We received many great comments and topic suggestions recently and I will address a selection below.
Several readers asked about whether there are supplements or vitamins that have a role in slowing the progression of IPF.
If you listen to the radio, watch television or frankly are alive and have either functioning eyes or ears then you have no doubt heard the sales pitch from vitamin and supplement makers. Unfortunately, supplements are not regulated in the same way as medications. The FDA does not require nutritional supplement makers to prove that their products are effective in any way. The result is that you have to be very suspicious of the grand promises made.
Many readers have inquired specifically about Vitamin D & Pulmonary Fibrosis.
Vitamin D has been in the news frequently lately. To summarize a large field of study, vitamin D is now recognized to be important in lung health. Vitamin D deficiency is very common. Low levels are associated with many types of lung disease including pulmonary fibrosis. Unfortunately, vitamin D supplements have not been evaluated in high quality studies.
My practice is to evaluate IPF patients for vitamin D deficiency and prescribe appropriate supplementation if their levels are low. If patients would like to take an over the counter dose of Vitamin D (400-800 units per day), I have no objection. However, there is no data that there is benefit in IPF.
We continue to receive questions about how to manage pulmonary fibrosis patients who also have severe pulmonary hypertension.
This is a very complex management problem. First we have to acknowledge that we have very little high quality data to guide our decision-making. That being said, we can say with reasonable confidence that endothelin receptor antagonists (Tracleer/Bosentan, Letairis/Ambrisentan, Opsumit/Macitentan) do not have any role. That being said, there is a small amount of data that supports the use of PDE 5-inhibitors (Revatio/Sildenafil) and infused Remodulin/Treprostinil.
In my practice, I work to tease apart how severe the pulmonary hypertension is relative to how severe the fibrosis is. Next, I consider whether the patient is or could be a lung transplant candidate. If the patient has advanced pulmonary fibrosis and would be a lung transplant candidate then I would move in that direction. If lung transplantation were not an option, then I would very carefully undertake treatment with Sildenafil. If the pulmonary hypertension were severe (assessed by right heart catheterization) then I would consider adding infused Remodulin/Treprostinil.
Here are all the important disclaimers: 1) There is little high quality data supporting my strategy, 2) The medications are being used “off label” meaning that they were not approved for use in this indication, 3) this strategy may lower the oxygen saturations, and 4) Echocardiography is not accurate in estimating pulmonary artery pressure in patients with pulmonary fibrosis. Right heart catheterization is the only way to accurately measure pulmonary artery pressure and pulmonary blood flow. With those warnings, others and I have been quietly treating certain patients with pulmonary fibrosis combined with pulmonary hypertension in this fashion for years.
Looking forward, this question is finally attracting some attention from drug companies and a group of studies are set to begin enrolling patients with combined pulmonary fibrosis and pulmonary hypertension. Hopefully in the not too distant future we will have better quality data supporting a specific management strategy for this difficult problem.