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Taking CHARGE

Posted Aug 09 2009 12:27am
I don’t know why I didn’t read " Choices in Deafness " from cover to cover. Looking back on it, I probably should have read it at least twice. But when we began our journey with a profoundly deaf daughter, the initial decisions were fairly straightforward. Would we begin sign language or would we pursue hearing and speech through a cochlear implant? There were bumps in the road, but relative to all our other decisions, it was not a hard choice to shoot for the moon with a cochlear implant. My husband and I are optimistic people and I don’t like to apologize for that, but in the case of our daughter, Adele, things don’t always go as planned.

Adele made quite an entrance to the world just over 5 years ago. With no warnings or complications during pregnancy, everyone was surprised when she needed resuscitating at birth. She was whisked to the NICU and there were not many encouraging words for us first-time parents. There was literally no time for a single “congratulations” before the silence of worry and concern set in. Within a few days, Adele was diagnosed with CHARGE Syndrome, a complex genetic disorder that affects multiple areas of development. Over the next three months in the hospital, almost every specialist had something to add to her list of diagnoses, which included inability to swallow, heart abnormalities, and possible blindness; so the fact that Adele had failed several hearing tests just fell into the pile of bad news. It would be almost a year before she was medically stable enough to really consider our options for hearing.

Because she was in-patient at Children’s Hospital for several months, the Hearing and Speech audiologists were able to see her often. We also had time to learn more about CHARGE Syndrome and found out that 90% of patients have hearing loss and many are also vision impaired. In fact, CHARGE Syndrome is the leading contributor to the deaf-blind population. Thankfully, by the time Adele was 6 months old, she was showing signs of good vision and we knew she would not be blind. We were elated that she could see and continued to think positively and prayerfully about her hearing.

Adele was wearing a hearing aid at 9 months and multiple tests had confirmed that she was profoundly deaf. As her medical condition became more stable, we began making visits to the HEAR Center to discuss our options. As with all implant candidates, it was a team effort. Our speech therapist was focused and realistic, our surgeon was experienced and thorough, and we as parents listened and processed everything as best we could. It was decided that she was a good candidate, but due to inner-ear abnormalities and facial paralysis, there were a few extra steps necessary to determine which ear to implant. Dr. Audie Woolley referred us to the University of Michigan for a second opinion with his friend and colleague, Dr. Steve Telian , and also for a procedure to test the nerve viability on both sides (promontory stimulation). The results of that visit were positive to go forward on her right side, (opposite facial paralysis) and we allowed ourselves to get excited.

Adele’s surgery day did not go as expected, and midway through the procedure Dr. Woolley let us know she would probably not be able to get an implant. Though undetectable on all the previous imaging, Adele had a large blood vessel blocking the path to the cochlea, putting her at increased risk for complications. It was a huge disappointment, but, thankfully, it didn’t last long. A few weeks later, Dr. Woolley had a new plan to work around the vein. Working together with Dr. Telian, Dr. Woolley successfully implanted Adele’s right ear in December 2005; it was a very good day.

Adele’s activation day was not as dramatic as some, but we were delighted to begin Auditory-Verbal thera py . Adele made some progress, but after nine months of therapy she had not mastered the first-level benchmarks. Our concern was strong enough that along with our AVT (auditory verbal therapy) therapist's recommendation, we began looking at sign language options. Up to this point, our decisions about Adele’s hearing had not been that difficult. All of our hopes had been in the implantation and with hearing and speech. Choosing which type of sign language – Signing Exact English or ASL – was much more difficult.

We knew virtually nothing about either type of sign language and were very anxious. We immersed ourselves in reading materials, the Internet, and tried to find unbiased opinions. Even now, over 2 years later, I don’t know how anyone can make a decision between SEE and ASL when you have so little personal experience. Nonetheless, we officially chose SEE because of the emphasis on English word order and our long-term goals of strong reading and writing skills. However, ASL was also a part of our world because of our involvement with other special-needs children at The Bell Center and Hand in Hand , and also the wonderful PBS program “Signing Times.”

This was a period of quick acquisition of sign language and a first introduction to Deaf Culture. What a new world for us! Watching Gallaudet’s documentary Through Deaf Eyes made us excited for how accomplished people with hearing loss can be. But it also made us very sad because we were starting to realize that often people with multiple disabilities, such as CHARGE Syndrome, are not a part of Deaf Culture. Through other readings and discussions, we learned that sometimes this is by choice but oftentimes it is by exclusion, which made us extremely concerned for Adele’s future within the deaf community, SEE or ASL.

Balancing that sadness, however, was the delight that Adele was learning signs as quickly as we were. With CHARGE Syndrome, there is a possibility of cognitive impairment and since she was so young, we did not have any clear indicators of what her intellectual abilities were. Adele was proving to be very bright and as many people like to say “as quick as a whip.” It was reason to celebrate and one of several factors that led our therapist to remind us about another option for communication: Cued Speech. From "Choices in Deafness" and other sources, we learned that Cued Speech is a method for visually communicating English using the phonemes of the language, not symbols for the words like sign language. In simple terms, it is a combination of lip-reading and about 40 hand signals that match the consonants and vowel sounds of English. In addition to Adele’s cognitive strengths, several events led to our consideration of Cued Speech.

We realized that even though we were very devoted to learning sign language and were gaining vocabulary rapidly, we knew there was a strong possibility that Adele’s communication needs could outpace us. We did the math and the number of words we needed to learn per day was somewhat overwhelming. If it was overwhelming to us as parents, what could we expect from everyone else in our lives? Without a strong circle of signing friends, including our own adult friends and family, it would be difficult for us to learn sign language beyond a third-grade level (about 5,000 words) and it would never be our natural language (as it is for deaf parents of deaf children). It seemed more common than not, that at some point, parents were not able to say everything they wanted to say to their children because of vocabulary limitations. That was very concerning to us, especially since there was a chance that the signing deaf community would not embrace her special needs.

Of more immediate concern was that I was finding myself unprepared to talk to Adele about what she was interested in on a daily basis. On a trip to the zoo, I prepared thoroughly and thought I knew the sign for every animal she could possibly want to see: monkeys, lions, tigers, elephants, zebras, rhinos, parrots, giraffes, turtles, bears, seals, etc. But when she stopped to watch the flamingos and the ostriches, I did not know those signs and, even worse, could not find them in the sign language book we had in our bag. We had lost that teachable moment forever and there would be more to come.

From our initial feelings on sign language, we still had a strong appreciation for the literacy-building opportunities in Signing Exact English. Cued Speech offered even more advantages for learning to read and eventually write. The phonemic awareness from Cued Speech allows a child to learn to read the same way a hearing child can learn. Through the hand cues, they have a visual reference for the sounds in a word and can “sound it out” allowing them the same opportunities as a hearing child. If Adele is like most kids, we foresee a lot of emailing and texting in her future and we would like her writing to reflect strong language skills and not be limited just because she cannot hear.

As we learned about Cued Speech from the book and other writings and research, we were intrigued but not without concerns. We read as much as we could and found one invaluable resource in another parent of a child with CHARGE Syndrome. Their daughter was 30 years old, doing ver y well, and had begun using Cued Speech in preschool. We were impressed enough to give Cued Speech a try. However, our biggest concern was (and continues to be) how well Adele would be able to express language with Cued Speech. Because part of the visual system depends on the mouth shape of a phoneme, Adele’s facial palsy and oral motor weakness posed a problem. It was an unknown risk that we talked about but felt that even if her expressive language fell into the “worst case” scenario, there would be ways to overcome it. The pros seemed to outweigh the cons and we moved forward.

With help from our HEAR Center therapist and a DVD tutorial, my husband and I learned the hand shapes and placements for Cued Speech in just a few days. Within a short time, we could put it all together to say anything we wanted to Adele without having to look it up. Our speaking was very slow at first, but it was very liberating to be able to say anything without first consulting a book. It also didn’t take long to notice that because Cued Speech forces your focus to the mouth area, Adele was paying more attention to our voices and using the hearing that she does have from her cochlear implant. We know that she can hear some common routine words without cues and her lip-reading skills are also quite good. Given her challenges, these skills can only add to her competence long-term in the hearing world.

Thankfully, Adele’s preschool embraced our decisions and we have been supported by them as well as the HEAR Center. In the two years we have been cueing, Adele’s receptive language has flourished and she is still proving how quickly she can learn. Literacy continues to be a driving force and reading children’s books has been one of the best ways to advance her vocabulary and increase the speed of our cueing. To her delight, both Adele’s grandmothers have learned to cue, as have her aunt and several therapists. We have taken her to 2 summer camps for Cued Speech and met families from all over the country who cue. We have even met a few more families with children with CHARGE Syndrome who are cueing. Even though our Cued Speech network is small, it is growing!

It is worth noting that most Cued Speech advocates recommend that a child learn ASL at some point, and we agree. The social aspects can be very rewarding and we hope that will be the case for Adele. Even though we, her parents, will probably never be as fluent as we would like in ASL, she can learn in other settings when she is a little older.

Though most of our efforts are focused on Cued Speech, signing still has a place in our home. Adele uses the initial signs she learned as a base of expressive language, as well as cues and gestures. Her expressive language has not progressed as rapidly as her receptive and that gap has been widening rather than narrowing. This is not all bad news, though, because her receptive language is amazing; we just need to help her match it with expressive language. Once again, we began looking for another piece to our ever-growing communication puzzle.

We posed our situation to as many professionals as we could over a period of several months. It was challenging to find people who felt they had adequate experience with a child like to Adele to make a recommendation, but we did get feedback from New York, North Carolina, Maryland, Michigan, and, of course, from our home team in Birmingham. The evaluations were not formal, but the recommendations were unanimous for incorporating an additional approach: Augmentative Communication . At Cued Speech camp, one of the therapists recommended a book with an unusual title, Schuyler’s Monster . It is a father’s story about his voiceless (but hearing) daughter’s journey to communication. While I do not agree with a lot of the author’s feelings, their story related to ours in many ways. They were using an Augmentative Communication device and it seemed, more than not, that this could be a good option for us.

It took almost a year before we were convinced to try Augmentative Communication. We did not want to overwhelm Adele, ourselves, our family, our therapists, our school syste m, etc. But over the summer we have gone through the evaluation process (and insurance process for coverage) and with the help of Easter Seals we are thrilled to have received our own device this very week. It’s official name is “ Vantage Lite ” from Prentke Romich Company, but we affectionately call it “Adele’s Pink Talker.” If you are not familiar with this type of device, it uses symbol icons to offer many choices of language, up to 5,000 words, and it speaks them for the user. Another benefit of the device is that it spells the words and sentences on the screen, furthering her opportunities to build literacy.

Because Adele cannot necessarily discriminate the device’s voice output, we are cueing everything to her as she learns what words the icons represent. We hope it all comes together smoothly but know it will take time. In the few days she has had her “talker,” she is already surprising us with what she wants to say.

We are not sure what Adele’s communication will look like in the future, whether it will be Cued Speech, output from her “talker,” ASL, or quite possibly from her own mouth. In the five years that we have been working on her hearing and speech, we have also been working on her oral motor skills for eating, to the schedule of up to three therapy sessions a week. Over the summer, Adele has surprised us all again by learning to eat small meals by mouth and drink enough nutrition through a straw to skip a few of her tube feedings. It almost feels like a miracle, but we will save that for another story. In the meantime, we remain optimistic that Adele’s head is filling with language and that someday she will be able to tell us everything that is on her very special mind. Her little hearing sister Margo (two and a half) has no problems communicating with her big sister. She's even picked up a few cues as well by watching us.








Sometimes it's the challenges that teach us appreciation. What seems small to some, can be great accomplishments to many families with children who have special needs.







Written by Julie Brandrup, mom to Adele (CHARGE Syndrome) and Margo
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