Living the Unexpected

I've been dragging my feet on writing this blog entry.  If you have spent any time reading posts on Hazel, you'll probably recognize that there aren't many instances where I am posting solely for the purpose of an update on how well everything is going.  Mainly it's because our life is moving at a pretty frantic pace right now and it's easier to cover the things that are going on in chunks instead of short bursts of information.

It's been about a month since Hazel had her most recent eye surgery and to be honest, her eyes are doing pretty well right now.  I mentioned last post that Hazel has her own tiny sunglasses to help with her sensitivity to bright lights and Aubree is taking her in on Friday to get a prescription done for her glasses.  We are still doing steroid drops four times each day in both eyes...but that seems like nothing compared to what she has needed in the past.  Hazel's most recent eye pressure check was on Monday, where both eyes registered pressures in the low-20s (that's a good range for her).  For the first time in her life, Hazel doesn't have an eye appointment for TWO MONTHS!  I wish this meant that Hazel was getting a break from doctor appointments and medical attention....but that's not the case.

The only reference we have for Chromosome 6 deletions is a comprehensive document on all the known active cases in the world -- which is 45...well, 46 counting Hazel.  In this document (linked here) it explains everything we know about C6 deletions and issues it can cause.  One feature of this deletion is that two-thirds of the people with this deletion experience some level of hearing loss.  To this point, the assessment of Hazel's hearing has been a bit of a roller coaster ride.  She failed two hearing screenings when she was born, had an inconclusive hearing test when she was a few weeks old, then had a completely successful detailed hearing screening in November 2013.  On July 14th, Hazel went to the ENT for Children to have tubes placed in her ears to drain excess fluid that was affecting her hearing and to have another detailed hearing test.  The procedure was a quick one and went really well... but the hearing test revealed that Hazel has suffered nerve damage and has "moderate hearing loss".  We are not 100% sure at this time if the hearing loss is permanent or the root cause of the nerve damage.  The top two theories are that the excess fluid in her head has been applying pressure to her nerves for too long or that this is related directly to her chromosome deletion.  Either way, she has been fitted for tiny hearing aids and will need them immediately to help avoid a delay in her speech development.  Aubree and I have also discussed learning sign language to help with her development or if she doesn't have her hearing aids in a noisy environment.

To this point, Hazel has had an MRI on three different occasions and we've determined that she will also need a tube shunt in her head to alleviate the fluid that has built up.  The neurologist and neurosurgeon believe there is a possibility that her hearing could rebound from the nerve damage that has already occurred -- but who knows.  The official date has not been set, but the surgery will be at the end of August and will unfortunately require a short hospital stay.

I'm trying my best to stick to the facts about Hazel's health issues...but it's hard not to feel like our family is being kicked while we're down.  This month was supposed to be the first time in a long time where there weren't any surgeries, we thought there would be less doctor appointments, more positive news instead of getting blind-sided by more health issues.  Guess that is just not in the cards right now.  I guess this is just life.

It would be hard enough to get Hazel to leave glasses on her face without ripping them off....so I'm not sure how we're going to get her cooperation when we also include hearing aids.  Our plan is to start both at the same time so that hopefully she will just see this as a single change instead of adding two different things.  Unfortunately, the hearing aids will need updated ear moldings every month or so to keep up with her growing ear size....which means more scheduling appointments, time, money, etc...

I know everything is going to be fine in the long run, but the relentless nature of Hazel's health issues doesn't seem to be letting up any time soon.

Post Op Update

We got a slight break this morning and didn't have to be at the surgery center until 6:30 am which is an hour later than the previous three surgeries.  Guess you've gotta enjoy the little things....  

Hazel had an uninterrupted night of sleep last night and was in pretty good spirits by the time we made it to the waiting room.  It was still dark outside when we arrived and the building was basically empty, but early surgery times are always better than any time later in the day.  Hazel stayed in good spirits up until about 7:30 in the pre-op room when her hunger started to outweigh any toys or funny faces that Aubree and I could offer.  All the nurses who have check Hazel in for previous surgeries always like to come by and say hello or see how much she has grown.  When Dr Smith arrived in our pre-op room Hazel was making lots of noises and getting anxious, but she LOVES Dr Smith and got a big smile once they were face to face.  Overall, this was a pretty uneventful day as far as surgeries go.  Everything went according to plan and there were not any "on the fly decisions" that needed to be made by Dr Smith or by us.  Both of Hazel's tubes have been inserted into her eyes and are fully functional.  It will be really interesting to see what her eye pressure is like when Aubree takes her in for her check up tomorrow morning.

Hazel is in good spirits and is essentially acting like nothing happened;  Aubree needed to be at work today so today I stayed home so that one of us will be with Hazel all day.  We have been playing, laughing, watched team USA vs. Germany in the World Cup, had some pureed sweet potato, had a bottle and right now she is taking a nap.  And as a bonus, Aubree works close enough that she was able to come home at lunch and see everyone for an hour or so.  

From this point, we don't have any planned eye surgeries for the immediate future...so we are in the monitoring phase for her eyes to see how she progresses.  We did get her fitted for tiny sunglasses and tiny glasses last week since she is very nearsighted, she will probably start wearing them in the next few weeks but who knows how long it will take for her to get used to them.  

I will keep everyone updated on some of the other issues that are on our plate for Hazel, including her excess head fluid, the ear fluid which is affecting her hearing and if anything happens with her minor heart condition.  The list of things we haven't addressed yet seems daunting when you list them all together, but they seem so secondary compared to her eye problems and finding out about her chromosome deletion.  We're really just trying to take this one step at a time and enjoying Hazel while she is still a baby.

I can't even tell you how encouraging it is to have people praying for Hazel so constantly and consistently.  It is tough to quantify the impact that prayer can have on a situation.  It's just impossible to know how things would be different if no prayers or fewer prayers had been said for our daughter.  Prayer is about faith and humility and turning things over to the Lord...not just for yourself, but for those around you.  Thank you to everyone who has been so faithful to God in this time and lifted Hazel up to Him in prayer.

Eye Surgery on Thursday

It's that time again...surgery week.  On Thursday, our little family will go to the surgery center in Dallas for Hazel's 6th eye surgery.  By this point everyone who reads the blog knows how we feel about these surgeries days...they are just about the worst.  We will go through the same motions that we've done 5 times before: Go to bed early the night before (which never works out).  Set our alarms for 4:15 am or so and barely get out of the house in time.  We'll be the first car in the parking lot, first patients in the building, we'll go through the same check-in process and sign all the same forms.  The hardest part is usually that Hazel can't eat for a few hours beforehand and can be unpredictable.  It is what it is I guess.

Overall I would say that Hazel is doing really well.  That doesn't mean that things are perfect...just that we've been in worse positions and things are slowly getting better.  There are still lots of concerns that we have about Hazel's health and more procedures to schedule.  I probably just need to make a list of Hazel's medical needs that could use prayer.  You know what...I'll add that to the bottom of the posts from now on.

I don't expect anyone besides Aubree and I to keep up with exactly what is happening at each surgery or appointment, so here is a refresher on what is planned for Thursday.  Dr Smith always starts her surgeries with an in-depth EUA (exam under anesthesia) to check Hazel's eye pressure, how previous surgeries are healing and to document how various parts of her eye are doing (Corneas, lenses, optic nerve, etc).  From that point, Dr Smith will be making a path for a tube to be placed in her right eye.  Six weeks ago, Hazel had a procedure which placed a plate and tube ON each eye and a path was created for the left eye where the tube can be placed IN her eye.  Following her surgery this Thursday, both eyes will have the tubes placed in her eyes to relieve fluid and get the pressure into a safe range.  Overall, we are hoping that this surgery is pretty straight forward and uneventful.

As usual, I will try and keep everyone up to date on how everything goes on Thursday and beyond.  If you would like specific things to pray about for Hazel, here is a list:

-  Successful eye surgery on Thursday

-  Focus and precision from Dr Smith on Thursday

-  Quick healing process

-  Hazel has fluid in her ears that is affecting her hearing... the fluid needs to be drained with a minor procedure.  It will require anesthesia, which is always pretty serious.

-  That Hazel's excess fluid in her head would normalize and not require a procedure to drain the fluid.

MRI and Eye Surgery #6

Just a quick update on Hazel's upcoming doctor appointments and procedures...

On Tuesday afternoon, May 27 Hazel will have her second MRI to get a good look at the excess fluid in her head.  This will be a good measure of how much fluid is building in her head since we have the MRI result from a month ago for comparison.  Depending on the results of this MRI, we will make a decision on when and how to relieve the pressure that is building from the fluids.  The most likely scenario is a procedure performed by a neurosurgeon to place a tube shunt in her head and drain the fluid.  I will update this issue when there is more information to share.

Secondly, we have a date set for Hazel's next eye procedure which will be on Thursday, June 26.  Her most recent surgery placed one plate and tube on each of her eyes and they made a path in the left eye to place the tube inside her eye.  The procedure in June will make a similar path for the right eye to place the tube and relieve pressure from her eyes.  This will also be an opportunity for our glaucoma doctor and cornea doctor to do an EUA (exam under anesthesia) which provides accurate eye pressures and will give them a chance to monitor her past surgeries.

There is not much else to report right now besides schedule updates -- but that's a good thing!  Hazel's little personality is really starting to take shape recently and she can be lots of fun.....or the opposite :)

Thanks to everyone who has been praying specifically for Hazel's upcoming MRI and for healing from recent eye surgeries.  I could probably start a separate blog that is filled with thank yous to all the individuals, families and church groups who have let us know that Hazel is on their hearts.  Sorry if the last paragraph always sounds repetitive with all the thank yous, but every week we are reminded somehow that our daughter is being lifted up in prayer.

Hazel Wristbands

Recently, our sweet friends Brooke Suarez and Amy Kraus from Valley Ranch Baptist reached out to Aubree and I with an idea for fundraising and promoting prayer for Hazel.  The idea was to produce Hazel Wristbands that would serve as a reminder to pray for Hazel's health and maybe the sanity of her parents!  After some discussion, we decided to put Hazel's name on one side of the wristband and a favorite verse on the opposing side:

"For we walk by faith, not by sight" - 2 Corinthians 5:7 (ESV)

This is a special verse to us since it perfectly reflects Hazel's vision problems and the path our family's life is on right now.  We are moving forward with faith in God's plan as our guiding light and not dependent on our own vision.

We hope that anyone who wants to purchase a wristband will use it as a reminder to pray and spend time with the Lord.  A wristband is more than a reminder to pray for Hazel; it is a reminder to pray for others in your life and a way to spread the message of Christ and the importance of prayer to others.  Don't get me wrong -- I want you to pray for Hazel!  But if anyone asks about your wristband, don't make it about Hazel's surgeries or chromosome deletion...make it about God and how He has impacted a tough situation with unbelievable support and love.

The secondary goal of the wristbands is to contribute to Hazel's medical costs and any proceeds will go towards medical bills.  It is important to us that everyone knows that any donations go directly towards medical bills and medical bills only -- not diapers, not clothes, not toys, not children's Tylenol, not everyday stuff.  Aubree and I are blessed with jobs and have always been prepared financially for "regular" expenses.

If you would like to purchase a wristband, Brooke and Amy are handling all the logistics and are selling them for $5 each. 

To order, please contact Brooke or Amy:

Brooke Suarez: Brooke82a@yahoo.com

Amy Kraus: Amykraus11@yahoo.com

Post-Op Update and Upcoming Surgery #6

Last week we took Hazel in to see her glaucoma specialist, Dr Smith, to get an accurate measure of her eye pressure and look at the progress of her pressure-relieving surgeries.  The best case scenario would be that Dr Smith does an EUA (Exam Under Anesthesia) and tells us that the previous surgeries were a success and that her eye pressures were in the goal range of less than 20.  What actually ended up happening was closer to worst case scenario.  Hazel's eye pressures were both in the mid-30s and scar tissue had formed in her eyes which had negated the passages that Dr Smith had created in her eyes to help fluid drain out.  As a reference, these are the options we determined are best for fighting Hazel's glaucoma....

1.  Control eye pressure with eye drops and see if pressure decreases naturally as she grows.  

• Result: Failed -- The glaucoma was too aggressive to be controlled long term with drops.

2.  Additional surgery to both eyes to create passage ways which will aid in fluid drainage and be a long-term solution for pressure control.  

• Result: Failed -- scar tissue formed over the passages and pressure is rising again even with eye drops.

3.  Plate and tube shunt.  A small plate is placed on both eyes, which is connected to a tube...an opening is created for the tube to be placed inside the eye and draw out fluid which dissipates into her body.

4.  [Out of options]

Last week we took the initial steps for option 3, resorting to a plate and tube.  This option is the higher risk because it involves placing foreign objects in the eye and a path must be created for the tube to go inside the eye.  There is a narrow area for Dr Smith to place the tube without hitting Hazel's cornea, lens, iris or causing structural damage.  Last Thursday Hazel had surgery to place a plate on both of her eyes and the path was created inside her left eye for the tube.  Both plates have been placed on her eyes....but only the left eye has a tube inside the eye at this time.  We will have to go back in 5 weeks +/- to do surgery on the right eye to insert the tube.  This will be eye surgery number six for Hazel...2 cornea transplants, 2 failed pressure relief surgeries and now 2 plate/tube placements.

The good news is that the plate and tube option is almost guaranteed to work....but the flip side of the coin is the higher risks involved for placing the plate and tube.  Before the surgery her eye pressures were at 35 and 37...at today's checkup her eye pressure was 13 in the left and 35 in the right.  This is a big deal for her vision and long term outlook -- Hazel's eye pressure hasn't been below 20 since she was three weeks old.  We're making progress but it is accompanied by risk.

Hazel's eyes will be bloodshot for a few more weeks and we are dealing with some swelling that looks pretty nasty.  Through it all, she is in good spirits and does not seem as bothered by her eyes as we thought she would.  We are trying to stay focused on all the other things a 7 month old needs to do: work on sitting on her own, eating solid foods, establishing a sleep schedule and other developmental benchmarks...but its hard to do it all.  

I will update the blog if anything new happens or when the surgery date is scheduled for the right eye.  Sometimes they don't give us much notice for the surgery date and other times we have a few weeks notice.  I can't write a post without thanking everyone who is praying for our daughter and showering us with support...it really does mean a lot!

Hazel the day after surgery

Update: Eye Exam and Surgery

Quick update from the Eye Institute of Texas Surgery Center. Hazel's eye pressure is high in both eyes today...they were in the high 30s and we need them to be below 20 ideally.  This is pretty rough news to handle. 

Dr Smith is placing a plate on both eyes, which will be directly above the eye and hidden beneath her eyelids. She is also placing a tube into her left eye to relieve pressure. Hazel will need the same type of tube to be surgically placed in the right eye in six weeks. 

This news sucks really bad but we are out of options...we've been fighting this option for a long time but this is what has to be done despite the risks involved. 

I'll try and update or post other pictures when she gets out. Should be done by 8:30 am or so. 

Eye Exam and Possible Procedure

This Thursday, Hazel will make another trip to the surgery center located on the top floor of the Texas Eye Institute.  The initial reason for this visit is to get an EUA (exam under anesthesia) where both her glaucoma doctor and cornea doctor can exam her progress...but there is a possibility that there will need to be another surgery in addition to the exam.  

Let me explain...

This will be Hazel's third trip to this particular surgery center...the first two visits were for her recent surgeries to relieve pressure from her eyes in a procedure known as a combination trabeculotomy-trabeculectomy.  Basically, the surgery is meant to create additional passages in her eye where fluid can drain.  These two procedures were a success at the time of surgery, but there is not a real definitive way to tell if they were a complete success without having her eyes re-examined and getting an accurate measure of her eye pressure.  The biggest threat to Hazel's vision at this point in her life is glaucoma and the eye pressure it creates.  If her eye pressure is too high it can pinch or strain the optic nerve in the back of her eye.  After a certain amount of time the nerve can become damaged to the point that it cannot function -- and that's when the lights go out for good.

In a perfect scenario, we would only be giving Hazel steroid eye drops to help the cornea transplants...but right now her pressures have been high enough that an additional eye drop is needed to keep her pressure in the preferred range.  We usually try and get an eye pressure check when we go to doctor visits, but it is getting harder and harder to convince Hazel to cooperate.  On Thursday they will get an accurate measure of her eye pressure and make a decision on whether or not to take a more drastic step to fight eye pressure.  The possible surgery would involve placing a plate on the upper portion of her eye that would be hidden behind her upper eye lid.  This small plate would also have a small tube attached which CAN be inserted into her eye if her eye pressure ever decides to spike and cannot be controlled with eye drops.  This is the last stand against eye pressure and carries with it some higher risks of damage to various parts of her eye if the tube is ever used.  If we ever had to place the tube into her eye is would be because there is no other option -- it's place the tube or risk losing her vision.

There is no guarantee that Hazel will need the plate to be placed on her eye, but I've been dread this day ever since I knew it was a possibility.  Nothing about her eyes has been easy and Thursday will no exception.  We are hoping that her eye pressure will be low enough that there will not be a need for more drastic measures to fend off her glaucoma.  But who knows...

We would appreciate any prayers this week for Hazel's eye pressure to be between 15 and 20, for positive reports about her previous surgeries and a sense of calm for Aubree and I.  I will try and post again on Thursday night or soon after with any news.  This will be the 7th time Hazel has gone under anesthesia and possibly her 5th eye surgery, so we will take all the prayer that we can get.

Hope everyone is having a great week. 

Detailed Chromosome Info

We have known about Hazel's chromosome 6 micro-deletion for a few months now, but recently we have been reading some detailed information about C6 Deletions which was provided by our geneticist (Dr Golder Wilson).  The PDF (found at the bottom of the post) is a lengthy document but is a good read with information about other children with this micro deletion and to what extent it impacts their life.  

I don't think that everyone will want to read through the document, but it is worth posting if even one person wants to learn more.  I wanted to post it just in case anyone wanted to thumb through it, glance at a few pictures of the C6 kids or learn more about what this could mean for Hazel.

If you decide to take a look, just know that Hazel's deletion is 6p 25.3 through 25.2.  Meaning....6th chromosome, "p" meaning the short arm of the chromosome and 25.3-25.2 is the specific portion where her deletion occurred.

Hope everyone is having a great week!

http://www.rarechromo.org/information/Chromosome%20%206/6p%20Deletions%20from%206p25%20and%20the%20end%20of%20the%20chromosome%20FTNW.pdf

Hitting the Reset Button

I feel like there has been a lot of changing and evolving information that I've put on the blog in the last six months, so now might be a good time to hit the reset button and give everyone a "current status" of sorts.  There are a lot of things about Hazel which some people know, but not everything we know about her makes it to the blog.  It's unrealistic to think that I will post every little thing that happens and many people have been focused on praying for her eye condition...unfortunately, there is more to Hazel's life than an eye condition.

After going through a detailed screening (a few months ago), we were able to determine that Hazel has a genetic deletion on the short arm of Chromosome 6.  It is considered by our geneticist to be a micro-deletion.  A chromosome deletion is exactly what is sounds like....if you were to take the long strands of DNA from her sixth chromosome than you would find that a section never developed and is simply not there.  There are a very limited number of Chromosome 6 deletions in the world (around 40 active cases) and it is very likely that Hazel is the ONLY person in the world with her specific deletion.  If you can't tell from the description, this is rare.  Really rare.  Like..."they don't have a name for it because its too rare" territory.  You won't find a fun run to raise awareness or a certain color of ribbon that people wear to raise funds for research.  Hazel is a 1-in-7 Billion scenario.

Her condition carries with it a number of potential issues, but there is not much data to work with to determine how likely health issues are to arise.  It's safe to say that more articles will be written about Hazel's condition than are available to read about her C6 deletion.  Being born with eye issues is on the list of possible problems, which is why she was born with cloudy corneas and has required four surgeries in her first six months.  She also has a few other things that could happen down the road because of her C6 Deletion, such as: heart conditions, spinal issues, hip dysplasia, enlarged head size, cognitive delay, emotional delay, delayed growth and a few others that I can't remember without consulting our genetics report.  Hazel's C6 Deletion is the top of the pyramid for her health issues and everything else that happens is a result of the genetic issues.

We have been going down the list of possible issues and addressing them one-by-one to try and get ahead of anything that we can.  Hazel has had two echocardiograms of her heart which found a slight defect called PDA, she has had a hip and spine ultrasound which ruled out any early spine and hip problems and most recently, Hazel had an ultra-sound of her head and an MRI.  The MRI from earlier this week revealed that there is extra fluid around her brain, resulting in a condition known as hydrocephalus.  We are being referred to a neurosugeon in order to get a second opinion and talk options for draining the excess fluid.  It might seem obvious, but conditions involving the brain and head are pretty serious...so it's been a stressful week for us.  We thought we were past the biggest hurdles when we did her fourth eye surgery, but now we have a new area of concern.  The relentless nature of these health issues is really taking its toll...

There was a time where Aubree and I did not want to fully disclose what is happening with Hazel and her genetic deletion.  It might sound strange since she is still a baby, but we were concerned about Hazel being thought of as a medical case or as the girl who has a disorder instead of just being a child.  At the end of the day, this is Hazel's life and this is how it's going to be...but this has been the hardest six months of lives.

We both feel comfortable now talking about her genetic deletion, especially since it is creating new health issues which can use the power of prayer.  We know how many people are praying for our daughter, including people who have never met us or know us personally.  Thanks to everyone who keeps Hazel in their thoughts and for all the encouragement over the past six months.

Hazel's 6 Month Photo