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CardioResp > (4) Paediatric Cystic Fibrosis > Flashcards

(4) Paediatric Cystic Fibrosis Flashcards

1
Q

Hoe does CF affect the body?

A
  • CFTR proteins in the body regulate salt and water movement
  • In CF these proteins may not be produced at all or not work properly
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2
Q

How is CF diagnosed?

A
  • Heel prick test
  • Newborn screening
  • Genetics
  • IRT (ImmunoReactive Trypsinogen)
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3
Q

What are some CFTR therapy drugs?

A
  • Kaftrio
  • Ivacaftor
  • Orkambi
  • Symdeko
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4
Q

What is one of the most important airway clearance techniques used in CF?

A

ACBT

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5
Q

What are some principles of ACT?

A
  • Optimize lung function
  • Consider inspiratory pause
  • Consider collateral channels
  • Optimize expiratory flow
  • Positioning
  • Huffing (N.B glottis)
  • Controlled cough
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6
Q

What are two breathing techniques used?

A
  • ACBT
  • Autogenic Drainage (AD)
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7
Q

What is autogenic drainage?

A
  • Flow modulation
  • Breathing at different lung volumes
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8
Q

What is PEP therapy?

A
  • involves breathing out against a resistance
  • airways are splinted open
  • collateral channels allow air behind secretions
  • pressure gradient allows movement
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9
Q

What is the huffing (FET) technique?

A
  • increases expiratory airflow
  • add to PEP cycles
  • can intersperse with exercise
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10
Q

What is involved in airway clearance technique for babies?

A
  • Education on CF & PEP mask
  • Assessment of chest
  • Positioning
  • Supported bouncing
  • Age-appropriate play and exercise
  • Huffing by age 3
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11
Q

What is a sample infant airway clearance session?

A
  • Assessment of chest
  • PEP mask + gymball bounce (10-12 mins)
  • Mat work (positioning / play / development
  • Build up to 2 minute cycles
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12
Q

What is Assisted Autogenic Drainage (AAD)?

A
  • therapist hands on patients chest
  • aim manually increase expiratory flow & prolong expiration
  • avoid excessive force
  • difficult to teach
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13
Q

What are manual ACT that may be used?

A
  • Percussion (cupped hand/palm cup)
  • Vibration (hands over chest wall)
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14
Q

What are some precautions for manual ACT?

A
  • reduced bone mineral density
  • fractures
  • low platelets
  • support head & avoid head shaking
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15
Q

What are examples of oscillating PEP?

A
  • PEP + internal oscillations
  • Acapella
  • Aerobika
  • Flutter
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16
Q

What is an Acapella?

A
  • counterweighted lever + magnet
  • not gravity dependent
  • resistance 1-5
  • oscillates frequencies 13-30 Hz
17
Q

What is an Aerobika?

A
  • Oscillatory PEP device
  • can combine nebulisation
  • breath actuated vs continuous mode nebulisation
18
Q

What is Bubble PEP?

A
  • 10-15 cm water
  • +/- washing fluid
  • patient blows into tubing
19
Q

What is Haemoptysis?

A

the coughing up of blood or blood stained mucus

20
Q

What are the categories of haemoptysis?

A
  • Scant < 5mls
  • Mild 5-50mls in 24 hrs
  • Moderate 50-250mls 24 hrs
  • Massive >250mls 24 hrs
21
Q

What are the recommendations for the different grades?

A
  • Mild: no immediate change ACT
  • Moderate: vigorous activity should be ceased
  • Massive: ACT & vigorous activity ceased, position bleeding side down
22
Q

What is the cause of pneumothorax?

A

formation of small sacs of air in the lung tissue that rupture, causing air leak into pleural space

23
Q

How is pneumothorax managed?

A
  • conservative or tube insertion
  • ACT but avoid PEP
  • avoid heavy weightlifting 2 weeks post
  • ACT prior to exercise
  • Inhaled therapies
24
Q

What are risk factors of incontinence?

A
  • multi-factorial
  • imbalance muscles resp, posture & continence
  • increased intra-abdominal pressure
25
Q

What are other complications of CF?

A
  • CF related liver diseases
  • CF related diabetes
  • CF bone health
  • Postural issues
26
Q

What are some recommendations for transplant in children <18 yrs?

A
  • FEV! <50%
  • 6MWT <400m
  • Hypoxaemia (88%)
  • Pulmonary hypertension
27
Q

How important is exercise in CF?

A
  • Cornerstone of treatment
  • Slows decline in lung function
  • increases QOL and muscle
28
Q

Why might someone with CF have exercise limitations?

A
  • deadspace causing minute ventilation increase
  • mucus obstruction + hyperreactivity cause increase metabolic cost breathing
29
Q

What is deconditioning?

A

It is an impaired ability for exercising muscle to extract and utilize oxygen from the blood

30
Q

What type of strength training should be performed?

A
  • start supervised bodyweight
  • start large muscles progress smaller
  • generally 3-4 times a week
  • not on consecutive days
31
Q

What are exercise considerations in CF?

A
  • Haemoptysis
  • CFRDM
  • CFRLD
  • Pneumothorax
  • Low BMD
  • Urinary incontinence
  • Advanced lung disease
32
Q

Why exercise test in CF?

A
  • unique insight into CV health
  • how body responds exercise
  • identify exercise related adverse events
  • assess interval status
  • results are motivating for patient
33
Q

What are the recommendations for exercise testing in CF?

A
  • performed at least at an annual basis
  • ideally before/after an intervention
  • CPET children >10yrs
  • Field test children >6 yrs
34
Q

What were the recommendations by Saynor in 2023 regarding exercise testing?

A
  • 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
34
Q

When would you use the 6MWT?

A

advanced lung disease or transplant assessment

35
Q

When would you use the 1 minute sit to stand test?

A

more severe lung disease or deconditioning

36
Q

What is the modified shuttle walking test (MSWT)?

A
  • externally paced
  • can be maximal
  • 10 metres
  • measure sats, HR, distance
  • no encouragement
  • note why patient stops
37
Q

How is the 6MWT performed?

A
  • 20-30m corridor
  • patient rest if needed
  • standard prompts
  • measure sats, HR, breathlessness
  • measure distance covered
  • minimal clinically important difference 33m
38
Q

How is the 1 minute sit to stand performed?

A
  • 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

CardioResp (9 decks)

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