Cystic fibrosis, lung tumours and obstructive sleep apnoea

Question 1

What causes cystic fibrosis?

Answer 1

An inherited CFTR gene on chromosome 7

Question 2

What kind of gene is it?

Answer 2

A recessive gene

both parents need to have the gene in order for the child to have CF

Question 3

What does cystic fibrosis affect?

Answer 3

Causes a defect in the chloride channels of cells

= excess mucous production

Question 4

What organs des cystic fibrosis mainly affect?

Answer 4

It is a multi system disease however the lungs and pancreas are worst affected by it.

Question 5

How do you diagnose cystic fibrosis?

Answer 5

Heel prick at birth

Sweat test:
Those with CF have a higher salt content in their sweat.
(chloride channel defect = more chloride.
Salt is sodium chloride.)

Question 6

What are the symptoms of cystic fibrosis?

Answer 6

Cough - mucous build up

Repeated chest infections - Can’t clear the excess mucous out of the system, contains bacteria which can cause infection.

Diarrhoea - Mucous blocks pancreatic secretions = no digestive enzymes produced to digest fat.

Diabetic symptoms: Mucous blocking pancreatic secretions = no insulin.

Osteoporosis: lack of nutrient absorption

Question 7

What is the treatment for cystic fibrosis?

Answer 7

Physiotherapy:
10 - 60 mins daily
Must occur at different angles since the lobes of the lungs are situated at different angles. Allows the mucous to drain into the larger airways and be coughed up.

Medication:(lungs)
Bronchodilators: Keep airways open and allow mucous to be coughed up.

Anti-inflammatory: Prevent narrowing of the airways

Dnaase: Break down the mucous

Antibiotics: Fight the chest infections that occur from mucous build up. (have to be given cautiously to prevent resistance)

Exercise:
Maintains optimum lung function
Rapid breathing from exercise encourages mucous to be cleared.

Transpant:
Not a cure!
Used in end stage where the heart has to work harder to compensate for the lungs.
This can lead to heart disease. 
A dual transplant may be required.

Question 8

List the main types of lung cancer.

Answer 8

Squamous cell carcinoma
Small cell cancer
Large cell cancer
Adenocarcinoma

Question 9

What is the most common type of lung cancer?

What is tis caused by?

Answer 9

Squamous cell carcinoma

Caused by smoking; the smoke irritates the lungs causing metaplasia of the respiratory epithelium into squamous cell epithelium.

Question 10

Describe the characteristics of a lung cancer.

Answer 10

Almost all are malignant and spread.

Take a long time to develop - can appear years after the patient stops smoking.

Usually asymptomatic until very late stages.

Question 11

Describe the effects of lung tumours.

Answer 11

Cough: tumour irritates the airway.
Coughing up bloody sputum.

Repeated infections i.e. pneumonia

Recurrent laryngeal nevre palsy (unilateral)

Superior vena cava obstruction.

Metastasis: Brain, liver and bone common.

Dysphagia

Question 12

What is obstructive sleep apnoea?

Answer 12

Airway obstruction for > 10 seconds whilst sleeping caused by a reduction in airway muscle tone.

Question 13

What occurs in sleep apnoea?

Answer 13

Due to the lack of airway muscle tone, the tongue falls back into the soft palate and the soft palate falls on to the airway, obstructing it.

Question 14

What are the types of sleep apnoea?

Answer 14

Obstructive sleep apnoea - most common

Central sleep apnoea - brain not sending signals to promote breathing.

Can have a mix of both

Question 15

What are the effects of sleep apnoea?

Answer 15

Drowsiness during the day.
Chronic hypoxia at night can lead to a myocardial infarction.
Snoring - those with obstructive sleep apnoea.

Question 16

How is sleep apnoea treated?

Answer 16

CPAP: Continuous positive airway pressure.

Mandibular advancement devices:
Device projects the mandible forward - tongue is attached to the mandible therefore it prevents the tongue from falling onto the soft palate and the soft palate from obstructing the airways.