Is this Cystic Fibrosis, Asthma, or some other Pulmonary Disorder

[smart_squared]

I have been researching information on CF and was wondering if it's possible to have the disease without the normally associated GI problems. My 19 mo old nephew has been sickly from almost the day he was born with recurring ear and bronchial infections, RSV, and pneumonia. Although he doesn't seem to cough up mucus, his chest is almost always congested and he breathes with a wheeze/rumble sound and vibration. His pediatrician has had him on almost constant antibiotics and nebulizing steroid treatments with only short term improvement. Although CF doesn't run in our family, I think it should be looked into. The pedi has poo-poo'd an inquiry saying Cole would have GI problems and diahrea (CFF says stools are usually "bulky") if he had CF. They are now trying to get my nephew well enough to put tubes in his ears and his pedi thinks this will cure all. The problem is that when they get him well enough via antibiotics and steroids, then they can't seem to schedule him in for 1-2 weeks. By then he has regressed back to his congestive pulmonary condition and they won't do the surgery. What's the relationship between his ears and his lungs? Your archives mention bronchiectasis. Is this common in kids without CF?  Or, as the pedi has also suggested, could this be the beginnings of childhood "asthma" (doesn't run in our family but both parents have allergies)? I think my sister needs to press her pedi regarding the whole pulmonary and/or CF problem (and get a pedi who'll be a true advocate for Cole's health). Am I an overbearing aunt or does my sister need a kick in the butt?

[National Jewish]

Cystic fibrosis (CF) is a genetic disease of the exocrine glands affecting the gastrointestinal and respiratory systems.  It is possible to have CF without having gastrointestinal (GI) problems.  A child may have CF even though it “doesn’t run in his family".  The diagnosis of CF can be made by a "Sweat test" and also by genetic testing.

Bronchiectasis is a disorder of the airways.  Inflammation and infections create areas along the airways where mucus collects.  This can lead to recurring infections.  Bronchiectasis is common with CF.  It can also occur without CF.

Recurring infections are the most common problems with immune deficiencies.  These commonly include sinus infections and chest infections like bronchitis and pneumonia.  Blood tests will reveal if there are any cells lacking from a complete immune system.  Blood tests will also show if the cells are present but lack adequate amounts for the system to function effectively.

[RTFLY]

I certainly don't think you are being overbearing. I have limited experience with CF as I work with adults. I teach critical care for adults as well, so my neonate/pediatric experience is little. What I do know is that I agree with you in that I don't think it is out of the question to have your nephew tested for CF. I am guessing that your sister is a little more passive then you are? I, too, am aggressive when it comes to quality care and taking care of loved ones. Maybe you should seek out another physician who will be more kin to your suggestions or testing requests.

As for your question about bronchiectasis. I would be hesitant to think that this would be the issue. Generally, bronchiectasis may be something we would see in patients with a history of CF, or chronic lung disease, someone older than 19 months. There are also GI issues that you had mentioned. It doesn't sound as if your nephew is presenting with those specific to CF, although increased frequency is one of those manifestations. The recurrent pneumonias is a "red flag" but again, your poor little nephew may just be a sickly baby! A sweat test is the current standard in CF testing to define if one has CF, that I am aware of. The Nat'l Jewish Center nurse that hosts this forum will certainly have much more info on this as well as CF in general. I am sorry to hear of your nephews short but troubling history. I certainly wish him well. Good luck and keep us posted if you wish.

                                                JCI, RRT RCP

[nurse12hr]

I am sure the forum moderator will correct me if I am wrong, but I believe there are mild forms of CF.  I have seen (work as a nurse) about 2 or 3 adults with mild CF.  

Interesting question--let us know what you find out!

[RTFLY]

I agree with you that there are mild SYMPTOMS of CF and these progressive complications associated with CF will vary widely from patient to patient. Playing devil's advocate,  I would be hesitant to call CF mild in any form. Recent progress in CF research is making a big difference in the lives of those who suffer this disease. The nurse that hosts this forum, I'm sure, is an excellent resource to acknowledge such advancements and improvements in care. I look forward to hearing more about the above individual as well. Take care...
                
                                              JCI, BS RRT

[Concerned lady]

I agree with the others. You're doing the right thing in pushing your sister to get better answers and help for the baby.

Call the LUNG LINE nurse, at 1-800-222-LUNG(5864) between 8am & 4:30pm, Colorado time, Mon-Fri, to get names of good docs in your area, who trained at Nat'l Jewish.

Such doctors may include pediatric pulmonlogists (lung docs), allergists (could refer you to pulmo's, etc.), pediatric gastro-enterologists (in case the baby has Pediatric GERD, alone, or in addition to other problems), etc.

Where are you, and what's your email? Perhaps people could recommend docs they know are good, in your area.

I hope the baby is seen and helped very soon.

[smart_squared]

My sister is in the north Dallas area.  If anyone knows of a good pediatrician or pediatric pulmonologist in this area (she's willing to drive), please post it here.  We all would greatly appreciate it.

[Concerned lady]

Please go to a helpful website called PAGER (Pediatric and Adolescent Gastro-Esophageal Reflux) Association.

ASK YOUR QUESTION ABOUT WHO IS A GOOD DOC (Pediatrician & Pediatric Gastro doc) in North Dallas.

Here's a "link" to their website:

<a href=http://www.reflux.org>http://www.reflux.org</a>

Also, ask a LUNG LINE nurse if she can recommend a good doc in the North Dallas area to you. Call the LUNG LINE at 1-800-222-LUNG(5864), and then press "5" to speak with a lung line nurse. Call Friday, between 8am & 4:30pm Colorado time.

Ask the LUNG LINE nurse for advice, too!

Sincerely, Concerned lady
http://cantbreathesuspectvcd.com

[Concerned lady]

Email me at ***@**** and I'll email you several names of medical professionals in the Dallas area, who can recommend good pediatric & pediatric gastro doctors to you, for your nephew!

Concerned lady
http://cantbreathesuspectvcd.com

[Concerned lady]

Sorry, I meant to say Pediatric Pulmonologists (not just Gastro docs).

[asabio623]

ALTHOUGH YOU MAY NOT SEE CF IN YOUR FAMILY, CF IS A RECESSIVE TRAIT AND COULD BE PASSED ON FROM GENERATION TO GENERATION WITHOU SHOWING ITSELF. IT IS MORE COMMON IN CAUCASIANS. THE PROBLEM WITH CF IS THAT THE LUNG SECRETE TONS OF MUCUS, AND PATIENT BECOME HOSPITALIZED FOR IT MANY TIMES A YEAR FOR INFECTIONS.