Sunday, December 9, 2012

Off the reservation

When Mighty Z was born I went through all the stages of grief Shock, Denial, Pain, Guilt, Anger, Bargaining, Depression, Reflection, Loneliness, Acceptance, and Hope. All these stages took their toll on me in one way or another.


I knew there was no cure for Congenital Central Hypoventilation Syndrome and because of the effects the mutations has on neuro-development. Simply put, Mighty Z's ANS neurons aren't where they are supposed to be, and this loss of communication causes many of the symptoms of the disease.


However even armed with that knowledge I still knew that Mighty Z would be a trailblazer. The problem with being a trailblazer, as I have found is that it is a hard road to walk. Not only are you trying to push the world out of their box with trying new things, you are also trying to push your own community of people with children who have the same disease out of their box as well. It all goes back to the fact once you become comfortable in your situation you do not want your cheese moved (although you might say you do)


I have always been off the reservation with Mighty Z never wanting to stay stagnant always wanting to move. I had no problem picking up and moving to Las Vegas for a doctor never mind the fact that my husband was still working in Oklahoma, When we decided on the breathing pacers I was all for Mighty Z being the only baby (at the time) with them implanted, in hopes that it would give Mighty Z a slim chance of having a more typical life. So when it came around that the French found a drug that might make Mighty Z’s life better I jumped on it.


Will this medication cure Mighty Z? No, but it does help her through sickness. Before the medication a simple cold could throw Mighty Z for a loop and land her back to being 24 hour dependent on her life support machines and of course a nastier bug such as strep would send her back to the PICU. Recently Mighty Z caught a respiratory form of strep and she was able to handle it with ease.


Mighty Z recently had a sleep study to confirm if the drug was working. Even though the sleep study report is still being read this is what was found to be an overview.  Last time Mighty Z tanked within the first 10 minutes of being off her machines with a CO2 of 80 and oxygen at 75%. After the drug she maintained her CO2 at an average of 44 and oxygen 95% or greater for 4 hours. She did rise to a CO2 56% and low oxygen 80% for a couple of seconds. Blood gas this morning 19.  Mighty Z has never in her whole life been able to sleep off of life support machines for longer than 10 min. It looks like the drug may be working.


Mighty Z is the world’s youngest child and the only person in the United States to try this drug, and you know what? In just three months it has already made her life even easier. Trailblazer is what we are and I am so proud and thankful for the opportunity to be part of Mighty Z’s little life no matter how hard being a trailblazer is.     


I don't think anyone knows what the future holds for CCHS. I know for a fact that even the thought of a pill that could help any part of CCHS was a pipe dream 11 years ago. So I think we should all try to always move forward in trying to find ways to help our children.
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