Harper's First Birthday

On May 16 we celebrated Harper's first birthday. This year has FLOWN by!

We had a small party at home, but Harper had a blast! She really dug into her cake. We even had to clean it out of her nose! Notice how well she is doing with her hearing aids. The majority of the time she wears this headband instead of the hat. I bought her these cute headbands at Kids Unlimited in Cullman when she was younger and didn't have enough hair to wear a bow. I had no idea how handy they actually would be!
She got WAY too many toys, but I have to say she plays with it all. She especially loves this baby doll that she puts into the recliner and says, "Rock, rock". Luke and I got her a pink electric car. When she's old enough the stick comes out of the back and she can make it go by pressing a button on the steering wheel. She loves it!

A Start in the Right Direction

I finally talked to Dr. Robin, Harper's geneticist, concerning the CT scan results. He said that it looks like she has enlarged vestibular aqueducts. Here's a snippet I found from nicdc.nih.gov:

Research suggests that most children with enlarged vestibular aqueducts (EVA) will develop some degree of hearing loss. Scientists also are finding that five to 15 percent of children with sensorineural hearing loss, or hearing loss caused by damage to sensory cells inside the cochlea, have EVA. However, scientists do not think that EVA causes hearing loss. Instead, scientists regard EVA as an important clue about hearing loss and its possible causes. This information helps physicians talk with families about how their child's hearing loss may change over time.
The presence of EVA can be a symptom of a genetic disorder called Pendred syndrome, a cause of childhood hearing loss. According to a study by the National Institute on Deafness and Other Communication Disorders (NIDCD), approximately one-third of individuals with EVA and hearing loss have Pendred syndrome. With Pendred syndrome, the hearing loss is progressive, which means that a child will have less hearing over time. Some children may become totally deaf.
In addition to its association with hearing loss, EVA may also be linked with balance symptoms in a small percentage of people. However, the brain is very good at making up for a weak vestibular system, and most children and adults with EVA do not have a problem with their balance or have difficulty doing routine tasks.

This isn't great news but it isn't horrible news either. There are a number of syndromes that have much worse effects than Pendred (i.e. blindness, heart defects, etc.) I am very encouraged that the doctors may finally be heading in the right direction. Dr. Robin is consulting with several other doctors about the test results. He said that the genetic counselor would get back in touch with me soon.

Another Day at Children's Hospital

Since all of Harper's genetic tests have had normal results, the next step is for her to have a CT scan of her inner ear to look for structural abnormalities. Even though it is just a simple, sedated procedure, I am much more nervous this time around compared to when she got tubes. She is old enough to realize that she is at the hospital or doctor's office and get really worked up. She even gets upset when we go to the HEAR Center for speech therapy and all they do is play with her there. I'm pretty sure that we won't know any results tomorrow. They usually send the report to the doctor's office who will call us with the results. I'll keep you posted!!

Be anxious for nothing but in everything by prayer and supplication with thanksgiving make your requests known to God. And the peace of God, which passes all understanding will keep your hearts and minds through Christ Jesus. Phil 4:6-7

Not-So-Happy Father's Day

After eating a great Father's Day meal even before gifts or dessert, Luke and I made a little trip down to Children's Hospital. Harper has started shoving her high chair tray when she wants out or gets angry. While I was putting in her hearing aids, she got mad and shoved the tray. Well, somehow the tray wasn't latched and she went face first onto the tile. We were afraid that she had bit through her lip or chipped some teeth but couldn't tell because her lip immediately puffed out. We grabbed her up and took off to downtown.
All in all, the doctor said everything was okay. Her teeth weren't loose. She had bit through her lip but not bad enough to need stitches. The worst part was her frenulum (the part that attaches your lip to your gum) is caught between her front two teeth. He said it would come out on its own. She was laughing before we left and doesn't seem to be in pain at all.
Not a great way to spend your Father's Day.

Genetics Update

The genetics counselor called today to tell me that Harper's tests were normal. Good night, can they make up their minds? The ENT clinic told us that she tested positive for the Connexin 26 gene mutation. Then, the geneticist told us that she was only a carrier for the Connexin 26 mutation so they did further testing. He felt sure that one of the tests would come back positive. Unfortunately not. You may be thinking, "Why does this crazy woman want the test to be positive??" Well, it would really help to know what we are dealing with--if the loss will progress, what type of therapy works best, or if any other body parts (eyes, heart, kidneys) will be affected.
What next? Harper will have a CT scan of the head to check for any structural abnormalities. Poor Harper will have to be sedated again and that means getting to Children's super early on an empty tummy. After putting her through all this, I'm just hoping that we'll get some answers.

Walk Walk Walk

Harper has really been making some progress with her walking. Her pediatrician told us that she would probably be a late walker because hearing loss and balance issues go hand in hand. Take a look at the bumps and bruises she's already gotten on her face! Go Harper Go!

Hot Head

It is finally June, and it is scorching outside!! I love summer and hope to get the pool going so we can swim this weekend.


That being said, I need your help! Harper is having to wear hats to keep her from pulling her hearing aids off and putting them in her mouth. It is WAY too hot to wear a hat in the middle of summer. Her poor little head is so sweaty when I take it off. Here's the ad from hannaandersson.com

Doesn't this look like Harper? Big cheeks and everything!

The audiologist only suggested this hat and toupee/wig tape to deter her from taking them off. We have been moderately successful using a stretchy headband but if she ever gets a hold of them OFF THEY COME! This is especially bad if we are in public and she happens to drop one.

Any ideas on what we could do???