Respiratory

Question 1

Who should be commenced on regular ICS/LABA combination Rx?

Answer 1

COPD with FEV1 < 50% AND 2 or more exacerbations in the past 12 months

Question 2

What is the recommended vaccination regime for COPD patients?

Answer 2

Annual flu shot

Pneumovax

• At time of Dx, then at least 5 years later
• 3rd dose 5yrs after the 2nd dose, if pt not previously vaccinated at time of COPD Dx

Question 3

At what FEV1 should theophyilline be considered?

Answer 3

FEV<40% (severe)

Question 4

What are the criteria for home O2?

Answer 4

PaO2≤55mmHg, or PaO2≤59 PLUS polycythaemia, pulmonary HTN or right heart failure

Question 5

What investigations are relevant in pulmonary arterial hypertension?

Answer 5

• Cardiopulmonary exercise test: assessment of exercise capacity
• LFTs: PAH Rx may affect LFTs
• NT-pro-BNP: elevation predictive of 1yr mortality

Question 6

How are CMV-negative recipients of CMV-negative lungs managed when they require blood products?

Answer 6

Leucoreduced or blood from CMV-negative donors

Question 7

What needs to be monitored post lung transplant?

Answer 7

HTN
Hypercholesterolaemia
DM (higher in CF)
Vaccinations (no live vaccines)
Cancers
- Skin checks yearly
Pregnancy
- Unsafe with mycophenolate and mTOR inhibitors
Drug monitoring
- Therapeutic drug monitoring
CYP450 inhibitors (CNI and mTORi are substrates)
- Verapamil, diltiazem
- Amiodarone
- Macrolides
- Ciprofloxacin
- Azoles
- HIV antiretrovirals
- Glucocorticoids
- Grapefruit juice
CYP450 inducers
- Carbamazepine
- Phenytoin
- Rifampin
- Glucocorticoids

Rejection

• Acute cellular rejection (T-cells) => steroids or T-cell depleting antibodies
• Humoral rejection (B-cell mediated) => IVIG, plasmapheresis
• Bronchiolitis obliterans

CMV

• Prophylaxis with valgancyclovir
• Alternatives are cidofovir or foscarnet in resistant CMV

PCP
- Bactrim

Fungal infection (candida, aspergillus)

• Oral azoles for candida
• Voriconazole for aspergillus

Question 8

What drugs may interact with mycophenolate?

Answer 8

Decrease concentration

• Bile acid sequestrants
• Antacids
• PPI
• Antacids
• Rifampin

Increase concentration

• Acyclovir
• Valacyclovir

Question 9

How is obesity hypoventilation syndrome diagnosed?

Answer 9

BMI ≥ 30 and daytime PaCO2 ≥ 45 mmHg in the absence of other causes of hypoventilation:

• COPD, ILD
• Kyphoscoliosis, neuromuscular disease, electrolyte disturbance
• Cerebrovascular disease
• Hypothyroidism
• Congenital alveolar hypoventilation syndrome
• Sedatives/hypnotics/opiates/EtOH

Question 10

What are the diagnostic tests in cystic fibrosis?

Answer 10

Sweat test

• Sweat Na ≥ 60 is confirmatory
• Sweat Na 30-59 should be referred for further testing

Immunoreactive trypsinogen
- Heel stick dried blood spot at birth

Genetic testing
- Mainly ΔF508

Question 11

What are the components of cystic fibrosis management?

Answer 11

Respiratory

• ABx for acute LRTIs
• Flutter valve or other Positive Expiratory Pressure (PEP) device
• Mucolytics (N/saline, hypertonic saline, mannitol, dornase alfa)
• Inhaled tobramycin if Pseudomonas positive
• Alternate day corticosteroids if severe
• Azithromycin 3 times weekly
• Homozygous ΔF508 => Lumacaftor/ivacaftor
• G551D => Ivacaftor monotherapy

Lung transplant
- Check for Burkholderia cepacia, as this is a C/I

Exocrine dysfunction

• Creon
• PPI/H2 antagonist improves creon function
• Vit A/D/E/K
• Dietitian for high caloric, high fat diet
• May need gastrostomy

Endocrine dysfunction
- OHAs and insulin

Liver disease
- Ursodeoxycolic acid

GORD
- PPI/H2 antagonist

Distal Intestinal Obstructive Syndrome
- Monitor bowel patterns

Intussusception
- May need surgical intervention

Fertility
- Bilateral absence of vas deferens, aka obstructive azoospermia

Osteoporosis
- Ca intake, Vit D level, DEXA, exercise prescription

Depression
- CBT, psychologist, psychiatrist, community groups, antidepressants