Living with lung transplant

Question 1

How many people will give up smoking when asked

Answer 1

Only 1% of patients will give up smoking when asked- need to reduce other lifestyle factors

Question 2

What is important when prescribing medications

Answer 2

Think about whether they will interact or not- think about allergies

Question 3

What should you look at when looking at family history

Answer 3

Any significant conditions in first degree relatives

Question 4

Describe how social history is important in disease

Answer 4

Occupations, drinking, smoking, any social barriers that will prevent their adherement to a management plan
Consider psychosocial health

Question 5

Describe the sweat test

Answer 5

Used to diagnose CF- prognosis of CF has improved dramatically
The sweat test measures the amount of chloride (a component of salt) in the sweat

Question 6

Describe airway clearance in patients with cystic fibrosis

Answer 6

2x daily, end of disease process

5 or 6 hours daily

Question 7

What is the final treatment option for patients with cystic fibrosis

Answer 7

Lung transplant

Question 8

Describe the psychosocial care for cystic fibrosis

Answer 8

Important to hear about its potential impacts on jobs and social relationships
May require physiotherapy

Question 9

What is the life expectancy for someone with a lung transplant

Answer 9

Less than 3 years

Question 10

Describe the burden associated with lung transplant

Answer 10

Long inpatient stay- can’t be too underweight or too overweight- otherwise the outcomes are poor
Burden is the large number of medications that you have to take- see transplant centre two times a week, only 5 in country- may have to travel long distances
Patient compliance is essential (immune suppressants)

Question 11

What is key in any transplant

Answer 11

Balance between infection and rejection

Question 12

What is the diet like for patients with a lung transplant

Answer 12

it is akin to being pregnant

Question 13

What medications to patients with CF require

Answer 13

Medications to help digest food
Due to sinus infections
Eating can make breathlessness worse

Question 14

Describe the emotional burden associated with CF

Answer 14

Large emotional burden for family to deal with children with CF
Survivor’s guilt can have an impact on mental health

Question 15

What is the purpose of the consultation

Answer 15

Diagnosis
Management plan
Lifestyle modification

Question 16

Describe the patient complaint

Answer 16

Brief

Patients own words

Question 17

Describe the history presenting complaint

Answer 17

Mixture of open ended & closed questions
Tailored questions to rule in/out diagnoses
Details of previous episodes with similar complaint

Question 18

Describe past medical history

Answer 18

Medical

surgical

Question 19

Describe medications and allergy

Answer 19

Dose and duration

OTC, alternative therapie

Question 20

Describe family history

Answer 20

1st degree relatives

Question 21

Describe social history

Answer 21

Occupation
Smoking/ alcohol/ recreational drugs
Living situation
Social support
ADL
Pets

Question 22

Describe cystic fibrosis

Answer 22

Multi system disease
Mutation in CFTR gene
Autosomal recessive
Diagnosis usually in childhood:
Presence of 2 CFTR mutations
Positive sweat test >60mmol/L
Clinical features
Median survival ~40 years, newborn >50y

cycle of infection, inflammation, lung damage and colonisation

Question 23

Describe the respiratory management of cystic fibrosis

Answer 23

Antibiotics
Port-A-Cath/ IVAD
Airway clearance
Recombinant DNase/ Inhaled mannitol
Hypertonic saline
Macrolides (Azithromycin)
Ivacaftor (G551Dm, 5%)
Oxygen, nocturnal NIV
Lung transplant

Question 24

Describe other things you need to consider in the management of cystic fibrosis

Answer 24

High calorie high protein diet
Enteral tube feeding
Fat soluble Vitamins A/E/K/D

Question 25

What are the co morbidities of cystic fibrosis

Answer 25

Diabetes
GORD
DIOS
Osteoporosis
Chronic rhinosinusitis
Subfertility
Psychosocial

Question 26

Who needs a transplant

Answer 26

FEV1<30% of predicted
PaO2<7.3 kPa
PaCO2>6.7 kPa
Increasing frequent of pulmonary exacerbations
Recurrent/ refractory pneumothoraces
Recurrent hemoptysis despite bronchial artery embolization

Progressive chronic end stage respiratory disease
Life expectancy
2-3 years

Question 27

What is the criteria for those who can have a transplant

Answer 27

No renal, heart or liver disease
No osteoporosis
BMI >17 or <30
No untreatable psychiatric condition
No history of cancer within 5 years
Reliable social support
Good adherence to medication and appointments

Question 28

List some transplantable respiratory conditions

Answer 28

COPD
Interstitial lung disease
Cystic fibrosis
Bronchiectasis
Idiopathic pulmonary arterial hypertension

Question 29

Describe some post transplant complications

Answer 29

Infection
Immunosuppression
Viral, bacterial, fungal

Rejection
Cellular (acute)
Antibody mediated (chronic)

Question 30

Describe life after a transplant

Answer 30

Lifestyle adaptation
Diet, travel, appointments

Medications and their side effects

Frequent appointments and admissions
Antibiotics
Steroids
Bronchoscopy + Biopsy

Question 31

List some history patient complaints

Answer 31

Current symptoms
Cough
Dyspnoea
tightness/ wheeze
 haemoptysis
Sputum
Fevers
Fatigue
weight loss
anorexia
Unwell contacts
Spirometry/ FEV1
Number of illnesses in last year
Need for admission

Question 32

Describe the past medical history that may present with cystic fibrosis

Answer 32

GORD
Diabetes
GI 
Renal
Sinusitis
surgery

Question 33

Describe the medications that the patient may be on

Answer 33

Inc PT/ nebs/ Ivs/ O2/NIV

Question 34

Describe the family history associated with CF

Answer 34

1st degree relative with CF

Question 35

Describe the social history in patients with CF

Answer 35

Impact on ADL/ work
Physical activity
Occupation
Smoking/ alcohol/ drugs