Esbriet Brings Hope to Those with Idiopathic Pulmonary Fibrosis

Implications of the IPF treatment for caregivers and patients.

Patients with idiopathic pulmonary fibrosis (IPF) can breathe a sigh of relief as the results of a recent study reveal that the drug Esbriet can slow disease progression and help with symptoms of the disease.

The results have major implications for patients with IPF, a serious and deadly disease that wreaks havoc on the lungs, scarring them and damaging them beyond repair, many times leaving patients waiting on transplant lists. For the first time in medical history, patients have medications available to them that can help them combat this deadly disease.

In an interview with Specialty Pharmacy Times, Dr. Marlies Wijsenbeek discussed Esbriet at length and the implications the medication has for both patients and caregivers. Dr. Wijsenbeek also discussed the possible side effects and adherence issues that could arise with use of the medication, as well as additional findings from the Esbriet study.

Patients should ask their physicians for guidance as to whether or not Esbriet may be a viable treatment option for them.

SPT: Can you discuss the ongoing developments and research regarding Esbriet?

Wijsenbeek: As you are probably well aware, Esbriet has been for some time now available in most of the countries. So at this moment, there is increasing evidence about how it works in real-time with patients with IPF. These data are collected in registries and will give us insights in how real-time behavior is.

On the other hand, there is also a need to see if there is an extension of the indication for Esbriet, if possible, because if you hypothesize that it works in one fibrotic disease, you could also think that it might work in other progressive disease, like for instance, collagen vascular disease, pulmonary fibrosis or more undifferentiated fibrosis. So I think currently most of the research will be aimed at seeing if more patients could benefit from these drugs.

Also, I think that there will be research into how the drug can be administered more efficiently. It’s now a 3 times 3 pill. So I think most of the research will be focused on these things.

SPT: What implications does Esbriet have for patients with IPF?

Wijsenbeek: Major I think, because as you are well aware, IPF is a devastating disease, a deathly disease with an average survival of 3 to 5 years. So that there is a drug available now that slows down disease progression and increases survival is, I think, extremely hopeful and there’s also a second drug available. So for the first time in years and years of research there are drugs that can actually improve patients’ survival. But we’re not there yet. Because still the patients, they will live longer, but they still die, it still remains a deadly disease.

So more research is needed to improve the situation for these patients. And also I think we have to learn more about how to improve quality of life for the patients and relieve symptoms. So still there is a lot of work that needs to be done.

SPT: How does the nausea associated with Esbriet affect patients’ adherence to the medication? How are researchers working to resolve this issue?

Wijsenbeek: We see that in a group of patients, Esbriet can give nausea or loss of appetite. I think there is a bit of a cultural difference in how it is perceived. What we usually try is to instruct patients that if you take the pill during the meal instead of 3 at a time, it usually already helps. It also helps, for instance, if there is a special moment when you have a lighter meal that you have more side effects, it will help to reduce the dose so that it is very patient tailored use of medication.

In the vast majority of patients, it will result in better management of the side effects and a very limited amount of patients will still have the side effects and will have to stop because of the side effects. With management that will work. Also there are some indications that in some countries we have less side effects with nausea than in other countries, so maybe dietary things will influence that. So there will also be research directed at these things.

SPT: What factors go into nausea affecting people differently in different countries?

Wijsenbeek: We haven’t researched it well enough, but talking to each other and seeing what everyone comes up with, we have a feeling that maybe a Mediterranean diet with olive oil and everything might have a different interaction than more in the Northern countries where we have more animal fats in the diet and less olive oil. But that needs to be established. And probably other dietary measures can also play a role but we are looking at that.

SPT: How do you feel that timing plays into the effect of Esbriet on patients with IPF?

Wijsenbeek: I think timing is crucial because we still unfortunately get patients referred in a very severe state of the disease and it’s quite disappointing, then you think if we had seen this patient earlier we might have been able to do more for the patient. The current idea is to start quite early with the medication.

There are not direct trials showing effect of Esbriet in very early and well-preserved long function, but what is shown is that in the studies that have been performed, the patients with the better long functioning in trials have a comparable effect than the patients with worse pulmonary function. So there is no reason to think that patients who still have a very mild disease would not react on the drug. You can imagine if you are a patient and you have this diagnosis that you’d rather have your medication presented as early as possible to slow it down as early as possible than to wait until you actually get worse to start medication that slows down disease progression.

SPT: What implications do the results of the Esbriet study have for specialty pharmacist?

Wijsenbeek: I think it’s very important that there is an awareness for this disease. Not only for the treatment of it, but it has long been thought that if you have IPF that there is nothing to do. But it’s very important to realize that there is now medication that can actually improve their survival and can improve the slowing down of their disease, so awareness of the disease is crucial. Also I think it’s crucial that patients are referred to centers that have some experience with these drugs, because the side effects of drugs is something you have to learn and the more patients you treat, the better you will know.

Patients are in need of more than medication, they also need counseling. A minor group will need pulmonary transplants. So there’s a lot more to treating a patient with IPF than only giving pills.

SPT: Besides just administering the prescription, what else do caregivers also need to be aware of in treating patients with IPF?

Wijsenbeek: I think people should realize what an impact a diagnosis of IPF has on a patient and on their family. You need counseling for that. You need support when the patient needs that, and you need to discuss with patients what they want and what they feel, so that is more the emotional part of support. You see with patients that there is an enormous need for information and wanting to know things. So I think we still have gaps in our care that we should have good information available for patients.

Also, I think that treating physicians should be aware that rehabilitation physical therapy might be beneficial for patients’ quality of life. Supplemental oxygen will definitely improve quality of life and having a good system delivered and access to that is crucial I think. I think new supporting is very crucial and having patients be compliant on the medication and managing side effects. I also think that timely referral to a transplant center for patients that are eligible for transplant is also crucial, that they are not too late to get on the transplant list. I also think that, because it’s still a deadly disease, that palliative care should be talked about and end-of-life issues should be discussed timely.

SPT: What do patients need to be aware of prior to agreeing to treatment with Esbriet?

Wijsenbeek: I think that patients should realize that it slows down disease. I don’t think they should be so afraid of side effects, because if you read about other medications you also have side effects. Sometimes you have the feeling that patients are a bit hesitant to start. But we also see that, in reality, three-fifths of patients actually don’t report side effects at all and tolerate very well, and then you have two-fifths who have side effects.

Only a small group between 15 to 20% actually discontinue medication because of side effects. I think that is something the patient should discuss carefully with the doctor that it’s usually quite well-tolerated and you should not be afraid. They should also be aware that this drug is not a wonder drug. It slows down disease progression, but patients need to be realistic. Because some patients expect that if you take a drug it will cure you and obviously that is not the case. It’s quite hard to handle if you do not have the right expectations beforehand.

SPT: What do physicians need to know about the importance of patient adherence to Esbriet?

Wijsenbeek: I think the first month of treatment is crucial, because the dose will be increased to the maximum dose and that is the time that side effects will develop and you can actually help patients in dealing with that and getting better. Also, the side effects of loss of appetite and nausea can be very well-managed by dividing the doses or lowering one dose and going slower up. So I think that’s very important to discuss beforehand, and to have a number or an email where the patient can actually reach the doctor to discuss problems.

Sometimes you have an issue where you have some patients where it’s not completely clear with the current guidelines if they qualify for having IPF. I would suggest that patients always discuss with an expert center because there might be changes in the international guidelines and some patients might benefit from medication.