Not Diagnosed dF508 & R117H 7T/9T

dmatherne

New member
I need a little input from the members of this site. My son, which is 5 1/2 months, was found to have CF through the Louisiana newborn screening with the gene combo dF508 & R117H 7T/9T. We have since did all of the ancillary test and he has passed as normal.

Sweat Test: 23 & 25
Stool Elastase : 496

We have been to two CF Clinics: Tulane University in New Orleans, LA and Baylor University in Houston, TX. Both clinics are apprehensive about diagnosing him with CF. They have stated that his gene combination (with the poly-T variant) is thought to cause some milder form of CF related disease. They each have young patients that have presented with no symptoms of CF and are on a 6 month check-up and treatment plan. Both CF clinics basically told us that we would do 6 month check-ups with the sputum cultures and when he is old enough do lung function tests. But for the meantime, we have to just wait and see if he presents with any symptoms of CF.

Our problem stems from this "information age". My wife has become friends with a number of CF moms through Facebook. One mother has a son with same gene combo around the same age and her CF clinic has diagnosed her son with CF and they seem to be on a much more rigorous treatment regiment. Her child is doing chest therapy twice a day and is going to be getting "The Vest" when he is old enough to use it. He also has a cold where the congestion is building up and he is on a nebulizer to break-up the congestion.

Finally, that brings me to my question. What are some advantages or disadvantages of a child with a mild form of CF being not diagnosed? To me he has it and I can see for insurance reasons not wanting to diagnose someone with something that isn't actually presenting itself for treatment. Obviously insurance won't pay for any prescriptions or treatments for a patient that hasn't been diagnosed with CF. I don't have any problem with this line of thinking, but should we be doing chest therapy and as he gets older think about getting the vest? Or be doing something else preventative now? We really haven't been told to do anything except: good hand hygiene, keep him away from smokers, flu shoots when 6 months and diet.

There seems to be a lot of controversy over whether the R117G 7T/9T gene should even be part of the genetic testing for CF. I'm having a hard time trying to figure out if it would be advantageous for him to be diagnosed and push for a diagnosis from his CF Dr. or just trust the doctors and be cautiously optimistic. Sorry for the long post, but if I have left anything out please feel free to ask.
 

dmatherne

New member
I need a little input from the members of this site. My son, which is 5 1/2 months, was found to have CF through the Louisiana newborn screening with the gene combo dF508 & R117H 7T/9T. We have since did all of the ancillary test and he has passed as normal.

Sweat Test: 23 & 25
Stool Elastase : 496

We have been to two CF Clinics: Tulane University in New Orleans, LA and Baylor University in Houston, TX. Both clinics are apprehensive about diagnosing him with CF. They have stated that his gene combination (with the poly-T variant) is thought to cause some milder form of CF related disease. They each have young patients that have presented with no symptoms of CF and are on a 6 month check-up and treatment plan. Both CF clinics basically told us that we would do 6 month check-ups with the sputum cultures and when he is old enough do lung function tests. But for the meantime, we have to just wait and see if he presents with any symptoms of CF.

Our problem stems from this "information age". My wife has become friends with a number of CF moms through Facebook. One mother has a son with same gene combo around the same age and her CF clinic has diagnosed her son with CF and they seem to be on a much more rigorous treatment regiment. Her child is doing chest therapy twice a day and is going to be getting "The Vest" when he is old enough to use it. He also has a cold where the congestion is building up and he is on a nebulizer to break-up the congestion.

Finally, that brings me to my question. What are some advantages or disadvantages of a child with a mild form of CF being not diagnosed? To me he has it and I can see for insurance reasons not wanting to diagnose someone with something that isn't actually presenting itself for treatment. Obviously insurance won't pay for any prescriptions or treatments for a patient that hasn't been diagnosed with CF. I don't have any problem with this line of thinking, but should we be doing chest therapy and as he gets older think about getting the vest? Or be doing something else preventative now? We really haven't been told to do anything except: good hand hygiene, keep him away from smokers, flu shoots when 6 months and diet.

There seems to be a lot of controversy over whether the R117G 7T/9T gene should even be part of the genetic testing for CF. I'm having a hard time trying to figure out if it would be advantageous for him to be diagnosed and push for a diagnosis from his CF Dr. or just trust the doctors and be cautiously optimistic. Sorry for the long post, but if I have left anything out please feel free to ask.
 

dmatherne

New member
I need a little input from the members of this site. My son, which is 5 1/2 months, was found to have CF through the Louisiana newborn screening with the gene combo dF508 & R117H 7T/9T. We have since did all of the ancillary test and he has passed as normal.

Sweat Test: 23 & 25
Stool Elastase : 496

We have been to two CF Clinics: Tulane University in New Orleans, LA and Baylor University in Houston, TX. Both clinics are apprehensive about diagnosing him with CF. They have stated that his gene combination (with the poly-T variant) is thought to cause some milder form of CF related disease. They each have young patients that have presented with no symptoms of CF and are on a 6 month check-up and treatment plan. Both CF clinics basically told us that we would do 6 month check-ups with the sputum cultures and when he is old enough do lung function tests. But for the meantime, we have to just wait and see if he presents with any symptoms of CF.

Our problem stems from this "information age". My wife has become friends with a number of CF moms through Facebook. One mother has a son with same gene combo around the same age and her CF clinic has diagnosed her son with CF and they seem to be on a much more rigorous treatment regiment. Her child is doing chest therapy twice a day and is going to be getting "The Vest" when he is old enough to use it. He also has a cold where the congestion is building up and he is on a nebulizer to break-up the congestion.

Finally, that brings me to my question. What are some advantages or disadvantages of a child with a mild form of CF being not diagnosed? To me he has it and I can see for insurance reasons not wanting to diagnose someone with something that isn't actually presenting itself for treatment. Obviously insurance won't pay for any prescriptions or treatments for a patient that hasn't been diagnosed with CF. I don't have any problem with this line of thinking, but should we be doing chest therapy and as he gets older think about getting the vest? Or be doing something else preventative now? We really haven't been told to do anything except: good hand hygiene, keep him away from smokers, flu shoots when 6 months and diet.

There seems to be a lot of controversy over whether the R117G 7T/9T gene should even be part of the genetic testing for CF. I'm having a hard time trying to figure out if it would be advantageous for him to be diagnosed and push for a diagnosis from his CF Dr. or just trust the doctors and be cautiously optimistic. Sorry for the long post, but if I have left anything out please feel free to ask.
 

dmatherne

New member
I need a little input from the members of this site. My son, which is 5 1/2 months, was found to have CF through the Louisiana newborn screening with the gene combo dF508 & R117H 7T/9T. We have since did all of the ancillary test and he has passed as normal.

Sweat Test: 23 & 25
Stool Elastase : 496

We have been to two CF Clinics: Tulane University in New Orleans, LA and Baylor University in Houston, TX. Both clinics are apprehensive about diagnosing him with CF. They have stated that his gene combination (with the poly-T variant) is thought to cause some milder form of CF related disease. They each have young patients that have presented with no symptoms of CF and are on a 6 month check-up and treatment plan. Both CF clinics basically told us that we would do 6 month check-ups with the sputum cultures and when he is old enough do lung function tests. But for the meantime, we have to just wait and see if he presents with any symptoms of CF.

Our problem stems from this "information age". My wife has become friends with a number of CF moms through Facebook. One mother has a son with same gene combo around the same age and her CF clinic has diagnosed her son with CF and they seem to be on a much more rigorous treatment regiment. Her child is doing chest therapy twice a day and is going to be getting "The Vest" when he is old enough to use it. He also has a cold where the congestion is building up and he is on a nebulizer to break-up the congestion.

Finally, that brings me to my question. What are some advantages or disadvantages of a child with a mild form of CF being not diagnosed? To me he has it and I can see for insurance reasons not wanting to diagnose someone with something that isn't actually presenting itself for treatment. Obviously insurance won't pay for any prescriptions or treatments for a patient that hasn't been diagnosed with CF. I don't have any problem with this line of thinking, but should we be doing chest therapy and as he gets older think about getting the vest? Or be doing something else preventative now? We really haven't been told to do anything except: good hand hygiene, keep him away from smokers, flu shoots when 6 months and diet.

There seems to be a lot of controversy over whether the R117G 7T/9T gene should even be part of the genetic testing for CF. I'm having a hard time trying to figure out if it would be advantageous for him to be diagnosed and push for a diagnosis from his CF Dr. or just trust the doctors and be cautiously optimistic. Sorry for the long post, but if I have left anything out please feel free to ask.
 

dmatherne

New member
I need a little input from the members of this site. My son, which is 5 1/2 months, was found to have CF through the Louisiana newborn screening with the gene combo dF508 & R117H 7T/9T. We have since did all of the ancillary test and he has passed as normal.
<br />
<br />Sweat Test: 23 & 25
<br />Stool Elastase : 496
<br />
<br />We have been to two CF Clinics: Tulane University in New Orleans, LA and Baylor University in Houston, TX. Both clinics are apprehensive about diagnosing him with CF. They have stated that his gene combination (with the poly-T variant) is thought to cause some milder form of CF related disease. They each have young patients that have presented with no symptoms of CF and are on a 6 month check-up and treatment plan. Both CF clinics basically told us that we would do 6 month check-ups with the sputum cultures and when he is old enough do lung function tests. But for the meantime, we have to just wait and see if he presents with any symptoms of CF.
<br />
<br />Our problem stems from this "information age". My wife has become friends with a number of CF moms through Facebook. One mother has a son with same gene combo around the same age and her CF clinic has diagnosed her son with CF and they seem to be on a much more rigorous treatment regiment. Her child is doing chest therapy twice a day and is going to be getting "The Vest" when he is old enough to use it. He also has a cold where the congestion is building up and he is on a nebulizer to break-up the congestion.
<br />
<br />Finally, that brings me to my question. What are some advantages or disadvantages of a child with a mild form of CF being not diagnosed? To me he has it and I can see for insurance reasons not wanting to diagnose someone with something that isn't actually presenting itself for treatment. Obviously insurance won't pay for any prescriptions or treatments for a patient that hasn't been diagnosed with CF. I don't have any problem with this line of thinking, but should we be doing chest therapy and as he gets older think about getting the vest? Or be doing something else preventative now? We really haven't been told to do anything except: good hand hygiene, keep him away from smokers, flu shoots when 6 months and diet.
<br />
<br />There seems to be a lot of controversy over whether the R117G 7T/9T gene should even be part of the genetic testing for CF. I'm having a hard time trying to figure out if it would be advantageous for him to be diagnosed and push for a diagnosis from his CF Dr. or just trust the doctors and be cautiously optimistic. Sorry for the long post, but if I have left anything out please feel free to ask.
 

Ratatosk

Administrator
Staff member
Gene mutations don't necessarily predict CF outcomes. I tend to cringe when the word mild CF is thrown around because CF is a progressive disease that affects people differently, yet will eventually affect the lungs, etc.

Heck, there can be people in the same family (same genetic mutations) and very differing symptoms.

DS started off with more digestive issues; however, his doctor is very proactive when in comes to lung care. We've done CPT and nebs since he was a week or so old.

Oh and DS had a 32 for his sweat test.
 

Ratatosk

Administrator
Staff member
Gene mutations don't necessarily predict CF outcomes. I tend to cringe when the word mild CF is thrown around because CF is a progressive disease that affects people differently, yet will eventually affect the lungs, etc.

Heck, there can be people in the same family (same genetic mutations) and very differing symptoms.

DS started off with more digestive issues; however, his doctor is very proactive when in comes to lung care. We've done CPT and nebs since he was a week or so old.

Oh and DS had a 32 for his sweat test.
 

Ratatosk

Administrator
Staff member
Gene mutations don't necessarily predict CF outcomes. I tend to cringe when the word mild CF is thrown around because CF is a progressive disease that affects people differently, yet will eventually affect the lungs, etc.

Heck, there can be people in the same family (same genetic mutations) and very differing symptoms.

DS started off with more digestive issues; however, his doctor is very proactive when in comes to lung care. We've done CPT and nebs since he was a week or so old.

Oh and DS had a 32 for his sweat test.
 

Ratatosk

Administrator
Staff member
Gene mutations don't necessarily predict CF outcomes. I tend to cringe when the word mild CF is thrown around because CF is a progressive disease that affects people differently, yet will eventually affect the lungs, etc.

Heck, there can be people in the same family (same genetic mutations) and very differing symptoms.

DS started off with more digestive issues; however, his doctor is very proactive when in comes to lung care. We've done CPT and nebs since he was a week or so old.

Oh and DS had a 32 for his sweat test.
 

Ratatosk

Administrator
Staff member
Gene mutations don't necessarily predict CF outcomes. I tend to cringe when the word mild CF is thrown around because CF is a progressive disease that affects people differently, yet will eventually affect the lungs, etc.
<br />
<br />Heck, there can be people in the same family (same genetic mutations) and very differing symptoms.
<br />
<br />DS started off with more digestive issues; however, his doctor is very proactive when in comes to lung care. We've done CPT and nebs since he was a week or so old.
<br />
<br />Oh and DS had a 32 for his sweat test.
 

JazzysMom

New member
I will have to gather my thoughts & get back to you, but in the meantime you can do a search for these genes on the site.

We have other members with that combo & it might give you some insight!
 

JazzysMom

New member
I will have to gather my thoughts & get back to you, but in the meantime you can do a search for these genes on the site.

We have other members with that combo & it might give you some insight!
 

JazzysMom

New member
I will have to gather my thoughts & get back to you, but in the meantime you can do a search for these genes on the site.

We have other members with that combo & it might give you some insight!
 

JazzysMom

New member
I will have to gather my thoughts & get back to you, but in the meantime you can do a search for these genes on the site.

We have other members with that combo & it might give you some insight!
 

JazzysMom

New member
I will have to gather my thoughts & get back to you, but in the meantime you can do a search for these genes on the site.
<br />
<br />We have other members with that combo & it might give you some insight!
 

Shelbyville

New member
Welcome:

Sorry to hear about your new born's diagnosis however he may lead a normal life for a long time. The attached link may give you some help. You may want to take Milissa's advice. Loads of information and out comes here along with caring souls.

<a target=_blank class=ftalternatingbarlinklarge href="http://pediatrics.aappublications.org/cgi/content/full/118/3/1260?submit.y=14&submit.x=161&submit=sendit&gca=118%2F3%2F1260&
">http://pediatrics.aappublicati...t&gca=118%2F3%2F1260&
</a>
Best Regards, Steve
 

Shelbyville

New member
Welcome:

Sorry to hear about your new born's diagnosis however he may lead a normal life for a long time. The attached link may give you some help. You may want to take Milissa's advice. Loads of information and out comes here along with caring souls.

<a target=_blank class=ftalternatingbarlinklarge href="http://pediatrics.aappublications.org/cgi/content/full/118/3/1260?submit.y=14&submit.x=161&submit=sendit&gca=118%2F3%2F1260&
">http://pediatrics.aappublicati...t&gca=118%2F3%2F1260&
</a>
Best Regards, Steve
 

Shelbyville

New member
Welcome:

Sorry to hear about your new born's diagnosis however he may lead a normal life for a long time. The attached link may give you some help. You may want to take Milissa's advice. Loads of information and out comes here along with caring souls.

<a target=_blank class=ftalternatingbarlinklarge href="http://pediatrics.aappublications.org/cgi/content/full/118/3/1260?submit.y=14&submit.x=161&submit=sendit&gca=118%2F3%2F1260&
">http://pediatrics.aappublicati...t&gca=118%2F3%2F1260&
</a>
Best Regards, Steve
 

Shelbyville

New member
Welcome:

Sorry to hear about your new born's diagnosis however he may lead a normal life for a long time. The attached link may give you some help. You may want to take Milissa's advice. Loads of information and out comes here along with caring souls.

<a target=_blank class=ftalternatingbarlinklarge href="http://pediatrics.aappublications.org/cgi/content/full/118/3/1260?submit.y=14&submit.x=161&submit=sendit&gca=118%2F3%2F1260&
">http://pediatrics.aappublicati...t&gca=118%2F3%2F1260&
</a>
Best Regards, Steve
 

Shelbyville

New member
Welcome:
<br />
<br />Sorry to hear about your new born's diagnosis however he may lead a normal life for a long time. The attached link may give you some help. You may want to take Milissa's advice. Loads of information and out comes here along with caring souls.
<br />
<br /><a target=_blank class=ftalternatingbarlinklarge href="http://pediatrics.aappublications.org/cgi/content/full/118/3/1260?submit.y=14&submit.x=161&submit=sendit&gca=118%2F3%2F1260&
">http://pediatrics.aappublicati...t&gca=118%2F3%2F1260&
</a><br />
<br />Best Regards, Steve
 
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