Respiratory assessment

Ideally consultation by a respiratory physician should be initiated soon after diagnosis.  

Symptoms or signs of respiratory insufficiency (including symptoms of nocturnal hypoventilation) should be checked at each visit (Andersen and others 2007). Serial monitoring is important... trends may be more useful than absolute measurements (Murray and McKenzie 2007).

Miller and others 2009a

Nocturnal oximetry may be considered to detect hypoventilation (regardless of the FVC) (Level C). 

Supine FVC and MIP may be considered useful in routine respiratory monitoring, in addition to the erect FVC (Level C). 

SNP may be considered to detect hypercapnia and nocturnal hypoxemia (Level C).