Emma Updates (December 2003 Archive)

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December 2003 Archived Emma Updates

12/27/2003-Well it has been an interesting week to say the least. We took the kids to see Santa on 12/23. We waited in line over two hours for them to see him. As you can see from the photo above, Emma seemed to be more traumatized by seeing Santa than by getting her hearing aids. While eating dinner on Christmas Eve, Emma got sick all over the table. She had been coughing and wheezing all day and we assumed she had what her brother had. After getting the kids down Santa, Mommy, and me got to work on putting toys together and wrapping gifts. The kids had a blast Christmas morning and we went to Grandpa Ken and Grandma Judi's house for Christmas Dinner and gift exchanging. Emma really opened up to her Grandpa and got to see and play with her Uncle Luis. We got home about 8pm and put the kids to bed. Emma woke up an hour later and really could not breath well so Amy and I turned the shower on hot and steamed up the bathroom for her to breath in some warm moist air. We also did a Xopenex breathing treatment. She seemed to breath better after all that and we decided she would sleep with us. Shortly after 1am she awoke with difficulty breathing again and Amy took her to the ER. She was admitted and more breathing treatments were given. She got to come home the next night after all the steroids they gave her reduced the bronchiole inflammation. Today, she is playing normal with a slight cough. She seems to be getting more frustrated today than we have seen, but we are told to expect that from kids her age. We seem to think it has to do with the fact that she is deaf but we just never saw the "terrible twos" with Landon and were not expecting it with Emma. Truth is all kids usually go through this "easily frustrated" stage.

Emma seems to be trying to say "mama" more but that is the only word we seem to notice. But that is also more than she has been saying. Her personality has also developed a lot in the past 6 weeks. She continues to lose more and more shyness as the weeks go by.

12/21/2003-GOOD NEWS TO REPORT!!! It seems as though Emma is taking in sound through her hearing aids!!! We were not sure at first because she did not respond to loud noises but we also remember that hearing aids keep everything limited to a specific volume. Amy worked at the kitchen table with her Saturday for about an hour on saying "mama" and Emma seems to be repeating it. I also caught Emma in the office going through all the CD ROMs and I said "what are you doing" while her back was turned and she turned around and smiled as I said it. She also seems to be soaking up all the sound she is hearing. Amy was changing her diaper and she started making noises that we have never heard. She sounded as though she was trying to make "formed sounds" or the beginning of words. She also is great about not touching her hearing aids. We put them in in the morning and they stay in all day. She actually seems to like wearing them! She will have her first AV Therapy session this Wednesday. We are looking forward to it. We were told not to expect a drastic change in the first month so we are cautiously optimistic. But we think that for Emma to begin repeating "mama" after Amy working with her is a major accomplishment for 2 days!!! We are still leaning heavily towards the cochlear implant. All the studies suggest that Emma will do great with a cochlear implant and I have now heard the language of three kids with implants ranging in age from 2 years to 8 years and I could not tell any of them were deaf. Some had distinct geographic accents. All studies also say that children with severe hearing loss do better with cochlear implants than their counterparts using hearing aids. One study said that the main influential factor in successful outcomes of cochlear implants is aggressive AV Therapy, young implantation age (before 3 1/2), and being put into mainstream schools with hearing peers. If Amy and I had it our way we would not do the implant. But the hard part for us is we have to make a decision based on what we think Emma would want in 10-15 years. And all studies show that 90+% of cochlear recipients are glad and feel they lead better lives because of the implant. Personally, Amy and I are terrified of the risks even though they are very very low. But we have to make a decision based on what Emma would want us to do and that is difficult for any parent. We will continue to work with hearing aids until we get more detailed info (like her MRI and AV Therapy session). We are excited but also guarded about getting our hopes up to much.

12/19/2003-Emma got her hearing aids today at the Callier Center at the University of Texas at Dallas. They tell us that she can now hear all the way down to 40 dB with them but that has yet to be seen at home. We got loaner aids and something tells me they are not the best quality because of all the squealing and feedback they produce. But hey, they were free. The "experts" say that is normal but I told Amy if the feedback and noise from them continues that Emma's first words will be "SSSSSSSQQQQQQQQQUUUUUUUUEEEEEEEEAAAAAAAAAALLLLLLLL!!!!" The right hearing aid and matching ear mold seem to be fine but her left (the better ear) seems to be having all the problems with feedback. I guess she is responding a little more but it still seems too early to tell. Amy said that it feels like we are re-living the day they told us she was deaf. Part of me thinks that the hearing aids are only a temporary solution but We shall see.

We also received some bad news today. CIGNA (the crappiest insurance in the world) told us that they would only authorize 3 auditory verbal therapy sessions. I guess they think that Emma will learn to speak perfectly in three hours. It seems as though we are dealing with two extremes here: experts and complete idiots (CIGNA being the latter). The people at the Callier Center have been our light of hope. Jennifer and Kathy are our audiologists and spend so much time and effort working with Emma and explaining things to us. They are not covered by insurance but are well worth the money spent.

Amy and I are actually going out to dinner and to a movie tonight with friends. It will be the first movie Amy and I have seen together since "Titanic." Of course we have seen all the Disney movies since then but we have not been on a "date" together since----well, we can't remember. Grandma Judi is coming up to keep the kids. Tomorrow we will begin working on getting Emma to listen. We are going to start with her learning the basics (mama, dada, drink). We plan on giving her 60-90 days to respond and then we will move on to the cochlear implant.

12/13/2003-Well today is Saturday. We had further testing done yesterday at the Callier Center at the University of Texas at Dallas. They confirmed all the same thresholds for the third time (80 dB hearing loss). They ordered Emma's hearing aids. Many seem to think that they will help with therapy but we are still moving on as though we are going to go with the cochlear implant. We spoke with the Auditory-Verbal Therapist that everyone seems to be recommending, Brenda Bliss. We can already tell she is going to be a delight to work with. She returns all our calls and takes time to discuss and answer our questions. We are now feeling much more support from the medical community. Landon is feeling more left out given that we are continuously having to leave him with Grandma while we take Emma to the doctor. He sees us drop him off and drive away with Emma. I worry that he feels we are spending "fun" time with her and not him. If he only knew where we were taking her everytime we leave. Before diagnosis, Landon had friends coming over two to three times a week. With the flu outbreak and holiday rush, he has not had a friend over in quite some time. I am quickly becoming his favorite playmate. It is frustrating because Landon needs to be playing with other kids now more than ever. He thinks that, not only are we spending more time with Emma, but I am afraid that he thinks that it is us not letting him have friends over to play. The good news is that he has made some new friends because of Emma's condtion. Our new little friend Megan has a brother named Travis that Landon talks about often. They are coming over Monday to play. He is learning more sign language everyday and uses it as he says "thank you," "please," "boy," "girl," and many other things. He is going to be a great teacher and helper for Emma. Emma is learning to play with her brother. They are going to be great friends. Grandma and Grandpa are coming over tonight to eat some of my homemade Guinness Chili and play Texas Hold'em.

We found a new website with a little girl named Laney Jane Anderson. She is a cochlear recipient at the age of two. Her dad has been recording audio clips of her progress and posting them on the internet. She is now five and HAS A GREAT GEORGIA ACCENT and sings the ABC song. She was profoundly deaf from birth. Her progress is very inspiring! You can see their site by CLICKING HERE.

12/11/2003-Today we got a second opinion from Dr. Paul Bauer. He specializes in Pediatric Otolaryngology and performed our new friend, Megan's, CI surgery. He saw us FOR FREE and spent about 1 1/2 hours with us dicussing Emma's condition. He also said that we should receive out MRI at Children's Hospital Dallas as it is much more high resolution. We discussed specific cochlear implants and which ones were better for kids. There is so much information to digest and I told Amy that we really need to meet more CI recipients that received implants as children to help us make that choice. Dr. Bauer said that he would go with the MED-EL implant if it was his child because he can get the electrode string further into the cochlea than the Cochlear Nucleus device. He also said that the Clarion Corporation has the highest tech and latest technology but that they were the company that had problems with their device causing meningitis in recipients. Apparently they have redesigned this and it has been proven safe. We are still, of course skeptical. The MED-EL is the thinnest requiring less drilling into the skull and it is also the only device that DOES NOT have to be surgically removed before an MRI. Cochlear corporation and their Nucleus device is what 70% of all cochlear implant patients have, is made of titanium, has the most electrodes, most channels, and has the best reputation. They are an Australia based company. MED-EL is Austrian based and Clarion is American. We are told to stay away from Advanced Bionics as they are too hard to program. We also found out that Dr. Peter's cochlear audiologist and programmer lives in our neighborhood about three blocks over.

Dr. Bauer also said today that he was also of the opinion that this was, indeed, due to genetics. Since Emma is otherwise very healthy and Amy had a normal pregnancy with her, nothing else seems to be a probable cause other than genetics. If this is genetic, we can test for what causes 50% of all recessive deafness: a gene called Connexin 26 and Connexin 30.

Barbara Luetke-Stahlman called and said that she was going out of town at the end of the month. Her daughter, Marcy, is going to come spend three days with us. It should be a great learning opportunity for us and I am sure it will also be very inspirational. We are looking forward to her visit.

Amy's mom has been keeping Lando while we go to all the doctor's visits with Emma. She has really helped us in having meals prepared when we get home, keeping Lando happy, and holding down the fort. Landon is doing good now that his cast and splint is off. He now knows the anatomical names of all the bones in the arm (thanks to Leap Pad). He knows something is going on but we still worry about spending enough time with him through all this. He will stay with his Grandma Judi again tomorrow while we get Emma's hearing aids fitted. We hope to have those before Christmas.

We have taken the kids to see Christmas lights twice this week. They love it and we have a blast spending the time with them. Emma has developed a passion for rocking horses ever since our visit to Pottery Barn Kids. When we pass by a house that has a rocking horse as a yard decoration, Emma screams loudly and laughs and points. She seems to be increasing in her vocalization. We have also been hearing her say "Ba Ba" and "Ma Ma" more lately. That is a good sign. She will be 21 months next week and according to ECI's evaluation, Emma is ahead in all areas of development except for communication which she is on a 10 month old level. Socially she is at 30 months (gets that from her mommy) and her cognitive skills are at 23 months which shows that her brain is very effective at processing information. Since we think she has had some hearing at some point and considering her cognitive level, she should learn very well how to communicate. She is also becoming more and more loving everyday giving lots of hugs and kisses to her mommy and daddy.

12/8/2003-Today we saw Dr. Peters for a second opinion. Audio behavioral testing was done as well as an OAE (otoacoustic emissions test) and it seemed to confirm that Emma is severely deaf in both ears. We have scheduled another ABR test for the end of the month to confirm her exact thresholds. We also discussed cochlear implant and all things indicate that Emma is going to be a great candidate. Dr. Peters did say to us "your child WILL hear and speak," and that was all we needed. We also got the opportunity to meet a brave little girl by the name of Megan who has received cochlear implants in BOTH ears. She was profoundly deaf at 6 months and is now two years old. She speaks and it is VERY clear. She is one cute little girl and looks and sounds like she is going to do great. She was an inspiration to Amy and I. Landon stayed with his Grandpa Ken and Grandma Judi while we took Emma to Dr. Peters' office. Grandma Judi made the best fried chicken and mashed potatoes for us. It was so nice to have a home-cooked meal after weeks of eating whatever whenever. Emma is happier than ever and plays so hard. She always has a smile on her face and we now feel comfortable that she is going to be just fine. Barbara Luetke-Stahlman is going to meet us Monday night to discuss Emma's condition and status. Megan's mom has been another angel for us. She has shared her one and a half year ordeal with her daughter with us and all the heartache that has gone along with it. She has been a wealth of information, help, and support for us. We feel like she is long lost family. She also has a son that is Landon's age and they got along great! We are having dinner with them Friday night and it looks like the we are all going to be very close friends for life. Emma and Megan got along great and it was great to see Emma interact with someone her age. Emma seems to really be coming out of her shell. Sunday night she warmed up to her Uncle Luis and last night warmed up to her Grandpa Ken. She flirts and bats her big brown eyes at everyone she sees. For the first time we feel like things are heading in the right direction and everything is going to be alright.

12/7/2003-If you are curious to see how a cochlear implant may sound to Emma once she qualifies and is implanted then click here. Please note that before you hear this the last saying right before the clear speech is spoken without the implant is where Emma should fall based on the specific Coclear Implant we are placed with. Keep in mind many of what you are hearing are only 4-16 channels. The lowest number of Channels Emma will have will be 22+. So her hearing would be better that this link.

12/6/2003-Emma has another ear infection. We know because we saw what appeared to be dried drainage from her ear tube outside the right ear. Amy immediately called Dr. Suzanne who immediately called in some Augmentin for her. We wasted no time in getting her first dose in her. Emma seemed to have a "good" day hearing today. When I went in to get her out of her crib, she was already awake and I snuck in the room. She had her left ear (the better of the two) up and she could not see me. I said "Boo!" at a regular conversational level. No Response. Then I increased my volume to a louder conversational level (but not yelling) and she immediately looked over and sat up. We know that there is hearing loss. If she had normal hearing she would be talking. But we all went to the grocery store together today and in the back seat we heard Emma saying, "Ba Ba Ba." It may simply be more baby babble but I like to think she is trying to talk. She is getting good at grunting and telling us what she wants. Today she was drinking juice in the back seat and began grunting and when we turned around she was trying to hand her juice to her mom. Tonight as we were putting her down to sleep she was pointing to the beanie babies on the shelf and grunting. We have to make sure we acknowledge what she is pointing at and grunting at so that she continues to try to vocalize and verbalize her thoughts. If we do not then she may think that we are not hearing her and give up altogether.

Much more testing is in store for Emma. First, she will have an MRI to help discern the cause and prognosis and treatment of her condition. Next, we will undergo a second ABR to confirm the original thresholds. Next she will have a auditory-behavioral tests done in which she will respond to sound by pointing at lights. Next we will probably have a test to evaluate her cortical auditory response (this test is done while awake and determines thresholds in an alert state). Emma seems to hear better when awake (as most of us do). All the while we will be working with hearing aids and speech therapists to see if we can get any progress. If not we will immediately begin the process of getting her a cochlear implant. Studies show that the younger the child receives an implant, the better they do in their lingual development. My goal is to have her speaking by age 2 1/2. I want to hear words by then. Amy and I will not rest until we know that is going to happen. We have also decided to teach her to sign as well as speak. We want Emma to be accepted in both the deaf and hearing cultures. We also see it as an advantage to her in the workforce. She will essentially be bi-lingual (and if she learns Spanish like her brother she will be multi-lingual!) We like the idea of participating in both cultures as a family.

12/1/2003-Today we set up an appointment with Dr. Brian Peters at Medical City Dallas so Emma could be screened as a cochlear implant candidate. We had a long and very imformative conversation with one of his staff who said that further diagnostic tests were warranted. It is so strange how we feel as though we were abandoned in this process by the medical community soon after diagnosis. We have been essentially shooting in the dark at what happens next. Emma's pediatrician and staff have been the most helpful in spending long conversations on the phone with the insurance company trying to get "permission" for her to have a chance at hearing. We realized today that we have been running in circles and are learning quick that there is a large part of the medical community that could care less about your child's hearing and are more interested in how to use your emotions to get into your wallet. Apparently further diagnostic testing is warranted before we make a move on hearing aids or cochlear implants. We need to test Emma at different pitches to determine exactly what she can and can not hear. I DO know that she could hear me coughing last night because everytime I did she would copy my cough. Amy has been working hard at teaching Emma to sign and setting up appointments. Emma seems to be picking it up rather quickly. We also SPOKE to a cochlear implant recipient ON THE PHONE tonight and could not tell that there was anything wrong with her hearing or speech. One of Dr. Peter's staff that works closely with the implant program said that often when PROFOUNDLY deaf children are implanted early enough they grow up to speak and hear on such a level that you can not tell they are deaf and they do just fine in school. We were also told that most insurance companies WILL cover cochlear implants. We hope and pray that ours is one of them!!! The other good news is that if Emma is a candidate for the program, she can participate in the hearing aid "loaner" program for $80 a month----much better than the $4000+ we were going to shell out to try hearing aids not knowing if the would work or not. Our hopes are high that Emma will hear again someday.

I have also been researching the many possible causes of Emma's hearing loss (dad has also been researching and has found much "interesting" information) and have come up with possible causes:

Sensorineural Hearing Loss Causes

1. Genetics-(A frameshift mutation in a gene called Connexin 26 is the cause in 20% of cases). This is also unlikely since the gene is passed through heredity and given the lack of deafness in family history, it is unlikely the case. Irreversible.

2. Auto-Immune Inner Ear Disease- Caused by the body's immune system attacking a specfic part of the body. Can be caused by Systemic Lupus Erythematosis (SLE) or many other immune disorders. (This is what Rush Limbaugh has and sounds most like Emma's symptoms to me and also possibly reversible with high dose steroid therapy).

3. Mondini's Malformation- a normal cochlea has about 2 3/4 turns (like a snail) and in this disorder there is only a small level of turns. Irreversible and not a great cochlear implant candidate

4. Meniere's Disease- usually has loss of balance symptoms, comes on in attacks that result in dizziness, nausea, and vomiting although 1/3 of these patients do not have these symptoms. Caused by an inner ear rupture between membranes and the mixing of perilymph and endolymph. Irreversible.

5. Neurofibromatosis- This usually occurs only on one side but can be "bilateral." These are small benign tumors that grow on the auditory nerve. These tumors also tend to grow on other nerves causing other problems with eyesight and other symptoms (obviously not likely here but still possible). In rare cases it is reversible through removal of the tumors but at high risk.

6. Viral infection- Herpes, CMV (cytomegalovirus), Varicella (Chicken Pox), and the Mumps viruses can cause this but most likely only if the baby is delivered vaginally. Emma was born C-Section so again unlikely. Irreversible.

7. Aminoglycoside antibiotic use- antibiotics such as Tobramycin (Tobradex often given in eye drop form for pink eye) and Gentamycin are in a class of antibiotic that can cause "ototoxicity" when given IV or in high doses. This was the cause of deafness for Miss America, Heather Whitestone McAllum. Emma DID receive antibiotics last winter when she was hospitalized for three days with a "viral" G.I. infection. HOWEVER, I DO NOT KNOW IF THIS IS THAT CLASS OF ANTIBIOTICS THAT WAS USED. WE WILL SOON KNOW. Sometimes antibiotics are given to restore the normal bacterial balance in the G.I. tract. We have requested Emma's file from the hospital and will be exploring this further.

8. MMR vaccine- has been linked (which does not imply that it was the cause---just a link) to deafness in six pediatric subjects since 1979. This is VERY unlikely but still a possibility. This is a vaccine given to kids at one year of age. Was also implicated in Heather Whitestone McAllum's deafness. Irreversible.

9. Acoustic Trauma- Very unlikely since she has never been around much loud noise other than her brother!

If anyone comes up with any other possible causes please email me here.