Just what ARE mitochondria anyway???

My son, Oliver, is one year old and is under investigation to determine what mitochondrial disorder he has, if he does indeed have a mitochondrial disorder. A couple of months ago he had a muscle biopsy. The surgeon removed two grams of tissue from his right thigh for testing. When the results were in we were told two things. One, that his muscle fibers were not bundling properly. A muscle is made of two types of fibers, conveniently labeled type one and type two. If you were to look at a cross section of a muscle sample you should see the two fibers equally distributed, so at a 50% mix. Oliver’s fibers are bundling at 60%/40%. This is somewhat mild. The second thing we were told was that everything else was at normal levels, but they wanted to do one last test. Why? I asked (later, of course, through a series of phone messages between myself and the R.N.) should we do one more test if everything was mostly normal? I finally got a response back from the geneticists R.N. explaining that while the mitochondrial activity levels were normal, he had more mitochondria than they would expect to find.

Mitochondria, hmmm, I remember making a cell cookie for a middle school biology project once, and I recall that we used some type of candy, jelly beans I think, to represent the mitochondria, but that’s about all that I remember. This post is to rediscover mitochondria and why they’re so important.

What are mitochondria? Google provided me with this succinct description: mitochondrion – A spherical or ovoid organelle found in the cytoplasm of eukaryotic cells, contains genetic material separate from that of the host; it is responsible for the conversion of food to usable energy in the form of ATP.

Mitochondria are the body’s powerhouses, the batteries if you will. Interestingly, mitochondria have their own DNA separate from that of the individual they reside inside of. Mitochondria were once a parasitic bacteria that has degraded over time until now its an integral part of our bodies and is passed on from mother to child in the egg. Even more fascinating, mitochondrial DNA can trace the maternal geneology back fifty thousand years and has been used to prove theories, such as we are descended from Neanderthals, false.

Facts:

Mitochondria contain 2 types of genetic material:
• mitochondrial DNA, which can only be passed on from the mother
• nuclear DNA, which is passed on from both parents

Autosomal recessive inheritance
The nuclear DNA in mitochondria is inherited from both parents (half from
each parent). Mitochondrial disease can be passed on only if BOTH the mother and
father are “carriers”. This means that they carry the mutated gene, but not the
disease – so they don’t have any symptoms. This is called autosomal recessive
inheritance.
When both parents are carriers, there is:
• a 25% chance of having a child with the disease
• a 50% chance of having a child who is a carrier like the parents
(has the mutation, but not the disease)
• a 25% chance of having a child that is not a carrier and does not have
the disease

http://www.hamiltonhealthsciences.ca/documents/Patient%20Education/MitochondrialDiseaseUnderstanding-lw.pdf

While I read many articles, Mitochondrial DNA and Human History from the Human Genome website (http://genome.wellcome.ac.uk/doc_WTD020876.html) was the simplest to digest.

But back to my original questions, what are mitochondria and how can I better understand mitochondrial disorders? Mitochondria reproduce faster than other human cells because they do not have a system check to identify mutated DNA. Because they don’t check, they reproduce faster, twenty times faster than other cells. One mutated mitochondria reproduces twenty times in X amount of time, and is why some mitochondrial issues do not manifest until adolescents or adulthood. It takes that many years for the original few defective mitochondria to reproduce to the level that they can impact their host.

What does this mean for Oliver? I have no idea, but it is a fascinating subject. If they’d told me in middle school biology that mitochondria are actually the remnants of an ancient parasitic bacteria that we now use to power our bodies I may have gone into genetics. Why do they keep all the cool stuff a secret?

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Kindergarten!

We made a conscious decision to not make a big deal out of the approaching First Day of School, yet even so, last night when I asked Hazel if she was nervous at all I was still surprised at her answer. “Nervous about what, mom?” Well SCHOOL for one! I laughed and said never mind and tucked her in. This morning, however, she popped out of bed shining and happy and excited – Today I Go To School! was her morning greeting to me.

So, y’know those moments in the movies where the moms are kissing their kids at school and the kids are like, “get off me, mom!” Well, in case you were wondering, kids really DO shove you off when you get to kissy at school in the morning! Hazel, my oldest and dearest, started her first day of kindergarten this morning. I was so excited for her while we waited in the gym for them to call for lineup. Then they actually DID call for lineup and it hit me, my baby was about to be taken from me, from my control, and I wasn’t going to be allowed to participate in her day any more. I realized she was walking off without her lunchbox and called her name. When she came back for it I scooped her up in a big hug, kissing her sweet little face and told her I loved her so much! She said, “OK MOM, GOOD BYE!” and squirmed out of my grasp to run to her spot in line behind her BFF Avery. (Luckily she had three friends she’d known for two years or so going to kindergarten in the same school. It might have been different had she not known anyone.)

Poor little Walter, he wanted to go so badly! I had to call him back out of the line. I reminded him he wasn’t quite old enough, to which her responded with a healthy stomping of feet, “YES I HAM!” He has a funny way of talking sometimes. There was a coffee social after all the kids had cleared out and we stayed for coffee and donuts. Walter started crying at one point, pining for his Hazy already. Tomorrow is his birthday. These last two weeks have been overwhelming with NEW things. Husband new job, Hazel new school, Walter turned three, Oliver turned one, Auntie moving to New York, New York… *sigh*

Well, its done. She’s in school for better or for worse, and now I just can’t forget to go pick her up!

This Ain’t My Grandpa’s Zucchini Bread!

My clearest memory of my grandparents’ house is the smell. My grandfather was a bread baker in his retirement, and he made everything from sourdough bread to brownies from scratch. My grandmother had been a Home Ec teacher and between the two of them the house was always refreshingly clean and full of amazing smells. My grandmother, years ago, put together a family cookbook, and I’m eternally greatful. I can bring back some of my happiest memories by simply reading over the recipes. A couple of years ago I decided to make my grandfather’s zucchini bread from the family cookbook, and I was SHOCKED at the amount of sugar in it. His recipe called for THREE CUPS OF SUGAR. No wonder we loved it so much! I played with the recipe a bit and after a while perfected my version. Enjoy!

3 eggs

1/2 cup oil

1/4 cup applesauce

1/4 cup crushed pineapple

1 3/4 cup sugar

2 cup grated raw zucchini

2t cinnamon

1t baking soda

1.5 t baking powder

3 cups unsifted flour

1 cup nut pieces

1 t salt

Grate unpeeled zucchini and set aside. Beat eggs until foamy, gradually adding oil and sugar. Add zucchini, cinnamon, salt, baking soda, and baking powder. mix well with an electric mixer. Gradually blend in flour, then fold in the nuts. Grease a loaf or Bundt pan and fill 3/4 full of batter. Bake at 350 for one hour.

*This recipe is also delicious with a quarter cup of flax meal mixed in, and I love using roasted and salted pecans as my nut of choice! I always make a couple sans nuts for my son.

A five year old tries to tell a three year old a knock knock joke…

hazel: “walter, knock knock…” walter: “what?” hazel: “say, who’s there.” walter: “umm… chicken.” hazel: “no, walter, say who’s there?” walter: “ummmm. … . . chicken!” hazel: “no walter, i’m telling the joke, you say, who’s there? walter: “knock knock” hazel: “who’s there? wait! no, you say who’s there. knock knoc…”

can you guess who’s five and who’s three?

My son, Oliver.

My son, Oliver, was unanticipated. My husband and I had decided to wait to have our third child another year, but the joke was on us. Even as we made that decision little Oliver was growing in my belly – we’d already conceived! The pregnancy was my healthiest to date, completely uneventful. At my 38 week checkup I asked the P.A. to “sweep the membranes” as my first two pregnancies had had to be induced. I wanted to help my body initiate labor as much as possible. Each pelvic exam I’d had previously had shown that Oliver was head down – but when the P.A. swept the membranes we discovered he was actually breach, and that when they thought they were feeling the sutures of the head they were actually feeling his sweet, baby bum.

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Painting Hazel’s Room

My mom recently repainted her room, and the first can of paint she brought home was too “purple.” So she gave it to us! We’re so excited to be making a big change. Hazel started helping me paint two days ago. We hope to move on to a mural once the walls are finished, but its the little steps we’re focusing on for now.

Little Oliver has turned ONE! Happy Birthday!

One year ago, Oliver was born via c-section. My third and most problematic child is also the sweetest, cutest little thing you’ve ever seen. His special needs have kept him on the baby side of life, and while he is progressing, toddler hood is still a ways away for us and I am unbelievably thankful for the extra moments of baby cuddles and love.

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