Health Information Management

Q&A: Should cystic fibrosis always be first-listed diagnosis

CDI Strategies, October 25, 2012

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Q: I have heard that there is an AHA Coding Clinic for ICD-9-CM, which states that the exacerbation of cystic fibrosis (CF) should always be listed first. Should we be querying for the specific pulmonary exacerbation of the cystic fibrosis?

A: Because the codes for CF are combination codes, the provider needs to state a relationship between the pulmonary exacerbation and the CF to assign 277.02, CF with pulmonary manifestations. If the physician does not document this link then, yes, you would need to query to establish this relationship.
However, use caution because it is the nature of the admission which determines the principal diagnosis. If the focus of treatment is the exacerbation of the CF with pulmonary manifestations, then this would be the principal diagnosis and a code will be added for the specific manifestation. If the manifestation is the focus of treatment then the manifestation is the principal and the CF code is reported as a secondary diagnosis.
Either way, yes, you would need to query to identify the manifestation/complication and the relationship to the CF so you can either list it as the principal or secondary diagnosis depending on the circumstances of the admission. This response is based on Coding Clinic, 4th Quarter, 2002. 
Editor’s Note: This question was answered by Cheryl Ericson, MS, RN, CCDS, CDIP, AHIMA Approved ICD-10-CM/PCS Trainer, CDI Education Director at HCPro, Inc., in Danvers, Mass. Contact her at For information regarding the CDI Boot Camp or ICD-10 for CDI Boot Camp visit

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