Hi, I just want to answer your question. I am 42 years old. I will die from CF. There will never be a cure in my lifetime or for anyone reading this. There will be drugs..yes. I get frustrated when I see others write how fortunate we are in the last 20 years with the developement of drugs. OPEN YOUR EYES! We are not. We have Pulmyzyme, TOBI, Hypertonic Saline, albuterol, Xopenex, Cayston....ummm, am I missing one or two. THATS IT! We are only 30,000 people. Do you think the drug companies are falling over themselves for a drug that will only help 30,000 people? Oh, and when one does come along it cost $300,000 (Kalydeco)...which only helps 4% of the CF population. There is a cure for CF...its called lung transplant...that will only give you a few years if your lucky
You are using an out of date browser. It may not display this or other websites correctly.
You should upgrade or use an alternative browser.
You should upgrade or use an alternative browser.
When cf will no longer be an incurable disease??
- Thread starter sweetninis
- Start date
MissT:
I'm 30 years older than you are and I really don't expect to die from CF.
My Mother, her Mother, My Dads Mother, my Dads Sister, my Brother and my Sister all died from cancer. I had an Uncle who died when he was hit by a bus. Another Uncle fell from a ladder and died.
There are just too many other ways to go, all of which I have no control over.
Bill
I'm 30 years older than you are and I really don't expect to die from CF.
My Mother, her Mother, My Dads Mother, my Dads Sister, my Brother and my Sister all died from cancer. I had an Uncle who died when he was hit by a bus. Another Uncle fell from a ladder and died.
There are just too many other ways to go, all of which I have no control over.
Bill
Oh missT, I don't know if you have not caught the amazing news that Kalydeco is helping people with your mutation!! There is one girl on here named marcij that it has helped more than the people with G551D at her clinic!! I totally understand your point of view, but they are researching your R117H <img src="i/expressions/face-icon-small-smile.gif" border="0"> Kalydeco is helping my daughter right now, praise God! If your insurance company is not allowing you to get it, hopefully with the new trials it will not be too long before your mutation is added to the list to be on label with Kalydeco. So you have 2 possible helps!! Yeah! You might even qualify to get in on the trials. I wonder how they would interact together? :/ I wish you all the luck in the world!! If you have a minute look in the CFTR Modulation and Gene Therapy for Marci's posts!!! <img src="i/expressions/face-icon-small-smile.gif" border="0">
imported_Momto2
New member
Printer, I think there is somewhat of a TREND towards late diagnosis/less severe, nothing more, and I am entitled to my opinion. It has been my experience that of all my friends with CF, they passed away from lung complications, lung and GI, or Liver and some combo of the previous. Now, this being said, its possible I was in a a biased hospital, or who knows what. But this is what I have lived. Every single person I knew as a kid with severe lung and GI CF died before age 33. Every single one, sample size of roughly 20, and some before they were school age. One distinct possibility is that the GI is so detrimental, it sets off lung and other stuff, and what you primarily see is the pulmonary effects. Certainly failure to thrive status leads to overall compromise of the entire system, and can lead to death, but I have not personally had a friend who was FTT pass away from that alone, though I am sure it has happened. I do not consider myself to have severe GI and pulmonary. I am sure your experiences are different, they would have to be. But lets just drop this before it gets out of hand.
S
Swallowtail66
Guest
I try to honor other peoples decisions and I try not to judge, but I do have an opinion. I am sure some will not like my comments here, but here goes. I think we have to help our future by being careful not to have children with CF. I am pro-life so I am not suggesting creating embryos and testing them and only keeping the ones without CF or aborting fetuses with CF. I think CF carrier testing and gene mapping should be easier to attain and education more readily presented. I think if you know you carry CF, you should be very careful about passing it to your future.
First: I didn't know about my CF status when I had children. I was crushed to learn we had CF. I had a tubal ligation when the second child was 3 because she had the symptoms of the first child and I knew it was going to be bad. I made sure, somewhat against their father's wishes, that I didn't have another child. He has remarried and has had another child...with CF. I have never had more children. My oldest child dated a guy for over 7 years. They were talking about marriage and the issue of children came up. She indicated her reluctance to have children naturally unless she was sure he was not a carrier. She told him that she would be glad to accept adoption or someone else's egg; just as long as the baby did not risk CF. They had a very hard time finding a doctor to test him because she was not ALREADY pregnant. Hello, that would be a little late. Finally, he was tested. He broke up with her the week after he got his results. I guess we know what his carrier status is. Better now that after they were married. He would not have handled having a baby with CF very well either.
Second: I know a young woman who was pregnant and tested positive for DF508. Her husband was tested and he was positive for DF508. They spent several months waiting to find out if their child had CF. He is not even a carrier. She is so pleased now that they dodged the bullet that they are considering another child. I gently suggested she study CF fully befores she makes a decision. I told her that they were blessed once and that the next time she might face having to watch her child suffer in ways she could not yet imagine.
I just think that there are many children in the world that need love and my child with CF has much love to give. Rather than risk her health or the health of her child, I would rather she adopt. I will love her child no matter how it becomes her child. I am very proud of her stand to protect her child long before it comes into her life. I would like our CF to stop here and not continue for future generations to face after I am gone.
First: I didn't know about my CF status when I had children. I was crushed to learn we had CF. I had a tubal ligation when the second child was 3 because she had the symptoms of the first child and I knew it was going to be bad. I made sure, somewhat against their father's wishes, that I didn't have another child. He has remarried and has had another child...with CF. I have never had more children. My oldest child dated a guy for over 7 years. They were talking about marriage and the issue of children came up. She indicated her reluctance to have children naturally unless she was sure he was not a carrier. She told him that she would be glad to accept adoption or someone else's egg; just as long as the baby did not risk CF. They had a very hard time finding a doctor to test him because she was not ALREADY pregnant. Hello, that would be a little late. Finally, he was tested. He broke up with her the week after he got his results. I guess we know what his carrier status is. Better now that after they were married. He would not have handled having a baby with CF very well either.
Second: I know a young woman who was pregnant and tested positive for DF508. Her husband was tested and he was positive for DF508. They spent several months waiting to find out if their child had CF. He is not even a carrier. She is so pleased now that they dodged the bullet that they are considering another child. I gently suggested she study CF fully befores she makes a decision. I told her that they were blessed once and that the next time she might face having to watch her child suffer in ways she could not yet imagine.
I just think that there are many children in the world that need love and my child with CF has much love to give. Rather than risk her health or the health of her child, I would rather she adopt. I will love her child no matter how it becomes her child. I am very proud of her stand to protect her child long before it comes into her life. I would like our CF to stop here and not continue for future generations to face after I am gone.
Printer, I lived in a fantasy for many years thinking, hoping, and begging god for a cure. I fully expect that this disease will kill me. God bless you for surviving till 72.
JennyC, yes, I do know about the Kalydeco trial for my mutation and I also know Marci. I am trying to get into the trial which starts soon. I have to blow a forty...dont know how Im going to do that but I will try my hardest. I am soooooo happy for your daughter. I have read your posts.
JennyC, yes, I do know about the Kalydeco trial for my mutation and I also know Marci. I am trying to get into the trial which starts soon. I have to blow a forty...dont know how Im going to do that but I will try my hardest. I am soooooo happy for your daughter. I have read your posts.
LittleLab4CF
Super Moderator
I think the opening question was ‘how long will it be when we find a cure for CF and no more deaths will result from CF’. A cure, for the living CFers most probably will involve something to mitigate what results in thick mucus. For CFers that have pulmonary presentations the root of all their problems stems from production of mucus made thick by high a concentration of salt. A normal lung secretes mucus from its mucus membrane, which is nearly water thin. The mucus keeps the lungs moist, and it catches everything our nasal system doesn’t catch. In the mucus catch are pathogens, foreign bodies like pollen and mold spores. There is a network of hair cells, or cilia that are designed to move unwanted debris by a wave action of the cilia up and out of the lungs including most of the mucus. Thick mucus overwhelms the alveoli and at best interferes with oxygen transport and at worst collapses the alveoli sac. On some scale of viscosity, the normal mucus transport is either slowed or stopped resulting in both an unhealthy medium for the lungs and an opportunistic medium for infection. And just as a very viscous mucus through multiple pathogen infections, the lung becomes nonfunctional. Apply the same logic to the nasal/sinus system and the infection fighting mucus, catches its quarry of microbes and allergens and right along with the host mucus, it stays. Although it would be disfiguring, we can technically live without a nasal system, non functional lungs result in death. In the GI tract, any and all sources of mucus can be similarly thick this time instead of blocking off effective oxygenation, the transport of digestive enzymes from the pancreas, bile and sticky walled intestines and bowels interfere with nutrition. For the uninitiated, an intestinal blockage, bowel blockage including constipation can be fatal. If caught in time a blockage can be removed. But auto digestion of the pancreas can result in organ death very quickly for newborns, and can be living hell with indescribable pain for anybody going through it.
<strong>Why don’t we have the mucus equivalent of a blood thinner? </strong>That could go a long way if it, like my analog of a blood thinner, thins all mucus, everywhere in the body. I do not have an answer to this, and for sure it would pay off in spades, eliminating a host of diseases not remotely related to CF.
CF is a genetic disorder specifically it is a monogenetic disorder meaning all our misery is a malfunction of a single gene. It is not a disease and it cannot be cured in conventional terms. It isn’t common knowledge, but in an odd way, CF has one parallel with Mendel’s pea plants. It is a seminal discovery, and one of the first genes that is understood at a level, few genetic disorders have enjoyed. Being monogenetic like Mendel’s choice of peas to determine dominant and recessive genetic characteristics, if almost any other plant or even higher life forms would have yielded something requiring a super computer to figure out.
Like war, in order to win, the first rule is to know your enemy. What help is it to continue to tease more and more mutations out of the CFTR gene? We seem to know less as we learn more. Why would an army of well equipped labs staffed with scientists continue to hammer on this disorder with little promise of future profit? The short answer is it is a perfect genetic disorder to learn the big lessons and make the big discoveries that apply to any and most likely all disorders and diseases. Many genetic therapies have been tried as a result of the CFTR gene mutation. I so far has been too little for too much side effects and of course cost. But the CFTR gene, mutation or not presents all the fundamental problems in effective gene therapy. We need a way of harvesting and “growing” large amounts of the healthy gene. We need a vector to deliver it to every cell’s nucleus and further, to deliver it to the correct spot on the correct chromosome. Finally we need something to activate this gene. This will probably result in a few Nobel Prizes, but considering how vast the genetic playground is, having a model to work on and develop tools for is encapsulated in CF genetics. Yes we now have one CF drug that affects 4% of the CF community. If you were in that 4% you would be yelling hosannas and turning cartwheels. If the sum of my life was to ‘cure’ 4% of 30,000 people I would die with a big wide smile.
I see a lot of promising work on a genetic resolution to all CF sufferers. We may have to settle for some time with mutation specific genetically based drugs, but as many people who are taking the steps it took to affect 551 mutation, imagine the impact when DeltaDF508 has a specific drug. I fully expect a panacea genetically based drug in my lifetime. I am 62 and if all goes well, I will see 75, but no longer. So this is my official prognostication of 13-15 years to a panacea CF genetically based drug. If I am wrong, it will be from earlier rather than later discovery.
<strong>Why don’t we have the mucus equivalent of a blood thinner? </strong>That could go a long way if it, like my analog of a blood thinner, thins all mucus, everywhere in the body. I do not have an answer to this, and for sure it would pay off in spades, eliminating a host of diseases not remotely related to CF.
CF is a genetic disorder specifically it is a monogenetic disorder meaning all our misery is a malfunction of a single gene. It is not a disease and it cannot be cured in conventional terms. It isn’t common knowledge, but in an odd way, CF has one parallel with Mendel’s pea plants. It is a seminal discovery, and one of the first genes that is understood at a level, few genetic disorders have enjoyed. Being monogenetic like Mendel’s choice of peas to determine dominant and recessive genetic characteristics, if almost any other plant or even higher life forms would have yielded something requiring a super computer to figure out.
Like war, in order to win, the first rule is to know your enemy. What help is it to continue to tease more and more mutations out of the CFTR gene? We seem to know less as we learn more. Why would an army of well equipped labs staffed with scientists continue to hammer on this disorder with little promise of future profit? The short answer is it is a perfect genetic disorder to learn the big lessons and make the big discoveries that apply to any and most likely all disorders and diseases. Many genetic therapies have been tried as a result of the CFTR gene mutation. I so far has been too little for too much side effects and of course cost. But the CFTR gene, mutation or not presents all the fundamental problems in effective gene therapy. We need a way of harvesting and “growing” large amounts of the healthy gene. We need a vector to deliver it to every cell’s nucleus and further, to deliver it to the correct spot on the correct chromosome. Finally we need something to activate this gene. This will probably result in a few Nobel Prizes, but considering how vast the genetic playground is, having a model to work on and develop tools for is encapsulated in CF genetics. Yes we now have one CF drug that affects 4% of the CF community. If you were in that 4% you would be yelling hosannas and turning cartwheels. If the sum of my life was to ‘cure’ 4% of 30,000 people I would die with a big wide smile.
I see a lot of promising work on a genetic resolution to all CF sufferers. We may have to settle for some time with mutation specific genetically based drugs, but as many people who are taking the steps it took to affect 551 mutation, imagine the impact when DeltaDF508 has a specific drug. I fully expect a panacea genetically based drug in my lifetime. I am 62 and if all goes well, I will see 75, but no longer. So this is my official prognostication of 13-15 years to a panacea CF genetically based drug. If I am wrong, it will be from earlier rather than later discovery.
sweetninis
New member
Printer:
Maybe u got me wrong I didn't wrote or mean anything about eliminating carriers of cf I just mean that cfers r full sick and cf carriers are half sick so why not start off with the half sick it makes sense doesn't it!
Maybe u got me wrong I didn't wrote or mean anything about eliminating carriers of cf I just mean that cfers r full sick and cf carriers are half sick so why not start off with the half sick it makes sense doesn't it!
sweetninis
New member
Swallowtail
You r right Prevention is better than Cure.
After reading all people's comments I feel so sad to know that it is going to take way too long to find a cure for cf but I m hopefull that someday a cure will be discoverd! Right now I think that all people should get their genetic testing done to know if they r carriers of CF or thallaseemia and all carriers should never ever marry another carrier and if u get to know it after having ur child diagnosis of cf then do not risk bringing another child into this world with being so positive mind that"may god this child does not have cf"its selfishness!!cf is painful and those who have it can tell.
You r right Prevention is better than Cure.
After reading all people's comments I feel so sad to know that it is going to take way too long to find a cure for cf but I m hopefull that someday a cure will be discoverd! Right now I think that all people should get their genetic testing done to know if they r carriers of CF or thallaseemia and all carriers should never ever marry another carrier and if u get to know it after having ur child diagnosis of cf then do not risk bringing another child into this world with being so positive mind that"may god this child does not have cf"its selfishness!!cf is painful and those who have it can tell.
sweetninis
New member
I said half sick bc when they marry another carrier they pass their sickness to their child in the form of cf. I was wondering if dr could find something when 2 carriers marry and their children do not suffer from cf just a thinking I m not a dr<img src="i/expressions/face-icon-small-sad.gif" border="0">
Its not about the cure, its about learning to prevent excerbations. Its about knowing your body well enough to prevent further break down. Its about doing what you need to do and making yourself a priority. Yes CF sucks. Their are so many varying degrees of this illness, so many genotypes it is hard to quantify and treat patients accordingly. Just because you may have the same genotype as someone else DOES not mean you will respond the same to their treatment plan. Can we take what we read and learn from each other? Absolutely but remember we are all differnt individuals.
I am 54 and was officially diag. at 24 . I always knew I had something so wasn't surprised. My FEV1 hovers 50-56%. Breathins sucks I have a bad asthmatic component to my CF. I'm on everything, allergic to most pseudo meds, have MRSA all the time and deal with horrifying pancreatic attacks. Can I say it sucks again.
I was a medical researcher for years then went to med school. So I get all of this. I practice 4 days a week and then come home and take care of my wonderful daughter who was born a week after my 50 birthday. Am tired but I never give up. I don't beleive in cures I believe in the power we have within. Never stop believing and don't feel sorry for yourself,I've seen others who have things much worse. Who cares about a cure, who cares who is more mild or severe. Just live each day. Grab life in your hands and hold it tight till our last breath. Never let CF defeat you mentally it might physically, but remember our mental outlook can transform many things.
By the way vit.C cured scurvy not lyme.
Tina 54 I have 3mutations they know nothing about! Lucky me.
I am 54 and was officially diag. at 24 . I always knew I had something so wasn't surprised. My FEV1 hovers 50-56%. Breathins sucks I have a bad asthmatic component to my CF. I'm on everything, allergic to most pseudo meds, have MRSA all the time and deal with horrifying pancreatic attacks. Can I say it sucks again.
I was a medical researcher for years then went to med school. So I get all of this. I practice 4 days a week and then come home and take care of my wonderful daughter who was born a week after my 50 birthday. Am tired but I never give up. I don't beleive in cures I believe in the power we have within. Never stop believing and don't feel sorry for yourself,I've seen others who have things much worse. Who cares about a cure, who cares who is more mild or severe. Just live each day. Grab life in your hands and hold it tight till our last breath. Never let CF defeat you mentally it might physically, but remember our mental outlook can transform many things.
By the way vit.C cured scurvy not lyme.
Tina 54 I have 3mutations they know nothing about! Lucky me.
LittleLab4CF
Super Moderator
CF genetics is a real hair puller. CrisDopher lamented over the discovering a correlation between CF and the CFTR gene mutation. Until knowing translates into action, for all practical purposes it is useless. Here comes G551D with a genetic magic bullet. Big deal, so we eliminate CF symptoms for 4% of CFers. If Delta F508 is the next mutation specific drug then there is potential benefit for 66% more of the CF population. Even if we make similar drug therapy for the top 10 or 30 mutations, those with multiple homozygous mutations will be covered will be largely under control.
I am reading between the lines here but I hear a bit of my CF is far worse than yours because you have lived past 60. First, I have nor argument because I can't relate to somebody who at 15 will die without a double lung transplant, like now. I don't want to try to understand this pain.
I have learned that the worst pain there is, is the worst pain you have felt. Not pain somebody else felt even though I can imagine, and I am good at imagining, but for me, the worst I have felt is the worst I know. Same for everybody else. My parents died young. To me this is a terrible loss of potential, and a personal loss of what I will never be able to share had they lived long. My wife's parents were in their late 90's and she sees her pain as having been able to share so much of their lives, and now they are gone and it must hurt more to lose so much time they had together. I think this is fairly universal and it is absolutely subjective. I read of one person who posts often how her medical file cabinet filled up with over half a dozen surgeries on her young pancreas. She has a bundle of other conditions that parallel the pancreas. Suffering hard and dying young sounds like the absolute worst, until you hear how wonderful and full their lives are. Living long, suffering less for many more undiagnosed years has its own unspoken baggage. I have had every invasive test and all the usual ones over and over and over again. Each time I got a big zero. Idiopathic GI problems just HAVE to be psychosomatic so after each circle on the medical merry go round ends up with me on a couch, whith a Psychiatrist. This has its own pain and the constant unknown cause(s) can be so frustrating and keep in mind late diagnosed CFers have been going through a similar idiotic pattern for how long? In my case, diagnosed at 51, I had been treated for what they knew, which in many cases would amount to malpractice if I weren't such a willing pariticpant. Cfers have their own brand of over and over and over, but they know why they are going through it.
I am reading between the lines here but I hear a bit of my CF is far worse than yours because you have lived past 60. First, I have nor argument because I can't relate to somebody who at 15 will die without a double lung transplant, like now. I don't want to try to understand this pain.
I have learned that the worst pain there is, is the worst pain you have felt. Not pain somebody else felt even though I can imagine, and I am good at imagining, but for me, the worst I have felt is the worst I know. Same for everybody else. My parents died young. To me this is a terrible loss of potential, and a personal loss of what I will never be able to share had they lived long. My wife's parents were in their late 90's and she sees her pain as having been able to share so much of their lives, and now they are gone and it must hurt more to lose so much time they had together. I think this is fairly universal and it is absolutely subjective. I read of one person who posts often how her medical file cabinet filled up with over half a dozen surgeries on her young pancreas. She has a bundle of other conditions that parallel the pancreas. Suffering hard and dying young sounds like the absolute worst, until you hear how wonderful and full their lives are. Living long, suffering less for many more undiagnosed years has its own unspoken baggage. I have had every invasive test and all the usual ones over and over and over again. Each time I got a big zero. Idiopathic GI problems just HAVE to be psychosomatic so after each circle on the medical merry go round ends up with me on a couch, whith a Psychiatrist. This has its own pain and the constant unknown cause(s) can be so frustrating and keep in mind late diagnosed CFers have been going through a similar idiotic pattern for how long? In my case, diagnosed at 51, I had been treated for what they knew, which in many cases would amount to malpractice if I weren't such a willing pariticpant. Cfers have their own brand of over and over and over, but they know why they are going through it.
with the up coming medicines such as kalydeco coming out, i have high hopes and prayers that it will be soon. My lifes ambition is to watch my son grow up and have me some grand babies i can spoil with cookies and send home. lord only knows how i drive to be the typical grandmother, cakes and cookies, spoil them and send them home.
E
ElenaFalcon
Guest
<div class="FTQUOTE"><begin quote><em>Originally posted by: <strong>LittleLab4CF</strong></em>We need a way of harvesting and “growing” large amounts of the healthy gene. We need a vector to deliver it to every cell’s nucleus and further, to deliver it to the correct spot on the correct chromosome. Finally we need something to activate this gene </end quote>
LittleLab4CF: is there any future of cf gene therapy currently underway in the UK? See link to these trials here: <a href="http://cfgenetherapy.org.uk/">http://cfgenetherapy.org.uk/</a>
Is it workable for CF lungs? If it is successfull and IF it can save cf lungs we will see a-kind-of-cure-for-cf-lungs in 5 or 6 years.
LittleLab4CF: is there any future of cf gene therapy currently underway in the UK? See link to these trials here: <a href="http://cfgenetherapy.org.uk/">http://cfgenetherapy.org.uk/</a>
Is it workable for CF lungs? If it is successfull and IF it can save cf lungs we will see a-kind-of-cure-for-cf-lungs in 5 or 6 years.
Elena,
Thanks for that link! To see this going on in the UK I have a bit more hope for it actually getting somewhere. I remember hearing when I was around 10 - 11 yrs old or so about gene therapy and being told "We expect a gene therapy cure in maybe 10 yrs or so just make sure you do all you can to keep yourself healthy for that time".....needless to say I'm now 30 and nothing yet!
Thanks for that link! To see this going on in the UK I have a bit more hope for it actually getting somewhere. I remember hearing when I was around 10 - 11 yrs old or so about gene therapy and being told "We expect a gene therapy cure in maybe 10 yrs or so just make sure you do all you can to keep yourself healthy for that time".....needless to say I'm now 30 and nothing yet!
CrisDopher
New member
I've got to go with Sara on this one.
*In general*, I think Sara is right. People w/ severe lung disease don't generally survive into their fifties and beyond. The existence of a handful of you who do have severe lung disease and have for a long time and weren't diagnosed until late is simply a statistical variant - an outlier on the graph that in no way affects the general correlation of disease severity to shorter life spans. Sara may have assumed that all late-diagnosed cystics have enjoyed a mild lung disease until recently. Not true, of course. But if you replace "all" with "most", then the statement is true.
But she was trying to make a larger point that I find interesting and also very true: that better detection of CF and the recent uptick in late diagnoses has inflated the numbers of people w/ CF who are older, thus raising the statistical median age of survival in a way not accounted for by the new medications and therapies. I had not thought of this before, myself. To me, it is a cause for celebration. I am not disappointed that the increase in median age isn't all because of meds; because the very fact of cystics in their 60's and beyond brings so much hope to all of us!
As for a cure... well... there's only one way to truly and actually wipe out this disease and it is so drastic a violation of human rights that no-one will ever do it. You would have to test every person on earth of child-bearing age and younger for CF genes. (Assuming you knew what all the variants are - and we don't yet!). All carriers (and cystics) forcibly sterilized the world over. You'd have to continue this program for fifty to sixty years. THEN you could say that CF has been wiped out.
But that isn't going to happen. And genetic therapy of an individual that can replace defective genes in every cell in the body is still science fiction. The first inroad to this kind of cure will be at the gamete level. Far too late for any of us! LOL
*In general*, I think Sara is right. People w/ severe lung disease don't generally survive into their fifties and beyond. The existence of a handful of you who do have severe lung disease and have for a long time and weren't diagnosed until late is simply a statistical variant - an outlier on the graph that in no way affects the general correlation of disease severity to shorter life spans. Sara may have assumed that all late-diagnosed cystics have enjoyed a mild lung disease until recently. Not true, of course. But if you replace "all" with "most", then the statement is true.
But she was trying to make a larger point that I find interesting and also very true: that better detection of CF and the recent uptick in late diagnoses has inflated the numbers of people w/ CF who are older, thus raising the statistical median age of survival in a way not accounted for by the new medications and therapies. I had not thought of this before, myself. To me, it is a cause for celebration. I am not disappointed that the increase in median age isn't all because of meds; because the very fact of cystics in their 60's and beyond brings so much hope to all of us!
As for a cure... well... there's only one way to truly and actually wipe out this disease and it is so drastic a violation of human rights that no-one will ever do it. You would have to test every person on earth of child-bearing age and younger for CF genes. (Assuming you knew what all the variants are - and we don't yet!). All carriers (and cystics) forcibly sterilized the world over. You'd have to continue this program for fifty to sixty years. THEN you could say that CF has been wiped out.
But that isn't going to happen. And genetic therapy of an individual that can replace defective genes in every cell in the body is still science fiction. The first inroad to this kind of cure will be at the gamete level. Far too late for any of us! LOL
You must remember that many reasons that genetic mutations survive is because they also have a good component. The majority of cystics do not have heart disease. If you have your cholesterol checked cystics usually average around 100 not the norm of 200. Considering the amount of salt intake that most of us need its interesting that we don't have many issues of hypertension either. In the early days of cf transplants with heart/lung, some cf hearts were transplanted in patients who needed a heart because our hearts were good. I'm not saying that some cystics will never develop heart disease but at 54 years of age my cholesterol and blood pressure are amazing. I also have the gene for a blood disorder called thalessemia. I'm a minor while my niece is a major. The good component is that people with this trait cannot develop malaria. So, you see in many gene mutations there is a positive that helps that gene survive. By trying to wipe out a mutation completely you open the door for something else to become problematic. It's a catch-22. All I ask for is new meds to keep us going. Without antibiotics it is hard to win this race. Even with an aresenal of preventive methods almost all of us have had to tune-up once in awhile. Embrace the fight, stay strong and may we all live very long lives. Tina