Revisiting Ethan

Happy Valentines Day!

It's been an up and down, bumpy road for my oral feedings. However, it seems like my swallowing issues are almost nil. Clinically I look fine when I swallow, it's just making my swallow team at Mac believe this without subjecting me to another icky Barium Swallow Test.

I have started to take my sippy cup again over the past few days. I must mention that we have modified the top so the hole is bigger so my formula just rolls into my mouth, as I don't suck. It seems to be working great! I take between 3-4 ounces of formula orally at meal time. I am trying to work up to a full meal which is 7 ounces. I still take about 3 tablespoons of sweet potato for a morning snack, in the afternoon I am more picky and usually take only 3 infant size spoons of banana. Mommy tricked me and got me to eat apples mixed with bananas yesterday, I ate it... We'll see if I do today!

My Kidsability appointments with my PT and OT will be changed to biweekly. At my last appointment, I had better spine position sitting (I wasn't so Quasimodo). However, I have shoulder hiking! Man, I can't catch a break! Shoulder hiking, is exactly what it sounds like. Instead of my rotator cuff rotating to reach up for an object while the shoulder stays stationary, I raise my shoulder with my arm to reach up, the PT says it's because my arm is so heavy for me. As you guys know my shoulders are so weak. Also it was pointed out that my elbows hyperextend when I put pressure on them, because my arms are so weak. Moreso my left as I have been using my right to spin around in circles on my bum. Talking about that, I was content with that for all of maybe a day, now I am as frustrated as ever that I can't move straight!

On that note, I took my first plunge off the kitchen counter this past weekend. My daddy has been working long hours at the office, so I guess he hasn't seen me at my finest on my bum. He turned around for a few seconds to get my formula, and found me on the ground.... Yes folks we have CERAMIC floors in the kitchen. Mommy came running downstairs once she heard daddy yell OMG! She found me on the floor screaming! I had fallen on my side. Daddy picked me up, mommy moved my arms and legs, and checked my head. Everything looked fine, and I am happy to report that I am very pliable!

My mommy has been trying to create a new friendship with another mommy in our library group. She has a son named Theo and he was born on May 3rd! Which happens to be my Grandpy's birthday. We had our first playdate outside of the group and the mommies hit it off, but I of course wasn't making it so easy! Theo is crawling, I am not. Theo kept coming over to me and grabbing my arm wanting to play. I turned so my back was facing everyone and proceeded to try to ignore this child and played with some toys... Theo would make a loud noise and I would cry at him. Like head poke cry! I was in total distress! Do I sound like anyone we know yet? Has to be uncle Brian. Anyway we will try again despite my antics.

Barkeep please KEEP the other round!

Well, I had just reported on how well I was doing with food on my last post. So this post will be telling you that I am now having a hateful relationship with food.

I should also mention that I had a fever of 101.5 this past Saturday night, but no other symptoms. My fever broke Sunday morning, however for the last couple days I have just wanted my soother and mommy to carry me around. Lucky her! She was always complaining that I never wanted to cuddle.. well she got her wish this week! I had a clinical assessment with my speech therapists, I of course didn't want to eat anything again today. My therapist said that that they often see kids who are just learning to take food by mouth go through phases where they are not feeling 100%, start to reject food, and it takes awhile for them to start accepting it again. So be patient and keep offering food to me, however read my signs and I need to feel in control. So mommy was right! The therapists suggested giving me something called stix or crunchies, apparently kids usually start with these before mastering cheerios because they dissolve faster, so we'll try those, because they have different flavours.

I had my second appointment at Kidsability with my OT and PT. My OT says I am doing well, I have progressed from raking to pinching and palming lower in my hand. I can take objects out and put them back in, bang them together and hold my hands. This is all age appropriate!

My PT has been working on my transitions (getting into sitting, and from sitting to standing, and back down again). She has also been helping me correct my sitting, as I occasionally have a posterior sitting position, so she occasionally has to use a small wedge under my bum. I have been working on knee exercises to strengthen my hip extensors and hopefully that will help me with my four point (on hands and knees) as right now gravity is working against me!
I have learned to pivot on my right arm while sitting so right now all I can do is go around in circles, but it makes me a little happier to be moving a bit!

I had a checkup with my pediatrician this past Monday.
weight: 20.5 lbs
length: 29 inches
My weight is down to the 50th%, which makes everyone happy, this is where I should be because of my low muscle tone. She says I am progressing well, and I am such a cutie!

I have been overly cranky this week, and the only thing that has made me happy is when I found out that Katy Perry and Russell Brand are divorcing... meaning Katy Perry can be mine again!

Barkeep, another round please!

Hello everyone!

There are so many things to update you guys on. I have been neglectful in my duties as host of this blog! I must apologize!

We left off starting with my adventures with carrot purée. Well to say it didn't go well would be an understatement. The result was I did not eat anything orally for a full week!  Mommy freaked and started giving me Cheerios instead, I love them so much! I was then tricked back into eating my oat cereal. From there banana purée and then sweet potato purée, and rice risks. Mommy has been given the okay to introduce a third purée but she's scared!  My sippy cup is going well. I have been taking about 3 ounces in a sitting for the last couple days.

How am I dealing with so much food all the time! My parents have dropped another feeding, so now I get formula 4 times a day and snacks of 2-3 teaspoons of purée in between. I eat sooooo much! I get weighed next week so we'll find out how much of a little big man I am.

I am frequently seen with my fingers in my mouth as I cut my first tooth on January 3rd, and my second came through today! Mommy was hoping that all my teeth would come in at the same time. Silly, silly lady.


My CCAC nurse was discharged this week. No more home visits for me!

Nurse Michelle

My last PT session with Cathy was 3 weeks ago she did the AIMS for my 8th month assessment. I scored between the 25th-50th percentile. She said I was closer to the top of this range! The AIMS if you remember is a scale which is used to assess an infants gross motor skills and the 50% mark is an average kid's score.

I started Kidsability a week later for physio and occupational therapy. I will be going there every Wednesday until the end of February, where I will receive another assessment to evaluate the frequency of my visits. My new therapists are Pam and Laurie (not to be confused with my speech therapist Laurie)
Both were impressed with my progression since the last time they saw me.
They understand my hatred of my tummy, so my exercises will focus more on my knees! Yay!

Today marked another visit to McMaster to meet with Dr. Tarnopolski.  He gave us my Carnitine level results and apparently I'm "saturated". Dr T assessed my progress and was pleased that I was sitting so well, making noise, and showing signs of stranger anxiety.  He gave dad the go ahead to reduce my Carnitine by half and my vitamin C entirely over the next few weeks.

The discussion moved from medications to hypotheses regarding my genetic make up.  Dr. T confirmed that my mom was born with and has the same genetic condition that I do.  Obviously the symptoms were less severe in my mommy.  Dr T surmised that the symptoms could be mitigated by the presence of estrogen or the double X chromosome that mommies have.  It could also be more severe in my case due to an interaction between my mommy and daddy's DNA.  Lots of possibilities!

We finished up with Dr T and cruised down to the cafeteria for a quick lunch.  Mom grabbed some hot water and warmed up my food and dad poured five ounces in my sippy cup.  I was soooooo excited that I polished that off in no time!  Dad filled up another ounce and after a quick break I downed that too!  Yes, you read that right!  I ate six ounces IN MY MOUTH!!!!

Looking forward to more yum yum my tum tum!

Bye for now.

Santa

Grandma and Mommy took me to meet this man named "Santa". Apparently he brings me presents for 1 day a year! I am going to be nice to him!

Mommy said I was so good! I was the only baby in the line who didn't cry with Santa!

Ethan Update

I have been doing well off my meds since the middle of November.
However I am still on Creatine, Carnitine, Vitamin C, Omeprazole and Domperidone.

I contracted a cold last week, had a runny nose, cough and hands full of boogers. I spread my sickness to daddy and grandma, but somehow mommy was left unscathed. We left my blood test to this week as mommy thought that colds could alter the levels. However, we since found out that colds don't alter carnitine levels.

Mommy and I headed to Mac on Monday for the blood test. She reassured me that there would be no head pokes, and they could draw the blood from my arm. I was happy with this fact and agreed to go.

The blood line was ginormous! The number was at 33 and we pulled a 55. Mommy read the sign that said "If you have cold/flu like symptoms please ask for a mask" She knew there was absolutely no way that I would keep a mask on my face, so we waited in the hallway instead.
Mariya ran into us, and was excited to see me! She said I was looking good and couldn't believe I was doing so well. She said that the genetics department has been super busy and that we would be in for genetic counseling in the new year and that mommy and daddy would have blood taken. Mommy told her happily that she had already given her blood!

A few hours later I was laying down in a crib with tourniquets on my arms. I was not bothered by this as I was still chatting to mommy. Then I heard the blood lady say she was going to find someone to help with the holding.... I knew this meant business and as soon as the tourniquet went back on my arm I was screaming! Mommy was instructed to hold my legs and chest down. She told me that I was super strong and that she had to use pretty much her whole weight to keep me from moving.

Another thing to note is that Dr. T had ordered Full Carnitine, which apparently isn't orderable any more since Sept since it costs so much to run, so they took blood to run my acetylcarnitine. Hopefully this is okay with the Dr. as I don't want to have to have blood taken again.

Dave (Infant Development) did my 8 month assessment last week and I scored consistent to my 6 month assessment, however my communication mark went down a little as I was not saying any consonant sounds. He also noticed that my pincher on my right hand isn't as developed as my left. It must be due to my middle finger issue.

8 Month Assessment

A few days after Dave assessed me, I started to say "ba"!  Mommy has also started some signs with me, however it is hard for me as my shoulders are weak and I don't like the effort that goes in. She's teaching me up, which I normally flail around to, so we consider that a win. She is also teaching me book, ball and of course hi and bye.  I don't quite understand these but hopefully I will soon.

I have been enjoying my oat cereal, but had a set back when I got sick and would hardly take any cereal. I was still given a few spoons a day, but I had lost my love for it. I still enjoyed my sippy cup with formula and mommy had started to give me sips of water from her glass. Which I love! After I started to feel better last weekend I started to eat my oat cereal again, which was good timing because my swallow assessment was today!

Mommy told Cheryl and Laurie about my sippy and glass adventures. They especially liked how eager I was and I presented good clinically. So they just told mommy to proceed with extreme caution... which she has been doing anyway. Onto my cereal assessment, I still looked good so I have graduated to orange vegetable purees! Of course we will have to add water to thin them down for me. But! I am excited! There was talk of getting me another Barium swallow study to assess my ability to swallow thin liquids safely. We'll keep you posted if they actually do set me up with one. Laurie indicated that it would probably take 2 months to set up a time.

That is it from me!

Merry Christmas and Happy Holidays!

What's been going on with Ethan?

I thought I would take a few moments to update my fellow readers on my progress before there are too many things to report!

As of today:
20 days: no Alpha Lipoic Acid
13 days: Carnitine went down to twice a day from 4 times daily
6 days: No Vitamin E
My CoQ10 gets D/C'd on Monday

I will be left on Creatine, Vitamin C, Omeprazole, Domperidone and Carnitine
My Carnitine levels will be checked mid December to see if I can be rid of the med, however a lot of metabolic/mitochondrial disorders have low carnitine levels, and Dr. T just wants to be sure that my levels are normal before I am taken off.

Since the last post I have learned to stand with support for a few minutes.  This makes me so happy as I am frequently heard chatting up a storm from this position.  I still don't make any consonant sounds, but the sounds now resemble language, not ear piercing squeals and grunts anymore. I can sit for hours playing with my toys... and am happy as long as someone is playing with me. To my mommy's dismay I have started to chew on my feet.  I have been known to roll from my back to tummy, but I don't do this very often and it's still very difficult for me to lift my head up from the tummy position. Often only for seconds at a time. Mommy was told to exercise tough love, but I make it very difficult for her. We have been working on my transitions, which I did not like, but am slowly getting used to the new positions.

Mommy relentlessly argued to have my total calories decreased before my diagnosis came in (so I have been eating less for about a month). Since she did this, I am more eager to take rice cereal orally. I had only a few days mixed in where it wasn't interesting enough. I am up to about 20mls of rice cereal taken orally before I start to get grumpy. My swallow therapist was impressed with me clinically, so the next step is to try a different grain cereal (like oat, barley or wheat).

My pediatrician said that she would be challenging me more on my oral skills. So my daddy took this to the extreme and introduced me to "The Sippy Cup". Do not tell my therapist! I'm just lucky that daddy told mommy! Mommy has allowed this for the time being....

Grandma and Mommy have been taking me to the Kidsability pool Wednesday nights (this also gives daddy a night off to himself!).
I have been four times. I started off really cautiously, splashing the top of the water only a few times, my legs were so stiff and I didn't want anyone to see that I was having fun, so I would quickly cover up my smile. However last time I was having the time of my life! Squealing and laughing up a storm! Kicking and splashing with my arms and legs! Grandma is trying to get me to relax on my back, which makes mommy tense with fear! I still do not know what to think about floating on my back....

The last thing to report is what came out of my visit with my pediatrician.  Daddy gets to PURPOSELY sleep through my overnight feeding!  My pediatrician says that now we know that I don't have some obscure glucose disorder and that I am now big enough to NOT eat overnight!  Tonight will be my third night not eating!  My mommy finishes feeding me at 11:30pm and my daddy starts feeding me at 6am!
Of course I go to bed around 8-8:30pm.

In the following weeks, I will have a second Kidsability assessment, carnitine levels, progression to a new cereal, and a photoshoot with Emerson!

Article

If you want to read the article that was referenced in my previous post about my disorder and the study that was done please visit the following link:

http://brain.oxfordjournals.org/content/early/2009/08/31/brain.awp221.full.pdf+html

Also please send your prayers and thoughts on to my buddy Emerson who just went through various metabolic tests at Sick Kids in Toronto.