Nystagmus Surgery Trip Day 4 ~ Discharge, headed home and next steps!

WE ARE HOME!  It feels amazing to be back home as a family and in our own beds tonight.  It was a long week, but it really flew by quickly.  We woke up this morning at about 5:30am to Cole crying in pain.  The doctor suggested not waking him to give medication so it was expected that he would wake up with some irritation.  After getting more medication and a new cold compresses he settled down and went back to sleep.  Johnny and I, of course, were still pealing ourselves off the ceiling from not knowing what to expect from the crying.  More blood on the pillow?  A problem that needed a trip to the ER?  Of course we were briefed on all the possible complications and they were all running through our heads.  Luckily it was just "normal" pain and even better...we could fix it with medication!  Unfortunately it was much harder for us to fall back asleep.  Through all the medical issues we've been through the hardest parts are when there is nothing that can be done and you feel completely helpless in fixing the pain or problem.


We woke up in time to get to our appointment with Dr. Hertle at 8:45am.  We needed to wipe down our room, vacuum, put all the sheets and towels in the laundry and remove all trash.  We let Cole sleep until the last minute, made our donation for the accommodations, packed the car and headed to our appointment.  Luckily there was virtually no wait once we arrived in the office.  He wore a pair of sun glasses they gave him the previous day since he pupils would remain dilated for several days.  As soon as he walks outside he stops and covers his eyes - it is very painful for him.  Since he was able to open his eyes the nurse ran through simple tests having him look at letters on the screen again.  Although it was much harder than on the day of testing, he was able to answer with about the same size letters.  You could tell he got discouraged, but he worked really hard at trying to answer her questions.

Next Dr. Hertle came in and immediately engaged Cole and asked how he was doing.  He showed Cole an app on his phone to gauge the shaking in his eyes and the head tilt.  He said he sees a lot less shaking but there was still some head tilt.  He also said that he did 10 surgeries this week and statistically 1-2 of those will not be successful and may need to be repeated.  Unfortunately we may not know this until we have a follow up appointment with him and complete the testing again.  We may get some inkling if we don't see any decrease in the shaking, but he is hopeful that we will.  He has asked that we return for a visit in 2-6 months.  He said to expect the redness to continue for 6-8 weeks but the irritation should lessen significantly by the end of the weekend.  Cole is doing a great job telling everyone he meets that he had eye surgery...which is good, because the whites of his eyes are completely red right now. It really takes you off guard when you look at him and you have to double take.  I love that he feels comfortable enough to talk about it though because it will help him when he returns to school on Monday.

In addition to repeat testing, he has recommended the contacts for the reasons mentioned in yesterday's post.  He wrote a new prescription and suggested we get it filled immediately.  We have a regularly scheduled follow up with Cole's regular ophthalmologist on Monday so it's perfect timing.  He would also like us to consider giving him Baclofin.  He said we should wait 6-8 weeks so that we can get a clear idea of what progress we see with only surgery, but then adding the medication later.  He is about to publish an article that studies the progress of 85 patients after a combination of 1) surgery, 2) contacts, and 3) Baclofin. I've spoken with several patients with Nystagmus through a Facebook group who have chosen a combination of these so it is very interesting to learn this new information.  Our currently plan is to talk with Cole's ophthalmologist Monday and see if she concurs with these suggestions.  We plan to schedule a follow up appointment with Dr. Hertle in November or December and wait to consider medication until after that appointment and retesting.  Overall the appointment was very positive and he really gave us a lot of hope for with regards to Cole's long term prognosis.  He said that it isn't out of the realm of possibility that he may drive a car one day and he also said he sees no reason for Cole to learn Braille.  He said there is no harm in doing so, but that he expects him to be able to learn without it.

After our appointment we made a quick stop for breakfast since we let Cole sleep until just before his appointment then headed back to VA.  He was doing very well and we continued to give medication throughout the drive.  We had him all snuggled up in his blanket so he could rest.  We did stop for lunch and although he ate, he seems to get tired easily.  We headed to pick up Reagan from Nana and Pop's house in Leesburg and she was sad to leave.  She told us all about her visit there and showed us "her" room.  We were so blessed to have them watch her so she didn't have to miss school and so that we could focus on what Cole needed.  We headed home, unpacked, and went to pick up Shelby (our dog).  The kids were super excited to see her. So thankful our dog trainer and her Weimaraner kept her this week.  She has been wiped out since she got home!  Needless to say, we had a quick dinner, showers and the kids were in bed.

I will post an update after we meet with the ophthalmologist Monday.  I also created a short "before" video to try to capture the shaking and plan to take an "after" video so we can see the difference.  I will try posting those once he has healed enough to get a good video. Thanks again to everyone for their prayers!  We feel very blessed that surgery went smoothly and we are seeing some improvement already!

As I close my post from this week I feel compelled to share feelings from last night that I struggled to express then.  Hopefully I can express them now in a way that is understood by all and sensitive enough for those who can related. When we told Cole he was having surgery to help him see better he said, "I see just fine."  My purpose for this blog is not only for family and friends, it is for my children as they get older and for others who may travel this journey to feel less alone....less lost.  As I lay in bed with Cole last night I couldn't tell when he was awake or asleep because he didn't open his eyes.  He would lay still then all of a sudden say something when I thought he was sleeping.  He had no sight so he couldn't be entertained with TV or with his iPad.  We tried listening to the movie Cars, but it didn't hold his attention very long without the visuals.  I asked if he wanted to listen to music and he said no.  He listened as Johnny and I conversed asking many clarifying questions because he had no visual cues.  "Johnny, can you pass me another one."  Cole, "another what?"

Cole asked his dad to get him a Robin toy and dad came back with Batman too.  As I opened the package he asked which was which because he had no sight.  I ran his fingers over Robin's boots, over Batman's pointy ears and over the buttons down Robin's shirt.  I was so intrigued by the difference in my relationship with him by not having sight.  It completely changed our conversations.  In no way can I say that I understand the perspective of a blind child's parent, but for a very short period of time, I realized how drastically different that parent must be to help their child experience life. I catch myself pointing out sunsets or birds to Reagan and cringe when I realize Cole cannot share in these experiences.  Dolphins, boats, and rainbows.  I will ask if he can see something and he will say yes as he is looking out the wrong window.  I often wonder if he is embarrassed he can't see it?  Is he worried I will be disappointed? Obviously I wouldn't be but I find myself torn - do I avoid pointing things out that are so beautiful to prevent these feelings?  I share this tonight because I think it creates perspective for all of us.  And it makes me realize that instead of avoiding it, I should explain it in a way he can understand - as a parent would for a child who is blind.  Do we all do enough to describe the world to our children?  Authors do this all the time. That is what makes a good book hard to put down - when you FEEL what the character feels or SEE what the character sees.  Last night made me realize we really should do this with everything in life. It is just one more reminder that every experience brings new perspective....

Nystagmus Surgery Trip Day 3 ~ Akron Children's for Surgery

Well the big day is here and Cole was a champ...a true Ironman.  Although the plan was to sleep in, the construction crew woke me up at 5:30am and got Cole up by 6:30am.  We had an easy morning in the room and then ventured down to the family room and put in a movie and played with a few other boys who were staying here at the Ronald McDonald House.  I was very worried Cole would struggle not having food after midnight, but he only asked for a snack a few times and did very well without.  He insisted he wear his new jersey that we bought last night - seemed fitting since we call him The Cole Man.

We walked over to the hospital and arrived by 12pm and met with the nurse to take vitals.  Upon arrival everyone was very edgy about his cough.  The nurse and tech were very skeptical and said it would be up to the anesthesiologist whether surgery would move forward as planned.  Johnny and I both think he walked in to the room with a plan to cancel.  Luckily, though, he listened to Cole's lungs and had him cough and said he didn't see any problem with it.  We reviewed Cole's LONG history...AGAIN...and got him changed and ready to go.  I was prepared for the tears as he was escorted away by the nurse and Child Life Specialist, but as Johnny said, Cole was voted "ladies man" last year at school.  The look he gives her before they walk out is priceless.  He had to be reminded to give his mom and dad a kiss good-bye.  He was all smiles!

We walked out at about 1:07 PM and surgery lasted just about an hour.  We met with Dr. Hertle in a consult room after the surgery and he said everything went smoothly.  He said Cole's left eye looks pretty good considering...but that the right eye is not in very good shape.  Nothing we didn't know already, but he did confirm the ROP did no internal damage (only damage to the periphery where it is expected). He also said that he expects Cole to see progress in his vision up until the age of 15 years old.  That was really good news.  He thinks his vision is probably closer to 20:300 (improving from the 20:500 or 20:800 we've heard before).  He said that Cole has Periodic Alternating Nystagmus and therefore he recessed all four muscles during the surgery.  The testing showed that he looks at something straight on until his eyes get tired, then he tilts his head to the right to get a different null point until he tires and then tilts his head to the left for yet another null point.

We talked about the cause of his low vision and Dr. Hertle said all his limitations are not caused by the nystagmus.  He said the majority is due to the developmental disruption from premature birth.  Some is related to the bleed and pale optic nerves.  Again, nothing new but good to have confirmation from a second source.  Unfortunately that damage cannot be reverse and the vision will not be regained.  He did say there are medications we should consider and that contacts are very helpful for children with nystagmus.  We will talk more about these in the appointment tomorrow.  There is also a risk that about 20% of patients end up needing a second surgery...we will also ask that question tomorrow as far as when we will know.

We were taken back to see Cole about 15 minutes later and he was moaning as we walked into the recovery room.  He was unconsolable as he was coming out of anesthesia so they gave him more morphine.  He was still in a lot of pain and wouldn't open his eyes so it was hard to see if he was fully waking up.  I was able to get on the stretcher so he could lay on my chest but continued to moan until the meds finally kicked in and he fell asleep.  We let him sleep for about 30 minutes and then woke him up to get back to the Ronald McDonald House to rest.  Unfortunately he was still in a lot of pain.  We called for Security to drive us back to the house and filled his prescription before we left.  He moaned the entire way back to the house and rested on my chest once we got there.  Unfortunately his eyes are swollen, the whites are dark red, the pupils are very large and he was bleeding from his eyes.  He hasn't really opened his eyes and when he sits up he immediately grabs his head in pain.  So we're taking it easy relaxing in the room, ordered pizza and Cole convinced dad to go to the hospital gift shop and get him the super hero figure he wanted.  I redosed his Tylenol and later his hydrocodone and he seems to be feeling much better.  He and dad have been playing with Batman and Robin for the last half hour.  We meet with the doctor tomorrow at 9am and will then head back to VA.  More tomorrow night with next steps!

Nystagmus Surgery Trip Day 2 ~ Testing and Pre-Op

If I had to sum up today in one word it would be "exhausted."  Wow.

It was a long day but Cole was a champ and held it together until we walked out of the hospital.  We arrived at our appointment 15 minutes early as requested, at 9:45am.  We filled out some paperwork and were able to meet with the insurance coordinator.  There is quite a bit that we will need to cover, but we have no doubt it's worth the investment.  We waited about 40 minutes and were finally taken back to answer questions about history and symptoms.  The nurse performed the standard vision tests requiring Cole to look at images on a screen and she continued to decrease the size until he was unable to read them.

Dr. Hertle then came in and did similar tests and talked to cole for a little bit asking him questions and if he knew why he was there to meet him.  We was wonderful with Cole and answered all our questions.  It was a little heartbreaking to hear him say, "I don't need formal testing to see that he is struggling." Apparently he categorizes his nystagmus surgery into 9 different sub categories depending on the results from the intense testing.  After his initial exam he sent us off to see several different technicians to have those tests completed.

Just a reminder - you can click on any of the images to see them larger...

Eye Movement Recording (EMR)
The first test was an EMR.  This test is a high speed recording of the movement of the eye consisting of 500 pictures per second.  The recording documents the waveform characteristics, amplitude, direction and frequency of the eye movements.  The test diagnoses the type of nystagmus Cole has and determines the surgical approach.  Cole had to sit in a chair (on my lap) and I had to hold his head still.  He watched as images were displayed on a screen.  The images (Bert, Ernie, Dora) probably switched every 5 seconds and this continued for probably 10 minutes.  By the time this test was done it was almost noon and they gave us a $15 voucher and suggested we go get lunch and come back.

Color Differentiation
After lunch Cole's first test was to take about 12 different color dots and line them up in order.  Although this sounds easy, he had to match each color with the next closest color dot.  He also had to have a patch over one eye and then repeat the test moving the patch to the other eye. This was testing his ability to differentiate the colors which ranged from blue to orange.

The next test included another eye chart on the wall.  He had to look at two stacked dots and indicate which one of the two had lines in the circle. The lines were of differing clarity. The first test he was able to stand right next to the screen.  Then he had to sit across the room and repeat the test.  When he was close he was 100% accurate.  When he moved back he was not able to see even one accurately.

Visual Evoked Potential (VEP) Test
The last test was REALLY taxing for Cole.  The VEP is also painless and checks the function of the rods and cones.  It involves three electrodes placed on the back of the head, one on the forehead and a patch over one eye (and then the other).  He was asked to sit still, back against the chair and stare at a red dot on a screen while the background changed.  It would change from tiny yellow and black moving checkerboard changing into varying sizes.  Very mesmerizing and hard to explain.  He wasn't able to see the red dot so they hung a frog from a wire in front of the computer screen.

He had to stare at the frog until the screen went black.  I would say the timer was probably set to 30 seconds at a time and he probably did this for 30 minutes (15 minutes on each eye) including time to switch the patch.  By the end you could tell it was getting increasingly difficult and he kept leaning forward and tilting his head to see better.  The challenge was the machine had to read his eyes so if he looked away we had to start that test over again.  Keeping his attention on the frog (who never moved) was really hard.

Dilation
When we finally finished the VEP they dilated his eyes and we waited for the medication to take effect.  Then we were taken back in to see Dr. Hertle.  Unfortunately he said it's really hard to see into his pupils since the nystagmus causes him to move.  He said he will get a much better view once he is sedated.  He said that he definitely expects surgery to help Cole


The nurse called over to the Pre-OP department and let them know we were running late.  We finally wrapped up the testing at 2:45pm and ran across campus to meet with the surgical team.  There we were met by the Child Life Specialist who walked through all the tools Cole would be seeing the following day.  Explained the gown, the BP cuff, pulse ox and face mask.  He got to pick his flavor for the mask and picked pink lemonade AND orange.  We then met with a nurse practitioner, the surgical consultant and a technician.  Each spent some time getting history, getting diagnostics and height/weight etcetera.  We were SO nervous his cough was going to stop surgery, but after meeting with the team they said his lungs sounds good and his throat is clear so they don't see any reason anesthesia will not be safe.

Overall we had such an amazing team of professionals who met with us today. They all went above and beyond to really work with COLE to make sure he felt comfortable and had fun.  This last pic is the one that proves he had the biggest muscles in the room (as measured by the blood pressure cuff...which is really a muscle meter).  She was so much fun.

So after being at the hospital for 6 hours we headed to the car and decided to drive to find dinner since the choices in walking distance from the hospital were rather limited.  After a quick dinner and shopping trip for a new jersey for Cole, we headed back to the Ronald McDonald House to relax and gear up for surgery!  No food for Cole after midnight so it's going to be a LONG morning!  Arrival at the hospital at 12:15pm for a 1:30pm surgery time.  It's a go! Prayers can commence !  And thanks to all those at home who are helping watch over Reagan.  She's with nana and pop this week (Johnny's parents) and is having SO much fun!  

Nystagmus Surgery Trip Day 1 ~ Ronald McDonald House

After we put Reagan on the bus we finished up packing, did a little work and left VA by about 9:30am.  We decided to stop for breakfast at Bob Evans and then had a quick lunch on the way to Akron, OH at the Somerset stop on the Pennsylvania Turnpike.

It was a smooth drive and Cole stayed occupied with movies and games on his iPad.  We drove straight to the Ronald McDonald House to check in and get a tour of the house.  This house is VERY different from the one in Baltimore.  Although just like Baltimore, they are building a brand new facility directly behind the existing house.  So far everyone is super nice.  It's a much quieter house and not as many people, although it is fully booked this week.  The biggest benefit is that we can walk across the street to the hospital.  In 2014 this house served 12,872 individuals with 135 core volunteers each month.  They had 55 families who stayed over a month.  The families coming to this house were from 46 counties across Ohio, 23 different states and 8 countries.  So if you visit McDonald's this week please think about all these families who benefit from your support and put your extra change in the donation box.

After we got settled into our room and got a tour of the house we drove the car to the parking garage down the street and decided to walk into town for dinner.  As with many cities, there are areas with more activity and areas with less.  We found a few hip bars, but the dinner options were rather limited.  We were able to find a nice Irish restaurant, though, and had dinner.  On our walk back we decided to venture down by the lock system.  Although the Ohio & Erie Canal in Akron was abandoned in 1913 due to the increased popularity of the rail system, it still had much of its original facade in place.  The history said there were families living and working on these boats for 7 months of the year.  We talked about locking system, how it works, looked at the water fall and of course Cole had to throw some rocks in the water.

Then it was off to bed early for Cole so he's ready for tomorrow.  Unfortunately he has been up since about 3:50am when Reagan woke them both up, had them dressed, teeth brushed and beds made.  I woke to hearing their laughter and asked what was going on and Reagan said she was ready to go to Nana and Pop's house for her sleep over.  We asked them to go back to bed and I fell asleep only to wake to find them standing next to me at 5:30am asking if it was time yet.  Clearly they were excited (or nervous) about the adventure.

We are all looking forward to meeting Dr. Hertle tomorrow and learning more about what to expect on Thursday.  We asked Cole if he had any questions and he said he wanted to ask if there was a way to make the tools not hurt him.  Good question!  We told him yes but that we will be sure to cover that tomorrow.  We are hopeful that his cough doesn't preclude him from having surgery.  I brought him to the pediatrician Thursday and spoke with the surgical team at Akron Friday and everyone thought he would be fine.  I am still worried given we've had MRI's cancelled for Reagan due to a stuffy nose.  Any time someone has to be anesthetized there is a concern about the airway.  Luckily his throat is open and his lungs are clear.  The surgical team said as long as he doesn't have a fever we should be ok.  Praying they are right - we will find out tomorrow!

1st Day of School, New Piano, Retainer is IN and Akron Children's Here We Come!

First Day of School

Wow, it's already back to school time!  We had a great summer, but I think all of us were ready of the structure of the school year to return.  Reagan was very excited to start 2nd grade at Potowmack Elementary School in Mrs. Arthur's class.  Cole was excited to start Kindergarten in Mrs. Bretcko's class.  This will be Cole's 4th year at Potowmack so it's old hat for him, but he was excited to have three kids from preschool in his class.  Both have completed their first two weeks and are adjusting very well.  Cole is in full day kindergarten so his day is a little longer than last year.  He comes home a little tired by Friday afternoon, but is doing much better than I expected!  He has a calendar of homework and we are working at keeping up with it.  This is new for him and will take a little adjustment especially since some tasks like writing are very hard for him.

I'm not sure who stole our oldest child, but the little girl in her place is very excited about reading, doing science experiments and finishing her homework as soon as she gets home.  It is such a shift from last year and a pleasant surprise.  I am soaking in the excitement and encouraging it the best I can.  It's definitely hard to juggle them both doing homework at the same time, helping both and stopping Cole from chiming in on Reagan's homework (which he loves and does easily).  She is learning ordinal numbers and he keeps up easily.  I can already tell it is going to be a fun year!

Old Piano, New Purpose

Some of you may have heard we recently adopted a piano.  After going with me to Cole's music lessons Reagan decided she wanted to try taking piano lessons.  She is having a lot of fun and it has been a very positive experience.  Using her left hand is very hard, but she does her best and we are seeing improvement!  Her teacher, Jessica, is amazing and has just the perfect balance of being supportive and encouraging.  I'm so proud of Reagan.  Upon mentioning the lessons to a friend, she asked if we would be interested in inheriting her piano.  We were thrilled!  The piano is a 1954 Wurlitzer spinet and looks wonderful in our formal living room.  I've known my friend, Stephanie Bridgewater, since KINDERGARTEN!  The piano was one she learned to play on, as well as her father.  It was purchased by her grandmother who started playing after an accident playing on a hitching post (for horses).  The game involved standing on the rope (between the posts) and the kids would pull them tight and the person on the rope would go flying - she went flying and broken her arm so severely she needed surgery and it left a very prominent scar.  The doctors suggested she play piano to help strengthen her hand, and that is how she started generations of piano players!  When Stephanie shared her story it just felt like this was the perfect piano for Reagan.  I love the history and pray Reagan continues to see progress and loves to play.

Orthodontia Journey Begins

A year ago the dentist suggested we visit an orthodontist.  I called and they recommended we wait until after she was seven.  We started with a new dentist (after 40 years!), Dr. Kim and he also encouraged us to see an orthodontist.  We visited Wiger Orthodontics and the doctor said a palate expander would really help resolve her cross bite.  Unfortunately they said if we wait that her jaw bone could develop longer on one side.  Reagan was OVER THE MOON excited about this.  We had to wait one week to have the mold made and then two weeks for the retainer to be made. She asked almost daily when she was going to pick it up.  I was convinced that upon pickup she would change her tune.  Nope.  She picked glow-in-the-dark hot pink and as you can see in the picture she is still excited!  She picked it up September 7th, 2016 and has worn it consistently since we got it.  We have a key and "turn" it once or twice weekly.  She has already asked me to turn it.  Hmm...over achiever?  Perhaps :-)  Given we have a $1500 lifetime coverage on orthodontia work we opted to pay out of pocket at the low-low price of $1300 (instead of the $4300 that they would have billed insurance)!  Yikes, just the beginning of a very long journey.  We had a very serious conversation with Reagan about where to put the retainer and where NOT to put it...like on a lunch tray at school.  Reagan is very responsible, though, and I know she will take good care of it.  Apparently dogs love retainers.  We are hopeful the rule, "if it comes out, it goes in (the case)" will prevent it become victim to Shelby's toothy mouth (the dog's).


Baseball !
We went to a NY Yankees game at Camden Yards last weekend and I sighed as I thought about how normal life feels right now....in a good way.  I stress about the things other families stress about like retainers, the dog getting dirty and homework getting done.  Although Cole still has the occasional headaches and threw up the other night (all over the bed, pajamas, wall, and carpet), we explain them away and try to go back to living.  There are always stressors "behind the scenes" like ensuring his teacher knows how to set the accessibility settings during computer lab or even knows they exist.  I still stress about Reagan having a seizure - crazy we haven't raised her meds since KINDERGARTEN.  The neurologist seems to think her level is high enough but she has had a LOT of weight gain in two years.  Anyway, life is settling down and we are enjoying the feeling of not having so many doctor's appointments.


With that said, we are now gearing up for Cole's second eye surgery.  The surgery will be done at Akron Children's Hospital by a world renowned opthalmologist who specializes in nystagmus surgery.  For more information on the surgery feel free to watch a short video, although it is not for the faint of heart.  We will drive up Tuesday, September 20th and will return home the 23rd.  Day 1 is testing and surgical prep, Day 2 is surgery, and Day 3 is a final check up and discharge.

We are very thankful that Johnny's parents have offered to keep Reagan so she doesn't miss school.  We've been on a wait list for this surgery for almost a year and unfortunately were not able to get an appointment over the summer (that was our hope).  Reagan is SO excited about her sleep over and asks every day when she can start packing.  Cole on the other hand is starting to get nervous and ask a lot of questions. "Mom, will they take my eyeballs out of my head? Will my eyes get bigger?  Will they look different or be a different color? Who is doing it? Why do I have to have surgery? Do they use a drill in my head? How do they get into my eye?"  We are very upfront with him but it's hard for him to understand.  The vision he has is "normal" for him so when I asked if he has trouble seeing things he says no.  I've been in touch with several people on a Facebook group for Nystagmus and most don't know that their eyes shake or wiggle.  Anyway, we are gearing up mentally and praying that the doctor believes the surgery will help.  Only time will tell!  I will update once we get settled in at the Ronald McDonald House !