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cystic fibrosis Flashcards

1
Q

treatment of cystic fibrosis

A
  • airway clearance techniques
  • inhalation therapy
  • education about the disease
    ACT
    assessment of adherence
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2
Q

pathology of cystic fibrosis

A
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3
Q

diagnosis of cystic fibrosis

A
  • sweat testing
  • high sodium because the skin cannot take in salt
  • immunoreactive trypsinogen - dried blood, heel prick test in infant s
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4
Q

problem list with cystic fibrosis

A
  • obstruction
    -hyper secretion
  • infection
  • hyper inflation
  • bronchiatesis
    -hyper reactivity
    -inflamation
    -instability
  • mucus retention
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5
Q

outcome measure with treating cystic fibrosis

A
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6
Q

aim of airway clearance techniques

A
  • improve mucucillary clerance
    – decrease airway obstruction and resistance
  • increase ventilation and airflow
  • dela disease progression
  • maintain optimal respiratory function
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7
Q

how airway clearance improve mucocillary function

A
  • decrease mucus plugging
  • tilises physiological effect of airflow and volume
  • remove secretions containing bacteria
  • homogeneous filling of alveoli
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8
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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9
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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10
Q

effective mucocillary clearance requires

A
  • adequate ventilation, air behind the obstruction
    -expirator airflow
  • effective cough
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11
Q

3 types of CF modulator drugs

A
  • amplifies
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12
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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12
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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12
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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13
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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13
Q

3 types of CF modulator drugs

A
  • amplifies
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14
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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15
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
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15
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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16
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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17
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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17
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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17
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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18
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
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19
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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19
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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19
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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20
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
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21
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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21
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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21
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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22
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
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23
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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23
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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23
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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24
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
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24
Q

how do correctors work

A
  • overcome the folding effects of the protein
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25
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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25
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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25
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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26
Q

how do correctors work

A
  • overcome the folding effects of the protein
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26
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
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27
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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27
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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27
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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28
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
    CFTR potentiators
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28
Q

how do correctors work

A
  • overcome the folding effects of the protein
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29
Q

how do correctors work

A
  • overcome the folding effects of the protein
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29
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
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30
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
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30
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
    CFTR potentiators
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30
Q

how do CFTR potentiators work

A
  • facilitate the chloride transport by modulating the gating , characteristics of the channel
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31
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
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32
Q

how does sputum rentenion affect HR

A
33
Q

how does sputum mention affection respiratory rate

A
  • compensation for loss of respiratory function
34
Q

what does Sp86 indicate

A
  • SpO2 of below 90 indicate a sign of hypoxemia , low oxygen levels
  • brain gets affect below 80-85
35
Q

cause of tenacious mucus

A
  • lack of water mention
36
Q

MDT of cystic fibrosis

A
37
Q

treatment of burden

A
  • high because they require other therapies such as enzymatic therapies
38
Q

what would you see on the CXR for cystic fibrosis patient

A
39
Q

difference between AD and ACBT

A
40
Q

types of CF classes

A

five

41
Q

name classes of CF

A
42
Q

how do correctors work

A
  • overcome the folding effects of the protein
42
Q

how do CFTR potentiators work

A
  • facilitate the chloride transport by modulating the gating , characteristics of the channel
42
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
    CFTR potentiators
42
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
42
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
42
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
43
Q

main presentation of CF

A
  • reoccurring chest infections
  • wheezing
    -coughing
    -shortnes of breath
  • damage to the airways
44
Q

how do correctors work

A
  • overcome the folding effects of the protein
44
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
44
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
    CFTR potentiators
44
Q

how do CFTR potentiators work

A
  • facilitate the chloride transport by modulating the gating , characteristics of the channel
44
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
44
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
45
Q

how would a wheeze look on auscultation

A
46
Q

what would go in the airway section of assessing cystic fibrosis

A
47
Q

how common is cystic fibrosis

A
48
Q

how does kaftrio work

A
49
Q

expect clinical presentations between adults and children to differ

A
50
Q

how do correctors work

A
  • overcome the folding effects of the protein
50
Q

how do CFTR potentiators work

A
  • facilitate the chloride transport by modulating the gating , characteristics of the channel
50
Q
A
50
Q
A
50
Q

how do amplifier drug work

A
  • increasing the amount of functional protein on the cell
50
Q

physiotherapy lead techniques in cystic fibrosis airway clearance

A
  • high frequency chest wall oscillations
    -non invasive ventilation
  • intermittent positive airway pressure
  • cough assist
51
Q

3 types of CF modulator drugs

A
  • amplifies
  • correctors
    CFTR potentiators
51
Q

self administered airway clearance techniques

A
  • ACBT
  • autogenic drainage
  • ## force expiratory techniqueexercise
  • force expiratory pressure
    -oscillating positive airway pressure
52
Q

signs of cystic fibrosis exacerbation

A
  • drop in lung funciton
  • increase sputum volume
  • increase breathlessness for cough
    -reduction in exercise tolerance
    -night sweats or fever
  • increase headache or daytime sleepiness
  • hemptysis
    raisedCRP/ white cell count
53
Q

what is hempytiss

A
54
Q

what would airway component of A-E assessment look like

A
  • using accesory muscles
  • diminished breathing sounds or added sounds, wheezing,
    sea saw breathing
55
Q

what is sea saw breathing

A
56
Q

what would the breathing component of a-e assessment look like

A
  • high respiratory rate greater than 12-20 breaths
    -osygen saturation SpO2 82
    shortness of breath
    cough
    stridor
    -tracheal position should be central
  • evidence of reduce chest wall expansion
    percussion
    occultation
    arterial blood gases
57
Q

what would the neurologist component of the multi system component of CF look like

A
58
Q

high respiratory rate may indicate

A

tackypnea due to airway obstruction

59
Q

normal spO2 for COPD patient

A

80-92

60
Q

can CF be classed as COPD

A
61
Q

what does the circulation of A-E assessment include

A
  • HR high
    blood pressure
  • fluid balance assessment
  • pulses and blood pressure
62
Q

disability component of A-E assessment

A

AVPU

63
Q

what are the components of AVPU

A
64
Q

effect of CF on airway surface liquid height

A
65
Q

purpose of NIV

A

to treat hypercapnia and type II respiratory failure

66
Q

contraindication to positive pressure

A
  • undrained pnumothorax
  • moderate hympytiss
67
Q

what is pneumothorax

A

air in the lung around the visceral Plura

68
Q

what is the incision site for a laporatomy

A
  • incision site down the middle of the abdomen along the lines abdomen
69
Q

incision site for thoracic surgery

A
  • on the right or left side of the chest through the breast bone
70
Q

treatment post laporatomy

A

positioning
mobilisation
deep breathing exercises
CPAP
intermittent positive airway pressure breathing
assisted cough ACBT

71
Q

purpose of a exploratory laparotomy.

A

explore the abdominal contents
- cancers
- stomach problems , pain

72
Q

problems after abdominal surgery

A
  • paint with coughing due to incision site
  • high secretion load
    low lung compliance
72
Q

problems after abdominal surgery

A
  • ## paint with coughing due to incision site
73
Q

main components of pre has

A
74
Q

outcome measures for exploratory laparotomy treatment

A

6 minute walk test, spirometry
sputum retention
auscultation

75
Q

management after abdominal surgery

A
  • if secretion progresses mobilisation is still a property
  • ACBT cycles
  • assisted cough
  • BIRD
  • suction depending on situation
75
Q

management after abdominal surgery

A
  • if secretion progresses mobilisation is still a property
76
Q

post op pulmonary complications following abdominal surgery

A
  • infections
  • pneumonia
77
Q

main approaches for upper GI surgery

A
78
Q

why is the success rate of upper GI surgery so poor

A
79
Q

why does abdominal surgery lead to low FRC and tidal volume

A
80
Q

how tot each a supported cough

A
81
Q

how would ACBT differ for a upper GI patient

A
82
Q

different types of anaesthesia

A

general
regional
sedation
local

83
Q

effects of general anaesthesia

A
  • hematoma
    -pneumothorax
  • loss of cough reflex
  • increase secretions
  • bronchospasm
84
Q

components of an effect cough

A
  • glottic closure
  • inspiration against closed glottis
85
Q

how to maintain secretion clearance post op

A
86
Q

precautions before moving patient out of bed post surgery

A
  • drips and drains
  • blood pressures
    AbGs
87
Q

pain management following surgery

A
88
Q

organs involved in upper GI surgery

A
  • small bowel
    gall bladder
    pancreas
89
Q

explaining pre hab to patient with learning difficultie s

A
  • speaking in short sentences and watching vocabulary.
90
Q

airway portion of A-E assessment

A

sitting up in bed and not wearing the oxygen mask

91
Q

why would someone continue to take their oxygen mask off despite being chesty

A
92
Q

type I respiratory failure

A
  • when the person can not get enough carbon dioxide
93
Q

effects of type I respiratory failure

A
94
Q
A

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