Official Conference Photos

October 3, 2018

We've decided to pursue the Porous Implant option with Dr. Lewin.  We've had a few phone appointments with her and her staff since our trip to Chicago to get the ball rolling.  This whole process is complicated.  Our first goal is to prove to our insurance that her reconstruction was unsuccessful and that revision is necessary.  We need them to grant us a Gap Exception in order to afford this as a surgical option.  We've submitted the following letter to an attorney (who specializes in this) for review and then we will submit it to the insurance company and cross our fingers.  It is almost guaranteed that the insurance will deny us (no insurance company willingly covers things like this), at which time we will appeal it as many times as needed.  It could be a long process but we are up for the challenge.

What is Porous Implant Ear Reconstruction "PIER"?  A porous polyethylene material that is hand-carved and implanted to give the ear a more realistic appearance.

September 25, 2018

To Whom It May Concern:

                                                                          

Hello! We are writing this letter in hopes of getting approval for a Gap exception and we wanted to share our story with you as you consider our request.

In June of 2009 our daughter Kambri was born without an ear unexpectedly.  When the doctor held her up so we could see her sweet face, we first noticed the large skin tag on her cheek and then said, "her ear is folded over" just as we realized that there was actually no ear but just a little formation of skin which we later lovingly nicknamed her nubin.  The next few days held a whirlwind of events.  Doctors, specialists & test after test consumed us.  During this time we learned the official title of her diagnosis: Microtia (deformity of the outer ear) Atresia (absence of the ear canal).  The next several years proved to be a learning curve as no one seemed to be very familiar with her diagnosis.

She was a very fussy & stiff baby.  When she was 2 1/2 months old, we thought that the eardrum in her good ear had ruptured.  The doctor insisted that because she only had one ear that we do surgery because this ear needed to function to the best of its ability.  So on August 19, 2009 she had her first surgery.  When the doctor got in there he was in awe at what he found.  The ear drum hadn't ruptured but there was "drainage that had thickened" and he removed almost a foot of nasty gunk from her ear.  The pressure from the buildup had made her miserable and she was a different baby immediately after surgery.  The constant tears nearly ceased & she started to smile.  She relaxed and was no longer stiff.  We were able to rule out almost all of our other concerns shortly after. 

Just a short time later she went under anesthesia for the second time, this time for an ABR (Auditory Brainstem Response) test.  The test proved that she does have an auditory nerve which meant that she could potentially gain some hearing and we were thrilled.  These results led us to Primary Children's Hospital where we met with Dr. Muntz for the very first time.  We learned a little bit more about Microtia-Atresia & what our options were.  He introduced us to the possibility of surgeries but told us we just needed to let her grow up first as they can't do anything before the age of 5.  He sent us to an Ophthalmologist, a Dentist and an Occupational Therapist.  The Ophthalmologist confirmed that her eyes were developed & functioning normally.  The dentist confirmed that she did have teeth, although they didn't come in until she was 14 months old.  With the whole right side of her face being affected, her little tongue hung out of her mouth all the time so the Occupational Therapist worked with her to gain muscle control so she could learn to eat & eventually speak.

We did as the doctor ordered and let her grow up...but not without a heck of a lot of intervention.  Over the years we've learned the in's and out's of unilateral hearing loss and a whole lot of life lessons.  She started speech therapy at 3 months old and finished at the age of 6.  She didn't make her first noise (other than crying) until she was almost 7 months old.  We had been working with her for months to help her realize that she had a voice and how to use it.  Once she found her voice she let it ring loud and clear.  

On January 21, 2010 (7 months) she was fitted with her first trial Baha - a bone conduction hearing aid.  Every doctor seemed to have a different opinion on whether or not it was necessary & if it would help her.  With a price tag of $5000 and insurance refusing to cover any of it, we weren't sure what we should do so we started with a trial to see what we thought.  We noticed almost immediately that she was able to locate sound which she had never been able to do before. Still with many unknowns Jared and I finally decided that we never wanted to look back and say "we wish we would have" so we decided to purchase one of our own. 

Kambri started school at Sound Beginnings when she was just 18 months old.  We were blessed with incredible teachers & staff and an opportunity to rub shoulders with many amazing families who were also dealing with hearing loss.  Kambri made leaps and bounds of progress during her time at Sound Beginnings. In May of 2013 she graduated from Sound Beginnings!  She was ready to be mainstreamed into a regular classroom and we were so excited! 

Her first 6 years brought hundreds of appointments.  She's been poked, prodded and examined a million times. I don't know how many hearing tests she's had but it feels like thousands.  She's become well acquainted with the sound booth.

In August of 2013 she had a CT Scan.  With it came with devastating news.  In my journal after we were given the results I wrote, "I watched intently as they did the scan last week and had my own ideas of what was in store.  I told Jared that I thought she had all the parts but I also thought it was possibly deformed.  I was anxious to see the official results.  For whatever reason I was shocked when he confirmed my ideas.  I guess I secretly hoped that it would be different.  He confirmed that the structure of the inner ear is fairly normal, however, it is surrounded by bone which means that we will never be able to restore hearing.  My heart literally sank.  Tough words to hear.  I held back the tears and squeezed Jared's hand just a little tighter.  He showed us the scan and pointed out specific things.  He showed us a diagram of a normal ear and compared it to her abnormalities.  He sat with us and answered our questions.  We explained it to Kambri who cried and said, "I just want a hole like my other ear".  Talk about heart wrenching.  We will be scheduling an appointment with a physician at Primary Children's Hospital to discuss what this means for our future.” 

It was then that we decided that if it wouldn't function, there was no reason to pursue reconstruction and for a time we thought the door was closed...

After going through a range of emotions once again we finally decided to implant the Baha (hearing aid) into her skull permanently in order to get rid of the headband.  The surgery was scheduled and we went to the ENT for our pre-op appointment.  When we walked into the office that day the doctor acted as if he had never seen us before.  He started explaining what a Baha is and what our options were, as if he didn't have a clue who we were.  I was stumped.  We had been seeing him for years and suddenly he knew nothing about us.  He stumbled all over himself when I pointed out that she had been a patient since birth and that surgery was scheduled in just a few days.  I was mad.  How could he forget us or at the very least not read his notes?!  I couldn't trust him to do surgery on my baby girl when he didn't seem to care.  We cancelled the surgery and walked out of that appointment.  We decided to go to a different doctor in hopes of starting the process all over again.  This doctor answered many of our questions and then he said, "I will do whatever you want me to do, but I will not do anything until you know all of your options and can make an educated decision" and with that he sent us back to Dr. Muntz at Primary Children's Hospital.  

So in June of 2015 we headed back to Primary Children's Hospital for a couple of appointments.  Our first appointment was with Dr. Harlan Muntz, a pediatric Otolayngologist, the same doctor we had met with when she was a baby.   He told us that it was a good thing that we hadn't implanted the Baha because that would make reconstruction extremely difficult because of the scar tissue.  He reassured us that there are absolutely things that can be done to restore hearing.  He explained all of our options.  Next stop was a hearing test at University of Utah Hospital followed by an appointment with Dr. Clough Shelton, also an Otolaryngologist.  He is the one who would make the ear canal & functioning ear drum.  He suggested that she would not need the Baha any longer as he can restore hearing in the 30's which is just below normal.  And with that, we were sent home to make a decision.  Over the next few months, together Jared, Kambri and I decided that if the ear would function, reconstruction would be worth it so we went ahead and scheduled the surgeries.

In December we had another appointment with Dr. Muntz.  He spent about 45 minutes with us answering all of our questions, easing our concerns, and even drawing pictures in the simplest of terms to help us understand every detail.  He showed us pictures of past patients and helped us to feel confident in our decision.  He explained the process of each of the 5 necessary surgeries and what we should expect. 

Her first surgery to reconstruct the ear was on January 5, 2016.  She had 6 surgeries that year with the last one on December 28, 2016.  It was a year of sleepless nights, countless doctor visits, many miles back and forth to Primary's, many tears, pain meds & barf bags.  She was a trooper but it did a number on all of us.

We've had several doctor appointments since then.  The healing process has been long so we've just been taking things a day at a time and trying to live life as normal as possible.  A few months ago we had an appointment at the University of Utah hospital and walked away with some tough news.  None of the information was shocking or unexpected yet it was still hard to hear.  The purpose of this whole process was to restore hearing and that was unsuccessful.  It's frustrating and heart breaking.  Several surgeries, appointments and follow-ups not to mention the thousands of dollars we've spent was all for not.   The inner ear refuses to heal.  Dr. Shelton compared it to trying to do a skin graft in the mouth - it's too moist to heal.  We've tried everything from creams & antibiotics to expanding ear plugs to cauterization & everything in between yet the ear canal continues to get smaller and smaller at every appointment.  After 15 months (post surgery) we concluded that it's probably not going to heal in the way we had hoped.   We don't love the way the ear looks & the placement of it but even more so we are disappointed that she still can't hear.

So once again we started to explore our options.  We met with new doctors, explored different procedures & even took a trip to Chicago to attend a conference for Microtia-Atresia patients, which is where we met Dr. Lewin.  We were able to meet with her individually, meet patients who had worked with her and were able to see (& feel) her work on actual patients.  We met with Dr. Bonilla (from Texas) who specializes in the rib graft surgeries (which is what she had done) and he told us that she is not a good candidate for the operation again.  He would not consider doing surgery and recommended us to Dr. Lewin.

We feel like Kambri is a good candidate for surgery with Dr. Lewin because we have explored all of our options.  We have researched countless options and we feel like this is the best fit for our situation.  Please consider granting us the Gap Exception so that we can move forward and offer Kambri all that she deserves. 

THANK YOU for your consideration!

Jared, Stephanie and Kambri Strawn