So what does this mean? Well, Cloey was due for several maintenance consultations and procedures 2 years ago when we started the whole Hospice journey, so now all the things we put off need to be addressed. We also need to determine if she is truly "all better", whether by miracle, medicine or simply outgrowing her breathing (or lack of breathing) condition. Or, if she is just declining more slowly than we had anticipated. Figuring this out is VERY important. It's the difference between taking her off of her AND (allow natural death, formally DNR do not resuscitate) order and putting our efforts into teaching her life skills for her *gasp* future, or continuing to focus primarily on her quality of life and making her comfortable. Her doctors and specialists in Phoenix are not sure what to do with her. They are good doctor's but are simply not equipped with the multidisciplinary model of care or knowledge and medical advancements to manage a disorder never seen before in the world. To complicate matters, our sweet girl is also becoming a young lady. This means changes in her dietary needs for her feeding tube, and her hormone levels need to be evaluated. Not to mention, no one expected her to reach this point, so no one knows what these changes will mean for her or how they will affect her delicate system.
First, we need to determine if Cloey is a "Chiari kid". Basically, Chiari malformation is a congenital condition where part of your brain protrudes into the spinal column at the base of your skull, this condition is often seen in Spina Bifida. When Cloey was 2 1/2, on our first trip to Mayo, it was discovered that she had some stenosis (pinching) in her spinal cord at the base of her skull just under her brain stem. She had a decompression surgery done, where they took away some bone at the base of her skull and the first 2 vertebrae to relieve the pressure on her spinal cord. She did great and everything looked fine. The neurosurgeon explained that the procedure was the same they do for Chiari malformation, but that she did not have it. 2 years later on a follow up scan, it looked as though her spinal cord was at risk for damage again, especially because she was now walking and more active. Because her entire spinal cord has low fluid the only option was to fuse her entire cervical spine and eliminate all movement in her neck, otherwise further damage would leave her paralized from the neck down. The fusion was done at Barrow by one of the best surgeons in the nation and went very well, with no complications. Fast forward to July 2010, we took her to Cincinnati Children's hospital to sort out her breathing issues. The doctor reviewed her history and evaluated her. He said he felt very strongly that she had had Chiari, all of her symptoms align with damage to her brain stem where her autonomic functions are controlled. Things such as bladder control, breathing and heart rate regulation, eye dilation, digestion, everything your body is supposed to do automatically without thinking about it. She lines up almost perfectly to a "Chiari kid" symptom wise. Unfortunately, the fate that comes with this particular malformation is an inevitable pacemaker, colostomy bag and 24 vent dependence, among other things. I called her neurosurgeon that did the original decompression and basically said "but she doesn't have Chiari, right?" his heart was obviously heavy as he shared with me that he technically couldn't say she had Chiari because it is medically defined as brain tissue protruding more than 5mm, and hers was not quite 5. He went on to share with me that, when he did the surgery her brain (at the brain stem) looked like a bruised peach. He said he believed that damage lined up with her symptoms as well, making her unofficially have Chiari malformation. This is when and why we decided to put her on Hospice. But the decline didn't happen the way it should have if she had had a true Chiari malformation at birth, so we need to determine if the brain damage has repaired itself, basically if she "outgrew" the autonomic problems, or if we are still looking at Chiari, she is just stronger than most ;)
We will also be seeing-
- Dietitian and GI to determine adequite nutrition and the possibility of future g-tube removal.
- Urology to discuss managing her incontinence.
- Orthopedic surgeon, to evaluate her growth and abnormal bone structure.
- ENT to check her ears and evaluate her Tracheostomy
- Sleep disorder specialist to determine if she is still not breathing on her own when she sleeps
- Endocrine to evaluate her growth and hormone levels
- Neurology to determine the extent of her brain damage