The first and most important point is that regular evaluations of your lung function and exercise capacity are important to identify if your IPF is getting worse. Patients may feel worse for many reasons—some lung related and others in no way related to lung function. As a result, I am a believer in regular pulmonary function testing and six minute walks.
In the patient that has not yet started treatment with one of the two FDA approved therapies (OFEV and Esbriet) I start one or the other agent. I generally allow three to six months on one therapy to determine if the medicine is working. Unfortunately as these medicines do not result in improvement in lung function, “working” means stability in your lung function. At the three-month mark, I repeat a spirometry to measure the forced vital capacity (FVC) and a six-minute walk test.
If there is clear evidence of progression and the patient has been able to tolerate the full dose of the chosen medicine for the full three-month period, then we discuss changing to the other medication. I will usually schedule a short interval follow up and repeat testing to verify that the change is sustained. If it has taken a month or so to gradually increase the dose then I will be patient for another six weeks and repeat testing. If at that time, there continues to be evidence of progression then I would encourage switching to the other medicine. If all is going well then I do a full pulmonary function test and six minute walk at the six-month mark.
Currently, there is no data supporting the use of both OFEV and Esbriet at the same time. That does not mean that it might not be useful, just that no one has studied it. As a result, your insurance is not likely to pay for both drugs together. I have not yet been successful in persuading a payer to approve both drugs at once. I hope that over the next few years we are able to study this question to better guide our treatment decisions.
Pulmonary Hypertension and IPF
In many patients with IPF, the pressure in their pulmonary arteries increases. This can lead to increased stress on the right side of the heart. Over time, some patients develop progressive impairment in their right heart function that leads to right heart failure. The treatment of pulmonary hypertension in patients with IPF is a very active area of clinical research. In our practice we have a variety of clinical trials that are ongoing for such patients. In patients that do not qualify for research trials we selectively offer treatment for their pulmonary hypertension.
The majority of clinical research studies now and looking forward will be comparing an experimental medicine to placebo but will allow treatment with either OFEV or Esbriet. It is imperative that patients participate in clinical research studies so that we may make further progress in treating IPF.
In patients that are getting worse, it is important to have an early discussion about lung transplantation as a treatment option. This is not the right path for every patient and even for many patients that might be interested. The evaluation process is rigorous and excludes patients with other advanced medical problems, obesity, inadequate supports and very advanced age. Nonetheless, I still have a frank and open discussion about lung transplantation with all of my patients.
End of Life Planning
In patients that have very advanced disease and are not candidates for lung transplantation, it is appropriate to discuss end of life planning. Although this topic is scary for patients and hard for doctors, an open discussion about this is important and productive. My experience is that patients are often afraid to ask about this topic but are receptive to the discussion when offered in a supportive environment.