Breathing problems

Try and keep a note of, or remember what the latest tests of breathing (e.g. forced vital capacity, FVC) were. This information can be useful for the doctors assessing your son if he does become ill acutely.

The main risks with breathing problems come when FVC and coughing strength have reduced:

  • Help with clearing the chest may be needed;
  • It may be important to help with coughing;
  • Antibiotics may be needed;
  • Sometimes it may be necessary to give support with a ventilator;
  • Risk of the breathing muscles needing extra support during an infection can be high in those with borderline respiratory function. Care in the use of opiates and other sedating medication is essential, as is care in the use of oxygen without ventilation due to the risk of rising carbon dioxide in people with compromised breathing muscle strength;
  • If nocturnal ventilation is already used, then access to the ventilator is essential during any acute event or intervention. For those who are already ventilated, the team involved with the respiratory care should be involved as soon as possible.

If you have a ventilator (or similar equipment) it is a good idea to bring it with you to the hospital.

Information based on consensus statement (published in January 2010)