CF, bronchiectasis and pneumonia

Question 1

How common is CF?

Answer 1

Autosomal recessive, carrier frequency of 1 in 25, CF incidence 1 in 2000-2500, more than 2000 mutations

Question 2

CF can affect many systems in the body- what are they?

Answer 2

Repeated chest infections, pancreatic insufficiency, male infertility etc.

Question 3

What is the cause of cystic fibrosis?

Answer 3

Genes code for the CFTR protein- moves to cell surface- acts as a chloride channel

Mucociliary clearance being affected- bacteria stuck in the lungs as mucous isn’t removed

Cystic fibrosis- mutations divided into 7 classes- class 1 and 7 results in most severe, class 6 least severe disease

Question 4

CF can causes bronchiectasis and pulmonary exacerbation what are they?

Answer 4

Bronchiectasis- dilated airways and signet ring sign
Pulmonary exacerbation- increased cough, increased sputum production, change in sputum colour, fever/ malaise (feeling of discomfort)

Question 5

What is used to treat CF?

Answer 5

Therapy- nebulised antibiotic, oral/ IV antibiotics, DNase, Vitamin A, D, E, and K (fat soluble vitamins- as pancreas broken down in CF patients) , pancreatic enzymes and insulin

Question 6

What are the symptoms of CF?

Answer 6

Symptoms- weight loss, increase cough- 60 ml of thick green sputum, haemoptysis, fatty stools and reduced vision

Question 7

What clinical signs may be present for CF?

Answer 7

Clubbing, coarse crackles, wheeze, none

Question 8

Why doe someone with CF lose weight?

Answer 8

Infection and pancreatic malabsorption- not taking enzymes

Question 9

Why has Helen got diabetes?

Answer 9

Over 30% of adult have CF related diabetes (CFRD)- exocrine and endocrine part of pancreas becomes more damaged

Question 10

What is causing Helen’s loss of vision?

Answer 10

Vitamin A deficiency- fat soluble vitamin

Question 11

What is bronchiectasis?

Answer 11

• Chronic dilation of one or more bronchi
• Poor mucous clearance
• Predisposition to bacterial infection

Question 12

What are the causes of bronchiectasis?

Answer 12

Detected in about 40% of cases

Post infection- measles, whooping cough, childhood pneumonia, TB

Immune deficiency- common variable hypogammaglobinaemia, late onset idiopathic Ig deficiency, selective immunoglobin deficiency, HIV, lung transplant

Allergic bronchopulmonary aspergillosis

Defective clearance- primary ciliary dyskinesia (Kartafener’s), cystic fibrosis

Toxic- aspiration

Mechanical- tumour, lymph node (middle lobe syndrome), foreign body

Question 13

What are the symptoms of bronchiectasis?

Answer 13

Symptoms- persistent. Purulent sputum (non smokers), haemoptysis, breathless, wheeze, sinusitis and nasal symptoms, weight loss, pleurisy, nasal polyps (growth within your nose)

Question 14

What are the clinical signs?

Answer 14

Clinical signs- clubbing, coarse crackles, wheeze, none

Clinical features- clubbing- increased curvature, loss of angle

Question 15

What sort of pathogens may cause an exacerbation?

Answer 15

Staphylococcus aureus, streptococcus pmeumoniae, haemophilus influenzae, pseudomonas aeruginosa, aspergillus

Question 16

Could the history of asthma and mucus plugs be relevant?

Answer 16

Allergic bronchopulmonary aspergillosis (ABPA)- affects people with asthma and CF

Question 17

What investigation should be undertaken for bronchiectasis?

Answer 17

History, genetics/ sweat test/ faecal elastase (exocrine part of pancreas and is secreted shows how good the exocrine function is), ciliary beat frequency/ nasal NO, sperm analysis, autoantibody screen, sputum- MC&S, AFB, fungal, aspergillus antibodies, lung function, immunology review

Question 18

What is the treatment for bronchiectasis?

Answer 18

Treatment- physiotherapy, mucolytics/ hypertonic saline, prompt antibiotics (10+ days), IV antibiotics as required, nebulised antibiotic, annual influenza vaccination

Question 19

What is pneumonia?

Answer 19

An infection that inflames the air sacs in one or both lungs. The air sacs may fill with fluid or pus (purulent material), causing cough with phlegm or pus, fever, chills, and difficulty breathing.

Question 20

What are the symptoms of pnuemonia?

Answer 20

Progressive breathlessness, fever with shivering attacks, left sided sharp chest pain, general malaise, lethargy

Question 21

How is chest pain described?

Answer 21

Pleurisy- sharp and worse on inspiration

Question 22

What are the signs of pneumonia?

Answer 22

Hot, flushed with peripheral dilation, dull to percussion/ increased breath sound on auscultation, crackles, pleural rub

Question 23

What is hypotension?

Answer 23

Inflammatory stimulus (peripheral dialation), decreasing peripheral arterial resistance, cardiac output increases.

Question 24

What is the treatment for bronchiectasis?

Answer 24

Antibiotics, oxygen, analgesia, fluids, physiotherapy, nutrition (infection- need a higher calorie intake to stop muscles wasting away) and stop smoking

Question 25

How would you classify the severity of bronchiectasis?

Answer 25

0-1- likely suitable for home treatment
2- consider hospital Rx
3 or more- manage in hospital as severe pneumonia

Question 26

What investigation and procedures should be undertaken to find out if a patient has bronchiectasis?

Answer 26

Antibiotics, pleural aspiration, drain, VATS/ surgery, rhDnase/ TPA (MIST2)