Im tired!

Of living in my own hell.... i know i have done it to myself... now there are several ways that i could stop living in my hell and i have thought about all of them... but at the end of the day there is only one that is the right choice for me and that is change it. instead of looking at it like hell i need to look at it totally differently! EASIER said then done right?????  Yeah i just seem to be in this black hole in my head and no matter how much i talk about it i just cant seem to get out of it and it is driving me nuts!!!! But i have not been completly honest with those i talk to... becasue i cant seem to be honest with me... god idk what i want to say im just going to go ill be back when im back

I am 1 in 150,000

Spinocerebellar ataxia 3 

Spinocerebellar ataxia (SCA) is a progressive, degenerative,^[1] genetic disease with multiple types, each of which could be considered a disease in its own right. An estimated 150,000 people in the United States are diagnosed with Ataxia, SCA's are the largest group of this hereditary, progressive, degenerative and often fatal neurodegenerative disorders. There is no known effective treatment or cure. Ataxia can affect anyone of any age. It is caused by either a recessive or dominant gene. Many times people are not aware that they carry the ataxia gene until they have children who begin to show signs of having the disorder.^[2]

 

SCA3[6] (MJD) (ATXN3)

4th decade (10–70)

10 years (1–20)

Also called Machado-Joseph disease (MJD)[7] Gaze-evoked nystagmus (a rapid, involuntary, oscillatory motion of the eyeball) upper motor neuron slow saccades

Azores (Portugal)

CAG repeat, 14q

 

^{'Machado–Joseph disease' (MJD) or Spinocerebellar ataxia type 3 (SCA3) is a rare autosomal, dominantly inherited neurodegenerative disease that causes progressive cerebellar ataxia,[1] which results in a lack of muscle control and coordination of the upper and lower extremities.[2] The symptoms are caused by a genetic mutation that results in an expansion of abnormal "CAG" trinucleotide repeats in the ATXN3 gene [1] that results in degeneration of cells in the hindbrain.[2] Some symptoms, such as clumsiness and rigidity, make MJD commonly mistaken for drunkenness and/or Parkinson's disease.}

 

The disease is caused by a mutation in the ATXN3 gene, which is located on chromosome 14q. The gene contains lengthy irregular repetitions of the code "CAG", producing a mutated protein called ataxin-3. (Normally, the number of copies is between 13 and 41.)^[7] MJD is an autosomal dominant disease, meaning that if either parent gives the defective gene to a child, the child will show symptoms of the disease. Therefore, if one parent suffers from this disease and the other parent does not, then if they decide to have children, there will be a 50% chance of their child inheriting the disease.^[2]
The pons (a structure located on the brain stem) is one of the areas affected by MJD. The striatum (a brain area connected to balance and movement) is also affected by this disease, which could explain both of the main motor problems cause by MJD: the tightening and twisting of the limb and the abrupt, irregular movements.^[8]
In affected cells, this protein builds up and assembles intranuclear inclusion bodies. These insoluble aggregates are hypothesized to interfere with the normal activity of the nucleus and induce the cell to degenerate and die.^{[citation needed]}

The hereditary ataxias are categorized by mode of inheritance and causative gene or chromosomal locus. The hereditary ataxias can be inherited in an autosomal dominant, autosomal recessive, or X-linked manner.

• Many types of autosomal dominant cerebellar ataxias are now known for which specific genetic information is available. Synonyms for autosomal dominant cerebellar ataxias (ADCA) used prior to the current understanding of the molecular genetics were Marie's ataxia, inherited olivopontocerebellar atrophy, cerebello-olivary atrophy, or the more generic term "spinocerebellar degeneration." (Spinocerebellar degeneration is a rare inherited neurological disorder of the central nervous system characterized by the slow degeneration of certain areas of the brain. There are three forms of spinocerebellar degeneration: Types 1, 2, 3. Symptoms begin during adulthood.)

• There are five typical autosomal recessive disorders in which ataxia is a prominent feature: Friedreich ataxia, ataxia-telangiectasia, ataxia with vitamin E deficiency, ataxia with oculomotor apraxia (AOA), spastic ataxia. Disorder subdivisions: Friedreich's ataxia, Spinocerebellar ataxia, Ataxia telangiectasia, Vasomotor ataxia, Vestibulocerebellar, Ataxiadynamia, Ataxiophemia, Olivopontocerebellar atrophy, and Charcot-Marie-Tooth disease.

• There have been reported cases where a polyglutamine expansion may lengthen when passed down, which often can result in an earlier age-of-onset and a more severe disease phenotype for individuals who inherit the disease allele. This falls under the category of genetic anticipation.

MJD can be diagnosed by recognizing the symptoms of the disease and by taking a family history. Physicians ask patients questions about the kind of symptoms relatives with the disease had, the progression and harshness of symptoms, and the ages of onset in family members.
Presymptomatic diagnosis of MJD can be made with a genetic test.^[9] The direct detection of the generic mutation responsible for MJD has been available since 1995.^[10] Genetic testing looks at the number of CAG repeats within the coding region of the MJD/ATXN3 gene on chromosome 14. The test will show positive for MJD if this region contains 61-87 repeats, as opposed to the 12-44 repeats found in healthy individuals. A limitation to this test is that if the number of CAG repeats in an individual being tested falls between the healthy and pathogenic ranges (45-60 repeats), then the test cannot predict whether an individual will have MJD symptoms.<sup>[9]



 So this is me and my fate! This is what my father, grandfather and grandmother died from! 









http://en.wikipedia.org/wiki/Spinocerebellar_ataxia
 http://en.wikipedia.org/wiki/Machado-Joseph_disease</sup> 

 

BEEN really SICK!!!

Well this last cold knocked me right on my butt for about 6 days!!! I got nothing done other then school and managing the boys!! I havent done much of the 30 day shred well im only on day 7... i think i restarted day one like 3 or so times!!! So nothing to report there... NOTHING to report on the job front which is killing me!!! My ex is still late on child support but im getting things handled on Monday!  So that will help!! IDK i have been fighting with my mom and TON and i am losing the positive out look and motivation i had so much of a few weeks ago!! THank goodness i see my therapist on Friday!!! IDK I just need to keep writing which i have not and i need to get further along with this 30 day shred!!! GAH ok i will write more later!!

30 Day Shred!

So I know I haven't written all weekend because I have been really sick!!!! BLAH kind of put the working out and losing weight at a halt... well not all together.... I am 2 tenths of a pound from being in the 170's again I am SOOOO EXCITED!!! Bringing my weight loss to 16lbs I cant wait until i say 26lbs by April!! Man i am feeling more confident every day!! My MOM even made a comment about noticing my weight loss.... So that being said I have decided thanks to my friends on My Fitness Pal i am going to do Jillian Micheal's 30 day shred!! I have looked at the YouTube results and a few people my size have lost up to 19'' on this thing!! And dropped two dress sizes!! I WOULD LOVE to be in an 8 or even a 6 again god what it would do to my self esteem!!!! So today I started day 1 and boy was it harder then I thought!! But Im SOOOO EXCITED!!! ONLY 29 days left!!! SO since you change levels every 10 days im going to do measurments every 10 days starting today!! And yes pictures BLAH I hate pictures of my body!!!  Anyway here we go!!!

Measurements

Shoulders-17"
Bicep-14.5"
Neck-15"
Bust-41"
Back-38"
Waist-36"
Hips-43"
Thigh-25"
Calf-16"

Ok I hurt!!

This whole working out and dieting thing is killing me!! Not really its actually REALLY good for me but im so sore i can barely move!!!  But im happy.... lets see here i need to look back at my old blog to see when i started this weight loss journey because im thrilled!! i start officially dieting again 5 weeks ago but tracking my weight!! I have lost 14.6 lbs in 5 weeks!! Which means i am right on track for my Vegas goal!! Might even be a little below!!! I AM FREAKING OUT EXCITED!!! Now if i could just help my friend who has helped me SO much find her motivation!! I dont know how losing 50 something pounds so far isnt motivation but everyone is different!! I am so proud of her and thankful to her at the same time!!!! I LOVE her like a sister and i am so so so so so so so so glad we became friends again!! I guess it was just meant to be..... or in the cards... now lets find he motivation button and we will all be happy!! Anyway im am starting to feel like no one can tell me what to do anymore!! I am so tired of being shot down... its quite refreshing to not be so hard on myself its just hard to not want to punch the person in the face that put me there to begin with!  a few days ago my grandfather looked me in the face and said i was a "waste of resources money and air"  WOW did that hurt!!!! All because i cant balance a full time job full time school and full time motherhood single!!!  God sometimes its hard to remember he is old and becoming MEANER with his age because it is a dagger through the heart!!!!  It makes it harder and harder and harder to live with him when all his goal is to put me DOWN!!!! HELL whatever i am so burnt!! But im not going to let this shit get me down!!!! Alright its bed time i need to rest my sore aching body goodnight no one!!!


R


MY THEME SONG!!!

TOO MUCH!!!

This is not going to be a long post because i have too much to post and i dont want to get into it all right now... shit my chest and left shoulder blade hurt enough for the night!! well i guess its morning at this point but w.e.... Long story short and NASTY stupid shit aside i think change is coming my way and when i say change i mean a good change but i have to get a job first and make some more positive strides towards a better happier life!! i just have to say i couldnt have done it without my friends right now!! I owe my life to you at this point because i think i might have taken mine by now if you werent there!!! so thank you!!! ok im going to bed because this is sooooo scattered and not making a whole lot of sense.... until tonight....


R

What a weekend!

I don't even know where to start.... this weekend has been an interesting one that is for sure!!!!  It started on Friday my oldest son had been sick all week and i planned on picking him up but instead his dad sends me a text an hour before he is dropping him off... so that ruined my plans because i didn't have a sitter and i had an apt @ 10 so instead of taking one boy i had to take both... i was really worried because this apt didn't allow me to sit and watch them and they couldn't participate in what i was doing so they had to sit in the waiting room....  anyway at my apt i was told i didn't need to keep going to these apts because i have surpassed the program but i have to see what my doc wants me to do so we will see there!!!  Then i went and took my oldest to get his haircut he was 3 weeks past due and his dad finally said i could... then i get a phone call from my mom that my grandpa went down in my house aka our basement and was digging around looking for stuff... now mind you i had a sick kid all week another one i was trying to keep out of the sick so i had sheets off of beds and laundry in piles in the living room and i hadn't done my dishes from the night before so basically it look like it was a total mess.... although i had it under control...... so i get this phone call my grandpa is on the war path and to be careful when i get home....  so i decide i am not going into the house threw the front im gonna go around back... well thats fine i spent the next few hours cleaning and getting everything in line.... i dont let the kids go upstairs like i normally do and i stay downstairs the whole time... i dont even go up to see my mom.... then around 7:30 I hear the basement door open and my grandma screams "if you dont clean that basment you find yourself a new home!  You fucking lazy shit" This being done without calling me up and in FRONT of my children!!! WOW!!!! First off over react much!!!! ANd second they dont own the house they arent even on the title my mom is so legally they cant do squat to me!! HAHAHAH poo on them.... well that sealed my fate for the weekend i decided i was going to keep the boys away and i was going to stay away and not say a damn word to them ALLLLLL weekend...... Well that same night i went upstairs after my grandparents went to sleep to talk to my mom... my therapy apts came up while talking about my grandparents and their goal to always knock me down!! I am not exaggerating that its their life goal to make me feel like shit they have done it ever since i was little little... anyway that's beside the point im not going to sit here and blame them for my issues although they MIGHT be a little piece of how i view myself now.... who the hell knows all i know is it feels like an excuse.... I am trying to live without excuses you can see how that is going so far....so anyway that night my mom and i had a heart to heart i finally admitted how much i hate myself and most of the choices i have made since i quit my sport... and i emphasize MOST i dont regret my kids at all or ever will.... but there are still choices i have made that i hate and i admit i HATE myself... i hate mostly what i stand for i hate what i have done with my life i hate who i am plain and simple. im not good enough pretty enough strong enough or even smart enough im not a good friend mother daughter or even sister.... im angry about this im extremely angry and i dont even know where to start to fix this because it has been burned into my brain for years and i finally believe it so i have to learn how to rewire my brain.... i started and new mood stabilizer but its going to take a few weeks before i know if it helps me get out of my blue funk and on my way to rewiring.... in the mean time FINALLY talking about my issues and not my issues with others is slowly making me feel better.... god i have been so good and have slowed down on seeing my therapist and calling her but god i wanted to call her sooooo bad this weekend... but i was a good girl and didn't... so Saturday i took the boys to the hardware store and they got to build monster trucks and had a BLAST!!! Then we decided to go to the McDonald play place which wasn't the best of ideas for my diet since i hadnt really eaten in the last two days...... i have been doing pretty good on my diet since i started recording my food again thanks to a friend nudging me and telling me its good for me.... im up to a 10.9 lb weight loss putting me at 185.1 only 15.1 lbs to go before April 10th i hope this keeps up because according to MFP i should be down to 169.8 in the next five weeks which would be KILLER!!!! so anyway back to the weekend... i have never felt so isolated in my entire life.... yes my choice but i couldn't handle the abuse warranted or not and i KNOW this time it is FAR from warranted... But thankfully its Sunday night and tomorrow starts a new week.... only other issue is we got like over a foot of snow which could shut a few things down or at least delay them tomorrow... and we are supposed to get dumped on again on Tuesday so this could make for an interesting week.... also i have a second interview on Tuesday with the hospital so fingers crossed on that one..... and then i have my oldest son again next weekend because his dad is going out of town which i don't mind but then i am going two weekends in a row only seeing him a total of 4 days out of 14 that is going to torture.... And i almost forgot i got a letter saying they are taking away my help for paying for my younger sons daycare but they didn't give me an explanation and only a week to figure it out.... so fingers crossed i can get that taken care of this week because he loves his daycare and i really don't want to have to take him out because the state is stupid!!!! GAH i want to be a kid again some days.... im just ranting and making excuses now.... but i cant help it..... on the other hand i had this brilliant idea about how my friend and i should get the same exercise DVDs and Skype each other while doing them because we both have the excuse we hate working out alone.... and her schedule is unpredictable and so is mine and we live about 35-40 min away which makes planning things a tiny bit harder.... she thought i was going completely crazy but I think im brilliant.... we could also make a MFP group and start working out like a class without a gym!!! I LOVE IT but it probably is really stupid.... anyway im going to leave a random song at the end of this terribly LONG ass rant.... see yall tomorrow


R


http://youtu.be/40dxDSXsrL8