Archive for February, 2009

The Mountain

Tuesday, February 24th, 2009

Both of my girls and I have always worn glasses.  Thus, in July 2006, we went together for our annual ophthalmology appointments.  It was the first year we had switched doctors, and we were seeing two new doctors at Emory, the hospital where Elliot works.  I went in to see one doctor while Rachel went into another.  Jessica was to follow Rachel.  When Rachel and I met up in the waiting room waiting for our eyes to dilate, she asked me if anyone had ever said anything before about her having a constricted visual field.  I felt my stomach drop but tried to remain outwardly calm for Rachel’s sake.  Early on, we had learned of a genetic cause of deafness, Usher Syndrome.  All of those with Usher Syndrome type 1 are born profoundly deaf with vestibular issues.  However, all those born profoundly deaf with vestibular issues do not have Usher Syndrome.  We had found many traits on my mother’s side of the family of another genetic syndrome which can include vestibular issues, and so that is what we had thought and hoped we were dealing with.  Usher Syndrome includes retinitis pigmentosa (RP), the gradual deterioration of the retina.  Most children with this syndrome are identified with RP by age 10.  However, even though the ophthalmologist we had been seeing knew that I was concerned about it, it had gone undetected until Rachel was 19.  As difficult as it had been 19 years earlier to get the news of Rachel’s deafness, learning that both of my girls had Usher Syndrome was far worse.  Only Rachel understood it at the time, and watching her crying and trying to cope with the news was the most painful thing I have had to live through.  Finding this out a month before she was to start her freshman year at a visual arts college only added to the pain. 

I called my husband, who was across the street in another Emory building seeing patients.  He left them with his physician’s assistant and called the Chief of Ophthalmology while he was on his way over, and he met us with Elliot.  While the assistants in the office where we were insisted that the retinal specialists were too busy to fit Rachel in that day for conclusive testing, it was one of the few times we used connections and let the Chief of Ophthalmology take them aside and tell them that they would fit Rachel in.  Thus, a very long day ensued, with Rachel going through an electroretinogram and a visual field test.  Both confirmed the news of RP.  What didn’t help was the retinal specialist who looked at the tests and gave us the results.  She epitomized a bad bedside manner as she yelled at Rachel, asking her how she could not have noticed that she had lost some of her visual field.   I made the decision right then and there that we were never seeing that doctor again. 

We had started out our “routine” appointments at 8:00 that morning and did not get out of Emory until 2:30 that afternoon.  While we wanted to go home and be left alone, we couldn’t because Rachel’s friend from France was on a plane all day making his way to Atlanta to spend two weeks with us, and we had to go straight to the airport to meet his flight that was landing at 3:00 p.m.  While, in some ways, having company then was difficult, in other ways it was a good thing because it forced us to go on with life and even enjoy it.  We not only toured Atlanta but also took her friend to Washington, D.C., for a few days, something only Rachel had done before, and so Adam and Jessica also enjoyed it. 

Elliot stayed behind and spent the two weeks calling every retinal expert in the U.S. to pick their brains.  He learned of the first ever Usher Syndrome conference planned for that fall and attended the conference in October.  Through the conference, he learned of one genetic mutation that causes Usher Syndrome that is classified as Usher 1f and runs in 2.5% of all Ashkenazi  Jews, which both Elliot and I are.  The gene had only been identified three years before and, therefore, even though we’d had genetic testing done twice before, our mutation had gone undetected.  At the conference, Elliot had the good fortune to meet the researcher who had our blood samples.  Although it usually takes months to even a couple of years to get results from this lab, the researcher pulled Rachel’s sample when he returned to work and called Elliot to let him know he had found two copies of the Usher 1f mutation in Rachel’s sample.  A lab at the NIH, in conjunction with a lab at the Technion in Israel, is studying our gene, and so the NIH performed DNA testing on the rest of us except for Adam.  Not surprisingly, they found one copy of the mutation in Elliot and me and two copies in Jessica.  Later testing through Harvard found that Adam is not even a carrier.  Talk about luck of the draw!  He had a 2/3 chance of being a carrier like Elliot and me but had escaped it.

One amazingly coincidental story that is worth mentioning – When I first learned of Rachel’s Usher diagnosis, I joined a few Usher and RP listservs on YahooGroups.  On one of those listservs one day, a mother wrote in.  She said she was from Ottawa and had a daughter with Usher Syndrome type 1 who was now 22 and had gotten a cochlear implant at NYU when she was four years old.  Rachel was only the 13th child to receive a cochlear implant at NYU, and so I knew that this girl from Ottawa had to be the same one whom Judy Simser had mentioned to Lea Watson so many years before.  I emailed her mother, and sure enough she was.  Both Rachel and this girl have since connected via email and Facebook.

While Rachel struggled initially with the diagnosis, especially once her friend from France left, she once again proved her strength when she picked herself up, said that she had never let her disabilities stop her from doing what she wanted before and wasn’t going to let this latest hurdle stop her either.  She is now in her junior year at SCAD and is an outstanding documentary photographer.  You can see her portfolio online at http://rachelchaikof.com/portfolio/ .  She has continued to travel and is easily one of the most independent women I know of any age.  She has traveled by herself to several areas in France, staying with host families there, to Italy to meet a family with a son with a cochlear implant, to Australia to meet an online friend of mine who has a son with a cochlear implant, to New Zealand where she met another implant recipient, and to Israel.  She is now planning four weeks this summer in South America where she will participate in intensive classes in Spanish, which she hopes will increase her chances of acceptance into the Peace Corps after she graduates from college in the spring of 2010.

Rachel has continued to be an advocate for cochlear implants, both through www.cochlearimplantonline.com, through speaking up on other blogs and through becoming a Cochlear Awareness Volunteer.  This past December, while in Australia, she gave several talks to the employees of Cochlear Corporation about her life as a cochlear implant user.  Then, Cochlear flew her to Adelaide where she gave a talk about bilateral cochlear implants at an Auditory-Verbal center there.  Also after her freshman year of college, she won the Graham Clark Scholarship Award.  The Graeme Clark Cochlear Scholarship Foundation recognizes the remarkable achievements of Nucleus cochlear implant recipients annually by providing tuition assistance.”    

Rachel cites her philosophy in the form of a quote, “Today is a gift.  That’s why it’s called the present.”  That is how she views her RP.  There is much promising research into an effective treatment for RP that Elliot and I follow very closely.  One particular treatment is already in phase II/III clinical trials, and our hope is to get Rachel into the next phase of clinical trials, although we don’t yet know when that will be.  There is so much promising research, including gene therapy, stem cell transplants, and drugs, that we believe there will be an effective treatment in our girls’ lifetime.  It just can’t come soon enough.  Meanwhile, Rachel and Jessica continue to live their lives from day to day, taking full advantage of all that life has to offer.

 

Jessica and Rachel, Paris, Summer 2008

Jessica and Rachel, Paris, Summer 2008

Catching Up– Quickly!

Monday, February 23rd, 2009

When Elliot was about 4 or 5 months old, Kevin and I went to a week long session to learn S.E.E., which is Signing Exact English. It was overwhelming. I remember that I was lost most of the time, barely able to recall a few words and parts of songs. I was trying to take care of my baby at the same time, and that was a full-time job. Kevin did a little better than me, but in the end when we would catch Elliot’s attention (because babies at that age do not have the visual acuity and awareness to watch moving hands much) we never could remember the signs were for what we wanted to say. At the same time I was learning more about Auditory Verbal Therapy. I distinctly remember riding to Indianapolis with Kevin saying “so why did we take that class, then?!?” when I explained the tenets of AVT and why I thought it made the most sense. At the time I could only say that I was trying to figure out what we were doing and how to proceed. We had lunch with a parent at the workshop whose son had a common cavity rather than a cochlea and was getting a tiny benefit from cochlear implants… what if that happened to us? We also met a teen who had been implanted as an older child, and we could see the struggle to communicate. It became immediately evident that, while the world assumed that we would learn sign language to communicate with our son, it would not be a way for us to truly communicate WELL with him.

In early October, the Fort Wayne newspaper covered our story:

Hearing ability will develop as boy grows

A 9-month-old is the state’s youngest to receive a cochlear implant.

By Nancy Nall
of The News-Sentinel

In her “crying in the shower moments,” Amy Kwilinski thinks of the beach.

The beach, where one of the rituals is taking off your watch, your jewelry, and any other valuables that might be harmed by exposure to sand and grit and water.

“But how do you tell a child to take off a $5,000 device that allows him to hear?” she wonders. It’s not the device she worries about; it’s her son Elliot’s ability to experience the beach the way everyone else does.

Those moments pass pretty quickly, though. Kwilinski and her husband, Kevin, know that $5,000 device – a cochlear implant – will give their son an excellent chance at experiencing life the way everyone else does.

Next week, if all goes as scheduled, Elliot will travel to Riley Children’s Hospital in Indianapolis and there become the youngest Indiana resident to receive one of the electronic devices, which help the profoundly deaf to hear in a manner approaching natural hearing. He’s only 9 months old, still nursing, not yet crawling. But his young age will allow him to adapt to the implant in a way older recipients might not – and get the maximum benefit from it.

“We wanted to take advantage of the brain’s plasticity,” said Amy, 33, who has been researching deafness, deaf culture, hearing aids and cochlear implants since Elliot’s disability turned up in a newborn screening. In infancy, the brain is wired to learn, and learn quickly; they want Elliot experiencing the world of sound before his personal learning window narrows or closes.

A cochlear implant works through its three main parts – an external microphone, which picks up sound; a microprocessor, which codes the sound into a series of electrical impulses; and a wire that carries those impulses into the human cochlea, where they are sent to the brain and understood as sound. It is an imperfect way to hear, but an enormous improvement over conventional hearing aids.

And for an infant, whose brain is especially open to learning about sound and what sounds mean, it’s a golden opportunity, Amy believes.

“Who really knows if the way I hear is the way you hear,” she said. “The important thing is, we understand one another. . . . (People who’ve lost their hearing and then received cochlear implants) say that the voices sound like Darth Vader and Minnie Mouse at first, but you get used to them. And you recognize the voices as speech and know what they’re saying.”

Cochlear implant technology is advancing in huge strides, as well, Amy said. The latest implants represent enormous improvements over the earliest ones, and the technology itself is only a few years old. By Elliot’s adulthood, she hopes, the devices may be so small and so sophisticated that know one else need know a person is wearing one.

Many in the deaf community are opposed to the implants, particularly in children. Deafness, they believe, is not a condition to be cured but a trait of human diversity. Adults might choose CIs, but children should not have the choice imposed upon them. The Kwilinskis reject that argument.

“It’s much more difficult for a person to learn to process sound the older they are,” Amy said. “And for Elliot to talk until then, he’d have to use sign language. I want him to be able to communicate with everybody, and to choose any career he wants. What if he wants to be a surgeon? How can he work with his hands if he has to talk with them, too?”

The surgery is only the first step for Elliot. It’ll be a month before the device is activated, after which he will spend time with audiologists and speech therapists to calibrate and adjust it, effectively turning on an entirely new sense in a person who lacks any framework to understand it. After that, “it’ll be pretty noisy around here,” Amy said. She expects lots of crying, followed by sounds of delight.

“People who’ve done this with their toddlers say within a few days, the kids don’t want to take them off,” she said.

“We went to a convention (of cochlear implant recipients), and I sat there with tears in my eyes, watching these teen-agers talk to one another,” she said. “I know this is the right thing to do.”"

We completed all of the candidacy requirements for the surgery, and Elliot’s first surgery was at 9 months of age. It would have been a month or so sooner had we been able to get the combination of insurance in order and the special exception for the MEd-el, which was only FDA approved immediately prior to his surgery. But we were pleased with his age at implantation.

On October 9th, I reported the surgery news to my friends and family:

“Everything went well! We’re happy to be home. The worst part was before surgery, when he couldn’t have anything to eat for 4 hours prior we were admitted, due to the anesthesia he was to have. He awoke at 3 AM and got very upset…we walked with him for an hour and a half…then got in the car and drove for an hour and a half (fortunately, he fell asleep). Part of that time we circled a Starbucks parking lot, waiting for it to open. Then we went to Riley Hospital at 6 AM. There is a lot of waiting, but he was administered a sedative (?) med. for part of that…we got regular reports from the staff and the actual surgery began at 8:50, about an hour after they took him to anesthesia.

It lasted a little over 2 hours, after which Dr. Miyamoto came and spoke to us. He was beaming, and obviously loves his work! Things went very well. Elliot is the youngest recipient of a Med-El in the U.S. and the youngest IN resident to receive an implant.

Elliot was sleepy most of the day after surgery. He nursed immediately when we woke up, and never cried during the day except when I would turn him around to the other side, and only momentarily. He slept Monday night, too…we left the hospital at 8 PM, after the Doctor’s approval, so that we could all get a good night’s sleep. We returned in the morning to have the bandage removed, and everything looks great. His scar is smaller than many I’ve seen, running from behind his ear and up for about 3 inches. The implant is not directly under the scar, but rather up further. No hair was shaved, and the stitches are dissolvable. Elliot is perky today and, except for a bit of swelling, looks back to normal.”

The day after surgery

The day after surgery

Back at home, we enjoyed the fun of a newly hearing baby. I wrote to a friend:

“Oh it’s just hilarious around here. The big boys are in bed and Elliot sneezed for the first time with his CI and just chuckled about that and again when I imitated it. He keeps staring at my face while nursing, something he didn’t do as much, and I keep talking to him…it falls off when he nurses on the left so I’ll probably end up lopsided after awhile…”

In early November, the newspaper did a follow-up story about Elliot:

Family grateful for gift of technology

A cochlear implant is allowing their 10-month-old son to hear.

Kevin Kwilinski catches his infant son’s eye and croons, “Ah ah ah ah ah.”

“Ah ah ah ah ah,” Elliot, the baby, croons back. In any other house with a 10-month-old, this would be commonplace, pleasing to parent and child, eliciting smiles all around.

In the Kwilinski house, it is something of a miracle.

A month ago, Elliot’s reply might have been silence, or a random growl or vocalization. Born profoundly deaf, Elliot had no way of interpreting his father’s open mouth as imparting anything of significance. His own baby babbling, incessant in hearing children, was starting to quiet, as his ears failed to carry the message of his own voice back to his brain.

But today, three weeks after his cochlear implant was activated, his brain is getting the message.

“Mm mm mm mm mm,” Kevin prompts.

“Mm mm mm mm mm,” Elliot replies. Another miracle.

On this Thanksgiving weekend, the Kwilinskis have much to be thankful for. Elliot, one of the youngest children to receive a cochlear implant, is responding beautifully to it, the delights of his new sense evident on his face.

“At his two-week check, they turned it up a little bit,” said Amy, his mother. Because Elliot can’t tell them what volume is most comfortable for him, such adjustments will be a regular part of his therapy until he can. Audiologists at Riley Children’s Hospital in Indianapolis look for cues as they tune the device – crying, or a blink.

The cochlear implant does not replace natural hearing, but it is by far the most sophisticated device for deaf people who want to hear. Working through three parts – an external microphone, a microprocessor that codes sound into a series of electrical impulses and a wire implanted into the cochlea – it serves as a sort of mechanical ear, bypassing the faulty one and sending signals directly to the brain.

What an implant wearer hears isn’t natural sound, but it is close enough that it can enable a deaf person to speak normally and interact with the hearing world. And for an infant like Elliot, whose growing brain is wired to learn language rapidly, it offers the next best thing to a cure.

Of course Elliot doesn’t understand what is happening to him. He wears a knit cap, tied under his chin, to protect the wire from his exploring hands. The microphone and processor – $6,000 in equipment about the size of a small cigarette lighter – hangs from it by a safety pin. So far, so good, but both parents keep a sharp eye on him as he scoots around on his hands and knees.

“Children can learn pretty early to leave it alone,” said Amy, who relies heavily on Internet mailing lists to communicate with other parents whose children have cochlear implants. “But we have a few spare parts.”

In just a few months, the Kwilinskis, who also have a 7-year-old and a 4-year-old, have gone through enough changes to unnerve any family. In the new year comes another – they’re moving to Denver. But this change is welcome, taking them to an urban area large enough to support the highly trained therapists Elliot will need.

“He needs an auditory-verbal therapist,” said Amy. “Indianapolis has three. Fort Wayne has none. We won’t have to drive so far to get him to one.”

The Kwilinskis are well aware of how fortunate they are. Elliot’s deafness was diagnosed during a newborn screening at Parkview Hospital, giving them an immediate start on how best to treat it. Kevin and Amy are both college-educated and capable researchers on-line and in libraries. Kevin has a good job with excellent health insurance that covered Elliot’s surgery and cochlear implant, and Amy is able to stay home to work with Elliot – all advantages other children born deaf might not have.

“We know we’re lucky,” she said. “The average age for this sort of deafness to be diagnosed is around 2, 2«.” By the time Elliot is that age, it’s entirely possible his speech and language development will be on a par with hearing children his age; only the device behind his right ear will indicate he’s different.

For now, Amy is Elliot’s primary therapist, playing games to teach him to associate sound with speech and speech with communication. But he’s doing much of the learning on his own, as he processes the sounds of life around him.

“It’s been incredible,” said Amy. At a musical performance last weekend, he was “clapping and dancing” on Amy’s lap. “I was walking around the mall yesterday, and for the first time it was clear to me that he was acting like a hearing child. He was turning his head toward the sound of a little girl talking, looking for the music in the stores.

“It really works,” she said. “Elliot hears.”"

Christmas, 2001 (Elliot was 11 months old)

Christmas, 2001 (Elliot was 11 months old)

During the candidacy process for the cochlear implant, we had met Elizabeth, a wonderful speech therapist on Dr. Miyamoto’s team who specialized in oral methodologies and we asked her to be our speech therapist. The problem was that the drive was impossible to do weekly (and take and pickup a 1st grader and preschooler to and from school). She would not do anything less than weekly sessions. Distraught, we finally found that there was an AVT near Indianapolis who would see us on a less regular basis. We saw her two times before we found out that we were going to be moving to Colorado. She referred us to a therapist who had trained her, and we contacted Nancy immediately. She agreed to work with us as soon as we arrived in Colorado! We had begun to be convinced that we really believed in the tenets of Auditory Verbal Therapy, anyway. As an La Leche League Leader, I was amazed at how the philosophies matched up in one particularly important area– that the parent is of crucial importance to a child. Even the teaching of my religion agrees: the parent is the child’s most important teacher. We know our child best and can best meet his needs.

We began official, weekly AVT right about at Elliot’s first birthday. He was already showing great signs of hearing well, and things took off. About 1 out of every 4 sessions was difficult because of behavioral issues. Retrospectively, I think that this is common– toddlers are this way!– but at the time I was nervous and worried and did cry a few times on the drive home. Would it work? Were we wasting time and money? What if we never communicate easily with him? There were always exciting words being learned, though, and before long, phrases. Every month we saw more and more progress. Mappings went well, therapy progressed. Nancy reported that his progress was very quick.

By the time Elliot was 2 we knew that he was nearly on-par with his hearing peers. We found out that summer that we would be moving to Georgia. When we told Nancy, she let us know that we were almost “finished” anyway! I was excited and pleased. Although I knew he was doing well, the thought of being finished with AVT already was a bit surprising and even frightening. So, when we moved to Georgia, I met with the team at the Auditory Verbal Center in Atlanta. They watched and listened… and finally, they basically asked me why I was wanting more therapy for him! It was clear from their reactions that they did not feel that he needed any more. I realized that I had no interest in making the long drive to the city if it wasn’t needed. We were in the process of requesting insurance coverage for a 2nd cochlear implant, and that battle would end up taking a lot of time.

Colorado baseball fans

Colorado baseball fans

When Elliot was first implanted, there were not children being bilaterally implanted in the United States. Before long, though, a few kids began getting 2nd sides implanted. We knew that we wanted this for Elliot for many reasons– in case of failure on the other side, for better hearing in noise, for localization of sound, and hearing during battery changes. We knew that the longer we waited, the worse his outcome could be on the unimplanted side. We spent about 18 months battling with Blue Cross and Blue Shield. They denied three times, and then we took it to the State Insurance Commissioner’s office, with the help of the Let Them Hear Foundation. The denials were overturned and we scheduled his 2nd surgery!

(Note: Melissa and I had become fast friends on CI circle, but we also shared another link at this point. Her daughter’s beloved therapist, Mary Ann, had been trained by our therapist, Nancy. So we both had very similar styles… and clearly it was a GOOD style! When I figure out how to do it, I will be posting video examples of Elliot’s AVT.)

Jessica

Saturday, February 21st, 2009

Before I continue with Rachel’s story, though, I need to talk about her sister.

When Rachel was in first grade, we had our third child, Jessica.  I had a feeling during my pregnancy that Jessica couldn’t hear, even though the “expert” neurologist had told us that Rachel’s deafness had been caused by a virus.  During my pregnancy with Adam, I looked for responses to sound and noticed that he would move around when my stomach growled.  In addition, although I am almost embarrassed to admit it, at about seven months into my pregnancy with Adam, I had started banging first pots and pans and then large cymbals in front of my stomach and was able most of the time to get Adam to move around.  With Jessica, I could repeat none of that.  

 

Rachel and Adam with Jessica

Rachel and Adam with Jessica

 

While newborn hearing screening was not yet routine in 1995 when Jessica was born, we were able to have her hearing tested before she left the hospital because we had a family history.  Jessica was not yet 24 hours old when the hospital audiologist told us that she could not get any response to sound from Jessica.  Rachel’s so-called “viral” hearing loss had turned out to be genetic.

So, just as Rachel was nearing the end of her Auditory-Verbal therapy, it was time for us to start over again.  Unlike Rachel, Jessica never had any residual hearing.  Thus, although we tried hearing aids, they proved useless except to annoy us with their feedback. 

When I told Rachel after bringing Jessica home from the hospital that Jessica was deaf, she quietly said to me, “I was hoping my little sister would be deaf like me.”  That was the beginning of a bond between the two girls that is still very much there today.  Jessica adores and looks up to Rachel as her role model.

One thing we knew was that we didn’t want to wait until Jessica was two to get her hearing because we knew all too well how many years we had spent working with Rachel to catch up her language.  Dr. Todd said that he would be willing to implant Jessica at 22 months even though the FDA guidelines at the time still specified age two.  We had heard that Noel Cohen, our NYU surgeon, had implanted a 20-month-old, and so we decided to make a trip with Jessica up to NYU, hoping that he would agree to implant her at 18 months.  Jessica was 14 months old at the time, and I will never forget sitting in Dr. Cohen’s office hearing him ask me, “So when do you want to do this,” to which I replied, “As soon as  you are willing.”  When he then asked me, “How about next month?,” I practically fell off my chair.  I beamed at him and told him he had a patient.  Thus, at age 15 months, Jessica became the youngest pediatric cochlear implant recipient at that time.

 

Jessica at age three weeks at AVCA after having ear molds made for her first pair of hearing aids.

Jessica at age three weeks at AVCA after having ear molds made for her first pair of hearing aids.

 

The early implant proved to be very beneficial for Jessica.  We began our weekly sessions with Mary Ann, and Jessica’s receptive language progressed very quickly so that, by age three, it was age appropriate.  However, she was barely talking.  Because we were experienced Auditory-Verbal parents at that point, we knew that something was not right.  In particular, we noticed that, while Jessica could spontaneously make certain sounds, she could not produce them voluntarily when we asked her to repeat them.  For example, she would point to a tree and say “ee” but could not see “ee” if we asked her to imitate it.  We took her to a pediatric neurologist who diagnosed her with speech apraxia, a condition where the signal from the brain to the mouth gets jumbled so that the mouth cannot figure out how to form the sounds based on what the ear, or in Jessica’s case, her CI is sending to her brain.  Mary Ann referred us to an oral motor therapist who specialized in apraxia, Sharon Wexler.  Sharon proved to be yet another incredible professional who came into our lives.  Within two months of working with Sharon, Jessica began to talk.  Within five months, her expressive language jumped a year.  It was as if all of that language had been inside of her waiting to come out.  At age six, Jessica “graduated” from Auditory-Verbal therapy with language that tested age level to two years above in all areas.

 

Jessica with me during a session with Mary Ann at AVCA

Jessica with me during a session with Mary Ann at AVCA

 

After seeing Rachel struggle with her CI failures, we also opted to have Jessica bilaterally implanted.  Jessica heard incredibly well with her one CI, which was a good thing because, no matter how hard I actually tried to teach her, she could not lipread at all, not even a single word.  We felt that she needed a “spare ear” so that she would never be left without hearing.  Jessica was nine years old when she received her bilateral CI about two months after Rachel received hers.  Jessica had the advantage of being younger than Rachel, and she made rapid progress with her new ear.  After about five months she was testing 60% auditory-only comprehension in that ear alone.  After about two years, her new ear was indistinguishable from her original ear.  According to the research on bilateral implants in children, Jessica was beyond the range of time between implants where this should have been possible, which just goes to show that researchers don’t know everything!  The icing on the cake for Jessica was that her hearing in background noise improved.  Normal hearing for testing in extreme noise is considered to be anything 80% or above.  Jessica tests at 84%.  In addition, she scores 100% on the most difficult test of auditory-only comprehension that involves her listening to and repeating single syllable words with no context and no visual cues.

Like Rachel, Jessica had gross motor delays.  She was even later than Rachel, not sitting until 11 months and not walking well until 2 years 3 months.  Between that and her expressive language delay, we opted to start her in preschool a year late and put her in with a class of children a year younger.  While she started preschool at Rachel’s and Adam’s old preschool, because Adam was at the private school where Rachel had been, we opted to switch Jessica there for her last year of preschool so that she could continue on to kindergarten there.  Jessica stayed at the school through first grade.  The teachers were wonderful and caring, but Jessica has some mild learning disabilities unrelated to her hearing that run in Elliot’s family.  Thus, like Rachel, she needed more resource help than the private school could provide.  We were very fortunate in that, for that next year, our county was starting a program in a nearby very good elementary school that was just like the program Rachel had attended for middle school.  For the next three years in grades two through four, Jessica attended this public school.  At the same time, I continued to take her to her resource teacher at the private school once a week for tutoring with the goal of keeping her on track to where the students were in her grade there so that she could eventually return to the school. 

In fifth grade, Jessica returned to the private school, and she has thrived there.  She is a very hard worker with a strong desire to do well.  She still works with an excellent resource teacher, but now the time that she gets in resource is sufficient.  She is in seventh grade, and the school ends after eighth grade.  So, once again, we’ll soon be looking at choosing a high school.

One of Jessica’s favorite activities is drama.  She recently had her first major lead in a school play, Cheaper by the Dozen.  She was wonderful, having memorized all her lines and reciting them with emotion.

 

Jessica in Cheaper by the Dozen

Jessica in Cheaper by the Dozen

The Rest of the Story … Almost

Friday, February 20th, 2009

 

In 1991, Elliot finished his surgical residency in Boston.  He had decided to do a fellowship in vascular surgery.  He created his list of the best programs, and I created mine of the best Auditory-Verbal programs and therapists.  They corresponded for Atlanta, Boston and Texas.  Thus, in June of 1991, we said goodbye to Boston after nine years and moved to Atlanta, initially just for the one year of his fellowship.  At this point, we had two children.  Our son Adam, who was born with normal hearing, was five months old when we moved to Atlanta, and we decided that I would take the year off of work and be a stay-at-home mom. 

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Rachel with her new baby brother, Adam

 

We began working with Mary Ann Costin at the Auditory- Verbal Center in Atlanta, then known as the Auditory Education Center (AEC).  Mary Ann evaluated Rachel, identified the areas of language we needed to focus on next, and began piling the work on me, which was a good thing!  Rather than daycare, Rachel attended a regular preschool five mornings a week, leaving my mornings free to focus on Adam and my afternoons free while he napped to focus on working with Rachel.  During that year, I saw how greatly Rachel’s language thrived from having me have so much more time to spend with her.  Thus, after we ended up deciding to settle in Atlanta, I still opted not to return to work right away.  Rachel’s language, while progressing steadily, was still delayed by about two years, which made sense since she had not heard anything until she was two years eight months old. 

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Rachel with Mary Ann Costin

 

As clearly as I remember the early, more difficult days, much of the rest of Rachel’s childhood in terms of her progress with spoken language is more blurry.  It was not as if I can point to a time when we got “there.”  Her progress was always even and steady, some language concepts coming more readily and others taking more work.  I know that past tenses, pronouns and articles were harder concepts for her.  Mary Ann was very organized in her approach, and, over the years, took Rachel through multiple areas of vocabulary and language and cognitive concepts.  She worked in thematic units, which served not just to encompass large areas of focused vocabulary but also to keep the therapy fun and interesting by focusing on a new unit once every month or so.  The thematic units included the zoo, the farm, the circus, the beach, school, the house, including each room, each of the seasons, holidays, feelings and emotions, children’s literature and more.  For each thematic unit, Mary Ann would spend hours putting together a scene that would sit on a board on the table.  Rachel would walk into the therapy room very eager to get started when she would see the circus, farm, etc on the table all set up to play with.  Mary Ann also used an interdisciplinary approach, including tasks and games to work on auditory sequential memory, speech, and even language concepts such as pronouns using the same props from the thematic unit. 

We opted to keep Rachel in preschool an extra year to give her more time to close her language gap.  When she entered kindergarten in 1993, she was fully mainstreamed with no special education and did very well, including socially, making friends very quickly. 

When she was six years old, the Auditory-Verbal Center passed out entry forms for the poster child for Better Hearing and Speech Month, an annual awareness event held May of every year.  Much to our surprise, Rachel won that year.  Much excitement followed.  The spokesperson that year was jazz musician Lionel Hampton, and we went with Rachel to New York to take the photo for the poster and to record her speaking for a radio public service announcement.  Then, in May, we all went to Washington, D.C., to participate in the Better Hearing and Speech Month festivities.

 

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Rachel with famous jazz musician Lionel Hampton for Better Hearing and Speech Month

 

Better Hearing and Speech Month 1994 also marked the beginning of my time as an advocate for cochlear implants and Auditory-Verbal therapy.  The editor of Contact, the publication of Cochlear Implant Club International (CICI), asked me to write an article about our experiences.  After that, he asked me to write more articles and eventually to be an associate editor.  It was a great experience for me, and I continued it for six years before I finally returned to work part-time.

In first grade, Rachel’s teacher taught phonics through weekly spelling lists.  She would give the weekly spelling test while walking around the room.  The early lists were based on phonetic chunks, such as “at” words or “et” words, which meant that her list would consist of words such as mat, bat, pat, sat, rat.  While Rachel could easily hear the difference in those words with someone nearby, with her teacher walking around her classroom of about 25 children, it became very difficult for her.  Even though she didn’t have an individualized education plan (IEP), her teacher was more than willing to help her out, and so she had her teacher’s aide give Rachel the spelling test one-on-one each week in the cafeteria. 

Rachel has always been a very focused, determined and motivated student.  Thus, we worked very hard with her throughout elementary school, both on her schoolwork and her continuing Auditory-Verbal therapy.  She continued to go to therapy once a week through second grade, and then in third grade we dropped to once a month.  At the end of third grade, she “graduated” from Auditory-Verbal therapy. 

Our public elementary school began to go downhill.  Thus, for fourth grade, we pulled Rachel out of public school and enrolled her in a wonderful private school.  Over the course of the year, Rachel began to have difficulty with reading comprehension.  Her school offered resource help, and we opted to begin having Rachel work for about 30 minutes a day with a resource teacher to work on vocabulary and reading comprehension.  This continued through fifth grade, and, while Rachel could have remained at the school for middle school, we felt that some more intensive resource help could help her finally close the remainder of her language gap.  However, the only place she could get that while still attending a mainstream school was in public school, and our public middle school was even worse that our elementary school.  We decided to look into it anyway, and the public school tested Rachel.  According to her testing results, she was not behind academically and so did not qualify for special ed.  So, we began talking to the resource teacher at her private school about making things work there for the following year.  Then, I received a phone call from a woman who said that she was the hearing impaired resource teacher for our county and had been given our name and contact information.  I was confused because our local school had already told us that Rachel did not qualify for special ed.  However, this woman explained that they turned Rachel over to her because Rachel did qualify for the hearing impaired program, and I had a choice of either having her go to our home school to work with Rachel or having Rachel go to her school.  Her school happened to be an outstanding middle school and was located only eight miles from our house, and so we seized the opportunity. 

While Rachel was not happy with our decision initially because she loved her private school, it proved to be one of the best moves we made for her.  Rachel was very fortunate her first year in her new school to have an incredible group of teachers.  In addition, her resource teacher also proved to be excellent and very dedicated.  Rachel worked with her one-on-one for sixth and seventh grades for reading and language arts, which meant that she got intensive resource help for 90 minutes a day.  Those two years proved to be very beneficial to her, and by eighth grade she was able to return to the regular classroom with monitoring by the resource teacher.  In addition, she found a great group of friends at that middle school and so was very happy socially.

Middle school also marked the beginning of Rachel’s awareness of her role as one of the earliest pediatric cochlear implant recipients and her decision to assume the role of advocate.  For her birthday, Mary Ann had given Rachel a gift certificate to Barnes and Noble.  Rachel used it to buy herself the book, “Web Design for Dummies.”  When Rachel was in eighth grade, she used this book to create the first iteration of her website, www.cochlearimplantonline.com.  She entered her website in her school’s technology fair and went on to win not just first place at the school level but also first place at the county and then state level.  By that time, Mary Ann had become like a member of our family, and she accompanied us to Macon, Georgia, and so was there to cheer Rachel on when she won the statewide award.

 

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Rachel wins first place at the state technology fair

 

For high school, we were fortunate that our local school had the arts magnet program for our county.  Rachel  had always been a very gifted artist, and so this program was a perfect fit for her.  Returning to school in district was also a welcome change.  Rachel thrived in high school, getting very involved and excelling academically.  Her school would give out VIP awards at the end of each year.  Each teacher was allowed to give out only five.  Throughout her time in high school, Rachel won 12 of these awards, with at least two every year.  She also became involved in filmmaking, and won her high school film festival’s awards for best documentary and best overall film two years in a row.  In ninth grade, her film was about the cochlear implant.  In tenth grade, it was about the daughter of a friend of mine, a remarkable little girl who not only hears with a cochlear implant but who also has physical disabilities but who leads a remarkably upbeat life.

The one downside to high school, or I should actually say two downsides, were Rachel’s cochlear implant failures.  One morning in the spring of her ninth grade year, she came to us very agitated, saying that she had put on her processor but was only hearing loud, painful clashing sounds.  I immediately took out her spare processor, but when she put it on, she burst into tears.  Fortunately, I had the audiologist’s home phone number.  I called her, and she told us to meet her at her office with Rachel at 8:00 a.m.  It didn’t take her long to confirm what we already suspected, that Rachel’s internal implant had failed.    While we felt as if the NYU cochlear implant team was almost like family, we had stopped traveling there for mappings when Rachel was about six years old when a cochlear implant program began at our local children’s hospital, and an experienced pediatric cochlear implant audiologist from the House Ear Institute began mapping the children implanted locally.  One of our main concerns was to have Rachel reimplanted as quickly as possible, and, besides the logistics of traveling back and forth to New York, we also knew that getting insurance approval to return to New York would delay scheduling Rachel’s surgery.  The surgeon in Altanta, Wendell Todd, was wonderful and worked Rachel’s surgery in for six days later. 

Rachel received a Nucleus 24 Contour, which meant a technology upgrade, in March 2003.  We had been warned by other parents whose children had had to be reimplanted that the road to returning to her previous level of comprehension would not be immediate.  Thus, we tried to keep our expectations low.  Rachel had some auditory comprehension immediately, and, with her excellent lipreading skills, the combination meant that she could function well in most situations, although not on the phone.  Her school provided her with a CART reporter for the duration of the school year, which also helped a great deal. 

It took Rachel about ten months to attain a level of auditory comprehension comparable to what she’d had with her Nucleus 22.  Unfortunately, she only got to enjoy that for three weeks.  One morning, I received a phone call from her school.  Rachel was hearing static.  Would I please bring her spare processor to school.  I had a bad feeling about it, although I’m sure part of that was having gone through an internal CI failure so recently.  However, when Rachel put on her spare processor, we had a repeat of the previous year with her starting to cry as the spare processor did not resolve the situation.  Throughout that day, occasional pops and crackles were added to the static.  I called our audiologist, and we were very fortunate that the Cochlear Corporation audiologist was going to be in Atlanta that day doing integrity testing on a few other children and was able to fit Rachel in at the end.  While the integrity testing did not show a problem with Rachel’s implant, because Rachel at 16 was an accurate reporter of what she was hearing, Cochlear Corporation told us to go ahead and reimplant.  What happened to Rachel had less than a 1% chance of occurring.  We were very upset to have Rachel go through yet another surgery, but, at the same time, we were fortunate that Cochlear Corporation was so cooperative.

We had already been planning to have Rachel bilaterally implanted that summer because research was showing the benefits of having two ears, and Rachel very much wanted to hear out of both ears.  However, we didn’t want to have her have to undergo two surgeries, and thus we decided to simultaneously have her original ear reimplanted and her other ear implanted for the first time.  After one very long eight hour day, Rachel had her two new implants. 

We were weary at that point of adjusting to a new CI.  Once again, even though she went from a Nucleus 24 to another Nucleus 24, Rachel could not hear as well with her new CI.  While we did not expect her to hear clearly from her new ear, we did not expect her readjustment to take as long this time.  I repeatedly took her back to her audiologist for mappings, but they didn’t help.  Four months into it, I made the decision to take her up to Bill Shapiro at NYU.  That proved to be one of the best decisions we could have made.  Cochlear implants have two basic settings, the threshold level and comfort level.  The threshold level for each electrode is the softest sound the user can hear for that electrode, and the comfort level is the loudest sound that the user can tolerate for that electrode.  Bill found two problems.  First, Rachel’s threshold levels were inaccurate.  She could not hear any of her threshold levels.  In addition, he discovered that she had something called T-tails, where as the threshold, or T, level increased, she did not immediately perceive an associated increase in loudness until the T level reached a certain point.  Bill said that that first point where she perceived the increase in loudness was where her threshold levels needed to be set.  He also found that her comfort levels were too low.  We spent two days with Bill.  The difference in Rachel’s hearing after those two days was nothing short of incredible.  In the booth, her auditory comprehension jumped 20%.  In everyday life, that meant the difference between struggling to hear to using her cell phone with the regular earpiece without the telecoil while walking down the noisy main hallway of her school at the end of the day and not missing a beat.  

Over time, Rachel not only attained her previous level of hearing with her Nucleus 22, but, especially with her bilateral implants, she exceeded it.  With bilateral implants, she enjoys music more and reports that sounds are richer and fuller.  Those of us who interact with her notice that her overall clarity of hearing and distance hearing are also improved.  It’s been almost five years since Rachel received her bilateral implants, and, while her hearing with her bilateral ear is not as good as with her original ear, it still continues to improve and has now reached the point that she could get by with just that ear if she had to, a comforting thought because it means that the chances of her ever going completely without hearing again are slim since it’s very unlikely that both implants would ever fail at the same time. 

Another highlight of Rachel’s time in high school was French.  During her IEP meeting before ninth grade, she stated that she wanted to study French.  The special ed director told her no, that she felt it would be too difficult for her.  I admit that I, too, was hesitant, but it was the last time that I would ever doubt Rachel’s ability.  Rachel was insistent and got her way, starting French in ninth grade.  She was fortunate to have a wonderful French teacher who worked with her after school once a week one-on-one for an hour of her own time on the pronunciation of the French words Rachel was learning.  At the end of the semester, Rachel happily stopped by the office of the special ed director, waving her report card with her A in French.  Rachel went on to excel in honors and then AP French.  In addition, she participated in two exchange programs arranged by her French teacher that awakened her love of traveling, particularly to France and the French culture, while also giving her the opportunity to improve her French.  Also, in eleventh grade, during awards night, Rachel was surprised and all smiles when she heard her name announced as the winner of her school’s foreign language award, an award given to one junior or senior out of about 800 students.

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Rachel and her high school French teacher

 

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Rachel in Paris

 

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Rachel with her award for excellence in foreign language in 11th grade

 

Rachel had always excelled in and loved art.  Thus, when it came time to choose a college, she opted for the Savannah College of Art and Design (SCAD).  That made the college process a very easy one for us as she visited SCAD, decided that it was the place for her, applied at the end of her junior year as they have rolling admissions, was accepted that June, and was finished with the whole process before most of her peers had even gotten started.  Thus, she was able to sit back and enjoy her senior year without the added stress of the college application process.

Rachel gave us one more thrill at the end of her senior year of high school.  Her school would give out all of its awards at awards night before graduation except for the three most major awards.  Graduation night, with nine of us looking on and cheering, Elliot, myself, Rachel’s two siblings, both sets of grandparents and Mary Ann Costin, Rachel walked up on stage to receive her school’s Faculty Cup award, given to her for excellence in academics and community involvement.  I sat back and thought to myself that we’d come to the end of our long journey, almost like climbing a very tall hill for 19 years, and that we could now sit back and enjoy the view.  Unfortunately, we only got to enjoy that view for two months before we found the mountain looming in front of that hill.

 

“Yep, nothing.”

Thursday, February 19th, 2009

When I got home I immediately researched and read that fluid is uncommon after a few hours after birth; Elliot was more than 24 hours old when they had administered the test. We spent all week going back and forth between hope and concern. One person we were thinking about was my mother-in-law, Sharon, who had become deaf in her late teens. Why had we never thought about that? She had been told that it was caused by loud rock music at dances. Now, of course, Otolaryngologists laugh at that idea– when one has damage from loud sounds, especially music, it is usually a gradual loss over a lifetime, not a dramatic thing taking a year or two. Was it hereditary?

I remember that the ABR test took a long time and that we didn’t know what was going on until the first ear was completed. The audiologist said something like “yep, nothing.” Was it his experience with hearing loss that made him so flippant? Who knows. But when he did the 2nd ear, I was hoping against hope that it would come out OK. It did not, of course.

I remember sobbing through the parking lot as Kevin carried the car seat. I don’t remember much for a few days after that. Kevin had to call my parents to tell them… I couldn’t bear to hear their reaction. I do know that I was immediately researching the words which the audiologist had mentioned: cochlear implant.

On January 19th, when Elliot was 15 days old, I wrote to a friend:

” I am still working thru the grieving for all the things he won’t hear and difficulties he will have educationally, but I began getting a list of books together to read and have ordered them thru interlibrary loan. I will let you know as soon as we know the cause. Of course I am sort of hoping in the back of my mind that it is the kind that can perhaps be assisted by a cochlear implant, which they now do as early as 1 year. That would be a difficult decision but one we can’t really think too much about since we don’t know the type of hearing lossthat he has. We will know more after seeing an ENT.”

I learned a lot in the immediate few weeks. I read a wide variety of books about deafness– from the educational perspective, from the Deaf Culture perspective, etc. By 3 months, Elliot had hearing aids. That was an interesting struggle in itself. Not to get them– the audiologist referred to Yoshinago-Itano studies about early amplification and identification being crucial, which I found really interesting, but she refused to turn the aids up enough to benefit the potential residual hearing. My husband had to download the manual about that particular brand of HA and insist. She called the company before finally doing it. (What was she saving his hearing for– a rainy day?) I spent hours trying to keep hearing aids on a baby. Earmolds take a couple of weeks to get back from the company, so if they are sent to the audiologist and then you go pick them up, by then they are often outgrown. Who knew that ears grew so fast? There are many kinds of plastics and silicones that can be made into earmolds; some fit more tightly than others. Some are squishy, some are hard. We didn’t get a pair that fit well for months. When earmolds are loose, there is a feedback loop created by sound into the hearing aid, and they whistle. I remembered my grandfather’s hearing aids whistling in church; now my son’s were whistling all the time. Every time his head bumped his car seat or his high chair or my shoulder, or he touched them with his fingers, he whistled. Then, he figured out how to pull them out. Earmolds detatch quite easily, too, and so you have two built in choking hazards on your baby at all times. We tried several substances to maintain the seal– Otoease, Otoferm and even Lansinoh. Nothing was perfect.

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Within a few weeks of the diagnosis I had found CI Circle, a listserv group for parents of children with cochlear implants. This, of all the sources, was the most useful for getting information, since we knew already that we were incredibly interested in cochlear implants. There were a variety of opinions, but I was able to narrow down which ones I agreed with and pick the brains of those parents. One very useful and exciting tip was from Melissa Chaikof regarding early implantation. She knew of a baby who was implanted under 12 months, which was (and still is) the FDA “recommended” age. We wondered if we could accomplish this for our baby, and we ate up research about the benefits. It seemed a “no brainer” that kids who are implanted younger learn language more easily and more naturally, like their hearing peers. Some of the kids who were implanted young had suffered from meningitis, which can ossify the cochlea quickly, and we did not have that reason to implant early.

On February 20th, when Elliot was 6 weeks old, I wrote to friends:

“Today Elliot had the second ABR (a hearing test which measures brainstem response by glueing/taping electrodes to the head while asleep), another tympanogram and Otoacoustic Emmision test (measures replies from the hairs in cochlea)…we had an appt. with the ENT a couple of weeks ago but didn’t learn much more then since it was an initial consult. The ABR was the same– no signs of any hearing up to 90 decibels, which is quite loud and the limit of the machine. Any residual hearing (which most profoundly deaf people have, even if just a little) will be over 90 dbls and will only be found out when he’s older and can show that he hears noise in other tests. We don’t know what caused the deafness, but in over 50% of congenital deafness cases, it is genetic. So we may end up having testing for that. We will also eventually have a CT scan in preparation for potentially getting a cochlear implant. We will be going to a CI center, either at U of M or Riley Children’s hospital eventually. They do them as early as 1 year now.”

We were so lucky to be in Indiana. Actually, for some reason, in the weeks after Elliot was born, the words a “charmed life” kept popping into my head by itself. I have no idea why, because that is a strange word… but it appeared to me that we were extremely lucky. We had a deaf baby, yes, but he was born in such an age of promise with the cochlear implant. I had two solid weeks of meals brought in by friends in La Leche League and the church. I had old friends write me long, encouraging letters. I felt that I was learning a lot about myself, what we could do when we needed to. I felt that I could be the mother that this child needed. So, when we visited Dr. Miyamoto at Indiana University and he was also gung-ho about early implantation, we knew that things were going to proceed.

One reason that we were clear about our course toward giving our child hearing was because of my husband’s experiences with his mom being deaf. There are people who argue that deafness is a god thing; for us, this did not ring true. Kevin remembered being the little boy who had to be all grown up in the days before email and phone services for the deaf. One time, he and his brother cowered in the back seat of a car while a police officer berated and roughly treated their mother, who had not heard his siren and not pulled over very quickly. Another time, he had to tell his mother that a friend of the family had been killed in a car accident moments before, when she had him call their house about something else. When he was in elementary school, he and his father arrived home to flashing lights of police cars because his mother had been raped while taking a nap on the couch. Then there were the little things– the water left running, the car door ripped off when she backed out the garage and didn’t notice that a door was open, the times he was locked out of the house while she was at home in her bedroom. In grocery stores, people would assume that she could hear because she spoke clearly, having heard throughout her childhood. Then when she would turn her back, they would assume she was being rude. She read lips well, but some people always speak quickly, and Kevin would have to interpret with his lips for every conversation like this. She had a lot of spunk, though, and had a zest for life. Unfortunately, Elliot never got to know Sherry because she died of ovarian cancer when he was five months old.

Elliot's brother, Alex, with my mother-in-law

Elliot's brother, Alex, with my mother-in-law

The other main reasons were because we wanted him to be able to communicate easily with his family, including his brothers, grandparents and extended family. We wanted him to be able to talk to anyone he might meet without an interpreter, if possible. We also hoped that he would be able to hear enjoyable sounds– birds, music, fireworks. Actually, music is one of the first things that I thought of, but after reading more about educational issues and deafness, I realized that it was the least of my worries.

On March 3rd, 2001, when Elliot was 2 months old, I wrote to friends:

“Our feeling tend to be that the we want Elliot to hear as much as possible and therefore be a part of our culture and our community rather than the d(D)eaf culture, which is shrinking due to advances in technology, health, etc. Although I know he probably will want to have deaf friends, he will be out there in a hearing world and we want him to be able to freely choose his occupation, spouse, etc. This doesn’t end up being the case often for those within the Deaf Culture. I respect their decisions but it is not what we want for Elliot. The Deaf Culture is often very opposed to CIs because they see it as taking people away from their community, destroying their culture. They have likened it to the holocaust. However, it is a one-day, go home the next, kind of surgery, and studies show that prelingually deaf children who have it develop speech similar to hearing children. In my opinion deafness is a disability (deaf culture denies this although they reap benefits of the Americans with Disabilities Act, etc.) much like the loss of a limb, eyesight…and we all know we would do what we could for our child in those cases. such as prosthetic limbs. ”

And on March 11, I mentioned CI circle to my friends:

You wouldn’t believe the support I have gotten on the CI email list and what I’ve learned. I am understanding so many things about him and finding that little things he does are common with deaf children. For example, he may have some vestibular issues and balance issues because he never lets me hold him upright on my shoulder, something I noticed right away before I even knew he was deaf. I have had many other parents comment that their kids were the same way, and that they have since had PT or OT to work with motor skills. There’s so much more than simply a loss of hearing. There’s heated debate about communication methods, heated debate about ways to stimulate sound, heated debate about medical vs. other models of looking at
kids…well, it’s overwhelming but I’m glad to be able to find out now. I’m one of the fortunate ones who has found out early. There are very few out there.

There have been about 200 children implanted with CIs between 9 and 18 months of age, so Elliot might be entering a very select group in a year. We hope that we can learn as much as we can and make a very informed decision. We will be going down to Riley in Indy in about a month and we hope to have a good mtg with the implant surgeons at that time.”

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Cochlear Implant

Thursday, February 19th, 2009

Lea was a very active member of Auditory-Verbal International (AVI).  One day, she was speaking on the phone with another AVI board member, Judy Simser, in Ottawa.  Lea mentioned that she was working with a child who was totally deaf.  Judy told Lea that she, too, had a child who was totally deaf, and the family had gone to New York to the cochlear implant center at New York University and gotten her a cochlear implant.  By this time, it was no longer a single channel cochlear implant but, instead, was the Nucleus 22, the first multichannel cochlear implant.  Judy told Lea that she should pass on the information about the NYU center to us because she was very impressed with what her student was hearing.  This was in August of 1989.  In October, we spent three days in New York with Rachel, having her undergo evaluations not just by the audiologists but also by therapist at the League for the Hard of Hearing and by a child psychologist. 

While were at the League, the therapist there introduced us to a mother and her daughter.  The mother was Melody James, and her daughter was Caitlin Parton, then four years old.  Caitlin was hearing and speaking as well as any four year old.  We were beyond amazed and knew from that moment that this was without a doubt what we wanted for Rachel.  Two years after we met Melody and Caitlin, they appeared on “60 Minutes” and showed the world the potential of the cochlear implant for children.  Over the years, Melody has become one of my closest friends and my second mentor.  Cochlear implants were still in phase III clinical trials for children, and so Caitlin was the only child I knew who followed a similar path to Rachel with spoken language and full mainstreaming, and I looked to Melody for guidance at every new stage along the way.  I still do!  Caitlin has inspired us through the years and let us know what was possible, from playing the piano to attending a top university.

At the end of our three days at NYU, Bill Shapiro, the head audiologist, told us that Rachel was definitely a candidate for a cochlear implant.  FDA protocol at that time was very strict, and so Rachel had to spend the next eight weeks wearing a body worn FM hearing aid at all times.  If, at the end of the eight weeks, she still showed no response to sound, then she would definitely be a candidate.  However, Bill was so sure that the FM would not make a difference that he scheduled Rachel’s surgery for eight weeks and one day later. 

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Rachel wearing her body worn FM hearing aid

 

Eight weeks later, we returned to NYU and, as suspected, Rachel still couldn’t hear anything, even with the powerful FM hearing aid, which we happily returned to the League’s audiologist.  That evening, we checked Rachel into the hospital.  In 1989, children actually checked in the night before their surgery.  The next morning, Rachel was prepped for surgery, and then we got the news that a water main had ruptured in front of the hospital, cutting off power to the hospital.  While the hospital had a backup generator, it did not supply sufficient power for the autoclave, the machine that sterilizes surgical instruments.  While the surgeon had one set of instruments already sterilized, he did not have a backup set.  We waited for hours, with me getting more and more upset as time went by.  I had taken four weeks off of work prior to Rachel’s surgery because she was in group daycare three days a week and was always catching a cold or other minor illness, which would have postponed her surgery.  Thus, she and I had spent the past four weeks home to keep her healthy.  I had visions of the hospital sending us home and rescheduling.  Fortunately, we were in New York, which had multiple cochlear implant centers, something very unusual at that time.  Our surgeon called one of the other implant centers and was able to borrow a backup set of instruments.  So, about five hours late but, nonetheless, on the day that she was scheduled, Rachel finally received her cochlear implant.  The surgeon was able to insert all the electrodes and pronounced the surgery a success.  Because Elliot was a surgical resident, he even pulled him aside at the end to show him Rachel’s head xray showing the implant right in place.

Today, cochlear implant surgery is outpatient, with children staying 23 hours to include one night or even going home the same day.  However, back then it was four nights in the hospital, including the night before surgery.  Rachel had a drain coming out of her incision, and she could not be released until the incision stopped draining.  They also kept the IV in her hand for that entire time, and, at one point, the IV needle slipped out.  Replacing that needle was the most traumatic part of the entire surgery and hospital stay for Rachel.  A medical student, intern and resident tried to put the IV back in and were not experienced enough and kept poking and hurting Rachel, who was crying.  Elliot was no longer there.  He was still in his surgical residency, and taking any time off was almost unheard of.  I was there with my parents and my father-in-law.  My mother saw what was going on and became very upset and went and found someone who knew what they were doing.  For a few weeks afterwards, whenever anyone asked Rachel to see her “boo boo,” she never pointed to her head but, rather, to the bruise on the back of her hand.

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Rachel sitting on her grandmother’s lap recovering from her cochlear implant surgery

 

Following Rachel’s release from the hospital, I went to Baltimore with my parents for a week to have some help while Rachel recovered.  Then, three weeks after that, my mother met me in New York for the activation of Rachel’s implant.

We had no idea what to expect.  There were so few children with cochlear implants then.  In fact, at one point Cochlear sent out a list to the families with the first 200 children in the U.S. and Canada to receive a cochlear implant, and Rachel was on that list.  All that Bill Shapiro could tell us was that Rachel would hear environmental sounds.  The cochlear implant team could give us no guarantees.  My mother and I sat in the little room with the audiologist and the computer with bated breath.  When he started stimulating the first electrode and reached the point where Rachel could hear the signal, she started to cry.  My mother and I were cheering!  Her crying meant that she could hear!  She proceeded to react the same way to each additional electrode. 

At that time, all of the electrodes were not programmed the first day.  Instead, programming occurred gradually over three days.  On the last day, Bill turned on the four highest frequency electrodes.  A speech pathologist from the League was there to help with the programming.  After Bill finished programming Rachel, she said, “ssss.”  Rachel was sitting in my lap and turned around and hit me on the mouth.  We all started laughing.  We were so thrilled.  Not only had Rachel heard it but also she recognized where it came from.  Even if she got the person wrong, she knew that someone was speaking.

Bill warned us that, although Rachel appeared to hear in that little room, when we left she would likely appear as deaf as before for awhile as her brain learned to make sense of the sound from the CI.  This proved to be true.  However, after two weeks, right before we were supposed to return to New York for Rachel’s second mapping, she began to respond to loud sounds by turning her head to look for them.  At her second mapping, Bill found that her tolerance for sound had greatly increased, and he was able to give her considerably more power, which meant considerably more hearing. 

Within a week of her second mapping, Rachel began to respond consistently not just to environmental sounds but also to voices.  That marked a turning point for us, and we were able to wholeheartedly return to Auditory-Verbal therapy with Lea, this time with hearing.

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Lea working with Rachel while Elliot and I look on

 

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Lea and me with Rachel

 

From that point in, we never looked back.  Before “sandwiching” was a known technique, it’s what I decided on my own to use with Rachel.  I would say something to her with my hand in front of my mouth so that she could focus just on listening to what I said.  Then I’d say it again, letting her lipread, and then I’d repeat it one more time with my mouth covered.  In this way, Rachel transitioned from lipreading to having fully auditory language within six months of receiving her CI.  In addition, two months after beginning to hear, Rachel began to use her voice.  I remember the first time I was able to reason with her.  She wanted to do something, and I held up one finger telling her that first we had to do something else, then we could do what she wanted.  I held up two fingers for that.  I saw the light bulb go off as she smiled and repeated what I had said over and over again letting me know that she’d gotten it.

I do need to add, though, that it was not all easy with Rachel.  She was always a very strong willed child who wanted control.  She would fight me about various things and would never give in.  For example, she refused to take medicine, and trying to disguise it in applesauce or even chocolate did not work.  I would have to force it down her, sometimes by lying her on the floor with her arms by her side with me sitting not on her but with my legs on either side of her holding her arms to her sides while I used by hands to hold onto her chin and slowly pour the medicine into her mouth.  She also fought bedtime for years, first flinging herself out of her crib and then refusing to stay in her bed.  This would go on for an hour-and-a-half every night, with Elliot and me constantly picking her up and returning her to her bed until she would finally fall asleep exhausted on the floor, and we’d then put her into her bed.  We finally put a gate in the door of her room, but then she started climbing over that, and so we put a second gate on top of that, and she would stand there crying and shaking the gate.  Not immediately, but early into her time with her cochlear implant, Rachel decided she would fight me on putting her cochlear implant processor on in the mornings.  It was not about not wanting to hear because she was not only fine with it once it was on but would then fight me about taking it off at night.  For awhile, we had to let her go to sleep with it on and remove it once she was asleep.  However, to put it on her in the morning I had to resort to the same sitting over her method that I used to give her medicine.  I used to tell myself then that her strong willed personality would stand her in good stead when she was older, and I was so right as her motivation and determination have taken her far, but it was not fun then!

Trying to Find Our Way

Wednesday, February 18th, 2009

I spent the first 24 hours after receiving the definitive diagnosis of Rachel’s deafness crying.  We were living in Boston and had no family in town, and so I mostly spent those 24 hours on the phone crying.  We knew nothing of deafness.  We wondered about whether Rachel would have to attend a special school, whether she’d ever be able to speak, where she’d find friends, and we thought about all the joys that hearing brought to our lives that we took for granted and were sad for what she’d be missing. 

Then, I made the first connection that helped me immeasurably.  My father was the lower school principal at a private school in Baltimore.  Unbeknownst to me, he had a deaf student in his school.  I think she was in 5th grade at that time.  Her parents were grateful to my father for giving their daughter a chance, and, after getting my permission, he called them for me.  I was on the phone with the mother , Esther, within an hour.  She told me about the oral approach and about the A.G. Bell Association for the Deaf, which, at that time, gave parents a free first year of membership.  When we went to Baltimore to visit my parents a few weeks later, she had us over to her house along with a friend of hers who also had a daughter who was deaf and oral.  She had reams of information ready for me, and having someone guide me through the early stages of learning about deafness was both invaluable and comforting.  This mother became my mentor.  I kept in close touch with her in those early years. 

Even though we were convinced by speaking to Esther that we were on the right track with the oral approach, we still explored all of our options.  We contacted the Massachusetts Association for the Deaf and received names of a few families in the area who were using the various approaches.  We spoke at length with two families, one that was oral whose ten-year-old son was doing beautifully and could even carry on a conversation while riding his bike, and one whose daughter had had meningitis at age three so had had speech and language already.  However, the family decided that hearing aids were a pain, got rid of them, and committed to using ASL.  Their daughter was now eight and had lost all of her spoken language.  To us, there was no comparison in which child we wanted Rachel to be like.  We wanted her to have the ease of communication that the boy did. 

This was also in the pre-internet days.  Thus, information wasn’t as readily available as it is for parents today.  Instead, I went to the library and copies reams of articles on deaf children and language, many of which I still have in my file today.  I also discovered the A.G. Bell Association’s book catalog and started ordering and reading books which still line my shelf today, books by Daniel Ling and Arthur Boothroyd and my early “bible,” Learning to Listen.  Later, I added books by Warren Estabrooks.  We also contacted the John Tracy Clinic and began their correspondence course.

When Rachel was three months old, she received her first hearing aid.  It looked something like this:

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The buttons attached to earmolds that stuck out of her ear.  When she was four months old, she got one for her other ear.  I sewed pockets into onesie t-shirts to hold them.  The difficult part, though, was that the microphone was not on the earpiece as with today’s body worn cochlear implant processors.  Instead, it was on the box.  So, keeping the aids under her clothes was not a good idea because the sound would then become mufflied.  Here is a photo of Rachel at about 6-8 months old wearing her hearing aids.  While we got used to them, I have to admit that it was difficult for me to take her out in public with these big things, and I always had the onesie with the boxes inside her clothes when I took her out.  Overalls worked well because then the tops of the boxes, where the microphones were, were not covered.

 

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When Rachel was four months old, we started out at a program that was overseen by a professor at a graduate school of Communication Disorders.  What benefitted me the most in that program was the parent group the professor had set up once a week.  The children stayed in the nursery while we all had a chance to talk.  Being able to share my feelings with other parents who understood how I was feeling was invaluable in helping me get past the grieving process and on to focusing all of my efforts on helping Rachel.  To this day, almost 22 years later, I am still in touch with one of those mothers. 

When Rachel was five months old, I returned to work part-time.  I remembered Ralph Guertin, the oral deaf man whom I’d interviewed for a job in my department when I was pregnant with Rachel.  He had not gotten a job in my department, but I looked him up in the company directory and saw that he was now working in another department.  I walked over to his building one day shortly after returning to work.  He remembered me and was very surprised to learn that I’d had a deaf baby.  He told me all about the Clarke School in Northampton and how happy he had been there.  A few weeks later, he came to my office and asked me to join him during lunch for a disability fair taking place at our company.  He took me to a table where a teenage girl was with her mother.  The girl was deaf and had gone for five years to Clarke.  I spoke with her and her mother at length and left very encouraged. 

Elliot and I drove two hours to Northampton and visited the Clarke School with Rachel when she was six months old.  While we were impressed with the facilities and with the professionals there, we left discouraged by the students.  At that time, Clarke specialized in very profoundly to totally deaf children.  They were all tethered to a center bank by FM cables, and most had very poor speech.  In addition, we sat in on a class of 15-year-olds and were very discouraged by the low academic level.  This was not the future that we wanted for Rachel.  We wanted her to be able to hear and speak so that she could communicate with the entire world and not just a small portion, but, at the same time, we didn’t want her to struggle so greatly to communicate.

When Rachel was 10 months old, we switched hospitals to Boston Children’s Hospital, and she got her first pair of ear level hearing aids.  I was so happy to get rid of the big, clunky body worn aids.  At the same appointment, the audiologist got her first accurate hearing test results through behavioral testing, and these results showed that Rachel had more hearing than we’d initially thought.  Where the ABR had shown no hearing above 1000 hertz, this showed hearing across all the frequencies, albeit at the level of a profound loss from 1000 hertz on.  

Unfortunately, unbeknownst to us at the time, Rachel began to gradually lose her residual hearing right after this appointment.  We noticed her diminishing response to sound but thought that it might be because she was experiencing recurring middle ear infections.  The ENT agreed and put tubes in her ears.  The ear infections stopped, but her hearing did not improve. 

One other thing worth mentioning is that Rachel was very late sitting and walking.  It went hand in hand with what her pediatrician had observed about her head control.  She didn’t sit until age 10 months and walk until age 21 months.  Once she started walking, her balance was very poor.  We now know that this is par for the course for deaf children whose vestibular system is also affected, but none of the professionals knew that then.  We even visited a new pediatric neurologist who was supposed to be world renown, and he proclaimed his theory that a flu-like virus I had had five months into my pregnancy had resulted both in Rachel’s deafness and in an “isolated insult to her central nervous system,” resulting in her gross motor delays. 

As good as the parent group at the graduate school was for me, the part of the program that was for Rachel was not overly beneficial.  It was run by grad students, some of whom wanted to be there and others who didn’t but had to be there because they needed the credit.  We were very fortunate to have one of the best of the group of grad students, Shari.  Shari was very bright and very eager to learn and to help Rachel.  She was outgoing, animated and full of energy.  We even started using her as a babysitter because she was so wonderful with Rachel.  That summer, though, we no longer had Shari.  Instead, we had another student who was not enthusiastic, who had no idea what to do to help Rachel, and who had no energy.  I became anxious about Rachel losing valuable time. 

We decided that we needed to find a better program for Rachel and, once again, I went back to my mentor.  I was speaking to Esther on the phone, and she said to me, “If I had to do it all over again, I’d go the Beebe Center.”  She went on to say that she had met children at an AG Bell convention who were learning language through the Auditory-Verbal approach and that she was incredibly impressed by them.  So, I got on the phone to the Beebe Center in Easton, Pennsylvania.  At the time, their Larry Jarret House program was a program for families from out of town to come learn about the Auditory-Verbal approach and immerse themselves in it for a week.  The center was in an old house with the therapy rooms and offices on the first floor and the apartment for the Jarret House on the second.  This was in 1988, and, since there were very few Auditory-Verbal therapists and centers then, this program was greatly in demand with a waiting list as long as five months.  However, we got lucky as they had just had a cancellation for the next month, September.  So, off Rachel and I went for a week, accompanied initially for the first three days by my parents and then by Elliot for the last three. 

The Jarret House program was amazing.  Rachel had an hour of formal therapy every morning, including with Helen Beebe herself, one of the founders of the Auditory-Verbal approach and a living legend at the time.  We were fortunate to have that time with her as she passed away about a year later.  During the rest of the day, we would watch other families’ therapy sessions, speak with other parents, even visiting one family in their home, and watching informative videos.  During three evenings, one of their therapists visited us in the evening and showed us how we could incorporate language learning into our everyday lives.  I remember her asking me if we had a dishwasher, to which I replied yes.  She told me I couldn’t use it all the time, and then she proceeded to demonstrate for me how to stand Rachel up on a step ladder and have her help me wash dishes, all the while feeding in language about washing and soap bubbles and hot and cold, wet and dry, etc. 

After that week was over, I was supposed to go to Philadelphia with Rachel to stay with my college roommate for the weekend.  However, I was so eager to get started that I cancelled that and returned home with Elliot, running out the very next day to an educational toy store to get materials so that I could get started on working with Rachel at home.  We also left the program at the graduate school and began working with Lea Watson at the Auditory-Verbal Communication Center in Gloucester, Massachusetts, for an hour twice a week.  It was a 54 mile drive each way that took me a full hour, but I was so committed to the Auditory-Verbal approach after seeing how well the kids were doing with it that I was willing to do whatever it took.

One of the early activities involved using toy animals and vehicles to teach Rachel the “learning to listen sounds.”  These sounds comprise the full range of speech frequencies, and, if Rachel could learn to discriminate those, then she could learn to discriminate speech.  One of those sounds was, “ahhh,” which we said while flying a toy airplane.  I remember how excited I was the day Rachel picked up the airplane and said, “ahhh.”  I even called the Beebe Center to tell them.  However, when Rachel was 18 months old, one day she picked up the airplane and mouthed “ahhh” with no voice. 

It was around this time that we began to experience professionals giving us their unsolicited opinions about our communication choices for Rachel.  Rachel was in a daycare/preschool the three days a week that I worked.  After we’d been there a few months, the director and school social worker called Elliot and me in for a meeting.  They informed us that we should be signing with Rachel, that they had already spoken to their teachers and that they were willing to learn some sign.  We were angry but calmly explained that we had done our research and made an educated decision and that we had chosen not to sign with Rachel and didn’t want anyone signing with her.  They accepted our choice because they had to, but they continued to badger us throughout Rachel’s time there.  In addition, we experienced a similar situation with some of the professionals at Boston Children’s Hospital, Rachel’s ENT in particular, who let me know at almost every appointment that she was aggravated with our choice and even called the Chief of ENT in one day during Rachel’s appointment, and he proceeded to lecture and argue with me for a half hour.  He told me that Rachel would never be able to learn to speak English well.

Lea was having difficulty getting Rachel to demonstrate any consistent response to sound or any indication that she was comprehending any spoken language through her hearing.  She referred us to one of the only Auditory-Verbal audiologists in the country, Judith Marlowe, in Winter Park, Florida.  She had seen other students of Lea and had made them new earmolds that resulted in a 10+ decibel gain in their hearing.  We had three days booked with Dr. Marlowe.  However, after the first day, she told us that Rachel appeared to have absolutely no residual hearing and that there was nothing she could do to help her hear more.  She gave us literature on a vibrotactile aid from a company that, coincidentally, was located about 20 minutes away from where we lived, and on the cochlear implant.  However, she told us that results from a vibrotactile aid were about equivalent to what they were seeing from the cochlear implant.  The cochlear implant she was referring to, though, was the early single channel implant. 

We did not have much money then.  My husband was still in his surgical training, and I was working three days, but between paying for daycare plus for Auditory-Verbal therapy, which our insurance refused to cover, things were very tight.  However, we needed to try that vibrotactile aid, and so $1000 later, Rachel was wearing a box with two wires that ended with disks that we taped each day to her chest.  One vibrated for low frequency sounds and the other for high frequency sounds.  At the very least, we hoped it would make Rachel aware that people were using their voices because she continued to speak by mouthing words with no voice.  The vibrotactile aid proved to be totally useless.  Rachel did not protest wearing it, but neither did she pay any attention to it.

After returning from Florida, we spoke with Lea.  Her first reaction was that we needed to find another approach to use with Rachel, that she clearly could not be Auditory-Verbal without the auditory part.  The approach is based on the premise that deaf children can learn language naturally through their hearing if they are trained to use their residual hearing to the maximum.  At the time, that meant hearing obtained through hearing aids, but, even with the most powerful set of hearing aids, Rachel could hear nothing.  We found another therapist who taught spoken language in conjunction with sign, and we were set to meet her.  Then we returned to Lea, and she said that she had been thinking about us and was reluctant to let us go because we were one of the most Auditory-Verbal families she knew even though Rachel couldn’t hear.  She wanted to help us, and she decided that she would use the same types of activities that she did with her other students but would incorporate lipreading and other visual and tactile cues into her lessons. 

We started holding everything we talked about up to our mouths.  Rachel proved to have a gift for lipreading, and she very quickly developed a receptive vocabulary list of 100 words.  She would also vocalize a few words, although most she still mouthed.  We were beginning to get discouraged and were beginning to think we’d have to add some sign language.  Then Lea made a connection for us that changed Rachel’s and our lives.

Another Beginning

Tuesday, February 17th, 2009

In 2001, I was a happy mother of two boys, expecting my 3rd child. We lived in Indiana, and were enjoying living close to my parents in Michigan. I was also deeply involved in my local La Leche League group as a Leader and also as a District Advisor. I enjoyed leading meetings for mothers and helping them through those first few days and months with their cherished babies. There is nothing more satisfying, as a mom, than helping other moms have a good bonding experience with their breastfed babies.

When Elliot was born, he was perfectly healthy, 8 lbs. 3 oz. However, in the hours following, he would choke or gag in a strange way. Being an experienced mom, I knew it was not normal. Finally, he did it when the pediatrician was there for the checkup. He admitted him to the NICU. Nothing specific was determined with tests but at one point, after he seemed fine, it was almost time to leave the hospital and the newborn screening machine was brought down for the hearing test. The nurse was young and inexperienced, so she couldn’t get it to work– or so she thought. She tried twice; it failed to work both times. Finally she got help, at which point they decided that it was really and truly a referral for an ABR in a week. I didn’t know what to think, and they furthered my hopes at that point by saying that it was probably just fluid in his ears. Incidentally, I believe that the reason for his gagging problem was vestibular in nature, though I have no way to confirm this.

Elliot with his family

We loved this baby up, and waited for the appointment.

The Beginning…

Tuesday, February 17th, 2009

Before I had children, I worked full time at an engineering consulting company.  When I was near the end of my pregnancy with my first child, Rachel, my supervisor asked me to interview over lunch a perspective candidate for a job in our department.  The man I interviewed was named Ralph Guertin, and he was profoundly deaf.  At the time, I knew nothing about deafness or deaf education.  Ralph communicated orally, using what little he got from his hearing aid and lipreading.  He impressed me greatly.

About a month later, on May 7, 1987, my father’s birthday, Rachel was born, two weeks before she was due.  I actually went into labor the night of May 6th, and I kept asking the nurses and doctors throughout my labor if she was going to be born on the 7th.  I knew that my parents wanted a girl, and so when Rachel turned out to be a girl, I couldn’t wait to call my father with his birthday surprise.  Because she wasn’t born until after 5:00 in the evening, I didn’t get to a phone until after 6:00, which was about the time of day that I usually spoke to my parents anyway, and so my mother didn’t suspect anything unusual when I called.  She thought I was calling to wish my father a happy birthday and said that I had just missed him, that he had gone out for a run.  I tried very hard to disguise my excitement when I said to her, “I have a birthday surprise for him.”  My mother asked me what, and I replied, “a granddaughter.”  She gasped and said, “What did you say?”  Once she got over her surprise, I made her promise not to tell my father, to let me call and surprise him.  However, when he came back from his run, she couldn’t contain herself.  When she threw her arms around him despite his being all sweaty, he knew something was up.  When she told him that he had a granddaughter born on his birthday, he had to sit down.  I am an only child, and this was their first grandchild.  They were wonderful parents and couldn’t wait to be grandparents.

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Elliot and me with one week old Rachel

I came home from the hospital two days later, and my parents flew to Boston from their home in Baltimore to see the baby and help Elliot and me during the first week.  I don’t know exactly when or what first made me suspect a problem with Rachel’s hearing, but my first conscious memory of thinking about it was when I took my mother to the airport and she told me not to slam the door because I would wake the baby.  I know that I was already aware then because I remember thinking that I wasn’t so sure it would wake her.  At some point during Rachel’s first week of life, I had already noticed that she didn’t startle to loud sounds.

Elliot was a surgical resident at the time.  I mentioned my concerns to him, and he looked up babies and hearing in one of his medical school textbooks.  He found something saying that babies don’t respond normally to sound until the age of four months, and we both latched onto that.  However, we continued to grow uneasy.  When I said something to our pediatrician at Rachel’s first visit when she was about three weeks old, he told me that I was just “an overly anxious new mother who didn’t understand how newborns react to sound.”  At her two month checkup, he noticed that her head control was poor, something I wasn’t aware of because Rachel was my first child.  She would bob her head up and down on my shoulder because her neck muscles were weak.  He suspected cerebral palsy and referred us to a pediatric neurologist.  The neurologist did all sorts of tests but did not mention Rachel’s hearing until I asked him about it.  He then did a few rudimentary tests and said that we could have her tested.

The night before Rachel’s hearing test, the smoke alarm went off in the hall right outside her  room when I burned something cooking.  She was happily playing in her crib and didn’t even flinch as the alarm was blaring.  I knew it wasn’t good.  The next day, we took her to Massachusetts Eye and Ear for an ABR test.  It was only supposed to take about an hour, but Elliot and I waited for 2 ½ hours in the waiting room knowing as time went on that we weren’t going to get good news.  When the audiologist brought us back into the room, she told us that our suspicions were correct, that Rachel had a severe-to-profound hearing loss.  That terminology meant nothing to me at the time, and so I asked her if Rachel was deaf.

The audiologist immediately took us to meet with another audiologist and a social worker.  Elliot and I were in shock and were not thinking straight.  Plus, we had a very fussy two month old baby to deal with.  The audiologist and social worker proceeded to ask us if we knew anything about the controversy in deaf education.  When we told her no, she told us that there were those who believed in sign language, those who supported an oral approach, and those who believed in using both.  I don’t remember their exact words, but they pushed the Total Communication approach in their presentation.  Knowing nothing, we replied that we guessed both would be good.  They then gave us one referral, to a TC school, and sent us off.