Cystic fibrosis with other manifestations

This code signifies cystic fibrosis (CF) where the patient presents with symptoms or complications beyond the typical pulmonary and gastrointestinal manifestations. It covers less common or atypical presentations of CF, such as those affecting the liver, pancreas (beyond exocrine insufficiency), or reproductive system.

Use this code when documentation specifies CF with manifestations like CF-related diabetes, meconium ileus equivalent, CF-related liver disease (e.g., cirrhosis), or male infertility due to congenital bilateral absence of the vas deferens (CBAVD). It is appropriate when the "other" manifestation is clearly linked to the underlying CF.

• Always sequence the CF code (E84.9 or more specific E84.xx) first, followed by this code if applicable, and then codes for the specific "other" manifestations.
• Ensure the medical record clearly links the atypical manifestation to the patient's cystic fibrosis diagnosis.
• Do not use this code for common pulmonary or gastrointestinal manifestations, which are covered by more specific E84 subcategories.