Monday, February 17, 2014

More on Predicting the Future in IPF

John R. asked the following question:

"What is your take on using the GAP Risk Assessment System as a tool to help determine a basic prognosis for the IPF patient?"

Here's my answer:

Thanks for bringing up the GAP score, John. For those who don't know about the GAP score, it is a simple tool that combines Gender, Age, and Pulmonary function test results (see the G-A-P there?) to help doctors predict how likely it is that someone with idiopathic pulmonary fibrosis (IPF) might die over the next three years.

The GAP score is an accurate tool that helps us identify people with IPF that should be considered for enrollment in clinical trials of new treatments or who might need lung transplantation. But the GAP score, just like any other prediction of the future, can't tell us what is going to happen to an individual person. Here's an example of what I mean.

Imagine that someone's GAP score was "4". About 1 out of 3 people with a GAP score of 4 will die over the following two years. This might mean that we should consider lung transplantation or enrollment in trials of new treatments -- in this way, the GAP score is indeed helpful. But this information actually does not help us know how long someone has left. Are you one of the "1 out of 3"? We still don't know the future.

Bottom line: Risk prediction tools like the GAP score can be very helpful for making decisions about treatment options, but are not really helpful if the question you are asking is "when am I going to die?" They can't answer those questions unless they predict a very high (>95%) or very low (>5%) risk, and in most cases, we don't need risk prediction tools when your risk is very high or very low, because it is often quite obvious when someone is very sick or very well.

While I do NOT recommend calculating your GAP score (for the reasons discussed above), I know everyone has already googled it. Here is a link to the calculator.

One more thing: the GAP score was developed in idiopathic pulmonary fibrosis (IPF) and may not apply to all of the other forms of Pulmonary Fibrosis. Many other forms of Pulmonary Fibrosis have better outcomes than IPF.

Sunday, February 2, 2014

Pulmonary Fibrosis Support Group at NYP/Columbia, Feb-June 2014

We will be holding our Pulmonary Fibrosis Support group at NYP/Columbia in Manhattan on the following dates during the first half of 2014:

February 20
March 20

April 17
May 15
June 19

Time: 3pm-5pm

Location:
Atchley-Loeb Conference Room
Presbyterian Hospital Building, 8th floor
622 West 168th Street
New York, NY 10032


Please try to bring your own oxygen, but we will tanks for those who need them.

All are welcome! Call Susanne or Julie in our ILD office at 212-305-8203 with any questions.

Tuesday, December 24, 2013

Am I Too Old for a Lung Transplant?

I'd like to talk about lung transplantation again. In this post, I'll try to answer the question "Am I too old for a lung transplant?" As you will see, it's not a simple thing...

Sunday, November 17, 2013

Pulmonary Fibrosis/ILD Support Group in Manhattan

We will be holding our next Pulmonary Fibrosis Support Group for patients and their caregivers at Columbia University Medical Center/New York Presbyterian Hospital on Thursday, November 21, 2013 from 3-5pm. I will be there along with our Pulmonary Fibrosis team. All are welcome!

The address is 622 W 168th Street, New York, NY 10032
Take the Presbyterian elevators to the 8th floor and go to room E107. 
Support Group is held in the Atchley-Loeb Conference Room. 
Contacts: Julie or Susanne at 212-305-8203

Hope to see you there!

Dr. Lederer


Sunday, November 10, 2013

"Should I take prednisone for my Pulmonary Fibrosis?"

I've received a number of questions about whether or not prednisone should be used to treat pulmonary fibrosis. In this post, I am going to try to help you understand the role of prednisone in the treatment of pulmonary fibrosis. I first want to remind you that you should not make any changes to your current medical treatments without discussing things with your doctor. In particular, if you stop taking a medication like prednisone,

Tuesday, October 29, 2013

The Honest Truth about Lung Transplantation: It’s a Wonderful and Terrible Thing

I want to begin a conversation about lung transplantation as a treatment for Pulmonary Fibrosis. This is a huge topic and the conversations that I have with my patients about lung transplantation range from “we need to move forward with evaluating you for lung transplantation right now” to “lung transplantation is a dangerous option for you, and we should avoid it.” There is no single conversation about lung transplantation. So writing this post has been a challenge. Perhaps this will be the first in a series of posts about lung transplantation for Pulmonary Fibrosis.

Thursday, October 24, 2013

Pulmonary Fibrosis Support Group in New York City

Hi everyone!

I wanted to alert everyone to a new Pulmonary Fibrosis Support Group that we started at Columbia University/New York Presbyterian Hospital in Manhattan. Our next support group will be held on Thursday, November 21 from 3pm to 5pm at 622 W 168th Street in Manhattan. We will be meeting on the 8th floor of the Presbyterian Hospital Building in the Atchley-Loeb Conference Room. I will be there along with the rest of our Pulmonary Fibrosis team at Columbia. We will talk about clinical trials, lung transplantation, and any other topics that people want to talk about.

Please call 212-305-8203 to RSVP.  More information available here.

New post on lung transplantation coming soon!

Thanks
David

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.

Friday, October 4, 2013

Dr. Swigris' Pulmonary Fibrosis Research Website and Blog

Dr. Jeff Swigris from the Pulmonary Fibrosis Program at National Jewish Health in Denver, Colorado has developed a new "patient-centered" research program for patients with Pulmonary Fibrosis. He is a world leader in the clinical care of patients with Pulmonary Fibrosis and he is also an outstanding clinical researcher studying new ways to improve the everyday life of patients with Pulmonary Fibrosis. He's got some great ideas and I encourage everyone to take a look at his website at http://pulmonaryfibrosisresearch.org. You can join his "P3F Registry" if you are interested in being considered for enrollment into research studies about Pulmonary Fibrosis.

Thursday, October 3, 2013

The Sixth Sense: Why you should use oxygen if you have Pulmonary Fibrosis!

Hi everyone!

I’m just completely blown away the overwhelming response to my blog. I’ve received comments, emails, and a few in-person thank yous. I’m pleased that you are finding the site useful. Please keep sending me your ideas for future blog posts.

Right now I’m flying at 30,000 feet on my way to Denver, Colorado for a meeting about Pulmonary Fibrosis. I’m a lung doctor, so I’m always thinking about how lungs are working (or not working), and right now I’m wondering if I’m harming myself by spending a few hours at altitude.

As you might know, the air is “thinner” at higher altitudes, which means there is simply less air to breathe. Now, I’m not breathing “30,000 feet” worth of thin air – which would be really bad. Instead, since the air in the cabin of this Boeing 757 is pressured a bit, I’m breathing about “8,000 feet” worth of thin air. For someone with normal healthy lungs breathing comfortably at sea level, about 99% of the hemoglobin in the blood leaving the heart might be carrying oxygen -- that’s a normal healthy percentage. But with the thin air up at 8,000 feet, only about 90% of the hemoglobin is carrying oxygen.

Should I be worried?

Maybe. I’ll explain why…

Tuesday, October 1, 2013

Should I join a Pulmonary Fibrosis support group?

Kathy Lindell, the nursing manager for the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease at the University of Pittsburgh is guest blogging today on our site on the importance of joining a Pulmonary Fibrosis support group. Kathy serves on the Medical Advisory Board for the Pulmonary Fibrosis Foundation and actively participates in the clinical care of patients with Pulmonary Fibrosis. Please share this with loved ones and friends and leave comments and questions for Kathy.
 
- David Lederer, MD

Kathleen O. Lindell, PhD, RN






Should I join a Pulmonary Fibrosis support group?
Kathleen Lindell, PhD, RN
Research Assistant Professor
Division of Pulmonary, Allergy, and Critical Care Medicine
Dorothy P. & Richard P. Simmons Center for Interstitial Lung Disease
University of Pittsburgh, Pittsburgh, PA

http://www.dept-med.pitt.edu/paccm/faculty/Lindell.html

Hi everyone! I am thrilled to be a guest blogger on Dr. Lederer's website. As a reminder, this post represents my personal views, not those of the University of Pittsburgh.

Attending a Pulmonary Fibrosis support group is a crucial part of helping you and your caregiver(s) adapt to the many unknowns that come with a diagnosis of Pulmonary Fibrosis. The disease course of Pulmonary Fibrosis is variable, so there is no set timeline on how the disease progresses.  There is no way to predict your future, and this can cause a great deal of angst and fear of the unknown for you and your caregiver(s).

The support group should provide information so that you can learn more about your disease; topics might include:

  • What is Pulmonary Fibrosis?
  • Importance of Exercise
  • Use of Home Oxygen
  • What are the treatment options?
  • Is a lung transplant the right thing for me?

The support group members can provide you with a home where you can get help with the day-to-day life of IPF;  explaining what IPF is to your family/friends, how to travel with oxygen, etc. The benefits of attending an IPF support group include meeting other people who are going through exactly what you are going through.

Maybe you’re not the type who would attend a support group? We advise our patients (and actually write a prescription) to attend at least once and then make their own decision if it’s for them.

Maybe you do not have a support group near you. There are on-line support groups that are easy to join. Here is a link to see if there’s a support group near you or how to join an on-line support group.  http://www.pulmonaryfibrosis.org/supportgroups

Our experience has been that this disease is not patient friendly. I wish I could say it was.  BUT, our experience has also been those who attend and participate in support group live better with their disease because of their interactions with other patients and caregiver(s) who are going through the same journey.

Monday, September 9, 2013

Five reasons you should use oxygen if you have pulmonary fibrosis

Your doctor told you to use oxygen for good reasons. It's a difficult transition, but smart use of oxygen is one of the most important things you can do if you have pulmonary fibrosis. Here's why you should maintain your oxygen saturation >90% 24hrs/day:

  1. You will suffer less. Oxygen saturations < 90% make most people feel short of breath.
  2. You can do more with oxygen. Using oxygen during activities will allow you to accomplish tasks that you simply can't do any other way.
  3. You can avoid pulmonary hypertension. Avoiding low oxygen levels can help prevent a serious complication of pulmonary fibrosis called pulmonary hypertension.
  4. You can improve your chances of getting a lung transplant. Lung transplant doctors want you to be strong, active, and have a healthy weight. Using oxygen allows you to be active and participate in rehabilitation, which can turn you into a better surgical candidate!
  5. You might live longer. While not proven to be the case for patients with pulmonary fibrosis, oxygen use does help people with chronic obstructive pulmonary disease (COPD) live longer. 

In an upcoming post, I will discuss some of the practical aspects of using oxygen in and out of your home. 

    Thursday, September 5, 2013

    The Pulmonary Fibrosis Checklist: Do these 8 things!

    This is a quick post to introduce the Pulmonary Fibrosis Checklist. If you've been diagnosed with any form of pulmonary fibrosis, talk to your doctor about these 8 things...

    The Pulmonary Fibrosis Checklist

    1. Use enough oxygen to keep your oxygen saturation >90% 24 hrs/day.
    2. If eligible, participate in pulmonary rehabilitation.
    3. If you have idiopathic pulmonary fibrosis (IPF), avoid long-term use of prednisone.
    4. Consider a sleep study to check if you have sleep apnea.
    5. See if you are eligible to enroll in a clinical trial of experimental therapies.
    6. Obtain a consultation from an ILD Center for help establishing your specific diagnosis and providing management advice
    7. See if lung transplantation is an option for you. 
    8. Visit the Pulmonary Fibrosis Foundation online for information, resources, and support.
    What do you think should be added to this list?

    I will focus on each one of the Checklist items in future posts.

    Thursday, August 29, 2013

    Should I undergo a lung biopsy to diagnose my pulmonary fibrosis?

    Of course only you and your doctor can really answer this question. But there are some important things you should know before you go under the knife...

    Yes, sometimes lung biopsies are necessary in PF...
    First thing is that a lung biopsy is sometimes (but not always) needed to help diagnose the kind of PF you have. And it's important to try to figure out which kind of PF you have for the following reasons:
    1. Different kinds of PF respond to medications differently. For example, prednisone is widely used to treat PF when inflammation is present in the lung, but it turns out that prednisone is harmful if you have IPF. Sometimes a lung biopsy is needed to sort this out before deciding on a treatment plan. If I don't think the results will influence the immediate treatment plan, I sometimes still recommend a biopsy since the results will often influence future treatment decisions.

    2. Sometimes infections, serious inflammatory conditions (such as a "vasculitis"), and other conditions may be alternative explanations for the appearance of your CT scan. Lung biopsies can be very helpful in these settings.

    3. Different kinds of PF progress at different rates. Sometimes a lung biopsy helps us understand how quickly you might get sicker. This information might be helpful (for example in deciding when to seek a lung transplant evaluation), but is probably not enough of a reason to undergo a lung biopsy in most cases. 

    Tuesday, August 27, 2013

    The Alphabet Soup of Pulmonary Fibrosis

    The Alphabet Soup of PF
    It is not surprising that there's a lot of confusion out there about the different kinds of PF. Here's why...
    • There are over 100 different kinds of PF that all look very much alike
    • There is an alphabet soup of confusing and sometimes poorly defined abbreviations for many types of PF (IPF, PF, ILD, NSIP, RB-ILD.... the list goes on and on).
    • Even experts will often disagree about which kind of PF you have.
    Experts typically do agree, however, that it is important to try to sort out which kind of PF you have, since different kinds of PF behave differently both in terms of how quickly the disease might progress as well as whether it might respond to medical treatments (such as prednisone). I'll talk about how we sort out which kind of PF is affecting the lungs in future posts. In this post, I'll try to clear up the confusion about the different terms used to describe different kinds (and families) of PF

    Friday, August 23, 2013

    What is Pulmonary Fibrosis?

    The word "pulmonary" means lungs and the word "fibrosis" means scar tissue -- just like the scars you might have on your skin from previous injuries or surgeries. So, in its simplest sense, pulmonary fibrosis (sometimes called PF) means scarring in the lungs. But many people use the term PF to describe a family of over 100 diseases that affect the walls of the air sacs* of the lung. While in many cases, scar tissue is present in the air sacs, there can also be inflammation or other changes to this delicate lung tissue. The details depend on which kind of PF you have (more on this later).

    There are four consequences to the build-up of scar tissue or inflammation in the lungs. Doctors use these consequences to recognize that someone has PF.

    • Stiff lungs. Scar tissue and inflammation make your lungs too stiff. Stiff lungs are hard to stretch,

    Thursday, August 22, 2013

    Why a blog about pulmonary fibrosis?

    Why a blog about pulmonary fibrosis? You already understand why.

    There is a world of confusion out there about PF. What is it? What's the difference between IPF, PF, and ILD? What causes it? Will my kids get it? Which treatments work? Should I try stem cells? prednisone? NAC? Do I need a lung transplant?

    Top that off with the nearly universal problem that in the months and years before you are finally told that you have PF, you were probably told that your cough or difficulty breathing was due to asthma, COPD (chronic obstructive pulmonary disease), emphysema, heart disease, or sleep apnea, or that "you just need to lose weight and exercise." 

    It's no surprise that just about every single one of my patients with PF is frustrated and confused when he or she walks in the door. 

    I created this blog to try to clear up the confusion about PF. Here we go...