Questions about pft

[threecutekiddos]

Hello,
My son has not been offiically been diagnosed with c.f. . He has a appt. with a c.f. clinic with his sister in two weeks. My question is on all most all of his pfts for the past couple years says Early obstructive pulmonary impairment and a couple when they where really low said moderate to severe ventilatory defect. But why dose the first one i spelled out say earliest stages of emphysema he is only 8 years old and has never been around any type of smokein his life! I don't smoke or his father? Any Ideas thanks ,kathy

 

[anonymous]

Hi - Emphesema is caused 80% of the time by exposure to smoke. It can be evident at earlier ages and not related to smoke. It is a condition of the many air sacs not acting properly. It is probably more of a description of his pft results. Hope this helps. If you type the word into your web browser, there are some good web sites with better descriptions of Emphesema itself. Hope this helps. Jo Ann

 

[luke]

kathy,

Other than smoking there is a rare genetic disorder alpha 1antitrypsin deficiency, more rare than CF, that also causes emphysema. Even with this disorder it takes a while for the "lung" portion of the disease to manifest, I haven't seen anyone diagnosed younger than 30. Please keep in mind that there are different diseases that cause obstrutive defects, CF and Emphysema would be considered the more severe of the 5. You also have Asthma, Bronchitis and bronchiectasis. In a child as young as yours you can probably remove emphysema. As for bronchiestasis, children can develop this one but it is normally secondary to another disease such as CF of ciliary motility issues, not always but normally. So probably one of the others would be the most likely. Please keep in mind that if it is CF is not the death sentence it used to be considered, not to mention we all are smarter and better looking, ask around!

I will keep you in my prayers.. if you have any question just repost


luke bailey, MBA RRT 29/cf

 

[threecutekiddos]

Thanks !! You made me laugh already this morning. I do not think they will get a c.f. diagnosis. We have already had sweat test and a 26 or something genetic test that was negative . But the Doctor said it is possible for them to have a rare gene. My son is very sick and has been since he was born despite the fact he has always taken asthma medicine treatments three times a day last year or two with duaneb advair 500 those little times he is not on steroids and he has built up a resitince to a lot of antibotics for his chronic sinus disease. When i picked up his pft from the time of age 4 to 8.5 years it said emphyesima the asthma Doc. never mentioned it to me. He also thinks my kids just have hard to manage severe asthma. But this pulmonary Doc I talked to thinks something is wrong and its worth looking in to. Hannah just has failuer to thrive with mild asthma. O.k. I Know no body asked my life story I am grasping at straws . We go to Robert Wood Johnson Hospital has a C.F. clinic on the 19th They gave a list of test they are going to perform . Thanks, kathy

 

[2sickkids]

What are ciliary motility issues? I have severe asthma chronic bronchitis broniectasis. I should know the answer to that question but right now it is not clicking.

 

[luke]

Part of the lung defense system are cilia. Basically, they are hair like structures that move out infection in a wave like fashion. When they do not work properly the infection just sits there. This happens for several reasons, the most common is smoking but various other things including genetic disorders can cause it as well. The treatment is much similar to CF, chest pt and positioning to clear out secretions. good luck with your tests.


luke

 

[2sickkids]

Well now I remember thanks. Sorry sick right now so my head is fussy. Having a hard time thinking clearly.