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! :)

Well let me start off here by introducing myself. I'm Mary(aka Mama Bear) my awesome counterpart Craig(aka Dado) and my two little angels with horns! Deanna Bear(whom this blog is going to be centered around..) and Brenya(aka BamBam). There will be many pictures to follow in the coming days, just getting the feel for this whole sharing my life through blogging idea. I am really looking forward to building a strong audience to share my crazy ideas with and to raise awareness about Mito kids, and how we are coping. I am also planning to put up some recipes in the future for the ketogenic diet(will go into more detail in later posts as to what this is). Anywho, back to a day in our crazy hectic life. Lastnight rocked, allow me to share with you the awesomeness! lol So some nights i have some pretty bad insomnia, and lastnight was no exception. I was just getting ready to try and settle down for the night around 2am and whatta you know I hear screaming from the kids bedroom. Not like normal I'm thirsty kind of screaming, but bloody murder come and save me mama kind of screaming. So me being me, I rush upstairs to make sure no one has busted into their room and is trying to make the 2 story jump to flee with them(wild imagination. lol). Thankfully, they were there just wide awake and terrified. Found Deanna under her sisters bed crying and BamBam just wanted to get in on the screaming I think. So anyway, gullible me I brought them down to comfort them and assured myself they would be back in bed within the hour. Hah! was I wrong, no matter how much I cuddled coerced and fed them the warm milk they were just being stubborn little ladies and there was nothing I could do about it. So long story short I have pretty much been up all night They did not doze off until 6am and I may have dozed from around 7-8 when they woke up for the day. So it was a very long night needless to say, ah well atleast I got some cuddles out of the deal.  And now I will leave you with a photo introduction to my 3 true loves. have a good day people. Come back often and check me out! I will only get better at this, so be patient! <3