The value of time for IPF patients
Over 200,000 patients across Europe and the United States have idiopathic pulmonary fibrosis (IPF), an incurable lung disease more deadly than most cancers. IPF causes progressive scarring of the lungs that cannot be reversed, making it harder and harder for oxygen to find a way into the body.
Before IPF treatments were available, patients with IPF on average live between 2 – 5 years following an IPF diagnosis. Shortness of breath and cough are the most disabling symptoms. Up to 80% of IPF patients suffer from cough, with the median patient coughing over 500 times per day. This can make ordinary activities like taking the stairs or a walk in the park into a daily challenge. It is not known why some people develop IPF, while others with similar risk factors do not.
We spoke to Liam Galvin, Secretary of the European Idiopathic Pulmonary Fibrosis & Related Disorders Federation (EU-IPFF), an organization that brings together IPF patient associations across Europe, about some of the challenges facing IPF patients and their carers.
Liam has experienced the shocking impact of IPF first hand. After losing his wife and her two siblings to the disease, he has been actively engaged in raising awareness and creating better treatment and care environments for patients. His work with the EU-IPFF involves encouraging European countries to follow the IPF Patient Charter. The charter makes recommendations to healthcare policy decision makers on actions needed to improve IPF patients’ quality of life, as well as to support the continued development of long-term treatments and ultimately a cure.
Diagnosed with a deadly disease
Not knowing what to do upon diagnosis is a fundamental issue for IPF patients. Liam notes that with insufficient information and support available, patients can be easily overwhelmed.
“Often patients feel relieved when they hear their diagnosis is not cancer or some other disease they perceive as more serious. This relief does not last long and what follows is a strong sense of uncertainty. Because IPF is a rare and unknown disease, patients struggle with this diagnosis - they do not know what they are dealing with. Many patients ask for a prognosis and what they can do to prolong their life.”
It is important for physicians to have an honest conversation about what to expect and to provide suitable disease management options. “A multi-degree approach with medical but also non-medical treatments like oxygen therapy, pulmonary rehabilitation and psychological counselling can help patients maintain quality of life and do what they love for longer.”
Delaying disease management
With the low life expectancy of IPF, time is of critical value to patients. Liam notes that one of the biggest hurdles is the delay for accurate diagnosis and disease management. It is important that disease management starts as early as possible following diagnosis to slow disease progression, because the damage to the lungs caused by the disease is irreversible and patients cannot get back what they have lost.
“It can take more than a year between first seeing a GP and the referral to a specialist for a correct diagnosis,” Liam said. ”Even after the diagnosis, patients often experience treatment delays due to a lack of resources or logistical issues.” Liam also explained that there are often few or no specialty centres in the patient’s vicinity. Low mobility and, for some, lack of personal resources makes travel nearly impossible, causing further postponement of appropriate disease management. These are among the types of challenges that the EU-IPFF is working to address to help improve care for patients with IPF.
Hope for IPF patients
Liam is hopeful that improvements are on the way. He recalls a patient who volunteered to be part of a patient study for medical students for diagnosis practice. Initially, none of the students would accurately make a diagnosis of IPF, but in recent years knowledge has increased and has led to students beginning to recognise the disease. But Liam is convinced there are other improvements on the way as well.
Things have moved a lot in recent years. Up until five years ago there was no treatment and now with approved treatments available, the collaboration between countries for better care and increasingly informed healthcare practitioners, there are more options to help patients maintain their independence and quality of life for as long as possible.
At Roche, we believe that ensuring better quality of life for patients and empowering patients and caregivers is an essential part of our work. We therefore endorse the patient driven IPF charter and are committed to address unmet needs for IPF patients.