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A2. Respiratory System > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

Which organs does CF particularly affect?

A

Pancreas
Lungs
Intestines
Reproductive System

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2
Q

How many babies are born with CF?

A

1 in 2500

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3
Q

How many people in the UK have CF?

A

9,000

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4
Q

What is CF characterised by?

A

The abnormal transport of Cl- ions across the epithelium leading to thick, sticky, viscous secretion

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5
Q

What gene is mutated in CF?

A

F508

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6
Q

What protein is affected in CF?

A

CFTR Protein

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7
Q

What does the CFTR protein do in physiology?

A

Transports Cl- in to the lumen dragging water with it

Hydrating secretions

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8
Q

In CF what happens to the CFTR protein?

A

It doesn’t work
Water isn’t dragged into the lumen
This makes secretions thick

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9
Q

What does thick secretions lead to?

A

Pathogenesis in various organs and systems

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10
Q

What is Type 1 respiratory failure?

A

Decreased PaO2 only

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11
Q

What is Type 2 respiratory failure?

A

Decreased PaO2

Increased PaCO2

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12
Q

What are the early stage CF symptoms?

A

Incessant coughing
Large volume of phlegm production
Shortness of breath

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13
Q

What are the later stage CF symptoms?

A

Haemoptysis

Pneumothorax

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14
Q

Why is the thick mucous in CF ineffectively cleared?

A

Because their is a poor mucocilliary beating

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15
Q

What does poor clearing of mucous lead to?

A

Bacterial infection
Recurrent inflammation
Infection causing structural changes to the lung

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16
Q

Patients with CF get alot of bacterial infections what does the type of infection they acquire depend on?

A

Age

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17
Q

What does pseudomonas aeruginosa reduce life expectancy to?

A

From 39 to 28

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18
Q

Where is pseudomonas aeruginosa acquired from?

A

The environment

Other CF patients

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19
Q

How does pseudomonas aeruginosa affect the lungs?

A

Causes a rapid decline in lung function

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20
Q

Once colonized what does pseudomonas aeruginosa do?

A

Undergoes mucoid change

Forms a biofilm, which protects the bacteria from the hosts defense system

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21
Q

Why does pseudomonas aeruginosa form a film?

A

To protect itself from the hosts immune system/response

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22
Q

Is staphylococcus aureus a common bacteria that affects CF patients?

A

Yes

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23
Q

How is CF diagnosed?

A

By a sweat test

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24
Q

What is type 1 respiratory failure?

A

When there is decreased PaO2 ONLY

25
Q

What is type 2 respiratory failure?

A

Decreased PaO2 and increased PaCO2

26
Q

What are the early stage symptoms of CF?

A

Incessant coughing
Large volume of phlegm production
Shortness of breath (dypsnoea)

27
Q

What are the later stage symptoms of CF?

A

Haemoptysis - this is commonly associated with infections of the respiratory tract
Pneumothorax

28
Q

What is the last resort treatment for CF?

A

Double lung transplant

29
Q

When is double lung transplant called upon for CF?

A 
When:
FEV1 < 30% predicted
Significant weight loss 
Hypoxia even at rest 
Recurrent worsening sepsis 
Worsening quality of life 
If without surgery their prognosis is <2 years
30
Q

What % of CF patients die whilst on the waiting list for transplant?

A

30-40%

31
Q

On what chromosome if the F508 gene located on?

A

Chromosome 7

32
Q

When is lung transplant considered for a CF patient?

A 
When FEV1<30% predicted 
Significant weight loss 
There is hypoxia even at rest
Recurrent worsening sepsis 
Worsening quality of life 
If their prognosis without surgery is <2 years to live
33
Q

Under what circumstances does a CF patient not qualify for transplantation surgery?

A

Other organ failure e.g liver, kidney
Malignancy
Drug, alcohol smoking dependency
Active systemic infection M.Abscessus - terrible prognosis but cannot have surgery

34
Q

What other organs are affected by CF?

A 
Pancreas
Liver
Fertility 
Bones
Endocrine
35
Q

What does the pancreas produce?

A

Strong destructive enzymes

36
Q

How does CF affect the pancreas?

A

The thickening of secretions can lead the pancreatic duct to becoming blocked

37
Q

What do persistent respiratory infections cause?

A

A negative feedback cycle

38
Q

What does poor clearance of mucous lead to?

A

Increased bacterial infection

39
Q

What does the colonization of pseudomonas aeruginosa increase with?

A

Age

Over 80% colonized over 26 years of age

40
Q

What is the treatment or recurrent respiratory tract infection in CF patients?

A

Treat early and aggressively with antibiotics

41
Q

Which bacterial infections should be given oral antibiotics?

A

Staph
Haemophilus
Pneumococcus

42
Q

Which bacterial infections should be given IV antibiotics?

A

Pseudomonas
Stenotrophomonas
Burkholderia

43
Q

What 3 questions are asked at a multidisciplinary meeting prior to a patient being put on the transplant waiting list?

A

Is the condition bad enough?
Is the rest of the body good enough?
Does the patient actually want this?

44
Q

What does the blocking of the pancreatic duct lead to?

A 
Pancreatitis 
Pancreatic cysts
Scarring 
Malabsorption
Malnutrition
45
Q

How does CF affect the intestines?

A

Poor intestinal secretion can lead to harm

Immovable faeces which causes constipation and distal intestinal obstruction syndrome

46
Q

What duct can CF block in the liver?

A

Bile duct

47
Q

What does the blockage of the bile duct lead to?

A

Lead to liver scarring

Cirrhosis

48
Q

What is the 3rd most common death associated with CF?

A

Liver disease

49
Q

Which cells produce insulin?

A

Islets of Langerhans

50
Q

What does Islets of Langerhan cells produce?

A

Insulin

51
Q

What can CF do to the pancreas?

A

Damage the cells that produce insulin

Leading to a unique type of diabetes known as cystic fibrosis-related diabetes (CFDR)

52
Q

What % of CF males are infertile?

A

95%

53
Q

How does CF cause infertility in males?

A

Blocks the ducts that connect the testes to the ejaculatory ducts

54
Q

What is ICSI?

A

Intra-cytoplasmic sperm injection

55
Q

Describe the process of ICSI?

A

Sperm is injected directly into the egg
3 attempts are given on the NHS
2-3 year waiting list

56
Q

What is the success rate of ICSI?

A

25% success rate per cycle

57
Q

What should be taken into consideration when CF patients are wanting children?

A

The partner should be screened as to if a carrier
That the mother is at optimum health
That the father will still be alive to raise the child

58
Q

Be aware of the danger of prolonging life rather than extending death

A

Their can be a fine line

59
Q

Give examples of the disciplines involved in the care of CF patients

A 
Consultants 
CF nurses
Dietician 
Physiotherapists 
Pharmacist 
Micro-biologist 
Ward staff
Phycologist 
Diabetic team 
GI team 
X-ray staff
GP 
Obstetricians
Ect...

A2. Respiratory System (15 decks)

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