Because there was no approved medication treatment for idiopathic pulmonary fibrosis (IPF) until mid-October, 2014, physicians primarily focused on non-pharmacological treatments to help patients improve. Even with FDA’s approval of nintedanib (Ofev) and pirfenidone (Esbriet), physicians will continue to recommend certain therapies that generally ease symptoms and improve patients’ quality of life.
Because there was no approved medication treatment for idiopathic pulmonary fibrosis (IPF) until mid-October, 2014, physicians primarily focused on non-pharmacological treatments to help patients improve. Even with FDA’s approval of nintedanib (Ofev) and pirfenidone (Esbriet), physicians will continue to recommend certain therapies that generally ease symptoms and improve patients’ quality of life.
One of pulmonologists’ top recommendations is pulmonary rehabilitation, a structured exercise program for people with chronic lung diseases, which is supervised by a respiratory therapist. The goal of pulmonary rehabilitation is to restore patients’ ability to function without extreme breathlessness and typically includes conditioning, exercise training and breathing exercises.
In addition, pulmonary rehabilitation incorporates nutrition counseling, education, and management of anxiety, stress and emotions.
“In Chronic Obstructive Pulmonary Disease [COPD], it improves quality-of- life measures as well as walk distance and shortness-of-breath scores. Now, there is more evidence that it helps improve IPF as well,” said Kevin Gibson, MD, professor of medicine and clinical director of the Dorothy P. & Richard P. Simmons Center for Interstitial Lung Disease at the University of Pittsburgh Medical Center.
In fact, two Cochrane Reviews stated the validity of incorporating pulmonary rehabilitation in patients with IPF, he added.
“This treatment has become an important part of the standard of care for people with chronic lung diseases, and recent studies indicate that pulmonary rehabilitation improves both exercise capacity and one’s quality of life,” according to a statement on the Pulmonary Fibrosis Foundation’s [PFF] website. Pulmonary rehabilitation programs can be inpatient, outpatient or home-/community-based.
Another effective non-pharmacological treatment for IPF is oxygen therapy. “Supplemental oxygen is very helpful at improving exercise capacity. If patients’ oxygen levels are low, they get a big benefit by using supplemental oxygen within the normal range,” Gibson said. IPF patients can monitor their own oxygen levels throughout the day using handheld pulse oximeters, available over-the-counter at many pharmacies. “Patients should try to maintain their oxygen saturation levels above 90% throughout the day and night,” according to PFF’s web site.
More frequently, physicians are also referring IPF patients to palliative care, which helps relieve pain, symptoms and stress caused by serious illnesses. “As the disease progresses and they experience symptoms such as shortness of breath – which limits their activity and causes symptoms such as panic reactions – we will often get palliative care involved in helping with those symptoms, Gibson said.
While pulmonary fibrosis is now the leading indication for lung transplantation in the United States, only about 13% of IPF patients get lung transplants, according to Gibson. “Lung transplantation is offered to patients who would be appropriate candidates for it. Some have other illnesses that make transplantation not a good idea, such as heart disease,” he said.
While it is not a solution for most IPF patients, transplantation can improve longevity and quality of life in PF patients who have no other signficiant health problems, according to the PFF. “As surgical techniques and outcomes have improved, more centers are performing transplants in individuals over age 65,” PFF stated on its web site.
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