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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
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
19
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
19
how do amplifier drug work
- increasing the amount of functional protein on the cell
20
3 types of CF modulator drugs
- amplifies - correctors
21
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
21
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
21
how do amplifier drug work
- increasing the amount of functional protein on the cell
22
3 types of CF modulator drugs
- amplifies - correctors
23
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
23
how do amplifier drug work
- increasing the amount of functional protein on the cell
23
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
24
3 types of CF modulator drugs
- amplifies - correctors
24
how do correctors work
- overcome the folding effects of the protein
25
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
25
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
25
how do amplifier drug work
- increasing the amount of functional protein on the cell
26
how do correctors work
- overcome the folding effects of the protein
26
3 types of CF modulator drugs
- amplifies - correctors
27
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
27
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
27
how do amplifier drug work
- increasing the amount of functional protein on the cell
28
3 types of CF modulator drugs
- amplifies - correctors CFTR potentiators
28
how do correctors work
- overcome the folding effects of the protein
29
how do correctors work
- overcome the folding effects of the protein
29
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
30
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
30
3 types of CF modulator drugs
- amplifies - correctors CFTR potentiators
30
how do CFTR potentiators work
- facilitate the chloride transport by modulating the gating , characteristics of the channel
31
how do amplifier drug work
- increasing the amount of functional protein on the cell
32
how does sputum rentenion affect HR
33
how does sputum mention affection respiratory rate
- compensation for loss of respiratory function
34
what does Sp86 indicate
- SpO2 of below 90 indicate a sign of hypoxemia , low oxygen levels - brain gets affect below 80-85
35
cause of tenacious mucus
- lack of water mention
36
MDT of cystic fibrosis
37
treatment of burden
- high because they require other therapies such as enzymatic therapies
38
what would you see on the CXR for cystic fibrosis patient
39
difference between AD and ACBT
40
types of CF classes
five
41
name classes of CF
42
how do correctors work
- overcome the folding effects of the protein
42
how do CFTR potentiators work
- facilitate the chloride transport by modulating the gating , characteristics of the channel
42
3 types of CF modulator drugs
- amplifies - correctors CFTR potentiators
42
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
42
how do amplifier drug work
- increasing the amount of functional protein on the cell
42
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
43
main presentation of CF
- reoccurring chest infections - wheezing -coughing -shortnes of breath - damage to the airways
44
how do correctors work
- overcome the folding effects of the protein
44
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
44
3 types of CF modulator drugs
- amplifies - correctors CFTR potentiators
44
how do CFTR potentiators work
- facilitate the chloride transport by modulating the gating , characteristics of the channel
44
how do amplifier drug work
- increasing the amount of functional protein on the cell
44
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
45
how would a wheeze look on auscultation
46
what would go in the airway section of assessing cystic fibrosis
47
how common is cystic fibrosis
48
how does kaftrio work
49
expect clinical presentations between adults and children to differ
50
how do correctors work
- overcome the folding effects of the protein
50
how do CFTR potentiators work
- facilitate the chloride transport by modulating the gating , characteristics of the channel
50
50
50
how do amplifier drug work
- increasing the amount of functional protein on the cell
50
physiotherapy lead techniques in cystic fibrosis airway clearance
- high frequency chest wall oscillations -non invasive ventilation - intermittent positive airway pressure - cough assist
51
3 types of CF modulator drugs
- amplifies - correctors CFTR potentiators
51
self administered airway clearance techniques
- ACBT - autogenic drainage - force expiratory technique - exercise - force expiratory pressure -oscillating positive airway pressure
52
signs of cystic fibrosis exacerbation
- 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
what is hempytiss
54
what would airway component of A-E assessment look like
- using accesory muscles - diminished breathing sounds or added sounds, wheezing, sea saw breathing
55
what is sea saw breathing
56
what would the breathing component of a-e assessment look like
- 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
what would the neurologist component of the multi system component of CF look like
58
high respiratory rate may indicate
tackypnea due to airway obstruction
59
normal spO2 for COPD patient
80-92
60
can CF be classed as COPD
61
what does the circulation of A-E assessment include
- HR high blood pressure - fluid balance assessment - pulses and blood pressure
62
disability component of A-E assessment
AVPU
63
what are the components of AVPU
64
effect of CF on airway surface liquid height
65
purpose of NIV
to treat hypercapnia and type II respiratory failure
66
contraindication to positive pressure
- undrained pnumothorax - moderate hympytiss
67
what is pneumothorax
air in the lung around the visceral Plura
68
what is the incision site for a laporatomy
- incision site down the middle of the abdomen along the lines abdomen
69
incision site for thoracic surgery
- on the right or left side of the chest through the breast bone
70
treatment post laporatomy
positioning mobilisation deep breathing exercises CPAP intermittent positive airway pressure breathing assisted cough ACBT
71
purpose of a exploratory laparotomy.
explore the abdominal contents - cancers - stomach problems , pain
72
problems after abdominal surgery
- paint with coughing due to incision site - high secretion load low lung compliance
72
problems after abdominal surgery
- paint with coughing due to incision site -
73
main components of pre has
74
outcome measures for exploratory laparotomy treatment
6 minute walk test, spirometry sputum retention auscultation
75
management after abdominal surgery
- if secretion progresses mobilisation is still a property - ACBT cycles - assisted cough - BIRD - suction depending on situation
75
management after abdominal surgery
- if secretion progresses mobilisation is still a property
76
post op pulmonary complications following abdominal surgery
- infections - pneumonia
77
main approaches for upper GI surgery
78
why is the success rate of upper GI surgery so poor
79
why does abdominal surgery lead to low FRC and tidal volume
80
how tot each a supported cough
81
how would ACBT differ for a upper GI patient
82
different types of anaesthesia
general regional sedation local
83
effects of general anaesthesia
- hematoma -pneumothorax - loss of cough reflex - increase secretions - bronchospasm
84
components of an effect cough
- glottic closure - inspiration against closed glottis
85
how to maintain secretion clearance post op
86
precautions before moving patient out of bed post surgery
- drips and drains - blood pressures AbGs
87
pain management following surgery
88
organs involved in upper GI surgery
- small bowel gall bladder pancreas
89
explaining pre hab to patient with learning difficultie s
- speaking in short sentences and watching vocabulary.
90
airway portion of A-E assessment
sitting up in bed and not wearing the oxygen mask
91
why would someone continue to take their oxygen mask off despite being chesty
92
type I respiratory failure
- when the person can not get enough carbon dioxide
93
effects of type I respiratory failure
94

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