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FAQs About Pulmonary Fibrosis/ILD

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In this video Dr. Meena Kalluri discusses some commonly asked questions received by PF/ILD patients. Dr. Meena Kalluri is an interstitial lung diseases and pulmonary fibrosis expert. Dr. Kalluri is the founder and director of the Multidisciplinary Collaborative Interstitial Lung Diseases (ILD) Clinic at the University of Alberta.

My name is mina kaluri and i’m the medical director of the multidisciplinary collaborative ild clinic at the university of alberta i think when patients are dealt with this diagnosis it’s a it’s a tumultuous time in terms of emotions um so one of the questions that i often get asked is about prognosis you know how much time do i have doc kind of thing and lots

Of patients these days are doing their research they’re reading things online and they often come back with statistics like the survival in ipf is two years sometimes it’s three sometimes it’s two to five three to five you’ll find different statistics online now statistics should always be interpreted in context so i always tell my patients that that’s just an

Average from a population data meaning lots of patients will study then they figure out an average from that so those statistics are not you you are your own individual person and your course may be very very different unfortunately we don’t have tools at our disposal that help us predict a timeline like telling you a six month time frame or a six week time frame

Or a six year time frame unfortunately we don’t have the right kind of tools in terms of uh your breathing tests i can’t just look at your breathing tests or just your ct scan or even just your symptoms but they can give me a good indication of what’s in in store so we can use a combination of these things and give you an estimate but i’ll tell you more often

Patients have surprised me so when peop when patients come in and they’re asking about prognosis this is what i tell them it’s good to be informed it’s good to read statistics but don’t be discouraged by it and remember that you are your own person and your experience your disease experience can be very very different you might exceed what what was predicted by

Those statistics three four years i’ve seen patients live far longer than that but i’ve also seen patients who who succumb to their disease earlier than that so it’s very very individualized the second thing i would say is the best way is to have an open attitude an accepting attitude i find that the patients who accept their diagnosis are then able to move on to

The next stage which is what can i do focusing on what can i do with this now and i think once you change your attitude and you become focused on the present and dealing with the present your outcomes are going to be much much better than someone who’s still struggling with the acceptance of the disease phase it’s the hardest thing to do but once you cross that

Bridge then the real healing can begin in so many ways you can start to empower yourselves about how to live well and cope well with this condition and move on with it because we can’t we can’t afford to get stuck in the initial phase having an open attitude uh positive attitude uh will change perspective whether it’s for treatments or oxygen or transplant

Or even end of life perspective matters so the world is as you perceive it so you can change your world by changing your perception so i find that patients who are able to adopt that kind of a positive attitude do the best no matter what their diseases yup so prednisone is a steroid and in general we say that ipf is a non-inflammatory condition meaning it’s a

Fibrosis or a fibrosing condition so in general we don’t use prednisone for long-term therapy of ipf but in certain situations we we do and we can prescribe steroids so one of those examples would be if you’ve had a flare of your underlying ipf to control the acute flare of your ipf your physicians might put you on a short on a course of steroids the other reason

Sometimes if you have cough low dose prednisone sometimes can be used or if rendizone is used for other purposes but in general we don’t put patients on long-term courses of steroids if they have ipf so that is a controversial question and the guidelines addressed it but they provided very low quality evidence so the initially the guidelines said yes it may not

Be unreasonable to consider treatment for asymptomatic reflux but the research to date does not show significant benefit in terms of either treating your cough or changing your long-term disease course with treating asymptomatic reflux but i think future research that will hopefully be done and published will help inform that decision what i do is generally start

With non-pharmaceutical approaches so things like identifying your triggers modifying your diet perhaps losing weight trying not to eat a large meal three hours before your sleep time not going to a reclining position as soon as you’ve had a meal even for a nap in the afternoon and raising the head end of your bed by at least six inches all of these strategies

Work better so you might want to consider discussing these and trialing these out so there are different ways of looking at it as far as medications are concerned we we try and think of the disease as being mild moderate or severe and that depends on where your fvc is it’s one of those numbers we look at when you’re doing your breathing tests it’s called the

Forced vital capacity and most countries most states most provinces within canada they think of 50 to 80 as being mild to moderate so if you’re above 80 percent in your fec you could you’re considered to have mild disease if you’re 50 to 80 you have moderate disease anything less than 50 would then be considered severe but that’s only based on one particular

Number we have to look at a lot more than just those numbers on a breathing test i would say another way to look at ipf and trying to understand where you are and in your own disease journeys to understand with respect to oxygen right so if you’ve not started oxygen you’ve not qualified for oxygen you might be in the early phases once you figure out that your

Oxygen numbers are going low on a six minute walk test that’s mostly a progressive sign of a progressive disease and maybe once you switch from low flow oxygen to high flow oxygen anything more than four or five liters of oxygen i would call that maybe a more advanced phase because after that disease starts to progress so one way of looking at progression and

To figure out where you are in your own process it’s are you on oxygen how much oxygen are you on high flow oxygen might be another way to figure that out there isn’t a specific kind of staging so to speak but in general with ipf we don’t really categorize it like that so mycophenolate is one of those drugs i think we very briefly spoke about in the beginning

It’s an immunosuppress suppressive drug in other words it’s used in some cases of pulmonary fibrosis or interstitial lung diseases where we also believe that inflammation is a big part not just scarring but inflammation just like ipf is just mostly scar not so much inflammation so we don’t use these drugs but if you have other types of pulmonary fibrosis which

Are not ipf for example you have rheumatoid arthritis you have scleroderma you have hypersensitivity pneumonitis these are all types of pulmonary fibrosis we might consider using an immunosuppressive drug to keep that inflammation down so the hope is if the inflammation is down it’s not going to trigger more scarring in the lung so we might use many of these

Medications sometimes alone sometimes in a combination to prevent inflammation hopefully to reduce scar formation in the lungs diagnosed early and correctly so that you know which type of pulmonary fibrosis you have do you have the type that actually requires steroids and immunosuppressive drugs or do you have the type that actually requires anti-fibrotics like

The ipf type so it’s very important to know that difference very very very clearly so i think this is also one of those questions hopefully that future research will address but for now most of the experts around the world believe that oxygen should be started if on a walk test you’re measuring your oxygen numbers and your numbers are below 88 percent and you

Have some symptoms like you have shortness of breath you have cough you have fatigue you’re struggling with activities if you have those two things then it’s probably not a bad idea to trial oxygen and see if it benefits you now in a lot of provinces maybe other countries in the world it’s approved at that point but in alberta it’s not approved so in other words if

You have symptoms and your oxygen levels are going below 88 you might self-fund oxygen do a small trial and see rent a portable concentrator and see if it makes a difference if it helps your symptoms it helps your activities it helps your quality of life then i would say discuss it with your physicians if you’ve not already done so and you might want to advocate

For yourself try and see if you can get the auction funded if not paying out of pocket or finding some other grants to support that would be useful so specific oxygen number less than 88 plus you have symptoms and poor quality of life i would say that’s a point to consider oxygen and discuss it so biologics are a specific type of medications mostly used with a lot

Of these autoimmune conditions in other words if there’s inflammation in your body whether that that inflammation is happening from underlying autoimmune diseases like scleroderma or rheumatoid arthritis so in that it’s in that world it’s in that context that biologics are used quite a bit in in the ild world so yes if you have any of those conditions and there

Is evidence that there is progression either of the lung problems that you have from that disease or other issues like joint problems skin problems muscle problems if there is evidence of progression then absolutely those drugs can be added ones that are already on can be changed or escalated up and that decision is taken in a discussion between the lung doctors

And your rheumatologists along with you and your family and then the decisions are made so absolutely there is room to change to add you

Transcribed from video
FAQs About Pulmonary Fibrosis/ILD By Alberta Lung