Cloey does not have, or did not have Chiari. She has some old brain damage to her brain stem from the congenital malformation of her skull and spine, but the damage is not signifigant to her airway issues and is rebuilding neuro pathways at this point. This is HUGE because it means she is NOT on an inevitable decline to a pacemaker, colostomy bag, 24hr ventilator, etc.
The next big issue was her autonomic dysfunction. The sleep disorder specialist/neurologist, autonomic dysfunction specialist (also a neurologist), the endocrinologist, the pulmonologist and the POTS specialist, worked hard together all week to sort out her symptoms. I mean, besides visits, they had several meetings together. Based on testing and a recent study out of Stanford University (the sleep doctor consulted his colleague over there) It was determined that the majority of her problems are a result of her spinal fusion. Cloey's entire cervical spine was fused at age 5 to prevent paralysis. It was necessary and she would be paralyzed from the beck down if we hadn't done it. When they fuse the neck they don't do it completely straight, they fuse it a little bit forward to make life skills such as eating and using a computer more possible, unfortunately this study out of Stanford has shown that in some cases (particularly where the patients anatomy is narrow, like Cloey's) This increases pressure on the airway, and putting a trach in and using a vent, take away even more of the space making things worse. Let me clairify, even though we didn't know this when the Trach was placed or when we placed her on the vent, it wouldn't have mattered. She needed those things because she was not able to breath adequately on her own, and as it turned out, They bought her 6 years to grow and open up more space in her airway. Her sleep study from 6 years ago before trach and vent showed 70 apneas in an hour. This week, with the trach capped off and no vent or oxygen itshowed 7. Miracle. The patients in this study behave like quadrapaligics, meaning while they may have avoided complete paralysis, some side effects are still evident. For Cloey, the fusion combined with her genetically small airway are causing her lack of bowel and bladder control, her weak intestines, her central apnea (brain not telling you to breath) and her irregular heart rate. The good thing is this will stay the same or get better as she grows. It is also the missing piece of the puzzle as to why she got BETTER on hospice without the vent. It also explains some of her symptoms that they thought might be POTS. The conclusion is that she does have some issues with her autonomic system, specifically orthostatic tachycardia (basically low blood pressure) but does not have POTS. We were given recommendations to add water and salt to her diet, and also begin an exercise routine. Again, these symptoms are also expected to stay the same or get better as she grows.
Than was ENT, we had a scope done on Wed, and everything looked good, not great but good enough to remove her Trach! It was done in the hospital and she stayed overnight in the PICU for observation and did great. The plan is to use prilosec, flonase and singular to protect her airway from irritation and inflammation, also to watch her weight diligently because she can not afford anything else to cause airway obstruction. Then we can use oxygen as needed. There is a chance this won't work and she will have to be re-trached. We won't really know for six months or so and will be doing scopes and sleep studies at home to monitor her during this time. If it doesn't work, we may be looking at a trip back up here in a few months to re-evaluate. But, she is doing really well and we are all hopeful this will "stick"
We also completely redid her diet for adequate adolescent nutrition, and only 1/2 time tube feedings! There is whisperings that the reflux medication and removing her trach could increase her appetite too, meaning a possibility of no g-tube in the future. Guess we are so used to tings snowballing in the negative direction it is hard to comprehend that they can snowball the other way too :)
Endocrine evaluated her as well, and ran some precautionary tests that we will get, and could be managed at home. She mostly looked and things to rule anything else out and agreed with the yeam on the source of her symptoms.
I think that is everything...
All of this is an absolute miracle, one of her Doctor's said "No one expected her to be 11 and doing so well" I am sure this is the direct result of her super awesome medical team, hard work, service, sacrifice and prayer that she has received. We could not be more full of Gratitude. I was literally jumping and clapping my hands when we knew she would have her trach out. I can't wait to see her quality of life and independance blossom. My brain looks at her and can't believe this is possible, but when my heart looks at her I see that baby in the NICU and say "I knew it"