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Flashcards in CF Deck (18):
1

aetiology

mutation in CF gene on long arm of chromosome 7

autosomal recessive mutation

2

what does the CF gene normally do

Codes for CF transmembrane conductance regulator (CFTR) channel.

Normally transports Cl- balancing salt & water on epithelial surface.

CFTR normally inhibits sodium channel

3

what does the mutated gene cause in CF

decreased Cl- secretion -> thicker mucous secretion on epithelium. Can happen in airway, GI, reproductive organs


in CF the Na channel flows loads of sodium into cells, water follows it and you get a thick, dehydrated mucous on the epithelial surface

4

pathology

Abnormal CFTR proteins ->
Cilia collapse & inflammation ->
Chronic Infection ->
Mucous plugging, ulceration & inflammation ->
Bronchiectasis

5

what can catching CF early improve

lung function and nutrition

6

what are the cardinal signs of CF

recurrent broncho-pulmonary infection
- bronchiectasis, abscesses
- haemoptysis, pneumothorax

pancreatic insufficiency
- pale/orange oily offensive stools
- failure to thrive
- fat soluble vitamin deficiency

GI symptoms

7

what are the GI symptoms of CF

dysmotility
- GO reflux
- constipation/rectal prolapse

co-existant disease
- crohns
- coeliac disease

8

what are some other symptoms of CF

Bilateral absence/blockage of Vas Deferens -> Male Infertility

Arthritis/Osteoporosis

Nasal Polyps & Sinusitis

Hepatopathy

Diabetes

Heat Exhaustion

Vaginal Candiasis

9

what are some of the signs of CF

cyanosis
clubbing
bilateral crackles
increased salt in sweat
cough
wheeze
failure to thrive
dyspnoea

10

how can CF be tested for

neonate screening - GUTHRIE TEST
- bloodspot test for immunoreactive trypsinogen (lower levels in CF)
- if +ve follow with mutation analysis and CF sweat test

bloods, cough swab, CXR

record/chart growth to monitor pancreatic insufficiency and GI blockage

11

who is involved in the management of CF

MDT - primary care handling surveillance and infection treatment

12

what is the management for pancreatic insufficiency

Enteric Coated Enzyme Pellets

High Energy Diet

Fat soluble mineral/vitamin Supplements

~H2 antagonist or proton pump inhibitors

13

what is the management for respiratory pre-infection

Segregation/cohorting to prevent cross-infection

Airway Clearance & adjuncts

Mucolytics

Influenza Vaccine

Prophylactic Antibiotics

14

what is the management for respiratory chronic infection

Treat Infective Exacerbations

Reduce Inflammation

Suppress Bacterial Load

Ibuprofen - Azithromycin - Prednisolone

Bronchodilators

15

what transplants can CF patents have

Liver, Pancreas & double Lung transplants

16

name some mucolytics and how they work

e.g. Alfladornase
- breaks down DNA released from neutrophils, making sputum easier to clear.

e.g. Hypertonic Saline
- does the same thing & is cheaper

17

what is involved in airway clearance

Percussion & Drainage

Autogenic drainage

Active Cycle of Breathing

18

what are some airway clearance adjuncts

Positive Expiratory Flow Mask

High frequency Chest Wall Oscillation

Chest physiotherapy vest