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.
