The day after...

There is a lot to say about our appointment yesterday but not enough time to write it out yet. The abbreviated version is that Dr. Antonelli has agreed to take Lily on as a patient at this point and he and his team will do a study on her. There will be lots of testing to be completed and then he and his team will have a meeting to discuss her implant candidacy. I liked him. I was so worried that I wouldn't like him but he was nice.... -Kriste

3 days and counting

There are three days left until we go to see Dr. Antonelli at UF Shands. Part of me is terrified that he will say that she is not a candidate. The other part of me is terrified that he will say she is a candidate. The surgery seems scary and it is hard to imagine them shaving off half of Lily's pretty blonde curls and making a permanent, quarter-sized hole in her skull.

Part of the process in getting ready for this appointment has been gathering up copies of all the evaluations that Lily has had and of all the audiological testing. In an earlier post, I described my memory of Lily's early tests. It turns out I was pretty close. Lily did pass the newborn screening in her right ear and it was because she was too awake that testing on the left ear was incomplete. It was 3 weeks after the newborn screeening (not 6 like I thought) that we were at the audiologist. The report written by the audiologist says that Lily passed the ABR in her right ear. It says with more description what parts of the ABR she passed in her left ear but does not come out and clearly say that she fails in her left ear. The OAE says she passed in her right ear and that further testing is necessary for her left ear. There is some terminology and a few graphs that I am unable to interpret at this point. (I recently did an audiology quiz on a site for audiology students and I did pretty well. Not an expert by any means but I know a lot more about ears than I ever thought I would.) I am hoping that Dr. Antonelli will be able to explain to us what he thinks about those tests from 2 years ago.

It is very likely that we will never find out why Lily has hearing loss. There is only one conclusion to make about Lily's hearing when you compare those early tests to Lily's most recent ones: her hearing loss has been progressive. There are many possible causes of progressive hearing loss, such as Unknown Nonsyndromic Genetic, Syndromic Genetic (like Enlarged Vestibular Aqueduct Syndrome) , Meningitis, congenital Cytomegalovirus (CMV), Neural degeneration, Teratogen (Chemotherapy), Low birth weight/premature, and Perilymph Fistulas. I haven't explored all of these causes yet and am not sure if I ever will. Some are easy to rule out, like meningitis, Perilymph Fistulas, and chemotherapy. She has never been on any antibiotics (of which some are ototoxic) and has never had much more than a minor cold or stomach bug. If Lily is a cochlear implant candidate, then we may find some answers. Or not.

I talked on the phone today with the mother of a 15 year old girl here in Tallahassee who has bliateral implants. Her daughter received her first implant at age 2 and was, at the time, the youngest person in Florida to receive an implant. Children as young as 6 months are receiving implants in some parts of the country these days. Anyway, we are meeting with this family on Wednesday evening next week. It is my hope that we will have Deaf/Hard-of-hearing role models for Lily as she grows up. Maybe this girl will turn out to be one of them.

Oh, and we are still waiting for Lily's hearing aid but I have recently had a lot of help from our service coordinator/Program Director of Early Steps. She tells me that I am keeping her on her toes. She has been talking with Lily's audiologist and with someone from the State of FL and it looks like we will likely be able to get even more financial help with Lily's aid. We have been told to hold off until after our appointment with Dr. Antonelli because they think that he will want to aid both ears initially. I am not sure why there has been such a struggle in getting this aid. Surely there are other parents of children with hearing loss that have been through this process. 1 in 600 children in Florida each year are born with hearing loss. There were 3900 babies born in Tallahassee last year. That means roughly 6 kids per year, which means about 18 kids 3 and under that are served by the Early Steps/SHINE program. I just don't understand why it seems like they don't know what to do with us. Maybe I ask too many questions....

-Kriste

Cochlear War

I have been almost forcing myself to read the other side of the argument regarding cochlear implants. This argument comes from the Deaf community. It seems important to understand their views, since they have actually know what it is like to live the life of deaf person, and I have no firsthand knowledge of this. For Lily's sake, I have sought out the deaf persepective. I came across this site, Cochlear War, which has an open letter to parents who are considering implanting their prelingual children. It was an eye-opening thing to read and gives me lots to think about in this week before our appointment with the cochlear implant surgeon.

There is a lot to read in that letter and I have read it a couple of times now. In my last re-reading, I picked up on this part, which I missed the first time around. Makes me feel better somehow...

We don’t believe that a cochlear implant should be installed in prelingually deaf babies and young children. If a child already has experience in hearing and speech before losing her hearing, and has become profoundly deaf as a result of sickness or trauma after learning how to speak, or was born hard-of-hearing but lost her hearing gradually, we have no objection to the implant, because the child is simply reconnecting with her previous mode of communication. That child already has a basis for spoken and aural language. An implant will enable the child to build on the existing foundation.

On the opposite of the spectrum, this story came out about a little girl in Boston who was just bilaterally implanted: Special Ear Implants Help Girl, Born Deaf, To Hear. There is a short video to watch and you can hear her converse just like a hearing person. I wonder how hard she had to work to speak that clearly. I wonder if we can pull off Auditory-Verbal Therapy in a way that won't make Lily need therapy as an adult.
- Kriste

The background on Lily's hearing loss...

There is a bit of background on Lily's hearing loss that we haven't mentioned yet and is an important piece to this story. Lily was born at The Birth Cottage on the bed in the Hydrangea Room on June 26, 2004 at 9:15pm. It was a normal birth, although much harder than Charlotte's birth (that is probably because Lily had a 15.75" head!). Because we were not at the hospital, Lily did not have a newborn hearing screening right away. Chris switched jobs not long after Lily was born and we were in insurance limbo for a few weeks.

We traveled to Tallahassee Memorial Hospital when Lily was 6 weeks old for her to finally have the newborn hearing screening. Her hearing was tested with the ABR test, or Auditory Brainstem Response. Her right ear was checked first and she quickly received a "pass" for this test (remember- this is the ear that has the profound loss!). She was deeply asleep for this ear. When it came time to switch to the left ear, Lily woke up a bit. Babies need to be asleep for this test to be accurate. We went ahead and tested the left ear even though she was not fully asleep. It took forever for this ear and the test administrator had to keep adjusting the wires that were attached to Lily's head because she was wiggling so much. After a long trial, the tester indicated that she didn't think we were going to get a good reading that day and she was going to have to refer us to Audiology Associates for further testing. I was glad to go, as I had a 2 year old Charlotte along with me and she was ready to go as well. The tester said she thought that Lily's abnormal results in her left ear were likely due to Lily's squirminess and that there was nothing to worry about.

The appointment was set for us with Audiology Associates for a date 6 weeks later. Since there was no emergency on their part to get us in as soon as possible, I still had little concern about Lily's hearing. I have not yet seen Lily's complete file so I am not positive about what tests were given to Lily that fall day in 2004. I can only guess at this point what tests she was given that day based on what I now know about audiology. I believe that the audiologist tested her ears first with the Otoacoustic Emission Test (OAE). She did not share the results with me and I didn't know anything about audiological testing then so I didn't know what to ask. Since Lily's left ear was the questionable one from the newborn screening, the audiologist started the ABR test on this ear. Once again, Lily was very difficult to test. She was squirmy and restless and her normal cranky self. Each time Lily squirmed, the test had to be reset and started over from the beginning. We were there for over 2 hours. Finally,the audiologist told me simply that the results were "inconclusive" and that it was hard to get a good reading due to Lily's restless behavior. She did not tell me that there was something wrong with Lily's ears (something that she could tell from the OAE), she just told me that further testing would be necessary in a few months when Lily was older and could be sedated for testing. That did not sit well with me- I did not like the idea of sedating my infant for a test that didn't seem necessary. I blew this test off. The audiologist missed her chance to tell me how important it was for us to find out definitively if Lily had hearing loss or not. Telling me that the results were "inconclusive" was just not enough information. Had she explained to me that sometimes babies are just born with unexplainable hearing loss and had given me the pamphlet I later saw this past June in the ENT's office about infant hearing loss and signs to look for, I might have taken all of this more seriously. Believe it or not, we are still seeing the very same audiologist.

In my mind, Lily was a colicky, very crabby, hard to soothe baby. That was why she didn't get normal test results. She didn't have a hearing problem. How could she have a hearing problem? We don't have any known risk factors. She was not a preemie -she was a 4 days late 9lb4oz baby. There was no birth trauma- she was pushed out in 25 minutes with no drugs. As a matter of fact, I did not take anything harder than an occasional Tylenol during my pregnancy with Lily. She has never had an ear infection, we have no known relatives with childhood-onset deafness, she never had any high fevers. The only thing close to a pregnancy complication was that Charlotte had Roseola when I was 25 weeks pregnant with Lily. Charlotte endured 105 degree fevers and febrile seizures with this illness. I came down with a cold or some virusy thing towards the end of Charlotte's bout with Roseola. But it seems unlikely that this would be the cause since babies are generally protected from most illness in utero.

I will admit that the questionable test results planted a seed of doubt in my mind about her hearing. At each well-baby check-up, I would ask our family doctor his opinion. He also agreed with us that it was not a great idea to sedate a tiny infant. He also agreed that since she had one ear that easily passed the newborn screening, even if there was something wrong with the other ear, she would get the sounds that she needed from her "good" ear and that we could do further audiological testing with her when she was older and no longer needed to be sedated for testing. He even did an old-fashioned tuning fork hearing test on her when she was about a year old because I brought the hearing worry up at her 1 year check up. She responded to the tuning fork being struck behind her back, but I now know that this is a common way for unknowing family doctors/pediatricians to check for hearing loss. Deaf kids are incredibly good at picking up on visual cues. She probably noticed my eyes following the doctor behind her or she watched to see what Charlotte was looking at or she noticed subtle movements that the doctor made.

So that pretty much explains the history of Lily's hearing loss. The doctors believe that her loss has been progressive, although we are not sure about that. Lily has from day one been a difficult baby. When she was a day old, I had this feeling that there was something off about her, although I never would have guessed hearing loss. There was something about the way her eyes darted all over the place instead of focusing in on me that seemed strange. She stared so intensly at lights when she was tiny and could not fall asleep if there was any light in the room, not even the lights from the clock on the VCR. One of the signs of infants with hearing loss is being unable to soothe your child by voice alone- there was no soothing Lily by speaking softly to her. It is hard to look back at pictures of Lily as an infant. There is a blank look on her face that I recognize now as the face Lily makes when she is not sure what is going on around her because she cannot hear. As Lily got older and more mobile, it seemed as though she had an invisible steel helmet on. She felt very distant to me and so unbelieveably stubborn. Now we know why.

-Kriste

Maybe next week will be the week...

It looks like we have been reassigned and the program director of CHS has taken our case. She has been out of the office for a week so this explains last week's continued delay with the new aid. Maybe this upcoming week will be the week when everything falls into place. From what I understand, CHS must get a denial from our insurance company regarding hearing aid coverage. I was told by our very first service coordinator that all they needed was a verbal denial but it appears that this is no longer true and there must be a written denial (and I hear that this is a new rule imposed by the state). From what I have learned from a Yahoo group for parents of children with hearing loss, hearing aids are denied by insurance companies everywhere and across the board. Seems silly to need a written denial for something that insurance companies are known to deny.

The Dora doll has made all of the difference. Lily has consistently worn her aid since Dora joined the household. She has even asked to wear her aid a couple of times! Maybe she is starting to realize that she has been missing out all this time...

-Kriste

Bilaterally implanted Miss America and more hearing aid waiting

So it has been a month and we are STILL waiting for Lily's hearing aid. It was July 10 when we choose Lily's pink Oticon hearing aid. Our new service coordinator, whom we have never even met, has resigned, and with her resignation comes more waiting for the proper paperwork to go through to get a little financial help with Lily's aid. In reality, Lily needs 2 aids and if we could just get our hands on the first aid then I could talk to the audiologist in person about why she does not think it is important to aid Lily's right ear. Even with a loss as great as hers in her right ear, she would still benefit from stimulating the auditory nerve. It is frustrating because sometimes it feels like we are dealing with people who don't really care every step of the way.

A new Dora the Explorer doll that can only be held when she is wearing her hearing aid seems to have made the difference today. Lily wore her aid for most of the day. It felt terribly manipulative but we are desperate to get her to wear her aid.

In other news, Heather Whitestone, the former Miss America, has been bilaterally implanted. Here is a story that came out this week about it:

Life -- now in 'surround sound':Ex-Miss America has 2nd implant to aid hearing
By Chris Emery August 8, 2006

Just before doctors turned on her new bionic ear yesterday, Heather Whitestone McCallum saw that her son, John, 6, had lost a baby tooth as he ate a sandwich.

When the device was activated, McCallum got to hear John explain what happened. "A seed in the bread took it off," John said, holding the tooth out to show his mother, who smiled and nodded.

"I like his voice," said McCallum, who underwent cochlear implant surgery six weeks ago at the Johns Hopkins Center for Hearing and Balance in Baltimore and returned yesterday for the activation. It was the most sound she had heard through her left ear since it was damaged by a bout with haemophilus influenza that left her deaf in both ears when she was a child.

McCallum, 33, is an Atlanta resident who in 1995 became the first Miss America with a disability. She had her first cochlear implant, in her right ear, in 2002, and is one of a growing number of people with little or no hearing who opt to have both ears augmented with the devices, which convert sound waves into electrical impulses and transmit them directly to the inner ear.

Strange sounds
After the first operation, McCallum used a hearing aid in her left ear in concert with the implant in her right ear. Some sounds took her a while to identify. Once, sitting in a car with her husband, she heard something she could not identify. "That was me sniffing," he told her.

Other sounds, such as her sons' screaming when they wrestle, were disconcerting. "They weren't in pain, but I had never heard it and didn't know that," she said.

Six months ago, when McCallum completely lost hearing in her left ear, she decided she wanted to get an implant in that ear, as well. "Suddenly, I misunderstood what people were saying to me," she said. "People had to struggle to communicate with me, even my boys."

Having two implants will allow McCallum to hear in "surround sound," an important ability for a mother raising two children, said Dr. John K. Niparko, the Hopkins surgeon who put in McCallum's new implant. Hearing with both ears helps people pinpoint where noises are coming from and focus on one speaker in a noisy room, Niparko said.

He said about 10 percent of the 100,000 people worldwide with implants have them in both ears.

Cochlear implants, developed in the 1970s, now consist of two parts. One is surgically implanted under the skin near the ear, and the other, which is detachable, rests on the ear and is held to the skin by a magnetic attraction between the parts.

Digital signals
The outer portion of the device captures sound waves, converts them into digital signals and beams them across the skin to the internal portion.

From there, minute electrical signals travel down a wire into the cochlea, the snail-shaped portion of the inner ear, where they stimulate the nerve cells connecting to the brain.

When implants were developed, they could stimulate only a few frequencies of sounds in the cochlea. McCallum's implant, on the other hand, can transmit up to 24 frequency bands. "There is a spectrum of sound that is missing, but it is much better than nothing," said Niparko.

The operation, including purchasing the device, costs about $35,000 and is covered by many insurance plans, and by Medicare and Medicaid, he said. People who understand fewer than one-third of words spoken to them might qualify for the implants, he said.

Yesterday, Hopkins staff members first calibrated McCallum's implant by plugging it into a computer and stimulating different tones of sounds inside her ear. Jennifer D. Yeagle, an implant audiologist, tested the 24 frequencies' bands of sound to see which ones McCallum could hear.

"She seems to be hearing the high frequencies better," Yeagle said. "That's because those are the ones her brain hasn't heard in the longest time, so it's noticing them more. As [the neurons] wake up after all these years, they will adapt to what they are hearing."

Later, when the computer was disconnected and the external portion first connected and turned on, McCallum said people's voices sounded high-pitched.

"You sound like a 3-year-old girl," she told Yeagle.

A few minutes later, she said voices were starting to sound more normal and turned on her older implant along with the new one.

"They have their own sound when they are apart, and a different sound when they are together, " McCallum said.

"When they are together, I love it."
________

-Kriste

Trip to Gainesville setup

Kriste emailed Dr. Antonelli one morning and got a reply 30 minutes later.

Since then she got an out of town referral form and we will be making the 300 mile roundtrip to Gator land. technically I think it is a 2nd opinion form... No one like him in Tallahassee and Shands one of the best.

We will be at Shands on August 28th. We hope that Dr. Antonelli will consider Lily a cochlear implant candidate. She is likely a borderline candidate since she has some residual hearing in her left ear.

-Chris

Blues Clues, Scottish Rite and "Mine"

Lily said - "I want mine." (sounds like "I wah my") today - referring to a bowl of cut up melon. Her sister had a bowl too. She also has picked up on "in" and "on" concepts. like put my clothes "on". put your foot "in."

Lily saying "Cheese" for camera

She cannot stand her hearing aid - I will say Lily do you want your hearing aid? And she will run away and say - no hear, no ear, no horsey..."Hearing" sounds like "horsey" I guess. (Lily's version of the word horsey sounds like "whforfin" but we know what she means)

---

No new hearing aid yet... since Children Home Society rep left and didnt send in paperwork... apparently they will pay $500 of cost... why just $500 and why doesnt insurance cover ? Luckily we have credit cards and at this point what is the difference between $2000 or $1500?

---

Lily loved the Blues Clues movie on last night... Charlotte loved it more but it caught Lily's attention for a good stretch.

---

Good News for Tallahassee - (I copied whole article bec it will soon disappear)- Kriste met the girl and mom in the article on August 1 at the library.

---

FSU speech and hearing clinic awarded $20,000
Civic group pledges future endowment totaling $500,000

By Diane Hirth
DEMOCRAT SENIOR WRITER

Jennifer Koch's daughter Karen Moore lost her hearing at age 3 and a half and was deaf for a year before receiving a cochlear implant in one ear.

In her year without sound, the little girl regressed to a 16-month-old's language level.

However, with the help of auditory therapy at Florida State University's L.L. Schendel Speech and Hearing Clinic, the 8-year-old has been catching up. She will be in a regular second-grade class this fall at W.T. Moore Elementary School.

"I love my clinic," Koch said.

The clinic on FSU's campus received a $20,000 check Wednesday from the Scottish Rite Foundation of Florida. The money will be used to increase the number of area children with speech, language and hearing disorders who are diagnosed and treated.

The Scottish Rite Foundation's commitment to the FSU clinic goes beyond the one-time contribution.

"When we come, we come to support this forever," said Robert Goldsmith of Jacksonville, Scottish Rite Foundation of Florida president.

His group will raise money for a $500,000 endowment to support the clinic's work with children in Gadsden, Leon and Wakulla counties. With adoption of the FSU clinic, the Scottish Rite Foundation will be associated with 14 such clinics in Florida.

Stephen Berry of Tallahassee, who is associated with the foundation, said, "It's a dream of ours" to support the clinic's work with children.

Part of FSU's Department of Communication Disorders, the clinic is a teaching and research tool in addition to a service for about 300 children and adults a year. About 18 faculty, 75 undergraduates and 100 master's and Ph.D. students are in the department. The clinic also works with schools, health care facilities and private clinicians.

While some clients pay through their private insurance or programs like Medicaid, "We also provide services at low or no cost," said clinic Director Juliann Woods. Thanks to the $20,000 gift, "That's where we're able to expand," she said.

"There's a critical link between language skills and reading and ultimately school success," Woods said.

One goal of the clinic is to create summer camps for children with communication disorders.

"I hope to create an autism camp," graduate student Nikki Greenwald said.

Florida State University's L.L. Schendel Speech and Hearing Clinic provides diagnostic and therapy services for children and adults with speech and hearing disorders. Call 644-2238 or see http://speechandhearing clinic.fsu.edu for more information. Fees are charged only to clients with the ability to pay.

To contribute to the Scottish Rite Foundation of Florida endowment fund for the clinic, call Robert Goldsmith at (904) 378-8957.
--------

-Chris

Appointments, appointments, and more appointments

The real whirlwind of appointments and phone calls began in early June.

We were assigned a "service coordinator" through Children's Home Society. Probably not important enough to mention but the the service coordinator was actually assigned by the SHINE program, which is an off-shoot of the Early Steps program, which is an off-shoot of the Children's Home Society. Our initial service coordinator promised many wonderful and helpful things, but in the end, was not really able to deliver all she promised- most likely due to having too many cases and not enough time (and I imagine she was not paid enough either).

Actually, she has already left her position and a person equally as overwhelmed has taken over her cases as of mid-July. The service coordinator is supposed to be someone who helps with coordinating appointments for a family, attending appointments if need be, and just generally a helpful guide through the early days of discovering that your child has a disability.

Here is a quick run down of all of our appointments thus far:

April 13- Appointment with FSU's First Words Project, where we discovered for sure that Lily was language delayed. Also was noted that the "quality of Lily's utterances" was very nasal and unusual.

May 25- Lily has OAE hearing test. Audiologist recommends more testing at a later date. I leave not knowing the name of the test, just that the results were far from normal.

June 8- Lily and Charlotte and Kriste see Dr. Roberts, an ENT, at Tallahassee Ear Nose and Throat. The only ENT center in town. It was a nightmarish appointment with Lily and Charlotte battling over lollipops during the entire appointment. I left knowing that Lily likely had a "moderate" hearing loss based on the OAE- that was devastating enough news. I also left thinking that I didn't want to see that ENT again as he had no rapport with children - He yelled down the hallway - "I am going to need a holder!" He had to hold Lily down physically just to check in her ears... a task done without difficulty by our family doctor, and audiologists.

June 15- Lily has a soundbooth hearing test, which gives us a more complete picture of Lily's loss. This is the first time I see Lily's audiogram and hear the words "profoundly deaf in one ear and moderate-severely deaf in the other". Really devastating news.

June 20- Lily has an evaluation at the Children's Home Society to qualify her for services. It is a formality though as she already qualifies due to her level of hearing loss. The first sentence of the evaluation was "This is a large child" Her weight 38.6 pounds, length 38 inches...97th percentile."

June 22- Lily has a 2 year check up with our family practitioner. We tell him the news. We hope that he will suggest to other parents to be more aggressive if they have a concern about their child's hearing (there is more to this story but will share in a later post)

June 26- Lily's 2nd birthday and appointment with a new ENT. Since the first ENT appointment went so badly, I thought it might be wise to meet with another one in the practice. This one had better rapport with Lily but he was not very helpful. He didn't tell us anything we didn't already know and he didnt make any suggestion that Lily was a cochlear implant candidate. He told us that further testing , MRIs, genetic testing really wasnt necessary and that we don't really want to know why Lily is deaf. If we find an answer, it won't be something we want to hear he said. He also did not suggest a 2nd hearing aide to stimulate the right ear and he said "see you in 4 months".

June 29- More hearing testing and confirmation of what we already knew- Lily is deaf.
We are given a loaner aid today with no instruction on how to use the aid or take care of it. A mold of Lily's left ear is also taken. The audiologist informs me that there is no need to aid Lily's right ear, as it is non-functional. We hear later that this is not true, that the auditory nerve needs to be stimulated even if only loud, environmental sounds are heard.

July 6- A representative from the SHINE program came our house to explain all the options in deaf education. Options ranging from ASL to Total Communication to Auditory-Verbal Therapy are presented. We ultimately choose AVT, which we hope will give Lily the most normal life possible.

July 10- Lily has an appointment to receive her new ear mold. We chose pink and white swirls. The audiologist gives us a different loaner aid until Lily's real aid is ordered and received. She calibrates this loaner aid in Lily's ear (which was loud and scary)and Lily is terrified. From then on, getting the aid into her ear is nearly impossible.

July 14- The SHINE representative returns and Kriste shares with her what communication method we have chosen. Auditory verbal. We hope that this is the right choice for Lily. Some days I see it as the only choice. There is an ongoing debate over Sign Language vs. AV - and whether a person can fit in to the deaf community if they get implant or use AV...or if you are deaf and don't know sign language you cant communicate with other deaf people. Right now with her limited hearing she can say many words so we feel like this is the way to go.

July 21- We meet Janet Kahn of FSU for the first time finally. Childrens Home Society covers cost until age 3 (we hope!). We spend almost 3 hours at FSU as Lily is tested to determine her language capabilities. Lily keeps her hearing aid in the whole time and does pretty well. The deficiences are evident in following multiple step directions, pronouns, prepositions.

July 31- Lily's first AVT session. It will be mid-September before we have another session because of FSU schedule and it is now up to us to keep the momentum going.

-Kriste