I have a quick question in regards to inhaled GM-CFS. What is the best solution to use when mixing the GM-CFS. The document that comes with the medication does list a few different options. If I use distiled water the shelf life in the documention mentions that it only good for a max of 6 hours. I will be splitting the bottle into 2 (every 12 hours).. So disstilled water wouldn’t be an option for the second dose. Other option is bacteriostatic water which give a shelf life of 20 hours or day (sorry don’t have the paper work handy). With the bacteriostatic i google it in regards to using it in a nebulizer and there are issue with it causing bronchitis.
I anyone could help fill me with some information and the best way to do this it would be great as i can not find a lot of information in regards to this.
Thank you,
Daryn
0
Chris Hameetman
Feb 1
Daryn:
For inhaled GM-CSF, I only use sterile saline. I once mistakenly reconstituted GM-CSF with sterile (regular) water and I went into an immediate coughing fit. Most people I know can not tolerate breathing in regular vaporized water. [https://www.atsjournals.org/doi/abs/10.1164/arrd.1983.127.6.691?journalCode=arrd]
And you are correct that the nebulization of bacteriostatic saline, containing benzyl alcohol as its preservative, can cause bronchitis in healthy adults. [https://www.ncbi.nlm.nih.gov/pubmed/7807035]
Unfortunately, I do not have any data on the shelf life of GM-CSF reconstituted with sterile saline. A brute force idea for consideration by your doctor would be to take your 2 doses within 6 hours of each other (assuming the shelf life of saline would be equivalent to distilled water.)
I will reach out to our doctors to see if they are aware of the shelf-life of a split GM-CSF vial reconstituted with saline.
Warmly,
-Chris
Daryn Wynne
Feb 2
Hi Chris, thank you for your quick reply.
My last 2 doses were mixed with sterile water which didnt seem to bad. But if saline would be a better option I will go get some tomorrow.
I have a few question if you dont mind as I dont have very much information on the medication and my doctors seem to be in the same boat.
In the next day or two I am planning on making a post with a little bit about myself and what has been going on since I got diagnosed with aPAP. Hopefully it can help in some way.
So I was discharged from the hospital this afternoon, I had my right lung lavage on Wednesday with 40L (Left is scheduled for next Friday) which went pretty good other then a little bit of a fever yesterday which they decided to keep me for an extra night. I was also pretty surprised Wednesday to find out that the Government of Canada approved to fully fund my GM-CSF treatment. I think my doctor only put the application in a few weeks ago.
My doctors along with most people have very little information in regards to PAP and even less when it comes to treatment. So I was hoping I could find some answers here.
I was directed to take 125mcg twice daily using the PARI LC PLUS nebulizer for one week on and one week off. I mixed the GM-CFS with 1 unit of sterile water, extract half a unit and add another 4.5 units which goes into the Neb. The pharmacist expressed that the bottle is for 1 use only and the remaining should be discarded which I my mind is crazy waste of money. So what i was planning on doing was just to space the doses 6 hours apart.
With that being said the pharmacy gave me 2 weeks worth 15 bottles which doesn't added up if im tossing out half it.
So if you or anyone could suggest the best way to get the most out of this medication it would be greatly appreciated. Looking for info like:
-Time of day to take day
-Time between treatment daily
-is one week on one week off is that the way to go?
-How much water/saline to mix for the nebulizer....
Would like to thank you and everyone else for any help with this. Also in regards to aPAP if anyone has any questions for me feel free to ask, I am pretty much an open book. I will also have a lot of information about me and my history when I make that post.
(Apology's for the run on sentences and poor grammar, i dont have Grammarly lol)
Regards,
Daryn
Emily
Jun 27 · Edited: Jun 27
Hi Daryn,
I hope all is well with your treatment. I had good stability on inhaled GM-CSF for about 4 years but had it pulled by the government in early 2017. My specialist has been trying to get it back for the past few years with no luck. Since 2017 I have had two sets of Lavages and am now headed into my third. I am located in Vancouver, BC and am wondering if you might be able to share with me who your physician is and where in Canada you are located?
My email is emilyvickery09@gmail.com and I would love learn more about how you got access to GM-CSF as I have been under the impression that there was no access to it in Canada.
Appreciatively,
Emily
Chris Hameetman
Feb 2
Daryn:
Sounds like you were given the "standard" starting treatment. 125ug x2 a day one week on & off. This is how many of the early clinical trials were designed, so it is not surprising.
As many have found, the treatment that works best for you may be different. So any thoughts I (or any PAP Patient on inhaled GM-CSF) share will be a reflection of their personalized treatment refinements.
That being said, here is my quick summary:
Started on inhaled GM-CSF 125ug x2 a day, every day -- no week off.
Splitting a vial got really annoying very quickly (as you are experiencing), so after about 2 weeks I started doing the full single 250ug dose once a day.
After 6 months, 250ug a day did not work for me, and I had to go in for another lavage.
Immediately afterward, I upped dosing to 250ug x2 a day -- no week off.
Since then (December 2016) I have not needed another lavage, and I feel pretty good!
I recently had a CT, and my lungs are not clean yet, but they are clearly not degrading as before (my previous cycle was to have a lavage every 6 months.) So at over 2 years without a lavage, inhaled GM-CSF has been a very effective treatment for me.
-Time of day to take day
I take my dose at 8AM and 7PM; although this is more a function of what is convenient vs. medial methodology
-Time between treatment daily
See above
-is one week on one week off is that the way to go?
Tricky question. I do not think this would work *for me* but may be right for someone else with a different physiology. My personal opinion (and I am NOT giving any medical advice here) is that more consistent treatment is better, as you are trying to differentiate/mature macrophages with GM-CSF so they start the process that naturally clears your lung surfactant, so why take your foot off the gas? A counter argument would be not wanting to take more drug than necessary, which is understandable, yet my personal opinion is the risks associated with frequent lavages is greater than more frequent GM-CSF inhalation.
-How much water/saline to mix for the nebulizer....
For each 250ug vial, I reconstitute with about 2 to 2.5ml of sterile saline.
I currently use a PARI SPRINT LC nebulizer FYI.
Warmly,
-Chris
Daryn Wynne
Feb 2
Hi Chris,
I picked up some sterile saline this morning. Do i still mix the medicine with the sterile water or are you just using the saline? I was also searching around on the web and saw a few things on bringing the vial up to room temp before mixing it?
Once again I really appreciate your help.
Thank you,
Daryn
Chris Hameetman
Feb 2
@Daryn Wynne (disclaimer: I am not giving medical advice - just sharing my own methodology -- please consult with your doctor!) OK, with that out of the way, I inject/reconstitute the vial with sterile saline, agitate and then extract for use in my nebulizer. As to temperature, I always thought that was more an issue for injection (vs inhalation), but warmth should help the solution mix more quickly regardless. I just put the vial in my pocket when I take it from the refrigerator, and by the time I have gotten to my neb, it has heated it up a bit. This is going to be a daily part of your life for it to work, so I have found it most effective to keep things as simple as possible.
Daryn Wynne
Feb 2
I totally understand the the disclaimer and I am making a list of questions I have for my doctors which I plan on going over with them next Friday when I go in for my lavage. I greatly appreciate and thankful for all your help!
Chris Hameetman
Feb 4
@Daryn Wynne Follow-up on " shelf life of GM-CSF (Leukine) reconstituted with sterile saline" -- I was unable to get an answer from any clinician, as I suspect no one ever did a study on this issue, and thus there is no trustworthy answer available. However, I did learn that the Savara form of GM-CSF (Molgradex) currently in clinical trials for aPAP treatment comes as a liquid fully mixed (no reconstitution required) as compared to Leukine (GM-CSF) that comes as a powder requiring reconstitution and is currently being used off-label for aPAP. So hopefully this more easily usable form of GM-CSF will be available soon to aPAP patients via Molgradex.
I've recently been ordered the inhaled Leukine treatment and received it in the mail just yesterday. I've read all the literature and attended the PAP Education Day in Cinncinati, so I feel confident that this is the treatment for me. But before I take the first dose, I'd like to hear if anyone has experienced any side effects from the inhaled treatment? I'm a nurse, so I know any of the listed reactions are possible, but would rather hear what is most likely, if anything. Any thoughts? Thank you in advance for any reassurances.
Hello fellow PAP survivors! I wanted to start some discussion regarding inhaled Leukine for PAP treatment. I was diagnosed in Dec, 2017 and had my lavage in Denver with Dr. James Ellis. I have been on inhaled Leukine (250mg per day) since then and although my lungs are still cloudy on the xrays, my O2 levels are pretty good during the daytime (unless I exert myself) but still need oxygen at night. Would increasing the dose to twice per day help clear the lungs better? Anyone else have input on dosage with leukine and how long to see results? Thanks!
Daryn: For inhaled GM-CSF, I only use sterile saline. I once mistakenly reconstituted GM-CSF with sterile (regular) water and I went into an immediate coughing fit. Most people I know can not tolerate breathing in regular vaporized water. [https://www.atsjournals.org/doi/abs/10.1164/arrd.1983.127.6.691?journalCode=arrd]
And you are correct that the nebulization of bacteriostatic saline, containing benzyl alcohol as its preservative, can cause bronchitis in healthy adults. [https://www.ncbi.nlm.nih.gov/pubmed/7807035]
Unfortunately, I do not have any data on the shelf life of GM-CSF reconstituted with sterile saline. A brute force idea for consideration by your doctor would be to take your 2 doses within 6 hours of each other (assuming the shelf life of saline would be equivalent to distilled water.) I will reach out to our doctors to see if they are aware of the shelf-life of a split GM-CSF vial reconstituted with saline. Warmly, -Chris
Hi Chris, thank you for your quick reply.
My last 2 doses were mixed with sterile water which didnt seem to bad. But if saline would be a better option I will go get some tomorrow.
I have a few question if you dont mind as I dont have very much information on the medication and my doctors seem to be in the same boat.
In the next day or two I am planning on making a post with a little bit about myself and what has been going on since I got diagnosed with aPAP. Hopefully it can help in some way.
So I was discharged from the hospital this afternoon, I had my right lung lavage on Wednesday with 40L (Left is scheduled for next Friday) which went pretty good other then a little bit of a fever yesterday which they decided to keep me for an extra night. I was also pretty surprised Wednesday to find out that the Government of Canada approved to fully fund my GM-CSF treatment. I think my doctor only put the application in a few weeks ago.
My doctors along with most people have very little information in regards to PAP and even less when it comes to treatment. So I was hoping I could find some answers here.
I was directed to take 125mcg twice daily using the PARI LC PLUS nebulizer for one week on and one week off. I mixed the GM-CFS with 1 unit of sterile water, extract half a unit and add another 4.5 units which goes into the Neb. The pharmacist expressed that the bottle is for 1 use only and the remaining should be discarded which I my mind is crazy waste of money. So what i was planning on doing was just to space the doses 6 hours apart.
With that being said the pharmacy gave me 2 weeks worth 15 bottles which doesn't added up if im tossing out half it.
So if you or anyone could suggest the best way to get the most out of this medication it would be greatly appreciated. Looking for info like:
-Time of day to take day
-Time between treatment daily
-is one week on one week off is that the way to go?
-How much water/saline to mix for the nebulizer....
Would like to thank you and everyone else for any help with this. Also in regards to aPAP if anyone has any questions for me feel free to ask, I am pretty much an open book. I will also have a lot of information about me and my history when I make that post.
(Apology's for the run on sentences and poor grammar, i dont have Grammarly lol)
Regards,
Daryn
Hi Daryn,
I hope all is well with your treatment. I had good stability on inhaled GM-CSF for about 4 years but had it pulled by the government in early 2017. My specialist has been trying to get it back for the past few years with no luck. Since 2017 I have had two sets of Lavages and am now headed into my third. I am located in Vancouver, BC and am wondering if you might be able to share with me who your physician is and where in Canada you are located?
My email is emilyvickery09@gmail.com and I would love learn more about how you got access to GM-CSF as I have been under the impression that there was no access to it in Canada.
Appreciatively,
Emily
Daryn:
Sounds like you were given the "standard" starting treatment. 125ug x2 a day one week on & off. This is how many of the early clinical trials were designed, so it is not surprising. As many have found, the treatment that works best for you may be different. So any thoughts I (or any PAP Patient on inhaled GM-CSF) share will be a reflection of their personalized treatment refinements. That being said, here is my quick summary:
Started on inhaled GM-CSF 125ug x2 a day, every day -- no week off.
Splitting a vial got really annoying very quickly (as you are experiencing), so after about 2 weeks I started doing the full single 250ug dose once a day.
After 6 months, 250ug a day did not work for me, and I had to go in for another lavage. Immediately afterward, I upped dosing to 250ug x2 a day -- no week off. Since then (December 2016) I have not needed another lavage, and I feel pretty good! I recently had a CT, and my lungs are not clean yet, but they are clearly not degrading as before (my previous cycle was to have a lavage every 6 months.) So at over 2 years without a lavage, inhaled GM-CSF has been a very effective treatment for me. -Time of day to take day
I take my dose at 8AM and 7PM; although this is more a function of what is convenient vs. medial methodology
-Time between treatment daily
See above
-is one week on one week off is that the way to go?
Tricky question. I do not think this would work *for me* but may be right for someone else with a different physiology. My personal opinion (and I am NOT giving any medical advice here) is that more consistent treatment is better, as you are trying to differentiate/mature macrophages with GM-CSF so they start the process that naturally clears your lung surfactant, so why take your foot off the gas? A counter argument would be not wanting to take more drug than necessary, which is understandable, yet my personal opinion is the risks associated with frequent lavages is greater than more frequent GM-CSF inhalation.
-How much water/saline to mix for the nebulizer....
For each 250ug vial, I reconstitute with about 2 to 2.5ml of sterile saline.
I currently use a PARI SPRINT LC nebulizer FYI. Warmly,
-Chris
Hi Chris,
I picked up some sterile saline this morning. Do i still mix the medicine with the sterile water or are you just using the saline? I was also searching around on the web and saw a few things on bringing the vial up to room temp before mixing it?
Once again I really appreciate your help.
Thank you,
Daryn
@Daryn Wynne (disclaimer: I am not giving medical advice - just sharing my own methodology -- please consult with your doctor!) OK, with that out of the way, I inject/reconstitute the vial with sterile saline, agitate and then extract for use in my nebulizer. As to temperature, I always thought that was more an issue for injection (vs inhalation), but warmth should help the solution mix more quickly regardless. I just put the vial in my pocket when I take it from the refrigerator, and by the time I have gotten to my neb, it has heated it up a bit. This is going to be a daily part of your life for it to work, so I have found it most effective to keep things as simple as possible.
I totally understand the the disclaimer and I am making a list of questions I have for my doctors which I plan on going over with them next Friday when I go in for my lavage. I greatly appreciate and thankful for all your help!
@Daryn Wynne Follow-up on " shelf life of GM-CSF (Leukine) reconstituted with sterile saline" -- I was unable to get an answer from any clinician, as I suspect no one ever did a study on this issue, and thus there is no trustworthy answer available. However, I did learn that the Savara form of GM-CSF (Molgradex) currently in clinical trials for aPAP treatment comes as a liquid fully mixed (no reconstitution required) as compared to Leukine (GM-CSF) that comes as a powder requiring reconstitution and is currently being used off-label for aPAP. So hopefully this more easily usable form of GM-CSF will be available soon to aPAP patients via Molgradex.