Cystic Fibrosis, Lung Cancer + Sleep Apnoea

Question 1

What type of disorder is Cystic Fibrosis?

Answer 1

Inherited disorder in cell chloride channels

CFTR gene - Chromosome gene

Question 2

What type of a gene is CFTR?

Answer 2

• Recessive gene

- Both parents need the gene

Question 3

How many children are affected by CF?

Answer 3

1 in 4

Question 4

How does CF affect the lungs? (2)

Answer 4

• Produces excess sticky mucous

- Lung and pancreas mainly affected (malabsorption of nutrients by the pancreas)

Question 5

How is CF diagnosed? (2)

Answer 5

1. Perinatal testing
   - all children now screened at birth by blood
2. Sweat test
   - Measures salt content of sweat
   - Greater salt in CF patients

Question 6

List the main symptoms for CF (4)

Answer 6

1. Troublesome cough
2. Repeated chest infections
3. Prolonged diarrhoea
4. Poor weight gain

Question 7

List other symptoms for CF (4)

Answer 7

1. Liver dysfunction in some
2. Prone to oesteoporosis
3. Diabetes symptoms
   - Prolonged pancreas disease
4. Reduced fertility
   - Mainly male

Question 8

List examples of CF tx (4)

Answer 8

1. Physiotherapy
2. Medication
3. Exercise
4. Transplantation

Question 9

How is physiotherapy used for CF tx? (2)

Answer 9

1. Used to help remove the mucous secretions from the lungs

2. Performed at home by parents

Question 10

What meds are used to treat the lungs for CF? (4)

Answer 10

1. Bronchodilators to open the airways
2. Antibiotics to reduce chest infection frequency
3. Steroids to reduce airway inflammation
4. DNAse to break down mucus

Question 11

What meds are used to treat the digestive system for CF? (2)

Answer 11

1. Pancreative enzyme replacement

2. Nutritional supplements

Question 12

How is exercise useful for treating CF?

Answer 12

1. Necessary to keep lung function optimal

2. Necessary to build physical bulk + strength

Question 13

How long do CF patients survive with a heart lung transplant? (2)

Answer 13

• 70% survive up to 2 years

- Longest survival about 12yrs

Question 14

What is the most common type of lung tumour?

Answer 14

• Squamous cell carcinoma

- Smoking related

Question 15

List some lung tumour effects (5)

Answer 15

1. Cough
2. Haemoptysis (blood stained sputum)
3. Pneumonia
4. Metastasis - bone, liver, brain
5. Dysphagia

Question 16

How does haemopytsis occur? (2)

Answer 16

1. Irritation to the airway from a tumour

2. Tumour is vascular and it bleeds easily, so the patient coughs up blood stained sputum

Question 17

Define sleep apnoea

Answer 17

Airway obstruction whilst asleep

• 10secs or more duration
• Airway muscle tone drops

Question 18

How does sleep apnoea present? (3)

Answer 18

1. May snore
2. Drowsiness during the day
   - Fall asleep driving
3. Increased risk of MI (low O2)

Question 19

Name the 2 types of sleep apnoea

Answer 19

1. OSA (most common)

2. Central Sleep Apnoea

Question 20

How is sleep apnoea measured?

Answer 20

Measure with AHI

• Apnoea/hypopnoea index
• Score <10 means few problems

Question 21

What is used to treat sleep apnoea? (2)

Answer 21

1. CPAP
   - Continuous Positive Airway Pressure
   - Not ventilation, just blows air into upper airways
2. Mandibular Advancement Devices
   - Will pull mandible and tongue forward so kept off the airway