I can't even think of a title...

SO here goes. We spent the majority of the day at Duke Children's today, meeting with a pulmonologist, getting lung function tests, meeting with the pulmonologist again, having a chest CT scan.

I wish I could say that we bypassed the new diagnosis we were dreading, but no such luck. Kennedy was officially diagnosed with severe restrictive lung disease. Symptoms have been exacerbated due to the worsening scoliosis, but her rib cage is the main factor causing this. A normal lung function is 80 or above. Kennedy's overall lung function was in the 30s today. The one caveat to that is that we don't know what Kennedy's normal baseline was, before she started to show symptoms. It could be that even though her lungs are normally clear (minus illnesses) and that her pulse ox is usually great (minus illnesses), that her normal function has been in the 50s for years. We just don't know. But no matter what, 30s is not good. How she is functioning in normal everyday life as well as she is is beyond anyone's guess.

So, will spinal fusion fix her? Bottom line is no one knows. It might help lung function. It might not help lung function. But what it will do is put her rib cage back in a better place which will help stabilize her lungs and keep it from getting any worse. Right now, her lung on the left is being squeezed and twisted due to the shifting of the spine which is pulling the rib cage with it. So does she need spinal fusion? Absolutely. And soon. This has all happened so fast and we do not want it to progress any further and put her in any more danger.

Kennedy will also be going for an exercise study and a sleep study within the next few weeks. The immediate needs that she might have based on these tests will be restrictions in the realm of physical exercise, and the possibility of needing a BIPAP for sleeping. We're praying we're not at this point yet. These tests will also give him a better perspective of how to handle her before, during, and after surgery as he will now be involved and trying to prevent any issues before they even arise. Although he has cleared her for the surgery, surgery on someone with lung function that low isn't ideal. The possible need for keeping her intubated after surgery is strong at this point.

So some questions we're left with:

- Will her CT show anything else that we're missing? It could, but the pulmonologist doesn't think so. It was done after our other appointments but he will call if there is anything else we need to know as a result of it.

- Will her MRI of her spine and brain scheduled for Friday show anything else we need to know about? We don't know and we won't know till next week. It's main function is to give the orthopedic surgeon a better look at her spine and how it is currently functioning. He wants to make sure we're not missing anything related to the spine. We've added the brain to rule out anything unusual there that could be causing her headaches, which we hope are just from the scoliosis and the lack of lung function.

- What does this mean for the future? There is no good way to fix the rib cage so this is a disease she will live with from here on. We hope that the spinal fusion will help stabilize and keep it from getting worse. If the sleep study shows sleeping issues because of this, then a BIPAP will be introduced. This is the first step in breathing assistance for those with restrictive lung disease. There will always be a chance for much more intense breathing intervention as she gets older, if the disease continues to progress. Will it progress? Great question with no certain answer. It will always be there. It might get worse, it might not. Only time will tell. As of this point, every cold, sinus infection, flu, and especially pneumonia becomes more dangerous than it ever has been. Think about how hard it is for you to breathe at times with just a cold and most of you have normal lung function.

- What does this mean for her new-found passion of scuba diving? The answer is still out on this one as well. We're on hold now until after the spinal fusion. The doctor will then perform more lung function tests to determine if she's improving, is stable, or is getting worse. The good news is he didn't immediately say no way, nuh uh. He is open to allowing her to continue depending on future lung function tests, especially given that she has been diving with no lung issues (only sinus) for the last year. And given that our awesome dive instructors are okay with his assessment. When we talked about this over lunch, she teared up but being the mature person she has always had to be...said that as much as she wants to do it, it isn't worth dying from. Of course, she's hoping for the best possible outcome on this question and to swim with the sharks soon.

This is one storm that will never pass, so it's a good thing we have learned to dance in the rain.