Nasal Potential Difference as Part of CF Dx process

hmw

New member
It would definitely be worth finding out if both mutations were on the same chromosome, since people can carry more than one and pass that single mutated gene on to their child (that is how the occasional baby with cf has 3 mutations.) That would not mean that one is benign but rather that you are a carrier and not a person with cf. As we all know, some carriers are symptomatic and that could be complicating your health (just like Shawn being a df508 carrier complicates his health with his asthma- his pft's are low enough to qualify for a lung disease dx.) Testing your parents just for your mutations could clear that up. For that matter, testing just ONE of your parents would clear that up- one parent would have either one, both, or none- simple elimination would tell you what the other parent carries. Genetic sequencing only looks at the cftr gene, I believe, so only yields results that are relevant to the disease. It really stinks that not even NPD is 100% accurate in ruling in/out the disease unless done jussssst right. I would think that it would have to be done under ideal circumstances to attain that high level of accuracy (We can agree to disagree on that, I don't mind... our accredited dr told us what he did and I have no reason not to believe him, just as you believe yours- so there is room to simply believe accuracy is important regardless of results. <img src="i/expressions/face-icon-small-wink.gif" border="0"> ) I don't think it's fair at ALL for any of your care providers to think you 'wanted' this dx, as you have fought hard to disprove it and never would have ordered the tests if you 'wanted' the dx- you HAD the dx without all those tests. Why would you have wanted the test if you were content with the dx?!? I hope things can be worked out. I am glad you've been taken off the registry. I think the registry is a valuable tool and that as many people as possible should be on it, but until your other health conditions can be completely clarified, it would serve to cause more confusion than not. I just wish YOU had the answers you needed so your care could be better managed (needing frequent intubation is very scary and if there was a way to prevent it that's not yet been found- if only you could!) <img src="i/expressions/heart.gif" border="0"> So NONE Of this CF stuff is simple. There are SO MANY shades of gray. In our case NPD ruled out CF. If things ever looked suspicious again in future we have another test we could try- testing Shawn for CBAVD, our last resort. It would be a powerful indicator that just maybe the NPD wasn't as accurate as we hoped. But for now, NPD is VERY STRONG evidence that 'symptomatic carrier' fits (make that a salty carrier- but even at 54, that's only half the score his sister had- she scored 108 and 2nd test confirmed) and we will continue to manage asthma and I have no worries unless his health took a big turn for the worse. I hope the rest of you here needing more answers get them! This test is a valuable tool. <img src="i/expressions/face-icon-small-smile.gif" border="0">
 
eta> I changed my response a bit since I didn't see your email response till after I posted.
 

hmw

New member
It would definitely be worth finding out if both mutations were on the same chromosome, since people can carry more than one and pass that single mutated gene on to their child (that is how the occasional baby with cf has 3 mutations.) That would not mean that one is benign but rather that you are a carrier and not a person with cf. As we all know, some carriers are symptomatic and that could be complicating your health (just like Shawn being a df508 carrier complicates his health with his asthma- his pft's are low enough to qualify for a lung disease dx.) Testing your parents just for your mutations could clear that up. For that matter, testing just ONE of your parents would clear that up- one parent would have either one, both, or none- simple elimination would tell you what the other parent carries. Genetic sequencing only looks at the cftr gene, I believe, so only yields results that are relevant to the disease. It really stinks that not even NPD is 100% accurate in ruling in/out the disease unless done jussssst right. I would think that it would have to be done under ideal circumstances to attain that high level of accuracy (We can agree to disagree on that, I don't mind... our accredited dr told us what he did and I have no reason not to believe him, just as you believe yours- so there is room to simply believe accuracy is important regardless of results. <img src="i/expressions/face-icon-small-wink.gif" border="0"> ) I don't think it's fair at ALL for any of your care providers to think you 'wanted' this dx, as you have fought hard to disprove it and never would have ordered the tests if you 'wanted' the dx- you HAD the dx without all those tests. Why would you have wanted the test if you were content with the dx?!? I hope things can be worked out. I am glad you've been taken off the registry. I think the registry is a valuable tool and that as many people as possible should be on it, but until your other health conditions can be completely clarified, it would serve to cause more confusion than not. I just wish YOU had the answers you needed so your care could be better managed (needing frequent intubation is very scary and if there was a way to prevent it that's not yet been found- if only you could!) <img src="i/expressions/heart.gif" border="0"> So NONE Of this CF stuff is simple. There are SO MANY shades of gray. In our case NPD ruled out CF. If things ever looked suspicious again in future we have another test we could try- testing Shawn for CBAVD, our last resort. It would be a powerful indicator that just maybe the NPD wasn't as accurate as we hoped. But for now, NPD is VERY STRONG evidence that 'symptomatic carrier' fits (make that a salty carrier- but even at 54, that's only half the score his sister had- she scored 108 and 2nd test confirmed) and we will continue to manage asthma and I have no worries unless his health took a big turn for the worse. I hope the rest of you here needing more answers get them! This test is a valuable tool. <img src="i/expressions/face-icon-small-smile.gif" border="0">

eta> I changed my response a bit since I didn't see your email response till after I posted.
 

hmw

New member
<p>It would definitely be worth finding out if both mutations were on the same chromosome, since people can carry more than one and pass that single mutated gene on to their child (that is how the occasional baby with cf has 3 mutations.) That would not mean that one is benign but rather that you are a carrier and not a person with cf. As we all know, some carriers are symptomatic and that could be complicating your health (just like Shawn being a df508 carrier complicates his health with his asthma- his pft's are low enough to qualify for a lung disease dx.) Testing your parents just for your mutations could clear that up. For that matter, testing just ONE of your parents would clear that up- one parent would have either one, both, or none- simple elimination would tell you what the other parent carries. Genetic sequencing only looks at the cftr gene, I believe, so only yields results that are relevant to the disease. <br /> <br />It really stinks that not even NPD is 100% accurate in ruling in/out the disease unless done jussssst right. I would think that it would have to be done under ideal circumstances to attain that high level of accuracy (We can agree to disagree on that, I don't mind... our accredited dr told us what he did and I have no reason not to believe him, just as you believe yours- so there is room to simply believe accuracy is important regardless of results. <img src="i/expressions/face-icon-small-wink.gif" border="0"> ) I don't think it's fair at ALL for any of your care providers to think you 'wanted' this dx, as you have fought hard to disprove it and never would have ordered the tests if you 'wanted' the dx- you HAD the dx without all those tests. Why would you have wanted the test if you were content with the dx?!? <br /> <br />I hope things can be worked out. I am glad you've been taken off the registry. I think the registry is a valuable tool and that as many people as possible should be on it, but until your other health conditions can be completely clarified, it would serve to cause more confusion than not. I just wish YOU had the answers you needed so your care could be better managed (needing frequent intubation is very scary and if there was a way to prevent it that's not yet been found- if only you could!) <img src="i/expressions/heart.gif" border="0"> <br /> <br />So NONE Of this CF stuff is simple. There are SO MANY shades of gray. <br />In our case NPD ruled out CF. If things ever looked suspicious again in future we have another test we could try- testing Shawn for CBAVD, our last resort. It would be a powerful indicator that just maybe the NPD wasn't as accurate as we hoped. But for now, NPD is VERY STRONG evidence that 'symptomatic carrier' fits (make that a salty carrier- but even at 54, that's only half the score his sister had- she scored 108 and 2nd test confirmed) and we will continue to manage asthma and I have no worries unless his health took a big turn for the worse. <br /> <br />I hope the rest of you here needing more answers get them! This test is a valuable tool. <img src="i/expressions/face-icon-small-smile.gif" border="0">
<p>
<p>eta> I changed my response a bit since I didn't see your email response till after I posted.
 

Beccamom

New member
My daughter's genetics came back with G542X mutation that is known to be cf disease causing and 2 polymorphisms that are not known to be disease causing. so that is why her diagnosis has been uncertain.  Alaso she had a sweat test at the age of 4 that was not diagnostic, but i dont know if it was normal or intermediate score.  she haaAs many symptoms including recurrent pneumonia and failure to thrive.  
 

Beccamom

New member
My daughter's genetics came back with G542X mutation that is known to be cf disease causing and 2 polymorphisms that are not known to be disease causing. so that is why her diagnosis has been uncertain. Alaso she had a sweat test at the age of 4 that was not diagnostic, but i dont know if it was normal or intermediate score. she haaAs many symptoms including recurrent pneumonia and failure to thrive.
 

Beccamom

New member
<p>My daughter's genetics came back with G542X mutation that is known to be cf disease causing and 2 polymorphisms that are not known to be disease causing. so that is why her diagnosis has been uncertain. Alaso she had a sweat test at the age of 4 that was not diagnostic, but i dont know if it was normal or intermediate score. she haaAs many symptoms including recurrent pneumonia and failure to thrive.
 

hmw

New member
It would be worth finding out the score of her sweat test.

Based on what you've told us so far about symptoms + her NPD results thus far, it sounds like she is headed for a dx.
 

hmw

New member
It would be worth finding out the score of her sweat test.

Based on what you've told us so far about symptoms + her NPD results thus far, it sounds like she is headed for a dx.
 

hmw

New member
It would be worth finding out the score of her sweat test.
<br />
<br />Based on what you've told us so far about symptoms + her NPD results thus far, it sounds like she is headed for a dx.
 

JennifersHope

New member
I agree Harriett. I am going to email UNC and ask them if they will blood test my mom. I think we need to stay in touch with each other over the years to see how things pan out for Shawn and I both.

I wish there was clear cut answers because the not knowing, having to live like this wondering what is going to make me sick next, not being able to go anywhere because we don't know what is going to trigger me, right now even my doctors office makes me wheeze... it is annoying to say the least..

I am not sure my story is over, I just don't know what else to do. I have a great respect for you.
 

JennifersHope

New member
I agree Harriett. I am going to email UNC and ask them if they will blood test my mom. I think we need to stay in touch with each other over the years to see how things pan out for Shawn and I both.

I wish there was clear cut answers because the not knowing, having to live like this wondering what is going to make me sick next, not being able to go anywhere because we don't know what is going to trigger me, right now even my doctors office makes me wheeze... it is annoying to say the least..

I am not sure my story is over, I just don't know what else to do. I have a great respect for you.
 

JennifersHope

New member
I agree Harriett. I am going to email UNC and ask them if they will blood test my mom. I think we need to stay in touch with each other over the years to see how things pan out for Shawn and I both.
<br />
<br />I wish there was clear cut answers because the not knowing, having to live like this wondering what is going to make me sick next, not being able to go anywhere because we don't know what is going to trigger me, right now even my doctors office makes me wheeze... it is annoying to say the least..
<br />
<br />I am not sure my story is over, I just don't know what else to do. I have a great respect for you.
 

JennifersHope

New member
Amy, I am sorry I didn't see this before now. I think to be diagnosed with a typical CF you have to have CF of some form and they don't believe I do.
I am not prescibed Tobi or Pulmozyme any more so I am able to get what I need.
 
Thanks, I hope we get answers some time soon, I am going to have to try to think harder about a way for solutions.
 
I can go to National Jewish Hospital in Denver Colorado, my insurance approved of it but I am so worn out an exhausted I was going to take a few months off, I already talked extensively with the CF doctor there as did my regular CF doctor. So I would just be going for a regular pulmonary work up.... I think I am going to go in September.
 

JennifersHope

New member
Amy, I am sorry I didn't see this before now. I think to be diagnosed with a typical CF you have to have CF of some form and they don't believe I do.
I am not prescibed Tobi or Pulmozyme any more so I am able to get what I need.

Thanks, I hope we get answers some time soon, I am going to have to try to think harder about a way for solutions.

I can go to National Jewish Hospital in Denver Colorado, my insurance approved of it but I am so worn out an exhausted I was going to take a few months off, I already talked extensively with the CF doctor there as did my regular CF doctor. So I would just be going for a regular pulmonary work up.... I think I am going to go in September.
 

JennifersHope

New member
<p>Amy, I am sorry I didn't see this before now. I think to be diagnosed with a typical CF you have to have CF of some form and they don't believe I do.
<p>I am not prescibed Tobi or Pulmozyme any more so I am able to get what I need.
<p>
<p>Thanks, I hope we get answers some time soon, I am going to have to try to think harder about a way for solutions.
<p>
<p>I can go to National Jewish Hospital in Denver Colorado, my insurance approved of it but I am so worn out an exhausted I was going to take a few months off, I already talked extensively with the CF doctor there as did my regular CF doctor. So I would just be going for a regular pulmonary work up.... I think I am going to go in September.
 

JennifersHope

New member
Harriett, I just emailed Dr Donaldson at UNC and asked him if he would be interested in drawing blood on my parents like he originally said they might be.

I don't know what is wrong with me or why I am so afraid to be bold when it comes to me but I am.....Both my parents are so upset with me that I am not going to National Jewish now and they think I am not being responsible by putting it off. I feel like I want a break and since it isn't going to change anything I just don't know what to do
 

JennifersHope

New member
Harriett, I just emailed Dr Donaldson at UNC and asked him if he would be interested in drawing blood on my parents like he originally said they might be.

I don't know what is wrong with me or why I am so afraid to be bold when it comes to me but I am.....Both my parents are so upset with me that I am not going to National Jewish now and they think I am not being responsible by putting it off. I feel like I want a break and since it isn't going to change anything I just don't know what to do
 

JennifersHope

New member
Harriett, I just emailed Dr Donaldson at UNC and asked him if he would be interested in drawing blood on my parents like he originally said they might be.
<br />
<br />I don't know what is wrong with me or why I am so afraid to be bold when it comes to me but I am.....Both my parents are so upset with me that I am not going to National Jewish now and they think I am not being responsible by putting it off. I feel like I want a break and since it isn't going to change anything I just don't know what to do
 

mom2owen

New member
I wish you so much luck JennifersHope. I really feel for you and it also scares me like it is a view into the future of Owen since he doesn't have a diagnosis either. I did want to add that as parents, we always want the best for our kids so while it feels like you parents are upset with you, I might be overstepping my boundaries here, but I would equate it to being upset that you have to live like this. There is nothing harder than seeing your child, always your baby, suffering and I am sure they just want answers for you so you can get the right kind of help and live the best life possible.
Oh, and I also wanted to add that I know how you feel about being bold with doctors. It is so tough because we need them to get through this but they are so often the big hinderance in getting help. And what is with the tight-knot group with CF docs especially? They will NOT contradict what any other doctor says about a diagnosis. Even in the same clinic, we had one doctor tell us Owen's sinuses were fine in the CT so he can't have CF but then his partner said his sinuses are a mess and totally swollen and "fragile" which is why he gets nose bleeds and mouth breathes. But, he added that it is not "bad enough" to be CF. When I asked about the first doctor he seemed very uncomfortable contradicting him but the evidence has always been there of sinus junk so he knew he couldn't take it back. Rollll those eyes!
I am so glad this post was started and I will definitely be giving it a lot of thought, with my dh. I want so badly to have it done but I fear more trouble with the results not actually clarifying things. I also have to laugh a little bit about the healthy nose thing for the test to work. Are there any CFers with healthy noses?? It seems like a tough thing to achieve, at least in Owen's case. He always has congestion and swelling. Will proceed with caution. In any case, I am eternally grateful for all of the information and support I get here. So big huge hugs to you all!
 

mom2owen

New member
I wish you so much luck JennifersHope. I really feel for you and it also scares me like it is a view into the future of Owen since he doesn't have a diagnosis either. I did want to add that as parents, we always want the best for our kids so while it feels like you parents are upset with you, I might be overstepping my boundaries here, but I would equate it to being upset that you have to live like this. There is nothing harder than seeing your child, always your baby, suffering and I am sure they just want answers for you so you can get the right kind of help and live the best life possible.
Oh, and I also wanted to add that I know how you feel about being bold with doctors. It is so tough because we need them to get through this but they are so often the big hinderance in getting help. And what is with the tight-knot group with CF docs especially? They will NOT contradict what any other doctor says about a diagnosis. Even in the same clinic, we had one doctor tell us Owen's sinuses were fine in the CT so he can't have CF but then his partner said his sinuses are a mess and totally swollen and "fragile" which is why he gets nose bleeds and mouth breathes. But, he added that it is not "bad enough" to be CF. When I asked about the first doctor he seemed very uncomfortable contradicting him but the evidence has always been there of sinus junk so he knew he couldn't take it back. Rollll those eyes!
I am so glad this post was started and I will definitely be giving it a lot of thought, with my dh. I want so badly to have it done but I fear more trouble with the results not actually clarifying things. I also have to laugh a little bit about the healthy nose thing for the test to work. Are there any CFers with healthy noses?? It seems like a tough thing to achieve, at least in Owen's case. He always has congestion and swelling. Will proceed with caution. In any case, I am eternally grateful for all of the information and support I get here. So big huge hugs to you all!
 
Top