Esbriet (pirfenidone) found to reduce IPF progression in all disease stages

When pirfenidone (Esbriet) was first approved for IPF, there was some concern initially that pirfenidone may be best suited for patients with mild to moderate disease.  A new study, “Efficacy of pirfenidone in patients with idiopathic pulmonary fibrosis with more preserved lung function,” was published in the European Respiratory Journal in 2016.

The study compiled outcome data from all three of the major pirfenidone Phase-III trials. The researchers re-analyzed the pooled data by stratifying patients based on their base-line lung function at the start of these clinical trials and compared the outcomes between patients with mild and more severe IPF at the beginning of these trials. Results from the study showed that the effectiveness of pirfenidone was independent of the patient having a more preserved or less preserved lung function at baseline. The researchers concluded that “These findings support the initiation of treatment with pirfenidone, irrespective of stage of baseline lung function in this patient population.” This gives us more confidence that pirfenidone is similarly effective in moderate to severe IPF stages and should be used in all patients with IPF. Continue reading

What are the most effective therapies for pulmonary fibrosis?

Idiopathic pulmonary fibrosis (IPF) is a devastating condition where the lung tissues progressively thickens and turn into scar tissues leading to breathlessness and ultimately respiratory failure and death. The treatment for IPF has changed drastically over the past few years. Traditionally, IPF were treated with high doses of corticosteroids in combination with other medications to suppress the immune system. New medications, pirfenidone and nintedanib, have made it possible to slow down the progression of the condition. Other supplemental agents such as N-acetylcysteine (NAC) has often been used as an adjunct therapy in pulmonary fibrosis. We often get asked about what the effectiveness of different therapies in pulmonary fibrosis. Continue reading

Pirfenidone related photosensitivity – Prevent sun burns this summer

Pirfenidone (Esbriet or Pirfenex) is one of the few medications that effectively treats and slows down the progression of idiopathic pulmonary fibrosis (IPF). While this medication is generally well tolerated, pirfenidone, like many other drugs, can increase the chance of a condition called photosensitivity. Photosensitivity means the skin is more sensitive to sun lights. People with photosensitivity can get sun burns more easily than usual and even have allergic skin reactions to sun light. We have had many patients after buying pirfenidone from our Canadian pharmacy service who called us with a skin rash thinking they were having an allergic reaction to pirfenidone, but in fact they were having photosensitivity and the skin rashes were results of unprotected exposure to sun lights. Continue reading

What You Might Not Know About Pulmonary Fibrosis

The air you breathe is essential to your long-term health, but for some people it’s difficult to get enough oxygen into your bloodstream. If you suffer from pulmonary fibrosis, you know that it’s a condition that limits the air that can get through your lungs and into your blood because the tissues around and between air sacs in the lungs thicken and scar over time. The shortness of breath and difficulty getting enough oxygen get worse over time, causing significant fatigue.

What you might not know is that the scarring you experience that leads to thickening of tissues over time could actually be caused by several different factors in your environment, such as airborne toxins, lung diseases, radiation, or medical treatments. It can be difficult for healthcare professionals to pinpoint the exact cause, which leads to a diagnosis of idiopathic pulmonary fibrosis (IPF). Continue reading