Cystic Fibrosis 2

Question 1

What are further issues with CF?

Answer 1

Fertility

Transplantation

Question 2

What effect does CF have on the sexual function of men and woman?

Answer 2

No effect

Question 3

What may CF do to puberty?

Answer 3

Cause it to occur later

Question 4

How does CF affect fertility in men?

Answer 4

>95% are infertile

Question 5

How does CF affect fertility in females?

Answer 5

They are fertile but poor nutrition may change that

Question 6

What options are available for infertile males?

Answer 6

Adoption

Artificial insemination with donor sperm

Intra-cytoplasmic sperm injection (ICSI)

Question 7

What is the success rate of artificial insemination with a donor sperm?

Answer 7

60-70% success rate

Question 8

What is the waiting list for intra-cytoplasmic sperm injection (ICSI)?

Answer 8

4 months

Question 9

What is the success rate of intra-cytoplasmic sperm injection (ICSI)?

Answer 9

25-30%

Question 10

What does ICSI stand up for?

Answer 10

Inta-cytoplasmic sperm injection

Question 11

How many attemps of ICSI is on the NHS?

Answer 11

3

Question 12

What is the cost of ICSI treatment?

Answer 12

£3000-4000

Question 13

What are the female fertility issues?

Answer 13

Major decisions (ethically and clinically)

Careful planning essential

Screening of partner

Counselling regarding long term impact of condition on family

Good communication with CF team

Optimum health (lung function and weight)

Question 14

What is the main risk for mothers with CF?

Answer 14

Baby is born premature, where the mothers can do badly

Question 15

What should be known about lung transplantation?

Answer 15

Last resort treatment

Not a miracle cure

Question 16

Where are lung transplants for grampian people done?

Answer 16

Freeman Hospital, Newcastle

Question 17

What kind of lung transplant is perfomed?

Answer 17

Double lung transplant

Question 18

When are patients bad enough for a lung transplant?

Answer 18

FEV₁ < 30% predicted

Lots of weight loss

Hypoxia at rest/desaturation on exercise

Hypercapnia

Recurrent worsening sepsis

Worsening quality of life

Question 19

Where are patients for a lung transplant often seen first?

Answer 19

Satellite clinic in Edinburgh

Question 20

What happens during the formal assessment for a lung transplant?

Answer 20

3 or 4 day admission

Must bring carer

Seen by multidiscipline team

Clinical investigations

Two way information sharing process

Multi-disciplinary meeting where the decision is made

Question 21

What are potential outcomes of the initial assessment?

Answer 21

Active list

Passive list

Too well

Decision not to go onto list (by patient)

Decision not to go onto list (by team)

Too ill

Question 22

What are 3 questions that you need to ask before doing a lung transplant?

Answer 22

Is the condition bad enough?

Is the rest of the body good enough?

Does the patient want this (prepared to take the gamble)?

Question 23

What are absolute conta-indications to transplant?

Answer 23

Other organ failure

Malignancy within 5 years

Significant peripheral vascular disease

Drug, nicotinne, alcohol dependency

Active systemic infection

Microbiological issues

Question 24

What are relative conta-indications for transplant?

Answer 24

Other organ dysfunction

Non-compliance

Steroids >20mg daily

Absence of recognised social support

Osteoporosis

Low BMI

Surgical risks (previous thoracic surgery)

Question 25

What are some psychological issues for a lung transplant?

Answer 25

Hope

Fear

Guilt/conflict

Stress

Question 26

What percentage of people who get double lung transplants survive 5 years?

Answer 26

61%

Question 27

What percentage of people who get double lung transplants survive 10 years?

Answer 27

48%