Thursday, 22 September 2011

Dr. T and the Baby Ethan Project

Daddy took the day off and joined mommy and me for my appointment with Dr. Tarnopolsky at Mac. We got underway with plenty of time to spare, unfortunately enroute I decided to have my morning poopies. I was thus changed in the trunk of our volkswagen just off of highway 6.

Soon we arrived at Dr. Taropolsky's office where we sat down to talk about my progress. Dr. T took a few moments to assess me, played with my arms, checked my reflexes and my head control. He said my reflexes were good and my head control was better. He'd planned to set up testing for my nuclear DNA however the funding takes 7 months for approval so he had cancelled this and went with the mtDNA test instead. The results are not back as of yet, but should be within the next couple of weeks.

Dr. T. believes that I show symptoms of reversible COX deficiency.  As my preliminary mitochondria test showed that I am only 8% efficient in the 4th stage.  He informed us that any one that is that severe should die and not stabilize like I have. However, this particular phenomenon has only ever been recorded twice in the history of medicine. Dr. T informed us that I will likely undergo another muscle biopsy in a years time to see if the percentage has increased.

Mommy asked about the glycogen that was found in my muscle, he said that it isn't unheard of that when the body starts to shut down it starts to store glycogen so it would make sense that there was an increased amount in me. He also mentioned that my symptoms are not consistent with glycogen storage disease. He increased my medicine volumes to coincide with my weight gain. He has taken me off Vitamin C (however my parents think they will keep me on it as it will be good for my immune system). He has also eliminated riboflavin as it is really only good for complex 1. Many of you that have been following me from the beginning know of my turbulent relationship with this vitamin and I am overjoyed to be rid of it!  However, he has added vitamin E to my mix, and mommy tells me that it tastes worse than the riboflavin, which she didn't think could ever be possible.

Dr. T cautioned my parents about my weight gain, he is concerned that because I am considered low tone it will be harder for me to move my muscles. This is in direct conflict with my metabolics team who want me to pack on the pounds.

We then decided to visit some old friends at the general peds ward. We ran into my old OT Kate, who told us that she will get in touch with my current speech therapist about trying me on 5mls of formula from a bottle.  She thinks its absolutely crazy to keep putting 0.01mls in my mouth... it won't get me too far. We saw our resident from the ICU, Serengy, and my nurses Deb and Janine who were all surprised to see me.

We could not leave until we visited the genetics department so I could see Mariya! She was happy to see me and couldn't wait for a cuddle!  She informed us that my skin biopsy results were not back yet.  However, the lab had found something interesting in the skin cells, they could not release the results to her yet as apparently they do not understand the results as they don't make sense.... They are not sure if it is due to how the skin cells were cultured or if it is in fact my cells.  They are going to hold another meeting and decide what the results mean and possibly ask for another skin biopsy.  We should know either way by the time we go to her clinic. Mariya does not believe that I have a mitochondrial disorder and mommy just thinks that I could be an X-Man.

We decided to proceed on to the Welland Staples store to see my friends Dayna and Nicola.  They were very excited to meet me in the flesh and had a big present full of soft blankets and stuffies for me.  They along with Tish have been working on "The Baby Ethan Project".  The project will invite Staples stores to run dress down days for each weekend in October.  The purpose of the days will be to spread my story throughout the Staples family and to collect some money to help support my medications, syringes, and other hospital costs.  I can't say thank you enough for their efforts, it truly is overwhelming to have their support.

We stayed in the store and played in the copy centre for an hour or so until it was time to eat.  We enjoyed the comforts of the store lunchroom and met some more new friends before hopping back in the car and high-tailing it home. 

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