Cystic fibrosis with pulmonary manifestation and bronchiectasis

What is the PDX when a patient is admitted with a Cystic fibrosis pulmonary manifestation and also has a worsening of their bronchiectasis? According to the coding clinic the complication should be sequenced as the principal diagnosis. Is bronchiectasis a complication of Cystic fibrosis and should bronchiectasis  always be sequenced as the principal diagnosis? In most cases I have seen, whenever the patient has CF exacerbation (pulmonary) the bronchiectasis will also exacerbate. Sometimes they might have a pneumonia also superimposed to the bronchiectasis.  It seems bronchiectasis is integral to CF. Most of the adult CF patient will have bronchiectasis. When bronchiectasis is coded as PDX it goes to the DRG 190 which is chronic obstructive pulmonary disease with MCC. I feel like 179 or 178 is the best DRG than 190 for a CF patient with pulmonary exacerbation and bronchiectasis because as the DRG (190) describes it is a respiratory infection and inflammation. The bronchiectasis is usually exacerbated by non-clearance of the mucus and colonization of organism such as PSA and MSSA. So I think coding CF with pulmonary manifestation with pseudomonas, MSSA as the PDX as the PDX and coding bronchiectasis without/with  exacerbation (CC) as the secondary diagnosis is more appropriate. What are your thoughts? How is this coded in your facility?

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