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.
Some of you have never heard of a lung transplant. Some are considering a visit to a transplant center. Some are undergoing evaluation at a lung transplant center. Some are on the waiting list for lung transplantation. Some have already had a transplant.  I will write this post as if you’ve never heard of a lung transplant. That way, we will all be on the same page.

Lung transplantation is the surgical replacement of one lung or both lungs with one or two lungs taken from someone else’s body. In most parts of the world, the new lung (or lungs) comes from someone who has died in a hospital, often in a tragic way. In some cases, healthy people can donate a part of one of their lungs (called a “lobe” of the lung) to a family member. This type of transplant is rare in most parts of the world. For example, only about 1 person undergoes a “living donor” lung transplant in the United States every other year.

If you have Pulmonary Fibrosis, lung transplantation may be an incredibly important treatment option for you. If you undergo a successful lung transplant, you can expect the following benefits after you recover from surgery:
  • Most people no longer require oxygen 
  • Most people have absolutely no shortness of breath or cough
  • Most people are able to return to an active lifestyle
  • Many people are able to return to work
For most people with Pulmonary Fibrosis, there is no other treatment option that has these life-altering effect. And if things go well, most people seem to live longer – in some cases, much longer – with a transplant than they would have lived if they had not undergone lung transplantation.

Sounds great, right? You might even think that everyone with Pulmonary Fibrosis should run to their nearest lung transplant center to be put on the waiting list. But, it turns out that not everyone can get a lung transplant. There just aren’t enough lungs for everyone.

Take a look at this chart that I made using information provided by the Organ Procurement and Transplantation Network, also called the OPTN. The OPTN is a federally designated organization
that coordinates all of the organ transplants performed in the United States. The OPTN makes their data available to all of us for free on their website. You can play with their online data tools here.  If you poke around their website a bit, you will find that in 2012, there were only 1,754 lung transplants performed in the entire United States. That same year, over 2,300 people were put on a waiting list for a lung transplant in the United States – that’s a shortage of about 500 lungs. And that doesn’t even include the 1,600 people already on the waiting list on any given day. That means that we would need to perform an additional 1,600 lung transplants today just to catch up with the current shortage, and then we would need to perform about 2,300 lung transplants (instead of about 1,700) each year to keep up with the ongoing demand.

(For those of you outside the U.S: The total number of lung transplants performed worldwide in 2011 – the last year we have data for -- was 3,747. International data is available in a huge powerpoint slidedeck designed for healthcare professionals here).

Funny thing – exactly half of the 1,754 lung transplants performed in the United States in 2013 were for adults with Pulmonary Fibrosis. That means that only 877 adults with Pulmonary Fibrosis underwent lung transplantation in 2012. And you may have heard that there is somewhere between 30,000 and 90,000 adults with Pulmonary Fibrosis in the United States.

Bottom line: we have nowhere near enough lungs for everyone.

This donor shortage has a tremendous impact on people with Pulmonary Fibrosis in two important ways:

First, since most people will need to spend time on the waiting list before a donor comes their way, it is important to visit a transplant center early in the course of your disease. The transplant team will need time to perform a comprehensive evaluation of your medical, surgical, and psychological candidacy for lung transplantation. They will also want to educate you and your loved ones about lung transplantation.  If you are approved for lung transplantation, then your waiting time might be anywhere from a few days to two years or more. My suggestion is that you should talk to your pulmonologist at your next visit about whether or not you should be evaluated for lung transplantation.

The second way that the donor shortage effects people with Pulmonary Fibrosis is that each lung transplant program takes the donor shortage into consideration when making decisions about who they will put on the waiting list. To some of you, this may sound sensible. Others may feel that this practice is inappropriate. Here’s the problem:

Most of the time when a healthcare decision about a treatment is made, your doctor will discuss the potential benefits of the treatment and its potential risks, and then together you and your doctor will develop a plan that maximizes your benefit and minimizes your risk. But when it comes to lung transplantation, we don’t have enough lungs to go around. So the transplant center team has to consider not only the benefits and risks of lung transplantation for you, but they also have to balance your benefits and risks against the benefits and risks of all of their other patients. In the end, your transplant team might decide not to offer you a lung transplant if your risk of serious complications is too high.

Transplant centers try to strike this balance because they want to help as many people as possible while also avoiding a scenario where the transplanted lung does not prolong someone’s life. 

Here’s an extreme example to help you understand this issue. Imagine that we had 10 lungs to give out to 15 people who need lung transplants. If 5 of the people had a fatal form of cancer and they weren’t going to live more than two years even with the lung transplant, then it would only make sense for us to give the lungs to the 10 people without cancer.

In real life, as you can imagine, the situation is often more complicated. Each person being evaluated for lung transplantation brings a unique set of circumstances that might increase the risk of serious problems after a lung transplant. Some people have heart problems. Others have life threatening infections that might get worse after lung transplantation. Some are weak and frail and may not survive the recovery period after surgery. Some may have a pattern of unhealthy behaviors that, if they continue, could put their new lung at risk.

It turns out that lung transplant programs very often decide not to put someone on the list because the risk of serious life-threatening problems is too high. We even make that decision if there are no other treatment options available. We even make that decision if they might benefit even in some small way from the transplant surgery. That’s a terrible and heartbreaking situation to find yourself in if you have a fatal lung disease.

You might think I’m making too big of a deal about the risks after a lung transplant – but even in the best of circumstances, lung transplantation is a risky thing. While most people do quite well for at least a few years, everyone who has had a lung transplant is at risk for some serious problems. Some have serious early complications after surgery that can be life-threatening or lead to a difficult months-long recovery period and a reduced quality-of-life. And while I did recently meet someone who had his lung transplant performed 22 years ago (!), only about 9 people out of 10 survive the first year after their lung transplant. By 5 years, half of the people who underwent lung transplantation have died. And only 1 out of 4 survive more than 10 years. Everyone is different (see my post about prognosis), but the risks here are real – I will discuss the specific complications and risks after transplantation in a future post.

You’ve seen a glimpse now of the two faces of lung transplantation: a life-saving treatment that leads to serious complications for many of the lucky few who undergo transplantation. I often say that lung transplantation is one of the most wonderful and terrible things that human beings do to each other. That might seem a little bit dramatic, but it seems to describe my experience in my transplant job pretty well.

I look forward to continuing this conversation with you in the comments section of the blog and in future posts. 

83 comments :

  1. Thank you for this very real, very true article about PF and lung transplantation. My mom is 3 months post transplant (single lung). As her team said, a transplant is not a cure. It comes with its own set of risks and complications. But I do know the past 3 months have been free of oxygen concentrators, wheelchairs and so many of the other PF side effects.

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    1. Hi Suzanne,

      Thanks for your comment, and congratulations on your mother's lung transplant! Best wishes to her for a long and happy life.

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  2. I look forward to your future posts discussing some of the very real risks and how we as patients can work with our medical team to minimize those risks such as obesity.

    At the time I was referred for my transplant assessment, it looked like I was heading for a downward spiral. Five months later after having the assessment followed by the decision a month later, my downward spiral had stopped and by the time my respirologist/pulmonolgist and I sat down at the end of summer, I had recovered back to my Nov 2012 level albeit with supplemental oxygen.

    The assessment decision was suitable candidate but too early to list although they did send me on for my cardiac angiogram (cardio says heart still under warranty - no blockages, no plaque, good pressure on both sides of heart). So, from the decision to move forward with assessment to assessment to decision to follow-up with your doctor is not a quick process. However, if this winter does it's number on me again, I am positioned to be put on the waiting list very quickly and as discussed with my doctor, if my good cardiac system helps me to pull out of it again by the end of summer 2014 and I haven't yet had the transplant, we have the option to either put me on hold or go forward.

    I don't want to be so sick and frail when I undergo the transplant because I don't believe that works in my favour to minimize risks and maximize benefits but the quality of life right now is good. I'm fully functioning albeit activity is down (do things slower and not for as long) but it's still good. The unknown on this side of transplant is when my IPF will decide to get real ugly again but I'm as ready as I can be for that.

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    1. Hi Barbara, Thanks for sharing your experience with your transplant evaluation. I think your experience is a good example of what to expect in the best case scenario.

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  3. It is so refreshing to hear all the good and not so good information about transplant. I am waiting for a double transplant at Papworth, UK. My day to day health is excellent, except for a very slow progression of breathlessness. I take no meds for my PF and have been on the list for 18 months. I have had two false alarm calls which were interesting and live my life as each day is the new normal. I so look forward to more of your posts. Murray XX

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    1. Hi Murray. Thanks for sharing your experience. Hang in there!

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  4. Gale Borders at ialwayspray4u@yahoo.comOctober 30, 2013 at 11:59 AM

    Thank you for sharing that info and I look forward to future articles. I do have a question about the transplant process. In 2009 I had a lung biopsy and told I had IPF I was also told I had 2 years to live unless I had a double lung transplant. Also told to make my funeral arrangements in case I didn't live long enough to get my transplant. I went through the process and told I needed to loose 6pds. to be listed. The visit I had to return when loosing the 6pds. I was told I didn't need a transplant at this time I was too healthy. I was 54 y.o. at that time and had high hopes of returning to my productive life. Now I am in limbo not healthy enough to do daily routines yet too healthy for a double lung transplant. Do you have any input on this kind of decision making? Thank you for taking time to respond to this question. I am sure I am not the only person out there that this has happened too.

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    1. I'm also in limbo and am currently waiting to see if I'm accepted into a clinical trial.I'm not sick enough for a transplant. I currently am a patient at the Columbia University Medical Center in Manhattan. Had been to another doctor and was told I was too old to be considered for a transplant. I did not listen and spent hours on the internet to find the best dr. and hospital. I have no doubt that if u keep looking and not accepting the negative you will take charge of your health and feel much better. Think about Columbia for eval. Good luck!

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    2. Hi Mr. Hopeful,
      I will be going to Columbia for evaluation for first time in two weeks. Very nervous. I am 25 year old, female on oxygen with Pf and scleroderma. I am scared I will be turned down because I am young. As you have been there, how is it like to be there. Most importantly, how do you feel going there ?

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    3. I can't really comment on anyone's individual situation. The decision about the timing of being placed on the waiting list is complex and needs to be tailored to each person's own situation. Happy to hear two of you are seeing us at Columbia. Always happy to hear feedback about our team.

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  5. It might be good for Dr Lederer to list the test required for the tx evaluation. It took me 5 full days of test. I was not too sick but was exhausted at the end of each day. That is why he says it is important to be evaluated early. If your PF progresses very quickly you will not be strong enough to do the test required. Gailinpa

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    1. Hi Gailinpa. Good idea. I will comment on the transplant evaluation process in a future post.

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  6. I was diagnosed in June 2008. The first tx center I went to said I was high risk because of my weight. I tried but could not meet their goal BMI. My dr there recommended me to another tx center that does more high risk txs. I also need small lungs. I have lost most of the weight. Been on the list 26 months. I got a call two months in, drive the 3 1/2 hours to the tx center, had the initial test then waited. 4 hours later the dr came in and said "I am sorry, before our plane even got there they were told the donor family changed their minds. We got in the car and came home. I had not expected a call that soon since I was not very sick. You never know when you will be a good match. At this point my disease has progressed to the point where I need the transplant. My PF has lead to pulmonary hypertension, so I now need both lungs instead of just one. I am on 8 liters sitting, 18 - 25 when moving. I go to pul rehab 2x a week and am able to walk on the treadmill. My quality of life is shrinking fast just can't get that much oxygen anyplace else. I also go to church every Sunday but I will have to stop that soon too when the cold and flu season gets rolling. It is just too much of a risk. Gailinpa

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  7. About organ donation. If everyone who wanted to be a donor could be a donor there would be plenty of organs to go around. The reality is that you must die certain way.... that you are on life support to keep the organs viable but brain dead. I suspect because I need a small lung my dry run call may have been a child or teen donor. I am not a parent so I can only try to imagine what those moments must be like. They ask do you want to donate.....you say yes....then you think about what they are going to do to your child to get the organs out. How horrible that must be. I did not feel bad, the time was not right for me.

    If you want to be and organ donor tell you parent, spouse, siblings. It is not enough to casually mention it. It is not enough to put it on your drivers license. You need to do the hard part, talk about it. I believe that most donors die suddenly and unexpectedly. In those moments after your loved one is no longer alive you will be in a state of shock, denial and grief. You will be asked about donating your loved one's organs. It will be a terrible moment of having to accept the reality of your loss. Can you imagine your self in that place and time? A simple talk with your loved ones could greatly help them at that terrible time and make the decision to donate much easier. How much easier will it be if you have already had this talk with loved ones........This is what I told my family. .I want to be an organ donor (of course no one would want these lungs!). It will be my last good deed before I leave this earth and it is very important to me. Many lives can be saved, what a wonderful gift to be able to give to someone else's family. So when the time comes and you are asked "Do you want to donate my organs"? JUST SAY YES. It doesn't not have to be deeply considered or debated with others. Make your response easily and with confidence because I want you to JUST SAY YES. Gailinpa

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  8. My brother in law had a double lung transplant 2 years ago. He had the surgery in March and passed away that Sept at age 50. You are so right about transplant being great but not without risks. The transplant team really was devastated because they were so hopeful for a good outcome, but some liver, kidney issues proved to be too much. My husband who is 54 was just diagnosed last Jan, so far it has not changed, praying for the same in Feb at the next Dr appt. I know there will come a time when we will have these decisions to make as well. Praying that a cure is found soon!

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    1. I am so sorry for your loss. PF is a devastating disease. There is a familial PF, sadly it seems to run in your husbands family. When it came time for my husband and I to make the decision to list or not we knew where PF ends. So do we gamble my life today in hopes of a better future? For me the answer is yes. When I fully understood what PF was going to do to my body I thought, what possible good could come out of this? It took a long time for me to find that good thing God can bring out of a bad situation...It was time. Time to say I'm sorry, forgive me, I love you, to heal old wounds by forgiving and most of all to say thank you. To have as few regrets as possible as I move forward. The other good thing I learned is that PF patients and post tx patients can be donors. The PF and transplant communities have been wonderful. Loving, caring, sharing and understanding. So many people like Dr Lederer giving of the expertise and time. Others sharing their stories, it helps to not feel so all alone. I'll be praying for you and your family. Gailinpa

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    2. Thanks for sharing your family's experience. Very sorry to hear about your brother-in-law's passing. I do want to point out to other readers that while the disease does seem to run in some families, most people with Pulmonary Fibrosis are the only one in their family with the disease. Most people do not pass the disease along to their children.

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  9. Great articles, I'm not a patient.. I have a niece that has been turned down twice. I'm trying to learn as much about this dreadful thing as possible. Keep posting, I will continue to follow. C.A.

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    1. Hi Loretta. I am sorry to hear that you are not a candidate for lung transplantation. I don't have any words that will ease your suffering. I would like you to know that when we turn down someone for lung transplantation, in many cases we are trying to save that person from a treatment that may be worse than the disease itself. Please also talk to your doctors about a number of medications that may become available to treat some forms of Pulmonary Fibrosis in 2014 or 2015. I will post more about this drugs as the clinical trials studying these medications finish early next year.

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  11. Thank you for the informative post. I look forward to the next installment. Does ILD/NSIP related to auto-immune connective tissue disease eliminate you from consideration for a transplant ?

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    1. Thanks for this question. I wish I could give you a simple "yes" or "no", but there is no single answer that applies to everyone. Each transplant center will use their own experience to decide whether or not any one person's "autoimmune" condition might interfere with the success of a lung transplant. So, some people with an "autoimmune" condition will be offered lung transplantation, and others will not. Please talk to your transplant team about whether or not your particular condition might confer additional risk.

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  12. Thank-you so very much for keeping these informative posts coming. My dad died 24 days after his first diagnosis of IPF. He never got a proper diagnosis, and was told he had everything from Allergies, to "a touch of COPD". Education + Awareness = a treatment/cure someday.

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    1. Hi Lorene. Thanks for sharing something so close to your heart. I LOVE your math: "Education + Awareness = a treatment/cure someday". I would add "Research" to the equation too.

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  13. Excellent article. My husband 's sister has been asking him why he can't have a live donor transplant. I guess that is one where someone donates a lobe of their lung to my husband, and that supposedly you can live with that. I would think that would be something that would not be enough, plus how would the person that donated do missing a lobe of their lung. Can you tell me anything about living lung donation?

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    1. Thanks for this question. Living donor lung transplantation is performed by removing about half of one lung from one donor and about half of one lung from another donor. These two "half lungs" or "lobes" are then transplanted into the chest of a child or a small adult. The recipient needs to be small because we are only using half a lung on each side. Keck School of Medicine at the University of Southern California is the world leader in this type of lung transplantation. Nowadays, this type of lung transplantation is rarely performed in the United States. There are risks to the donors that make some physicians shy away from the procedure. You can find out more here: http://www.2ndwind.org/donor_program/livedonor.htm

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  14. I think most people with lung disease would not be excluded from being considered for transplant except for cancer. I know people who have been transplanted who have/had Cystic Fibrosis, COPD, PF, PH (Pul hypertension). You can live with one lung so I assume that someone donating a live lung will live a normal life. Don't know how much of a lung the person waiting would need.
    Gailinpa

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  15. It is only a lobe that is used. The right lungs has 3 lobes and the left has 2. From what I've read on live donor transplant, not all transplant centers do them, and generally it's best if you have 2 live donors. The recipient receives 2 lobes increasing their lung capacity to 40%. With a single live donor, 2 lobes are removed reducing the donor's lung capacity by 40% so with 2 donors each donating a lobe, each of them loses only 20% lung capacity.

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    1. If you live in the US, start here http://www.transplantliving.org/living-donation/ to find out more about live donation.

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  16. Looking forward to reading more on this topic.

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  17. There is a PFF summit from bench to bedside Dec 5-7 LaJolla Ca. Some days are for professionals and others are for anyone interested in Pul Fibrosis. More info at Pul Fib Foundation's site. Gailinpa

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  18. Hi Doc,

    Thanks for the insightful post, you've done a great job of covering lung tx and weighing the pros and cons!

    I try not to think about these things but my dad died in March this year. He was in the hospital on 10L of continuous O2 (at rest..) and the doctors said he had lung failure. I asked the docs if it was possible to give more O2 to keep his stats healthy but they said that it's not worth it and that you can't give more than 10L of continuous O2. Eventually his heart failed because it was pumping too hard... But his X-Rays, etc. did not show any new scarring - but the docs said that this was it and didn't really want to help him along, even if he was in AE and may have made it out of it. (same doctor had earlier prescribed Azathioprine and Prednisone in a combination - known to be unsuccessful and extremely dangerous)

    Yet, I often read about people using more than 15-20L of O2 at times (another commenter right here said that he uses 18-25L while moving!). This kind of information confuses me. I know that we were up against a wall at the time, but I definitely want to understand if an IPF patient can take more O2 during an acute exacerbation to help them through it. I want to make sure that in the future, patients in India can demand what they can get rather than something about 10L being too much and an attitude of "Oh let him die cause he's going to die anyway".

    They told us mechanical ventilation was our only option to improve his stats (he was maintaining 68-70% at 10L of O2), they had tried a CPAP machine but he was not comfortable with it. Thus, they said that we should mechanically ventilate (but they recommended against it) or that we should give him morphine to just make him comfortable. He had already had atrial fibrillation the previous night which they had managed to control with some medication. He died within 15-20 minutes of administering the morphine.

    My question is this: Would it have been possible to provide more O2 to help improve his stats ? What is the max level of continuous flow O2 provided to IPF patients before the 'giving up' situation is reached ?

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  19. Hi Ashray. I can feel the sadness and frustration you experienced with your dad's disease and his last days. Your description of someone's last days on Earth after battling Pulmonary Fibrosis is heartbreaking and all too accurate. I am sorry about your father's passing. I hope he didn't suffer too much.

    You question about oxygen use is a really important one. In general, we try to give as much oxygen as we can on order to ease shortness of breath, minimize pulmonary hypertension (and strain on the heart), and to make sure that the brain and other organs are getting enough oxygen. Yes, we do often give oxygen at flow rates of 15 liters per minute or more. In the hospital, we can also now deliver up to 40 liters per minute using newer devices. Having said that, I don't know the details of your father's condition at the end of his life -- everyone's situation is different, and it is difficult for me to comment on anyone's specific medical condition or medical care without access to details about their condition and treatments. For example, in some cases, we avoid high oxygen levels in the blood because some people might slow down their breathing too much and even stop breathing all together. This particular situation applies to people who already have high carbon dioxide levels in the blood. In some areas of the world, it is difficult to achieve high flows of oxygen -- although I don't believe this is the case in India.

    I don't have any words that will ease your mind about your father's disease or his passing. It sounds like he was gravely ill and his doctors may have thought he had little time left. Morphine and oxygen are often used in this setting to ease suffering. I hope his passing was peaceful. Sometimes that is all we can ask for.

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  20. My father had a double lung transplant in 2010 becasue of IPF but passed shortly thereafeter becasuse of complications. To all those patients considering this option ask as many questions as you can. This is true for the family and support team too - you will all need each other so educate yourself as much as possible. Build a strong relationship with your doctors - in the months that followed from the time my Dad was put on the list until he received his transplant the relationship we had with the dr truly allowed us to hold on to our hopes. Dr. Lederer is a great contributor to the PF/IPF community as you can see from his posts - as well as other groups. Do not be afraid to ask all of these people the questions you have, no matter how small you may think they are, or how tough they are to ask - I promise...it makes a difference!

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    1. Hi Nick. True words! So good to hear from you. I hope you and your family are doing well.

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  21. I was diagnosed with IPF six years ago. I believe I've actually had the disease for almost 15 years but was ineffectively treated for asthma. I had a biopsy six years ago that confirmed fibrosis. I am on oxygen 24/7 but have painful stomach cramps that have kept me from staying on a weight loss diet that would enable me to be on an active transplant list. Do any other IPF people have this problem?

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    1. Hi. I know quite a few IPF patients and do not remember hearing about this issue often in connection with the disease. I know GERD is often present in people with lung diseases. It could be that the cramps are a separate issue and that seeing a gastroenterologist might helpful. I also wanted to say that many IPF patients in the early stages are misdiagnosed with asthma. I know I was. Gailinpa

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  22. Dr Lederer I am looking for information about trans tracheal oxygen deliver system. Do you know anyone who is using this system? I don't care about the "looking better" part I want to know if the system does deliver o2 in a way that is better than wearing a cannula. What are the dangers of having the tube inserted into the trachea though your neck? Could you wear a cannula at the same time? Their ad says ... improved mobility, greater exercise capacity, and longer lasting o2 sources. Any information or thoughts would be greatly appreciated. Gailinpa

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    1. Transtracheal oxygen is an excellent option. Information is available at http://www.tto2.com/. I also recommend reading "Adventures of an Oxy-phile", a terrific book about oxygen use written by the late Tom Petty, MD and a number of his colleagues. There is a chapter on transtracheal oxygen that may be very helpful. http://amzn.to/18tSjtI

      The risks of cannula insertion are small. Most people do not wear both a transtracheal cannula and a nasal cannula, but it can be done if oxygen requirement are high and there is a sufficient oxygen supply.

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  23. Per this article in the New York Times from January 2013 “Who Should Receive Organ Transplants?” (http://newoldage.blogs.nytimes.com/2013/01/08/who-should-receive-organ-transplants/?_r=0), the International Society for Heart and Lung Transplantation was to begin working towards revised guidelines this spring [2013?]. The International Guidelines for the Selection of Lung Transplant Candidates: 2006 Update—A Consensus Report From the Pulmonary Scientific Council of the International Society for Heart and Lung Transplantation are now 7 years old and based largely on data from the days when very few recipients over age 65 received transplants.

    Has this update been complete yet? Is there an expected release date?

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    1. Hi Barbara. I am on the committee of doctors writing the revised guidelines and we are hoping to have it done in early 2014. There will be some bureaucratic delay in getting it published, but hopefully the document will be available sometime in 2014.

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    2. Hi David. Sure you're up to your eyeballs getting ready for this weekend's ATS Conference but do we have a publication date yet for the revised guidelines?

      Enjoy your conference. Hopefully you can do a blog post on the Pirfenidone (ASCEND) results and maybe Nintedanib (INPULSIS)?

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    3. Transplant selection guidelines hopefully will be published before the end of the year.

      I will definitely be blogging about the clinical trials. :)

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  24. So Jeff Swigris was correct. He said you were on the committee. Also the bureaucratic delay but at least it's being updated and published soon. Good news! Thank you and Happy Holidays to you and yours.

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  25. I should say Jeff suspected you might be on the committee and asked me to let him know your reply, which I have done.

    Just wanted to take this opportunity to say I hope you really, really appreciate how much your extra time and effort in reaching out to us in your blog (and Dr Jeff in his blog and forum as well) means to us out here in the wilderness. You are both very special people!

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  26. I am Arpan Goenka from India. My mother is suffering from pulmonary fibrosis. She is on 24 hour oxygen concentrator. 4 litre / minute. I guess she can't survive long. I have just one question. Why Indian Government doesnt helps to start lung transplant in India. Population is more than 120 crore but still nothing here.

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    1. Hi Arpan. I think there may be a few hospitals in India that are performing lung transplantation. There is a discussion about this on the Inspire.com website. I recommend that you join inspire.com and search their website for this discussion. I wish you mother the best of luck battling her condition.

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  27. Hi, I am Pankaj Vishwakarma from India, 4 months back i was diagnosed with ILD. IPF my age is 30 yrs, at first i thought this is not a serious disease as i was able to do all my activities quite comfortably but as I started to get treatment suddenly my condition became very severe and i am facing respiratory falure and 24x7 on oxygen. All my life has changed suddenly I am teacher and have two dependents on me my wife and a daughter of 4 years. everything is changed now .. can not even walk.... doctors said there is no cure.. don't know what to do?

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    1. Hi Pankaj. Sorry for the delayed response. I am sorry to hear about your condition. I am largely unfamiliar with medical care in India. Please talk to your doctor about pirfenidone, a treatment for some forms of pulmonary fibrosis. It is available in India (made by Cipla). Please also talk to your doctor about the possibility of being considered for lung transplantation. You may need to travel outside of India for this. Also please take a look at pulmonaryfibrosis.in and pulmonaryfibrosis.org. Best of luck battling your condition.

      Dave Lederer

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    2. Hi Pankaj , My father is also in same situation now, How are you doing now? Is there any better treatment in india you come across. Please let me know.

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  28. Thankyou Dr Lederer.
    I live in new Zealand and we don't have much choice but to die of lung disease. You are doing amazing revolutionary work and I wish you all the very best! Thanks again! ( I have copd like Joanne )

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  29. Hi , I am Mathi , live in Jacksonville FL . My father was diagnosed with Interstitial Lung Disease which has been steadily progressive resulting in Class II dyspnoea.

    The Recent Chest X-ray shows scattered interstitial lung disease nodular and infiltrative - causing puzziness of the cardiac Silhouette.


    I am looking for hospitals for this disease in Jacksonville FL for the Permanent cure. Currently he is in India and getting treatment and no progress on his health. Still its getting worse.

    Please advise any better treatment here in Jacksonville for this.

    Thanks in Advance for your help and valuable suggestions if any,,

    Thanks,
    Mathi

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    Replies
    1. Hi Mathi,

      Sorry for the delayed response. You can find a list of ILD centers in Florida on the Pulmonary Fibrosis Foundation website: http://pulmonaryfibrosis.org/life-with-pf/find-medical-care

      Best of luck.

      Dave Lederer

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  30. Dr. Lederer,

    My father has a form of idiopathic pulmonary fibrosis. If it primarily affects the lower lung lobes, what would happen if the lower lobes were removed? If removing the lower lobes is not a good idea, what about replacing the affected lower lobes with a family members lower lobes? I assume that if it can be done it would be much less risky than a lung transplant? Thank you for your invaluable advice.

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    Replies
    1. Thanks for your question. This idea comes up a lot in conversations I have with people suffering from this disease. I don't know of anyone who has tried to surgically remove scar tissue from the lungs to help people breath better. If we surgically removed the parts of the lungs that appear scarred on a CAT scan, I would expect that new scar tissue would develop over time in the lungs anyway -- eventually that person would suffer from having smaller lungs (due to surgical removal of the scar tissue) and continued build up of scar tissue, leaving them worse off than they would have been if they hadn't had surgery. They would also face risks of undergoing lung surgery. Overall, I don't think this approach would help.

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  31. what about replacing the lower lobes from the healthy lobes of a family member? Is that a good, safe approach?

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    Replies
    1. generally we take out the entire lung and replace it with either a full lung or a lobe (if there are differences in size between the donor and the recipient).

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  32. Could I be a living donor for my mom? How much is the procedure?

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    1. Thanks for your question. Almost every person who undergoes lung transplantation in the United States receives lungs from someone who has died. In 2013, only 1 out of the 1,923 lung transplants performed that year used a lung (really a "lobe" -- or about half a lung) from a living donor. In 2014, all 1,925 lung transplants used lungs from someone who had died. Outside of the United States -- for example, in Asia -- the procedure is performed more frequently. I encourage you to talk to you mother's lung transplant team about this. They can also discuss the costs involved with lung transplantation.

      Best of luck to you and your mom.

      Dave Lederer

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    2. can 2 live related donors give up lung to fanily member?

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  33. i meant give lung lobe to patient from to live family members?

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  34. My dad has sarcoidosis and is struggling. He was told because of the presence of the scar tissue that he is not a candidate for lung transplant but that doesn't make sense to me... it would seem that the scar tissue that is interfering with his breathing would make him a prime candidate for a transplant. Perhaps I am not understanding this?

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    Replies
    1. Thank you for your question. I can't be certain about why a transplant center told your father that he is not a candidate. One possibility is that in some folks with sarcoidosis, the scar tissue is both in the walls of the airsacs (inside the lungs -- this is pulmonary fibrosis) and on the outside lining of the lungs. The outside lining of the lungs -- like the peel of an apple -- is called the "pleura". Scarring of the pleura can make transplant surgery more difficult. In some cases, this scar tissue can increase the risk of bleeding and other complications during and after transplant surgery. It is possible that your dad's doctors decided that the risks were too high because of this kind of scar tissue. I encourage you to ask your dad's doctors to give you more details about the reasons for this decision. Your dad can also consider being evaluated at other transplant centers. You can find a list of lung transplant centers in the US here: http://www.srtr.org/csr/current/Centers/TransplantCenters.aspx?organcode=LU

      Best of luck
      Dave Lederer

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  35. Doctor, your straightforward, understandable -to-laymen blog content and your replies to questions are incredibly helpful to this ILD sufferer. I am optimistic that I will get on a list. I surely am trying!
    Thank you. Thank you.

    Joe

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  36. Thank you Doctor Leberer for all the great replies ! I have three questions. First, I have had COPD and IPF for 4 years, on oxygen 2 years currently at 20 LPM and my tests show I'm down to 13 %. I had an assessment in March 2015, in Toronto, got a stent put in, had to wait 8 weeks, then at the last minute they said I have to wait 4 years because I had melanoma removed from my arm in 2014. My questions are 1- Is there any hope of a test that could detect the melanoma cells in my body so I wouldn't have to wait 4 years ? If I were to take a low dose of morphine or medical cannabis, would that disallow me to be considered in the future for a transplant ? 3- Is there any suggestions that you have that might help me stay around for 4 years ? Thanks again for your great service !

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    Replies
    1. Thanks for sharing your experience. I am sorry to hear about everything you are going through. You have asked three excellent and very important questions. From where I stand, I honestly don't know that I can answer them -- and I'd rather be honest than give you a long-winded answer that doesn't get at what you are truly asking me. Here's what I can say: First, each transplant program ends up making their own decisions about to whom they will and will not offer a lung transplant. Some programs say that any history of cancer within 5 years or any use of marijuana within the past 6-12 months would be an absolute barrier to lung transplantation. Other programs are more lenient. I encourage you to talk to your doctors to see what your options are. I wish you all the best.

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  37. hi David, thanks for the article. my dad has IPF and has just been told that he's to be put forward for lung transplant waiting list. he is 70. would 1 lobe from me and 1 lobe from another sibling (we're all in our 20s/30s) be any good to him if we donated? or does the 1 lobe donation only work when going from an adult to a child or young adult?

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  38. This is a great article. Comprehensive, lucid, easy to understand and in my estimation one of the best articles on Transplant I've seen. Have you thought about updating it with the advent of Esbriet and Ofev or any other changes in the technology of transplant or donor awareness Dr. Lederer? Thanks for writing this - it's needed.

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  39. Thanks Bill. Good idea -- will think about this.

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  40. My wife is a 24 year post lung transplant recipient and lives a great quality of life. She was transplanted at Barnes Hospital St Louis by Dr. Joel Cooper who pioneered lung transplantation. We are greatful to the donor family and blessing from above that has allowed her to watch our daughter grow up and know who her mother is. Thanks for your perspective in your article. Tim Merritt

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  41. Hi, I'm writing you desperately. My mother inlaw has PF. We are from South Texas and she got a call to go for an evaluation at San Antonio. We travel up there and after seeing the doctor he told us that she wants eligible for a transplant. One because she's to weak and another because cancer runs in her family. He said no other hospital would consider her for a transplant because of that. Which is heart breaking. She's on oxygen 24/7. Other than PF she has no complications. So my question is. Is it true that once 1 hospital turns you down, every other would do to? Just because cancer runs in her family? I hope you can answer my question. TIA & Thank you for taking time on us.

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    Replies
    1. Hi Alexia. No, it is not true. Just because one transplant program said "no" does not mean that all programs would say no. Please talk to your mother-in-law's doctor to discuss referral to other lung transplant programs. Please give you mother-in-law my best wishes.

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  42. I know I misspell words. I was trying to type that they told us SHE WASN'T ELIGIBLE FOR A TRANSPLANT.

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  43. Thank you for your article doctor. My post here is somewhat belated, but a conversation I had today got me to looking into lung transplantation statistics again. I lost my wife to IPF five years ago. She was a very young 50 year old woman. If ever there was a good candidate for transplant, it was her. She never smoked, didn't drink alcohol, and was very active with yoga, Zumba, dancing, etc. She was amazingly positive, not only before her diagnosis, but during her illness as well. While she was at the top of the transplant list, no organs became available. It's so very difficult to come to terms with loss when you think you're doing everything right, and things just don't work out. It's sad to see that things haven't changed much in the past 5 years. Since the statistics aren't likely to improve anytime soon in regards to the numbers of patients and available organs, and also in post-transplant survival rates, the best hope is in the development of new treatments. Research into causes and treatments of diseases such as IPF will hopefully improve the odds for people suffering from those illnesses.

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    1. Thank you for sharing your wife's experience here. I am very sorry for your loss. Yes, research into causes and treatments is critical. Many folks (including myself) are working to identify new treatments and work to prevent and cure the disease.

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  44. I am a young 54 year old woman, diagnosed with IPF 2 years ago. I have met the transplant team, and I've very close to being "activated" as they called putting me on the list for transplant. What if you have changed your mind. What is the treatment then? None of my doctors have ever really talked to me about this as an option. It's explained that a transplant is going from a terminal chronic disease to a manageable chronic disease. Even though I have seen some amazing people, with great outcomes from having undergone the transplant in my Peer Support group, I'm just not thinking I want to do this. There is literally only myself and my husband. We have no children or family members to help. We have friends but they're all dealing with full plates themselves. I've seen the stress health problems put on a marriage and I really don't want to put us through that. Having to live near the hospital for several months after transplant, rehab, "bumps" as they call them. Knowing both medication and other different medical problems are common. The 5 times a day of heavy medication for the rest of your life. The constant stress of worry over rejection. The lungs being an "open system" making you more susceptible to infections. The long drives back and forth to the hospital in the future. I've had a good life...how about if all you want to do is maintain what you can for as long as you can. Why do none of my doctors ever seem to want to suggest this as an alternative? I'm not sure what to do now. I know I have to say something to my lung specialist. How do you have this conversation?

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    Replies
    1. I want to thank you for bringing this up. The decision to proceed with listing for lung transplantation is an extremely important one. You should know that you are not obligated to be listed -- you can and should make your own decisions about your health after being educated by your healthcare team about your options. Not going on the list IS an option. I do encourage you, as you said, to talk to your lung doctor(s) about this.

      I will point out one important part of the conversation that comes up frequently in my own practice. I have had many patients tell me that they do not want a transplant -- and then months or years later, after their condition has substantially worsened, they change their mind. This is a major problem -- because by the time one's condition has worsened, there may not be enough time to be evaluated and then wait on the waiting list for a lung transplant. I have seen too many people die waiting for a transplant because they made the decision to proceed with listing too late in the course of their disease. If you think you might want a transplant in the future when you are sicker, more limited, and suffering more from your disease, talk to your doctors about when you should be listed for a transplant. better too early than too late in my experience.

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  45. There was a time i thought COPD emphysema has no cure and it only can be controlled,i guess i can proudly say there is %100 cure for copd disease,i was diagnosed of Chronic obstructive pulmonary disease (COPD) for over 9 years which i thought there was no cure for it just as everyone here thinks,my condition was very critical which i was very afraid that will not survive it.
    i took all the medication which doctor prescribe for me but nothing work out totally but i was able to live with it for the few years tho i still cough all day and seize of breath,i came across a post online which they were discussing how this herbal foundation help them cure their diseases,i was like wow does this really exist so i contacted the herbal foundation immediately so they gave me instruction on how the process goes which i followed exactly,i can proudly say i'm fully cure of copd emphysema without any side effect.
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    ReplyDelete