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KEY CONCEPTS

KEY CONCEPTS

  • Image not available. Good nutrition with appropriate pancreatic enzyme and vitamin supplementation are essential in the management of cystic fibrosis (CF).

  • Image not available. Airway clearance and anti-inflammatory therapies are key components to improve pulmonary health in CF patients.

  • Image not available. Antipseudomonal agents are the cornerstone of antibiotic therapy for chronic lung infections in the CF patient.

  • Image not available. Altered pharmacokinetics of CF patients can impact the dosing and clearance of pharmacologic therapy.

PATIENT CARE PROCESS

Patient Care Process for Cystic Fibrosis

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Collect

  • Patient characteristics (eg, age, sex, pregnancy status)

  • Patient medical history (personal and family) including genetic results, if available

  • Social history (eg, tobacco/ethanol use, school, job, insurance)

  • Eating habits (when and how much of what foods)

  • Current medications including over-the-counter (OTC) use, herbal products, and dietary supplements

  • Symptoms including

    • Respiratory symptoms (eg, cough, shortness of breath, sputum production)

    • Stooling frequency and description

  • Objective data

    • Respiratory rate (RR), height, weight, oxygen saturation, and forced expiratory volume in 1 second (FEV1) if available

    • Labs including: complete metabolic panel, complete blood count (CBC) with differential, serum creatinine (SCr), culture and sensitivity results

Assess

  • Medication adherence (what, when, and how medications are taken)

  • Nutritional status

  • Exercise tolerance

  • Financial ability to afford medications

  • School / job performance and limitations

  • Emotional status of patient and family caregivers

Plan*

  • Drug therapy regimen (including doses, route, formulation, frequency, duration, and timing with other medications and therapies)

  • Order Labs (including sputum cultures, vitamin concentrations, and A1C)

  • Obtain FEV1 and bone mineral density scan

  • Referrals to social work if needed

Implement

  • Provide patient and caregiver education on the treatment plan

  • Use coaching and teach back strategies to encourage adherence

  • Schedule follow-up

Follow-up and Evaluate

  • Results of sputum cultures

  • Evaluate stool frequency and description and adjust enzyme therapy, if needed

  • Patient adherence (check refill history and patient's ability to describe regimen)

  • Cough and FEV1 including changes in symptoms

*Collaborate with patient, caregivers, and other healthcare professionals (Pulmonologist, Gastroenterologist, dietitian, social work, and respiratory therapist).

PRECLASS ACTIVITY

Preclass Engaged Learning Activity

Read Novak KJ. Chapter 33: Cystic Fibrosis: Blood, Sweat, Lungs and Gut Level II. In: Schwinghammer TL, Koehler JM, Borchert JS, Slain D, Park SK, eds. Pharmacotherapy Case Book, 10th Edition. New York, NY: McGraw-Hill Education, 2017.

This practice case gives a real-life example of a typical CF patient who might present to a clinic or hospital, the medications that are typically used, and monitoring that should be performed. This case will help develop the learner's understand about the COLLECT, ASSESS, and PLAN steps in the patient care process.

INTRODUCTION

CF is a disease state resulting from a dysfunction in the cystic fibrosis transmembrane conductance regulator (CFTR). It is the most common life-limiting genetic disorder in Caucasians with a prevalence of 30,000 affected individuals in the United States. Historically, the saying “Woe to that child which when kissed on ...

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