From Verona - Your Frequently Asked Questions, Answered Here!

Hello from Verona!

Anna had a good day.  She is recovering nicely.  We have to visit the hospital daily for antibiotic and steroid therapy, but once she has had that, we are free to leave on our temporary passes.  We are technically still inpatients, even though we are spending our afternoons and evenings in our apartment.

I am unable to upload photos tonight (very spotty internet here), and besides that, nothing very exciting happened today, so I thought it might be a good day to answer questions.  Here are the Frequently Asked Questions from around the world.  I hope your questions or worries are answered here, but if not, it's open season on questions for the Italy contingent of the B Family!


Did you know Anna had this condition (Michel's Aplasia) when you adopted her?

No.   We knew Anna was deaf, and we happily chose to pursue her adoption.  We did not know that the cause of Anna's deafness was such a rare congential anomaly.  This was not any kind of deceit on China's part - - this is a rare condition only diagnosed by expert physicians in conjunction with CT and MRI imaging.  Personally, I confess that I am glad we didn't know.  I hope our family would have had the faith to keep walking forward, but in hindsight, I see the Lord's mercy in revealing little bits of the whole picture, piece by piece. It's like putting a big puzzle together - right now, we have the outline and some of the middle, but until Gabriel's trumpet sounds, we won't see the whole picture as He created it.


If you knew Anna was deaf, why are you pursuing hearing for her?

Before we met Anna, we had no preconceived ideas about whether we would pursue intervention.  We knew about hearing aids and cochlear implants, but truly, we had no ideas of whether we even wanted to pursue those routes.  Once we met Anna, and we saw how vocal she was, and how hard she tried to use her voice to communicate, we really believed she must have some hearing.  We were shocked to learn that not only did Anna have no hearing whatsoever, she actually lacked her hearing nerves.  In our pursuit of hearing aids (assuming she had some usable hearing), one thing led to another.  We also became acquainted with the school system, and one concern was that all of Anna's peers had some kind of aiding.  Anna would be the only child with no auditory input.  It seemed unfair to withhold that from her, if it was within our power to provide it.  After all, if your child is born with a visual impairment (and I have a child who was), do you refuse to seek medical care or refuse to allow your child to wear glasses because "this is how they were created?"  No.  In the end, once we learned about ABI, and after some initial floundering on whether this was something we wanted to pursue, all three adults in our family were unified in our feeling that we owed it to Anna to at least try.  We don't answer to the whole world or to the deaf community or to the adoption community.  We answer first to God, and then to Anna.  Looking at things in that prism, we determined that we would rather err on the side of more intervention than less intervention.  When Anna is older, if she doesn't want the ABI, she can stop using it.  But if we did nothing, and she decided she wanted it when she is older, it would not be an option for her because there is a limited amount of time for the brain to develop usable auditory pathways.


Why can't Anna have a cochlear implant?

Anna was born without her cochlear nerves and without her inner ear structures (cochleas, semi-circular canals, etc.).  A cochlear implant relies on the cochlea and the cochlear nerves to transmit sound to the brainstem. 


What is an Auditory Brainstem Implant (ABI)?

It is a device very similar to a cochlear implant.  Instead of being implanted in the ear structure, it is implanted directly on the auditory portion of the brainstem.  Sound is transmitted to an external microphone and processor and then transmitted to the electrodes, which penetrate the auditory brainstem.


How will an ABI affect Anna's quality of life?

We hope it will improve her quality of life by giving her access to sound and speech. We will need to be very careful around water, as her external processor cannot get wet. If Anna takes a bath or goes swimming, she will need to remove her device. Every night, the external portion will need to go into a drying container to remove the moisture from the day (I guess it's good we live in a desert climate!). ABI is okay for use in some of the lower-level MRI scanners, but if Anna needs a higher-level MRI, they will have to surgically remove the magnet (a very easy procedure), do the MRI, and then replace the magnet. Anna will not be able to use plastic slides (something to do with the plastic and the static electricity potentially 'erasing' the map) or go through the medical detector at airports.


Why can't Anna have surgery in the United States?

Until a few months ago, the FDA had approved ABI only for adults and only for treatment of a condition in which hearing people lose their  xhearing as the result of surgery to remove tumors that have grown on the cochlear nerve.  Recently, four centers in the USA have applied for approval to begin clinical trials in children.  Anna does not qualify for the trials because they are limiting the inital portions of their trials to younger children only.  Some of the surgeons from these centers were on hand in Italy to learn about the procedure as Anna had her surgery.  As these centers expand their programs, they may accept older children like Anna.


Why Italy?

The pioneer in ABI in children for conditions other than acoustic tumors is Professor Vittorio Colletti.  He has successfully performed an unparalleled number of ABIs in children of all ages, including children Anna's age.  He has assembled a team of dedicated professionals who manage the hospital stays and follow the children as they acquire language. 


Why is the cost so high?

Actually, the surgery would cost much MORE in the United States. The difference is that we would share that cost with the insurance company. Since the surgery is not FDA approved and performed abroad, it is not covered by insurance. Thus, we bear the cost alone, which is why it seems exorbitant. Actually, a two week hospital stay, with brain surgery, an ICU stay, multiple CT scans, mutiple blood tests and urinalyses, and round-the-clock physician care is a bargain at 40,000 Euros. This cost does not include our travel, lodging, and all the follow-up care (including travel and sessions costs for mapping in Los Angeles).


What is mapping?

Mapping is the term the medical field uses to refer to "fine-tuning" a cochlear or brainstem implant.  It is a somewhat tedious process, where they turn and test each electrode and then find a setting that works for the individual patient.  Some electrodes may have to be deactivated because they cause undesirable issues, like a bothersome tic or white noise.  Some can be activated but must be slowly turned up as the user gets used to sound.  For the first year, Anna will have mapping done every 2-3 months.  Most children who receive ABI in Italy are also mapped in Italy.  However, Anna and her American friends will be mapped by House Ear Institute in Los Angeles.  House plans to send their audiologist responsible for the ABI kids to Italy for the activation in March.


What will Anna look like when she gets home?

Anna will look like Anna - only with a funny haircut and a big scar.  It will take time for her scar to heal.  She will also be wearing an external device with wires, and a processor over her ear.  If you have ever seen anyone with a cochlear implant, it looks very similar to that.  The only external difference is that the magnet is worn lower on the back of the head, closer to the brainstem, where the implant is located.


Will Anna be able to hear when she gets home?

This is my MOST frequently asked question!  Yes and no.  Once the device is activated and working properly, Anna's brain will have access to sound.  However, her brain has to be trained at how to listen, hear, and interpret sound and meaning.  The doctors tell us to expect about one year for the auditory pathway to develop.  This could take longer in Anna because she is older, and therefore, her brain is less adaptable to change (so they say --I find Anna to be VERY adaptable!).  AT the very least, the surgery should give Anna access to "environmental sounds" - a dog barking, a train whistle, a drumbeat.  At the most, Anna will develop open-set speech discrimination -- the ability to hear what someone is saying to her without reading along by way of writing or sign or lip-reading. 


Will Anna be able to hear her own voice?

"But of course!" was the reply from the doctor when I asked this question.  Again, it will take time for Anna to learn what sound is and what sounds mean. 


What should we expect when we interact with Anna at home?

Imagine never hearing anything at all to suddenly hearing sounds when you chew, when you cough, when you speak - everything!  It will all be new to Anna.  It's exciting, but also overwhelming to the sensory system.  Be patient with Anna.  She won't understand everything everyone says right away.  Some parents even report that children want to take off their processors for a mental rest.  Keep talking to Anna with your face directed at hers and with whatever sign or gestures you can, as it will help her understand your speech.  Anna WILL hear some things...it will take time and patience and lots of hard work to develop that into something meaningful.  We have a wonderful team of teachers, speech therapists, auditory-verbal therapists and audiologists who are committed to helping Anna thrive.

Now it's your turn...if you have a question that I have not answered, please feel free to email me or to leave it in the comment section.  I will do my best to answer it.

Good night!

Amy