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RESP > 13. Cystic Fibrosis > Flashcards

13. Cystic Fibrosis Flashcards

1
Q

What is cystic fibrosis?

A

AR

Abnormal apical membrane chloride channel which prevents luminal transport of chloride

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2
Q

What are the causes of death in cystic fibrosis?

A

Respiratory failure
Sepsis
Bronchial haemorrhage

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3
Q

Where is the CFTR gene located?

A

Long arm of chromosome 7

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4
Q

How many classes of CF are there?

A

6

1-3 are severe, 4-6 more mild

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5
Q

What is the most common CF mutation in Ireland?

A

Delta F508

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6
Q

What class of mutation is delta F508?

A

2

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7
Q

How is CF diagnosed?

A

Clinical features/CF history in sibling/positive newborn screening
and
positive sweat test/positive nasal PD/2 identifiable CF mutations

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8
Q

How does CF present in infancy?

A

Failure to thrive

Intestinal obstruction

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9
Q

How does CF present in childhood?

A

Failure to reach growth milestones
Recurrent chest infections
Abdominal cramps/diarrhoea

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10
Q

What are the respiratory findings in CF?

A

Panbronchiectasis

Nasal polyps/sinusitis

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11
Q

What are the GI findings in CF?

A

Pancreatic insufficiency/CF related diabetes
Liver disease
Intestinal obstruction

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12
Q

What are the non-RT or GI symptoms of CF?

A

Renal disease
Congenital absence of vas deferens
Osteroporosis

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13
Q

What are the symptoms of an exacerbation of CF?

A

Cough with increase in volume, purulence and viscidity of sputum
Increased wheeze or haemoptysis
Nasal/sinus symptoms
Onset from a few days to a few weeks

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14
Q

Why does lack of pyrexia not rule out infection in CF?

A

Their body is so used to infection that it no longer produces the normal response

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15
Q

What are the most common infectious organisms in CF?

A 
Pseudomonas
Staph aureus and MRSA
H. influenza
B. cepacia
Stenotrophomas maltophilia
Atypical mycobacteria
Aspergillus and candida
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16
Q

What complications are associated with b. cepacia?

A

Can cavitate through the chest wall after a transplant: significant contraindication to be colonised with it

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17
Q

What is the defining feature of a stenotrophomas maltophilia infection?

A

Brick red sputum

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18
Q

What complications are associated with CF?

A 
Haemoptysis
Pneumothorax
ABPA
Atelectasis
Type 2 resp failure, pulmonary hypertension and CCF
19
Q

Why do CF patients have low BMIs?

A

Require lots of calories to breathe

20
Q

What are the signs of CF on inspection?

A 
Low BMI and delayed puberty
Clubbed and cyanosed
Hyperinflated chest
Portacath and/or PEG tube
Hepatosplenomegaly
21
Q

What are the signs of CF on auscultation?

A

Creps everywhere

Expiratory wheeze

22
Q

What nebulised therapies are given in CF?

A

Anti-pseudomonal antibiotics
DNAse
7% saline helps to clear sputum
Bronchodilators as needed (salbutimol)

23
Q

What bronchodilators should be given if a CF patient has asthma concurrently?

A

Salmeterol or symbicort

24
Q

What anti-inflammatory is given in CF?

A

Azithromycin to reduce the frequency of exacerbations

25
Q

What treatment is given for ABPA?

A

Daily oral prednisone or monthly IV methylprenisolone and oral itraconazole

26
Q

What maintenance treatments are given to overcome malabsorption?

A

Pancreatic enzyme tablets: Creon
vitamin A, D, E, K: aquadek
Proton pump inhibitors
PEG feeds/nutritional supplements

27
Q

What drug is used to prevent liver disease?

A

Ursofalk

28
Q

What potentiator is given in a G551D mutation?

A

Ivacaftor

29
Q

What corrects are given in a delta F508 mutation?

A

Orkambi and symkevi

Kaftrio

30
Q

What is the function of potentiators?

A

Increase ion flow by helping chloride channel come up to membrane and open more

31
Q

What is the function of correctors?

A

Help channel stay open longer

32
Q

What methods of airway clearance are used in exacerbations?

A

PEP mask
Flutter valve
Acapella
biPAP

33
Q

What should be monitored at every CF appointment?

A

FEV1
BMI
Sputum culture

34
Q

What annual measurements should be taken in CF?

A 
Oral glucose tolerance
Liver ultrasound
Vit levels
DEXA
CXR or recent high res CT
IgE for aspergillus
FBC, U&E, LFTs, ca/phos, protein, albumin
35
Q

What are indications for lung transplant?

A

Low FEV1
>1 exacerbation in the last 3 months
24>BMI>17
Age <24

36
Q

What score is used to evaluate if a CF patient is a candidate for lung transplant?

A

CF-ABLE

37
Q

What scar is seen post lung transplant?

A

‘Clamshell’

38
Q

What is bronchiectasis?

A

Localised, irreversible dilatation of part of the bronchial tree due to destruction of muscle and elastic tissue

39
Q

What does sputum normally look like in bronchiectasis?

A

Clear, frothy, white

40
Q

What is a typical history for a patient with bronchiectasis?

A

Productive cough
Recurrent lower RTI
Intermittent haemoptysis
Over a period of years

41
Q

What complications are associated with bronchiectasis?

A

Respiratory failure
Massive haemoptysis
Amyloidosis

42
Q

What signs are seen in bronchiectasis?

A

Clubbing

Coarse creps +- wheeze

43
Q

What investigations should be done in bronchiectasis?

A 
High res CT thorax
IgG, IgA, IgE
Mannin binding lectin
a1AT levels
Sputum culture and susceptibility
Sweat testing and CF genotyping
44
Q

What is the maintenance treatment for bronchiectasis?

A

Chest physio
Nebulised DNA and anti-pseudomonal antibiotics
Alternate day azithromycin

RESP (17 decks)

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