Need advice from parents who have been there

[mama2emmett]

Hi there,
My name is Meghan and we were just told last night that our son needs to be worked up for CF. He currently is being treated for his most recent pneumonia, but in 10 days is scheduled to have a baseline CXR. Here is a little history. He has had lung issues since he was about a year old. He has been on a neb since then pulmicort bid from october to may, albuterol and xopenex prn and many rounds of prednisone. It seems like his symptoms get worse every year. He also has had several incidences of hypoglycemia, and had a fasting glucose of 60 (while on steroids). He has had an immunology work up due to intermittent high fevers, long duration and frequency of illness.
This year we moved to a new state and his bad time hit early. Mid september he was admitted to the hospital with pneumonia and a left upper lobe collapse. Then he was diagnosed with another left upper lobe pneumonia this past monday. He has never had any issues with weight, though he does seem to be thinning out dramatically.
His doc thinks he needs to be worked up at our local pedi pulmonologist which is a cf center. she thinks he needs to possibly have a bronchoscopy too. She said that the lung collapse is very rare and suspicious.

What are you thoughts? What is it important for me to ask? I am an RN but feel unprepared...please any feedback welcome.

Meghan mama2emmett

 

[mama2emmett]

Hi there,
My name is Meghan and we were just told last night that our son needs to be worked up for CF. He currently is being treated for his most recent pneumonia, but in 10 days is scheduled to have a baseline CXR. Here is a little history. He has had lung issues since he was about a year old. He has been on a neb since then pulmicort bid from october to may, albuterol and xopenex prn and many rounds of prednisone. It seems like his symptoms get worse every year. He also has had several incidences of hypoglycemia, and had a fasting glucose of 60 (while on steroids). He has had an immunology work up due to intermittent high fevers, long duration and frequency of illness.
This year we moved to a new state and his bad time hit early. Mid september he was admitted to the hospital with pneumonia and a left upper lobe collapse. Then he was diagnosed with another left upper lobe pneumonia this past monday. He has never had any issues with weight, though he does seem to be thinning out dramatically.
His doc thinks he needs to be worked up at our local pedi pulmonologist which is a cf center. she thinks he needs to possibly have a bronchoscopy too. She said that the lung collapse is very rare and suspicious.

What are you thoughts? What is it important for me to ask? I am an RN but feel unprepared...please any feedback welcome.

Meghan mama2emmett

 

[mama2emmett]

Hi there,
My name is Meghan and we were just told last night that our son needs to be worked up for CF. He currently is being treated for his most recent pneumonia, but in 10 days is scheduled to have a baseline CXR. Here is a little history. He has had lung issues since he was about a year old. He has been on a neb since then pulmicort bid from october to may, albuterol and xopenex prn and many rounds of prednisone. It seems like his symptoms get worse every year. He also has had several incidences of hypoglycemia, and had a fasting glucose of 60 (while on steroids). He has had an immunology work up due to intermittent high fevers, long duration and frequency of illness.
This year we moved to a new state and his bad time hit early. Mid september he was admitted to the hospital with pneumonia and a left upper lobe collapse. Then he was diagnosed with another left upper lobe pneumonia this past monday. He has never had any issues with weight, though he does seem to be thinning out dramatically.
His doc thinks he needs to be worked up at our local pedi pulmonologist which is a cf center. she thinks he needs to possibly have a bronchoscopy too. She said that the lung collapse is very rare and suspicious.

What are you thoughts? What is it important for me to ask? I am an RN but feel unprepared...please any feedback welcome.

Meghan mama2emmett

 

[mama2emmett]

Hi there,
My name is Meghan and we were just told last night that our son needs to be worked up for CF. He currently is being treated for his most recent pneumonia, but in 10 days is scheduled to have a baseline CXR. Here is a little history. He has had lung issues since he was about a year old. He has been on a neb since then pulmicort bid from october to may, albuterol and xopenex prn and many rounds of prednisone. It seems like his symptoms get worse every year. He also has had several incidences of hypoglycemia, and had a fasting glucose of 60 (while on steroids). He has had an immunology work up due to intermittent high fevers, long duration and frequency of illness.
This year we moved to a new state and his bad time hit early. Mid september he was admitted to the hospital with pneumonia and a left upper lobe collapse. Then he was diagnosed with another left upper lobe pneumonia this past monday. He has never had any issues with weight, though he does seem to be thinning out dramatically.
His doc thinks he needs to be worked up at our local pedi pulmonologist which is a cf center. she thinks he needs to possibly have a bronchoscopy too. She said that the lung collapse is very rare and suspicious.

What are you thoughts? What is it important for me to ask? I am an RN but feel unprepared...please any feedback welcome.

Meghan mama2emmett

 

[mama2emmett]

Hi there,
<br />My name is Meghan and we were just told last night that our son needs to be worked up for CF. He currently is being treated for his most recent pneumonia, but in 10 days is scheduled to have a baseline CXR. Here is a little history. He has had lung issues since he was about a year old. He has been on a neb since then pulmicort bid from october to may, albuterol and xopenex prn and many rounds of prednisone. It seems like his symptoms get worse every year. He also has had several incidences of hypoglycemia, and had a fasting glucose of 60 (while on steroids). He has had an immunology work up due to intermittent high fevers, long duration and frequency of illness.
<br />This year we moved to a new state and his bad time hit early. Mid september he was admitted to the hospital with pneumonia and a left upper lobe collapse. Then he was diagnosed with another left upper lobe pneumonia this past monday. He has never had any issues with weight, though he does seem to be thinning out dramatically.
<br />His doc thinks he needs to be worked up at our local pedi pulmonologist which is a cf center. she thinks he needs to possibly have a bronchoscopy too. She said that the lung collapse is very rare and suspicious.
<br />
<br />What are you thoughts? What is it important for me to ask? I am an RN but feel unprepared...please any feedback welcome.
<br />
<br />Meghan mama2emmett

 

[JazzysMom]

WOW sounds like a real rough time. Even if it is CF it also sounds like allergies can be aggrivating things so I would somehow work allergy testing into the whole thing. Not just the blood test because that isnt accurate enough. THe actual skin test.

The glucose test really needs to be done when he isnt on steroids because they can knock results so out of whack. All the lung issues could very well be CF.

Did they by any chance test any sputum? He could have a bacteria that is just causing havoc OR it could be the thick mucous from CF or it could just be freaking bad luck.

The sooner they get results (I would ask for full panel genetic testing for CF as well as sputum cultures), the sooner the proper treatment can be given.

Whether it be CF or not.....you really dont want his lungs going through so much. It will lead to permanent damage.

I know its hard to be prepared especially as a Nurse & you know more about the medical side of things. Please just take one day at a time.

We are here for support before, during & after!

HUGS

 

[JazzysMom]

WOW sounds like a real rough time. Even if it is CF it also sounds like allergies can be aggrivating things so I would somehow work allergy testing into the whole thing. Not just the blood test because that isnt accurate enough. THe actual skin test.

The glucose test really needs to be done when he isnt on steroids because they can knock results so out of whack. All the lung issues could very well be CF.

Did they by any chance test any sputum? He could have a bacteria that is just causing havoc OR it could be the thick mucous from CF or it could just be freaking bad luck.

The sooner they get results (I would ask for full panel genetic testing for CF as well as sputum cultures), the sooner the proper treatment can be given.

Whether it be CF or not.....you really dont want his lungs going through so much. It will lead to permanent damage.

I know its hard to be prepared especially as a Nurse & you know more about the medical side of things. Please just take one day at a time.

We are here for support before, during & after!

HUGS

 

[JazzysMom]

WOW sounds like a real rough time. Even if it is CF it also sounds like allergies can be aggrivating things so I would somehow work allergy testing into the whole thing. Not just the blood test because that isnt accurate enough. THe actual skin test.

The glucose test really needs to be done when he isnt on steroids because they can knock results so out of whack. All the lung issues could very well be CF.

Did they by any chance test any sputum? He could have a bacteria that is just causing havoc OR it could be the thick mucous from CF or it could just be freaking bad luck.

The sooner they get results (I would ask for full panel genetic testing for CF as well as sputum cultures), the sooner the proper treatment can be given.

Whether it be CF or not.....you really dont want his lungs going through so much. It will lead to permanent damage.

I know its hard to be prepared especially as a Nurse & you know more about the medical side of things. Please just take one day at a time.

We are here for support before, during & after!

HUGS

 

[JazzysMom]

WOW sounds like a real rough time. Even if it is CF it also sounds like allergies can be aggrivating things so I would somehow work allergy testing into the whole thing. Not just the blood test because that isnt accurate enough. THe actual skin test.

The glucose test really needs to be done when he isnt on steroids because they can knock results so out of whack. All the lung issues could very well be CF.

Did they by any chance test any sputum? He could have a bacteria that is just causing havoc OR it could be the thick mucous from CF or it could just be freaking bad luck.

The sooner they get results (I would ask for full panel genetic testing for CF as well as sputum cultures), the sooner the proper treatment can be given.

Whether it be CF or not.....you really dont want his lungs going through so much. It will lead to permanent damage.

I know its hard to be prepared especially as a Nurse & you know more about the medical side of things. Please just take one day at a time.

We are here for support before, during & after!

HUGS

 

[JazzysMom]

WOW sounds like a real rough time. Even if it is CF it also sounds like allergies can be aggrivating things so I would somehow work allergy testing into the whole thing. Not just the blood test because that isnt accurate enough. THe actual skin test.
<br />
<br />The glucose test really needs to be done when he isnt on steroids because they can knock results so out of whack. All the lung issues could very well be CF.
<br />
<br />Did they by any chance test any sputum? He could have a bacteria that is just causing havoc OR it could be the thick mucous from CF or it could just be freaking bad luck.
<br />
<br />The sooner they get results (I would ask for full panel genetic testing for CF as well as sputum cultures), the sooner the proper treatment can be given.
<br />
<br />Whether it be CF or not.....you really dont want his lungs going through so much. It will lead to permanent damage.
<br />
<br />I know its hard to be prepared especially as a Nurse & you know more about the medical side of things. Please just take one day at a time.
<br />
<br />We are here for support before, during & after!
<br />
<br />HUGS

 

[mama2emmett]

Thank you <img src="i/expressions/face-icon-small-smile.gif" border="0">

As for allergies, we have an allergist in Boston and he has been through every screening allergies wise (including blood tests and 4 or 5 rounds of skin tests). He used to test positive to everything but once on singulair he is only allergic to cats, and a few minor other environmental. But he takes singulair once a day.
They have not done a sputum culture but his previous pneumonia responded very well to antibiotics. This one seems to be too. I think a sputum is a good idea though.
I am all to aware about the dangers with his lungs. The doc's concern is that it takes a very serious "plug" to cause a collapse or an anatomic anomoly, so she wants to do a sweat test as well as the bronch just to see whats going on in there. From everything I have read here I should be asking for a genetic screening too. He is still so diminished in that lobe <img src="i/expressions/face-icon-small-sad.gif" border="0"> and it seems to get better then worse and so on even in the span of a week!

Is it possible that it could be CF even without a failure to thrive or obvious GI involvement?

I cant remember if they screened me during pregnancy....if it was negative could that rule it out?

He just seems to be getting progressively worse as time goes on...it gets more severe.

Thank you for listening to me vent and answering my questions, I have so much going through my head and it is going to be a long couple weeks until we start to get answers.

Meg

 

[mama2emmett]

Thank you <img src="i/expressions/face-icon-small-smile.gif" border="0">

As for allergies, we have an allergist in Boston and he has been through every screening allergies wise (including blood tests and 4 or 5 rounds of skin tests). He used to test positive to everything but once on singulair he is only allergic to cats, and a few minor other environmental. But he takes singulair once a day.
They have not done a sputum culture but his previous pneumonia responded very well to antibiotics. This one seems to be too. I think a sputum is a good idea though.
I am all to aware about the dangers with his lungs. The doc's concern is that it takes a very serious "plug" to cause a collapse or an anatomic anomoly, so she wants to do a sweat test as well as the bronch just to see whats going on in there. From everything I have read here I should be asking for a genetic screening too. He is still so diminished in that lobe <img src="i/expressions/face-icon-small-sad.gif" border="0"> and it seems to get better then worse and so on even in the span of a week!

Is it possible that it could be CF even without a failure to thrive or obvious GI involvement?

I cant remember if they screened me during pregnancy....if it was negative could that rule it out?

He just seems to be getting progressively worse as time goes on...it gets more severe.

Thank you for listening to me vent and answering my questions, I have so much going through my head and it is going to be a long couple weeks until we start to get answers.

Meg

 

[mama2emmett]

Thank you <img src="i/expressions/face-icon-small-smile.gif" border="0">

As for allergies, we have an allergist in Boston and he has been through every screening allergies wise (including blood tests and 4 or 5 rounds of skin tests). He used to test positive to everything but once on singulair he is only allergic to cats, and a few minor other environmental. But he takes singulair once a day.
They have not done a sputum culture but his previous pneumonia responded very well to antibiotics. This one seems to be too. I think a sputum is a good idea though.
I am all to aware about the dangers with his lungs. The doc's concern is that it takes a very serious "plug" to cause a collapse or an anatomic anomoly, so she wants to do a sweat test as well as the bronch just to see whats going on in there. From everything I have read here I should be asking for a genetic screening too. He is still so diminished in that lobe <img src="i/expressions/face-icon-small-sad.gif" border="0"> and it seems to get better then worse and so on even in the span of a week!

Is it possible that it could be CF even without a failure to thrive or obvious GI involvement?

I cant remember if they screened me during pregnancy....if it was negative could that rule it out?

He just seems to be getting progressively worse as time goes on...it gets more severe.

Thank you for listening to me vent and answering my questions, I have so much going through my head and it is going to be a long couple weeks until we start to get answers.

Meg

 

[mama2emmett]

Thank you <img src="i/expressions/face-icon-small-smile.gif" border="0">

As for allergies, we have an allergist in Boston and he has been through every screening allergies wise (including blood tests and 4 or 5 rounds of skin tests). He used to test positive to everything but once on singulair he is only allergic to cats, and a few minor other environmental. But he takes singulair once a day.
They have not done a sputum culture but his previous pneumonia responded very well to antibiotics. This one seems to be too. I think a sputum is a good idea though.
I am all to aware about the dangers with his lungs. The doc's concern is that it takes a very serious "plug" to cause a collapse or an anatomic anomoly, so she wants to do a sweat test as well as the bronch just to see whats going on in there. From everything I have read here I should be asking for a genetic screening too. He is still so diminished in that lobe <img src="i/expressions/face-icon-small-sad.gif" border="0"> and it seems to get better then worse and so on even in the span of a week!

Is it possible that it could be CF even without a failure to thrive or obvious GI involvement?

I cant remember if they screened me during pregnancy....if it was negative could that rule it out?

He just seems to be getting progressively worse as time goes on...it gets more severe.

Thank you for listening to me vent and answering my questions, I have so much going through my head and it is going to be a long couple weeks until we start to get answers.

Meg

 

[mama2emmett]

Thank you <img src="i/expressions/face-icon-small-smile.gif" border="0">
<br />
<br />As for allergies, we have an allergist in Boston and he has been through every screening allergies wise (including blood tests and 4 or 5 rounds of skin tests). He used to test positive to everything but once on singulair he is only allergic to cats, and a few minor other environmental. But he takes singulair once a day.
<br />They have not done a sputum culture but his previous pneumonia responded very well to antibiotics. This one seems to be too. I think a sputum is a good idea though.
<br />I am all to aware about the dangers with his lungs. The doc's concern is that it takes a very serious "plug" to cause a collapse or an anatomic anomoly, so she wants to do a sweat test as well as the bronch just to see whats going on in there. From everything I have read here I should be asking for a genetic screening too. He is still so diminished in that lobe <img src="i/expressions/face-icon-small-sad.gif" border="0"> and it seems to get better then worse and so on even in the span of a week!
<br />
<br />Is it possible that it could be CF even without a failure to thrive or obvious GI involvement?
<br />
<br />I cant remember if they screened me during pregnancy....if it was negative could that rule it out?
<br />
<br />He just seems to be getting progressively worse as time goes on...it gets more severe.
<br />
<br />Thank you for listening to me vent and answering my questions, I have so much going through my head and it is going to be a long couple weeks until we start to get answers.
<br />
<br />Meg

 

[websterhome]

I'm really new to CF... my 9 week old daughter was diagnosed at 6 days of life. I too am an RN & know how frustrating it can be to feel like you maybe should know more about what's going on, but don't. Anyway, I don't have tons of info for you but here's what comes to mind. We knew my husband & I were both carriers, we started with having a genetic test done on her at 5 days old. Just a capillary or venous blood draw for 0.5-1ml, it often takes a while to get results, our hospital only runs them on Mon & Thursdays (weird). Anyway, once we knew Lucy had it, they still wanted to do a sweat chloride test because it's considered the "gold standard" of CF diagnosis. I asked if it was possible for her sweat test to be negative even though her DNA test was positive, they said it would be incredibly unlikely. From what I gather, it's possible someone could have CF without major GI symptoms (although not our case) - not everyone with CF has pancreatic insufficiency. As for your pregnancy screening question - if you did have the CF DNA screen while pregnant & it was negative, I'm 99% sure that would mean your child could not possibly have CF. That test is starting to be offered more & more to pregnant women. Please know that someone in WA is thinking about you tonight! I'm so sorry you're having to go through all of this.
Christy

 

[websterhome]

I'm really new to CF... my 9 week old daughter was diagnosed at 6 days of life. I too am an RN & know how frustrating it can be to feel like you maybe should know more about what's going on, but don't. Anyway, I don't have tons of info for you but here's what comes to mind. We knew my husband & I were both carriers, we started with having a genetic test done on her at 5 days old. Just a capillary or venous blood draw for 0.5-1ml, it often takes a while to get results, our hospital only runs them on Mon & Thursdays (weird). Anyway, once we knew Lucy had it, they still wanted to do a sweat chloride test because it's considered the "gold standard" of CF diagnosis. I asked if it was possible for her sweat test to be negative even though her DNA test was positive, they said it would be incredibly unlikely. From what I gather, it's possible someone could have CF without major GI symptoms (although not our case) - not everyone with CF has pancreatic insufficiency. As for your pregnancy screening question - if you did have the CF DNA screen while pregnant & it was negative, I'm 99% sure that would mean your child could not possibly have CF. That test is starting to be offered more & more to pregnant women. Please know that someone in WA is thinking about you tonight! I'm so sorry you're having to go through all of this.
Christy

 

[websterhome]

I'm really new to CF... my 9 week old daughter was diagnosed at 6 days of life. I too am an RN & know how frustrating it can be to feel like you maybe should know more about what's going on, but don't. Anyway, I don't have tons of info for you but here's what comes to mind. We knew my husband & I were both carriers, we started with having a genetic test done on her at 5 days old. Just a capillary or venous blood draw for 0.5-1ml, it often takes a while to get results, our hospital only runs them on Mon & Thursdays (weird). Anyway, once we knew Lucy had it, they still wanted to do a sweat chloride test because it's considered the "gold standard" of CF diagnosis. I asked if it was possible for her sweat test to be negative even though her DNA test was positive, they said it would be incredibly unlikely. From what I gather, it's possible someone could have CF without major GI symptoms (although not our case) - not everyone with CF has pancreatic insufficiency. As for your pregnancy screening question - if you did have the CF DNA screen while pregnant & it was negative, I'm 99% sure that would mean your child could not possibly have CF. That test is starting to be offered more & more to pregnant women. Please know that someone in WA is thinking about you tonight! I'm so sorry you're having to go through all of this.
Christy

 

[websterhome]

I'm really new to CF... my 9 week old daughter was diagnosed at 6 days of life. I too am an RN & know how frustrating it can be to feel like you maybe should know more about what's going on, but don't. Anyway, I don't have tons of info for you but here's what comes to mind. We knew my husband & I were both carriers, we started with having a genetic test done on her at 5 days old. Just a capillary or venous blood draw for 0.5-1ml, it often takes a while to get results, our hospital only runs them on Mon & Thursdays (weird). Anyway, once we knew Lucy had it, they still wanted to do a sweat chloride test because it's considered the "gold standard" of CF diagnosis. I asked if it was possible for her sweat test to be negative even though her DNA test was positive, they said it would be incredibly unlikely. From what I gather, it's possible someone could have CF without major GI symptoms (although not our case) - not everyone with CF has pancreatic insufficiency. As for your pregnancy screening question - if you did have the CF DNA screen while pregnant & it was negative, I'm 99% sure that would mean your child could not possibly have CF. That test is starting to be offered more & more to pregnant women. Please know that someone in WA is thinking about you tonight! I'm so sorry you're having to go through all of this.
Christy

 

[websterhome]

I'm really new to CF... my 9 week old daughter was diagnosed at 6 days of life. I too am an RN & know how frustrating it can be to feel like you maybe should know more about what's going on, but don't. Anyway, I don't have tons of info for you but here's what comes to mind. We knew my husband & I were both carriers, we started with having a genetic test done on her at 5 days old. Just a capillary or venous blood draw for 0.5-1ml, it often takes a while to get results, our hospital only runs them on Mon & Thursdays (weird). Anyway, once we knew Lucy had it, they still wanted to do a sweat chloride test because it's considered the "gold standard" of CF diagnosis. I asked if it was possible for her sweat test to be negative even though her DNA test was positive, they said it would be incredibly unlikely. From what I gather, it's possible someone could have CF without major GI symptoms (although not our case) - not everyone with CF has pancreatic insufficiency. As for your pregnancy screening question - if you did have the CF DNA screen while pregnant & it was negative, I'm 99% sure that would mean your child could not possibly have CF. That test is starting to be offered more & more to pregnant women. Please know that someone in WA is thinking about you tonight! I'm so sorry you're having to go through all of this.
<br />Christy