Atypical cf?

[LouLou]

Pneumonia is a very severe lung infection that is diagnosed by a certain sound audible by stethoscope and viewable by chest xray. It is not something a typical child gets as you know. In the cf community it is avoided like the plague by preventative measures and acting fast when a chest infection starts (precursor to pneumonia). Do your kids really have pneumonia 4-5 times a year or did you mean a chest cold? I'm not sure how any cf doctor would say that this child has no lung involvement. In fact this sounds very severe. At 32 I can tell you exactly the dates of the two pneumonias I've had. And for reference I am not considered disabled by the US government.
<br />
<br />Are you doing all the typical cf treatments? If not it's time to demand being armed with the techniques and medication to best care for your children to PREVENT any further damage.

 

[Ratatosk]

I wonder if they may not need more enzymes. Are they getting the enzymes prior to eating, are they slow eaters? If so, you may want to divide the enzymes. Half at first, rest halfway thru the meal or snack.

I'm concerned as well with the lung infections. DS' issues have pretty much been digestive, though we've done CPT and nebs since he was a newborn. When he's gotten a cough, his doctors have been very agressive in upping treatments and prescribing antibiotics.

BTW, DS is one of those with a normal sweat test -- 32 -- yet a genetic blood test showing cf.

 

[Ratatosk]

I wonder if they may not need more enzymes. Are they getting the enzymes prior to eating, are they slow eaters? If so, you may want to divide the enzymes. Half at first, rest halfway thru the meal or snack.

I'm concerned as well with the lung infections. DS' issues have pretty much been digestive, though we've done CPT and nebs since he was a newborn. When he's gotten a cough, his doctors have been very agressive in upping treatments and prescribing antibiotics.

BTW, DS is one of those with a normal sweat test -- 32 -- yet a genetic blood test showing cf.

 

[Ratatosk]

I wonder if they may not need more enzymes. Are they getting the enzymes prior to eating, are they slow eaters? If so, you may want to divide the enzymes. Half at first, rest halfway thru the meal or snack.
<br />
<br />I'm concerned as well with the lung infections. DS' issues have pretty much been digestive, though we've done CPT and nebs since he was a newborn. When he's gotten a cough, his doctors have been very agressive in upping treatments and prescribing antibiotics.
<br />
<br />BTW, DS is one of those with a normal sweat test -- 32 -- yet a genetic blood test showing cf.

 

[Lilliansmom]

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>LouLou</b></i>

Pneumonia is a very severe lung infection that is diagnosed by a certain sound audible by stethoscope and viewable by chest xray. It is not something a typical child gets as you know. In the cf community it is avoided like the plague by preventative measures and acting fast when a chest infection starts (precursor to pneumonia). Do your kids really have pneumonia 4-5 times a year or did you mean a chest cold? I'm not sure how any cf doctor would say that this child has no lung involvement. In fact this sounds very severe. At 32 I can tell you exactly the dates of the two pneumonias I've had. And for reference I am not considered disabled by the US government.



Are you doing all the typical cf treatments? If not it's time to demand being armed with the techniques and medication to best care for your children to PREVENT any further damage.</end quote></div>

All of their pneumonias have been dx by chest x-ray. Some at our children's hospital, others at our ped. I did not think it was that big of a deal b/c they handle it at home with antiubiotics and neb treatments for the most part. DD has been hospitalized once for it, but I guess I just assumed that since they were managing at home that it was ok. The pulm has them on flovent and said he wanted to start antibiotics closer to the fall to try and prevent them from getting it this fall/winter. He was able to see more scarring on the x-ray that they did in the clinic than what they had had when compared to ones from September and December, so I am guessing that is not good.

That is all we are doing so far. I will add this to my list of questions/concerns for when we see him on the 2nd.

 

[Lilliansmom]

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>LouLou</b></i>

Pneumonia is a very severe lung infection that is diagnosed by a certain sound audible by stethoscope and viewable by chest xray. It is not something a typical child gets as you know. In the cf community it is avoided like the plague by preventative measures and acting fast when a chest infection starts (precursor to pneumonia). Do your kids really have pneumonia 4-5 times a year or did you mean a chest cold? I'm not sure how any cf doctor would say that this child has no lung involvement. In fact this sounds very severe. At 32 I can tell you exactly the dates of the two pneumonias I've had. And for reference I am not considered disabled by the US government.



Are you doing all the typical cf treatments? If not it's time to demand being armed with the techniques and medication to best care for your children to PREVENT any further damage.</end quote>

All of their pneumonias have been dx by chest x-ray. Some at our children's hospital, others at our ped. I did not think it was that big of a deal b/c they handle it at home with antiubiotics and neb treatments for the most part. DD has been hospitalized once for it, but I guess I just assumed that since they were managing at home that it was ok. The pulm has them on flovent and said he wanted to start antibiotics closer to the fall to try and prevent them from getting it this fall/winter. He was able to see more scarring on the x-ray that they did in the clinic than what they had had when compared to ones from September and December, so I am guessing that is not good.

That is all we are doing so far. I will add this to my list of questions/concerns for when we see him on the 2nd.

 

[Lilliansmom]

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>LouLou</b></i>
<br />
<br />Pneumonia is a very severe lung infection that is diagnosed by a certain sound audible by stethoscope and viewable by chest xray. It is not something a typical child gets as you know. In the cf community it is avoided like the plague by preventative measures and acting fast when a chest infection starts (precursor to pneumonia). Do your kids really have pneumonia 4-5 times a year or did you mean a chest cold? I'm not sure how any cf doctor would say that this child has no lung involvement. In fact this sounds very severe. At 32 I can tell you exactly the dates of the two pneumonias I've had. And for reference I am not considered disabled by the US government.
<br />
<br />
<br />
<br />Are you doing all the typical cf treatments? If not it's time to demand being armed with the techniques and medication to best care for your children to PREVENT any further damage.</end quote>
<br />
<br />All of their pneumonias have been dx by chest x-ray. Some at our children's hospital, others at our ped. I did not think it was that big of a deal b/c they handle it at home with antiubiotics and neb treatments for the most part. DD has been hospitalized once for it, but I guess I just assumed that since they were managing at home that it was ok. The pulm has them on flovent and said he wanted to start antibiotics closer to the fall to try and prevent them from getting it this fall/winter. He was able to see more scarring on the x-ray that they did in the clinic than what they had had when compared to ones from September and December, so I am guessing that is not good.
<br />
<br />That is all we are doing so far. I will add this to my list of questions/concerns for when we see him on the 2nd.
<br />
<br />

 

[Lilliansmom]

of their Atypical cf?

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>Ratatosk</b></i>

I wonder if they may not need more enzymes. Are they getting the enzymes prior to eating, are they slow eaters? If so, you may want to divide the enzymes. Half at first, rest halfway thru the meal or snack.



I'm concerned as well with the lung infections. DS' issues have pretty much been digestive, though we've done CPT and nebs since he was a newborn. When he's gotten a cough, his doctors have been very agressive in upping treatments and prescribing antibiotics.



BTW, DS is one of those with a normal sweat test -- 32 -- yet a genetic blood test showing cf.</end quote></div>

They may need more. Lilly tends to gain for awhile and then stop till we up hers. Jack tends to be more os a slow staedy gainer. They usually take them before eating, although occasioanlly they are half way through by the time I get everything broken open. They eat pretty quickly.

Our ped always chalked JAck's frequent lung infections up to being a preemie - he was 10 weeks early, although his twin sister never gets pnuemonia, so maybe she is wrong on that.

Thanks for your advice.

 

[Lilliansmom]

of their Atypical cf?

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>Ratatosk</b></i>

I wonder if they may not need more enzymes. Are they getting the enzymes prior to eating, are they slow eaters? If so, you may want to divide the enzymes. Half at first, rest halfway thru the meal or snack.



I'm concerned as well with the lung infections. DS' issues have pretty much been digestive, though we've done CPT and nebs since he was a newborn. When he's gotten a cough, his doctors have been very agressive in upping treatments and prescribing antibiotics.



BTW, DS is one of those with a normal sweat test -- 32 -- yet a genetic blood test showing cf.</end quote>

They may need more. Lilly tends to gain for awhile and then stop till we up hers. Jack tends to be more os a slow staedy gainer. They usually take them before eating, although occasioanlly they are half way through by the time I get everything broken open. They eat pretty quickly.

Our ped always chalked JAck's frequent lung infections up to being a preemie - he was 10 weeks early, although his twin sister never gets pnuemonia, so maybe she is wrong on that.

Thanks for your advice.

 

[Lilliansmom]

of their Atypical cf?

<div class="FTQUOTE"><begin quote><i>Originally posted by: <b>Ratatosk</b></i>
<br />
<br />I wonder if they may not need more enzymes. Are they getting the enzymes prior to eating, are they slow eaters? If so, you may want to divide the enzymes. Half at first, rest halfway thru the meal or snack.
<br />
<br />
<br />
<br />I'm concerned as well with the lung infections. DS' issues have pretty much been digestive, though we've done CPT and nebs since he was a newborn. When he's gotten a cough, his doctors have been very agressive in upping treatments and prescribing antibiotics.
<br />
<br />
<br />
<br />BTW, DS is one of those with a normal sweat test -- 32 -- yet a genetic blood test showing cf.</end quote>
<br />
<br />They may need more. Lilly tends to gain for awhile and then stop till we up hers. Jack tends to be more os a slow staedy gainer. They usually take them before eating, although occasioanlly they are half way through by the time I get everything broken open. They eat pretty quickly.
<br />
<br />Our ped always chalked JAck's frequent lung infections up to being a preemie - he was 10 weeks early, although his twin sister never gets pnuemonia, so maybe she is wrong on that.
<br />
<br />Thanks for your advice.
<br />
<br />

 

[Ratatosk]

of their Atypical cf?

With DS, his baseline is no cough right now. Meaning, if he develops a cough, we're to contact the doctor and figure out a treatment plan. We do CPT/Vest 3 times a day (bare minimum of twice a day). If it looks like he's starting to come down with a cold or cough, we bump up his treatments to 4 times.

 

[Ratatosk]

of their Atypical cf?

With DS, his baseline is no cough right now. Meaning, if he develops a cough, we're to contact the doctor and figure out a treatment plan. We do CPT/Vest 3 times a day (bare minimum of twice a day). If it looks like he's starting to come down with a cold or cough, we bump up his treatments to 4 times.

 

[Ratatosk]

of their Atypical cf?

With DS, his baseline is no cough right now. Meaning, if he develops a cough, we're to contact the doctor and figure out a treatment plan. We do CPT/Vest 3 times a day (bare minimum of twice a day). If it looks like he's starting to come down with a cold or cough, we bump up his treatments to 4 times.

 

[izemmom]

of their Atypical cf?

I'm sorry you're not getting answers. Do have dealt with all of this for so long with no clear diagnosis must be so frustrating.

I hope you'll continue to fight, request blood tests until there is a clear answer and then be able to get the kids on the road to better health.

One thing that stood out for me in one of your original posts is that your son has not had breathing tests done. My daughter is 4 and has been doing PFT's for over a year. Yes, there's a fair amount of unreliability at this age, but we are able to get a baseline and detect changes which has been helpful. Before she was old enough to do this she had infant PFT's. Even without a dx, with your son's history, including the prematurity, he SHOULD be having some sort of annual lung function testing done, don't you think? Kids with mild asthma get at least spiromerty done on a regular basis and you're dealing with recurrant pneumonia.

I wish you the best of luck and hope for answers and better results soon!!!

 

[izemmom]

of their Atypical cf?

I'm sorry you're not getting answers. Do have dealt with all of this for so long with no clear diagnosis must be so frustrating.

I hope you'll continue to fight, request blood tests until there is a clear answer and then be able to get the kids on the road to better health.

One thing that stood out for me in one of your original posts is that your son has not had breathing tests done. My daughter is 4 and has been doing PFT's for over a year. Yes, there's a fair amount of unreliability at this age, but we are able to get a baseline and detect changes which has been helpful. Before she was old enough to do this she had infant PFT's. Even without a dx, with your son's history, including the prematurity, he SHOULD be having some sort of annual lung function testing done, don't you think? Kids with mild asthma get at least spiromerty done on a regular basis and you're dealing with recurrant pneumonia.

I wish you the best of luck and hope for answers and better results soon!!!

 

[izemmom]

of their Atypical cf?

I'm sorry you're not getting answers. Do have dealt with all of this for so long with no clear diagnosis must be so frustrating.
<br />
<br />I hope you'll continue to fight, request blood tests until there is a clear answer and then be able to get the kids on the road to better health.
<br />
<br />One thing that stood out for me in one of your original posts is that your son has not had breathing tests done. My daughter is 4 and has been doing PFT's for over a year. Yes, there's a fair amount of unreliability at this age, but we are able to get a baseline and detect changes which has been helpful. Before she was old enough to do this she had infant PFT's. Even without a dx, with your son's history, including the prematurity, he SHOULD be having some sort of annual lung function testing done, don't you think? Kids with mild asthma get at least spiromerty done on a regular basis and you're dealing with recurrant pneumonia.
<br />
<br />I wish you the best of luck and hope for answers and better results soon!!!

 

[Melissa75]

Pneumonia is weird. My 8-yr-old has had it twice, dx'ed via stethoscope--one with x-ray follow-up. He was ill, don't get me wrong, but not violently ill. Partly because he has never had wheezing or any asthma. Partly because the fluid hadn't filled the infected lobe.

I think this is called "broncho-pneumonia" which is a lower respiratory tract infection (and SHOULD NOT happen to a kid 4-5 times a year and be considered normal).

Then there is lobar pneumonia, when one or more of the lobes is filled with fluid. That is the one usually accompanied by rusty sputum, high fever, violent chills...quite memorable as LouLou says.

For people with mucus in their lungs all the time, pneumonia is a tricky dx. As my allergy shot nurse says, if I didnt know this was your baseline, I'd send you to the hospital for an x-ray and antibiotics, presumed pneumonia...not the lobar kind though.

Meh...in rereading some articles, the line btw these pneumonias is really fuzzy.

This is just plain confusing. Bottom line, 4-5 broncho-pneumonias is not normal.

 

[Melissa75]

Pneumonia is weird. My 8-yr-old has had it twice, dx'ed via stethoscope--one with x-ray follow-up. He was ill, don't get me wrong, but not violently ill. Partly because he has never had wheezing or any asthma. Partly because the fluid hadn't filled the infected lobe.

I think this is called "broncho-pneumonia" which is a lower respiratory tract infection (and SHOULD NOT happen to a kid 4-5 times a year and be considered normal).

Then there is lobar pneumonia, when one or more of the lobes is filled with fluid. That is the one usually accompanied by rusty sputum, high fever, violent chills...quite memorable as LouLou says.

For people with mucus in their lungs all the time, pneumonia is a tricky dx. As my allergy shot nurse says, if I didnt know this was your baseline, I'd send you to the hospital for an x-ray and antibiotics, presumed pneumonia...not the lobar kind though.

Meh...in rereading some articles, the line btw these pneumonias is really fuzzy.

This is just plain confusing. Bottom line, 4-5 broncho-pneumonias is not normal.

 

[Melissa75]

Pneumonia is weird. My 8-yr-old has had it twice, dx'ed via stethoscope--one with x-ray follow-up. He was ill, don't get me wrong, but not violently ill. Partly because he has never had wheezing or any asthma. Partly because the fluid hadn't filled the infected lobe.
<br />
<br />I think this is called "broncho-pneumonia" which is a lower respiratory tract infection (and SHOULD NOT happen to a kid 4-5 times a year and be considered normal).
<br />
<br />Then there is lobar pneumonia, when one or more of the lobes is filled with fluid. That is the one usually accompanied by rusty sputum, high fever, violent chills...quite memorable as LouLou says.
<br />
<br />For people with mucus in their lungs all the time, pneumonia is a tricky dx. As my allergy shot nurse says, if I didnt know this was your baseline, I'd send you to the hospital for an x-ray and antibiotics, presumed pneumonia...not the lobar kind though.
<br />
<br />Meh...in rereading some articles, the line btw these pneumonias is really fuzzy.
<br />
<br />This is just plain confusing. Bottom line, 4-5 broncho-pneumonias is not normal.
<br />

 

[anchored]

I had a very unusual call and got to speak with the CF director at Boston Children's yesterday. We have a child with crazy sinuses and is a carrier. I had a number of questions about nasal potential difference testing as he had a "normal" sweat test of 10 and 11. The director told me that you can never say someone has a normal sweat test. You can say that a sweat test is not indicative of CF but you can't diagnose on just that. To determine if someone has CF you have to walk through a number of different things like genetic testing, stool testing, lung x-rays, cultures, and medical history to come up with a diagnosis. I was relieved to hear that as another CF center told us "he has a normal sweat test, isn't that great! You can go home happy now." No, I can't I still have a sick kid and no clear path forward!

I'm feeling better about going to Children's to have an honest discussion and thorough eval to see where we really are. We are also seriously considering participating in a nasal potential difference study at Children's Hospital of Philadelphia: <a target=_blank class=ftalternatingbarlinklarge href="http://clinicaltrials.gov/ct2/show/NCT00589745
">http://clinicaltrials.gov/ct2/show/NCT00589745
</a>
I hope you are able to get some clear answers. Don't stop asking hard questions. Also I found it very helpful to Google for medical journal articles on atypical CF, nasal potential difference and such. I read the abstracts and if I thought something looked really good I asked our doctor if he could order an article for me. Then I highlighted all the stuff that seemed to line up with our son and talk to the doctors from that viewpoint. The European medical journals have a lot of info. Its slow reading and I certainly needed a dictionary at hand but it has opened up doors that wouldn't have otherwise been open.

Best wishes on this journey!