Surveillance & Prevention

  • You should begin to see a pulmonologist yearly starting at diagnosis, and begin pulmonary function testing as soon after diagnosis as possible. Testing should include measurement of forced vital capacity (or “FVC;” the largest breath that a person can fully and forcefully breathe out). Starting these tests early will allow children to become familiar with the equipment and the team and to assess baseline respiratory function
  • When you are unable to walk without support, a scheduled pulmonary assessment should occur at least every six months. The assessment should include pulmonary function testing with measurements of FVC, maximum inspiratory (MIP – how strongly you can breath in) and expiratory pressure levels (MEP – how strongly you can breathe out), and peak cough flow (PCF – the strength of your cough). These values will be trended over time by your pulmonary team
  • In addition, the assessment should include pulse oximetry (SpO2- measures the oxygen in your blood while you are awake) and when available, end-tidal or transcutaneous measurement of blood carbon dioxide levels (PetCO2/PtcCO2 – measures the levels of carbon dioxide in your blood while you are awake)
  • A sleep study may be needed if there are signs of restless sleep, morning headaches or extreme tiredness during the day. This study assesses breathing and measures oxygen levels and blood carbon dioxide levels while you are asleep
  • Pneumococcal vaccination (to prevent pneumonia) and yearly influenza vaccinations (flu injections; avoid live virus influenza nasal spray) will help prevent episodes of severe flu and pneumonia


  • Experiencing a prolonged illness with only minor upper respiratory infections
  • More tired than usual or sleepy for no reason during the day
  • Short of breath, seeming as if you cannot catch breath or have difficulty finishing sentences
  • Having headaches most mornings or all the time
  • Having trouble sleeping, waking up a lot, having trouble waking up, or having nightmares
  • Waking up trying to catch your breath or feeling your heart pounding
  • Having trouble paying attention during the day at home or at school
Figure 10. Surveillance, Assessment and Management of the Pulmonary Team