I'm Back!

Wow, have I been a horrible blogger!!! Even though I am super busy these days (job hunting) and the holidays are coming up, I hope I'll do a better job keeping this thing updated!
That being said, I'll keep this one short and sweet! :)
This morning Harper actually asked Luke "Where's Mama?" I was in the shower and heard her and almost passed out! This weekend she also started nodding her head and saying yes when you ask a question. I am so amazed by her progress!
Last week she had her first speech evaluation since starting speech therapy. Her baseline scores put her at a 3-6 month level but this test put her at age level 15-18 months! Can you believe it??? Only 20 weeks of therapy and she's scoring within her age group!

One Scary Night

Saturday night we were so excited to watch the Alabama/Virginia Tech game! Luke had been looking forward to the beginning of college football....well, probably since the last game last year. Our friends Cory and Linsy came over to watch and my sister had popped in for a minute. Harper was in typical Harper-mode. She had Cory and Linsy's shakers running around the living room. We were all eating hot dogs so she was going around to each of us so that we could give her bites of bread. She came up to me to get another bite of bread and she stiffened up and fell in the floor. Her falling down was nothing unusual but her eyes kept fluttering and I noticed that she had pooped in her diaper at the same time. I picked her up to go change her and her eyes kept fluttering. I called the pediatrician on-call but ended up calling 911 because she couldn't hold her head up. We all went outside to wait on the ambulance. Harper would cry, then her eyes would drift off to the side, then snap back to normal. I was absolutely terrified.
By the time the ambulance got there I noticed that she was burning up. They had no thermometer on the ambulance, had no motrin/tylenol, and couldn't start an IV. I happened to have some motrin in the diaper bag (thank you God) and gave her a dose on the ride to Children's Hospital. They also weren't concerned when Harper kept drifting to sleep which is TOTALLY unlike Harper.
When we arrived at the hospital her fever was 103.5 after the motrin. They gave her another dose of Tylenol, took a urine sample (through a catheter, mind you), and checked her over. The doctor told us that she had a febrile seizure. Supposedly these happen when a child gets a fever within a short time frame (like 60 seconds or so). They told us that it could happen again and that it wasn't a concern if the seizure only lasts 30-60 seconds. Easy for them to say! Any longer and we should call an ambulance like we had done.
I cannot believe that her fever shot up so quickly. She had NO symptoms--no runny nose, no fever, no ear infection. I am sooooooooooo very thankful that she is okay. I know God is in control and taking care of this precious child!

Audiology Appointment

Harper had an audiology appointment Friday afternoon. She had to have another hearing test, and we had planned on buying the hearing aids and FM system. The hearing tests take place in a soundproof booth with her sitting in a high chair and an audiologist sitting in front of her. This time I sat in there with them. There is another audiologist sitting behind a semi-one way window to conduct the test. They place microphones in Harper's ears and try to get her to turn toward the sound. She is then "rewarded" by a moving pig or moving duck. In true Harper style after three or four turns, she was bored with the rewards and wouldn't turn to look anymore. The few results that they did get were consistent with her past test, so that means that her hearing hasn't gotten worse. Yay!
However, the audiologist did not want to order the hearing aids or FM system at the appointment. Because of Harper's diagnosis of enlarged vestibular aqueducts, they would like her to use the loaner aids for six more months in case her hearing drops further. If that happens, she may need a different type of aid or only one aid in her better ear.
We also discussed with her our concerns about Harper's balance issues. She said that compared to some children with EVA Harper's case isn't nearly as severe. She said that some children have to wear a helmet because their balance is so poor. Any bump on the head for children with EVA can cause a drastic drop in hearing. We are so thankful that Harper is doing so well!

Test Results

We finally got a call from the genetics clinic regarding Harper's Pendred Syndrome test. The test came back as normal. However, the counselor told me that this doesn't mean that Harper doesn't have Pendred Syndrome; it just means that this test came back negative. She has been in contact with Dr. Richard Smith at the University of Iowa which is always ranked nationally in the top 3 in otolaryngology. He said that in the next 1-2 years that there will be several more tests available that may be more conclusive targeting different chromosomes. The counselor told me that as of now Harper's diagnosis is still large vestibular aqueducts with hearing loss.

Sing-a-long

Harper LOVES music. In the last couple of weeks, she has really started to pick up on songs. Of course, she likes some more than others. Here is a rundown of what Harper is singing these days:

• Wheels on the Bus---She does some of the motions (wipers) but only sings "all through" which sounds like ahhh foo. "The wheels on the bus go round and round...." Then I point to her and she says, "ahhhh foo" and I finish "the town"!
• Ants Go Marching--She claps, stomps around, and says the "Boom boom boom boom" part. It is absolutely precious!
• The Clean-Up Song--She loves this and sings it ALL the time. She'll just be playing or sitting in her car seat singing "Up, up, up up". Maybe this is a sign that she's going to be mama's helper!

Terrible twos???

The past couple of weeks I have wondered if Harper has already entered the "terrible twos".
Harper has done a great job wearing her hearing aids---until recently. Now, if I tell her "no", do something she doesn't like, or try to challenge her while doing her speech therapy, she will pull both of her hearing aids out! She is basically saying, "I'll show you!" She realizes that this is one way that she can exert her independence and have control of the situation. In addition Harper has become increasingly impatient. For example, she wants her breakfast IMMEDIATELY after the microwave beeps. If there is a cartoon on tv/dvd player that she doesn't like, she'll point and scream at it until we change it to one she does like.
I have been worrying if this is "normal" behavior for a fifteen month old child or if this is related to her hearing loss and a sign of frustration. I had a long talk with her speech therapist today who assured me that although it is a little early, Harper's behavior is perfectly normal and very common with hearing impaired children. Thankfully, she gave me some tips to deal with Harper's new sassy attitude, but also warned that it will get worse before it will get better. I'm going to have my hands full!

One Proud Mommy

Yesterday Harper's speech therapist wanted to try a new game. She thought that Harper would be too young to catch on, but Harper proved her wrong!
Susan lined up three toys (a monkey, car, and airplane) and told Harper to get the monkey ooo ooo ooo eee eee eee and throw it in the basket. SHE DID along with the car, airplane, horse, bus, dog, and cat!!! The first round she was using the object's name along with the "learning to listen" sound. She did so well that she played the next game only with the object's name. She only missed one!!!
After only four months wearing her aids, she is listening and learning way more than I thought. I am so proud of my baby girl!!!

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???

Another day of speech

Harper had speech therapy again yesterday. Usually she is great at participating and seems to enjoy it. Yesterday she wouldn't repeat most of what we were saying to her. I had noticed this pattern when we had practiced her "Learning to Listens" this past week. She has begun to default to her usual "bye bye or ba ba" as an answer to everything. Also, instead of saying "uh oh" very plainly, she has been saying "uh uh". I just chalked it up to a bad week, teething (3 or 4 new teeth this week for a total of 13), stubbornness, or laziness.
When I explained this to the speech therapist and she witnessed her behavior yesterday, she had cause for concern. Supposedly, this is a sign of progressive hearing loss. She will go see the audiologist tomorrow for further testing. I just don't think I am prepared for the hearing loss to get worse.
On a happy note, I am currently listening to Harper play "Peep-Eye" with herself when she is supposed to be napping. It sounds like "P-Pie P-Pie P-Pie". Hilarious.

Faith

I read a blog today that said "I don't want a life that requires much faith." This is SOOO the way I feel. I want things to just roll along without a hitch. He unpacked the thought further and said:"Here’s the thing I’ve realized, though. My natural inclination to have a secure life that needs little faith ends up being a life that needs very little God, either. That’s the deadly part of security and safety, we get used to living without God. There’s no need for God to show up. “All set, Lord,” becomes our silent, unconscious prayer." Boy, do I need to keep that in mind.

Lots of shots

Harper had her 12 month visit at the new pediatrician yesterday. His name is Dr. Johnston at Birmingham Pediatrics. I had asked around in some of my offices and to some of my friends, and his name kept popping up. Supposedly, he has a lot of experience with developmental delays. Although Harper didn't want much to do with him, I really liked his approach. She especially didn't care for the four immunizations!
He was very impressed with Harper's speech at 12 months. As you can see on the sidebar, Harper is up to around 12 words. He said that just 2 words at this age is within the normal limits. Yea!! He said this is phenomenal given her hearing impairment.

I also want to wish Luke a late "Happy Anniversary"! We've been married four years on May 16th and still honeymooning :) I'm so lucky to have a husband like him. He is so honest and trustworthy and really does have a desire to please God. Men like him are rare.

Harper's Birthday

Harper's first birthday is tomorrow. I can't believe it has already been a year since she was born! Last year at this time, I was in the hospital trying to watch Grey's Anatomy while the nurse was putting the IV in my arm and getting geared up for the cervadil.

What I wasn't expecting was the strange feeling I have at the one year mark. While I am happy that she is growing and learning more every day, I also feel sad that I am losing my baby. It is like every day there is a new step toward her independence--no more bottles, no more formula, self-feeding, etc. I just can't imagine never being able to witness again the amazing things that happen in the first year of a baby's life. God knows the desires of my heart, and I pray that He will let us have another child in His time.

By the way, this blog has been WAY too serious for my liking. I promise I will try to lighten it up soon!

Our Story

After months of dealing with constant ear infections, Harper went in to Children's Hospital on February 26 to have tubes and her tongue clipped. Before, the ENT had told us that she had mild hearing loss due to the infections and when the fluid was drained she would go back to normal hearing. The audiologist had suggested that she have an auditory brainstem response (ABR) done while she was under the anesthesia just to check things out. Luke and I thought this was common procedure. We were actually pretty excited about her surgery because we thought she would start making more speech progress when the infection was gone.

The doctor came in after the tubes were in place to tell us that the surgery was a success, but she had no fluid after all. He said the audiologist was performing the ABR and would be in to give us the results after it was complete. He said have a nice day, blah, blah, see you in a year.

In a few minutes the audiologist came in accompanied by the doctor. At that point I knew something wasn't right. She told us that the ABR showed a sensorineural hearing loss--moderate in the right ear, severe to profound in the left. I asked her to clarify what that meant, and she said that Harper could hear around 90 decibels in here left ear and a rock concert is 100 decibels. She recommended hearing aids in both ears. I asked, " How long will she have to wear them?" The audiologist said, "For life". There is no cure, no surgical fix. That is the point that reality set in, and I didn't hear much that was said after that.

The past two months have been life changing for Luke and me. We have dealt with the reality that things will be different for Harper, and things will be different for us as well. Of course, we still have a hard time dealing with this news, but God has allowed us to grieve quickly so we can spend our time focusing on helping Harper's development.

Today is my first Mother's Day. I am thankful for the blessing that Harper has been in my life. We are thankful for every single improvement. This situation has made us slow down enough to recognize the miracles that happen every day.

First Day at Speech Therapy

Yesterday was Harper's first day of speech therapy at Children's Hospital. They have a special clinic called the HEAR Center specifically for children with hearing loss. After we found out that Harper had hearing loss, we decided to proceed with a plan called Auditory-Verbal Therapy (AVT). This will allow Harper to be mainstreamed in school and live the most "normal" life possible. This approach teaches the child to learn to listen rather than lipread or use sign language. It seems that AVT is pretty rigid. We have to cover our mouth while talking to Harper because she is already such a great lipreader. This week we are working on Ahhhhhhh (airplane), Bu bu bu (bus), *raspberry sound* beep beep (car), and choo choo (train). We are also working on her understanding taking turns making the sounds.
The therapist said that they compare her to a two month old baby's development since she has only been aided for two months. We are so impressed by her progress so far!

creepy UAB

UAB has a clinic for EVERYTHING. Monday Harper had an appointment at the genetics clinic at the UAB Department of Human Genetics. A little creepy, yes. First, a genetic counselor came in and took a life history from Luke and me (and I mean life history). She actually drew up a family tree. She asked our ethnic origin. Come on, I'm from Jasper. Next, the doctor came in, looked at Harper's face, hands, and feet, and basically told us that she looked normal. Apparently, a lot of times they can look at children and tell if a syndrome is involved. He didn't like the results from the first round of genetic tests so he sent us over to Children's to get more labwork done. They are sending the blood to a special lab at the University of Iowa--they have a otolaryngology clinic that does chromosome tests specifically for deafness. Cool, huh? Another 4-6 weeks of waiting for results.

very, very new at this

Hello to all!
This is my first attempt at documenting our journey raising a personality-filled child who just so happens to have severe hearing loss. My hope is that this will help people to understand how difficult learning is for children who have never had the opportunity to hear spoken language the correct way and other challenges that come along with hearing loss like those ugly hats. I hope there are many more blogs to come.