(4) Paediatric Cystic Fibrosis Flashcards
Hoe does CF affect the body?
- CFTR proteins in the body regulate salt and water movement
- In CF these proteins may not be produced at all or not work properly
How is CF diagnosed?
- Heel prick test
- Newborn screening
- Genetics
- IRT (ImmunoReactive Trypsinogen)
What are some CFTR therapy drugs?
- Kaftrio
- Ivacaftor
- Orkambi
- Symdeko
What is one of the most important airway clearance techniques used in CF?
ACBT
What are some principles of ACT?
- Optimize lung function
- Consider inspiratory pause
- Consider collateral channels
- Optimize expiratory flow
- Positioning
- Huffing (N.B glottis)
- Controlled cough
What are two breathing techniques used?
- ACBT
- Autogenic Drainage (AD)
What is autogenic drainage?
- Flow modulation
- Breathing at different lung volumes
What is PEP therapy?
- involves breathing out against a resistance
- airways are splinted open
- collateral channels allow air behind secretions
- pressure gradient allows movement
What is the huffing (FET) technique?
- increases expiratory airflow
- add to PEP cycles
- can intersperse with exercise
What is involved in airway clearance technique for babies?
- Education on CF & PEP mask
- Assessment of chest
- Positioning
- Supported bouncing
- Age-appropriate play and exercise
- Huffing by age 3
What is a sample infant airway clearance session?
- Assessment of chest
- PEP mask + gymball bounce (10-12 mins)
- Mat work (positioning / play / development
- Build up to 2 minute cycles
What is Assisted Autogenic Drainage (AAD)?
- therapist hands on patients chest
- aim manually increase expiratory flow & prolong expiration
- avoid excessive force
- difficult to teach
What are manual ACT that may be used?
- Percussion (cupped hand/palm cup)
- Vibration (hands over chest wall)
What are some precautions for manual ACT?
- reduced bone mineral density
- fractures
- low platelets
- support head & avoid head shaking
What are examples of oscillating PEP?
- PEP + internal oscillations
- Acapella
- Aerobika
- Flutter
What is an Acapella?
- counterweighted lever + magnet
- not gravity dependent
- resistance 1-5
- oscillates frequencies 13-30 Hz
What is an Aerobika?
- Oscillatory PEP device
- can combine nebulisation
- breath actuated vs continuous mode nebulisation
What is Bubble PEP?
- 10-15 cm water
- +/- washing fluid
- patient blows into tubing
What is Haemoptysis?
the coughing up of blood or blood stained mucus
What are the categories of haemoptysis?
- Scant < 5mls
- Mild 5-50mls in 24 hrs
- Moderate 50-250mls 24 hrs
- Massive >250mls 24 hrs
What are the recommendations for the different grades?
- Mild: no immediate change ACT
- Moderate: vigorous activity should be ceased
- Massive: ACT & vigorous activity ceased, position bleeding side down
What is the cause of pneumothorax?
formation of small sacs of air in the lung tissue that rupture, causing air leak into pleural space
How is pneumothorax managed?
- conservative or tube insertion
- ACT but avoid PEP
- avoid heavy weightlifting 2 weeks post
- ACT prior to exercise
- Inhaled therapies
What are risk factors of incontinence?
- multi-factorial
- imbalance muscles resp, posture & continence
- increased intra-abdominal pressure
What are other complications of CF?
- CF related liver diseases
- CF related diabetes
- CF bone health
- Postural issues
What are some recommendations for transplant in children <18 yrs?
- FEV! <50%
- 6MWT <400m
- Hypoxaemia (88%)
- Pulmonary hypertension
How important is exercise in CF?
- Cornerstone of treatment
- Slows decline in lung function
- increases QOL and muscle
Why might someone with CF have exercise limitations?
- deadspace causing minute ventilation increase
- mucus obstruction + hyperreactivity cause increase metabolic cost breathing
What is deconditioning?
It is an impaired ability for exercising muscle to extract and utilize oxygen from the blood
What type of strength training should be performed?
- start supervised bodyweight
- start large muscles progress smaller
- generally 3-4 times a week
- not on consecutive days
What are exercise considerations in CF?
- Haemoptysis
- CFRDM
- CFRLD
- Pneumothorax
- Low BMD
- Urinary incontinence
- Advanced lung disease
Why exercise test in CF?
- unique insight into CV health
- how body responds exercise
- identify exercise related adverse events
- assess interval status
- results are motivating for patient
What are the recommendations for exercise testing in CF?
- performed at least at an annual basis
- ideally before/after an intervention
- CPET children >10yrs
- Field test children >6 yrs
What were the recommendations by Saynor in 2023 regarding exercise testing?
- advocates for a small selection of test performed at high standard
- CPET considered gold standard
- Wpeak cycle ergometry if CPET unavailable
- 6MWT
- Shuttle test
- 3 minute step test
- 1 minute sit to stand test
When would you use the 6MWT?
advanced lung disease or transplant assessment
When would you use the 1 minute sit to stand test?
more severe lung disease or deconditioning
What is the modified shuttle walking test (MSWT)?
- externally paced
- can be maximal
- 10 metres
- measure sats, HR, distance
- no encouragement
- note why patient stops
How is the 6MWT performed?
- 20-30m corridor
- patient rest if needed
- standard prompts
- measure sats, HR, breathlessness
- measure distance covered
- minimal clinically important difference 33m
How is the 1 minute sit to stand performed?
- how many times sit to stand
- no use of hands
- cue when 15 seconds left
- muscle and aerobic
- minimal important difference 5 reps
- *learning effect
