with GORD cough.(6)
  • Bronchodilator inhalers are often ineffective in TOF cough, and may aggravate TM. (6)
  • Steroid inhalers may help TOF cough in the context of asthma, or improve tracheal inflammation, but may not be successful in many. (11)
  • Rarely, severe cough may require more aggressive intervention if airway reflux is very severe, such as fundoplication, tube feeding, other surgical approaches due to risk to the lungs from aspiration.
  1. tbc