Yes, imuran or this class of drugs is not approve for this disease. As you stated, one of the risk of using immunosuppressants are the risk of infection like fungal.
Imuran may not be approved for this disease but it does possess radioactivity, this might be the reason behind its use here.
since you said she is showing signs of improvement then it may worth keeping imuran as part of the therapy while monitoring for any sign of infection
Yes, that would be great.
We can chat here as well- I understand that you are concerned. I will certainly try my best. Before we start, is there anything else I should know that may have her lung function to have decreased? Any other changes in medication? Anything else at all that you think I should know, please let me know.
I will be going through some literature tonight and tomorrow as well. Whatever information I find I will certainly pass along to you if that is OK with you.
It takes me a little bit of time to sift through all the clinical trials. There are literally millions of articles out there.
I will keep looking and posting until we found an answer for you. :)
Just checking to see if i can expect an answer by tomorrow, 5.13.12?
I just posted- and it all got erased- This is basically what I said :
PLCH is usually treated drugs such as Methotrexate, Etoposide, cyclophosphamide, and Vinca drugs such as Vinblastine. A main stay of treatment for PLCH is smoking cessation, since a good chunk of PLCH cases do occur because of smoking.
Imuran isn't used for this condition- I searched through indexes including Medline , to pluck some articles, but came up pretty short. The only thing that I did find was the use of Imuran in Pulmonary Fibrosis- However, the drug doesn't have the indication for this use.
Imuran is a potent immunosuppresant- It is possible that the drug is suppressing the immune system, and there may be a fungal/mold infection inside her lungs, including Aspergillus. Imuran, coupled with steroid use and the condition itself, may all lead to this condition.
I would certainly speak to the MD regarding this. They may be treating the Pulmonary Fibrosis itself, rather than the PLCH. Pulmonary Fibrosis can occur due to PLCH. Does this make sense?
Please reply, and let me know if you need any clarification. From the researched I've performed, using Medline, I've search the terms Azathioprine + PLCH . Nothing specific had come up in this search.
My reply to your last question got erased too. Did you get it? If not, i will retype it and forward.
Thank you for being so thorough. I truly appreciate it.
The PLCH was diagnosed from several biopsies from the right lung after several submillimeter nodules were noticed by a new doctor. She had the nodules for quite a few years without any noticiable change. The new doctor wanted to do a biopsy, so he did it via a video assisted thoraoscopy (VAT). There were no nodules noted in the left lung. However, since starting the prednisone (for about six weeks and then weaned off) and Imuran, both lungs have become seriously compromised. It is my understanding that PLCH in adults is relatively benign (but not always). However, her disease has progressed very rapidly and now involves both lungs per additional x-rays and CT scans. She needs oxygen constantly. Before the Imuran, she did not need oxygen any more than you or I.
Is there anyway you or I can forward these messages to one of your pulmonologists or oncologists? I would be happy to pay for another opinion or two about the actual disease, how the biopsies were obtained, if there was even a need to have the nodules biopsied, and the rapid deterioration of her lungs. The doctor is fixated on PLCH but i really doubt that is the reason for the rapide deterioration of her lung functioning.
I will wait for your next message. A day or two or even three is reasonable.
I guess I am not sure of how the billing works. Do I get billed each time you send me a message or for your time and research?
I am very pleased with, and grateful for,your interest, thoughtfullness, and determination to help me with this problem. I would like to continue our diaglog as the medication issue is, I believe, the major problem. The disease and its diagnostic pathway are of interest to me, but only as secondary issues. There is no doubt the disease exists. The treatment with Imuran is of primary importance as I think it has done more harm than good. I am looking forward to your next message.
No problem at all! And like I said, I will try to address all of your questions- I will give you an overview of the pathology of PLCH as well.
I would say it is POSSIBLE that the new onset infections can be due to Imuran. I don't want to say 100% because it is hard for me to see the whole picture of a patient via a computer screen.
Don't worry- I will keep you updated for sure. And if you have any more comments or questions, respond as much as you'd like.
I apologize for not getting back to you sooner. You have been extremely helpful. It is clear that you really care about what you do. I don't any more questions about the medications or maybe i just do not know what to ask or comment on. I will keep you in mind if something else comes up. If this conversation has stimulated your interest and you have any more to add in the future, I am more that happy to continue. At this time, I am concerned that the doctors will not let go of the PLCH diagnosis as I believe something much more serious is going on.
Thank you very much. I am 100% satisfied.
Still haven't heard back from any associates of mine. Let me give you some information.
The Biopsy would probably be your best piece of information to diagnose this. I would say it was pretty accurate in that sense.
I've pulled a relatively good article, albeit from Europe. It is from 2006, but gives a pretty nice summary. I can't send it to you, but I will give you the PubMed ID #:
I really don't like using articles unless they are usually within 1-2 years of the date, but this article seems pretty accurate. Let me summarize PLCH in terms of orphan disease status.
The article goes on to describe PLCH as a disease affecting people aged 20-40 years old, and usually due to smoking. The definite diagnosis is confirmed (as you mentioned) via lung biopsy. In this article, it describes PLCH as an Orphan disease.
An Orphan Disease relates to an Orphan Drug. Don't quote me on the exact numbers, it has been some time since I've learned about this topic in depth. Basically, Pharmaceutical companies want to make money, however, diseases that are rare (I believe "rare " was designated by the government if there are 2 issues . First is if the disease is affecting less than 1/200,000 people. Second, there is no other treatment option). PLCH, according to this article, seems to fit this category. Since there is no real avenue for profit for pharmaceutical companies (Nobody has the disease, so why try to make a drug?) a law was passed that allows companies that DO make drugs for orphan disease, can receive patent extension (generics cannot be made), and also receive stipends and other types of funding from the government. This could be why there isn't really a specific treatment for PLCH.
With that being said, the Oncologist that prescribed the Imuran may be attempting to use it to treat the PLCH just out of experience basis.
I can't say if there is something else going on to be honest- The biopsy SHOULD be pretty accurate. However, you should voice your concern with the use of Imuran in terms of infection related issues. As I mentioned, the Oncologist may have experience using Imuran in PLCH, or in LCH in general, since it can be a multi-organ disease.
Let me link you here. As I mentioned, I can't really send articles to customers, because I have access via my institution, but click this link tell me if it works for you. If it does, try to read through it. It gives a pretty good overview of the disease. It has a good amount of technical terms, so if you have any questions at all, please shoot me a reply. Here is the link:
Try to click the "Full Free Text" portion. If you can't open, PLEASE DON'T PAY FOR IT! IT is crazy- They make you pay an enomorous amount, such as 35 dollars, sometimes only for 24 hour use. If you can't open it, I will translate everything to you. I think it may be easier for you just to check it out, then we can discuss.
I will try to contact my associates tomorrow. I sent them an email, but I will try to give them a call if they do not answer. I'll wait for your response, and try to ask a doctor on JA- , and I will see if he/she has any input on this as well.
Let me know if that works for you Glenn.
Thank you for the information. I read the article and it makes sense except for the discussion of the disease at the cellular level. My mother-in-law was hospitalized and the Imuran stopped. I think they are looking at a possible lymphoproliferative disease - which i probably will never understand. She has new doctors, the pulmonologist who prescribed the Imuran is now out of the picture. I simply will have to trust the new doctors now.
You definitely have gone far beyond any expectations I ever had. Thank you for obviously being a very caring person.
Did you ever hear the short stories by Paul Harvey..."Now you know the rest of the story?" I would like you to know the rest of this story. It is 47 pages long. I will try to email it to you tonight at this address or if you have a private email I can send it to as it is for your information only. Please let me know.