Dr. Nasreen Khalil has dedicated her research career to unraveling the mystery of idiopathic pulmonary fibrosis (IPF) and finding effective treatments. She leads a team of researchers at the Vancouver General Hospital who are working to develop a treatment to stop IPF in it tracks. Idiopathic pulmonary fibrosis affects an estimated 30,000 Canadians and there is no known cure – yet.
What exactly is idiopathic pulmonary fibrosis?
Pulmonary fibrosis is one of a family of related diseases called interstitial lung diseases that can result in lung scarring around the air sacs. As the lung tissue becomes scarred, it interferes with a person's ability to breathe.
Essentially pulmonary fibrosis severely impairs important functions of the lung, causing the person who has the disease to develop shortness of breath, and over time becomes dependent on oxygen from oxygen tanks. Further, because the lung scar tissue that forms is very stiff, it becomes hard for the lung to naturally expand in order to breathe in good oxygen. This leads to great discomfort and minimizes a patient’s ability to be active.
How common is idiopathic pulmonary fibrosis?
It is believed idiopathic pulmonary fibrosis occurs in roughly 10 to 35 per 100,000 people. And, if we combined all other types of pulmonary fibrosis, we are looking at a slightly higher number per 100,000.
We often hear it takes as much as two or three years for doctors to diagnose the disease. Why?
Most people who are suffering with a health problem begin by visiting their family doctor. Consider how much a family doctor has to know in order to determine the source of health problems in every part of the body and in patients of all ages. It can be difficult. However, once a family doctor suspects a serious underlying lung condition, they will refer their patient to a lung specialist. And then, if the lung specialist suspects pulmonary fibrosis, they will refer the patient to one of two centres in British Columbia that specialize in pulmonary fibrosis diagnosis, decision-making and management. These centres are both located in Vancouver, one at Vancouver General Hospital and the other at St. Paul’s Hospital.
Can you speak to the importance of exercise and weight control in pulmonary fibrosis patients?
My moto is ”the less you do the worse you get and the more you do the better you get – that is as long as your oxygen levels are good enough”. I cannot stress enough the importance of good fitness and having a body weight as close to your ideal body weight for your height as possible.
What kind of research is being conducted currently with regard to pulmonary fibrosis?
My own research is involved in finding out what makes pulmonary fibrosis tick, especially idiopathic pulmonary fibrosis. Simultaneously, my team and I are working to develop a drug that stops the disease in its tracks.
Essentially, there are two types of research. One is to determine how we can help improve patient quality of life and what kinds of things a patient can do to slow disease progression. This research requires the participation of patients living with the disease as study subjects. The other kind of research is the kind I’m involved in. My team and I study the cells in the lung of people with the disease to uncover the cause and what makes the disease progress so we can learn how to stop it.
What have you learned about the disease through your research?
We were the first people to confirm pulmonary fibrosis and idiopathic pulmonary fibrosis had nothing to do with inflammatory cells, but is instead the disease was caused by abnormalities in the cells of the air sacs in the lung themselves. This is perhaps due to an individual’s genetics.
This discovery caused a huge change in the way we look at the cause of pulmonary fibrosis. Drug design was also influenced by this discovery. Drug development efforts are now focused on creating treatments capable of stopping abnormal cell tissue development in the first place.
But while we are making progress, the reality, unfortunately for the many suffering from pulmonary fibrosis today, is that it can take years before an idea in a researcher’s mind makes its way onto paper, is broadly accepted as known fact, and further informs the development, approval and availability of new and more effective treatment. We’re working hard to better understand and find a cure for pulmonary fibrosis, but we’ve got a ways to go yet.
To learn more about IPF and Dr. Khalil's groundbreaking research, read Dr. Khalil's full interview here.
Dr. Nasreen Khalil has a Bachelor of Medical Science, a Doctorate of Medicine and Fellowships in Internal Medicine and Respiratory Medicine as well as research training in cellular and molecular biology of the lung. Dr. Khalil is particularly interested in interstitial lung diseases and has commenced a clinic at the Vancouver General Hospital dedicated to the diagnosis and management of patients with interstitial lung disease, as well as being recently recruited to the University of British Columbia as a Respirologist. In addition, Dr. Khalil, who was the Associate Director of Tuberculosis Control at the British Columbia Centre for Disease Control from 1999-2002, has extensive experience in diseases caused by Mycobacteria tuberculosis and atypical mycobacteria.