3. Cystic Fibrosis, Lung Cancer and Sleep Apnoea Flashcards
What is cystic fibrosis (2)
Inherited disorder
Caused by CFTR recessive gene (chromosome 7)
What does CFTR gene cause
Defect in cell chloride channels
Result of cystic fibrosis (what does the CFTR gene cause)
Excess sticky solid mucus (rather than rubbery) that traps bacteria
Organs mainly affected by cystic fibrosis (2)
Lungs
Pancreas
Cystic fibrosis diagnostic methods (2)
Perinatal testing (blood test at birth) Sweat test
What does a sweat test measure
Salt content of sweat
Difference in normal/cystic fibrosis sweat test
Cystic fibrosis - greater salt content in sweat
Major symptoms of cystic fibrosis (4)
Troublesome cough
Repeated chest infections
Prolonged diarrhoea
Poor weight gain
Minor symptoms of cystic fibrosis (4)
Liver dysfunction Prone to osteoporosis Diabetes symptoms (prolonged pancreas disease) Reduced fertility (mainly male)
Cystic fibrosis treatment (4)
Physiotherapy
Medication
Exercise
Transplantation
Role of cystic fibrosis physiotherapy
Remove mucus secretion from lungs
Types of medications used for cystic fibrosis treatment (4)
Bronchodilators (lungs - open airways)
Antibiotics - reduce chest infection frequency
Steroids - reduce airway inflammation
DNase - break down mucus
(Pancreatic enzyme replacement and nutritional supplements)
Role of exercise in cystic fibrosis treatment (2)
Maintain optimal lung function
Build physical bulk and strength
When is transplantation performed
During end stage lung disease (ESLD)
What is ESLD
Extensive fibrosis of lung tissue causing dysfunction
