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INTRODUCTION

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CASE LEARNING OBJECTIVES

  • Describe the signs and symptoms of a respiratory exacerbation of cystic fibrosis (CF)

  • Determine appropriate pharmacotherapy, including antimicrobial choice and patient monitoring for a patient with an acute CF exacerbation

  • Describe appropriate nutritional assessment and management of a patient with CF

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PATIENT PRESENTATION

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Chief Complaint

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"Increased work of breathing and worsening respiratory symptoms" per stepmother

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History of Present Illness

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JM is a 12-year-old Caucasian female brought to the emergency department for worsening respiratory symptoms. The patient's stepmother states that JM was treated with ciprofloxacin 750 mg PO BID at home for the past 2 weeks without resolution of symptoms. JM has had increased cough with very dark-green colored sputum, SOB, and lethargy. She has lost 8 pounds (3.6 kg) over the last month and is having abnormal stools (4–6 stools/d) that are foul smelling and greasy.

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Past Medical History

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JM was diagnosed with cystic fibrosis (CF) at the age of 6 months. Her CF genotype is ΔF508 homozygous. Previous hospitalizations: she is frequently hospitalized ∼ every 2–3 months for acute pulmonary CF exacerbations. She has pancreatic insufficiency requiring supplementation. History of iron deficiency anemia, GERD, allergic rhinitis. Her baseline FEV1 is 80% (0.80) of predicted.

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Family History

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JM has one 19-year-old brother who no longer lives with the family who also has CF (same genetic mutations) and has frequent hospitalizations for acute CF exacerbations.

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Social History

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JM is a sophomore at a local high school. She lives with her father and stepmother. She is nonadherent to her nebulized medications and airway clearance (misses at least once daily) and occasionally forgets to take her pancreatic enzymes (about 1–2 times/wk). Her stepmother works full time and JM does not participate in any sports or extracurricular activities outside of school.

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Allergies/Intolerances/Adverse Drug Events

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NKDA

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Medications

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Home Therapy
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Vitamin A-D-E-K supplement (Source CF) 1 gel cap PO BID

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Albuterol 2.5 mg nebulized BID

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Dornase alfa 2.5 mg nebulized daily

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Pancreatic enzyme (Creon) eight 12,000 capsules PO with meals and 4 capsules with snacks

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Vitamin E 400 IU PO BID

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Tobramycin inhalation solution (TOBI) 300 mg nebulized bid, 28 days on/off alternating with aztreonam, currently "on" month

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Airway clearance via high frequency chest wall oscillation vest bid (patient does only once daily)

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Ferrous sulfate 325 mg PO BID

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Loratadine 10 mg PO daily

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Current Inpatient Therapy
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Meropenem 850 g IV q 8 h

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Ciprofloxacin 750 mg PO q 12 h

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