CT Scan Update

Good News!! Jesse spoke with Dr. Little on Thursday and he informed us that Little bit's CT was normal, meaning no calcification of the cochlea. Praise the Lord!!! Two tests down, 2 to go....

1)MRI: Sept. 9th, under sedation

2)ABR/Complete Auditory testing: Sept. 28th, under sedation

Please pray for Emerson's health and safety as they have to sedate her for these last 2 tests.

Rejoice in hope, be patient in tribulation, be constant in prayer. Romans 12:12

Rejoice in the Lord ALWAYS, again I will say REJOICE. Philippians 4:4

It's NEVER Too Early

Emerson spent this past Saturday in Knoxville with her Poppy and CC (my parents) while I was at work and Jesse tended to some business there. Poppy taught his little princess her first bible verses. When they called me to recite them to, the entire time Poppy was reciting the verses she babbled right along with him. It's NEVER too early to start teaching of the Lord's miraculous, undeserving, unconditional love.

Her 1st 2 verses:

For God so loved the world, that he gave his only Son, that whoever believes in Him should not perish but have everlasting life. John 3:16

Jesus said to him, "I am the way, and the truth, and the life. No one comes to the Father except through me." John 14:6

2 Month Check-Up

My how time flies! Yesterday, August 8th, was Emerson's 2 month check-up. Here are her stats:

Weight: 12 lbs. 2 ozs. 75-90%

Height: 23.5 inches 75-90%

Head: 15 inches

Dr. Good and I discussed developmental mile stones. Here are a few of the many that Emerson has attained:

Social Smile

Better head control

Rolls from her back to her side

Reaches to swat at toys

Tracks with her eyes

Watching her mobile

Has different cries for different things

I just had my shots cry

Too many pictures mom!

Last but certainly not least she is cooing and making all kinds of conversation!! PRAISE THE LORD!! She is even making a lot of guttural sounds, which Dr. Good says shouldn't happen for a couple more months. I believe it's because she can feel those sounds when they are made.

I have been anxiously awaiting these little noises to come out of her mouth. There is nothing sweeter to my ears than to hear her use her voice. You see, for many deaf babies they may start out making a little noise, but many stop because they can't hear themselves. The deaf educator that came to do her assessment for TEIS (Tennessee Early Intervention Services) explained that every time she makes these noises we should get in her face, be really animated, smile and point to our ear and say, "I hear you." This positive affirmation will hopefully help her continue to make noises, especially as she gets a little older.

Prayer request: That she continues to develop appropriately and continues to expand her baby babble!

As you may know, this appointment is when she got her first round of immunizations. I opted not to give her tylenol due to some studies that I've read recently that say mounting a small fever will help her mount a good immune response. She never ceases to amaze me, she took it like a champ! Only one loud cry and then she went to sleep in my arms. Yesterday she was a little more sleepy and fussy than usual, but she slept 6.5hrs through the night and is back to her norm this morning. GOD IS GOOD and HE IS ABLE!

Give thanks to the Lord for He is Good, His love endures FOREVER. Psalm 107:1

Genetics Update

This past Friday I received a call from, Dr. Steven's office regarding mine and Jesse's genetics testing. Connexin 26 is indeed the reason for Emerson's deafness, HALLELUJAH!. Jesse and I both passed her a mutation causing this recessive trait to be expressed. I am so thankful to say that deafness caused by connexin 26, is non-syndromic isolated deafness.

But I will hope continually and praise you yet more and more. Psalm 71:14

CT SCAN COMPLETE!!

On August 4th, at 8:30 Emerson was scheduled for a CT to determine if her cochlea is calcified. Let me start by saying this is quite possibly the hardest thing I have ever had to do. The CT was supposed to be sleep deprived. I was instructed to wake her up at 4:00am, keep her hungry and awake until we arrived at the hospital at 8:00 for registration. At that time I was supposed to feed her and she was supposed to fall sound asleep. Well, let's just say it didn't go as expected. Little bit has been sleeping 6-8hrs through the night for the past 2-3 wks, so when I woke her at 4am she had already been without food for 7 hrs. The first hour was a breeze. My mom and I were able to amuse her, but the ceiling fan did most of the work. After that, it was all downhill! She began to cry and cry and cry. She cried so hard for the next 3 hrs, that her little body was quivering. It got to the point that we couldn't even put her paci in or hold her for more than a few seconds because she would start to fall asleep. When we arrived at the hospital at 7:45 it was getting more difficult to keep her awake. I went into registration and when I came out my mom had her stripped down and was wiping cold baby wipes on her to no avail, Emerson was down for the count. The problem: she hadn't eaten in over 11 hrs. She was supposed to eat BEFORE she fell asleep. I tried everything to wake her up and she wouldn't so much as flinch. She was listless and I was a little worried she was hypoglycemic. Our CT wasn't scheduled for another 30 mins, but fortunately, a tech saw me desperately trying to get her to breastfeed in the lobby and I explained to her the situation. They immediately took us to the scanner, laid her little listless body on the table, and within 3 mins the scan was complete. Almost the very minute the scan was completed, her little eyes began to flutter, coincidence??? I think not!! Praise the Lord for all the prayers that went before her!! My little warrior stayed completely still for the scan so we didn't have to repeat it and then latched on and ate like a champ! She stayed awake for another hour before she crashed. I am so thankful that this test is complete. Now all we have to do is WAIT, WAIT, WAIT!! Isn't this a great game?!?!?

Immediately after the scan

Let us then with CONFIDENCE draw near to the throne of grace, that we may receive mercy and find grace to help in time of need. Hebrews 4:16

The prayer of a righteous person has GREAT POWER as it is working. James 5:16b

WHAT A MIGHTY GOD WE SERVE!!!

Hearing Aid Fitting

On Monday, August 1st at 9am, my little one was fitted with her first set of hearing aids! YAY!!! She was a trooper. She was fitted with a BTE (behind the ear) aid that has a mold for her ear. They are her new purple bling! They have a 55 dB (decibel) gain, which means they amplify the sound in her environment by 55 dB.

She's so young that it's very hard to objectively tell how much she is actually hearing, but when her left aid was put in, she got very still and looked to the left. When her right aid was placed she stopped sucking on her pacifier and her eyes were as big as saucers. After the initial placement it's been hard to tell what she is actually hearing, but it seems as if she hears deeper voices, such as her daddy's and poppy's (grandfather's) voice better than any other. We remain hopeful that as she gets a little older we will be able to more objectively tell what she is hearing. After we've completed the full auditory testing, which is scheduled September 28th, we'll know exactly what her aids allow her to hear.

After one day of using the hearing aids, we quickly realized this is going to be more of a challenge than we had anticipated. Her little ears are so pliable it's hard to keep the aid in place, not to mention her little hands seem to find their way to her ears and she pulls them off. We also realized that her right aid was having to be replaced more frequently because the mold did not fit as snug in that ear. Another appointment made for wednesday morning allowed a new mold to be fitted free of cost, (Praise the Lord!), for that ear. We are waiting for that one to arrive.

As the week moved on, we realized a headband was the way to hold these babies on! She is getting used to the idea of this and is pulling less frequently at them! Looks like she'll be wearing headbands a lot!

We are scheduled for a follow-up appt. on 8/16/11. At that time, Wendy, (the audiologist) will adjust the filter and the volume and we will recheck her molds to make sure they still fit correctly. She is growing so fast, her molds will have to be replaced often. I am so thankful she has her hearing aids and am anxious to see how they work for her. Now that she has her aids she will begin therapy in Knoxville. Our first session is August 31st! I had the opportunity this past Thursday to visit the therapy center and observe children with cochlear implants, ages 2-11 years receiving therapy. WOW!! It was AMAZING!! Oh, how I pray that little bit will be a candidate. I can't wait to get started and am so thankful to be moving forward! The Lord continues to be faithful and continues to show me everyday how nothing happens by accident. I am so thankful the Lord has blessed me with this sweet baby girl, she continues to amaze me everyday!

Psalm 100

Make a JOYFUL NOISE to the Lord all the earth!

Serve the Lord with gladness! Come into his presence with SINGING!

Know that the Lord, HE IS GOD! It is HE who made us, and we are HIS; we are his people and the sheep of his pasture.

Enter His gates with THANKSGIVING, and His courts with PRAISE! GIVE THANKS TO HIM; bless His name!

For the LORD IS GOOD; His STEADFAST LOVE ENDURES FOREVER, and His faithfulness to all generations.

Continuing the Journey,

Cristin

Our Unexpected Journey

On June 3, 2011 Jesse and I welcomed a new little blessing into our lives, Emerson Grace Doty. She weighed 6lbs. 9oz. and was 19.5 inches long. This little girl was all we had ever dreamed of and brought instant joy into our lives. It's amazing how quickly you fall unconditionally in love with someone. The pregnancy and delivery were uneventful and she appeared healthy. Our stay in the hospital was short and the day she was to be sent home she had her newborn hearing screening. The initial test was performed in my room, however, she was not having a good response, so the tech decided to take her to the nursery to repeat it. When she was returned to me I was informed that she failed this screening exam, but was reassured that many babies fail their initial test due to fluid or vernix in the ear. She was to be retested in 3-4 wks. As any mother would do, I immediately started to worry, but I tried to squash it, so as to completely enjoy my new little bundle of joy. We went home later that evening and enjoyed our first few days at home. Our first appointment on Monday, June 6th, to our pediatrician, Dr. Good, went well, she had lost a few ounces and was down to 6lbs. 4 ozs, but otherwise she had an uneventful check up. I did, however, voice my concerns about the failed hearing exam, but was reassured that MANY babies fail this screening exam and that there was most likely nothing to worry about. We rescheduled for the end of the week to have her weight rechecked, hoping that she would have regained enough to be back at her birth weight. On Friday, the little chunker weighed in at 6lbs 12 ozs. 8 OZS. gained in 4 DAYS!!!! What can I say, she is a GOOD eater!! At this visit Dr. Good held her and when he talked she seemed to calm to his voice. He was reassured that she could hear and that her repeat test would be normal.

I couldn't help but notice over the next couple of weeks that she didn't seem to startle to any loud noises, not to the door bell, the dogs barking, or the pans that I would intentionally drop from time to time. I tried to push the worry away and rest on the promises of the Lord, to be anxious about nothing, but pray about everything (Phil 4:6), but I found that sometimes that was easier said than done. I rescheduled our repeat hearing exam for June 27th. And so we waited. We waited and watched, anxiously anticipating her to startle to any loud noises. That never happened.

Test day arrived and as we entered TC Thompson Children's Hospital, my stomach was in my throat. Jesse met us for the exam and again we waited. Emerson's name finally called, we met an audiologist named Susie Keith. She repeated the OAE (otoacoustic emissions) exam, which is the screening exam. There was no response. I immediately started to cry because my gut had said all along that her initial test was not a false positive. Susie said, "no, we aren't going there yet, we have other tests to do." She then did a tympanogram to determine that there was no conductive issue with her ear canal or tympanic membrane, such as wax, or fluid. She passed this exam. We then proceeded to do the ABR (auditory brainstem response) exam. Jesse asked if there could be any false positives to this exam and Susie said, "no." So this was it. As the testing proceeded, it was becoming apparent that she was not having any reactivity. Susie looked at both of us and said, "It appears that she has severe to profound hearing loss." My heart sunk, tears began to flow uncontrollably and all I could do was hold and rock her. I couldn't believe what I was hearing. My daughter was deaf. Jesse was as shocked as I was, but immediately went into 'what's next' mode. Susie said we should see an ENT. Jesse immediately walked out of the room made a phone call and had us an appointment the next day with Dr. David Barnes.

As we left that day, I could barely breathe, walk, or talk. I couldn't believe this was happening. I immediately wanted to ask the Lord, "WHY?!?!?" Why my daughter? We waited so long to decide we even wanted a child and then we have one and she is deaf?? Why do all my friends have healthy children, more than one, and we have ONE Lord?? Why, Lord???? you know I love music, I love to sing and SHE IS DEAF?? I felt, hurt, anger, sadness, disbelief, many emotions flooded my heart as I drove home that day. All I could think was, 'Lord, I want her to hear us tell her we love her.' Jesse left the hospital to be with me and he called my parents to come down. As we sat that day, soaking in the news, many tears were shed, many prayers were said, and many questions asked, but I knew at the core of my being that this was NOT A SURPRISE TO MY GREAT SAVIOR. I knew that the Lord had chosen me and Jesse to be this sweet little, precious, perfect, little girl's parents. I knew that he MUST have a great plan for her life, one to prosper her and glorify him! It didn't change the hurt or the desperation, but I knew he was in control.

Today is July 23rd, and as the days pass I cling even tighter to his promises. I anxiously await the Lord's miraculous healing of my little girl, praying all the time for the grace to handle if this is not what he KNOWS would be best for her. His promises are more real to me everyday. I KNOW that this world is broken, but MY GOD IS NOT!! I KNOW that GOD IS GOOD ALL THE TIME and HE IS ALWAYS THERE, even when we feel like he's not. I KNOW that this little girl, the most precious gift we have ever been given, was chosen especially for us. I KNOW that the Lord IS WORKING EVERYDAY in Emerson's life. I KNOW He is preparing her to do GREAT things! I know that this journey is just beginning, but I KNOW that the Lord has already gone before us and prepared the way. He is granting us new mercies and grace everyday to walk this path put before us and I pray that we walk it with confidence and grace. The Lord has already changed me so much through this process and I know he is not through with me yet!

Where we are now:

I sing and talk to her all the time, as if she can hear me and she does calm to the vibrations of my voice when I'm holding her. I guess that means I have to hold her ALL THE TIME!!! As you can imagine with this diagnosis comes many doctors appointments and much waiting. We have seen a geneticist, Dr. Kathy Stevens, to have Emerson tested to make sure there is no syndrome associated with her deafness. We were told this past Wednesday that it appears that she has 2 mutated genes (connexin 26) that cause non-syndromic hearing loss. Jesse and I had to have our labs drawn to confirm she got one mutated gene from each of us, which would confirm this as being the cause of her hearing loss. We have been to see a pediatric ENT/surgeon, Dr. Little, in Knoxville, who would be the one to do her cochlear implants if she is a candidate. We are scheduled for a CT on August 4th, to make sure her cochlea is not calcified and an MRI as well as a complete hearing exam on September 28th. The MRI will determine if her auditory nerve is atrophic. These tests will determine if she is 100% a candidate for the implants. We have also been to see Wendy Richardson, an audiologist, to have her fitted for hearing aids, which we hope to get next week. All babies are fitted with aids initially to attempt to get any noise into their little ears to help stimulate them.

Specific prayer requests: 1)Pray for me and Jesse to each have passed a mutated gene to Emerson. This would confirm that connexin 26 is the reason for her deafness and it is NON-syndromic usually only affecting the hair cells in the cochlea, which would be a step in the right direction for being a candidate for the implants.

2) Pray that the hearing aids would bring enough noise into her little ears that we see a response. So that we know she is being stimulated. With her being severe to profound we just don't know how well the aids will work.

3) Pray that she is a candidate for the cochlear implants.

4) Pray for grace and mercy for us as we HURRY UP AND WAIT.

5) Pray that the Lord will keep Emerson healthy and developing appropriately, that she will come to know him at an early age and pray that he uses her little life to do GREAT things to bring GLORY to him!

Journey with us!

Love,

Cristin