Sorry to go missing for so long- update on the kids!

hmw

New member
I know I kind of disappeared for a few months and I want to update everyone on how things have been going here. I have missed everyone and hope to be back here again.
Emily got very sick the week of that awful end of October snowstorm. We weren't able to take care of her as we should and I know that didn't help. She was sick through all of November, despite a couple oral abx and ended up inpatient on IV's as well as cleanout for partial obstruction. She was on home IV's for barely a week when she ended up back inpatient with a horrendous GI thing. Everything shut down, she had a high fever (which of course also had them worrying about a line infection), she was pooping chalk white- her bile ducts stopped working, I have not seen her so sick in years. She was in for 5 days and was gradually able to start eating again but we dealt with poop misery for weeks trying new meds, etc.
We finally got things the best they have been in over a year on a combination of Miralax & benefiber in Gatorade by tube daily, Actigall, and Amitiza- a chloride channel activator. She's continued with a lot of respiratory infection off and on and a CT last week at her clinic appt showed onset of significant sinus disease for the first time. She has an appt with her ENT in a couple weeks for a scope/exam to see what needs to be done.
Then all hell broke loose with Shawn. We've known all along he was a cf carrier and he has horrible asthma, actually has worse lung function than his sister. we thought we'd ruled out cf with a neg NPD. His asthma dr finally insisted on a pulmonary consult due to lack of control. Our 'consult' turned into a 90min appt picking apart his hx and reopening the cf can of worms. Then a week later he got sick. Really, really sick- all 3 kids caught it, Tim and even Emily got over it fairly quickly but he just never did.
We are approaching 7 weeks later and he is STILL not better. An asthma consult turned into multiple ped and pulmonary sick appts, sputum culture which showed a heavy growth of staph. Consecutive courses of zithromax, Augmentin, bactrim and starting the levaquin- and another culture still showed staph, and he is still sick. The levaquin and high doses of prednisone have helped the most. So we will see what happens when he finishes them. He is having a chest CT and then a bronch. He's been dx'ed with CRMS and cf is under consideration pending future cultures, how well his pft's respond to tx, and results of the CT and bronch, etc. Right now he is doing albuterol 4x a day along with Acapella and symbicort, and we wait and see for the rest of this testing. The last couple months have been so hard. He's been so sick and trying to push through it to go to school- falling asleep in class, he's lost so much weight, it's just so hard. And I never saw this coming, NEVER.
So... A lot going on here! Sorry so long- just wanted to update and let you know where I've been. <img src="i/expressions/face-icon-small-smile.gif" border="0"> hopefully things will settle down.
 

hmw

New member
I know I kind of disappeared for a few months and I want to update everyone on how things have been going here. I have missed everyone and hope to be back here again.
Emily got very sick the week of that awful end of October snowstorm. We weren't able to take care of her as we should and I know that didn't help. She was sick through all of November, despite a couple oral abx and ended up inpatient on IV's as well as cleanout for partial obstruction. She was on home IV's for barely a week when she ended up back inpatient with a horrendous GI thing. Everything shut down, she had a high fever (which of course also had them worrying about a line infection), she was pooping chalk white- her bile ducts stopped working, I have not seen her so sick in years. She was in for 5 days and was gradually able to start eating again but we dealt with poop misery for weeks trying new meds, etc.
We finally got things the best they have been in over a year on a combination of Miralax & benefiber in Gatorade by tube daily, Actigall, and Amitiza- a chloride channel activator. She's continued with a lot of respiratory infection off and on and a CT last week at her clinic appt showed onset of significant sinus disease for the first time. She has an appt with her ENT in a couple weeks for a scope/exam to see what needs to be done.
Then all hell broke loose with Shawn. We've known all along he was a cf carrier and he has horrible asthma, actually has worse lung function than his sister. we thought we'd ruled out cf with a neg NPD. His asthma dr finally insisted on a pulmonary consult due to lack of control. Our 'consult' turned into a 90min appt picking apart his hx and reopening the cf can of worms. Then a week later he got sick. Really, really sick- all 3 kids caught it, Tim and even Emily got over it fairly quickly but he just never did.
We are approaching 7 weeks later and he is STILL not better. An asthma consult turned into multiple ped and pulmonary sick appts, sputum culture which showed a heavy growth of staph. Consecutive courses of zithromax, Augmentin, bactrim and starting the levaquin- and another culture still showed staph, and he is still sick. The levaquin and high doses of prednisone have helped the most. So we will see what happens when he finishes them. He is having a chest CT and then a bronch. He's been dx'ed with CRMS and cf is under consideration pending future cultures, how well his pft's respond to tx, and results of the CT and bronch, etc. Right now he is doing albuterol 4x a day along with Acapella and symbicort, and we wait and see for the rest of this testing. The last couple months have been so hard. He's been so sick and trying to push through it to go to school- falling asleep in class, he's lost so much weight, it's just so hard. And I never saw this coming, NEVER.
So... A lot going on here! Sorry so long- just wanted to update and let you know where I've been. <img src="i/expressions/face-icon-small-smile.gif" border="0"> hopefully things will settle down.
 

Beccamom

New member
Harriett,

I am soooo sorry to hear about Shawn. You have been so supportive of me as we are over a year now in Cf limbo even after two NPDs. We did go to UNC as part of the mucus clearance consortium to try to figure out what is going on. She had a NO test which was low and mucus clearance disorders PCD and CF are low and asthma is twice that of normal. The doctors are positive now she does not have asthma. That doctor completely agreed that my daughter has "A" mucus clearing disorder and we are still in limbo as to which CF or PCD. Meanwhile, she has been treated for CF for a year and her lung function Jan 2011 was FEV1 55% and Jan 2012 was FEV1 100%. She is using a vest, hypertonic saline, and IV antibiotics and she is a completely new person.

I hope they figure out what is going on with Shawn and he can experience the improvement my daughter has had.

PM me if you want to learn more about our University of North Carlolina experience.
 

Beccamom

New member
Harriett,

I am soooo sorry to hear about Shawn. You have been so supportive of me as we are over a year now in Cf limbo even after two NPDs. We did go to UNC as part of the mucus clearance consortium to try to figure out what is going on. She had a NO test which was low and mucus clearance disorders PCD and CF are low and asthma is twice that of normal. The doctors are positive now she does not have asthma. That doctor completely agreed that my daughter has "A" mucus clearing disorder and we are still in limbo as to which CF or PCD. Meanwhile, she has been treated for CF for a year and her lung function Jan 2011 was FEV1 55% and Jan 2012 was FEV1 100%. She is using a vest, hypertonic saline, and IV antibiotics and she is a completely new person.

I hope they figure out what is going on with Shawn and he can experience the improvement my daughter has had.

PM me if you want to learn more about our University of North Carlolina experience.
 

hmw

New member
We do know with Shawn that simply treating for asthma did little to help his lung function- we'd been doing that for about 15mos with little change when finally we had to see pulmonary. Really - I should have taken him sooner and I feel terrible that I didn't, but I thought we could get a handle on it and that we'd ruled out anything even close to cf related! He is certainly being treated more aggressively now than he has ever been before and this has resulted in pft's higher than we have EVER seen before- now just to see if he can maintain them once off the prednisone and Levaquin and start getting some energy back and weight back on.

That NO test sounds really interesting. I wonder how it would work for people with CF that also have a strong inflammation/asthma component. We know with Emily, by both her symptoms and her pft's, for example, that her small airways are very much affected and she needs a daily inhaled steroid in addition to more CF specific tx. Her pft's increased more in response to adding pulmozyme than they did with anything else though.
 

hmw

New member
We do know with Shawn that simply treating for asthma did little to help his lung function- we'd been doing that for about 15mos with little change when finally we had to see pulmonary. Really - I should have taken him sooner and I feel terrible that I didn't, but I thought we could get a handle on it and that we'd ruled out anything even close to cf related! He is certainly being treated more aggressively now than he has ever been before and this has resulted in pft's higher than we have EVER seen before- now just to see if he can maintain them once off the prednisone and Levaquin and start getting some energy back and weight back on.

That NO test sounds really interesting. I wonder how it would work for people with CF that also have a strong inflammation/asthma component. We know with Emily, by both her symptoms and her pft's, for example, that her small airways are very much affected and she needs a daily inhaled steroid in addition to more CF specific tx. Her pft's increased more in response to adding pulmozyme than they did with anything else though.
 

Rebjane

Super Moderator
Hoping things improve. You are such a good mom; it's so hard when you are doing everything you can and things still aren't where they should be.
 

Rebjane

Super Moderator
Hoping things improve. You are such a good mom; it's so hard when you are doing everything you can and things still aren't where they should be.
 

Beccamom

New member
As for the NO being affected by inflammation you are correct it would alter the numbers. For this reason PFTs are done the same day so if inflammation was present it would be considered as part of the overall picture. Also NO is a screening tool and not a diagnostic tool. It just helps the looked like a stopper attched to a hose to a computer. It just helps the doctor head in the right direction. It is a very simple breath test that checks air coming out of the nose. No pain and really short test.

It is great his PFTs are going up.
Best wishes
 

Beccamom

New member
As for the NO being affected by inflammation you are correct it would alter the numbers. For this reason PFTs are done the same day so if inflammation was present it would be considered as part of the overall picture. Also NO is a screening tool and not a diagnostic tool. It just helps the looked like a stopper attched to a hose to a computer. It just helps the doctor head in the right direction. It is a very simple breath test that checks air coming out of the nose. No pain and really short test.

It is great his PFTs are going up.
Best wishes
 

scrapper1264

New member
I do not post here a lot but just had to say how sorry I am all this is happening. You are so helpful to everyone and we appreciate it. You are a great Mom and prayers are definitely coming your way.
 

scrapper1264

New member
I do not post here a lot but just had to say how sorry I am all this is happening. You are so helpful to everyone and we appreciate it. You are a great Mom and prayers are definitely coming your way.
 
oh Harriet, hang in there. So sorry you're going through craziness. The GI thing really stinks. My kiddo's GI dr had us increase the amitiza dosage, doubled his dose of nexium and the thing that helped the most was adding a cyproheptadine tablet each night. It's usually used for appetite increasing, but we've not used it for years. A side effect of the cyprohep. is that it relaxes the GI tract. You may want to ask if it could help your kiddo.

Thanks for updating, hope all calms down for all the kids and you too.
 
oh Harriet, hang in there. So sorry you're going through craziness. The GI thing really stinks. My kiddo's GI dr had us increase the amitiza dosage, doubled his dose of nexium and the thing that helped the most was adding a cyproheptadine tablet each night. It's usually used for appetite increasing, but we've not used it for years. A side effect of the cyprohep. is that it relaxes the GI tract. You may want to ask if it could help your kiddo.

Thanks for updating, hope all calms down for all the kids and you too.
 
Top