Twists and Turns:
Journeys with Implants
Cochlear implant success stories: you can find them everywhere on the internet, in videos and on brochures. The cochlear implant has often been described as a medical miracle. Michelle Tjelmeland and her daughter, Ellie, had such a positive experience with one implant that they both obtained a second implant -on the same day. Michelle created a website to share their experience with other parents through www.iloveellie.com. "I always say that is the absolute best personal decision I've ever made. The implant has truly given me life back--I have regained my independence and freedom. I can use a drive-thru, talk on my cell phone, listen to my Ipod, hear the turn signal on my car, listen to the giggles of my girls running thru the sprinkler, and talk to my husband from another room--all things I couldn't do before my implants. My hearing still is not perfect, and I still sometimes struggle, but I will take what I have any day over what I experienced prior to being implanted."
Michelle was so thrilled with her newfound hearing from her implants, and with her daughter's progress, that she was inspired to set up a foundation: the Cochlear Implant Awareness Foundation. Through her website, www.ciafonline.org, Michelle provides information and resources for individuals who need assistance in obtaining implants.
Many children have benefited greatly from cochlear implant technology. But not everyone has the happy ending that they hoped for with an implant, and they often feel confused and frustrated. Some families encounter twists and turns during their journey with an implant when the outcomes they expected or hoped for weren't realized.
"We Didn't Know This Could Happen"
Jane Holtz was in the car, heading home from a play when her daughter Lauren suddenly said, "Mom, I can't hear anything!"
Jane was puzzled, because she had just changed the batteries in Lauren's implant earlier that day, and she knew that there was enough power in them. When they arrived home, Jane changed the batteries again. Nothing. She changed every part, one by one. Again, nothing. Jane's heart sank. Oh no, the internal device broke, she thought to herself.
"I was upset and worried all at the same time," Jane recalled. "I was feeling bad for Lauren, mad at the implant company and upset that she might need more surgery! And did I want that for her? I wasn't sure.."
Jane's husband felt there was no question that they would go ahead with surgery again, this time with a different brand of implant. They talked to Lauren and asked for her input. Lauren gave it a lot of thought and, being a huge Beatles fan, decided that she wanted to hear music again. It took three months to get insurance approval and a surgery date set and several months to get a comfortable mapping with the new implant.
"I was angry at the implant company," Jane continued. "And I don't know that I have ever resolved that issue. They didn't tell us everything-too many devices have failed for them not to know it was a possibility."
Looking back, Jane recalled that the surgeon never brought up the possibility of device failure and the potential for re-implanting the device.
"My assumption was that it would last as long as it was in her head," said Jane, "and it could just be updated with software." Jane noted that the surgeon did not discuss the durability of the implant during the consultation for the second surgery until she asked questions about future failures. Lauren's first implant was just four and half years old.
"It just doesn't seem to be on their list of things that parents get told," she said.
We Weighed the Risks
Sara Kennedy, however, had a different experience with her daughter's implant. The team did discuss the possibility of device failure and the difficulty with re-implanting.
"They indicated it was possible to re-implant during her lifetime, but difficult, and not always successful. They talked about the surgeon's success rate in getting it placed correctly." The implant team also shared two stories of kids who had been re-implanted and why they were re-implanted (the internal device stopped working).
"In the end," said Sara, "we decided we didn't have much to lose since she had no response to sound in that ear. But the other ear, -- for us anyway--we are not touching. If she wants to get a second implant later...it's up to her." Sara's daughter is gaining incidental learning and new listening abilities with her implant.
Sara continued, "When we were researching the surgery, I noticed that we quickly came to a glass ceiling in terms of talking about "success." The implant itself does what it is designed to do at a very high rate of success. We heard 97%, much like that expected of pacemakers. But that isn't the success that parents care about. The difficulty is predicting whether or not a person will actually learn to use the auditory channel...or really, what is the point? Our daughter, then age seven, was considered far past the desirable age for implantation and later language learning. Studies of 'who was successful and why' didn't seem to apply to her "aged" status. My gut tells me that it's the kids (or adults) who liked wearing hearing aids, who look down your throat as you speak, who find speech therapy strangely interesting, who can make sense even out of their limited hearing, and if they sign, their circle of family and friends keeps signing to bridge the switch from visual to auditory... these are the kids who will make better use of implants."
"I get so irritated," says Sara, "when I hear that 'Child X was mainstreamed after the implant.' Mainstreaming is not a success, it's a location."
The Wagner's Story
For Katy and Kevin Wagner, their journey with two implants was laced with disappointment, frustration and sadness. Their son Dylan was 22 months old when he contracted meningitis. They were told that they had to make a decision as soon as possible, as the window of time for implanting a cochlear implant was closing quickly. X-rays had shown the beginning of cochlear ossification.
"There was no time to do research," said Kevin. "We had to take the doctor's word for it, that it was the right thing to do."
Kevin recalled asking the implant surgeon about the success and failure rates with implants. The doctor reassured them by stating that the implant center had seen great success.
"Part of the problem," said Katy, "is that we received biased information. All of the materials from the implant center dealt with the success stories."
When the day for activation came, the Wagners waited with great anticipation for the hook-up. The doctor came in and announced that they needed to do another x-ray before they could proceed. The x-ray showed that the electrode was not in the right place. The only option was to do another surgery and re-implant.
"Our hearts dropped to the floor," continued Kevin. "We had just put our child through surgery and had to do it again."
"We didn't obtain any response during the activation of the second implant," said Katy. "It was emotionally exhausting to go to mappings over and over and get no response." After a year, the Wagners made the decision to stop using the implant.
Katy had met two other moms with deaf children at a parent-infant program and they became fast friends. The other children also received implants and did quite well with them.
"I felt jealous," said Katy. "It was hard seeing other children get good use out of their implants. I asked myself, 'Why not me?' Even though they're my friends, it was hard for me to feel happy because we didn't have the same outcome."
Deaf Plus with Implants
Leslie Kaufman shared similar feelings. She has a five-year-old daughter with CHARGE syndrome. CHARGE syndrome is a genetic syndrome that differs from child to child, but often affects vision, balance, and hearing. Katie received her implant at 18 months.
"We were excited at first," said Leslie. "We thought she would begin to hear right away and learn to speak." In the sound booth, Katie's responses were measured within the speech range, but Leslie discovered that she wasn't seeing the same results at home. "She turns to her name at home with it. But responds to very little else. She responds to voice and music much more than environmental sounds. I've never seen her respond to one environmental sound with (the implant)."
Leslie found it hard to watch the progress of another child the same age as Katie who received an implant at the same time, but with far different results.
"Even though I have totally accepted that Katie needs a Total Communication (including sign language, speech, gestures, etc.,) approach," said Leslie, "I'm still not looking forward to seeing this other little girl back in our district, watching her have optimal success with her implant while Katie struggles to communicate even simple needs. Just a bit of jealously, I guess."
The Kaufmans began to connect with other families with children who have CHARGE syndrome and noticed that many others had similar results with their implants.
"Almost every family I've talked to has had the same results-some detection with it, but very slow progress, and they usually need additional forms of communication besides just voice," said Leslie. "I personally think there are auditory processing issues. CHARGE seems to include a lot of different types of processing issues, some for more kids (like Katie) than others. I think CHARGE can include a lot of neurological "difference"-at least that's what we see with Katie."
Looking back, Leslie pondered what she learned over the last five years.
"If I could change anything," she says, "I would have researched it more to see what the results for others with CHARGE had been. I don't think it would have changed our decision to do the implant, but I would have been more prepared for what to expect.
"The implant team was great in terms of support, but they honestly didn't know what to expect from Katie." Leslie continued. "She was the first child here with multiple disabilities to be implanted, and I honestly think they thought she would have the same results as other kids.
"Our only exposure to implants before Katie got one was visiting an oral school that teaches kids with implants to talk. So that was our first expectation. When you set yourself up for those kinds of results, you're in for some big adjustments later."
What If We Don't Succeed?
"Families who choose implants are set up to either succeed with the implant, or fail," believes Susan Raad, owner of Communication Clubhouse in Illinois and mom to two deaf and hard of hearing children.
Susan's son, now 13, obtained an implant at 18 months with good results.
"The very first day, he responded to a dog barking!" Susan recalled. Despite a background in speech, language and audiology, Susan found herself vulnerable as a parent in a field that is often polarized. Prior to obtaining the implant, a doctor suggested that she pursue a certain method of communication, and Susan found herself pursuing it, even though her son's audiogram showed no response.
"Even with my background, I found myself acting in irrational ways based on my hopes as a parent, rather than what I knew from my academic background. We often talk about implants in an either/or situation. If the implant takes off, it is considered a 'success' and our child has 'succeeded.' But if an implant doesn't work for a child," she continued, "it is considered a 'failure' and parents begin to question what they did wrong. We begin to humanize the technology."
"What's more," said Susan, "it's a technological crapshoot. Most of the time, the odds are in our favor, but what do we do when it doesn't go in our favor? Families end up blaming themselves, feeling like they haven't done enough."
Susan believes that the idea of "choice" is a misleading one. "If parents are told they can simply choose a path for their child based on the decisions they make," she says, "then what happens if their child isn't able to function on that chosen path-does that mean they've failed?" She shakes her head.
Wendy Gugliemino's four-year-old daughter, Talia, experienced an unusual situation. Four days after the surgery, Talia began vomiting and couldn't sit up or walk. She was diagnosed with a perilymphatic fistula (fluid leaking into the middle ear) and sent home with medicine to treat the nausea. It took several days for the vomiting and nausea to subside. During the activation of the implant, Talia told her mom that it hurt and she began to experience nausea once again. The audiologist shut down several electrodes and tried a different mapping strategy. For five weeks, they struggled with mappings and different strategies. Nothing seemed to be working well, and Wendy noticed that her normally talkative daughter was becoming withdrawn. Wendy was persistent in getting an answer and requested another nerve response test.
"In the process of doing the NRT," says Wendy, "they uncovered a very interesting problem. One of the ground wires in the implant was not working correctly, and the malfunction was causing a power imbalance in the implant." The problem was corrected and they went back for another mapping.
"The electrodes were all turned on on a Thursday. By Monday, Talia was having no problem understanding speech and language. We had not expected it to happen quite that fast. It was so wonderful to have our happy girl back. I think she talked all weekend and asked a lot of, 'What's that?'"
Right Decision or Not?
"Our five-year-old daughter, Bo, talks non-stop and is an auditory learner," says Moira Allaby, "so when she lost nearly all hearing in her right year, the next logical step in our minds was an implant."
Bo has a progressive hearing loss in both ears. Even so, she still has good use of her hearing aid in the left.
"She was a borderline candidate," says Moira, "but after consulting with the audiologist and surgeon who we'd known since Bo was 18 months old, we decided to move forward. My husband and I researched and talked with families whose children had implants. Still, we weren't absolutely certain of our decision, more like 90%."
Moira explains, "That's what makes this decision so hard for parents, I think. Our son had to have a shunt implanted into his head in order to live. There was no question, and with him, we moved forward without hesitation. With the cochlear implant, we certainly had the luxury-or burden, depending on the perspective-of choice.
"Bo's surgery and recovery were textbook. What has been surprising is her response to the cochlear implant. Her first disappointment was not having 'princess music' piped into her processor." Moira explains that she told Bo she may be able to hear music better with the implant, and Bo believed that meant stereo sound following surgery.
"As a full-on Type A, inquisitive child who needs to know every detail of everyone's comings and goings," Moira says, "we believed Bo would easily become accustomed to and learn to use the implant. The opposite is true."
Moira explains that now, four months after surgery, Bo doesn't like wearing the processor, even though she is doing well in speech sessions, accessing language and environmental sounds quite well.
"It's hard, thinking you're opening something up for your child and then believing that perhaps you have instead taken something away-the little bit of residual hearing from the implanted ear and freedom from the clunky device itself."
Bo recently told Moira, "Mom, I talk. I don't sign." Bo still talks incessantly, but she actually is signing more than ever in her integrated (hearing/non-hearing) preschool program and is relying more on sign language.
Moira says, "On a gut level, I think we made the right decision-especially when it concerns the future. We have a lot of great support, including older kids and adults who have implants and can give us insight to their experience. As with mode of communication, every child has unique needs and styles of learning and approaching/perceiving the world around them. It would only make sense that each person's response to an implant would vary accordingly.
"I'm a firm believer that each child, if given the chance, will guide parents to their communication cocktail, if you will. We're not getting stressed about it, just moving forward, continuing with auditory training, and letting Bo lead us."
An Unexpected Turn
Within a week of getting an ear infection, Chris Stolinas' daughter, Abby, could no longer hear with her implant. Doctors suspected that a toxic strain of bacteria was to blame.
"I find it weird to explain our situation to people, because I have to say, 'My profoundly deaf daughter is now deaf!'" said Chris. "I cried a lot in the beginning, and to tell you the truth, finding out she lost her hearing this time was 1000 times worse than finding out she was deaf in the first place, over six years ago. Six years ago, she didn't know sound, so wouldn't have missed it if she never got to experience it. We weren't even sure she would be a candidate back then (because of her malformed cochlea) and were prepared to go in a different direction if need be--and we would have been fine with that. Our lives would be different than what they are now, but still just as good."
Chris is hoping that it is possibly a "soft" failure and that a future implant will help Abby regain the hearing that she worked so hard to acquire with the first implant, though she realizes it isn't likely.
Lee Ann Rotz, a pediatric audiologist at the ECHO Program at Carle Foundation Hospital in Urbana , Illinois , describes the difference between a "hard" and "soft" failure:
"A "hard" failure is when the device stops working due to either internal electronic malfunction or a structural problem such as electrode shearing from a severe blow to the head. This usually results in immediate "no sound" from the implant. Hard failures can be proven by the manufacturer (prior to re-implantation) when the company sends a representative to perform a series of tests which confirms device malfunction. On the other hand, "soft" failure occurs when a device malfunction is suspected but cannot be proven by the manufacturer's tests. Diagnosis of soft failure is determined by a very thorough and often lengthy evaluation of the cochlear implant user (by the CI team), which usually begins with awareness of declining performance, aversive unexpected auditory stimuli like a popping sound, and/or intermittent function. .All medical, programming and equipment issues are ruled out. The manufacturer representative is asked to come to the center and perform testing to determine device integrity. Often these tests come back as "normal." The usual management is device replacement when the aversive symptoms become intolerable or performance becomes unacceptable as determined by the CI team and family."
The Food and Drug Administration requires implant manufacturers to report adverse events related to medical devices, including implants. These include device failures, a death caused from a device or serious injury. Incidences are listed in the MAUDE database (link listed below). Failure rates are often reported to be in the one to two percent range among manufacturers. In their book, "Cochlear Implants in Children: Ethics and Choices," J.B. Christiansen and Irene Leigh report that out of 438 responses, the authors noted via parent comments that approximately 6% of the implants were replaced internally at one time. They caution that this is a conservative estimate since some of the answers were ambiguous, implying the possibility of a higher percentage.
Parent Preparation is Key
For parents considering implants, good preparation is the key to handling the twists and turns that can sometimes occur. Barbara Mellert, a mom to two sons with implants, found the online forums (see below) helpful to her in preparation for her son's surgeries. She says, "When Tom, then Sam, went through the workup, it was very thorough. We saw several folks--a social worker, of course our ENT and audiologist. When we started the process for Tom, we truly didn't know what we'd decide but figured since he had lost hearing; it would be helpful to see what was involved, during and after. That process was excellent and really helped us make our decision. So talking to multiple providers with different perspectives helped us a bunch."
Barbara also found it helpful to talk with other families as well as finding teens with implants to talk with her sons. Her sons now return the favor and have mentored other teens who are considering an implant.
Barbara continued, "We did not discuss potential re-implants when the boys were going through their work-ups. Granted, we went into the process with a good bit of knowledge--very different I think if your child is a baby as opposed to a teen-- so perhaps it was assumed we knew that the CI is a device that could fail. I will say I'm much, much happier now that both boys have two implants. They both are very dependent on their implants. And they love being bilateral! I know it's not for everyone, but for them, it's been the best thing we've ever done."
Leslie Kaufman offers this advice for parents considering an implant:
"Just to do your homework and learn all you can about the possible results from having an implant-and be prepared to accept and work with whatever results your child has, optimal or not."
As with all technologies, modalities and methodologies for communication, the key to a positive experience and healthy outlook is to never see the family and/or child as failing the method, but in recognizing that a method can indeed fail a child and family. ~
References and resources for more information: