By Kaye Estoista-Koo
There is a silent killer in our midst.
Slowly, but surely, a growing percentage of the population suffers from abnormal wound healing in the lungs. This eventually leads to respiratory lung failure, in some cases even lung cancer and, in most cases, it takes years before it is even discovered.
Many end up dying from it without knowing what really killed them in the first place.
What is its name? Idiopathic Pulmonary Fibrosis or IPF is a category of diseases under Interstitial Lung Diseases or ILD.
Dr. Dina Diaz, a pulmonologist with a sub-specialty in Pulmonary Medicine (Adult Pulmonology), Occupational Lung Diseases (including Interstitial Lung Disease), and Respiratory Physiology, is on a mission to spread awareness about this silent but deadly killer.
She wants to raise awareness because there is really no known way to prevent it, hence the name idiopathic.
Filipinos especially need to be more aware because in general, we have bad health-seeking behavior.
Know When to Worry
“Health is at the lowest level of priority. When you have money, extra or not, you won’t think immediately about getting an x-ray or a check-up. Only when you’re really sick, that’s when health becomes a priority,” she says.
She even notes in jest that there is a certain month, every year like clockwork, that her patients increase: the time when weather changes and allergens abound. That is the time she sees her once-a-year patients called asthmatics. She adds that more often than not, they stop taking medicine and maintenance the moment the asthma symptoms stopped. Dr. Diaz recommends for asthmatics in remission, meaning those who have not had attacks in three years or more, to continue taking the maintenance because asthma can come back any time.
Interestingly, Dr. Diaz became a pulmonologist because she comes from a family of asthmatics. She knows what she is talking about.
This is the most surprising insight: Many patients who have been treated for pneumonia or tuberculosis are possible misdiagnoses of IPF.
Dr. Diaz explains, “The presenting clinical manifestations are the same like any other pulmonary problem such as difficulty of breathing, shortness of breath, and chronic cough.”
She says IPF is more difficult to diagnose as it is not common, meaning there is not enough supporting data on it because people don’t know they have or had it.
“It is unrecognized unfortunately even by the specialists, it is always misdiagnosed as pneumonia. For example, if treatment is done for pneumonia and their symptoms keep coming back, they end up having several admissions for pneumonia or being treated for TB and feel bad because they are not getting better,” she says.
The problem with the difficulty in diagnosing it also lies in the kinds of tests done, as tests to rule out pneumonia, TB, and other ILDs are the same.
IPF, a debilitating and fatal lung disease, causes permanent scarring of the lungs, which results in difficulty of breathing.
“IPF is progressive-causing tissue damage in the lungs,” explains Dr. Diaz. “Interstitial is a tissue between your airways and blood vessels in the lungs and gas exchange should happen between these two but once this area is damaged, gas exchange is affected. It’s basic oxygenation.”
This build-up or fibrosis causes decrease in lung function, which results in decrease of amount of oxygen that goes around from the lungs to all the major organs of the body.
Given the number of years that she has now studied cases with IPF, Dr. Diaz can help diagnose when a patient has IPF using the same processes and procedures that her fellow pulmonologists use. She just knows what she is looking for.
Left untreated, she says a patient misses out on “proper treatment and supportive treatment, including preventive methods like avoiding infections to not add injury in the lungs. New treatments can slow it but it cannot be cured because it is progressive.”
Dr. Diaz is also adamant that more information on this health condition be spread because while it does target older patients more, chronic coughing can be caught early. “The danger of not being diagnosed is it becomes fatal if the case really is IPF and a patient is getting bad or wrong treatment that may hasten and can do harm. Patients can get treatment for pneumonia and be diagnosed late, but they don’t get the appropriate supportive treatment.”
Another reason that it is vitally important that IPF be diagnosed properly is because when patients don’t know what they have, they panic and turn to Dr. Google. In these cases, the Internet does not help at all.
Dr. Diaz, who was the first council head of the Council of Interstitial and Occupational Lung Diseases back in the ‘90s, is also a member of the Philippine College of Chest Physicians.
They have been studying and continue to study and focus on these diseases and, to date, they are still trying to increase awareness.
For her, red flags that signal something other than the usual ILD is when “you don’t get usual results from usual treatments, it must lead you to thinking if you really have that or something else so if you are not responding and it keeps coming back it is something else.”
Currently, Dr. Diaz heads the Department of Pulmonary Medicine at the Lung Center of the Philippines and says that many patients do come in because of referral basis. She wants to remind the public that if your x-ray doesn’t show improvement, watch out. “Under ILD some are treatable, some are uniformly fatal whatever you do, and IPFs, which account for 51 percent of the known ILD is considered uniformly fatal,” says the doctor. “You end up with fibrosis and you lack oxygen and at same point, lung scarring, so you can’t breathe anymore and you go into lung failure.”
Are you at Risk?
While there is no singular reason to be predisposed to IPF, again, it is idiophatic, there are high risk factors to consider and be mindful of, just like any lung disease: smoking, old age, and other causative factors which include occupational exposures, radiation, and drugs.
Since IPF is abnormal wound healing in the lungs, the fibrosis also predisposes one to cancer or heart issues.
She adds that for those who have IPF, they could still be functioning with a lung function that is more than 50 to 60 percent but these are hard to find or spot. “They may get more breathless than usual, or experience non-stop coughing, like a dry cough.
There are those who rapidly progress within three years of diagnosis and end up dying. And then there are those who stabilize but are still breathless every day. And some go into slow decline every year with a decreased lung function,” she says.
Dr. Diaz adds that while you can’t be cured from IPF, quality of life improves once you know what you have and you know what to avoid. Regular monitoring helps, along with a measure of pulmonary rehabilitation, to teach you how to manage breathlessness.
“We are hoping that now that treatments are available, more awareness and health-seeking behavior will happen. Treatments may trigger increasing awareness,” she says.
One of the new available treatments in the Philippines is called Nintedanib.
Dr. Diaz explains that Nintedanib can be measured objectively as it was made to specifically reduce progression in decrease of lung function as an objective measure by managing lung function at 60 to 70 percent and stabilizing it.
It has just been introduced and this targeted drug receptor directly involves itself in the abnormal fibrosis and helps it from progressing quickly.
Nintedanib with the specific name OFEV was developed by Boehringer Ingelheim and has been approved in the US, European Union, and Japan. It is given as an oral treatment in capsule form, twice daily.
She recalls that before Nintedanib became available, her most notable cases of patients with IPF didn’t have it easy.
She had one who came in, a patient who went through three years of suffering with multiple admissions.
Another one, her now longest-living IPF patient, was diagnosed in 2007 or 2009, and is now oxygen dependent. She shares how he has weathered all the problems because he is very positive and he has a loving wife by his side. In IPF, family support is critical.
Another case, this one a referral from the province, came to her after spending a lot of money and seeing a lot of doctors. He wasn’t left with much by the time he came to her. “When he came to see me, he said, “All I need is for you to tell me what I have,’” she recounts. “So when I broke the news to him, that his lungs were that bad, he was happy because he finally got a diagnosis. He knew what was happening to him.”
Living on Borrowed Air
Dr. Diaz remarks that disclosing IPF is like disclosing cancer when you have to tell a patient that you are not going to get better. Barring a lung transplant, which is the only guarantee that you will get better, IPF patients live on borrowed air, so to speak.
Of course, any time a patient finds out they have IPF and once it is explained to them, Dr. Diaz says they respond the same way anyone who gets news of a terminal illness.
Lung transplants are not yet available in the Philippines, but IPF patients can hope to retain lung function at 70 percent with the new treatments available.
While it can be genetic or familial, it is but a small percentage of the diagnosed population.
Dr. Diaz gives the following important pointers: “The first thing is if you have a family member who you think may have had this, get checked. If you have progressive shortness of breath that doesn’t seem to be improving and coughing that doesn’t get better for months, meaning it is chronic, get an x-ray. Any other lung condition should be gone in two weeks. Some people think it is part of aging when they start to cough.”
In most of the cases, IPF has been diagnosed in middle-aged men but it doesn’t mean women are off the hook. That is why Dr. Diaz does an eight-page questionnaire as it takes her a long time to process any referral, followed by a lot of work-up and two more consults.
The Royal Brompton Chest Hospital in the United Kingdom, the equivalent in the Philippines being the Lung Center of the Philippines, employs a multi-disciplinary team approach. Dr. Diaz says that in the Philippines we are just starting to copy the same approach when it comes to treating IPF.
In Royal Brompton, they have 1,000 cases every month which they can confer on, look at the cases, read the x-rays and CT scans, and decide if a case is IPF. In this approach, the pulmonologist is joined by the radiologist and in some cases the histopathologist if there is a need for biopsy.
She explains that in the United Kingdom all the centers refer because everyone is aware of it: “We want to put up something like that and train other hospitals to put up their own so that someone is always looking into the possible IPF cases.”
She has started by organizing case-based sessions so that more and more doctors are used to seeing cases and reading scans of IPF cases. In this way, they can all learn together and teach each other. Diaz says all CT-scans end up looking the same, so if it’s all interstitial, the attending clinician or pulmonologist can tell the radiologist his or her suspicion and they can discuss, sit down, and look at the cases, working together to figure out a course of action.
The last say will of course have to be the pulmonologist as he or she is the one who interfaces with the patient but at least, in this way, more eyeballs are looking at cases.
Aside from the Nintedanib treatments, they also recommend exercise tests and the Bi-Lung function test, which is a Pulmonary Function Test to check breathing by looking at actual numbers.
She can’t repeat enough the reminder to consider IPF or any ILD whenever a patient is not responding to treatments for a pulmonary problem and to consult a specialist or a center because this may be what you have. To Dr. Diaz, awareness and recognition is key.
“Get an x-ray some time in your life, especially if you are breathless and coughing. Or take a Lung Function Test. Yes, the elderly are more prone, especially the middle-aged males. The usual preventive measures would apply like not smoking but since IPF is idiopathic, it can happen to anyone as it is a reaction of your lung to injury. There is no evidence it is airborne! IPF is a rare disease in the lungs, not easy to recognize, so be more aware of the status of your health.”