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Chief Complaint

“I don’t feel good; I keep coughing up green stuff and have to go to the bathroom more and my stomach hurts more when I have to go.”

History of Present Illness

Danny Miller is a 6-year-old male with cystic fibrosis (CF, with genotype F508del / G542X) admitted for an acute CF pulmonary exacerbation and worsening symptoms of steatorrhea and frequent foul, smelling stools. Danny has lost 3 pounds since his last CF clinic visit six weeks ago and this is his third exacerbation requiring hospitalization within the past year. His respiratory culture is positive for Pseudomonas aeruginosa (for the 1st time) and methicillin-susceptible Staphylococcus aureus.

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Missing Information?


Patient Database

Drug Therapy Problems

Care Plan (by Problem)


  1. Describe the pharmacokinetic differences specific to patients with CF that would affect Danny’s antibiotic dosing.

    Hint: See Treatment in PPP

  2. What should be monitored, including therapeutic drug monitoring goals, during Danny’s antibiotic course (i.e., laboratory tests, clinical signs/symptoms)?

    Hint: See Treatment and Table 17-3 in PPP

  3. If a β2 agonist is not recommended by CF guidelines, why does a β2 agonist bronchodilator like albuterol accompany inhaled therapies in the treatment of CF as part of airway clearance?

    Hint: See Treatment in PPP

  4. What are important patient/caregiver education points with regards to any new therapy started for Danny?

    Hint: See Outcome Evaluation in PPP

  5. What is the most appropriate order of administration for Danny’s inhaled therapies? Why?

    Hint: See Treatment in PPP


Danny returns to the clinic 3 months after discharge and his respiratory culture is positive for Pseudomonas aeruginosa for the second time in 6 months. What additional chronic therapy should be recommended to suppress colonization?

Hint: See Treatment in PPP


Global Perspective

Cystic fibrosis is an autosomal recessive genetic disorder that has variable incidence worldwide. Although data suggests that CF is more prevalent in Caucasian populations (approximately 1:3500 in the United States and 1:2000-3000 in the European Union), it is important to recognize that CF impacts every race and ethnicity. The currently available data of incidence in other racial and ethnic groups in the United States (e.g., 1:15,000 and 1:30,000, in African-American and Asian-American populations, respectively) may be an underestimate as there exists a misconception that CF only impacts individuals with European descent.1,2 Improved diagnostics have broadened the identification of cystic fibrosis in non-European populations and in individuals with non-typical presentations of cystic fibrosis.2 With advances in newborn screening and awareness of CF worldwide, the previously reported prevalence of CF ...

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