Hey everyone!
     
     Thanks to all the people who have viewed me in the last 2 days! You guys rock :D and whoever is reading this right now, you rock too! Thank you :)


     I am going to start this off today with a little more details about Deanna Bear since she is my total inspiration for this blog. Even though she may look 'normal', underneath all that beauty and smiles there is some serious stuff going on. She has a mitochondrial disease, wolfe parkinson white syndrome, heart murmer, and needs to be on a strict ketogenic diet 1:1 ratio. It is really hard sometimes to explain to people that she is actually not the 100% healthy girl that she looks like!


    So i bet your wondering what exactly is mitochondrial disease, well I was trying to think of a simple way to explain it, but I am just going to give you the definition from the United Mitochondrial Disease Foundation(UMDF):
    Mitochondrial disease refers to any illness resulting from deficiency of any mitochondria-located protein which is involved in energy metabolism. Thus, deficiencies of the respiratory (electron transport) chain, either resulting from deficiency in one or more mitochondrial or nuclear-encoded proteins, are mitochondrial disorders. Also, by this definition, disorders of fatty acid (beta) oxidation, Krebs cycle and pyruvate dehydrogenase complex deficiency are mitochondrial disorders. Although the proteins involved are nuclear-encoded, they are located in the mitochondria and are involved in energy metabolism. Although genetically dissimilar, all of these disorders share clinical similarities in that they result in an energy deficient state. What I always tell people when they ask is 'her body has problems converting carbohydrates to energy, which is why she is on a high fat diet'. Which leads me into my next explanation, the ketogenic diet. 
      The ketogenic diet is actually quite simple on a 1:1 ratio, what is a ketogenic diet and what is that ratio all about? Well, let me explain! The ketogenic diet is normally used at a much higher ratio for people with epilepsy. There have been many known cases of it helping to stop seizures when no meds would work. Deannas ketogenic diet is also referred to as a 'modified atkins diet'. The ratio is referring to fat:carbs+protein. So for example say a hot dog has 9grams of fat, 2 grams of carbs and 7 grams protein that would be a balanced meal. Also she is not restricted on calories like people who are on it for epilepsy! So she can eat her little heart out with balanced food all day! Which makes me happy :), And if she desperately wants something that isn't 'balanced' then comes in the whipping cream(35%).. I'm weak! I can't say no all the time! I want her to enjoy being a kid, and am thankful for her everyday! Even the hectic stressful days where I just feel like crawling up into a ball! lol I'm not sure how much more I can say on the ketogenic diet, but if anyone is reading this who is on the ketogenic diet or knows someone who is I will be posting recipes very soon!
    Now onto the Wolfe Parkinson White syndrome(WPW)! This is the thing that freaks me out the most, because her heartrate has the potential to shoot up rapidly and I am powerless to help her(other than rushing to the hospital! which would be a nightmare all in itself where we are living right now!).  Wolff-Parkinson-White syndrome is a condition in which a particular type of abnormal, extra electrical pathway between your heart's upper chambers (atria) and lower chambers (ventricles) causes a rapid heartbeat (tachycardia). Not much more to that either, pretty straightforward. And thankfully she hasn't had any episodes with this that I am aware of yet, but there is always the potential which has me on edge most days when she is out running and screaming and having fun! Ah well, can't live everyday in fear, life is to damn short for that!


   In my next post I will be posting recipes, and more details on all of us. I just wanted to give everyone some background info on our little angel! Thank you all for taking time to read this! :)