Improvements and Obstacles: How Is IPF Treatment Advancing?

Patrícia Silva, PhD avatar

by Patrícia Silva, PhD |

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Sixty-six year old Dan Castner was living life to the fullest when he started experiencing difficulties in breathing. While he worked in the outdoor industry for more than 40 years and was used to biking, climbing, camping, skiing and hiking, in March of 2012 his life completely changed. Castner was diagnosed with Idiopathic Pulmonary Fibrosis (IPF), a disease that deepens the tissue and makes lungs thick, stiff and even scarred.

Following a hiking trip in Lake Tahoe, Castner came to realize that his lungs weren’t working properly and sought professional help. “That was the second time I heard about the disease,” he explained in a recent interview. His mother had also died suffering from the same disease 20 years earlier, and it wasn’t easy for him to deal with the diagnosis or discuss it with his family. “I want to be seen as who I am, and not based on the disease I have,” he added. In addition, when he started researching the disease on the internet, he thought he had only about two years to live.

Despite Dan’s initial fears, life expectancy for people who suffer from IPF has in fact increased over the past decades, and on average patients live about five years after being diagnosed. Last year, the U.S. Food and Drug Administration (FDA) approved the use of Esbriet (pirfenidone), from Genentech, and OFEV (nintedanib), from Boehringer-Ingelheim Pharmaceuticals, Inc. as treatments for IPF, which have substantially contributed to an increase in survival and quality of life for those with the disease, as Esbriet and OFEV are the first therapies approved specifoically for the treatment of pulmonary fibrosis.

The Impact Of New Treatments

“Both drugs reduce the disease progression, and they do it by about 50 percent. It is a great first step and they offer patients hope,” explained Dr. Imre Noth, a pulmonologist specialized in interstitial lung diseases and a reputed IPF researcher. “These drugs can help patients extend their lives, but I tease my patients by telling them my objective is not to make them live forever, it’s to make them leave better.”

There is currently no cure for IPF, and like many other patients, Dan Castner needs to take multiple pills each day in an attempt to manage the disease. However, the approval of Esbriet and OVEF represent major advancements in patients’ quality of life, as explained by Dr. Noth. “We want to make sure that people like Dan can ride their bicycle,” a health goal that involves pulmonary rehab, improving lung function and reducing the burden of the disease’s symptoms.

Two years after the diagnosis, Dan Castner is now retired and despite being aware of the disease, living with the possibility of lung complications at any time, and not being able to ride a bicycle anymore, he is otherwise able to live a normal life thanks to his medication. In addition to treating the more than 100,000 people who suffer from IPF in the United States, having access to IPF-specific medication also addresses the problem of managing lung transplant waiting lists.

“The reality is that in 50,000 cases a year, transplants are about 3,800, which IPF represents the largest group — about 40%,” continued Dr. Noth on the use of lung transplantations, which are one of the most common options for treating patients with IPF. However, like Dan Castner, most IPF patients need to wait years for a new lung. “The improvement of quality of life [with medication] is a reality, while lung transplant is a pretty serious business, it involves a lot of medication and a serious commitment from the patient,” added the pulmonologist, emphasizing the advantages of drug treatment.

Limitations in Improving IPF Patients’ Lives

However, not everything is positive in terms of IPF treatment advances, and there are also some negative aspects to the two new drugs as well as obstacles for advancing the field of IPF therapeutics. Lack of resources is the number one problem identified by both the physician and patient in the interview: increasing financial support would make the drugs more affordable and increase reachability. The cost associated with both OFEV and Esbriet is about $94,000 per year.

The amount is too high for the majority of the patients, but the burden is eased by Medicare and insurance companies, which help cover part of the drugs’ costs. However, it may not be enough, and Dr. Noth believes that the “patients are only receiving medical coverage because the system would look like the bad guy,” while there is still a lack of public investment in IPF treatments and research. When asked about the greatest obstacle in the field, Noth has no doubts: “Money.”

Misdiagnosis is also a limitation, since symptoms like shortness of breath can be confused with other lung diseases, including emphysema or COPD. Misdiagnosis, which can last years, results in a delay of treatment and consequent decrease in quality of life. “Going to an ILD [Interstitial Lung Disease] center actually translates into improved survival” and studies show that patients who visited an ILD center sooner “live longer, even if they have the same category of disease.”

In addition to these problems, there are two other obstacles in the way of improving quality of life of IPF patients: age and stigma. Patients are usually diagnosed after the age of 50, and the probability of developing IPF increases with age. However, Dr. Noth believes physicians need to pay more attention to other diseases as well. “Patients with IPF have higher rates of coronary disease, pulmonary embolism, lung cancer, reflux disease, sleep apnea, and a variety of other systemic problems. A lot of doctors end up ignoring them,” since the odds are that the patient will die of pulmonary fibrosis first.

“It turns out it does matter and a third of the patients die of comorbidities,” he added, noting the importance of patients with other diseases to participate in clinical trials in order to advance treatment. Prejudgment is also a problem, with smoking being one of the biggest issues. Patients don’t want to be associated with a lung disease that is the product of smoking, which can result in a delayed search for a physician and treatment or in social exclusion.

How To Overcome Obstacles

In order to overcome the difficulties associated with IPF, the doctor and the patient agree that there are two main approaches: improving prevention and diagnosis, and raising awareness and funding, both of which are directly connected. “There are certain ages we all know we need to get a colonoscopy or a mammogram, but nobody is doing that with IPF,” believes Dan Castner. Dr. Noth added that the lack of prevention is a major issue for improving IPF diagnosis, but he noted that “in a decade and a half, we have seen a relative increase in data and that makes all the game different.”

“We have now a far greater understanding of the genetics of the disease,” added the pulmonologist, explaining that there is a certain genetic predisposition that in addition to lung injuries are major risk factors for IPF. The discovery of the genomics related to the disease helps in the development of new and improved treatments, as well as in the improvement of diagnosis. Dr. Noth believes the future resides in the use of CT-scans not only to detect lung cancer, but also IPF.

In fact, the comparison between cancer and IPF does not end there. While lung cancer kills about 157,000 people annually in the United States, about 40,000 people die each year from IPF — the same as breast cancer. However, the attention given to the two diseases is quite different. “I have been on bike rides for cancer, but there are no bike rides for IPF,” added Dan. Similarly to funding, raising awareness about IPF may not only increase funding for research, but also improve diagnosis.

While IPD organizations, advocates, physicians and patients work towards this goal, one of the most critical steps is National Pulmonary Fibrosis Month, which is celebrated in September, and National Healthy Lung Month, which is held the following month. During the observance designated by the American Lung Association, activities will be organized throughout the country to promote awareness of maintaining healthy lungs, which is crucial for a good quality of life, happiness and overall health.

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