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Cardiorespiratory > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

What does PEP stand for, and what is it?

A

Positive Expiratory Pressure - a patient will inhale air at a normal flow, then resistance as they exhale will mean slower and more forceful, pushing secretions out

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2
Q

What is the pathophysiology of cystic fibrosis?

A

Defect of the CFTR gene - cells unable to transport chloride ions.

Mucous-secreting cells: prevents chloride from being secreted -> intracellular levels increase -> water not attracted to mucus to thin it out -> mucus abnormally thick -> build up of mucus

Sweat-producing cells: chloride ions not reabsorbed -> accumulate in sweat

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3
Q

What is the name of the test performed on newborn babies that screens for cystic fibrosis?

A

Guthrie Heel Prick test

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4
Q

What are three ways a diagnosis of cystic fibrosis can be confirmed?

A
  • Sweat test (chloride levels >60mmol/L = diagnosis very likely) - gold standard
  • DNA testing (gene mutation analysis)
  • Stool test (enzyme: elastase)
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5
Q

How does a person inherit CF?

A

It is an autosomal recessive disorder - inherited when a person receives a mutated copy of the CFTR gene from both parents.

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6
Q

On what # chromosome is the CFTR gene?

A

7

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7
Q

What organs/organ systems are affected by cystic fibrosis?

A
  • Respiratory system
  • Gastrointestinal system
  • Liver
  • Endocrine system
  • Reproductive organs
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8
Q

How does CF affect the respiratory system?

A
  • Abnormal, sticky secretions
  • Recurrent infection and inflammation
  • Wheezing
  • Chronic cough
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9
Q

How does CF affect the gastrointestinal system?

A
  • Malnutrition due to malabsorption of fat and fat-soluble vitamins
  • Poor weight gain (failure to thrive)
  • Frequent, foul-smelling, greasy bowel movements
  • Stomach aches
  • Excessive wind
  • Liver disease
  • Intestinal obstruction (constipation)
    - Meconium ileus (neonatal intestinal obstruction)
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10
Q

Signs and symptoms of cystic fibrosis:

A
  • Salty tasting skin
  • Slow weight gain or failure to thrive despite good appetite
  • Wheezing, coughing, pneumonia
  • Abnormal bowel movements
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11
Q

How does CF affect the reproductive system?

A

Men: blockage of or underdeveloped vans deferens (fertility problems, but not impotent)

Women: irregular menstrual cycles due to nutritional issues (but have healthy, fertile eggs)

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12
Q

What are some indications of infection in CF?

A
  • increased, moist, or more productive cough
  • yellow-green sputum
  • wheeze
  • SOB
  • decreased exercise tolerance
  • lethargy
  • loss of appetite
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13
Q

What S&S might you expect as disease progresses?

A
  • Respiratory distress
  • Finger clubbing
  • Chest deformity (hyperinflation, flattening of diaphragm)
  • Reduction of function
  • Respiratory failure
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14
Q

What proportion of Caucasian NZers are thought to be carriers of the CF gene?

A

1 in 25

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15
Q

The absorption of what vitamins is impaired (due to blocked ducts in the pancreas)?

A

Vitamin A, D, E, & K

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16
Q

What are six bacteria that can cause lots of trouble for CF patients?

A
  • Pseudomonas aeruginosa
  • Staphylococcus aureus (incl. Methicillin-resistant - MRSA)
  • Burkholderia cepacia
  • Aspergillus
  • Non-tuberculous mycobacteria
  • Influenza
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17
Q

Why are people with CF recommended to stay at least 6 feet from each other?

A

Cross-infection - more likely to pass bacteria on to each other

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18
Q

What is common medical treatment for CF?

A

Antibiotics and inhalation therapy

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19
Q

How are antibiotics commonly administered for CF?

A
  • Antibiotic route (oral, nebuliser, dry powder inhaler, TOBI Podhaler)
  • IV (peripheral cannula, PICC, portacath)
20
Q

Types of inhalation therapy for patients with CF?

A
  • Bronchodilators eg salbutamol (Ventolin)
  • Preventers eg Seretide
  • Hypertonic saline (before and during airway clearance techniques)
  • Bronchitol
  • Pulmozyme
  • Nebuliser
  • Trikafta
21
Q

When should bronchodilators be used?

A
  • PRN
  • Before airway clearance techniques
  • Before hypertonic saline
22
Q

Will a lung transplant cure CF?

A

No - as CF still affects organ systems other than the lungs. Median survival post transplant ~8.5 years. Transplanted lungs susceptible to infection and rejection

23
Q

How can CF affect the endocrine system?

A

Can lead to CF related diabetes in adolescents/adulthood - due to scarring of exocrine pancreas -> disruption to insulin secreting endocrine cells

24
Q

What is described as the ‘cornerstone of CF treatment’?

A

Airway clearance

25
Q

What Airway Clearance Techniques should be used on babies?

A
  • percussion (5 modified positions)

- assisted huffing & bouncing on therapy ball/infant PEP

26
Q

What Airway Clearance Techniques should be used on toddlers?

A

Same as babies plus:

  • blowing and huffing games
  • bubble PEP
  • trampoline and swimming
  • vibrations
27
Q

What Airway Clearance Techniques should be used on children?

A

Same as toddlers plus:

  • PEP mask or oscillating PEP
  • ACBT w/ percussion/vibrations and modified postural drainage
28
Q

What Airway Clearance Techniques should be used on adolescents?

A

Same as children plus:

- autogenic drainage

29
Q

What Airway Clearance Techniques should be used on adults?

A
  • Percussions/vibrations/positioning
  • ACBT/FET/autogenic drainage
  • PEP/Acapella/flutter
30
Q

What other methods can be used for airway clearance?

A
  • High frequency chest wall oscillation (vest)
  • Intrapulmonary percussive vibration
  • Physical exercise
31
Q

Head down positioning has been associated with reflux, when could head down positioning be considered?

A
  • No reflux symptoms
  • Very productive/bronchiectatic
  • Specific LL changes on CXR
  • Benefits outweigh risk
  • Patient or carer/parent reports more effective for clearance
    (Avoid doing 1-2 hours after eating)
32
Q

What are the contraindications for percussion and vibration?

A
  • Severe osteoporosis
  • Frank haemoptysis
  • Rib fracture
  • Chest injuries
33
Q

What can be used to help teach children the techniques in ACBT?

A
  • Deep breathing: balloons, party blowers, wind instruments, blowing windmills, bubbles etc
  • FET: get them to use a mirror and fog it up
34
Q

In ACBT, what part should you spend more time on if a patient has more atelectasis?

A

Thoracic expansion exercises

35
Q

In ACBT, what part should you spend more time on if a patient has more bronchospasm?

A

Breathing control

36
Q

In ACBT, what part should you spend more time on if a patient has more sputum?

A

Forced expiratory technique

37
Q

General physiotherapy treatment options for CF:

A
  • Percussion
  • Vibrations
  • ACBT
  • Autogenic drainage
  • Positioning
  • PEP
  • Bubble/bottle PEP
  • Oscillating PEP
  • Vest
  • Exercise
38
Q

What pressure should be used for PEP?

A

10-20cm H2O

39
Q

Contraindications, precautions for PEP?

A

CI: undrained pneumothorax
PC: increased WOB, increased ICP, haemodynamic instability, recent facial surgery, active sinusitis, active haemoptysis, drained pneumothorax

40
Q

What is the theory behind PEP?

A

Collateral ventilation - recruits obstructed or collapsed airways, increased volume of air behind obstruction will force secretions centrally

41
Q

Contraindications and precautions for oscillatory PEP?

A

CI: frank haemoptysis and undrained pneumothorax
PC: increased WOB, increased ICP, haemodynamic instability, recent facial surgery, active sinusitis, active haemoptysis, drained pneumothorax

42
Q

What are some musculoskeletal complications of CF?

A
  • Pain (chest and back pain common)
  • Low bone density
  • Fractures
  • Thoracic kyphosis
  • CF arthropathy
43
Q

What changes should be made to physio treatment if the patient experiences MODERATE haemoptysis?

A

Cease percussion, vibrations, oscillatory PEP, hypertonic saline, head down tilt
Cease vigorous exercise
Continue with controlled ACT

44
Q

What changes should be made to physio treatment if the patient experiences SEVERE haemoptysis?

A

Active bleeding: high side lying with bleeding side down

Cease ACT and exercise until active bleeding resolves

45
Q

What changes should be made to physio treatment if the patient experiences pneumothorax?

A

Gentle coughing, adequate humidification, cease PEP, avoid UL resistance exercises

Cardiorespiratory (14 decks)

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