Respiratory Long Case 2

Question 1

Mainfestations of CF

Answer 1

Respiratory
- Type of mutation
- Nasal polyps, sinusitis
- Micro, infections
- Sputum clearance techniques
Pancreatic insuficiency
- Nutrition/weight
- Osteoporosis
- Anaemia
- pancreatitis
- GI symptoms
Diabetes
Hepatobiliary disease
Nephrolithiasis
Fertility
VTE risk
Heat exhaustion

Question 2

Management of CF respiratory

Answer 2

• Infection prevention
		○ Avoiding sources
			§ Sick contacts
			§ Day care
			§ Don't mix with other CF pts
		○ Vaccination
		○ Hand washing
	• Optimise respiratory status
		○ Comprehensive chest physio plan including deep breathing, percussion, postural drainage, PEP techniques]
		○ Mucolytics
	• Acute
		○ Review exacerbation management
		○ Review microbiology
	• Longer
		○ Consider long term antibiotics/macrolides
		○ Consider regular tune-ups
		○ Longer term consider transplant?
	• Support
		○Support groups don't mix with other CF pts

Question 3

Infertility in CF management

Answer 3

• Genetic counselling
		○ Discuss risk of transmission
	• Discuss infertility issues
		○ May need assistance with conceptions
	• Assess pregnancy risk in females
		○ Poor nutrition, poorly managed DM, poor respiratory status (FEV1), pulmonary hypertension all concerning
		○ Risk reduction
			§ Optimise nutrition
			§ Optimise diabetes
			§ Optimise resp status
		○ Manage with multidisciplinary team in tertiary center
Contraception until optimised

Question 4

Sarcoidosis manifestation

Answer 4

• Pulmonary - BHL, ILD
	• Skin - lupus pernio, erythema nodosum
	• Eye - uveitis, sicca
	• Lymphadenopathy 
	• Cardiac - arrhythmia 
	• Neurological - 7th nerve palsy, neuropsychiatric, seizures
	• Fever, weight loss, arthralgia
Hypercalcaemia

Question 5

How would you manage Babak’s COPD?

Answer 5

Confirm diagnosis and severity
- PFT FER<70% with no bronchodilator response
Optimise function
- Pharmacotherapy (LAMA then LABA then ICS
- Adherence and inhaler technique
- Pulmonary rehab
Prevent deterioration
- Smoking cessation
- Prevent triggers
- Prevent exacerbations
- Infection prevention
- Oxygen therapy
Develop care plan
Manage exacerbations
- Early diagnosis , inhaled bronchodilators, systemic steroids
Surgical
- Transplant, lung volume reduction

Question 6

Causes of pulmonary hypertension

Answer 6

1. PAH
   a. FHx
   b. Drugs/toxins
   c. Connective tissue diseases, HIV, portal HTN, congenital heart disease
   
   2. L) Heart disease
      a. Systolic, diastolic, valve disease
   3. Lung Disease/Hypoxia
      a. COPD
      b. ILD
      c. Sleep disordered breathing
      d. Other mixed lung diseases
   4. CTEPH
      a. Chronic thromboembolic disease or other pulmonary artery obstruction (arteritis, stenosis, tumours)
   5. Miscellaneous
      a. Haem- myeloproliferative disorders, splenectomy
      b. Systemic- sarcoidosis, vasculitis
      c. Metaboic- thyroid disorders, GAUCHER’S DISEASE
      Other- fibrosis mediastinitis, CKD on HDx