Cystic Fibrosis

Question 1

What are the 3 key points in the day-to-day management of CF?

Answer 1

• Infection control
• Physiotherapy
• Nutritional management

Question 2

What are the main aims of these combined treatment strategies?

Answer 2

To ensure infants and children with CF do not have respiratory symptoms or symptoms of malnutrition

Question 3

What do many CF specialists recommend in order to reduce the risk of respiratory tract infection patients with CF?

Answer 3

Prophylactic antibiotic treatment

Question 4

What is usually recommended for prophylactic antibiotic treatment in patients with CF?

Answer 4

Flucloxacillin

Question 5

What is recommended if there is an increase in respiratory symptoms or decline in lung function?

Answer 5

Rescue antibiotics

Question 6

What is an indicator in CF for IV antibiotics?

Answer 6

Persisting signs or symptoms despite oral antibiotics

Question 7

Why is vigorous IV antibiotic therapy often required?

Answer 7

In order to prevent lung damage

Question 8

For how long are IV antibiotics given in more severe CF related infections?

Answer 8

14 days

Question 9

How are IV antibiotics usually administered in CF?

Answer 9

Via a PIC line

Question 10

What type of infection is more commonly associated with rapid decline in lung function?

Answer 10

Pseudomonas infection

Question 11

What is used to treat pseudomonas infection?

Answer 11

Specific anti- pseudomonal antibiotics

Question 12

What do more severe cases of CF often require?

Answer 12

Regular IV antibiotic therapy

Question 13

How can regular IV antibiotic therapy best be administered?

Answer 13

Via a central venous catheter with a SC access port

Question 14

What is the problem with using a central venous catheter with a SC access port?

Answer 14

They require monthly flushing and complications may develop

Question 15

When and how often should children with CF begin engaging with physiotherapy techniques?

Answer 15

From diagnosis at least twice a day

Question 16

What is the aim of physiotherapy techniques in CF?

Answer 16

To clear the airways of secretions

Question 17

What physiotherapy techniques are advised for young children?

Answer 17

Chest percussion and postural drainage performed by parents

Question 18

What physiotherapy techniques are advised for older children?

Answer 18

Controlled deep breathing exercises

Question 19

What else may be useful in the physiotherapy management of CF?

Answer 19

Physiotherapy devices

Question 20

What else is encouraged in CF?

Answer 20

Exercise

Question 21

What should be assessed regularly in CF patients?

Answer 21

Dietary status

Question 22

How is pancreatic insufficiency treated?

Answer 22

Oral enteric-coated pancreatic replacement therapy

Question 23

When should pancreatic replacement therapy be taken?

Answer 23

With every meal and snacks

Question 24

How is the dose of pancreatic replacement therapy determined?

Answer 24

Based on clinical response

Question 25

What is essential in a CF diet?

Answer 25

High calories

Question 26

How high must the calorie count be in a CF diet?

Answer 26

Around 150% of normal

Question 27

How may a high calorie diet be achieved?

Answer 27

Over night feeding via gastrostomy

Question 28

What else may CF patients require to supplement their diet?

Answer 28

Fat soluble vitamin tablets

Question 29

What is the only therapeutic treatment that can be considered in end stage CF lung disease?

Answer 29

Bilateral sequential lung transplant

Question 30

What is the current 10 year survival rate post Bilateral sequential lung transplant?

Answer 30

50%

Question 31

What must be considered before deciding to perform a lung transplant?

Answer 31

- Co-morbidities - Microbiology - Psychological preparation - Optimal timing - Post-transplant care

Question 32

How has the average life-expectancy of a CF patient improved?

Answer 32

From a few years to mid 30's

Question 33

What is the current predicted life expectancy of a baby born with CF?

Answer 33

Into the 40's

Question 34

What do 95% of CF patients ultimately die of?

Answer 34

Respiratory failure

Question 35

What are the other common complications of CF?

Answer 35

- Meconium ileus - Distal intestinal obstruction syndrome (DIOS) - Infection - Liver disease - Respiratory complications - Infertility - Psychological impact

Question 36

How many infants with CF are affected by meconium ileus?

Answer 36

10-20%

Question 37

What does meconium ileus cause?

Answer 37

Intestinal obstruction

Question 38

What are the typical symptoms of meconium ileus?

Answer 38

- Vomiting - Abdominal distension - Failure to pass meconium

Question 39

What is usually needed to treat meconium ileus?

Answer 39

Surgical treatment

Question 40

What can sometimes be useful in relieving the obsstuction caused by meonium ileus?

Answer 40

Gastrografin enema

Question 41

What is a DIOS?

Answer 41

It is the later equivalent of a meconium ileus by where the mucofaeculent material obstructs the bowel

Question 42

How is DIOS treated?

Answer 42

Combination of oral laxatives

Question 43

What can chronic infection in CF lead to?

Answer 43

- Damage to bronchial wall - Bronchiectasis - Abscess formation

Question 44

What organisms often cause infection in CF?

Answer 44

- Staph aureus - Haemophilus influenzae - Pseudomonas aeruginosa - Burkholderia

Question 45

Which organisms tend to cause rapid decline of lung function in CF?

Answer 45

Pseudomonas and Burkholderia

Question 46

What are patients often advised to do as a result of high risk infection with specific organisms?

Answer 46

Avoid socialising with other CF patients

Question 47

How many adolescent CF patients will have evidence of liver disease?

Answer 47

1/3

Question 48

What evidence can be seen suggesting liver disease?

Answer 48

- Hepatomegaly - Abnormal LFTs - Abnormal USS

Question 49

What may be beneficial to improve bile flow in patients with liver disease in CF?

Answer 49

Ursodeoxycholic acid

Question 50

What does CF associated liver disease rarely progress to?

Answer 50

- Cirrhosis - Portal hypertension - Liver failure

Question 51

What treatment is generally very successful in CF patients with severe liver disease?

Answer 51

Transplant

Question 52

What respiratory complications becoming increasingly more likely as CF progresses?

Answer 52

- Chest infections - Pneumothorax - Life-threatening haemoptysis

Question 53

Which gender are affected by infertility in CF?

Answer 53

Male

Question 54

Why do males almost always experience infertility in CF?

Answer 54

They do not have a vas deferens

Question 55

How may males with CF father children?

Answer 55

Through intracytoplasmic sperm injection

Question 56

Are females affected by infertility in CF?

Answer 56

No, they tolerate pregnancy well unless they have severe lung disease

Question 57

Why do patients with CF require psychological support?

Answer 57

They have to cope with a chronic and ultimately life-limiting disease

Question 58

Which age group require particularly special psychological consideration?

Answer 58

Adolescents