Unscripted - Understanding PH-ILD: Pulmonary Hypertension in Interstitial Lung Disease

00:00:00:00 - 00:00:29:04
Welcome to unscripted The Amcp podcast, a look inside managed care pharmacy. Listen in as experts explore the challenges, innovations and opportunities shaping health care for millions of patients. This episode of Unscripted, The Amcp podcast is sponsored by United Therapeutics. To quote Martine Rothblatt, CEO of United Therapeutics, United Therapeutics finds corridors of indifference and then runs like hell down them.

00:00:29:06 - 00:00:54:07
Today, we're focusing on a serious yet often under-recognized condition pulmonary hypertension. In interstitial lung disease, or PHILD. This complex overlap of two life altering conditions demands early recognition and a multidisciplinary approach. Our guest today is Franck Rahagi, executive director of Cardiopulmonary Global Medical Affairs at United Therapeutics. Welcome, Franck. Thank you very much. Yeah, it's a pleasure to get you on.

00:00:54:08 - 00:01:30:01
Why don't we begin with this sort of fascinating origin story for United Therapeutics? Yes. Our founder and CEO was involved in the field of satellite communication. In fact, Sirius satellite, which is known to many people, she was struck by a bad news of her daughter being diagnosed with pulmonary hypertension, which at that time was a fatal condition. She did not accept that poor prognosis and basically since then has dedicated her life to finding a cure for pulmonary hypertension.

00:01:30:05 - 00:02:02:04
She started United Therapeutics, her company, and since then we have continued to come up with new products. And in addition, a major part of our company efforts is in organ manufacturing and artificial organs. Fascinating. So can you sort of introduce us to pulmonary hypertension and interstitial lung disease? Yeah. So interstitial lung diseases are conditions where the lung develops inflammation and scarring or fibrosis.

00:02:02:06 - 00:02:39:21
It's an umbrella diagnosis that includes many different subtypes, including idiopathic ones, the ones where we don't know where it came from with, the major member being idiopathic pulmonary fibrosis, those related to connective tissue diseases such as scleroderma and those related to environmental factors, such as chronic hypersensitivity pneumonitis and others. The case of pulmonary hypertension, it's essentially the blood vessels of the lung tightening up and resulting in increased pressure.

00:02:39:23 - 00:03:15:07
That is then seen as pulmonary hypertension, a pressure that limits the patient's abilities and activities and eventually leads to right heart failure and demise. So what happens when you put these two together? So unfortunately, the effect is beyond additive. And patients with certain interstitial lung diseases that already have a poor prognosis, it basically halves that prognosis. And in the case of IPF and poor may have pretention it reduces to maybe median of two years.

00:03:15:09 - 00:03:48:10
And so how common is this and how common is the overlap between the two disease. Yeah. So in interstitial lung disease if you're a patient with interstitial lung disease there was a center that basically screened every single person that was referred to them. And in that referral group the prevalence was around 15%. Over time, that increases to 40% to 50% and towards advanced disease, for instance, when patients are referred for lung transplant.

00:03:48:10 - 00:04:08:23
And at that point everybody gets screened for pulmonary hypertension, it could be over 80%. So it is of significant prevalence within the ILD population. And so just thinking about this sort of from a payer perspective, obviously this kind of condition requires a fair amount of treatment. Look, and you mentioned lung transplants. How should payers look at this.

00:04:09:00 - 00:04:36:09
Obviously it sounds like it would be fairly expensive to be, helping these patients with, their care. Yeah. So the way to always look at this is for me through the eyes of the patient. And early intervention always makes a difference. And this could be done through pulmonary rehab. These are non-pharmacological interventions from a rehab psychological support oxygen and aggressive treatment of the underlying disease.

00:04:36:11 - 00:05:04:10
Or once pulmonary hypertension has been uncovered in this population, use of medications that are now available for treatment. And so when you think about this from that perspective, where would people most likely be able to get this diagnosis early enough? Does it require seeing a specialist or can that be picked up by primary care? Well, I mean, most of the patients with interstitial lung disease should be taking care of at some point by a pulmonologist.

00:05:04:10 - 00:05:36:07
So, the screening efforts have been focused on the pulmonologist looking at their patients with interstitial lung disease and noting where there is a discrepancy with the level of disease and the level of symptoms of the patient. But it's not just with the specialists. The patients themselves could also notice increased fatigue or worsening symptoms. And basically ask their doctors, hey, could this be from something else, such as former hypertension?

00:05:36:09 - 00:06:10:17
So I think the best outcomes come from, alertness by both the patient and the treating physicians. So, Franck, you mentioned some of the pharmacological treatments. What are some of the other options. And how are individuals treated for these two conditions when they're coexisting. So the pharmacological treatments are directed at interstitial lung disease. But as of late, Trupostinal inhaled has also been approved for the treatment of pulmonary hypertension in ILD patients with early improvements in walk distance.

00:06:10:18 - 00:06:57:02
So what would you say is the message when you consider from the different groups, the patient, the clinicians or the payers, what should they be thinking about or take from this? The ability to diagnose the patient early is important, and this is both a message for the pulmonologist to be aware of. Declines, unexpected decline in the patient's looking, at the Cat scans, looking for enlarged pulmonary arteries, looking for enlarged right heart, and looking for signs in the pulmonary function tests such as, low oxygen levels of oxygen exchange or discrepancy between, vital capacity and oxygen exchange as ways to, start screening the patient.

00:06:57:04 - 00:07:26:21
This happens with Echocardiograms and then subsequently right heart cath for confirmation and for patients the awareness that with increased symptoms, there's usually a reason. And the reason has to be discovered. If it's the interstitial lung disease may be treated more aggressively. And if it's a new conditions such as PHILD or pulmonary tension in lung disease, there is no new treatments available.

00:07:26:23 - 00:07:56:23
Well, I like to thank you for joining us, Franck and discussing. Thank you very much. This is a pleasure, and I'd like to thank you, the audience, for listening to this episode of unscripted, the Amcp podcast. This episode was sponsored by United Therapeutics. For more information about United Therapeutics, please go to www.unither.com.