Cystic Fibrosis (CF)

Definition: 

• A hereditary disorder characterized by thick secretions in the lungs, pancreas, liver, intestine, and other organs resulting in multi-system disease.   
• Median survival is approximately 50 years. 

Diagnostic Criteria

• Lab work:
  • Elevated immunoreactive trypsinogen (IRT)– released by the pancreas 
  • Sweat test- done at 2 weeks old-result >/= 60 mmol/L 
• Clinical involvement in one or more organ systems 
• About two percent will require DNA analysis for confirmation 
• There is no cure for CF, but treatment can ease symptoms, reduce complications, and improve quality of life. 
• Pulmonary involvement occurs in 90% of patients who survive the neonatal period, and end stage lung disease is the principal cause of death. 

Coding Considerations

• Codes for CF are assigned based on the chronic manifestations. 
• CF unspecified (E84.9) and other specified codes such as fecal impaction are CCs with an SOI of 2 
• CF with pulmonary manifestations (E84.0) and with meconium ileus (E84.11) are MCCs and SOI of 3 
• All CF codes fall under HCC 277 for Medicare Advantage patients. 
• Assign all CF codes as needed to describe all organ involvement. Most patients will have pulmonary involvement but also may have issues with other organs. 
• If a patient is admitted for a manifestation or complication of CF, the manifestation or complication will be the PDx, and CF will be SDx. 
• If the patient is treated mainly for cystic fibrosis, then it would be PDx. 

CDI Practice Considerations

• Review records for commonly associated organ system complications: 
  • Pulmonary–bronchiectasis, sinusitis, bronchitis, recurrent pneumonia, pneumothorax, allergic bronchopulmonary aspergillosis, COPD. Common sputum colonization–pseudomonas, staph and Hemophilus 
  • Gastrointestinal-meconium ileus (neonates/infants), intestinal obstruction, FTT, GERD, GI bleeding, biliary cirrhosis, cholelithiasis, liver disease, distal intestinal obstruction syndrome (DIOS), small intestine bacterial overgrowth (SIBO). 
  • Pancreatic-pancreatic insufficiency with malabsorption and secondary insulin-dependent CF-related diabetes, pancreatitis. 
• Treatment can be a clue for complications or manifestations. It is usually directed at organ involvement.
  • Chronic antibiotics 
  • Bronchodilator therapy: inhaled Pulmozyme which liquefies sputum 
  • Hypertonic saline nebulizer 
  • Pancreatic enzyme replacement therapy (PERT) 
  • Insulin for CF-related diabetes 
  • Treatment for GERD 
  • CFTR modulator therapy, which restores deficient CF-protein function. Used in certain CF genotypes. Medications include Elexacaftor, Tezacaftor, and Ivacaftor.   

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Earn FREE ACDIS CEUs when you join Staci Josten, RN, BSN, CCDS, Alyson Swinehart, BSN, RN, CCDS, and other CDI leaders for a roundtable discussion regarding important, timely industry topics! The topic for June’s discussion is Exploring Tricky Topics in CDI: Insights and Solutions We will provide background on this topic, share industry insights, and facilitate collaborative discussion with guided questions and answers.

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Learning Objectives:

• Understand the guidelines for selecting the principal diagnosis.
• Demonstrate the ability to appropriately select a principal diagnosis in scenarios involving interrelated or competing conditions, in accordance with official coding guidelines.
• Identify a reportable diagnosis. 

What is the e4health CDI Leadership Roundtable?
The goal for the virtual CDI Leadership Roundtable Discussion is for CDI leaders to explore specific topics within CDI, learn about the topic and from each other. During each roundtable, e4health CDI Leader’s will present a CDI topic, spend time sharing current industry standards or some education regarding this topic and then open with probing questions for group discussion.

Who should attend the e4health CDI Leadership Roundtable?
The focus of this group is for those who have influence over CDI program process, policy, and education.

Why should I attend the e4health CDI Roundtable?
This will be a wonderful place to learn, share your wins and challenges and collaborate with other CDI leaders across the industry. Also, after completing a survey, free ACDIS CEUs will be earned.