Cystic fibrosis lung cancer and sleep apnoea Flashcards
CF is an abnormality in the CFTR gene in chromosome 7. IS it a recessive or dominant gene and what does this cause?
- Recessive gene
- Both parents need to have the gene
- 1 in 4 children affected
- 2 in remaining 3 carriers
- 1 healthy
How many people in the population are carriers of CF?
1 in 25
How many births have CF to some extent?
- 1 in 2500
What is CF and what does it produce?
- Inherited defect in cell chloride channels
- Produces excess sticky mucous
Which 2 organs are mainly affected by CF?
- Lung and pancreas
What 2 tests can you use to test for CF?
Perinatal testing:
- All children now screened at birth - blood
‘sweat test’:
- Measures salt content of sweat (greater salt in CF patients)
What are the main symptoms of cystic fibrosis? (4 points)
- Troublesome cough
- Repeated chest infections (with pathogens that would not usually cause a problem - pseudomonas, staphylococci)
- Prolonged diarrhoea
- Poor weight gain
What are 4 less common symptoms of CF?
- Liver dysfunction in some
- Prone to osteoporosis
- Diabetes symptoms (prolonged pancreas disease)
- Reduced fertility (mainly male)
What are 4 treatments of CF?
- Physiotherapy
- Medication (antibiotics)
- Exercise (helps to clear out pipes)
- Transplantation
Which treatment is the first line of defence of treating CF?
- Physiotherapy
- Because the lungs are full of sticky substances - if this can be removed from the lungs, chances of infection are reduced
How often would someone with CF need physio treatment?
- Performed at home for 10-60 mins daily
What medications can be used on the lungs to help treat CF? (4 points)
- Bronchodilators to open the airways
- Antibiotics to reduce chest infection frequency
- Steroids to reduce airway inflammation
- Dnase to break down mucous
What medication can be used on the digestive system to treat CF?(2 points)
- Pancreatic enzyme replacement
- Nutritional supplements
Why is exercise a good treatment for CF? (2 points)
- Necessary to keep lung function optimal
- Necessary to build physical bulk & strength
IS transplantation a cure for CF?
- No