Does Kalydeco Eliminate Pseudemonas?
- Thread starter Gammaw
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S
stranger
Guest
There is nothing in the makeup of Kalydeco that addresses pseudemonas. What Kalydeco *does* do is mitigate the amount of junk that collects in the lungs thus, on paper, giving bugs like Pseudo, less opportunities to flourish.
Yes, stranger, that's why I ask. It's my understanding that a whiff, if you will, of pseudemonas for most people (those without CF) does not produce the colonization or infection and subsequent lung decline that occurs in the CF Lung. The non CF lung will rid itself of pseudemonas, clearing it away harmlessly. At least in most instances. It's that sticky CF lung that allows pseudemonas to colonize. So, if Kalydeco helps normalize mucous (does it?) does someone on Kalydeco have a chance of finally eliminating pseudemonas through normalized chanels? Any Kalydeco takers here that have experienced clear cultures where bacteria had been their chronic friend before?
B
BreathinSteven
Guest
I agree with stranger... Some of my take on our infections is that our mucus is so viscous and voluminous that it prevents the antibiotics in our bloodstream (either oral or IV) from getting to the bacteria flourishing in and on that mucus... The infection keeps raging because we can't get water to the fire... But -- I might be totally full of crap!!!
I had a double lung transplant 13+ years ago -- I don't have the mucus problem in my lungs any longer -- I imagine that's similar in someone for whom Kalydeco is a successful treatment... Though I'm immune suppressed and more prone to infections -- I'm usually amazed at how well, and how quickly antibiotics work, compared to when I was battling bugs with my old, CF lungs...
So -- I don't know about whether we will continue to colonize pseudomonas or other bugs, or whether we will finally rid ourselves of them with Kalydeco, but I imagine that may be a strong possibility as I believe that antibiotics that would allow eradication of those bugs should work much better... Love, Steve
I had a double lung transplant 13+ years ago -- I don't have the mucus problem in my lungs any longer -- I imagine that's similar in someone for whom Kalydeco is a successful treatment... Though I'm immune suppressed and more prone to infections -- I'm usually amazed at how well, and how quickly antibiotics work, compared to when I was battling bugs with my old, CF lungs...
So -- I don't know about whether we will continue to colonize pseudomonas or other bugs, or whether we will finally rid ourselves of them with Kalydeco, but I imagine that may be a strong possibility as I believe that antibiotics that would allow eradication of those bugs should work much better... Love, Steve
It does not eliminate pseudemonas :-( I have had some great improvements with the drug, however still end up going in for IVs every 3 months or so because both pseudo and staph grow back. I am very compliant with my breathing treatments, exercise strenuously 6x a week, and still require the IVs that frequently. That is the next step in helping CF patients... figuring out how to completely eradicate all the bugs!
Thanks for sharing your experience nmatthews! I keep thinking about this.....can you help me understand why it doesn't help? For example, does Kalydeco not help correct the sticky mucous problem in the lungs? Can bacterial colonization just become too severe over time? Or...? Any idea what's at work here?
J
jamest
Guest
One issue is the cilia - if there is enough damage to the lung, the cilia may be gone. Doesn't matter how thin the mucus is if there aren't functioning cilia there to move it out.
This sounds like a long term question though, I doubt there will be any conclusive answers on this for five to ten years.
This sounds like a long term question though, I doubt there will be any conclusive answers on this for five to ten years.
So I wonder if those who are younger or haven't cultured bacteria as long have had any better luck eliminating colonized bacteria or perhaps avoiding them altogether....Fascinating question - to me anyway. It makes me wonder about the efficacy of future drugs for other genotypes or other classes of mutuations too.....
Cell correction vs. reversing damage.
This is an excellent point. Kayladeco will not fix the damage from CF over years. It will fix the cause of the damage. Any immediate increase in lung function or pancreas function is due allowing the remaining healthy parts to function better. Just as repairing a dam will not reverse the damage of the resulting flood, it will stop any future flooding.
As the drug is given earlier in life, I suspect that colonizations will be greatly reduced if not eliminated. A person's ability to fight or reverse colonizations is directly related to the remaining healthy tissue. Possibly, some with low lung damage that are colonized will be able to eradicate it.
This is why it is important to start giving the drug to patients before any damage has occurred.
This is an excellent point. Kayladeco will not fix the damage from CF over years. It will fix the cause of the damage. Any immediate increase in lung function or pancreas function is due allowing the remaining healthy parts to function better. Just as repairing a dam will not reverse the damage of the resulting flood, it will stop any future flooding.
As the drug is given earlier in life, I suspect that colonizations will be greatly reduced if not eliminated. A person's ability to fight or reverse colonizations is directly related to the remaining healthy tissue. Possibly, some with low lung damage that are colonized will be able to eradicate it.
This is why it is important to start giving the drug to patients before any damage has occurred.
My two DDs (age 20 and 15) with G551D (for one of their CF genes), have been on Kalydeco since its FDA approval 2/2011. I keep reading about how some people stop enzymes, stop/back off on respiratory treatments, etc., but that has not been our experience. Both girls have been significantly more stable on Kalydeco, though. Younger DD ran cross country the last 2 fall seasons! Older DD had a history of frequent CF flares and was in and out of the hospital for what seemed like every 2 or 3 months for YEARS. On Kalydeco, they no longer take prophylactic oral antibiotics. Older DD still gets the usual bugs that flare her CF infection, but has responded very well to outpatient treatment (we increase treatments from 2x daily to 4x, go on oral antibiotics, take short bursts of steroids---stay on top of symptoms). There have been a few close calls every year, but NO hospitalizations. PFTs are improved (and great) for both of them as well. Their CF doctor and I credit the Kalydeco.
About PA, both DDs are still culturing abx resistant rough and mucoid strains ... no real reduction in that sadly. We are listening to their symptoms and treating those. I have kept my fingers crossed for 2 years that Kalydeco might bring about a significant reduction in symptoms and in their chronic infection. Disappointed about the PA, but still holding out hope. Stability is definitely the Kalydeco bonus for us, and that has greatly improved our family's overall quality of life.
About PA, both DDs are still culturing abx resistant rough and mucoid strains ... no real reduction in that sadly. We are listening to their symptoms and treating those. I have kept my fingers crossed for 2 years that Kalydeco might bring about a significant reduction in symptoms and in their chronic infection. Disappointed about the PA, but still holding out hope. Stability is definitely the Kalydeco bonus for us, and that has greatly improved our family's overall quality of life.
LittleLab4CF
Super Moderator
I think everybody is on the same page. The question really is, ‘Will the new environment in my lungs and body, due to Kalydeco, allow the body/antibiotics to eradicate resistant microbial colonies?” It is a great question, in fact a fantastic question for the future of all CFers.
The answer in part has to do with the types of microbes, bacterial, fungal and viral. Multiple Drug Resistant or MDR bacteria are mostly what the original question was asking about like p.A., MAC and MRSA. Ideally, once the environment in the body becomes undesirable for infection, those that can be eradicated, either by the body’s own defenses or antibiotics should be cleaned out. It looks like it will take a long time for the body to recover. This isn’t a big surprise, I would expect minimum of a year on Kalydeco to see any improvement beyond, not just being so sick. Being on Kalydeco definitely gives an advantage for resisting all future infections because the body can now move mucus and its quarry of pollen and pathogens up and out.
Those that have colonies in the lungs may be in for the long haul. When a colony of bacteria takes hold in the lungs and they can’t get rid of that colony, the lungs wall the infection in. This keeps the infection away from the lungs, but many colonies thrive in their new protective homes away from our blood supply. At some time, the chrysalise may be treated or otherwise removed. TB was famous for being bound up in chrysalise throughout the body, in fact they were said to be consumed by them, hence the other name for TB, consumption. MAC’s and MRSA’s are tubercular pathogens and my guess is they will be fully sealed in as general health improves. In a few years, the antigen levels for MRSA and such will begin to drop but never go away.
MDR bugs are near to impossible to treat by definition and it just makes matters worse when the body’s natural defenses are mechanically compromised. As lungs become moist and the cilia regenerate, assuming they can, and the mucus thins, our lungs begin to function like the filter queens they were meant to be.
The mechanical blocks, keeping our immune system at bay like thick, desiccated mucus lining everything is eventually cleansed after a time on Kalydeco. At least that’s the logic. Although the mucus is now thin and does its job properly, CF seems to have some other issues that I don’t know if Kalydeco can correct.
Most CFers have smaller than usual plumbing or duct work. My Eustachian tubes, pancreatic and bile ducts, sinus openings and every gland from the salivary to the urogenital system are replete with thin sticky tubing. From my understanding, this is all typical in CF. The potential for blockage in the lungs, is unlimited, it is the ideal target. Kalydeco shows it corrects the genetics in G551D and thins mucus throughout the body. This relieves about half of the stress on the body just by normalizing mucus.
I don’t know that the narrower than normal tubing and openings in our bodies will improve. I expect they will stop being physically adhesive or sticky and certainly the mucus will be less inclined to adhere to the walls of our plumbing systems. This common problem with CF may be the result of the CFTR gene error, if so it may reverse itself here after some time. I assume this would be something that affects the growth and structure of our sticky ducting from birth, maybe in the womb.
My question is in part why do CFers have narrow sticky ducting? If poor functioning mucus cells are engorging the walls of the fallopian tubes or common bile duct, maybe the duct works in our bodies will shrink at the walls, leaving closer to normal sized ducts and ports. This is not something I am reading anything about, has anybody leaned what effect Kalydeco might have on the structure of the ducting? This would be a thinning of the duct walls and potentially an opening or widening of all swollen tissue, aggravated by CF. At a larger scale scarring from repeated infections have deformed and distorted our lungs and such and that won't revert.
Anybody have any thoughts?
LL
This question and others remain to be answered and they may be part of our new leases on life.
LL
The answer in part has to do with the types of microbes, bacterial, fungal and viral. Multiple Drug Resistant or MDR bacteria are mostly what the original question was asking about like p.A., MAC and MRSA. Ideally, once the environment in the body becomes undesirable for infection, those that can be eradicated, either by the body’s own defenses or antibiotics should be cleaned out. It looks like it will take a long time for the body to recover. This isn’t a big surprise, I would expect minimum of a year on Kalydeco to see any improvement beyond, not just being so sick. Being on Kalydeco definitely gives an advantage for resisting all future infections because the body can now move mucus and its quarry of pollen and pathogens up and out.
Those that have colonies in the lungs may be in for the long haul. When a colony of bacteria takes hold in the lungs and they can’t get rid of that colony, the lungs wall the infection in. This keeps the infection away from the lungs, but many colonies thrive in their new protective homes away from our blood supply. At some time, the chrysalise may be treated or otherwise removed. TB was famous for being bound up in chrysalise throughout the body, in fact they were said to be consumed by them, hence the other name for TB, consumption. MAC’s and MRSA’s are tubercular pathogens and my guess is they will be fully sealed in as general health improves. In a few years, the antigen levels for MRSA and such will begin to drop but never go away.
MDR bugs are near to impossible to treat by definition and it just makes matters worse when the body’s natural defenses are mechanically compromised. As lungs become moist and the cilia regenerate, assuming they can, and the mucus thins, our lungs begin to function like the filter queens they were meant to be.
The mechanical blocks, keeping our immune system at bay like thick, desiccated mucus lining everything is eventually cleansed after a time on Kalydeco. At least that’s the logic. Although the mucus is now thin and does its job properly, CF seems to have some other issues that I don’t know if Kalydeco can correct.
Most CFers have smaller than usual plumbing or duct work. My Eustachian tubes, pancreatic and bile ducts, sinus openings and every gland from the salivary to the urogenital system are replete with thin sticky tubing. From my understanding, this is all typical in CF. The potential for blockage in the lungs, is unlimited, it is the ideal target. Kalydeco shows it corrects the genetics in G551D and thins mucus throughout the body. This relieves about half of the stress on the body just by normalizing mucus.
I don’t know that the narrower than normal tubing and openings in our bodies will improve. I expect they will stop being physically adhesive or sticky and certainly the mucus will be less inclined to adhere to the walls of our plumbing systems. This common problem with CF may be the result of the CFTR gene error, if so it may reverse itself here after some time. I assume this would be something that affects the growth and structure of our sticky ducting from birth, maybe in the womb.
My question is in part why do CFers have narrow sticky ducting? If poor functioning mucus cells are engorging the walls of the fallopian tubes or common bile duct, maybe the duct works in our bodies will shrink at the walls, leaving closer to normal sized ducts and ports. This is not something I am reading anything about, has anybody leaned what effect Kalydeco might have on the structure of the ducting? This would be a thinning of the duct walls and potentially an opening or widening of all swollen tissue, aggravated by CF. At a larger scale scarring from repeated infections have deformed and distorted our lungs and such and that won't revert.
Anybody have any thoughts?
LL
This question and others remain to be answered and they may be part of our new leases on life.
LL
LittleLab, Thank you. You have no idea how much I enjoyed reading your last post. So much to think about. I hardly realized how hungry I was for all that information, though posed as more questions. I've wondered not only about the biology of CF, but about the effects of Kalydeco and its mechanism for some time. Now I have things to think about.
I admit I'm beginning to miss the days where you could simply go to the library, pull out an encyclopedia for an overview, then find treatises and books that took you through all aspects of a topic - you know, those things with pages that educated you on a wide plane about a topic in a small space. This is what I want for my CF education. You gave me a taste of it. Today there is a murky collection of articles, studies, snippets, opinions, some reliable, some not, all over the place. Lacking cohesion. Making education difficult, unreliable, disjointed. It stymies the progression of thought needed to solve the huge challenges of CF. Like puzzle pieces scattered on a table. But I'm rambling now.
I picture the narrowed tubing in your post similar to an atrophied muscle. Growth stymied by the lack of vigorous exercise, and sluggish supplies of salty life giving fluid. The way the pancreas stops generating enzymes when it's efforts are rendered useless by sticky mucous after a time. Initially optimistic and vigorous in it's expectation of successful function, eventually giving up in defeat. Ive been told that even Kalydeco cannot revive it . . . Once the pancreas stops secreting enzymes that can't make their way through, it quits trying. But then I've also heard some say their enzyme intake decreases.
Are you aware of any authorities that collect this information in a palatable way for those of us who might want it? The CF team when my son was born recommended a book entitled "Cystic Fibrosis: A Guide for Families" that was a bit heady for an emotionally drained and shell shocked parent at the time. Now it would be outdated. Perhaps the author has kept up and updated. I will look. Perhaps it can answer some of these questions. Maybe even the questions about Kalydeco and its effect on the ductwork. Thanks for the stimulus. I will let you know if I find anything.
I admit I'm beginning to miss the days where you could simply go to the library, pull out an encyclopedia for an overview, then find treatises and books that took you through all aspects of a topic - you know, those things with pages that educated you on a wide plane about a topic in a small space. This is what I want for my CF education. You gave me a taste of it. Today there is a murky collection of articles, studies, snippets, opinions, some reliable, some not, all over the place. Lacking cohesion. Making education difficult, unreliable, disjointed. It stymies the progression of thought needed to solve the huge challenges of CF. Like puzzle pieces scattered on a table. But I'm rambling now.
I picture the narrowed tubing in your post similar to an atrophied muscle. Growth stymied by the lack of vigorous exercise, and sluggish supplies of salty life giving fluid. The way the pancreas stops generating enzymes when it's efforts are rendered useless by sticky mucous after a time. Initially optimistic and vigorous in it's expectation of successful function, eventually giving up in defeat. Ive been told that even Kalydeco cannot revive it . . . Once the pancreas stops secreting enzymes that can't make their way through, it quits trying. But then I've also heard some say their enzyme intake decreases.
Are you aware of any authorities that collect this information in a palatable way for those of us who might want it? The CF team when my son was born recommended a book entitled "Cystic Fibrosis: A Guide for Families" that was a bit heady for an emotionally drained and shell shocked parent at the time. Now it would be outdated. Perhaps the author has kept up and updated. I will look. Perhaps it can answer some of these questions. Maybe even the questions about Kalydeco and its effect on the ductwork. Thanks for the stimulus. I will let you know if I find anything.
Another important aspect is that Kalydeco helps people with G551D reach about 50% of normal CFTR channel function (carriers are at about 90% and normal is 100% according to the vertex graphs), and the airway surface liquid depth is about 50% of normal. This means the mucus is much thinner but still thicker than normal.
Kaydeco helps the lungs to be less hospitable to bacteria & also helps the mucociliary clearance/clearing of mucus from the lungs to improve (mucocilary clearance is better but not at normal levels).
For those that start Kalydeco at a young age, prior to bacteria colonisation, they hopefully will be able to stay more healthy (with less/no bacteria) for longer, especially as more treatments are developed.
Many people that I have spoken to on Kalydeco still have the same bacteria, but there lungs are more stable / lung function is higher. I have heard of a few younger patients who have had clear sputum cultures after starting Kalydeco.
I am on Kalydeco and started at age 27 after having pseudomonas for 24+ years. I still have pseudomonas and it is still multi resistant, but my doctors believe it is very likely that my amount of bacteria has decreased.
I am now able to clear sputum via my own mucociliary clearance pretty well, even with colds etc. It is much thinner and there is much less sputum. My only neb is pulmozyme (I do notice a difference if I do not use this) and I am not on long term antibiotics. Also have not needed chest physio in over a year.
When I started Kalydeco I cleared a lot of mucus & inflammation settled which helped my lung function improve significantly (from the 40s to 70% in 1 month, then it plateaued at 70). I also did very intensive physio for the first few months of Kalydeco.
Kaydeco helps the lungs to be less hospitable to bacteria & also helps the mucociliary clearance/clearing of mucus from the lungs to improve (mucocilary clearance is better but not at normal levels).
For those that start Kalydeco at a young age, prior to bacteria colonisation, they hopefully will be able to stay more healthy (with less/no bacteria) for longer, especially as more treatments are developed.
Many people that I have spoken to on Kalydeco still have the same bacteria, but there lungs are more stable / lung function is higher. I have heard of a few younger patients who have had clear sputum cultures after starting Kalydeco.
I am on Kalydeco and started at age 27 after having pseudomonas for 24+ years. I still have pseudomonas and it is still multi resistant, but my doctors believe it is very likely that my amount of bacteria has decreased.
I am now able to clear sputum via my own mucociliary clearance pretty well, even with colds etc. It is much thinner and there is much less sputum. My only neb is pulmozyme (I do notice a difference if I do not use this) and I am not on long term antibiotics. Also have not needed chest physio in over a year.
When I started Kalydeco I cleared a lot of mucus & inflammation settled which helped my lung function improve significantly (from the 40s to 70% in 1 month, then it plateaued at 70). I also did very intensive physio for the first few months of Kalydeco.
JustaCFmom
New member
re: an encyclopedia for an overview
LittleLab, Thank you. You have no idea how much I enjoyed reading your last post. So much to think about...
I admit I'm beginning to miss the days where you could simply go to the library, pull out an encyclopedia for an overview, then find treatises and books that took you through all aspects of a topic - you know, those things with pages that educated you on a wide plane about a topic in a small space. This is what I want for my CF education... QUOTE]
I don't know if this is what you had in mind, but Dr. Littlewood put this site together and it is up-to-date. He is authoritative and was personally involved in CF care. The fact that he is based in Europe, also adds another perspective.
http://cfmedicine.com/history/
We do live in exciting times.