So much has changed, but really nothing has...

At the request of our pediatrician, Jeff and I took Rylee to Chapel Hill on Friday, May 3rd for an appointment with the top pediatric pulmonary doctors in North Carolina. This winter has by far been the worst season for Rylee ever.  She constantly has what seems like a sinus infection and eventually drains into her lungs, causing pneumonia.   With the last bout of pneumonia wreaking so much havoc, taking a month to get rid of and conflicting reports on the structure of her cilia, her pediatrician thought it was best to take her to Chapel Hill.  After a 2.5 hour appointment, the head doctor thinks it's about a 5% chance that Rylee really does have PCD. The ultra-structure of her cilia are normal and her nitrous oxide result was within normal range. She said she can't say with 100% certainty that Rylee does not have PCD. Diagnosing is still a complicated task and it is possible that Rylee could have a variant that is not yet able to be diagnosed.
So while we all think this is great news, I'm having a bit of trouble. At least with a diagnosis, I knew what I was up against and Rylee was getting the best medical treatment she's ever received. It is a possibility that she still can develop bronchiectasis, a permanent widening of her airways. With each repeated lung infection, especially in the same area of the lung, bronchiectasis becomes a greater possibility. With this appointment, other than taking away the diagnosis, nothing changes for Rylee. The head doctor at Chapel Hill is in total agreement with all of Rylee's doctors that the Vest is necessary for her mucociliary clearance on a daily basis, and may be for the rest of her life. She still suffers severe infection and constantly seems to have a sinus infection and a cough. It is a high probability that she can still get atelectasis, that could possibly result in another bronchoscopy.
We're still in limbo with the infectious disease specialist. Rylee received the adult pneumonia vaccine, Pneumovax, in February 2012. Checking immune response to certain viruses is how they test for immunodeficiencies. Her titers responded wonderfully in March 2012. Her titers were checked again this past March and she responded to less than 50% of the strains they checked for. Not the results an infectious disease specialist would like to see, especially in a child who has received the polysaccharide vaccine, Pneumovax. The vaccine should last at least 5 years and should have made her respond to well over 50%. With that being said, he ID specialist was not ready to start her on immunoglobulin therapy. It could mean life-long shots, giving her immune system that push it needs to help fight infection. A few weeks ago, she received the Pneumovax vaccine again. Her levels were checked last week, but we won't receive results until next Friday. If she responds as she did the first time, she'll be checked again in 5 months. If she again responds to less than 50%, we will begin talking about treatment plans. The main goal will be to cut down on the infections and the severity of them.
We appreciate the support everyone has given us. These past 6 months have been quite crazy and stressful, to say the least. Diagnosis or no diagnosis, we will continue to fight for Rylee and the medical care that she needs and is entitled to. 
So, for now, we continue on, just as we have been the past 6 months or so, daily nebulizer and vest treatments and keeping her sinuses and lungs clear of gunk.  After just finishing 4 weeks of antibiotics spread over 6 weeks, Rylee went off to school today with a squealing nose and junk in her throat.  But just as she always does, she didn't complain at all and continues to smile and sing!