Complexities of Cystic Fibrosis Pulmonary Care
Monograph
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Course Summary
Cystic fibrosis (CF) is considered a rare disease, according to the National Organization of Rare Diseases, and is one of the most commonly diagnosed genetic disorders. Approximately 30,000 people are living with CF in the United States, with 1,000 new cases diagnosed every year. Pulmonary complications and clinical characteristics, including progressive obstructive lung disease, sinusitis, and infection, among others, contribute to significant morbidity. Although CF is a life-shortening disease, survival has improved due to earlier diagnosis through routine newborn screening, evidence-based guidelines to optimize pulmonary health, and the development of CF-specific interdisciplinary care centers. Pharmacists are well-positioned to engage in shared decision-making regarding treatments for CF pulmonary conditions and to remain aware of the comprehensive safety and efficacy of these treatments.
Course Objectives
Describe the common clinical pulmonary manifestations of cystic fibrosis (CF)
Identify laboratory and genetic studies needed to diagnose CF
Recognize the most common presentations of CF-related complications
Describe the current recommendations for long-term maintenance of optimal lung health in CF patients
I. Introduction
II. Epidemiology
III. Pathophysiology
IV. Clinical Presentation
V. Screening and Diagnosis
VI. Cystic Fibrosis Management
VII. Therapies to Maintain Lung Health
1. Clearance of Airway Secretions
2. Chronic Airway Infections
VIII. CFTR Modular Therapies
IX. Pulmonary and Extrapulmonary CF Complications
1. Pulmonary Exacerbation (PEx)
2. Hemoptysis
3. Pneumothorax
4. Chronic Rhinosinusitis (CRS) and Nasal Polyposis
X. Transitioning to Adulthood
XI. Patient Counseling
XII. Next Steps
XIII. Summary
- Read the course objectives and faculty planner disclosure
- Read the course material
- Complete the post-test with a minimum score of 70% and complete the course evaluation form.
- Results are automatically submitted to CPE Monitor
Faculty Planner Disclosure
The following individuals were involved in developing this activity: Pamela Sardo, PharmD, BS. Pamela Sardo was an employee of Rhythm Pharmaceuticals until March 2022 and has no conflicts of interest or relationships regarding the subject matter discussed. There are no financial relationships relevant to this activity to report or disclose by any of the individuals involved in the development of this activity.
Unlabeled Use Disclosures
The information provided in this course is general in nature and it is solely designed to provide participants with continuing education credit(s). This course and materials are not meant to substitute for the independent, professional judgment of any participant regarding that participant’s professional practice, including but not limited to patient assessment, diagnosis, treatment and/or health management. Medical and pharmacy practices, rules, and laws vary from state to state, and this course does not cover the laws of each state; therefore, participants must consult the laws of their state as they relate to their professional practice. Healthcare professionals, including pharmacists and pharmacy technicians, must consult with their employer, healthcare facility, hospital, or other organization, for guidelines, protocols, and procedures they are to follow. The information provided in this course does not replace those guidelines, protocols, and procedures but is for academic purposes only, and this course’s limited purpose is for the completion of continuing education credits. Participants are advised and acknowledge that information related to medications, their administration, dosing, contraindications, adverse reactions, interactions, warnings, precautions, or accepted uses are constantly changing, and any person taking this course understands that such person must make an independent review of medication information prior to any patient assessment, diagnosis, treatment and/or health management. Any discussion of off-label use of any medication, device, or procedure is informational only and such uses are not endorsed hereby. Nothing contained in this course represents the opinions, views, judgments, or conclusions of RxCe.com LLC. RxCe.com LLC is not liable or responsible to any person for any inaccuracy, error, or omission with respect to this course, or course material.
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- Target Audience: Pharmacist
- Contact Hours: 1.0 (0.1 CEUs)
- Activity Release Date: 10/17/2023
- Activity Expiration Date: 10/17/2026
- Activity Type: Knowledge
- UAN: 0669-0000-23-172-H01-P
- Topic: Disease State Management/Drug Therapy
- CeBroker Number: 20-1117190
Author:
RxCe.com, LLC is accredited by the Accreditation Council for Pharmacy Education as a provider of continuing pharmacy education.