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% |