It must be one of the hardest aspects of having idiopathic pulmonary fibrosis (IPF): breathlessness. The difficulties with breathing account for a poor perceived quality of life and a significant restriction of daily activities. Not surprisingly, effective management of breathlessness is a major goal of IPF patient care. But what is the current state of affairs in this challenging field? Researchers gave a summary in the journal called Expert Review of Respiratory Medicine.
What exactly happens in the body when you are out of breath because of IPF? This has to do with a 'mismatch' of neurological signals and mechanical response. Your nerves tell your lungs to work harder (the 'neurological signal'), but because of the IPF, your lungs are unable to respond appropriately. The mechanical response (the widening of the lungs to suck in the air), is not strong enough. As a patient, you are aware of this mismatch and that increases the sensation of unsatisfied inspiration. This mismatch happens primarily during activity, which causes you to limit your activities and negatively influences your quality of life.
Because of scarring in the smallest parts of your lungs, it's harder for oxygen to travel from your lungs into your bloodstream
But your lungs also have to work harder when you are at rest. Because of the IPF, your lungs become 'stiffer', which makes it harder to expand your lungs, and the air is blown out of your lungs faster than you would like. A third thing that happens in lungs with IPF, is something called 'impaired gas exchange'. Because of scarring in the smallest parts of your lungs, it's harder for oxygen to travel from your lungs into your bloodstream. All these things cause patients with IPF to adopt a more rapid and shallow pattern of breathing.
So, are we able to tackle breathlessness in IPF? The researchers mention a number of treatments. Unfortunately, evidence for the treatments positively influencing breathlessness in IPF, is rather weak. As a result, the researchers argue for more carefully designed studies. These studies should determine the efficacy of interventions in the management of IPF. Hopefully they will contain strong clues for how to combat this serious symptom.