Diagnosis in sight?

[Mommy2Zeke]

I've posted many time here on the boards before. I'm a 28 yr old mom to a gorgeous 21 month old boy with CF (DDF508). I have had an extensive personal history with respiratory illness. (Nasal polyps, severe asthma, pneumonia, chronic sinusitis, bronchitis.)

Since my son's diagnosis at birth with CF, my son's docs as well as my husband and I have wondered to what extent I might have CF or some other chronic lung disease. Several months ago I had the very basic genetic mutation panel and of course, I came back a carrier for delta f508 but no other mutation showed up. My sweat tests were in the 30's, so for the time being we decided to hold off on further testing unless I started having more issues. Cut to now, Where I've had 3 sinus infections and 3 bouts of bronchitis/pneumonia in just the last 10 months and now I'm on the third round of abx for bronchitis/cough that will not go away. I've gone through periods of this for years, I'd get better and make it for long periods of time with only sinuses or allergies bothering me, then I'd have a full year where I'd be sick almost constantly and get everything under the sun and it would hang on forever.

My doctor (an allergist/immunologist) has been talking to pulmonologists in the same practice and they've slowly been putting together the pieces as my illnesses have piled up. Today she finally brought up the possibility that I have Bronchiectasis and/or some form of CF and is now concerned enough to run a lot of tests to hopefully rule some things out and possibly find a diagnosis. Starting with an eNO test and sputum smear test, immunoglobulin panels, skin testing (to rule out allergies, although she said she doubts this is the issue), a CT scan of my lungs and sinuses, and some other smaller tests.

For now she's put me on Xopenex .63 every 6-8 hours until the cough subsides, and she switched me from Erythromycin to a high dose of Zithromax for another 7 days.
She asked me today about my son's vest and said even if she doesn't find bronchiectasis that she would like me to get a vest and do regular CPT for preventative care.

I know there are plenty of you who have bronchiectasis roaming here... does this plan of action sound reasonable to you?

 

[Mommy2Zeke]

I've posted many time here on the boards before. I'm a 28 yr old mom to a gorgeous 21 month old boy with CF (DDF508). I have had an extensive personal history with respiratory illness. (Nasal polyps, severe asthma, pneumonia, chronic sinusitis, bronchitis.)

Since my son's diagnosis at birth with CF, my son's docs as well as my husband and I have wondered to what extent I might have CF or some other chronic lung disease. Several months ago I had the very basic genetic mutation panel and of course, I came back a carrier for delta f508 but no other mutation showed up. My sweat tests were in the 30's, so for the time being we decided to hold off on further testing unless I started having more issues. Cut to now, Where I've had 3 sinus infections and 3 bouts of bronchitis/pneumonia in just the last 10 months and now I'm on the third round of abx for bronchitis/cough that will not go away. I've gone through periods of this for years, I'd get better and make it for long periods of time with only sinuses or allergies bothering me, then I'd have a full year where I'd be sick almost constantly and get everything under the sun and it would hang on forever.

My doctor (an allergist/immunologist) has been talking to pulmonologists in the same practice and they've slowly been putting together the pieces as my illnesses have piled up. Today she finally brought up the possibility that I have Bronchiectasis and/or some form of CF and is now concerned enough to run a lot of tests to hopefully rule some things out and possibly find a diagnosis. Starting with an eNO test and sputum smear test, immunoglobulin panels, skin testing (to rule out allergies, although she said she doubts this is the issue), a CT scan of my lungs and sinuses, and some other smaller tests.

For now she's put me on Xopenex .63 every 6-8 hours until the cough subsides, and she switched me from Erythromycin to a high dose of Zithromax for another 7 days.
She asked me today about my son's vest and said even if she doesn't find bronchiectasis that she would like me to get a vest and do regular CPT for preventative care.

I know there are plenty of you who have bronchiectasis roaming here... does this plan of action sound reasonable to you?

 

[Mommy2Zeke]

I've posted many time here on the boards before. I'm a 28 yr old mom to a gorgeous 21 month old boy with CF (DDF508). I have had an extensive personal history with respiratory illness. (Nasal polyps, severe asthma, pneumonia, chronic sinusitis, bronchitis.)
<br />
<br />Since my son's diagnosis at birth with CF, my son's docs as well as my husband and I have wondered to what extent I might have CF or some other chronic lung disease. Several months ago I had the very basic genetic mutation panel and of course, I came back a carrier for delta f508 but no other mutation showed up. My sweat tests were in the 30's, so for the time being we decided to hold off on further testing unless I started having more issues. Cut to now, Where I've had 3 sinus infections and 3 bouts of bronchitis/pneumonia in just the last 10 months and now I'm on the third round of abx for bronchitis/cough that will not go away. I've gone through periods of this for years, I'd get better and make it for long periods of time with only sinuses or allergies bothering me, then I'd have a full year where I'd be sick almost constantly and get everything under the sun and it would hang on forever.
<br />
<br />My doctor (an allergist/immunologist) has been talking to pulmonologists in the same practice and they've slowly been putting together the pieces as my illnesses have piled up. Today she finally brought up the possibility that I have Bronchiectasis and/or some form of CF and is now concerned enough to run a lot of tests to hopefully rule some things out and possibly find a diagnosis. Starting with an eNO test and sputum smear test, immunoglobulin panels, skin testing (to rule out allergies, although she said she doubts this is the issue), a CT scan of my lungs and sinuses, and some other smaller tests.
<br />
<br />For now she's put me on Xopenex .63 every 6-8 hours until the cough subsides, and she switched me from Erythromycin to a high dose of Zithromax for another 7 days.
<br />She asked me today about my son's vest and said even if she doesn't find bronchiectasis that she would like me to get a vest and do regular CPT for preventative care.
<br />
<br />I know there are plenty of you who have bronchiectasis roaming here... does this plan of action sound reasonable to you?

 

[Printer]

Jen:

I was dx at 47. The best advice that I could give you is to get yourself to an ADULT CF CENTER.

I can't begin to tell you how many Doctors that I have had contact with that don't have a clue about CF, much less have the ability to dx it. I am constanly educating my Primary Care Physician.

Best of luck to you,

Bill

 

[Printer]

Jen:

I was dx at 47. The best advice that I could give you is to get yourself to an ADULT CF CENTER.

I can't begin to tell you how many Doctors that I have had contact with that don't have a clue about CF, much less have the ability to dx it. I am constanly educating my Primary Care Physician.

Best of luck to you,

Bill

 

[Printer]

Jen:
<br />
<br />I was dx at 47. The best advice that I could give you is to get yourself to an ADULT CF CENTER.
<br />
<br />I can't begin to tell you how many Doctors that I have had contact with that don't have a clue about CF, much less have the ability to dx it. I am constanly educating my Primary Care Physician.
<br />
<br />Best of luck to you,
<br />
<br />Bill

 

[Melissa75]

Jen,

Hi, I am sorry you have been sick. I remember your son was struggling with C. dif. I hope that has cleared up.

If you get the bronchiectasis dx and are cleared for not having another CF gene, you would do some or all of the CF lung treatments, based on the severity of your infections/bacteria and lung function decline.

I do pretty minimal stuff these days because I was able to up my exercise to 3+ hours of cardio a week. My sweat test was 11 and have never cultured classic CF bacteria.
Your sputum cultures and high res CT scan will help figure out what is going on.

2x a day
Symbicort inhaler (steriod and long-lasting bronchodilator)
Neil Med sinus wash using distilled water, which I don't think is CF protocol (to wash out the allergens and viruses)
Acapella (loosen the collected mucus)

1x a day
Mucinex extended release (mucus thinner)
Singulair (allergy/asthma pill, not antihistamine)

As needed
Ibuprofen (inflammation reducer, lung and body aches)
PharmaNAC fizzy tabs (another mucus thinner)

Current antibiotic, but not prophylactic 3x/week like many CFers
Zithromax

Hope this helps. You can up what you are already doing by adding Mucinex and the Sinus washes. You don't need a script for that.

 

[Melissa75]

Jen,

Hi, I am sorry you have been sick. I remember your son was struggling with C. dif. I hope that has cleared up.

If you get the bronchiectasis dx and are cleared for not having another CF gene, you would do some or all of the CF lung treatments, based on the severity of your infections/bacteria and lung function decline.

I do pretty minimal stuff these days because I was able to up my exercise to 3+ hours of cardio a week. My sweat test was 11 and have never cultured classic CF bacteria.
Your sputum cultures and high res CT scan will help figure out what is going on.

2x a day
Symbicort inhaler (steriod and long-lasting bronchodilator)
Neil Med sinus wash using distilled water, which I don't think is CF protocol (to wash out the allergens and viruses)
Acapella (loosen the collected mucus)

1x a day
Mucinex extended release (mucus thinner)
Singulair (allergy/asthma pill, not antihistamine)

As needed
Ibuprofen (inflammation reducer, lung and body aches)
PharmaNAC fizzy tabs (another mucus thinner)

Current antibiotic, but not prophylactic 3x/week like many CFers
Zithromax

Hope this helps. You can up what you are already doing by adding Mucinex and the Sinus washes. You don't need a script for that.

 

[Melissa75]

Jen,
<br />
<br />Hi, I am sorry you have been sick. I remember your son was struggling with C. dif. I hope that has cleared up.
<br />
<br />If you get the bronchiectasis dx and are cleared for not having another CF gene, you would do some or all of the CF lung treatments, based on the severity of your infections/bacteria and lung function decline.
<br />
<br />I do pretty minimal stuff these days because I was able to up my exercise to 3+ hours of cardio a week. My sweat test was 11 and have never cultured classic CF bacteria.
<br />Your sputum cultures and high res CT scan will help figure out what is going on.
<br />
<br />2x a day
<br />Symbicort inhaler (steriod and long-lasting bronchodilator)
<br />Neil Med sinus wash using distilled water, which I don't think is CF protocol (to wash out the allergens and viruses)
<br />Acapella (loosen the collected mucus)
<br />
<br />1x a day
<br />Mucinex extended release (mucus thinner)
<br />Singulair (allergy/asthma pill, not antihistamine)
<br />
<br />As needed
<br />Ibuprofen (inflammation reducer, lung and body aches)
<br />PharmaNAC fizzy tabs (another mucus thinner)
<br />
<br />Current antibiotic, but not prophylactic 3x/week like many CFers
<br />Zithromax
<br />
<br />Hope this helps. You can up what you are already doing by adding Mucinex and the Sinus washes. You don't need a script for that.

 

[Mommy2Zeke]

Actually, I already do Mucinex daily and Neil Med Sinus washes pretty regularly. My doctor wants me doing them every day now. I was/am training for a half marathon in May and will be returning to a full schedule of working out now that my doc has cleared me to go ahead with exercise... so I'll be getting in 6+ hours of cardio a week. That should do something to help keep things loosened up. I will say that since I started running (except for this latest bout) I've been able to cough up stuff easier.

Actually... it wasn't C diff that my son had, it was VRE. He's a lot better and hasn't been in the hospital at all since June. He also got his central line removed after 18 months!!! He's doing great and is in the 60th percentile of weight for height and continues to amaze his doctors. He is culturing Psuedomonas, but is non-symptomatic. He's had no lung exacerbations at all this year!

 

[Mommy2Zeke]

Actually, I already do Mucinex daily and Neil Med Sinus washes pretty regularly. My doctor wants me doing them every day now. I was/am training for a half marathon in May and will be returning to a full schedule of working out now that my doc has cleared me to go ahead with exercise... so I'll be getting in 6+ hours of cardio a week. That should do something to help keep things loosened up. I will say that since I started running (except for this latest bout) I've been able to cough up stuff easier.

Actually... it wasn't C diff that my son had, it was VRE. He's a lot better and hasn't been in the hospital at all since June. He also got his central line removed after 18 months!!! He's doing great and is in the 60th percentile of weight for height and continues to amaze his doctors. He is culturing Psuedomonas, but is non-symptomatic. He's had no lung exacerbations at all this year!

 

[Mommy2Zeke]

Actually, I already do Mucinex daily and Neil Med Sinus washes pretty regularly. My doctor wants me doing them every day now. I was/am training for a half marathon in May and will be returning to a full schedule of working out now that my doc has cleared me to go ahead with exercise... so I'll be getting in 6+ hours of cardio a week. That should do something to help keep things loosened up. I will say that since I started running (except for this latest bout) I've been able to cough up stuff easier.
<br />
<br />Actually... it wasn't C diff that my son had, it was VRE. He's a lot better and hasn't been in the hospital at all since June. He also got his central line removed after 18 months!!! He's doing great and is in the 60th percentile of weight for height and continues to amaze his doctors. He is culturing Psuedomonas, but is non-symptomatic. He's had no lung exacerbations at all this year!

 

[AnnieT]

I have it, but I also have pseudomonas. I'm on TOBI now as I have another infection. Honestly, the Zpak doesn't do anything for me, but I also have that nasty bacteria in me.

 

[AnnieT]

I have it, but I also have pseudomonas. I'm on TOBI now as I have another infection. Honestly, the Zpak doesn't do anything for me, but I also have that nasty bacteria in me.

 

[AnnieT]

I have it, but I also have pseudomonas. I'm on TOBI now as I have another infection. Honestly, the Zpak doesn't do anything for me, but I also have that nasty bacteria in me.

 

[Melissa75]

Jen,
I am so glad your son is doing well and growing well.
It is great too that you are running, it offers a huge benefit to your lungs and immune system though I understand that sometimes it is just not possible.
It was this website and Jerry Cahill who inspired me to make exercise a top priority.
I will be interested to hear how your tests go.

 

[Melissa75]

Jen,
I am so glad your son is doing well and growing well.
It is great too that you are running, it offers a huge benefit to your lungs and immune system though I understand that sometimes it is just not possible.
It was this website and Jerry Cahill who inspired me to make exercise a top priority.
I will be interested to hear how your tests go.

 

[Melissa75]

Jen,
<br />I am so glad your son is doing well and growing well.
<br />It is great too that you are running, it offers a huge benefit to your lungs and immune system though I understand that sometimes it is just not possible.
<br />It was this website and Jerry Cahill who inspired me to make exercise a top priority.
<br />I will be interested to hear how your tests go.

 

[2005CFmom]

Jen,

My husband was diagnosed with bronchiectasis after our daughter was diagnosed with CF. He developed a severe cough that we could not get under control and was coughing up mucus plugs. His doc did the allergy/asthma route, but treatment didn't help much. He went to an ENT and found out his sinus were FULL of polyps, had them removed. Little to no improvement.

Still, recently having a daughter diagnosed with CF, and being on CF sites and hearing about adult diagnosis of CF made us want to be sure he does not have CF...so onto the pulmonologists. Lung scans done indicated bronchiectasis, thus his current diagnosis.

We already knew he was at least a carrier of DDF508, so they preformed a sweat test first. I think it was 18. The doctor was still not convinced that it was not CF so he ordered the Ambry CF Amplified test. He still showed only as a carrier.

Our daughters CF doctor and nurse were very interested in my husbands case. They set up an appointment for him to see the Adult CF doctor on one of his regular days (not a CF clinic day). He felt (as does my husbands pulmonologist) that although my husband may not have CF, his carrier status could be influencing his symptoms.

Long story to get to this, his treatment.

AM
Nebs -Albuterol, Hyper-sal, and Acetylcysteine (Mucomyst)
30 Minute Vest
Advair 500/50
Nasonex

PM
Nebs - Albuterol, Hyper-Sal, and Pulmozyme
30 Minute Vest
Advair 500/50
Nasonex

He deals more with inflammation issues rather than infection issues. His go to drug for when he develops his uncontrollable cough is prednisone. Starts at a high dose then tapers down over the next few weeks. Thankfully his treatment regime has been very effective and it has been over a year since he had to take prednisone.

Let me know if you have any questions. It was a very long process and I'm sure I left a lot of information out.

Good Luck on your journey!

P.S. Just some info on sputum test, both he and my daughter culture staph (not MRSA or PA).

 

[2005CFmom]

Jen,

My husband was diagnosed with bronchiectasis after our daughter was diagnosed with CF. He developed a severe cough that we could not get under control and was coughing up mucus plugs. His doc did the allergy/asthma route, but treatment didn't help much. He went to an ENT and found out his sinus were FULL of polyps, had them removed. Little to no improvement.

Still, recently having a daughter diagnosed with CF, and being on CF sites and hearing about adult diagnosis of CF made us want to be sure he does not have CF...so onto the pulmonologists. Lung scans done indicated bronchiectasis, thus his current diagnosis.

We already knew he was at least a carrier of DDF508, so they preformed a sweat test first. I think it was 18. The doctor was still not convinced that it was not CF so he ordered the Ambry CF Amplified test. He still showed only as a carrier.

Our daughters CF doctor and nurse were very interested in my husbands case. They set up an appointment for him to see the Adult CF doctor on one of his regular days (not a CF clinic day). He felt (as does my husbands pulmonologist) that although my husband may not have CF, his carrier status could be influencing his symptoms.

Long story to get to this, his treatment.

AM
Nebs -Albuterol, Hyper-sal, and Acetylcysteine (Mucomyst)
30 Minute Vest
Advair 500/50
Nasonex

PM
Nebs - Albuterol, Hyper-Sal, and Pulmozyme
30 Minute Vest
Advair 500/50
Nasonex

He deals more with inflammation issues rather than infection issues. His go to drug for when he develops his uncontrollable cough is prednisone. Starts at a high dose then tapers down over the next few weeks. Thankfully his treatment regime has been very effective and it has been over a year since he had to take prednisone.

Let me know if you have any questions. It was a very long process and I'm sure I left a lot of information out.

Good Luck on your journey!

P.S. Just some info on sputum test, both he and my daughter culture staph (not MRSA or PA).