Pam,
My ds is very similiar. He has had severe sinus problems dating back to age 6 months. He also has asthma (dating back to the same point). He really doesn't have bowel problems either but is very small for his age. He grew well for the first several months but once he started getting sick, he quickly fell from the top of the chart to the bottom which is where he is now (7 years). He had his first sinus surgery shortly after turning age 4 (after over a year of trying to clear it.) Within weeks, his sinuses were bad again. He also had a very low negative sweat test at a hospital that has an accredited satelite cf clinic that was trained by the children's hospital that the cf clinic is based in. At the request of our ENT, we did genetic testing through genzyme (he treats many cf patients and said ds's sinuses looked like a cfer's to him.) His genzyme panel came up completely negative - no genes found. It finds approx 90% of the mutations. We could follow up with an Ambry panel but at this point, ALL specialists have said they do not think ds has cf (pulmonologist, allergist, 2 seperate ENTs.) All this just basically to say that I totally agree with everything everyone else says. For your own piece of mind, request the Ambry genetics screen. However, it is very possible to have extremely severe sinus problems and not have cf. Has your ds had an in depth study of his immune system? ITs basically a series of blood tests that take several months. Has he been allergy tested? Our ds originally tested neg for everything (allergies, immune problems, etc.) We later found out his prevnar vaccine did not take so ds was vaccinated with the pneumovax and retested - it took. He also originally tested neg for all allergies but at age 5 was found to have many allergies and is now on allergy shots. Does your ds have a lot of ear infections?? The last test that has been recommended to us is a biopsy of his cilia to check for primary cilia dyskinesia (sp?) It's also a genetic disease that requires the person to inherit a gene from each parents. The effects are very similiar to cf but the disease is very different. Children with PCD have lots of ear, sinus, and can have lung problems. Basically, the cilia does not work correctly to sweep out debris from the sinuses, lungs, etc so it stagnates and becomes infected. Treatment is very similiar although prognosis is generally a little better. THis is the last test our ds needs. Our allergist pushed for it but the ENT has to do it under general anesthesia. He prefers to wait until ds's next sinus surgery. We (with 2 ENT opinions) have decided to wait until ds's symptoms can no longer be managed to do the surgery since the last surgery did not last for very long. Finally, how are they treating your ds? To help manage symptoms, ours is on a nasal steroid spray (nasonex) daily, daily nasal washes, zyrtec for the allergies (although antihistamines should be used sparingly since they can actually CAUSE the sinus infection). He's also on singulair and advair for the asthma plus frequent oral antibiotics and/or oral steroids. I hope that helps. Good luck in your quest for answers. Be prepared that you may find none. All of our specialists have said they think ds probably just has really bad sinuses and we'll probably never find an answer WHY. Not exactly the answer we wanted but an answer of sorts I guess. Good luck!
