Cystic Fibrosis Flashcards
1
Q
Pathophysiology
A
life limiting autosomal recessive disease that results in defective CT transmembrane conductance regulator proteins
effects cellular chloride ion channels and distribution of water and ions - effects all exocrine glands
salt/water imbalance results in the accumulation of a sticky mucus in the lungs
causes recurrent infections and colonisation with multi-resultant organisms
2
Q
Clinical Implications
A
impaired airway clearance impaired gas exchange airflow limitation dyspnoea reduced exercise tolerance musculoskeletal dysfunction
3
Q
Clinical Features
A
RESPIRATORY
- cough productive of sputum
- breathlessness on exertion
- reduced exercise capacity
- frequent exacerbations
- haemoptysis
- pneumothorax
NON-RESPIRATORY
- inadequate nutritional status and results loss of weight
- CF related diabetes
- distal intestinal destruction reflux
- CF related liver disease
- CF related arthroplasty
- postural abnormalities incontinence
4
Q
Medical Management
A
antibiotics inhaled therapies nutritional support oxygen therapy end-stage disease management
5
Q
Physio Management
A
airway clearance techniques EXERCISE musculoskeletal care - postural and muscle length continence management breathlessness management
6
Q
Exercise Management
A
3-5 days per week 30 mins per day work at 75% of max HR alternate strength training consider use of supplemental oxygen
