Cystic Fibrosis Flashcards
What is the CFTR protein?
A chloride channel in epithelial cells. Promotes efflux of chloride, promoting movement of water into the extrcellular space. Thins mucus
True or false. Female CF patients are infertile
False. Only males
Most common organism in lungs of adult CF patients?
Pseudomonas aerguginosa
Use of hypertonic inhalations in CF?
Creates a hypertonic extracellular environment which draws water out of the cells, causing thinning of the mucus which enhances mucociliary clearance
ADRs with hypertonic saline or mannitol?
Bronchoconstriction (often used with salbutamol to reduce this)
Discuss use of salbutamol in CF patients
Used prior to nebulised hypertonic inhalations or used PRN to treat exacerbations
ALWAYS use with a spacer
Explain use of ICS in CF patients
Reduces inflammation of the lung and the secondary damage as a result of the inflammation
CF patients often have trouble absorbing which macronutrient?
Fat. + fat soluble vitamins (ADEK)
Explain CF related diabetes and the relevant treatment
Caused by a destruction of the pancreatic islet cells that secrete insulin. Causes glucose intolerances.
Tx = insulin
Treatment for nutritional complications of CF?
Dietician review, high calorie diet, pancreatic enzyme replacement, vitamin supplements, sodium supplements
Is lung transplant a CF cure?
No. Effects more than just your lungs
In CF, chloride is trapped — the cell
Inside
Why are CF patients susceptible to more infections?
Because the mucus in the lungs is so thick that the cilia cannot clear it, making it breeding ground for infections
