Cystic fibrosis - Inhaled Antibiotics for Chronic Suppressive Therapy Flashcards
What is the underlying cause of cystic fibrosis (CF)?
Mutation of the gene that codes for cystic fibrosis transmembrane regulator (CFTR)
CFTR is a type of chloride channel
What happens to chloride transport in the pancreas of CF patients?
Impaired secretion of bicarbonate and digestive enzymes
Leads to maldigestion and malabsorption of fats and nutrients
What are the consequences of malabsorption in CF patients?
Reduced absorption of fat-soluble vitamins, especially A and E
Accumulation of digestive enzymes can lead to cell destruction
What major cause of morbidity and mortality is associated with CF?
Destruction of lung tissue
Chronic bacterial colonization and pulmonary infection are key factors
What pathogens are most commonly associated with lung infections in CF patients?
- Pseudomonas aeruginosa
- Staphylococcus aureus
These pathogens lead to chronic infections and inflammation
What percentage of males and females with CF are infertile?
98% of males and 70% to 80% of females
Infertility is due to obstruction of the vas deferens in males and cervical mucus issues in females
What are some common drugs used in CF therapy?
- Pancreatic enzymes
- Fat-soluble vitamins
- Antibiotics
- Dornase alfa
- Ibuprofen
These drugs help alleviate symptoms and delay lung injury
What is the function of ivacaftor in CF treatment?
It is a CFTR modifier that restores function of the defective protein
Approved in 2012 for specific CF mutations
What is the significance of lumacaftor in CF therapy?
It acts as a stabilizer to enhance the actions of ivacaftor
Approved in 2015, it expanded the number of eligible patients
What are common adverse effects of ivacaftor and lumacaftor?
- Abdominal discomfort
- Nausea
- Diarrhea
- Headache
- Nasal congestion
- Shortness of breath
Chest pain is a specific adverse effect associated with ivacaftor
What serious adverse effects should be monitored in patients taking CFTR modifiers?
- Hepatic injury
- Hypertension
- Cataract formation
Liver enzymes and blood pressure should be regularly monitored
What is the cost of 56 tablets of ivacaftor?
$28,675
Typical dosages vary for pediatric and adult patients
What are the four fat-soluble vitamins?
- A
- D
- E
- K
Common deficiencies in CF are in vitamins A and E
What is the purpose of inhaled antibiotics in CF?
To suppress chronic infection with P. aeruginosa
Inhalation achieves high concentrations in the airway
What are the dosages for tobramycin and aztreonam in CF treatment?
- Tobramycin: 300 mg every 12 hours
- Aztreonam: 75 mg three times a day
Both follow a cycle of 28 days on and 28 days off
What is the role of dornase alfa in CF management?
Decreases sputum viscosity by breaking down extracellular DNA
Administered via inhalation, it improves pulmonary function
What is the effect of high-dose ibuprofen in CF patients?
Slows progression of pulmonary damage
It suppresses the inflammatory response
What are the benefits of inhaled beta2-adrenergic agonists?
They improve lung function by causing bronchodilation
They also enhance ciliary function
