A doctor blogging about Pulmonary Fibrosis, Interstitial Lung Disease, Prednisone, Oxygen, and Lung Transplantation for patients and their caregivers. No one is average. Demographics are not destiny. Doctors are not fortune tellers.
Saturday, October 5, 2013
“The Internet Said I Have 3 Years to Live”
I’ve been having a hard time writing this post about “prognosis”, or how long you might expect to live with Pulmonary Fibrosis. It’s a tough subject. After struggling for about 30 minutes, I realized two things:
Number one – my difficulty writing about prognosis is a pebble in comparison to your mountain of difficulty living with Pulmonary Fibrosis. So I’m going to stop complaining right now.
And number two – it’s difficult to write about prognosis because…. Well, because prognosis is a lie. A myth. Let me explain.
But, before I get to the myth of prognosis, I first need to tell you that all that stuff on the internet about a short survival time in Pulmonary Fibrosis comes from studies of patients with one specific form of Pulmonary Fibrosis called Idiopathic Pulmonary Fibrosis (abbreviated IPF). So, if you don’t have IPF, none of that stuff applies to you – your prognosis might be substantially better than the prognosis in IPF. If you haven’t been to a Pulmonary Fibrosis center (also called an ILD center) to really sort out exactly which kind of Pulmonary Fibrosis you have, then go get yourself an appointment tomorrow.Check out the Pulmonary Fibrosis Foundations website for a list of ILD centers.
Ok, if you’ve been to an ILD center and the team there said you do indeed have IPF, then we can start talking about how prognosis in IPF is just a myth. Now, I don’t mean that IPF isn’t a serious disease – it IS quite serious and people do die from Pulmonary Fibrosis. But the idea that a doctor can tell you how many months or years you have left before your die is simply ridiculous. How can they know exactly when you are going to die? That would be magic.
You are probably thinking “Well, I don't really expect my doctor to know exactly how long I am going to live – I agree that is kind of silly -- but I would like an idea of what to expect.” And I agree with you – that is very reasonable. But, giving you a single landmark in time is simply misleading. Here’s why:
Many of you have seen something like this on the internet: “Patients with IPF live about 3 to 5 years from the time of their diagnosis.” Most people interpret this to mean: “I have 3 to 5 years left before I die.” But, that’s a false interpretation. It doesn’t mean that everyone with IPF lives 3 to 5 years. It means that “on average” people with IPF live 3 to 5 years.
Are you average? No, you’re not. No one is average. Everyone is either better than the average or they are worse than the average. Saying “you have about 3 years left” is like saying that people are 5’ 7’’ tall (the average adult height in the U.S.). Kind of unhelpful and ridiculous.
There is a lot of variation in how long people live with IPF – in fact, in some studies 1 out of 5 people live more than 10 years! Some doctors now think that there may be a very long “silent” period where there is scarring (fibrosis) in the lungs without any symptoms at all – and that the “3 to 5 years” really only begins when people become quite ill – so someone diagnosed in a very early stage might remain well for many years – we don’t really know how many years – before the disease begins to progress. One study even showed that “on average” people with IPF lived 7 years if you started the clock at the time that their symptoms began (like when they first developed short of breath with activities and exercise). That means that half of those people lived more than 7 years!
There’s also a whole other level of complexity here. Doctors can look at how sick you are today and they can use that information to refine their prognosis. Some of the things that predict a better prognosis are the need for less oxygen (or none at all), having better lung function test results (tests like the “FVC” and “DLCO”), walking farther during a walking test (usually a six-minute walk test), and how much shortness of breath you have during your day-to-day activities.
On top of all of this, the studies that found the average survival was 3 years were done more than 10 to 15 years ago. Nowadays, the survival seems to be better – at least “on average.”
And none of these numbers account for people who get a lung transplant – that’s a game changer. Lung transplantation is really to only treatment for IPF that seems to prolong life – again, at least “on average”.
Let me drive the point home by showing you an example of how I talk about prognosis with my patients. Realize that this is one scenario tailored to an individual (and hypothetical) patient. I would change the range of numbers used depending on my patient’s individual characteristics.
“No one can tell you how long you are going to live. You may have read on the internet that the average survival with IPF is 3 years, but that means that half of patients with IPF actually live longer than that – some live a lot longer. But, it also means that the other half don’t even make it 3 years. Also, I don’t know if the average is really that helpful, since no one is average. I find it more helpful to think about the range of possibilities. Is it possible that you are going to live longer than 10 years? Without a lung transplant, the truth is that that does seem unlikely to me. I am worried that you will become much sicker before 10 years were up. Could you live 5 years if you don’t undergo lung transplantation? Yeah, I could see that happening, but you might be quite ill 5 years from now, and while it is possible, it is kind of a stretch. I think the most likely thing is that before 5 years are up, your disease will have already progressed to the point where you need oxygen all the time – perhaps up to 10 or 15 liters of oxygen through a face mask, you are very limited in what you can do – each day will be a struggle, and sometime in the next 5 years, your lungs are likely to fail, which is a life-threatening event. I don’t know if all of those things are going to happen in 5 years, 4 years, 3 years, 2 years or even in the next year, but based on how you are doing today, it is unlikely that any of those things are going to happen in the next 6 to 12 months. So, let’s work together on figuring out how to prevent things from getting worse, and let’s get you as healthy as possible so that when your lungs are sicker, you don’t necessarily have to be so limited by your lungs. We can look into some of the clinical trials of new treatments for IPF – maybe one will work for you. And if lung transplantation is an option, let’s start looking into that now too.”
So, don’t let ANYONE tell you that they know how long you are going to live. You are alive today. You are living with pulmonary fibrosis. Living. Treasure that.
Number one – my difficulty writing about prognosis is a pebble in comparison to your mountain of difficulty living with Pulmonary Fibrosis. So I’m going to stop complaining right now.
And number two – it’s difficult to write about prognosis because…. Well, because prognosis is a lie. A myth. Let me explain.
But, before I get to the myth of prognosis, I first need to tell you that all that stuff on the internet about a short survival time in Pulmonary Fibrosis comes from studies of patients with one specific form of Pulmonary Fibrosis called Idiopathic Pulmonary Fibrosis (abbreviated IPF). So, if you don’t have IPF, none of that stuff applies to you – your prognosis might be substantially better than the prognosis in IPF. If you haven’t been to a Pulmonary Fibrosis center (also called an ILD center) to really sort out exactly which kind of Pulmonary Fibrosis you have, then go get yourself an appointment tomorrow.Check out the Pulmonary Fibrosis Foundations website for a list of ILD centers.
Ok, if you’ve been to an ILD center and the team there said you do indeed have IPF, then we can start talking about how prognosis in IPF is just a myth. Now, I don’t mean that IPF isn’t a serious disease – it IS quite serious and people do die from Pulmonary Fibrosis. But the idea that a doctor can tell you how many months or years you have left before your die is simply ridiculous. How can they know exactly when you are going to die? That would be magic.
You are probably thinking “Well, I don't really expect my doctor to know exactly how long I am going to live – I agree that is kind of silly -- but I would like an idea of what to expect.” And I agree with you – that is very reasonable. But, giving you a single landmark in time is simply misleading. Here’s why:
Many of you have seen something like this on the internet: “Patients with IPF live about 3 to 5 years from the time of their diagnosis.” Most people interpret this to mean: “I have 3 to 5 years left before I die.” But, that’s a false interpretation. It doesn’t mean that everyone with IPF lives 3 to 5 years. It means that “on average” people with IPF live 3 to 5 years.
Are you average? No, you’re not. No one is average. Everyone is either better than the average or they are worse than the average. Saying “you have about 3 years left” is like saying that people are 5’ 7’’ tall (the average adult height in the U.S.). Kind of unhelpful and ridiculous.
There is a lot of variation in how long people live with IPF – in fact, in some studies 1 out of 5 people live more than 10 years! Some doctors now think that there may be a very long “silent” period where there is scarring (fibrosis) in the lungs without any symptoms at all – and that the “3 to 5 years” really only begins when people become quite ill – so someone diagnosed in a very early stage might remain well for many years – we don’t really know how many years – before the disease begins to progress. One study even showed that “on average” people with IPF lived 7 years if you started the clock at the time that their symptoms began (like when they first developed short of breath with activities and exercise). That means that half of those people lived more than 7 years!
There’s also a whole other level of complexity here. Doctors can look at how sick you are today and they can use that information to refine their prognosis. Some of the things that predict a better prognosis are the need for less oxygen (or none at all), having better lung function test results (tests like the “FVC” and “DLCO”), walking farther during a walking test (usually a six-minute walk test), and how much shortness of breath you have during your day-to-day activities.
On top of all of this, the studies that found the average survival was 3 years were done more than 10 to 15 years ago. Nowadays, the survival seems to be better – at least “on average.”
And none of these numbers account for people who get a lung transplant – that’s a game changer. Lung transplantation is really to only treatment for IPF that seems to prolong life – again, at least “on average”.
Let me drive the point home by showing you an example of how I talk about prognosis with my patients. Realize that this is one scenario tailored to an individual (and hypothetical) patient. I would change the range of numbers used depending on my patient’s individual characteristics.
“No one can tell you how long you are going to live. You may have read on the internet that the average survival with IPF is 3 years, but that means that half of patients with IPF actually live longer than that – some live a lot longer. But, it also means that the other half don’t even make it 3 years. Also, I don’t know if the average is really that helpful, since no one is average. I find it more helpful to think about the range of possibilities. Is it possible that you are going to live longer than 10 years? Without a lung transplant, the truth is that that does seem unlikely to me. I am worried that you will become much sicker before 10 years were up. Could you live 5 years if you don’t undergo lung transplantation? Yeah, I could see that happening, but you might be quite ill 5 years from now, and while it is possible, it is kind of a stretch. I think the most likely thing is that before 5 years are up, your disease will have already progressed to the point where you need oxygen all the time – perhaps up to 10 or 15 liters of oxygen through a face mask, you are very limited in what you can do – each day will be a struggle, and sometime in the next 5 years, your lungs are likely to fail, which is a life-threatening event. I don’t know if all of those things are going to happen in 5 years, 4 years, 3 years, 2 years or even in the next year, but based on how you are doing today, it is unlikely that any of those things are going to happen in the next 6 to 12 months. So, let’s work together on figuring out how to prevent things from getting worse, and let’s get you as healthy as possible so that when your lungs are sicker, you don’t necessarily have to be so limited by your lungs. We can look into some of the clinical trials of new treatments for IPF – maybe one will work for you. And if lung transplantation is an option, let’s start looking into that now too.”
So, don’t let ANYONE tell you that they know how long you are going to live. You are alive today. You are living with pulmonary fibrosis. Living. Treasure that.
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