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B - Paediatrics MANAGEMENT > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

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

A
  • Infection control
  • Physiotherapy
  • Nutritional management
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2
Q

What are the main aims of these combined treatment strategies?

A

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

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3
Q

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

A

Prophylactic antibiotic treatment

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4
Q

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

A

Flucloxacillin

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5
Q

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

A

Rescue antibiotics

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6
Q

What is an indicator in CF for IV antibiotics?

A

Persisting signs or symptoms despite oral antibiotics

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7
Q

Why is vigorous IV antibiotic therapy often required?

A

In order to prevent lung damage

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8
Q

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

A

14 days

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9
Q

How are IV antibiotics usually administered in CF?

A

Via a PIC line

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10
Q

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

A

Pseudomonas infection

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11
Q

What is used to treat pseudomonas infection?

A

Specific anti- pseudomonal antibiotics

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12
Q

What do more severe cases of CF often require?

A

Regular IV antibiotic therapy

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13
Q

How can regular IV antibiotic therapy best be administered?

A

Via a central venous catheter with a SC access port

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14
Q

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

A

They require monthly flushing and complications may develop

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15
Q

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

A

From diagnosis at least twice a day

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16
Q

What is the aim of physiotherapy techniques in CF?

A

To clear the airways of secretions

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17
Q

What physiotherapy techniques are advised for young children?

A

Chest percussion and postural drainage performed by parents

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18
Q

What physiotherapy techniques are advised for older children?

A

Controlled deep breathing exercises

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19
Q

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

A

Physiotherapy devices

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20
Q

What else is encouraged in CF?

A

Exercise

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21
Q

What should be assessed regularly in CF patients?

A

Dietary status

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22
Q

How is pancreatic insufficiency treated?

A

Oral enteric-coated pancreatic replacement therapy

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23
Q

When should pancreatic replacement therapy be taken?

A

With every meal and snacks

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24
Q

How is the dose of pancreatic replacement therapy determined?

A

Based on clinical response

25
Q

What is essential in a CF diet?

A

High calories

26
Q

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

A

Around 150% of normal

27
Q

How may a high calorie diet be achieved?

A

Over night feeding via gastrostomy

28
Q

What else may CF patients require to supplement their diet?

A

Fat soluble vitamin tablets

29
Q

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

A

Bilateral sequential lung transplant

30
Q

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

A

50%

31
Q

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

A
  • Co-morbidities
  • Microbiology
  • Psychological preparation
  • Optimal timing
  • Post-transplant care
32
Q

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

A

From a few years to mid 30’s

33
Q

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

A

Into the 40’s

34
Q

What do 95% of CF patients ultimately die of?

A

Respiratory failure

35
Q

What are the other common complications of CF?

A
  • Meconium ileus
  • Distal intestinal obstruction syndrome (DIOS)
  • Infection
  • Liver disease
  • Respiratory complications
  • Infertility
  • Psychological impact
36
Q

How many infants with CF are affected by meconium ileus?

A

10-20%

37
Q

What does meconium ileus cause?

A

Intestinal obstruction

38
Q

What are the typical symptoms of meconium ileus?

A
  • Vomiting
  • Abdominal distension
  • Failure to pass meconium
39
Q

What is usually needed to treat meconium ileus?

A

Surgical treatment

40
Q

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

A

Gastrografin enema

41
Q

What is a DIOS?

A

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

42
Q

How is DIOS treated?

A

Combination of oral laxatives

43
Q

What can chronic infection in CF lead to?

A
  • Damage to bronchial wall
  • Bronchiectasis
  • Abscess formation
44
Q

What organisms often cause infection in CF?

A
  • Staph aureus
  • Haemophilus influenzae
  • Pseudomonas aeruginosa
  • Burkholderia
45
Q

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

A

Pseudomonas and Burkholderia

46
Q

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

A

Avoid socialising with other CF patients

47
Q

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

A

1/3

48
Q

What evidence can be seen suggesting liver disease?

A
  • Hepatomegaly
  • Abnormal LFTs
  • Abnormal USS
49
Q

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

A

Ursodeoxycholic acid

50
Q

What does CF associated liver disease rarely progress to?

A
  • Cirrhosis
  • Portal hypertension
  • Liver failure
51
Q

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

A

Transplant

52
Q

What respiratory complications becoming increasingly more likely as CF progresses?

A
  • Chest infections
  • Pneumothorax
  • Life-threatening haemoptysis
53
Q

Which gender are affected by infertility in CF?

A

Male

54
Q

Why do males almost always experience infertility in CF?

A

They do not have a vas deferens

55
Q

How may males with CF father children?

A

Through intracytoplasmic sperm injection

56
Q

Are females affected by infertility in CF?

A

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

57
Q

Why do patients with CF require psychological support?

A

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

58
Q

Which age group require particularly special psychological consideration?

A

Adolescents

B - Paediatrics MANAGEMENT (106 decks)

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