Sunday, October 28, 2012

Dear Sue

Inspired by the deaf woman I met this morning.

Dear Sue,

You don't know me,
I don't know you,
So how dare you presume
To think that you do

You have once piece of information
And give it the meaning
That I have rejected
The essence of your being

My child's CI does not imply 
I am one of those  mothers
Who refuses to accept
The differences of others

I will  not choose between
The black and white path laid ahead
I choose the course unseen
The path of acceptance instead 

Of misunderstanding

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:

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.

Tuesday, October 2, 2012

Finally, A Diagnosis !!!! - Pontine Tegmental Cap Dysplasia

This is just a brief update but I wanted to go ahead let everyone following this blog know about an interesting development today.  After months of exhaustive research online, I sent Eve's MRI images out to two separate doctors who have recently confirmed that she has an extremely rare (and I mean something like 1 in a million) brain disorder that has only recently been classified.  It is called Pontine Tegmental Cap Dysplasia.  Doctors still don't know much about the condition and there is a very large spectrum of possible outcomes so we cannot rely on the existing cases to tell us what Eve's potential is.  There are only about 20 case reports of people with this condition in the medical journals.

They think it is a genetic condition that involves a "de novo" mutation.  This means it is highly unlikely any future children we have would also have this condition!  I am in the process of collecting as many journal articles I can about the disorder.  I am hopeful that having an actual diagnosis might help us figure out better ways to treat her and possibly will enable us to connect with some of the other families of patients with this diagnosis.

Here is a link to a brief explanation of the condition:


Friday, August 10, 2012

Eve's Eye Condition - Corneal Anesthesia

The Diagnosis

As we were struggling with Eve's feeding problems and figuring out how to cope with her hearing loss diagnosis, we discovered yet another problem.  When Eve was about six weeks old she came down with a cold.  We started to notice a slightly cloudy spot over the middle of her right eye.  I remember calling the hospital's nurses hotline to ask about what over the counter medications I could give Eve for her cold and mentioned Eve's eye to the nurse.  The nurse told me that it is common for babies with colds to have mucous come from the sinus passageways into the eyes.  She advised us to wipe Eve's eyes with a soft napkin.  When I did this I noticed that Eve did not close her eye when I would wipe it.  I remember thinking this was somewhat odd but did not really dwell on it.  The next week I was on the phone with Eve's pediatrician and mentioned the issue with Eve's eye and she was not concerned.  Every day I would look at it and I realized that the spot was getting larger and larger and we became more and more concerned.

On February 25, 2011, Eve had another visit with her pediatrician and I asked the pediatrician to take a look at it.  She agreed something did not look right and said she would get us in with an ophthalmologist.  As I was heading home from the pediatrician I received a call from ophthalmology.  The pediatrician had called them and they wanted me to bring Eve in that very day.  So we brought Eve in to see the ophthalmologist.  He said he could not tell us what was going on unless he could examine the eye with Eve under anesthesia.  He told us he thought she might have glaucoma in her right eye.  Needless to say, John and I were alarmed.

On March 1, 2011, we were able to get Eve into the hospital for an examination under anesthesia.  We spoke to the opthamologist beforehand and he again told us he did not know what the problem us but he was certain he would have an answer for us following the examination.  We anxiously waited for about 30 minutes and they called us into a room to talk to the opthamologist again.  He told us it was not glaucoma.  He said the epithelial layer of her eye was extremely damaged and there was a central scar-like lesion over her cornea but he had no idea what could have caused this to happen.  He said he did not think there was an infection as it is highly unlikely for only one eye to become infected, and only her right eye was affected.  He gave us a cortical steroid to apply to the eye for a few days and said that if the condition did not improve by the next appointment, then it meant that the cause probably had something to do with her nerves.

Eve  on 3/1/11, just before we left home to have her first eye exam under anesthesia.
Three days later, March 4, 2011, we brought Eve back into her  ophthalmologist .  He confirmed our fears - her eye was not improving.  I remember waiting forever in the waiting room, then waiting even longer as he examined her and then left the room so he could confer with the head of the practice.  John and I were in agony as it was becoming more and more clear to us that our deaf child may have a serious problem with her eye that even her opthamologist was struggling to identify.  We waited so long that eventually I had to go back to the car to get my breastpump, as I could not go more than a few hours between pumps at that time.

I remember walking to the car in a daze and, as I was stumbling back I ran into a woman, a grandmother, whom we had spoken with briefly in the waiting room.  I think it was obvious that I was struggling because she asked me if everything was okay.  I could hardly vocalize a response, and I simply burst into a fit of tears on the sidewalk next to the parking garage at the Childrens hospital.  This woman simply gave me a hug and let me cry and told me we would figure it out.  Slowly I gathered my composure, thanked the complete stranger, and went back inside.  When I went inside, I found John just holding Eve tightly and he had tears streaming down his face.  It was so surreal.  I don't think either of us could believe what was happening.  I kept repeating in my head that Eve (and us) already had too much to deal with.  How could we possibly handle anything more?

Finally Eve's opthamologist, and the head opthamologist of the practice (who eventually became Eve's main opthamologist) came into the room.  Both of them examined her together and then they started bouncing ideas off of each other as to what the problem could be - naming rare conditions that John and I had never heard of.  They asked us if we were Jewish - to which we answered in the negative.  Finally, they told us they believe Eve has an eye condition called corneal anesthesia.

Corneal anesthesia is a condition in which the person has no or a reduced sensation of touch in the cornea.  There are a variety of causes for this condition, but in extremely rare cases, someone is born with the condition.  It involves either failure of the fifth cranial nerve, or the part of the brain that processes the signals from that nerve (the pons).  It is often associated with a condition called familial dysautonomia, which is why the opthamologists asked us if we are Jewish.  Familial dysautonomia occurs nearly exclusively in people of Eastern European Jewish ancestry.

Corneal anesthesia is an extremely difficult corneal disease to manage.  It has no known cure.  In fact, the article I have linked below says it is one of the most difficult and challenging ocular diseases that still lack treatment.  What happens is that, because there is no sensation in the eye, the eye does not blink, produce tears, and the internal processes that the eye has for repairing any damage that occurs to the eye does not function properly.  To make things worse, because the patient cannot feel any pain or other sensation in the eye, they often self-inflict wounds, simply because they cannot feel when they are causing themselves damage.  With an infant, this condition is that much more difficult to manage because the child simply does not understand that she needs to protect her eyes.

Here is an article that discusses this condition, its causes, and its progression.

The initial stage of treatment that the opthamologist suggested back in March 2011 was just to extensively use lubricating drops and ointments.  Over a matter of several agonizing weeks, Eve's eye did heal.  There were ups and downs during that entire process that were extremely frightening for John and I.  We had to bring Eve back in a few more times for additional eye exams under anesthesia but there was slow improvement.  Once the right eye healed from that incident, we used preservative free-lubricating eye drops every hour on the hour.  We hoped that would be enough.  Unfortunately, it wasn't.

The Second Incident

We went several months without incident.  We brought her into the ophthalmologist every few months and we thought everything was going to be okay.  Then, some time in November we started noticing both eyes looking more and more red more and more often.  Finally, towards the end of November, right before our planned trip to Dallas over Thanksgiving, we realized her eyes were consistently looking more and ore red.  The ophthalmologist was very concerned and said both eyes looked pretty bad.  He instructed us to put lubricating ointment in her eye every hour for the next week and said that if things didn't improve he would have to perform what is called a tarsarrophy.- which means, in layman's terms, sewing the eyelids together.

He said we could go ahead and go on our Thanksgiving trip to Dallas but I regret that decision now.  Every day of that trip Eve's eyes got worse and worse and worse.  Both eyes became blood red, had cloudy looking white circles in the middle, and the circular areas were putting off this gross mucous-like stuff.  The right eye seemed worse than the left.  Here are the photos we sent to her doctor via email when we were in Dallas:

Left Eye.  11/28/11

Right Eye.  11/28/11

It was an absolute nightmare.  I remember falling asleep one night in tears underneath her crib.  I was convinced she was going to be blind.  Night and day I hoped and prayed and pleaded that her eyes would start healing but they were just getting worse and worse.  I nearly brought her into the ER in Dallas but her ophthalmologist just said we should just bring her in in a couple of days when we returned to San Diego.  The day after we got back home we were at the ophthalmologist's office first thing.  I remember checking her in and just sobbing uncontrollably.  John managed to stay composed enough to talk to the doctor and explain what was going on.  That day or the next day the ophthalmologist had her sedated and performed the tarsarrophy.  

Here is a close-up of what the tarsarrophy looked like once the swelling went down:


The entire month of December I was a complete mess.  The stitching on Eve's left eye came out a few days after it was first sewn in so we had to bring her back again and put her under anesthesia again to sew it back up.  

Eve was simply unbelievable through all of this.  She could barely see anything yet she remained happy as can be.  She seemed somewhat more subdued than her normal self through it all but she really amazed John and I with how well she handled it.  Here is Eve on her first Christmas with her eyes sewn about 80% closed.  I just adore her precious smile in this photograph..

Eve Troy.  12/24/11.
We left for Colorado to visit John's family on Christmas Eve.  The next day, the stitching on her left eye came out AGAIN.  UGH!  It was so frustrating.  This was, however our first opportunity to see how her eye was actually doing.  Fortunately, it looked MUCH better.

Left Eye.  12/25/11.

If you look closely at this photo, however, you can see the spot where her eye is now scarred because of this incident.

Here is Eve in Colorado!  Her first time in the snow!
Eve Troy 12/27/11
And here she is on her first birthday!

I really strive to do my best to make sure Eve has all of the same experiences that any other child would have.  Even though she has these issues, I do not want her to grow up and use her disabilities as an excuse to not do something or accomplish anything anyone else could accomplish.  I want her to learn that we all have struggles and challenges in different ways even though, for some people, those struggles are more  visible and obvious than for others.

The Ongoing Struggle

In January 2012 I took Eve to Philadelphia for some testing to see if she might be a candidate for a cochlear implant.  I will talk about that testing in my next post.  However, while I was there, her  ophthalmologist suggested I take Eve to a colleague of his, a corneal specialist who happens to have an office in Philadelphia.   As part of a very busy day I took her to see this specialist.  His suggestion was that we perform a more permanent tarsarrophy at the corners of each eye.  This is done by splitting each eyelid down the middle and then sewing the eyelids together for a period of time while the eyelids fuse together.  Doing so would keep the eyes more closed and keep the eye more moist and able to heal itself.  We were reluctant to follow this advice at first, but slowly came to realize that lubricating eye drops alone were not enough to keep her eyes in good shape.  On February 7, 2012, she had the procedure done.  This is what it looked like right after the procedure.

Partial Permanent Lateral Tarsarrophy 2/7/12.
About an hour after we came home that afternoon I looked away for one second and noticed that the stitching on her right eye came out.  This is what it looked like:

2/7/12.  Tarsarrophy stitching came loose.

So I frantically called her doctor and was able to get him on the phone by calling the hospital's main line and asking them to transfer me to the surgery area.  He was between surgeries and said we needed to bring her back that day so it could be redone.  He redid the surgery on her right eye using a slightly different technique, with the hope that it would stay better.  Here she  at physical therapy the next day:

Eve Troy. 2/8/12.

 And here is my all time absolute favorite video of Eve.  Even with all this crap on her eyes, she is just the happiest child.

Anyway, here is what her eyes look like now:

Eve Troy.  3/5/12.

Eve Troy. 6/17/12
It breaks my heart to know that her peripheral vision is impacted by this procedure.  But at the same time, we are hopeful that it will help to maintain the integrity of her eyes long term.  She may need to keep her eyes like this for years, if not forever. 

As it stands, both of her eyes have some scarring and opacification.  The right eye is worse than the left.  Her doctor does not think the scarring over the left eye impacts her vision but it probably has some impact to the vision on her right eye, although she can still see very well with that eye.  

Our daily routine with her right now involves binding her arms with arm braces at night and any time she is unsupervised in the car seat.  We do this so that she does not poke her eye when we cannot stop her.  We use swim goggles outdoors and, of course, in the water.  We use preservative free lubricating eye drops every 15-30 minutes during waking hours and use a lubricating ointment at night.  We have to watch her constantly to make sure she is not poking her eye with her hands or toys.  Her eyes require constant attention and constant management.  We have to take her to the ophthalmologist sometimes every week and at least every few weeks.  On nearly a daily basis her eyes look red or opaque and we have to deal with it.  It is like riding a roller-coaster.

Even with all of this, in the last several months there have been a couple of incidents where her right eye became damaged, but it healed up without needing surgical intervention.  Every time this happens there is slightly increased scarring.  These minor incidents will add up over the course of her lifetime.  The truth is that it is a progressive disease that will cause her to suffer decreased vision over time.  How bad will it be?  We really do not know.  It largely  depends on how attentive we, and later she, is to the condition throughout her life.

The uncertainty of it all drives me mad sometimes.  Both John and I struggle with coping every time one of her eyes becomes damaged because we know every single one of these incidents causes further scarring or opacification that will  add up over time.  It causes both of us great stress and anxiety and we are still working on figuring out how to cope.  Of everything we are dealing with, we know that her vision will only get worse and worse despite everything we do to stop it.  I try not to dwell on the future for too long, because there is always hope that some better treatment will be discovered.  Right now, however, it feels like we are on a downhill battle - just trying to stave off the inevitable.  I cannot wrap my mind around the possibility that Eve could be both deaf AND blind someday.  It is just so terrifying and unfair.    


Friday, July 20, 2012

The Light at the End of the Tunnel

I have written extensively about Eve's up's and downs with feeding and so I want to share this video with you all.  Since Eve received her feeding tube over a year ago she has not been able/willing to drink more than a couple of sips at a time, and she certainly was not able to do it on her own.  Every day we offer her drinks, over, and over, and over.  So many times I have wanted to give up, thinking it is hopeless.

Well, this week we had a major breakthrough! She actually started to tip her head back and take several gulps on her own!   John and I were practically in tears when we saw her actually drinking!  For the last few nights she has been able to drink about 1-2 ounces of milk from a sippy cup.  Here is a little video I took of her doing it.

I encourage her by cheering her on and raising my arms.  She seems to enjoy the encouragement because she will take a few sips, put down the cup and copy my cheers.  I emailed this video to Eve's occupational therapist, Ann Mazzeo.  I told the OT "you are not going to believe this!"  Here was her response:

I do believe this!  You made my day!!!!  She needed the "skills & tools" to be able to do this on her own!  This is because she has two awesome parents, that carryover, challenge, and listen..... and they have a daughter that is determined and wonderful to work with!  Thank you for this very happy share.
 We certainly couldn't have done it without all of Ann's encouragement.  Way to go Eve.  Thank you for reminding me that I should never give up or lose hope in what you can accomplish.

And here is a photo and a video of my sweet girl at Gymboree today.


Tuesday, July 10, 2012

Accepting My Child's Deafness

The Diagnosis

Eve failed the newborn hearing screen at this hospital the day after she was born but I was not at all concerned. The audiologist who ran the test assured us that many babies who have perfectly normal hearing, especially those who were delivered via cesarean, fail the initial screening due to fluid in their ears. At the time, I simply did not have it within me to accept that Eve having hearing loss was even a remote possibility.

When Eve was three weeks old we brought her back for another screening with the same audiologist at the hospital where she was born. Eve failed that test. It was then that a knot of fear began to form at the center of my being.

I got Eve in to see an audiologist at Rady Children's hospital right away to perform two tests. The first test is called brainstem auditory evoked response (BAER) test. The BAER test works by using electrodes to measure brain wave activity that normally occurrs in response to certain sounds - clicks and beeps, etc. The second test is called oto-accoustic emissions (OAE). The OAE test records the sounds that are produced within the ear itself by hair cells inside the cochlea in response to sounds. The existence of these inner-ear sounds essentially confirms that the cochlea and other parts of the ear, including the auditory nerve, are functioning.

On the morning of January 20, 2011, John and I drove the hospital testing center. I clearly remember bringing Eve inside, feeding her until she fell asleep, and then watching as the audiologist ran the tests. My level of anxiety throughout this process rose and rose and I kept repeating in my head "there is simply no way my daughter has hearing loss." That morning was the longest morning of my life.

This is what the BAER test looks like. These photos were taken during Eve's second BAER test on April 15, 2011. Which simply confirmed the original results.

Once the tests were completed, the audiologist told us the results. She said the BAER test indicated Eve has something called "auditory neuropathy" and that Eve had absent (meaning no inner ear sounds) on the OAE test. The results were the same in both ears. At the time, John and I had absolutely no idea what any of this meant.

The audiologist explained that she diagnoses auditory neuropathy based upon specific abnormal readings on the BAER test that are believed to be related to either the functioning of the auditory nerve or the transmission of signals from the inner ear to the auditory nerve. There is not a lot of research or information about this condition as it has only been diagnosed since the early 1980's. The audiologist told also told us that people who have auditory neuropathy on the BAER can have normal hearing, or hearing loss ranging from mild to severe, or can have off-and-on hearing, with sounds fading in an out or sounding distorted in some way. The audiologist told us that the only way to determine how much Eve could hear is by testing her behavioral responses to sound. So all we knew at that point was that there was likely a problem with Eve's hearing, but we just had to wait to learn more.

As the audiologist was explaining all of this to us, I was overwhelmed with grief. I could hardly think clearly and spent the next thirty minutes trying to choke back my tears as I asked question after question to the audiologist. Eve (and John and I) already had enough on our plate with Eve's feeding problems. I simply could not believe what I was being told.

The Grieving Process

When John and I went back home, we handed Eve off to his mother who, fortunately, stayed with us through those first difficult weeks, and went to our bedroom. We just held each other and cried.

The next day, I was taking a much needed afternoon nap when John woke me up with a phone call from my sister, Hayley. She told me that my other sister, Hayley's twin, Heather had just called her briefly from Taiwan to tell her that Heather had just been contacted by a local hospital and informed that her boyfriend, Mark, had been hit and killed by a taxi. Shortly afterwards, I spoke with Heather when she was at the hospital after she had just identified Mark's body. To this day, that conversation replays over and over in my mind. It is just so so sad.

Although I never met him, I could tell what a wonderful person he must have been because he made Heather so happy. They were so in love. It was such a tragedy. These photos were taken just a few months before the accident.

I wanted to go be with Heather so desperately but I couldn't. Eve needed me. I spent the next several weeks simply going through the motions. Pumping breastmilk, trying desperately to feed Eve, and watching her so closely for any sign that she was hearing anything.

On March 1, 2011, since Eve was going under anesthesia for an eye exam, her ENT specialist went ahead and ordered an MRI. The next day she called me to tell me that, based on the MRI, it looked like Eve has no auditory (or vestibular) nerve on the right, and maybe a small auditory nerve on the left. The ENT told me she would order another MRI when Eve was closer to a year old and that it is possible that the nerves just appeared small because Eve was so young. The reason these results were significant is that a child simply is not a candidate for a cochlear implant if he or she does not have a functioning auditory nerve. I will discuss the cochlear implant decision in more detail in a later post.

Over the next few months, John and I became more and more convinced that Eve was not hearing anything. We started putting her in audiology booths for testing. Consistently, she did not respond to anything at all on the right. On the left side, we felt that she was possibly responding to very low tones at a very high volume. She was essentially profoundly deaf on the right and severely deaf on the left. We tried hearing aids. They did not seem to make any difference whatsoever.

Understanding and Acceptance

As it slowly became clear to John and I that we were, in fact, parents of a deaf child, we realized that we had a lot of learning to do. We spoke with doctors, audiologists, and teachers for the deaf. We also sought out parents of children with hearing loss and, most importantly, we sought out d/Deaf adults. We read books, blogs, medical journals, publications put out by the government, watched documentaries,etc, etc, etc, etc. All of a sudden, John and I were in the middle of an debate about how to raise and educate deaf children that has been ongoing for many decades. Practically everyone we spoke with had a different opinion, and believed deeply and passionately that their approach was the best approach. It is a strongly debated, and deeply divided issue.

It was the stories of the Deaf adults that were the most touching to us. So many of them had parents who refused to learn and teach their children American Sign Language and, instead, focused on an oral/aural approach - i.e. forcing their deaf/hard of hearing children to learn to speak and read lips. For some people with hearing aids and only a moderate hearing loss, this approach works. For others, with more severe hearing loss, this oral/aural approach made their lives incredibly challenging and much more difficult than necessary. These people eventually learned American Sign Langauge, and resented their parents immensely for not making the effort to learn how to communicate with them. Many Deaf people I have met are completely alienated from their families, simply due to an inability to communicate with them.

John and I have come to believe firmly that parents of deaf children should learn the sign language that is native to their region and teach it to their children. There is a plethora of evidence that supports this. Here are a couple of articles that discuss the latest research on the importance of a visual mode of communication for deaf children.

Even if a child is capable of learning spoken language with the support of an assistive device, there is absolutely no evidence that teaching a child a visual language will limit his or her ability to develop a spoken language. However, if a child is not provided with access to a full and complete language during the early-childhood period, that child's ability to develop ANY LANGUAGE AT ALL EVER is permanently, and irreversibly stunted.

No matter what, we want to be able to communicate with our child, and we want our child to be able to communicate with the world. John and I have spent thousands of dollars on private ASL tutoring. I have fought her school district tooth and nail and they are now providing her with a deaf tutor who works with her three days a week. We will be able to communicate with our child. It has been so amazing to watch Eve's ability to communicate progress and progress. Here she is signing some of her favorite signs:





I am still, and will likely always have some degree of sadness about Eve's hearing loss. Music is something John and I both enjoy tremendously and we are extremely saddened that we will not be able to share that aspect of our lives with her. But Eve loves to dance. I dance with her whenever I can. There are so many other things I will still be able to share with her.

In January, 2012 I was at a restaurant with only Eve and I was reading a book about the psychological impact that being deaf has on deaf children and adults. The man at the table next to me asked me if Eve is deaf and I replied affirmatively. His response was "its not a big deal". At the time, his comment seemed insensitive. Over time, however, I came to understand what he meant. It certainly has been a challenge for John and I to navigate through all the different approaches that can be taken with raising a deaf child. And learning an entirely new language has been costly, can be frustrating at times, and has been extremely humbling.

On the other hand, I have met so many wonderful Deaf people who have made so many wonderful accomplishments and contributions to society. I have come to realize that, in the bigger picture of Eve's life, while being deaf will shape her identity in many ways, it is not something that will limit her ability to succeed and thrive or to do anything that she sets her mind on doing. And today's technology makes communication for a deaf person even less of a barrier than ever before.

I have come to believe that deafness is only a limitation if a deaf child's parents, family, friends, educators, and even society at large, view it as such. If the people in a deaf child's life fail to accept who the child is, fail to fully support the child's natural form of communication, or fail encourage the child to reach his or full potential, then that child will certainly be set up for failure. But that failure is not because the child is deaf, but because the child's deafness was not accepted, properly encouraged, supported, and even celebrated.

Thursday, June 28, 2012

Eve's Feeding Disorder - Life with A Feeding Tube

On April 29, 2011, Eve had surgery for the anatomical deformity I mentioned in my last post.  We also coordinated that appointment so that she could also have an  MRI and an eye exam (I will write about her eye issues later) at the same time.  We stayed overnight  went home the next day.

For the next three days Eve's feeding worsened and so, upon the recommendation of her occupational therapist, we finally relented and agreed that Eve needed a feeding tube.  We were admitted on May 3, 2011.  Upon arrival, the nurses placed a naso-gastric feeding tube ("ng-tube") into Eve's nose.  Thus, our journey with a feeding tube began. My thinking was that we could just use the feeding tube to give Eve those extra few ounces of milk each day that she refused to eat on her own.  For the first couple of days this actually worked.  In fact, on the second day she had the tube, we did not need to use it at all because she was able to drink enough on her own.

This is what Eve's N-G tube looked like.
I remember getting into heated arguments with the resident on Eve's case at the hospital about how her feeds should be administered.  The resident insisted we should give eve about ten ounces of milk on a continuous drip throughout the night.  I, however, had done my research and learned that night feeds and continuous feeds should be avoided, if possible, because the body does not normally eat that way.  Continuous feeds do not allow the stomach to expand enough that the child is able to eventually consume large quantities of food at any given time.   Ultimately, this can make it more difficult to wean a child from the tube. Also, I did not want to give Eve any more milk through the tube than was absolutely necessary.  I felt that this resident was setting Eve up to become tube dependent.    

After we went home we continued our regime of giving Eve at night only what she did not consume during the day.  However, over the course of the next month, despite my efforts at preventing it, she refused her daytime feeds more and more.  Despite our efforts to avoid it, it only took about three weeks for Eve to become entirely dependent on the tube.  It was shocking to me how quickly this occurred.  Looking back, it really makes a lot of sense that this happened.  Eating was so difficult for Eve.  She quickly learned that she did not need to do it as long as the tube was in place.

I hated that ng-tube.  It was sooo difficult to deal with.  Her face had a horrible reaction to the tape and was always red and irritated.  We never managed to keep the tube in her nose for more than a few days, as she could easily pull it out herself.  Placing the tube was an excruciating process for all three of us.  One of us had to restrain her while the other measured the tube and shoved it into her nose. She would, of course, scream hysterically the entire time.  I will never forget the time I placed the tube into her nose and instead of going into her esophagus, it came out of her throat.  I tried again and that time it came out of her other nostril.  Finally, on the third attempt it went into her esophagus.  I was constantly worried it would go into her lungs but we had been taught how to use a stethoscope to listen to the sound her stomach would make when a little bit of air was pushed into it.

Family Photo Shoot on 6/14/2011.  

If you look at her left cheek in both of these photos you can see the red marks left by the tape.
Since Eve's feeding was worsening rather than improving with the feeding tube, her GI doctor suggested pretty quickly that we transition to a gastronomy tube ("g-tube").  We thought there was a possibility that she might do better with feeding with a g-tube instead of the ng-tube.  Ng-tubes cause irritation of the throat and nasal passages, and they tend to exacerbate any existing problems with reflux.  Eve's g-tube was placed on June 15, 2011.  We stayed at the hospital for two nights to ensure it was working for her.

Not long after the g-tube was placed, Eve started throwing up her milk.  Sometimes just once a day, sometimes, several times a day.  We tried different formulas, different medications, different feeding schedules.  We had to make sure not to make more than one change at a time so we could figure out what helped and what didn't.  Since she was throwing up so frequently, the doctor advised against giving her large volume feeds overnight because there is a risk of aspiration if she were to throw up during her sleep.  So we wound up having to giver he continuous feeds during the night after all.  The regime that wound up working best for us was as follows:

  1. Nexium (for GERD) 2x a day exactly 15 minutes before each feeding.
  2. Azithromyacin (an antibiotic that helps with GI motility) in the morning but only when her stomach was completely empty, otherwise she would throw it up.
  3. Overnight continuous feeds.
  4. 4 daytime bolus feeds of 4 ounces over a period of 30 minutes while she is in her high chair, and keep her in her high chair for another 30 minutes.
It took us four or five months to figure all of this out but with this regime she threw up less frequently, maybe a couple of times a week instead of every day.  Some weeks were better than others.  If she had any type of head cold, her stomach simply would not tolerate fees and she would throw up more frequently.  It was so difficult to see her struggle with throwing up.  Eve is such a happy child normally but when she would throw up, she would be miserable, and all I could do is hold her and comfort her while it passed.  Of course, all of the reflux and throwing up made her even less willing to try liquids.  Not long after the g-tube was placed she  started refusing all liquids of any kind altogether.

When Eve was about six months old, we began introducing solid foods.  Although she was refusing all liquids, I hoped that she would do better with solids.

Feeding Therapy - 7/6/11
Feeding Therapy - 7/6/11

Every time we would feed Eve during the day, we would offer her food by mouth.  The idea behind this is that, at minimum, she should associate being offered or tasting food with the feeling of her stomach being filled.  John and I are extremely consistent about offering her food any time we giver her feeds through the tube (if she is awake at least).  Unfortunately, more food winds up on her than in her.  Here are a few examples:

7/21/2011 - Avocado
7/21/2011 - Avocado

7/22/2011 - Banana
7/27/2011 - Carrot

She will typically open up for one or two bites but she never consumes very much volume of anything.  Earlier this year I thought we had a breakthrough with re-fried beans.  I remember she ate about a tablespoon of them and I was in tears because that was more than I had ever seen her eat before.  She showed interest in them for a few weeks but then lost it.  We continue offering and offering. Sometimes she shows some interest but most of the time the food winds up on her or on the floor.  Lately she has started spitting the food back on whomever is offering it to her.  She used to refuse by looking in the other direction and clamping her mouth shut.  Now she can sign "all-done" and will sign it repeatedly until you stop offering whatever it is you are offering.

I have a love-hate relationship with the feeding tube.  On the one hand, I hate that my child can't eat. I hate that she can't enjoy the wonderful sensory experience of food.  I hate that feeding her is a seemingly never-ending battle.  Food is an extremely important part of life in my family.  John loves to cook and, for us, it is food that brings people together.  Any time we have family over, we plan and cook delicious meals and consume them together.  It saddens me when I wonder whether if Eve will ever be able to be part of that.  On the other hand, I have a huge sense of relief that she is able to get the nourishment she needs to grow.  It wasn't until after we got the feeding tube that Eve really started developing her gross motor skills.  It enabled her to focus her attention on things other than eating.

Coping with a child with a feeding disorder has been extremely difficult for me.  It is a constant struggle and a constant worry every day.   I don't know when or how we are ever going to get her to eat normally.  I don't know if she will be able to enjoy food together with us as a family.  The problem is, the more anxious and nervous, and forceful I am towards making her eat, the less inclined she is to want to eat.  It is a vicious cycle.  Often feeding disorders start as a physical problem and develop into a psychological disorder that affects the entire family and the entire feeding process.  Feeding is no longer something the child can enjoy, it is something that they are constantly forced into.  I am still working on just letting it go.  There is only so much I can do.  The more I try to make it happen for her, the more she will feel imposed upon and forced into it and it becomes a battle.   I have to let it go. . .

Over a period of about six months we were able to increase the volume of Eve's feeds to five ounces and reduce the amount of feeds we give her during the day from four to three.  We are working on increasing her tolerance for higher volumes of formula.  Right now, Eve rarely ever throws up - so that is one minor success story.  When she finally stopped throwing up we were able to start giving her large volumes over a short period at night rather than continuous feeds.  She is consistently growing along a healthy and proportional height-weight curve.  As far as what she takes by mouth, I cannot get her to drink more than a couple of sips of any liquid.  On a good day she will eat about 5 goldfish or cheerios and maybe a few spoonfuls of a soup or puree.  Where we are at now took so many months of constant daily struggle.  These accomplishments may seem small because we have such a long way to go but, for us, these are huge accomplishments.  

Eve still has little or no desire to eat or drink.  Most of what I offer her winds up on me, on her, or on the floor.  Her GI doctor says he will not try to wean her from the tube unless she shows more skill at being able to drink.  However, because she is on the tube, she refuses to drink and so cannot gain the skill.  I honestly do not know how to break this cycle.  It is possible I might snap at the next person who asks me when Eve will be able to stop using the tube.  Although it may seem a relatively innocuous question coming from someone who has no familiarity with Eve's issues, for me, that question triggers so many feelings of anxiety, and anger, and helplessness.  I do not know the answer, and I wish I did.

For a long time I struggled with tube-feeding Eve in public.  People, especially children, stare.  I am sure it is because it is something they have never seen before, but it does make me self-conscious.  It was really the first visible sign that something is wrong with Eve.  It makes us look different.  I remember asking another woman I know who had a child (who passed away in her early twenties) with a feeding tube how she dealt with this issue.  She told me she would never feed her child in public - she would take her child to the restroom to feed her.  That woman's comment was really a wake-up call for me.  Feeding someone in a restroom is simply repulsive.  Would you go to the restroom to eat a sandwich?  There is a risk that her food could become contaminated, and certainly the odors of a restroom are not consistent with encouraging a person to eat . .  .

After thinking about the only real alternatives to feeding Eve in public - not going out of the house or feeding her in places like restrooms, I slowly became more comfortable with doing it.  I also realized that, as Eve grows older, she is going to have to learn how to cope with people's questions and looks, and I am going to have to be a role model for her and teach her how to handle those situations.  If I am ashamed of how she eats, she will pick up on those feelings, which could have long lasting implications on  her self-esteem.  Instead, if people stare or ask questions, I explain the situation.  Interestingly, children seem more understanding and less judgmental than adults.

A few weeks ago I was at a friend's baby shower.  I was drinking a Diet Coke from a can and, miraculously, Eve actually showed some interest in drinking it.  Now, certainly Diet Coke is not an optimal drink for a seventeen month old child.  On the other hand, I try to capitalize on the situation any time Eve shows the slightest interest in drinking.  In my judgment in that moment, I thought a sip of Diet Coke would not hurt her, and it could be an opportunity for her to actually get some practice drinking liquids.  So I offered Eve a sip of my Diet Coke.  Most of it spilled onto her and onto the floor.  Afterwards, I caught a glimpse of an older woman who had witnessed what had just happened.  She had an expression of utter disgust and judgment against me over what I had just done.  At first, I was angry.  Who did she think she was?  After thinking about it some more, I realized that, before having Eve, I might have reacted the same way.  As I  realized this, my anger at her judgment subsided. I am now a better person than I was before I had Eve.  Having gone through these experiences, I am much less likely to judge a person, especially a parent, based some initial observation.

To end this post on a positive note, here are some photographs of a moment in time where Eve did enjoy food.  It does happen sometimes.  These photographs help remind me that there may in fact be a day when Eve can consume and enjoy food on her own.

Eve's First Thin Mint - 2/3/2012

Friday, June 15, 2012

A Letter To an Amazing Father

To my husband, John:

Thank you for holding my hand and being by my side during Eve's difficult labor and delivery.
Thank you for changing all of Eve's diapers and taking over those middle of the night feedings during those first difficult weeks when I was still recovering.
Thank you for planning, shopping for, and making our meals every day.
Thank you for working so hard to provide for us.
Thank you for being my shoulder to cry on as I mourn the loss of the child that I thought I would have.
Thank you for feeling so deeply the pain and loss due to Eve's disabilities yet loving her more because of them.
Thank you for learning a completely new language  - and constantly encouraging me to do so - in order that we can communicate with our daughter.
Thank you for engaging me in those endless arguments over how to best care for her.  Two minds are better than one.
Thank you for helping to make those difficult decisions - the feeding tube, the cochlear implant, etc.
Thank you for letting me know I am not always right.
Thank you for being strong when I am weak.
Thank you for taking her to so many of those endless doctor and therapy appointments.
Thank you for constantly working with her, loving her, and encouraging her.
Thank you for always believing in her.
Thank you for always believing in me.
Thank you for sharing in the joy of her accomplishments.
Thank you for allowing me to see how a loving father interacts with his daughter.  I never experienced that.
Thank you for going above and beyond when many fathers would have ran in the opposite direction.
Thank you for giving me and Eve your whole heart all day and every day.

You do not know how amazing you are.

Men like you, the ones that not only support, but nurture their families, are rare indeed.  I honestly do not know what we would do without you. 




Here is a video of Eve on Fathers' day one year ago.  She has grown so much!

Wednesday, June 13, 2012

Eve's Feeding Disorder- The First Four Months

It is very difficult to describe the frustration and desperation that parent of a child with a feeding disorder must endure.  It is a struggle John and I endure all day, every day, with no clear end in sight.  Every time there is a step forward it seems to be followed by two steps back.  Although I believe this struggle is one that no one can truly understand unless they have been through it themselves, I will do my best to explain what happened.

We came home from the hospital on January 1, 2011.  We knew something was wrong with her feeding from day one but we did not yet realize the extent of the problem.  Because finger-feeding was so difficult for Eve, we reluctantly decided to transition her to a bottle.  It still took us an hour to feed her 20ml's of breastmilk.  She was clearly trying extremely hard but just could not coordinate the process.  She would pull away from the bottle, arch, and scream, or, alternatively, act like she was sleeping and refuse to open her eyes or move.  She did anything she could to avoid eating.  I continued trying to breastfeed but I became increasingly frustrated and she became increasingly averse to my efforts.  We bought every bottle at babies r' us.  None of them made any difference.  We had no idea know what to do.  Neither did any of our friends or family.

It often took two people working together to feed her.  One person holding her, the other manipulating her arms and legs or supporting her chin to help her engage the bottle.
On January 3, 2001 we took her to her first pediatrician appointment.  It was not her normal pediatrician but since we were having so much trouble feeding we thought we should get her in right away.  She was still below her birth weight.  She was a small baby - 6 lbs 15 oz and she was 6 lbs 5.5 oz at this appointment 5 days later.  It usually takes about two weeks for babies to regain their birth weight so this was not yet terribly concerning.  I told the pediatrician about what we were going through and did not take our concerns seriously whatsoever.  He explained to me that many women have trouble breastfeeding and said that maybe, if our society was different, and I had been exposed to more women breastfeeding their children, then I would be able to do it.  Apparently, the reason I could not breastfeed was because I did not know what I was doing.  He encouraged us to go ahead and move on to formula.  It is shocking to me that he was so dismissive.

Every day was a struggle.  I refused to give up on breastfeeding and even if Eve couldn't breastfeed I wanted to give her breastmilk and so I continued pumping every 3 hours around the clock.  We still spent an our or more during each feeding session six to seven times a day. One feeding session with her would sometimes run into the next one.  If Eve was sleeping, she would rarely wake up on her own to eat.  We would wake her up and offer her a bottle every 3 hours on the dot.    If it had been 4 hours and she still had not eaten anything we would do whatever it took to wake her up.  We took to taking her clothes off, move her arms and legs around, put cold cloth her face.  I am not sure which was better, the screaming hysterically or the utter lethargy when we would try to wake her to eat.  We methodically wrote down how much she ate at every bottle and how long it took.  We weighed her every day.  John even made spreadsheets in excel.

We weighed her every day on our kitchen scale.
We saw a lactation consultant that first week who diagnosed Eve with an "ineffective suck."  She had no suggestions on what I could do to breastfeed and refferred us to an occupational therapist.  I could not get an appointment with the occupational therapist for TWO WEEKS.

We saw Eve's regular pediatrician for the first time on January 7.  At that point, Eve was eating between 2-3 ounces per feeding session and it still took us an hour or an hour and a half each time.  At that appointment Eve weighed 6 lbs 10 oz so she had gained about five ounces.  This doctor did not take our feeding challenges seriously either.  The pediatrician told us that Eve was not a turkey.  She actually said that, verbatim.  Her implication was that we were force-feeding our child by stuffing her like you would stuff a turkey.  Her recommendation was that we be less forceful with feeding and that we let Eve go four hours between feedings instead of three. 

John and were extremely reluctant to follow her advice.  At this point we KNEW something was wrong with our child.  We knew she should be eating more and that it was not supposed to be this hard.  We knew that if we were any less forceful or persistent Eve would not gain weight.  We were right, but I guess we had to prove it before her doctor would take us seriously.  We spent the next three days following the pediatricians advice.  At that point Eve was eating only 1-2 oz and taking 45 minutes on average each session.  If Eve went to sleep before finishing the bottle we would just let her sleep and wait until she woke up.

At Eve's follow-up appointment three days later, she had lost two ounces.  At that point Eve was two weeks old and still several ounces below her birth weight.  At that appointment, Eve's pediatrician also noticed an anatomical defect that none of the other doctors, and even she, had failed to notice previously (the defect had to be surgically corrected when Eve was 4 months old but that is another story altogether).  Finally, the pediatrician took us seriously.  The pediatrician sent us straight over to children's hospital for monitoring and testing.

Eve's first visit to Rady Childrens' hospital was on January 10, 2011.  We spent three days and nights there while they evaluated her feeding and ran numerous tests.  They did a KUB test (Kidney, Urieter, and Bladder), an echochardiogram (heart), a renal ultrasound, a pelvic ultrasound, lumbosacral x-rays, and an upper GI study.  Everyhing was normal.  The doctors could not tell us what was wrong with Eve.  The most helpful thing that came out of that hospitalization was the occupational therapist that taught us some useful feeding techniques.   We were sent home on January 13, 2011 with only a prescription for omeprazole for acid reflux.  That was their best guess at what was going on.  Three days later we went to the ER because Eve was refusing to eat.  After we had been there for an hour she took a bottle and so we went back home.

Eve's first visit to Rady Childrens' Hospital.
John working with one of the occupational therapists.

1/12/11.  Eve with her great-aunt Cindy at Rady's.  Aunt Cindy is an ER doctor at Rady's and was a big support for us during those first difficult months.
1/13/11.  Eve with her "Bomsie" (my mother-in-law) and the little girl that shared our room at the hospital.
That first month was so challenging.  I think I cried every day.  I was pumping all the time, trying to take time out of each day to breastfeed, and spending the rest of the day trying to feed her.  The lactation consultants encouraged me to have lots of "skin-to-skin" contact.  The doctors were telling us to give her "tummy time".  I was rushing her in and out of lactation consultants, occupational therapy visits, doctors office, hospital visits, etc, etc.  I really don't know how we did it.

For the next couple of months, with constant effort and with the help of outpatient occupational therapy, Eve was able to start gaining weight. Some weeks were better than others but it was always a struggle.  We tried everything we could think of - different bottles, different nipples, one lactation consultant even convinced us she was "tongue-tied" and so we clipped her frenulum - the small piece of tissue between the tongue and bottom of the mouth.  She still struggled, cried, pulled away from the bottle, and screamed hysterically during nearly every feeding.  And she consistently did not eat enough.

Eve's occupational therapist, Ann Mazzeo was simply a lifesaver for us  during those first few months.  She was the only person who had any clue how to help us with Eve's feeding problems.  We still see her nearly every week.

We tried so many different feeding positions.

Eventually, at the suggestion of Eve's occupational therapist, we wound up using a bottle that is typically  used for babies with cleft lip and palate who have no ability to suck.  It is made of a thin plastic that you can squeeze to get the milk to come out.  We would squeeze the milk into her mouth and she could then swallow it.  She never developed a consistent sucking pattern but being able to squeeze the milk into her mouth helped.

The times Eve came down with a cold were the worst.  Having a stuffy nose made an already difficult process nearly impossible for her.  During the more difficult periods we fed Eve with eye droppers, finger feeding devices, and even medicine syringes. I remember many feedings when we would feed her by filling up a 3-ml syringe and refilling it and reoffering it over and over.  We could speed up the process by doing it together.  One of us would fill up 2-3 syringes while the other held her and offered them to her.  We would use the sring put the milk in her mouth and massage her throat to encourage her to swallow.  Many times the milk would simply spill back out her mouth and down her throat.  We would then sop up the milk and weigh the towel to see how much milk she had lost so we could figure out how much she had actually swallowed.  The situation was absolutely ridiculous and impossible. But what other choice did we have?  We had to feed our child?  Giving up was simply not an option.

I stayed home with her nearly 24/7 during those first few months, between having to pump and spending an hour to feed her, sometimes with her screaming hysterically and arching and pulling away, I was utterly embarrassed to leave the house.  I remember one of the rare occasions when we decided to go out to dinner with our friends, Rich and Jodie.  After we ordered our food it was time to give Eve a bottle.  Eve's reflux was flaring up and so even though we could tell she was hungry, she would pull away from the bottle turn bright red and scream every time we would offer it to her.  Since Eve was crying so loudly I decided to take eve to the restroom.  Jodie went with me.

Once in the restroom I offered Eve the bottle over and over and over.  Every time I offered, Eve would pull away screaming bloody murder.   I would then have to spend time calming her down.  Once she was finally calm I would offer the bottle again.  After about 30 minutes of this I was at my wits end.  I told Jodie, with utter desperation, "She is so hungry!"  Then, a woman who had just entered the restroom happened to overhear what I said and turned to me and said "Why don't you just give her a bottle then?"

Give Eve a bottle.  Seriously?!?  What a brilliant suggestion -  to give a hungry child a bottle.  I wonder why I hadn't thought of that.  I was so flabbergasted I could not think of an intelligent retort.  I had a vision in my head of handing Eve to Jodie and punching the woman in the face.  It is the first time I, as an adult, have had an impulse to be physically violent towards someone.  Instead, I just ignored the woman.  But I raged inside.  Why couldn't that woman have kept her mouth shut?  That situation taught me a valuable lesson.  Keep your  opinions to yourself when it comes to other people's parenting.  Every child is different and every situation is different.  Give people the benefit of the doubt, especially if you don't know anything about them or their child.

Eventually we were able to get into a consultation with a gastroenterology specialist but her only suggestions was just that Eve had reflux.  They did two endoscopies.  On the first one she had high levels of something called eosinophil's and we were referred to the allergy clinic for a possible condition called eosinophilic esophagits.  I tried a lactose free diet but it didn't help.  After the second endoscopy was clear, her doctor ruled out that condition and said they thought she just had reflux.   I read about the condition online and got the doctors to agree to switch her to Nexium at a higher dose and it seemed to help some.

At some point in April Eve's feeding started rapidly deteriorating.  I remember rushing her to the pediatrician one day and completely breaking down in tears in the office.   Eve would only take 2-3 oz per session and would consistently get several ounces less each day than she needed to eat to grow enough.  We tried fortifying the breastmilk with formula to make it more calorie dense but the more we fortified, the less she would eat.  By the end of April she was only eating 15-20 oz per day, it took us all day to give her that, and it still was not enough.  John and I were increasingly desperate and did not know what we were going to do.  Despite all of our efforts, Eve was not gaining enough.  I felt that every bit of my energy went towards feeding her yet she was withering away in my arms.  

I would go to bed nearly every night feeling defeated and in tears.  Crazy thoughts went through my head.  I was trapped and did not know how to get out.  A couple of times when Eve refused to eat I threw the bottle across the room.  I even punched the refrigerator.  John and I started getting into arguments over details like when to feed her or how. The truth is that neither of us knew what to do and we were taking our frustration out on each other.  We started going to counselling, and it helped.

While we were going through all of this a friend of mine who has a child who is a few months younger than Eve made a post on Facebook about how her child was growing too quickly.  My friend jokingly indicated she was going to wrap her child up in saran wrap to keep him from growing.  Reading that post made me feel so defeated.  My friend had no idea how fortunate she was that her child was healthy and growing.  I think many parents make jokes like this but they do not realize how blessed they are that their children are growing.  If your child is growing, you should take so much joy in that.  The alternative is simply a nightmare.