Sunday, November 20, 2011

November 2011 - "No More Surprises"

On Tuesday, November 15th, Brian took the day off from work to accompany Madison and I to her sound booth appointment at NYU.  That afternoon, Brian was walking back from lunch when his cellphone was Dr. Davis - our geneticist.

Dr. Davis asked Brian if he had time to speak.  Of course he did.  Dr. Davis began speaking and mentioned that the results are in and...Brian interrupted her and said, "Jennifer and I are hoping that it's Connexin."  Her reply, "It is."  A moment of silence occurred, Brian was speechless - not knowing exactly how to respond.  For 5+ months, the questions of "Why?" and "What Else?" were going through our heads.  Every time Madison leaned one way, or moved one arm more than the other - thoughts about her motor skills would go through our head.  Constantly dissecting all of Madison's actions, it was difficult to enjoy and appreciate everything without wondering (and fearing) what's next.  Finally he said, "So this means that the chances of having something else happen or anymore surprises is the same as any other (hearing) child?"  To which Dr. Davis responded, "Brian, there are no more surprises.  This is it."

As the conversation progressed, Dr. Davis and Brian discussed Madison's actual test results.  It turns out that Brian and I are carriers of a mutated gene called Connexin.  One of us has a mutated Connexin 26 gene, and the other a mutated Connexin 30 gene.  These are both recessive, so Brian and I (and any of our family members that happen to also be carriers) have never experienced hearing loss.  However, these mutations were passed to Madison from each of us, hence she was born with profound hearing loss.  Connexin 26 mutation is the largest genetic cause of hearing loss within infants.  The chances of two individuals having a 26 & 30 mutation is extremely rare.  Connexin mutations are more common in certain ethnic groups - but with Brian and I from different ethnic backgrounds, the chances of this happening was extremely remote.  They did not test for this in our genetic screening during the pregnancy.

With the questions of "Why" and "What Else" answered, we're finally able to focus on the "Now" and the next steps in our daughter's journey.  

November 8, 2011 - 5 months old

Mommy and I love to spend time together during naked baby massage sessions.  I get undressed and we do all sorts of stretches.  I think my mommy wishes she had massage time for herself too!
Alan is my older brother and I say hello to him every morning.  I love petting (grabbing) his fur.  He usually gives me a big lick across the face.
Daddy and I love reading books together.  My favorite book is "The Bellybutton Book".  I love my Bee bo.
I have a gas button on each of my feet that mommy can press when I'm backed up and ...
So in love with my feet! I found them a few weeks ago and can't stop playing with them.  I love putting them in my mouth.
Oatmeal.  I will be trying solid food for the first time next week.  Special baby oatmeal mixed with breast milk.  I am very curious to what it will taste like.  I'm sure I will make a huge mess during my first feeding session.
Night-time rituals consist of B-B-B-B.  Bath, Books, Bottle, Bed.   I'm finally sleeping through the night (most of the time).  I know my mommy and daddy are happy to finally catch up on sleep.

Sunday, November 6, 2011

November 1, 2011 - She's a Fighter!

I spoke with the nurse the night before, confirming Madison's CT appointment for Nov at 1pm at NYU Medical Center.  No food (in her case, milk) 4 hours prior to her appointment.  Luckly one of our friends forewarned us about her clothing... no metal is allowed in the CT-machine and most newborn onesie's have metal snaps.  Obviously we would have not even thought about this, but good thing we were able to plan ahead!

We arrived at the hospital around 12:30 and checked in at admitting.  Madison was given a cute beanie baby toy to play with as we filled out paperwork.  We arrived to the Radiology suite by 12:45.  It was a ghost town and nobody was in sight.  I began to pace the hallways (holding Madison, to keep her occupied)... a woman down the hall finally approached me, "This must be Madison."  I was so glad to find someone to help us, since our appointment was for 1pm.  I was getting anxious because Madison had not eaten since 8:45am... and she usually eats every 3.5 to 4 hours.  The woman told me the anesthesiologist was running late and that we probably wouldn't start until 1:15.  1:20pm rolled around... "It's looking like another 20 minutes." By now, Madison was passed out on my chest.  I was so glad that she was sleeping, despite being hungry.  Brian and I were amazed at how well behaved she was in the waiting room.  The nurse suggested that we try to place Madion in the bed and do the CT scan without sedation.  Why not give it at try, right? Miraciously she stayed asleep while they laid her into the table, draped lead apron over her and tucked her little arms to the side.  She was sucking on her pacifier and we were told "no sucking allowed".  By than Brian and I both realized this was going to be was a lost cause.... back to the waiting room we go.  An hour and 15 minutes after our scheduled appointment, the anesthesiologist finally shows up.  He tells us he was held up in another procedure and was sorry for the delay.  By this point, Madison was hysterically crying - she was tired, hungry and confused.  Everyone in the room was very rushed - trying to get the procedure underway.  Madison was in my arms... a gas mask was shoved over her face and held in place for about 60 seconds (which felt like an eternity for us).  In fact, the nurse commented "Oh my goodness, your daughter, she's a fighter!"  Madison was screaming the entire time, kicking her legs, waving her arms, and had such a confused look on her face, "What is going on mommy? Why are they doing this to me?".  By this point, Madison wasn't the only one crying... Brian and I left the room and the procedure began.  25 minutes later, the nurse came to get us.... "Madison was a champ and did just great!  She just woke up from the sedation. You can come get her now."  For the first time, it was somewhat comforting to hear our little girl cry.  We went into recovery, where we were able to calm her and feed her.  Within 20 minutes, Madison was back to her normal self.  We were discharged from the hospital by 3:30pm. 

Shortly after we got home, we sent an email to the ENT surgeon at NYU, Dr. Roland - letting him know the CT scan was completed and that we are anxious for the results.  Within the hour, Dr Roland responded, "The inner ear structures and cochlear appear to be normal."  What a sigh of relief.... This news meant that Madison is a candidate for Cochlear Implants!  Today was another GREAT day!

October 31, 2011 - MOOOO!!

My nanny took me trick-or-treating today.  No candy for me, maybe next year?


October 24, 2011 - "Yes, something seemed a little off"

We made Madison’s genetics appointment back in early July... The soonest available appointment was October 24.  With mommy in California, daddy and Gloria (our nanny) took Madison to Dr. Davis – our geneticist at Cornell Medical Center.  The appointment began with a lengthy conversation with an assistant to Dr. Davis, as she was teaching a class.  The assistant went through mommy and daddy’s family trees – asking numerous questions (all negative ones) about each family member, how old were they now, how old were they if they died, how they died, illnesses, children, if not children – how come, etc.  After Dr. Davis’ class ended, she met with Madison.  She gave Madison a routine physical and then went over the family tree.  The conversation with Dr. Davis felt a little weird, as her assistant didn’t tell her that Gloria wasn’t my wife.  In fact, Gloria told her a few minutes into the conversation, in which Dr. Davis replied, “Yes, something seemed a little off.”  With Dr. Davis getting a better understanding of the entire family history and situation (and now realizing Madison has European descent on her mother’s side, not South American) we went over the possibilities of what may have caused the hearing loss, as well as future concerns.

Hearing loss is either caused by problems during pregnancy/labor, structural impairments, or by certain genetic conditions.  Pregnancy was more than fine, mommy was very strict and stayed away from caffeine, splenda products and even deli meats!  Labor and delivery could not have gone any smoother.  Not a single day goes by with the thoughts of "what did we do to cause this??" Dr. Davis reassured us that Madison's hearing loss is not a result of something we did.  With Madison’s CT Scan still in the future – we haven’t been able to rule out structure.  In fact, Dr. Davis kept mentioning how adorable Madison is, and how coordinated she is – which may lead us to rule out structural issues.  This is because if there were structural issues, it’s not usually isolated to hearing loss.  For example, many children with hearing loss may require other types of therapy (i.e. occupational or physical).   If the hearing loss is not due to structural impairments, science is not always able to determine the genetic cause.  Because there are so many possible mutations, they are not able to test for every possibility.  The most frequent genetic cause is due to a mutation in the connexin gene.  In fact, the 3 most common ethnic groups are Ashkenazi Jews, Caucasians and Asians.  Apparently 1 in 33 people carry this recessive gene.

Based on Madison’s physical evaluation and her ethnic background (and not being half-Paraguayan) Dr. Davis encouraged us to have her tested for Connexin.  The only effect of the Connexin mutation is to prevent hair cells from growing within the ear/cochleae, hence, not allowing one to hear.  There are no other impacts to her vision or motor skills.  So, if she were to test positive, mommy and daddy can breathe a little easier.  If she tests negative, then there are several other syndromes out there that cause deafness in infants – each worse than the next, and many of them are impossible to detect before other symptoms appear.  

We thought that if they were to test for Connexin, it would be with a cotton swab.  How wrong we were!  Two vials of blood later, and a screaming baby…the doctor told us it would take anywhere from 3-5 weeks for the results.  And so we wait…

Wednesday, October 19, 2011

October 18, 2011 - Put a Smile On My Face

I received this video on my phone from Madison's therapist at The Center.   Absolutely LOVED watching this while I was at work...  It sure put a smile on my face!

Sunday, October 16, 2011

October 12, 2011 - Did You See Her Reaction?

Madison had her initial appointment with the staff at the NYU Medical Implant Center.  This is already the fourth hospital in NYC that she has visited in her 4 months of existence.  This appointment (along with several others) had been made about 7 weeks ago... The plan is to meet with their audiology team three times over the next few months, then with the surgeon in December.  The staff was extremely nice and answered our questions and tried to address all of our fears.  The best part of the visit was getting Madison exposed to the sound booth for the first time.  Madison sat on mommy's lap in a small room where sounds were emitted and daddy was across the window pane with the audiologist.  The audiologist said to daddy, "Did you see her reaction?"  Daddy's response, ""  The audiologist noted that Madison eyes moved the slightest bit.  However, as the audiologist increased the decibel level - sure enough daddy saw Madison react to sound!  It was great to see her startle.  The audiologist noted that Madison was able to hear sound between 70 - 100 decibels at lower frequencies with her hearing aids.  This is nowhere near conversation levels, but it does infer that she (hopefully) has an auditory nerve and can still be a candidate for an implant.  Our next visit at NYU will be the middle of November.  This will consist of a follow up testing in the sound booth and a CT-scan (to determine if Madison's inner ear structure will allow for an implant).  This was one of the better days we have had in a quite some time.

October 8, 2011 - 4 months old

Madison is 4 months old today... she weighs 14.8lbs and is 25inches long.  If she continues on this path, she will weigh 20lbs by the end of the winter... and hopefully scheduled to receive her cochlear implant!

October 2011 - 5 Days a Week

Madison's schedule just got busier, if that is even possible! She is now in therapy 5 days a week.  In addition to receiving speech services at The Center on Tuesdays and Thursdays, Madison began therapy at Clarke School just a couple of weeks ago.  She receives 3 30-min private speech sessions (Mondays, Wednesdays & Fridays) in addition to one 1-hr group session. (Fridays).  Madison's speech therapist's name is Lauren.  The goals here are no different than what they are at The Center.  We teach Madison that she needs to vocalize in order to request something, e.g., If we blow bubbles, in order to do it again, Madison needs to vocalize something, i.e., cooing.  Listening is a 4 stage process (identify, discrimination, identification & comprehension).  Right now the focus is on IDENTIFICATION.... the move from nothing to something.  You bring your hands together to clap and a sound is produced.  Recognizing this is the first stage in the auditory process to the brain.  As she gets older, the other areas will be taught.  Each session begins with a "HELLO" song, and ends with a "GOODBYE" song.  It is important to establish a sense of routine to each session.  In between we sing songs, read books and do baby exercises, i.e., tummy time.  Every Friday, Madison attends a group therapy session.  This consists of 7 other babies (similar age, all with different degrees of hearing loss) and their parents/nannys.  This is not only a great time for the babies to interact with one another, but gives Brian and I an opportunity to speak with other parents who are in the same situation as us.  So far it has served as a great support network.  It has been helpful meeting other families that have the same concerns as we do.  Brian and I plan to attend once a month with Madison. 

Saturday, September 24, 2011

Sept 2011 - Fine, You Can Have Three

On Thursday, September 22 we had our meeting with NY State to determine how many sessions of state-funded therapy Madison would receive until the age of 3.  This was our ISFP meeting, Individual Family Service Plan meeting.  Present at the meeting was: Madison, mommy and daddy, Tamara (our service coordinator), two representatives from NY State, and Meredith (director from Clarke) on via conference call.

The ISFP meeting is the third step in the early intervention (EI) process.  The first step was finding a servicer coordinator - we'll be using Tamara (who happens to also work at the Clarke School).  The second step was for Madison to get evaluated by therapists.  We had this done by two therapists at Clarke - Sarah and Lauren.  The EI program is not solely related to hearing loss.  In fact, it covers many other areas.  However, Madison will be using the program for speech therapy and we've aligned ourselves with the Clarke School as their speech therapists focus on infants/children with hearing loss.

At the meeting, we focused on Madison's development as well as our concerns (and goals) for the short-term and future.  We had been forewarned by numerous people that this meeting was tedious and intrusive, but we did not feel that way at all.  Maybe we were lucky with the two state representatives that ran our meeting.  We were hoping to receive three 30 minute therapy sessions a week, along with a parent/infant group therapy transportation to/from the UES. 

Even though they claimed our appointment was one of the first of its kind being performed online using their "NICE" system, it felt as if there was a ton of paperwork.  When we finally go to the section of which services the state will approve Madison for, one of the state administrators said, "We'll give Madison two sessions, 1 group and transportation."  We were shocked!!!  Tamara had said to us that all of her recent families were granted with three sessions.  Immediately, Tamara (politely) objected and Meredith weighed in with her opinions too.  My husband and I said, "We know these next few years are critical and don't want to have any regrets."  Next thing we know, the state representative said, "Fine.  You're approved for three therapy, one group and transportation."  Meeting adjourned.  We'll have meetings with the state every six months until she turns 3 years old, then we switch from the Department of Health to the Department of Education. 

In this difficult time, we are so happy to be living in the greatest city.  To have all of these options available to us, and a short car ride away is invaluable.  Now, it's up to us to utilize these resources and have Madison reach her full potential.

Sept 2011 - Are the Hearing Aids Working?

This is the most common question of the moment... Often asked by others and more often a question bouncing around our own heads on a daily basis. 

Madison won't be going into a sound booth until she is a bit older, so the best way to guage whether or not the HAs are working is based strictly by observation.  Most mornings, when the weather allows for it, Brian, myself, Madison and Alan go on a morning walk. Before receiving her HAs, she would fall asleep shortly after we leave the apartment.  Lately, she has been awake and much more alert in her stroller - hopefully because she is hearing some sounds - sometimes she doesn't fall asleep until we return back to our apartment and go into the elevator. 

There is a lot of construction going on in our neighborhood - which has been helpful to try and test out the HAs.  A couple of weeks ago, I went on a mid-day walk with Madison to run some errands - first stop was the pediatrician's office - she was starting to doze off as we walked inside.  Soon enough a loud screaming baby was brought by us - Madison quickly jerked and woke up.  Did she hear the scream or was it just a coinscidence? Later on that walk, she was sound asleep - we approached a jackhammer (literally 5 feet from us) and she did not budge.  On that same walk, (she was now awake) and we approached another jackhammer - her eyes buldged and it seemed as though it was in responce to the noise.  Earlier today, Brian and myself were on another walk (Yes, we enjoy our walks...I don't know what we are going to do when it starts to snow!) and about to cross the street - when a traffic guard blew his loud whistle 6 times.  Of course, I look immediately at Madison to see if I notice anything different - her eyes buldged just as they did with the jackhammer.

Aside from today's experience, I have shared these findings with Jessica and she says these are all very good signs that Madison is getting some input! Not a single day passes when I'm not constantly thinking about the noise in my surroundings... always wondering what it is like for Madison - if she is hearing anything.  We have been told to keep thinking positive thoughts, that most deaf people receive some benefit from the HAs.  There is a small chance (1%) that she does not have an auditory nerve.  In this case, she would not receive any input from HAs (and with this extreme, she would not be a candidate for a cochlear implant).  Yes, it is so rare, but than again so is our situation.  It is so hard not to keep thinking WHAT IF... We just have to continue taking one day at a time and pray for the best.  We are hoping that the HAs are working, giving Madison exposure to some sounds.  Even though she is not hearing sounds of speech, her nerve and brain are working to make use of the sounds that are being received.  This is priming the nerve/brain for implantation of the cochlear implant.  Madison should be going into the sound booth middle/end of October and hopefully the audiologists will be able to confirm that Madison is responding to sounds with her HAs.

Sept 2011 - Take This With a Grain of Salt

On Wednesday, September 22nd Madison met with an opthamologist - Dr. Levy.  Dr. Levy was recommended to us by Dr. Modi, during her first appointment with the audiologist at Cornell.  The reason for the meeting, is they want to make sure that her other senses (i.e. vision) are okay.

Madison sat in the patient chair (well, on mommy's lap) and Dr. Levy looked at her eyes with all of his tools.  Then, he performed a vision test.  Obviously, no baby can read a chart and repeat letters back to you.  He used pieces of cardboard-like material that were shaped like a rectangle.  One side of the rectangle would have a bar-code/stripe design on it.  He would track whether Madison correctly tracked which side the design was on after he flipped the sheet over and had her quickly glance at it.  The test started out with thick stripes, and slowly increased in difficulty - with the stripes getting smaller and thinner.  Well - she did great!  She has the same vision (if not better than) all of her hearing peers.

Then, we asked Dr. Levy about Usher Syndrome.  For those of you that don't know - a small percentage of deaf infants have a chromosome mutation passed onto them genetically.  In the case of Usher Syndrome, there are 3 main strands - where strand 1 causes blindness by the age of 5, as well as loss of balance.  The other 2 strands also cause blindness and balance issues, but the onset is not until age 10 or later.  (A prerequisite of Usher is deafness at birth).  Dr. Levy's response, "you can take today's results with a grain of salt."  If she has Usher, there would be no way to tell at this age, and unfortunately, the best thing to do is schedule another appointment in a few years.  Dr. Levy believes that 3-6% of deaf infants have Usher.  Hopefully, we will be able to find out if it's Usher during our genetic testing appointment - though Usher is not impacted solely by one (or a few) chromosomes so it's not easy to detect before it's onset. 

It's great to know that our daughter's vision is okay, but upsetting that we still have more questions.

Sept 8, 2011 - 3 months old!

Sept 2011 - Railroad, Hotdog

Madison began speech therapy sessions at The Center for Hearing and
Communications, which is conveniently located about a mile from our
apartment, in early August.  As mentioned earlier, these services are
through the DIP program.  When Madison's services began we had
sessions with the Director, Lois Heymann, until being assigned a
permanent therapist once the school year began.  After Labor Day,
Madison was assigned a speech therapist, Jessica.  Madison's sessions
are 1 hour long and take place every Tuesday and Thursday.  Initially,
Madison was sleeping through most of her sessions.  This gave me an
opportunity to ask questions to Jessica.  What can Madison hear with
her HAs? Since Madison's hearing loss is so severe - she will not hear
sounds of speech when she wears hearing aids.  Sound she may hear
consist mainly of environmental noises: airplanes, baby screaming,
doorbell ringing, whistle blowing, jackhammer to name a few.  What
types of responses should we expect from Madison when she is wearing
her hearing aids? An infant with normal hearing would not be turning
their head to sounds yet... Signs at this stage in development include
things such as eye brows raising, stop sucking a pacifier, startling.
At this age, it is very difficult to detect.  Once Madison is a little
older (4-5 months) she will be put in a sound booth to see if she
reacts to any of these types noises.  How should we interact with our
daughter? One of the most important things is to treat her as a child
with normal hearing.  Sing to her, talk to her, dance with her... do
all of the things you would do as if she had no hearing loss.  They
stressed the importance of constantly engaging Madison.  Any noise she
makes - we repeat back to her.  If we are playing with a toy that
makes a noise (i.e. rattle), once the noise stops... we wait for her
to make a sound, than continue the movement (i.e. shaking).  Madison
has been making so many noises, will they eventually stop? All babies,
regardless of hearing loss go through a routine with "talking".  First
they begin to coo, than they begin to babble, than jargin and
eventually begin speaking real words.  In babies with hearing loss,
they follow the first two steps... but eventually they stop.  That is
because they are getting no feedback to continue (ie hearing someone
talk back to them and have a "conversation").  Because of this, it is
extremely important to react anytime she makes a noise... hoping that
she will not stop making the noises.  The past 5 sessions, Madison has
been awake! She seems to really enjoy therapy and love Jessica! The
session usually begins with Jessica testing out her HAs to make sure
they sound normal.  She connects the mold of the HA to a long tube and
inserts the other end into her hear.  She makes sounds testing their
functionality "Ahhh, Eeee, Oooou, ChhChh, Ssss Ssss, Railroad,
Hotdog".  The vowels/constants and those exact two words include all
of the frequencies in the audiogram for speech sounds!  This usually
takes a few minutes than it is time to play! Madison spends most of
the time giggling, cooing, laughing and enjoying herself.  We have
played with a drum, cow bell, bells, bubbles and building blocks.
Madison's favorite toys seem to be the shiny bells.  Madison will
continue going to therapy twice a month.

Monday, September 5, 2011

September 2011 - Her Latest Trick

Madison absolutely hates tummy time... so she figured out how to get out of it!  Take a peak below at her latest trick!

September 2011 - Little Miss Sunshine

Mommy is making sure that I wear all of my pretty dresses before I grow out of them... Here are a few pictures, more to come...

August 2011 - Dear Madison

Most of you may know that we decided to have a doula assist with the birth of our daughter.  We were so glad to have met such a wonderful women, Rachel Weber - she arrived at our apartment when labor began and was with us in the delivery room up until Madison's arrival.  She eased my mind and helped soothe my pain... and truly made everything such an enjoyable experience.  We just received this letter in the mail from her last week.  Enjoy the read.

Sunday, August 28, 2011

Aug 2011 – Beep…Beep…Beep…

Madison was fitted to have molds made for her hearing aids (HAs) when she was 9 weeks old.  Green foam was inserted into her ear, almost like silly string, which hardens and takes an impression of her inner ear.  Two weeks later we went to Cornell to pick them up.  Our audiologist showed us how the hearing aids work and how to clean them.  Since Madison’s hearing loss is so severe, they don’t expect her to be able to hear sounds of conversation.  There is a chance that she may hear environmental sounds (fire truck, airplane, ferry boat, etc), but she may continue to hear nothing...  At the very least, the HAs will prime the brain for the CIs (pending she has an auditory nerve).  The HAs seem somewhat difficult – first, they seem hard to insert in her ear and second, they make a beeping noise when not sitting in her ear properly.  Hopefully both of these aspects will get easier with practice. 

A few hours after returning home from picking up the HAs, I realize something isn’t right.  “Beep… Beep… Beep…” The noise would not stop from her left HA.  I kept trying to reposition it – but quickly realized the HA was too small and not sitting in her ear properly.  I wish this was realized while we were still at Cornell.  What a headache… back we went the following day.  New impressions were made of both ears.  Since Madison is growing so quickly, this is completely normal.  It seems like she will need to have new impressions/molds made every few weeks for the first couple of months. 

Until this day, Madison’s hearing loss was always in disguise.  Since she will always be wearing HAs during waking hours – not only does it serve as a constant reminder of our situation, but I assume will stir immediate questions from others… friends/family and strangers.  This is something I haven’t quite prepared myself for… but than again, I haven’t quite prepared myself for any of this.  I keep telling myself that things will get better and easier – we just need to take one step at a time. 

Aug 11 - Uptown Girl

On August 11, we went to the Clarke School on 83/East End Ave for 3 things:
  • Tour of the facility
  • Meeting with our EI coordinator
  • Begin our EI State Evaluation Process
The Clarke School provides services for speech therapy in addition to having pre-K for children 3, 4 and 5 years old.  Plus, the 5 year old kindergarten class is integrated - half of the students have no hearing loss.  We met numerous students as well as the teachers. Everyone was extremely nice and it was great to see all of these children, with either CI or HA, they were talking (back) to the teachers! It was very encouraging to see the 'finished' product of where we would like to be in a few years.  All of these kids were very happy
and smiling and learning in a great environment.
We had our initial meeting with our EI coordinator - Tamara.  As we've mentioned before, we feel lucky to be in NYC with so many options available to us.  The EI program is actually a state-run program that will provide Madison with services X times a week.  Because of Madison's profound hearing loss, we hope to qualify for at least 2 days, and possibly 3 days a week.  Tamara has been involved with the EI program for several years and we believe she'll be a great asset for us as we embark on this journey.  Tamara was able to offer us a lot of advice and insights - into what we should expect with the EI program, as well as her experiences in dealing with children and parents in our situation.

The initial state evaluation process took about an hour and was conducted by two therapists.  The therapists asked us a ton of questions, and the test would not end until we didn't pass 4 questions in a row.  It was upsetting having to go through parts of the test, as it's used for children with numerous types of disabilities.  So, they would ask us if she responds to sound(s) and we all knew what the answer would be...  Then, the next question would be - does your child try and imitate your voice after you speak with her...  Followed by - does your child turn their head when you talk to them.  It was very frustrating to have to deal with this.

We are eager to have our follow up meeting with the state and Clarke in September.

August 2011 - DIP

DIP = Deaf Infant Program

We went on a tour of the Center for Hearing and Communications in Lower Manhattan.  In addition to being a facility that provides Early Intervention (EI) services, they conduct a special program called DIP.  The DIP program provides speech therapy services for infants until they are 3 years of age.  Because of Madison's profound hearing loss she qualifies for DIP and EI.  DIP gives Madison 2 speech therapy appointments per week for 60 minutes.
What could an infant with no hearing actually get out of a speech therapy session?  (This was one of our questions).  Initially, the sessions are geared more for the parents than the infant - considering Madison slept through her initial session.  The therapist tries to educate the parents, as well as instruct them on how to interact with their child.  The main goal is for you TO INTERACT with your child, just as if there was no hearing loss.  They encourage you to mimic your child's noises - if they speak some jargon, try to imitate that back.  Plus, they want you to constantly talk to your child - almost like the narrator of a story.
The therapists have seen the success stories and know the first few years are pivotal in her growth.  It's going to be a lot of hard work, but with the right therapy and supporting cast we're hopeful Madison will be chatting up a storm shortly after receiving CI.

Saturday, August 27, 2011

Aug 8 – Madison Is 2 Months Old

Madison is smiling every day and enjoying her morning bottle feed with her daddy.  She has already outgrown most of her newborn clothing.  She had her 2 month pediatrician visit and is doing great… She received 2 vaccines and, surprisingly, she did not scream! She grew almost 2 inches and weighs 10.2 lbs.  I am amazed how fast she is growing and how quickly she seems to be learning.  The one thing she hasn’t done yet was notice her older brother & soon-to-be best friend, Alan.  Alan is probably the most well behaved chocolate lab ever! Aside from trying to enter her jungle activity mat, AKA Costa Rica, he hasn’t bothered her one bit!  

Speaking of Alan, another thing Brian and I have discovered during our journey was about “hearing dogs”.  How would Madison know to wake up if there was a fire alarm? If she was napping, how would she know the door bell was ringing or someone was trying to call her? These are all questions going through our head.  Just like there are seeing eye dogs, there are also hearing ear dogs!  Well, dogs are trained to be able to work with people who have a hearing disability.  What a great job!  We plan on getting Madison a hearing dog (lab of course!) for her 5th birthday.  Please don’t tell her!

Aug 5 – 20 Is the Magic number

We had a surgical consult with Dr Brown, pediatric surgical ENT surgeon at Cornell Presbyterian, to discuss the possibilities of a CI.  He began by telling us that when this procedure is done at such a young age, the chances for success (meaning one being able to learn to speak) are extremely high… and chances that she could be mainstreamed into regular school at a young age is very likely.  But, not everybody is a candidate for this procedure.  On paper, Madison is an ideal candidate… but a MRI needs to be conducted to determine if she has an auditory nerve.  We were told that there is 1 in 100 chance that she will NOT have a nerve…it is extremely rare.  In addition, this physician has never encountered such thing.  We try to remain positive… but than again, given our situation already being very rare, it is very difficult to keep our hopes up.  Considering she is a candidate for a CI, she can be implanted as young as 6 months… but she needs to weight 20lbs.  Although this is a minimally invasive procedure, anesthesia is still administered and blood is still lost… thus the weigh requirement. 

August 2011 – Early Intervention

One of the best things regarding our daughter’s hearing loss is the amount of resources and support that are available, especially here in NYC.  There is a state-run program called Early Intervention (EI) that provides services from birth to 3 years of age to those with hearing loss.  Since Madison’s hearing loss is so severe, she automatically qualifies to receive speech therapy sessions.  Speech therapy for a deaf child??  If Madison is a candidate for a cochlear implant, she will be able to hear… and eventually will be able to speak.  We met with a surgeon to discuss what this would entail, but basically she would not receive the implant until she is 1 years old.  Anyway, back to Early Intervention.  In addition to speech therapy, Early Intervention will provide hearing aids (HAs) for Madison.  Although we were told that the HAs won’t allow her to hear conversation, it will give her access to more sounds than she currently receives… and prepare the brain to process sounds via the cochlear implant.  We were assigned a service coordinator that walked us through the EI process.  Boy, a lot of information was thrown our way...  I guess it’s a good thing that there are so many resources out there but it was and is very overwhelming! 

Cochlear Implant (CI)

A hearing aid amplifies incoming sound but there must be functioning hair cells and an intact nerve to transmit that amplified signal to the central auditory pathways. A CI, on the other hand, attempts to replace a function lost by the cochlea, usually due to an absence of stimulable hair cells. In a normal hearing ear, the hair cells within the cochlea act as transducers of mechanical and hydraulic vibration of the tympanic membrane, ossicles of the middle ear and perilymph and endolymph of the inner ear to chemo-electric energy capable of stimulating the eighth nerve. The decrease in hair cells causing a sensory hearing loss results in the cochlea losing its ability to stimulate the eighth nerve. The cochlear implant replaces the function of the lost hair cells by converting mechanical energy (sound waves) into electrical energy capable of exciting the auditory nerve.  CIs are surgically placed within the inner ear, bypassing the hair cells of the cochlea and directly stimulating the endings of the auditory nerve. Although there have been many variations on the theme, the basic design of an implant system has remained relatively stable over the years. It consists of an external microphone, processor and transmitter and an internal receiver-stimulator and electrode array.

Feel free to visit this website to learn more about Cochlear Implants:

August 2011 - Hope

Due to advancements in technology, there is a high probability that Madison will one day learn to hear and speak just like you and I.  Most people are surprised when I tell them this but basically there are two options regarding how to communicate/raise a deaf child – learn sign language or through spoken language.  There is a device called a cochlear implant (CI), which enables someone who is profoundly deaf to hear (see above).  We plan on learning as much as possible regarding cochlear implants and hope that this is an option for Madison.

July 2011 – A Trip to Holland

We received an email from my sister-in-law a few days after receiving this news… It was titled “Trip to Holland

A Trip to Holland
By Emily Perl Kingsley

I am often asked to describe the experience of raising a child with a disability – to
try to help people who have not shared that unique experience to understand it,
to imagine how it would feel. It’s like this…

When you’re going to have a baby, it’s like planning a fabulous vacation trip to
Italy. You buy a bunch of guide books and make your wonderful plans…the
Coliseum, the Sistine Chapel, Gondolas. You may learn some handy phrases in
Italian. It’s all very exciting. After several months of eager anticipation, the day
finally arrives. You pack your bags and off you go.

Several hours later, the plane lands. The stewardess comes in and says,
“Welcome to Holland!” “Holland?” you say. “What do you mean, Holland? I
signed up for Italy. I’m supposed to be in Italy. All my life I’ve dreamed of going
to Italy.” But there’s been a change in the flight plan. They’ve landed in Holland
and there you must stay.

The important thing is that they haven’t taken you to a horrible, disgusting, filthy
place full of pestilence, famine, and disease. It’s just a different place.
So, you must go out and buy new guidebooks. And you must learn a whole new
language. And you will meet a whole new group of people you would never have
met. It’s just a different place. It’s slower paced than Italy, less flashy than Italy.
But after you’ve been there for a while and you catch your breath, you look
around. You begin to notice that Holland has windmills. Holland has tulips. And
Holland even has Rembrandts. But everyone you know is busy coming and
going from Italy, and they’re all bragging about what a wonderful time they had
there. And for the rest of your life you will say, “Yes, that’s where I was
supposed to go. That’s what I had planned.” And the pain of that experience will
never, ever, ever, go away. The loss of that dream is a very significant loss.
But if you spend your life mourning the fact that you didn’t get to Italy, you may
never be free to enjoy the very special, the very lovely things about Holland.

July 26 – The Sound of Silence

Today was the day Madison had the ABR Diagnostic hearing test at Cornell Presbyterian.  She must be sleeping for this test as well.  Since it is more involved, she needed to be sleeping for 2 hours.  If we weren’t able to complete the test naturally, the test would be repeated using sedation.  Of course, we wanted to avoid this at all costs.  Madison behaved and she slept the entire length of the test! Tiny electrodes were placed on her head again and connected to a computer system which measured her brain’s response to different clicking sounds.  These sounds were played at 3 different frequencies and ranged from 60 decibels to 120 decibels.  Normal hearing ranges from 0 – 20 decibels.  The audiologist allowed 3 hours for the test.  Since we started on time, she was able to analyze the results and share them with us in her office later that afternoon.  Normally she would have us return the following day.  We were very anxious to hear the results and glad we did not have to wait one more day.  My mother was with us for the day so she was able to join us for the appointment.  The audiologist brought the four of us into a room and sat us down at her table.  Before she was able to say anything tears started rolling down my cheeks and my heart sunk.  Sitting in front of me was a piece of paper with Madison’s name written on top and a red line was written in across the bottom graph with the word PROFOUND.  The results showed that Madison has profound hearing loss in both of her ears.  Basically, if she is sitting outside on a runway, and an airplane takes off, she MIGHT be able to hear it.  So many questions were running through my head… WHY, WHY, WHY? Was this really happening to us? The audiologist spoke with us for a good hour discussing what her hearing condition means for us.  I couldn’t tell you what she said because all sorts of thoughts were running through my mind.  All along we had been praying for miracle… than realized we had already been given one.

July 21 – Tests, Tests and More Tests

After discussing potential outcomes between my husband and I – we were expecting the doctor to say that the left ear was better than the right ear.  We were not prepared for the news we were about to year….  Instead, we were told, “Your daughter has at the very least moderate hearing loss in both ears.”  Shocked, we asked about the different (better) results for the left ear vs. the right ear.  The doctor said that because the readings were so low, that he was unable to assume that one ear was better than the other.  Tears, followed by more tears…followed by the doctor saying, “It’s good that we’re finding this out at such a young age.  If this is her only issue, chances are she’ll be able to live a great life…It could always be worse.”  Another hearing test was scheduled to determine the exact level of hearing loss, this was called a ABR diagnostic test.  This is similar to the ABR screen test but the test is longer and results are much more detailed.  Additional appointments were scheduled – one with an Opthalmologist to determine if Madison has any vision problems and another with a genetics specialist – to determine if there is a genetic link to her hearing loss.

July 19 – “Good… She passed”

We needed Madison to sleep while the audiologist conducted the test.  The test itself was about 30 minutes.  After numerous feeding sessions she finally cooperated.  Tiny electrodes were placed on her head – one on her forehead and one behind each ear.  On the computer screen during the test was a graph with an X and Y axis.  The y-axis was numbered from 1 through 5.  The x-axis was more of a timeline.  We needed the plot to get to the number 3.  It would rise from 0 to 1, then 1 to 1.5 – and for the left ear it made it all the way to a tad above 2, but then would drop back down.  Reality started setting in, but we thought that there had to be some hearing.  Why else would the line move up? The next test was for fluid.  Upon seeing the results, the audiologist said, “Good…. She passed.” – translation = she didn’t have any fluid in her ears.  This was a test we were hoping that she would fail.  Unfortunately, that was not the case…

Auditory Brainstem Response (ABR) Hearing Screen

  • The normal auditory system generates small electrical currents when excited. These electrical responses are the measured response during ABR tests.
  • To record an ABR, small surface electrodes are attached to the head at several places.  An earphone is place in the ear, brief sounds are played, and the electrical signals across the electrodes are recorded.
  • These electrical signals, which include the ABR, are amplified and then fed to a computer, where they are averaged. Signal averaging is used to reduce the recorded level of other ongoing brain activity and to resolve the small electrical responses generated by the auditory system.
  • If hearing is normal, these electrophysiological responses should be observed for low level stimuli.
  • If hearing loss exists, the threshold of these responses will be elevated.
  • ABRs are less dependent on the status of the middle ear, compared to OAE tests, because the response is recorded across surface electrodes and does not have to travel back out through the middle and external ear.

July 8 - Madison Is 1 Month Old

Madison received her first round of vaccinations at her 1 month pediatrician visit…. Where she screamed more than ever! She is growing and now weighs almost 8 lbs.  The medical assistant performed the OAE screen again.  Brian and I were hopeful that she would pass…. But she did not.  Our physician still told us that this happens often and not to worry… the cause is most likely fluid trapped in her ears.  She than referred us to an audiologist where Madison would have another, more accurate, hearing test conducted, ABR hearing screen (see below).  In addition, they would conduct a which is a test to determine the amount of fluid trapped in the eardrum.

June 2011 – 1 in 1,000

Over the past few weeks Brian and I noticed that Madison was not responding to sounds.  Pans would drop on the floor of the kitchen and she would remain sound asleep.  Fire trucks would pass down our block and she would not stir.  We found ourselves purposely making loud noises to see if Madison would flinch… She would not.  This is when our research began… We started reading about different reasons babies do not pass the newborn hearing screen.  Since the pregnancy was healthy and there were no direct links to hearing loss, we didn’t even suspect this was an issue.  We learned that for every 1,000 babies born, 1 has profound hearing loss.  We felt the reason she wasn’t passing the tests was due to fluid in her ears.  Worst case scenario was she would need to have tubes surgically placed to remove the fluid, if it didn’t resolve on it’s own. 

Otoacoustic Emissions (OAE) Hearing Screen

  • The normal cochlea produces sound in response to external stimulation; this internally generated sound is the measured response during OAE tests.
  • To measure OAEs, a small probe is placed in the infant’s ear canal and sound is presented by either one or two tiny loudspeakers. Any response generated by the ear is recorded with a small microphone housed inside the probe.
  • If an infant’s cochlea is functioning normally, internally generated sounds will be recorded.
  • If cochlear hearing loss exists, the cochlea either will not generate a response or it will generate a response that falls below the level that is expected from an ear with normal hearing.
  • Normal external and middle ear function is important if OAE measurements are to be interpreted correctly as tests of cochlear function. Even if a cochlea is functioning normally and produces an OAE, the response may not be recorded if it is attenuated by fluid in the middle ear or any external ear canal anomalies.

June 13 - "Most Likely Fluid"

Madison had her first physician’s visit a few days after we got home from the hospital.  She was a healthy baby and would not receive any vaccines until her one month visit.  The medical technician performed the same “newborn hearing screen” that was conducted in the hospital.  This is known as the OAE hearing screen (see below).  Madison did not pass in either of her ears.  Our physician asked if we had any hearing loss issues in our families.  We told her that we did not.  Once again, we were reassured by our physician that things are okay and not to worry.  She stated that the cause is most likely fluid and that will drain on it’s own over time.  Brian and I didn’t really think much of it, but we did start to pay attention to Madison’s response to sounds…

June 9 - Newborn Hearing Screen

At the time, little did we know that this was going to be the first of many tests in Madison’s life.  An audiologist came into our recovery room saying she needed to conduct a “newborn hearing screen”.  She inserted tiny probes into Madison’s ears and measured the output of sound waves with a small hand-held device.  She didn’t pass… We were told not to worry, that most babies don’t pass at this age.  The likely cause is fluid in the ear from birth.  She reassured us that everything was going to be okay.  Brian and I thought nothing of it and continued enjoying the moments of Madison’s first day of life.

June 8, 2011 - Happy Birthday To You!

7:58pm Madison Elizabeth is welcomed into the world weighing 7lbs & 1 oz and 21 inches