10.1 Cystic Fibrosis (Respiratory)
Learning Outcomes
Be able to:
- Explain the pathophysiology of Cystic Fibrosis (CF)
- List the drug treatment options and place in a therapeutic context
- Describe the
- Mechanism of action
- Common adverse effects
- Outline the monitoring and counselling points
- Detail the principles of infection prevention and antibiotic treatment
- Apply this knowledge to a case study of uncomplicated CF
- Identify common clinical problems and create a pharmaceutical care plan (including therapeutic recommendations).
Cystic Fibrosis
This chapter focuses on the respiratory management of cystic fibrosis (CF). As pharmacists, we see patients with cystic fibrosis – both paediatric and adult. Many of their medications can only be sourced through the hospital pharmacy dispensary. However, pharmacy services are not limited to supply of specialised medications. I hope that this module will provide you with a solid knowledge base and help you to realise the essential role of a pharmacist as part of a multidisciplinary team caring for patients with CF.
Case study: Abi’s Story
Read Abi’s story. https://au.sports.yahoo.com/its-terrifying-teens-sage-advice-after-receiving-lifesaving-gift-for-her-18th-birthday-104541137.html
- Abi is an incredibly inspirational Townsville local living with CF. She is passionate about motivational speaking and sharing her story for the purposes of educating others.
Key Resources for Further Reading
- Therapeutic Guidelines (eTG) – Respiratory (Cystic Fibrosis)
- Cystic Fibrosis Australia website – https://www.cysticfibrosis.org.au/
CF is a Complex Multisystem Disease
CF primarily affects the respiratory system (lungs), it also affects the digestive system (particularly the pancreas) and the reproductive system.
📺 Watch the YouTube video for a reminder about the “Pathophysiology of cystic fibrosis” (2.31 mins).
The video can also be accessed at https://youtu.be/6IbP1ASGv9w
People with CF secrete very thick and sticky mucus that narrows and blocks the airways and traps bacteria, predisposing to infections and irreversible lung damage. In the pancreas, dehydrated secretions lead to exocrine duct obstruction and pancreatic damage. In turn, this prevents the release of pancreatic enzymes and thus malabsorption of fat and other nutrients.
For the purposes of this subject, we will focus on the impact of CF on the respiratory system. However, we will also see that some of the drug treatment options can alleviate the impact of CF on other organ systems as well.
Approach to Respiratory Management
The respiratory management of CF encompasses 5 essential considerations.
- Effective airway clearance. This may be achieved from a combination of chest physiotherapy, exercise, beta-agonists, and nebulised mucolytics (e.g., dornase alfa or hypertonic saline nebules)
- Anti-inflammatory drugs. In the lecture recording, we discuss the anti-inflammatory properties of azithromycin. There is a controversial/limited role for oral and inhaled corticosteroids (inhaled corticosteroids are generally only be used if there is evidence of concomitant asthma).
- Potentiator therapy. If the patient has a certain type of CF gene mutation, we may use drugs (e.g., ivacaftor) to correct the cystic fibrosis transmembrane conductance regulator (CFTR) protein function.
- Infection prevention and control strategies. These are essential because respiratory pathogens are highly transmissible, and can readily cause difficult-to-treat CF exacerbations.
- Optimising antibiotic therapy. In the case of a CF exacerbation, early & aggressive antibiotic therapy is essential to delay the progressive decline in lung function. Choice of antibiotic regimen (dose & frequency) is a decision made by the patient’s specialist CF centre.
Drug regimens in CF patients can quickly become very complex. You should aim to develop an appreciation of this.
Lecture Materials
The chapter resources have been divided into 3 parts. Video slides for all 3 parts can be downloaded here.
Activities
- What organism(s) are most commonly found in the airways of paediatric patients with cystic fibrosis?
- Describe the approach to antibiotic treatment.
- What organism is most commonly found in the airways of adolescent and adult patients with cystic fibrosis?
- Describe the approach to antibiotic treatment. What are the risks and benefits?
- List 3 different dosage forms used to deliver drugs for the treatment of CF.
- What are the advantages and disadvantages of each?