Little Trooper

Ben began his big day, December 23, at OHSU at 7:00 am. He won the hearts of all the nurses as he played and waited 3 hours for his surgery time.

After 4 hours of surgery and recovery, we were able to see him again. He had had trouble breathing and needed to be monitored a bit longer than usual. Hospital policy requires babies to eat before they are discharged. Ben refused. Reluctantly, after 12 hours in the hospital, we headed for home.

He finally ate at three the next morning and again at 8:00. Each time he generously shared his food by projecting it out and down the front of Daddy.

He perked up for his first Christmas eve at Grandma and Grandpa's.

This is his scar on day two. It's hard to see but the stitches actually go all the way around to the bottom of his ear. To the left of the stitches is a small dot. Under the dot is the internal magnet and processor.

By Christmas day, 48 hours after surgery, he was playing, eating and smiling. He is wobbly and has a hard time crawling, standing, and sitting up on his own. We have been told his balance will improve over time.

Overall, we have been impressed with Ben's recovery. Ten more days and he will be hearing for the first time in his right ear! He is quite the 'Little Trooper'.

This past week we spent another long day at Doernbecher. Ben was assessed by Speech Pathologist and a Cognitive Specialist. Their goal was to determine a baseline for Ben's language skills before he receives his implant. We will go back in a year and they will measure Ben's progress.


They were most impressed with Ben's signing. He will sign about 6 signs consistently. After a few minutes of torture in his highchair, I was able to record a few of Ben's favorites.

Ben signs Daddy ALL THE TIME.

This boy is all about food. He signs 'milk' when he wants a drink and throughout the day whenever he wants anything. 'More' and 'cracker' are his newest signs. Here he does both.

'Thank you' must be the cutest sign of all. Instead of tapping his chin he dramatically smacks his face.

The real heart breaker is when I rock him back to sleep in the middle of the night and he groggily signs 'mommy' over and over. I'd get up twice for that one.

We Have a Date!!!!

It's official:

DECEMBER 23!

Insurance approved and we are ready. Ben will have is pre-op next week and then we wait!

Yahoo!

First Thanksgiving

Ben's first Thanksgiving weekend was full of adventures.

Including.....

tasting and loving pumpkin pie,

wearing Grandpa's hat,
hanging out with the Grandmas,

helping Daddy with the new addition,

sorting Mommy's Tupperware,



and finding Daddy outside.

Ben also discovered what happens to rolls of toilet paper when they are put in the toilet, how fast Mommy moves when you shove moss in your mouth, and exactly what a dog's tongue tastes like.

Somehow I didn't manage to get pictures of those.

Choices

This past week we met up with Don at OHSU to talk about Ben's up coming implant. (For a brief review of what a cochlear implant is check out this earlier blog: MRI and CT.)

We discussed our options for which brand of implant Ben will be getting. The choices include Cochlear's Nucleus 5, MED-EL's Sonata, and Advanced Bionic's Harmony. After lots of debating, we chose the Nucleus 5.

The Nucleus 5 has the slimmest processor available and has some wearing options perfect (hopefully) for a little guy.

He can wear the processor on his ear and the battery pack (the portion at the bottom of this picture) clipped to his shirt. My hope is this will make the whole contraption slightly less easier to loose.

When his ears get bigger, he can wear the battery and processor on his ear.

It even has a remote with a full-color display so we will be able to wirelessly monitor and manage Ben's hearing.






Part of our visit included a hearing test. I held Ben in a sound proof booth while an intern distracted him with a toy. Don plugged Ben's ear with a device through which he pumped in sound at various decibels. Ben's job was to turn to a moving toy each time he heard the sound.

It took a few tries, but Ben soon caught on.

Unfortunately, Ben wasn't responding as well as he previously had in his left (good) ear. We know Ben's hearing loss will progress at some point. That time may be now. We can adjust his hearing aids, do some more tests, and maybe someday implant the left ear as well. We aren't as disappointed as we thought we would be; The implant date is coming so fast.

Oh...did I forget to mention? DECEMBER 23! We are just waiting on insurance approval. Keep your fingers crossed!

Lion King

Happy Halloween!

Perfect Pumpkin

Ben finally got to pick his pumpkin from our garden.

Pumpkin stems do not taste good!

But they are fun to carry.

Pumpkins, mud, flannel, and rubber boots... It must be fall.

P.S Rubber boots are HARD to put on a baby.

Farmer Ben

Ben loves feeding apples to Frank.

He tries so hard to stand on his own.

But, it doesn't last long. He came up smiling with bits of grass stuck in his two tiny teeth.

Racing Grandma!

It's hard to tell who has the coolest wheels!

One Year Ago Today!

One year ago today I went in for my final ultrasound and to find out if we were having a boy or a girl. Adam had been talking a bout his son for weeks and kept asking me how his boy was doing. He told everyone he'd be happy with a boy or a girl, but we all knew he hoped for a son.

The ultrasound technician looked at Ben's heart, bones, stomach, hands, feet, and brain. She took her time taking pictures and making sure he was healthy.

Out of the blue, she said, "It's a boy!" Adam leaped out of his chair, raised both arms in the air and shouted "YEAH!" It was like his team scored a Touch Down in overtime at the Super Bowl.

Our team definitely won this time!

Ahoy!

This past weekend we joined Grandma and Grandpa on their sailing trip in the San Juan Islands.

Ben helped Daddy and Grandpa steer the sailboat.

A good sailor always has a snack close at hand.

Ben also tried out his sea legs on Uncle Peter and Aunt Gerry's boat.

Tasting the wheel is an old Captain's trick for ensuring your crews safety.

Arr!

Favorite Sounds

Ben loves to blow raspberries.

All through the grocery store, car rides, in restaurants, on walks, in the swimming pool, to little old ladies at the retirement center, in bed at 4 in the morning ....everywhere.

Not always polite, but SUPER cute!

Crawling?

Ben's on the move!

It's kind of a crawl, scoot, and drag with a half roll.

Definitely an A+ for effort!

He couldn't be happier with himself.

Cousin Maddie

Ben spent a fun Saturday playing with his Cousin Madison.

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She took Ben on his first ride down the slide

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and pushed him in the swing.

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Over and over.

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Ben LOVES his swing and his Cousin Maddie!

Daddy's Big Helper

This weekend Ben helped Daddy drive an excavator.

First they discussed the job.

Then, Ben learned exactly which controllers to use.

Finally, Daddy showed Ben how to play in the dirt.

Two happy boys with one big toy!

Garden Helper

Ben loves to help in the garden. Today we picked green beans.

Well... he picked a green bean.

A lesson in Genetics...

Today from the Genetic Counselors at OHSU called. The results of Ben's genetic testing had arrived.

Ben was tested for an autosomal recessive disorder called Pendred Syndrome. Autosomal recessive is one of several ways that a trait, disorder, or disease can be passed down through families. An autosomal recessive disorder means two copies of an abnormal gene must be present in order for the disease or trait to develop. A mutation in a gene on one of the first 22 nonsex chromosomes can lead to an autosomal disorder.

Genes come in pairs. Recessive inheritance means both genes in a pair must be defective to cause disease. People with only one defective gene in the pair are considered carriers. However, they can pass the abnormal gene to their children.To have an autosomal recessive disorder two copies of the gene must be mutated for a person to be affected. One copy comes from the mother and the other from the father. The parents are usually unaffected and unknowingly each carry a single copy of the mutated gene (and are referred to as carriers).




If both parents are carriers, there is a 25% chance of a child inheriting both abnormal genes and, consequently, developing the disease or syndrome. There is a 50% chance of a child inheriting only one abnormal gene and of being a carrier, like the parents, and there is a 25% chance of the child inheriting both normal genes.

The results from Ben's tests show he has two mutated genes, what is expected with someone affected by an autosomal recessive disorder. One of the mutations is to a gene known as SLC26A4 on chromosome 7, which causes Pendred Syndrome. The other is a mutation that has not been seen before. The results stated the exact mutation is unclear.

Because Pendred syndrome is a recessive trait, a child needs to inherit two mutated SLC26A4 genes—one from each parent—to have Pendred syndrome. This means Ben's diagnosis is still a bit unclear. To determine if Ben truly has Pendred's both parents need to be tested to see if we are carries of the mutated gene. If we are carries, it is possible Ben has Pendred's with a mutation that has not yet been documented. Lucky Ben!

It is important to us to determine whether or not Ben has Pendred's because it can cause some other complications for him as he grows older. Mostly, we will need to monitor his thyroid as he enters puberty. He may also develop some balance issues as he begins to try to walk.

All in all, the answer is good for Ben and good for us. It's a bit strange, in a way, to think that people can carry this mutated gene around unknowingly. If they have a child, that child may or may not be affected. It may never surface. But then again, it just might. Lucky, lucky Ben!