Because of the debilitating symptoms and poor prognosis, patients face an uncertain future, and patients and their families or caregivers face daily challenges in terms of managing the disease and maintaining an acceptable quality of life.
At present, patients often have a tortuous path to a diagnosis of IPF, with little information about the disease when it is diagnosed, particularly if they are not treated at a specialist centre. Many patients do not immediately realize the gravity of their condition, and both patients and their families or caregivers can later be overwhelmed by the physical and psychological symptom burden, with relentlessly deteriorating quality of life and a continuous increase in distressing symptoms. For approximately 80% of patients, lung transplantation will not be possible because suitable donors are not available, and they currently have few other treatment options. The scientific literature supports the benefits of palliative care, but this is offered infrequently and usually late in the disease process.