Negative sweat test but lots of mucus

[Janae Warren]

I have two mutations and an Intermediate sweat test and I am diagnosed and treated for CF. Has your child been tested for PCD, Primary Ciliary Dyskinesia?

I'd never even heard of PCD till you asked. I've been researching it. They are sending her to a pulmonologist soon. I'm so nervous about going but really hope we find some answers soon.

 

[lauryn.tubes]

lauryn.tubes...if your daughter has two CF mutations - is she not already on a CF regime? Maybe I did not understand your statement? My son only has shown the digestive symptoms so far - and he does CPT three times a day and is on all kinds of nebulized lung meds plus kalydeco.

My daughter has DF508 and then F1052v which is defined within CFTR2 as "variable consequences". According to a California study it causes CF in 33% of the patients who have it, and I believe at least 50% is required to be classified as disease causing. My daughter is vulnerable to a more mild form of CF or atypical Cystic Fibrosis. But, of course, no one guarantees us anything

 

[Printer]

lauryn.tubes:

As a CF Patient who went mis-diagnosed for 47 years, I would strongly suggest that you find a more aggressive CF Clinic to care for your DD. Like your DD, I have NEVER had a positive sweat test.

Bill

 

[Janae Warren]

lauryn.tubes:

As a CF Patient who went mis-diagnosed for 47 years, I would strongly suggest that you find a more aggressive CF Clinic to care for your DD. Like your DD, I have NEVER had a positive sweat test.

Bill

Are your tests borderline? My daughter's test was around 10 on each arm,which of course is very low. The pulmonologist made me feel like I was obsessive mom last week. "When you hear hooves, Don't go looking for zebras." he said. Really upset me and made me start questioning myself. But my husband and family members have also seen what our daughter looks like and they all agree it is not normal. The pulmonologist said he thinks the gunk she coughs up all the time is from past nasal drip and he is going to put her on 3 weeks of broad spectrum antibiotics. He said he thinks it's a sinus infection. I've had MRI and shows I have chronic sinusitis and post nasal drip - and I cough from it, but it ain't nothing like what is daughter looks and sounds like. It will be great if it's really just a horse ? not a zebra.

 

[Ratatosk]

To simplify things... You're the customer, you're paying for these appointments to be told you're over reacting. Ask for testing, if the doctor refuses, the tell him you'd like that written in the file that parent requested culture, certain tests and he refused because....

Basic fact of the matter is there's something wrong with your child, you'd like answers. As for the broad spectrum abx, I guess I'd ask for a culture to see what's growing in the sputum or mucus sample and see what antibiotics it's sensitive to. If she's culturing pseudomonas and they're using a regular run of the mill abx, it probably won't do any good. Years ago I took our then 2 month old to the doctor with a horrible productive cough and was told by the doctor "they cough, it's what they do" -- meaning people wcf. At the time he was on an antibiotic (Keflex); however, he ended up having bronchitis caused by enterbactor choclae -- a bug common in NICUs and needed two completely different antibiotics to clear up the infection.

 

[Printer]

Janae:

If one never looks for zebras, even if they are present, they will never be found. Get another Doctor, you don't need to put up with that attitude.

I went 47 years before I found a Doctor willing to look for a Zebra (CF).

Bill

 

[Ank]

Hi
You are right you should suspect cf ,have you done cough culture to check which bacteria is causing infection.
Asthma and cf are diff.
cfers produce lot of sticky moucus ..get genetic test done.

 

[lauryn.tubes]

Printer - thanks for that comment. We have clinic on Wednesday and I do think I'm going to request we start doing the vest at least. My daughter started to cough after she would run around. We did CPTs for two days and it drastically improved. I thought that it was too good and too quick to be attributed to CF so we started to lay of. Cough has started to creep back. I appreciate your point of view so much...I don't want her to look back and say "why didn't you help me?!". It's such a hard spot to be in.

 

[Ank]

Ya u need to do CPT.Its v imp to do cpt till u get diagnosed..Inhaling albuterol & H Saline clears cough
Infact u will have to learn cpt techniques lik acbt and autogenic drainage.. To maintain healthy lung.It has to be done twice a day

 

[Printer]

lauryn:

I know that this sounds strange but her cough is her friend. It is a way for her to clear her lungs. Let her cough, not to the point of vomiting but a strong cough is good.

Bill