Thursday, October 11, 2012

To Implant Or Not To Implant, That Was The Question

Our decision to give Eve a cochlear implant was not an easy decision or one that we took lightly  I can only hope that some day, when she grows up, she thinks we made the right choices for her.  This blog post is about how we came to reach that decision for her.

The MRI Results

Eve was two months old when she had her first MRI.  I will never forget the phone call I received from her ENT, Dr. Carvalho the next day.  At that point I was pretty sure Eve could not hear anything at all so I was starting to seriously consider the idea of a cochlear implant.  From the beginning of that phone call, I could tell from her voice that Dr. Carvalho did not have good news.  What she told me is that, based on the MRI, it looked like Eve had no cochlear nerve on the right, and maybe only a very small nerve on the left.  She explained that this is significant because if a person does not have a functioning nerve, they are not a candidate for a cochlear implant.

The way cochlear implants work is that they hook up an external sound processor to a surgically implanted electrode that stimulates the cochlea.  The cochlea is a small spiral or snail-shaped part of the inner ear that sends sound vibrations into the auditory nerve, which are then processed by the brain to be interpreted.  If there is no auditory nerve, there is no way for the electrical sound impulses from the implant to connect to the brain.  If you want to understand more, these Wikipedia articles should help:

http://en.wikipedia.org/wiki/Cochlear_implant
http://en.wikipedia.org/wiki/Cochlea
http://en.wikipedia.org/wiki/Cochlear_nerve

Dr. Carvalho said that we could do another MRI when Eve was around one year old but if it turned out there were nerves, there was no way she would give Eve an implant.  Hearing this news was extremely upsetting to me.  At that time I still had not come completely to terms with Eve's deafness, at that point I had never met a deaf person, and the Early Intervention teacher that had come into our home had given me much hope about what cochlear implants can do.

As I mentioned in my earlier post, we tried hearing aids from when Eve was about four months old until she was about a year old.  When we could keep them in her ears, they did not make any difference.  We certainly never got any response on the right, but sometimes, at an extremely high decibel level, with the left hearing aid, we thought we might be seeing some kind of response.  This gave me some hope that she might have a working auditory nerve on the left side.

A few weeks after we received her initial MRI results, I was doing some research and came across the name of another ENT by the name of Dr. Germiller who works at the Childrens Hospital of Philadelpia (CHOP) with patients who have small auditory nerves.  I sent him an email out of the blue and asked if he had any suggestions.  I was completely surprised when he responded the next day and offered to take a look at Eve's MRI and provide a second opinion.  After he looked at the MRI, he agreed with Dr. Carvalho that Eve has no cochlear nerve on the right and maybe a small one on the left.  He then invited us to come out to Philadelphia to perform a test called promontory stimulation which he often performs on his patients with small nerves.

In his email to me, Dr. Germiller explained this test as follows:
Promontory stimulation is like a "pretend" cochlear implant.  It uses electrical stimulation of the cochlea via a temporary electrode, using the same stimulation pattern a cochlear implant does.  Then the responses are measured by a typical ABR  equipment.  we do it in children with questionable cochlear nerves on MRI, if they are being considered for cochlear implantation.  It tells us if there is an electrically stimulatable nerve, and how good the nerve sustains electrical stimulation over time.  We have found it extremely helpful in these cases.
Although Eve did have a second MRI in December 2011, it only confirmed the original MRI results.  Getting a cochlear implant was out of the question for the right side, but was she a candidate for an implant on the left side?  Eve's doctors believed that the only way to know was to go through with the promontory stimulation.

The Philadelphia Ordeal

Our insurance plan is an HMO,which only covers doctors that are "in-network" unless there is a prior authorization to go out-of network.  Dr. Carvalho worked with Dr. Germiller to request the authorization for the promontory stimulation testing in Philadelphia.  Our medical group almost denied the request because there is not a lot of published data on the usefulness of the test.  However, Dr. Carvalho spent nearly an hour on the phone with the director of the medical group and convinced them to give us the authorization.  The procedure was scheduled for September 27, 2011 and I bought our plane tickets.

About one week before we were scheduled to leave I received a call from someone at CHOP who told me that they were being told by my insurance company that the hospital fees for the procedure were not authorized.  I was confused because I thought that had all been taken care of.  So I called our AMAZING case manager over at our medical group and she got to the bottom of the problem.  Apparently, the medical group is contracted with our insurance company and there are some things the medical group is permitted to authorize but other things the insurance company itself has to authorize.  The director of the medical group thought she had the authority to authorize the procedure, including both the doctor and the hospital fees, but the insurance company took the position that the medical group could not authorize the hospital fees.  Anyway, after a few days I received a call from the case manager to let me know that the director of the medical group had gone to bat for us with the insurance company and convinced them to authorize the procedure.  Whew!  I thought we were all set.

Then, the Friday before the Sunday we were scheduled to leave for the trip, I received a call from Dr. Germiller.  He informed me that the procedure had been cancelled because CHOP felt that the device being used for the procedure had to first be authorized by the hospital.  Dr. Germiller explained that he has used the device at CHOP for many years with no problems whatsoever but apparently some administrator at CHOP recently got wind that the device had not been FDA approved and was forcing Dr. Germiller to jump through a few procedural hoops before permitting him to use it again.  Dr. Germiller was extremely apologetic but said his hands were tied.

Finally, in November I received an email informing me that the procedure had been rescheduled for January 12, 2012.  Fortunately, getting our insurance to extend the authorization was not a problem.  Everything was set!  John and I decided that, since we were funding the travel expenses ourselves, just Eve and I would go.  I knew I could handle it but it was an exhausting few days.  Eve and I flew out to Philadelphia on the evening of January 10.  We tried to get a room at the Ronald Macdonald house but it was full so we wound up staying at a Sheraton a few miles from the hospital.  On the 11th we had a couple of pre-op appointments.  Then, on the morning of the 12th we got up bright and early at 5:30 am in order to check Eve into the hospital at 6:30 am.  I anxiously waited while she was underwent procedure.

After about two hours, Dr. Germiller and the Michigan audiologist he works with to do the procedure, Dr. Kileney, spoke with me.  They told me, as expected, there was no response on the right.  However, on the left side they said there was a response, although the nerve seemed to fatigue after repeated stimulation.  Dr. Germiller was not 100% confident but said he has seen kids with nerves that responded similarly to this test develop some understanding of speech and speech ability with an implant.  He said that it takes a lot more mappings and a lot more time for the cochlear implant to start working, but he said he recommended it in her case.  He went so far as to tell John that if it were his child he would do it.  In February, we received confirmation from the CI team here at Rady Children's Hospital, San Diego, that, based upon their evaluation of us and of Eve, she was approved for the procedure.  We set the date for March 9.

The Decision

Although we had gone through a lengthy and arduous process to determine if Eve was even a candidate for a CI, John and I both struggled immensely with the decision.

By January 2012 both of us had had time to immerse ourselves in ASL and Deaf culture and meet many wonderful Deaf individuals.  We had spent a lot of time grieving over Eve's hearing loss but we were also coming to appreciate the wonderful new world that her hearing loss was able to expose us to.  We came to learn that so many deaf people did not view themselves as disabled in any way and most of the deaf people we met would not change being deaf even if they had a magic wand that could make them hearing.

I spoke with a family therapist over at Deaf Community Services and she told me some horror stories about children that had grown up with CI's and an "oral/aural" approach to language, who never learned ASL, and who, at about age 10 or 11 started acting out, throwing their CI's down the toilet and, upon working with them she discovered that those children were considered "CI failures" and, because they had never been exposed to ASL they had essentially no ability to communicate with anyone.  I learned that some children with CI's do remarkably well, but others do not.  And even the ones who are considered a "successes" often struggle in their lives with a sense of self-identity.  Are they hearing?  Are they deaf?  Both? Neither?  They cannot hear as well as a hearing person and even though they can hide it extremely well, there is still a lot they miss.  Especially in a setting that has a lot of ambient noise.  

Eve's early intervention teacher told me that the children she works with who have CI's "consider themselves hearing."  When I mentioned this comment to one of my deaf friends, she scoffed.  How could a child self-identify as Deaf if he or she has never been exposed to anything other than the "hearing" world.  So where does a child with a CI fit into the world?  The fact that some deaf people shun CI's and feel strongly against them gave John and I much reason to pause for consideration.

John and I read as much as we could about the "CI debate."  We wanted to know all perspectives and be as informed as we could before making a decision to surgically alter our one year old child.  I have always believed you should not alter a child until the child is old enough to make an informed decision.  From circumcisions to ear-piercings, why not wait and let the child decide himself or herself?  It is his or her body after all?  But study after study shows that the earlier a prelingually deaf child receives a CI, the better the outcome.  In particular, studies show that the prognosis for speech recognition is much better if the child received the CI under age 2.  So, either way, a decision had to be made before Eve was capable of making the decision herself.

We learned that the decision to surgically implant the CI is only the tip of the iceberg.  Because people with CI's do not hear sound the same way that children with normal hearing do, they require regular "auditory-verbal" therapy, which must be reinforced at home on a daily basis.  John and I both feared that with Eve's other challenges -  eating, motor delays, etc, was yet another therapy session going to be too much for her to handle?  We feared that Eve's teachers, and even our families would see the CI and treat her just as you would treat any hearing child.

We read horror stories about deaf children attending "oral" schools for the deaf where the students spent hour after grueling hour learning how to form their lips in order to speak.  As though being able to speak is more important than learning how to read and write, do math, and understand the ways of the world.  No matter what, we both felt strongly that that we did not want Eve's life and self-identity to be centered around listening and speaking.  There is so much more we want for her.  We were just not sure if it was worth it.

At the same time, what you have to remember from one of my earlier posts is that in November-January 2011-2012, when we were struggling with this decision, Eve had some significant damage to both of her eyes.  John and I were (and still are) both extremely fearful of what the long-term outcome is for her vision.  We debated back and forth ad nauseum   Despite all of our concerns about giving Eve a CI, I felt that even having some access to hearing could be beneficial to her, especially if she winds up having significant vision loss in the future.  Even if, because of her small nerve, she is never able to understand speech or talk, being able to hear a car honk could be helpful.  Despite it all, I still believe living life as a deaf person must be incredibly difficult and challenging.  The honest truth is that there are so many barriers that deaf people have to overcome.  Although there are many many deaf people have overcome those barriers, many do not.  I felt that if Eve is able to use the CI to listen and talk, it just might make life a bit easier for her.  She already has so many challenges, shouldn't we take one of those challenges away if we can?

So how could we overcome all of our concerns about the CI and still move forward with it?  Well, we decided that the only way to do it was to give her the CI, go to the auditory therapy, and yet still learn American Sign Language and use it with her as her primary language until and only until she shows a clear preference for and understanding of speech.  This is a very different approach than is taken by most hearing families.

In fact, some CI centers insist that families stop using sign language at all once their child receives a CI.  I am not sure where this concept originally came from but some doctors, audiologists, and speech therapists seem to think that you have to deprive a child of sign language in order to "force" them to communicate with listening and speaking instead.  As though giving a child ASL somehow hinders a child's oral/aural language development.  Even before CI's, this approach was used at "oral" schools for the deaf.  And the deaf children at those schools wound up using their own made up signed languages with each other, behind the backs of their teachers, in order to communicate.

John and I feel that the better approach is to give Eve every possible communication option and let the choice be hers.  Communication needs to be fast and easy and versatile.  We suspect that even in the best case scenario with the CI, Eve will feel more comfortable with signing.  This choice seemed like common sense to me; how can language possibly hinder language?  We intuitively believed that with a CI, speech/hearing and ASL are not mutually exclusive.  Plus, we feel strongly that the only way to avoid the worst-case outcome- that being Eve is deemed a "CI Failure" and unable to communicate, is to make sure she learns ASL first.

In fact, the majority of language development research that I have read indicates that knowing one language proficiently greatly assists in the acquisition of a second language and greatly improves a child's language comprehension in both languages.  I even came across an article recently that studied speech perception outcomes in CI children of deaf parents who use sign language and hearing parents who did not.  In that study, the CI children of deaf parents actually developed better speech perception than the CI children from hearing parents.   

The bottom line is that I don't necessarily think CI's in prelingually deaf children is a bad thing, but it can be if the child winds up being deprived of language and self-identity as a deaf person because the child's parents failed to accept that the child is deaf or embrace that the child's most natural form of communication is a signed language.  The worst case scenario is not that the child is unable to understand speech or talk.  The worst case scenario is that the child is unable to communicate.  The only way to ensure that a deaf child can communicate is to immerse the child in a signed language.

The Surgery and Outcome

Eve's CI surgery took place on March 9.  At one point after we checked her in I had the strongest urge to take her back home and just forget about the whole thing.  One of the potential complications of a CI surgery is that it could damage the facial nerve, which gives the person facial paralysis.  I remember playing with Eve and cherishing her beautiful smile and thinking that I would never ever forgive myself if I never saw that smile again.  On the other hand, Eve's doctor reassured us that the risk of that happening is very small and there was nothing unusual about Eve's anatomy that made the procedure any riskier for her.  Also, I felt confident that Eve's ENT knew what she was doing so I trusted in her experience and knowledge.

Eve on 3/5/12.  I just love her smile.
After a nerve-wracking few hours, Dr. Carvalho came out and said the surgery went perfectly.  Eve, however, had a difficult recovery.  At first the nurse asked if I wanted to give her just Tylenol or Tylenol with Codeine.  I regretfully elected just Tylenol and Eve was miserable for the next four hours, basically crying constantly.  We drove her home with her crying the most dreadful cries I had ever heard from her.  But I couldn't give her the Tylenol with Codeine for a few hours because I had already given her regular Tylenol and didn't want to overdose her.  So Eve was in pain and crying for a few hours before I finally was able to give her the Tylenol with Codeine and she calmed down.  It was a very difficult night and she slept fitfully and woke up crying frequently.  I kept asking myself over and over if it was worth it.

The next morning the entire left side of her face was very swollen and for the entire day she seemed very exhausted.  Fortunately, she started to perk back up to her normal self on the following day.
Eve 3/10/12, the day after her CI surgery.

Three weeks later, I was so excited for her CI activation.  I brought my videocamera and my sisters even came with us to the appointment.  The audiologist set everything up  and then said Eve was on but Eve did not show any responses whatsoever.  It has now been six months, 4 or 5 program mappings, and countless auditory-verbal therapy appointments later, and I can only sometimes get her to respond to high-frequency sounds like "SSSS" or "SHHHH".  Last week we brought Eve in for a sedated ABR test and the results were promising.  The audiologists said that Eve was showing responses at high medium and low frequencies and that the mapping we have been using for the last month should work for her but it may just take more time.  So we put the CI on her every day and take her to her auditory verbal therapies once a week and I hope that it is doing her some good or that it will be useful to her in the future.  But only time will tell.  What is wonderful is that she is signing more and more every day, so no matter what, I am certain we will be able to communicate with her.


4 comments:

  1. Good afternoon. First, I want to apologize for the mistakes in the translation to English. Now is when I most regret not further studied English language at school. We live in Spain. We are now in a situation very similar to yours. Our son has diagnosed an alteration on chromosome 6 and this is causing a motor delay, vision problems (myopia and glaucoma) and profound deafness. He is three years old. Doctors are seeing the option of cochlear implantation. The mother is sure of it, but I have doubts, the same questions that you have had:
    Is not it better to learn to talk by signs and not make the move to the other operation?
    What if something goes wrong?
    What if the cochlear implant does not work?
    I have a scared, very scared. I just want my child to be happy, deaf or not deaf. There are also the problems in their eyes ...
    Thanks for your experience. I've cried reading it. I hope that your daughter can be very happy. I hope everything goes well. I'll tell you. Thanks again. a hug

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  2. All of your questions are valid. The only way to ensure your child will have access to communication is to use sign language. If you decide to get a cochlear implant, there is absolutely no reason you cannot continue using sign language. If the CI does not work, you will still have sign language. Our number 1 focus is sign language. If the CI works, great! If it doesn't, then at least Eve will have a language that she can use to learn and communicate with others. If your child eventually loses his sight, then you can just convert the sign language he already knows to tactile sign language. Many deaf-blind individuals communicate by just putting their hands over the other person who is signing. Thank you for commenting and good luck!

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  3. I'm the editor of the Endeavor which is the magazine for the American Society for Deaf Children. Could you contact me at asdctami@aol.com?
    Currently, ASDC's website has been compromised, but you are able to see what our organization is all about...www.deafchildren.org

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