Table 1.

Supportive care intervention component delivery schedule

	Intervention component	Rationale	Approximate time
			Visit 1	Visit 2	Visit 3
1.	Education: disease, typical disease course, prognosis, treatment options, including mechanical ventilation, intensive care unit (ICU) admission	Rare disease, patients/caregivers are often uninformed about disease course and prognosis and therefore unaware of likelihood of ICU hospitalisation	40%	10%	5%
2.	Self-management training for most common and distressing symptoms	Progression escalation of incapacitating symptoms, for example, cough, dyspnoea, hypoxaemia, role of pulmonary rehabilitation and oxygen therapy	30%	30%	25%
3.	Caring for caregiver	Impacts family due to rapid change in life status for previously healthy individual—CG often neglects own health	20%	30%	25%
4.	Planning for future and development of shared end of life (EOL) goals	Rapid progression of disease and lack of discussion prior often leaves CG without adequate preparation for making advance care planning and EOL decisions	10%	30%	45%