Fall in New England

We have been very busy taking advantage of what autumn in Maine brings.  Mostly apple picking, several times a week.  Pretty much all of our food involves apples and pumpkins until the snow flies.

The boys all love apples and will eat them until they are full.  I have to watch them carefully when we are picking.

We finally had our visit for F's functional vision assessment with a Teacher of the Visually Impaired in mid-September.  While we are still waiting on the report, she told me that he seems to have a good field of vision with all four quadrants of vision functioning.  His acuity is great and he is compensating well with his remaining eye. They are not recommending direct therapeutic services at this time but wish to monitor him as his skills develop.

Both W and F have recently started walking, so life has gotten even crazier.  F has not had any troubles with perceiving where things are when he is walking.  We did have an incident at a play place where he fell a short distance to his left side and freaked out because he didn't see what was coming.  That was the first time in a while where that has happened.  He is our climber and has recently climbed up the five foot ladder into the swingset in the backyard.  I have a few more gray hairs this week.

We have had some significant issues with his eye rotating lately.  I have been able to take it out pretty easily with our handy-dandy suction cup/plunger and he is pretty good about it. In fact, F noticed it and pointed to his socket, then his eye, gesturing that he wanted me to put it back in.  We visit the ocularist again next week to make some adjustments, which will include trying to address the rotation issue.

B has taken to reciting a long list to people we meet.  "This is F, he had eye surgery.  Now he has a super hero eye.  This is W, he didn't have eye surgery.  I am B, I am three, and I call myself Big Brother B."  He is matter of fact about everything and we want to remain open about F and his history.

Some days are still difficult.  A lot of the time I just want this done with.  I know that this is something we will deal with for the rest of our life and I don't want to do it anymore.  F will always be monitored and we will always have to wonder if he will stay in remission.  He will always have only one eye.  Sometimes that is a difficult thing to face as a parent.  I imagine, someday, it will be difficult to face for F as well.  But for now, we do everything we can to make it as normal as possible and to keep him as healthy and happy as we can.

Early intervention...or how the heck do you give special education to an eight month old?

In my non-mommy life, I am a speech-language pathologist.  I graduated with my Masters in 2003 and began work with a pediatric center.  While in school, I always thought I wanted to work with older children in elementary or middle school, or even high school.  But I started working with preschoolers in my clinical fellowship year and suddenly became involved in early intervention.  For the next ten years or so, I worked primarily with 2-5 year olds, with some of my beloved elementary school aged children sprinkled in.  I became well versed in local early intervention and the services it provides.  In our state, Early Intervention is from birth to five years old (entering kindergarten).  In the past five years, they have divided these services into Part B and Part C so that the 0-3 set is under one set of criteria and 3-5 is under another.  Most states have 0-3 in Early Intervention and then their school systems get involved at age 3.

When we got F's diagnosis and knew he would have his eye removed, I immediately started the process for an evaluation. I did not want to "wait and see" if he developed issues following surgery.  He is high risk for motor development issues because he has monocular vision.  I wanted to meet with a team and get ideas of what I could do to help him with his gross and fine motor skills and get a baseline for his skills.  I emailed the Birth to Two Team Leader while he was in surgery. Sometimes it is good to have connections.

We waited for F to heal and recover and to complete some of our appointments. In the meantime, I forced F to use his left side as much as possible.  I sat his carseat in the backseat where he would have to turn his head to look out the window.  I sat on his left side when he was in his highchair and would place food on that side of his tray.  Within a few weeks of surgery, he was crawling and seemed to have no issues with left neglect, but I still wanted someone to look at him.

 A few weeks out, I contacted CDS to schedule to evaluation.  The intake case manager did not connect my name to who I was.  However, the OT that was sent out as part of the team remembered me from when we worked together.  They came at the beginning of May.  All of F's skills were well within the normal range, including his fine and gross motor skills.  He was compensating well with his functional eye and developing at a similar rate to his twin brother.  However, given that he was "at risk" for developing motor issues, they wrote an Individual Family Service Plan for us to have an Early Childhood Educator come to the house, watch his skill development, and give me ideas to help him every month.  They also recommended a Functional Vision Assessment by a Teacher of the Blind and Visually Impaired.  Assessments from the TBVIs run on a school year calendar, even for children under three, and our referral was a bit hung up, so we are still waiting on that assessment as I write.

Our ECE is someone I worked with a bit in my job.  She was very helpful in giving me things to watch for and activities to introduce to continue strengthening F's skills.  She had me play games with flashlights and changing lighting in the hallway to increase his visual awareness.  She showed me how to teach him to check walls and doorways to his left.  She helped me help him navigate stairs.  She and I know she is "front loading" me with information for the future.

F is pretty fearless.  He is our climber and has had no trouble navigating stairs, steps, or different types of flooring.  He has learned quickly how to compensate for his eye by turning his head or using his hands.

If I weren't a practitioner in the field and didn't have connections, I don't know that we would have gotten Early Intervention services.  But my advice to parents is to be proactive, learn the ins and outs of the system, and fight for what you believe you need.

Not a glass eye, exactly...

We get a lot of questions about F's eye.  I hope to address most of them here as best I can.

During his enucleation surgery, Dr. Mukai placed a silicone implant into the socket.  This takes the place of the eye.  He equated it to a rubber ball.  Typically, this is how prosthetic eyes are done nowadays.  They used to be entire balls that could be taken, leaving an empty socket.  As technology has developed, they have found that putting an implant in that a prosthesis fits over gives more natural movement and limits infection.  Dr. Mukai attached the muscles to this implant and then placed a conformer in before stitching the lid shut.  The conformer is a "place holder" for the prosthetic eye.  It is clear and has a few holes in it.  I found this out when F decided to rub it right out at meal time.  I freaked out.  I had not been brave enough to pull back his eyelid and look at what was in there.  He was having a tantrum, started rubbing his face, and *clink* there it was.

We met with our ocularist, Dr. K, about six weeks after the enucleation.  An ocularist is someone who makes prosthetic eyes.  There are not many of them in the country.  We have three that visit Maine and Dr. K is the one who is used by our opthamologist.  The boys immediately fell in love with him.  He explained to us that prosthesises are typically made with an impression.  This does not hurt but is a weird feeling.  With young children (F was 7.5 months at the time), they often will do this under anesthesia.  The last thing I wanted to do was put the poor kid under AGAIN if we didn't have to.  Dr. K was confident he could fit the eye without an impression but told us it would take extra visits to get it just right.  We were more than willing.  We left with a temporary prosthetic, which was stock but fit the measurements of his socket with a little grinding.

Prosthetic eyes are not made of glass.  They are typically made of an acrylic polymer.  They can be shaped to fit the opening.  They can be "built up" in areas or shaved down.  They are not round, as your eye opening is not round.  F's fits over his implant like a hard contact. When they are not sized correctly, they can rotate or fall out.  With our temporary eye, F got really good at rubbing it out when he was tantruming.  Amazingly, he will lay on our lap and let us put it back in.  It is really quite easy once you get the hang of it.  You just pull his upper lid up, slide it in, pull the lower lid over, and done.

After our first meeting, Dr. K started working on F's permanent prosthetic.  He tried several sizes in the socket and studied F's right eye and his brother's eyes.  He hand tinted the eye and experimented with the pupil and iris size. The results were amazing. 

It took a handful of visits and some work but he did a great job. We had some problems with rotation originally but he was able to fix them quite easily.

We don't take his eye out frequently. In fact, the more we can keep it in, the better. It reduces wear and tear. We have had to take it out to wash it when it gets particularly gross. We use a small plunger and he allows us to just like when we put it in.

I also want to answer a lot of the questions we get asked a lot.

• He can't see out of it and never will. Even if they make technology for prosthetic eyes to see, his brain won't adjust to understand vision with that eye.
• His eye will grow a little but much of your eye growth is in the first five years of your life. They can add a bit to the eye before he needs a new one.
• Yes, some children swallow the prosthetic. As long as there is no evidence of breathing troubles, they just instruct you to "let it pass."
• It does not float.
• He should be able to drive and do just about everything someone with two eyes can do. He can't be a fighter pilot or in the military. Everything else is fair game.

Some people hesitate to say things around him, like "it's all fun and games until someone loses an eye." It is just a saying. I don't let it bother me. In fact, there have been times where I have said "let's keep the eye we have, ok?"

At the beginning

I chose the name of this blog from something my oldest child told me. When his brother arrived home with a patch, he ran off to get some things, came back in the room, and told me "Mommy, we can be pirates TOGETHER!" I have tried to hold onto that through this journey. He didn't think anything of the patch, just showed his love for his family.

But back to the beginning. My oldest child, B, was born on the longest day of the year in 2010. I stayed home with him until he was six months old, then returned to my job as a speech-language pathologist part time. When he was 18 months, we decided to try for child #2.... And got identical twins! It was a huge surprise and hard to swallow. Initially, my first doctor thought we were having mono-mono twins, meaning there was no dividing membrane within the shared placenta. Big scary diagnosis, as it has a higher in utero mortality rate, likely extended bedrest, super early delivery, and high risk of complications. We later found out they were mono-di twins, which was still higher risk but much easier to manage.

 I delivered them at 37.5 weeks via c-section. F was born first, followed by W two minutes later. W spent a few hours in the NICU for observation with his breathing but we were all together by evening and discharged together several days later.

Life with twins and a toddler was hard. Recovering from my pregnancy and birth was hard. Adjusting to being a stay at home parent was hard. The boys were not good sleepers and slept either in bed with us or in their swings in our room for the first two months. The off set ticking of swings will haunt me to my death. I was so tired I would be delirious some days. I was battling really nasty depression and exhaustion.

Eventually, we got into a rhythm. We were able to leave the house each day and go to the library, go to play groups, go to the store. It was never easy, but I eventually got the hang of it. From the beginning, F was a fussy baby. W was feisty but he was able to go with the flow and be more independent. F was a Mama's boy and cried and whined frequently. I told my mom "I feel like I as missing something with him." 

At four months, I saw a white dot on his pupil. I was the ONLY one who saw it. My husband didn't see it, my mother didn't see it, the pediatrician couldn't see it even with an ophthalmoscope. But it bothered me. I saw it every time I nursed him or changed him, it was all I saw.

A month later, it started to change and grow and impact his iris. Other people noticed, but only when I pointed it out to them. I worried a lot and talked about it to the family. His pupil wasn't reacting like it should right before his six month pediatrician's appointment. 

When I brought it up, it sounded alarms to our nurse practitioner. He brought in our doctor and she didn't know what it was, but didn't like what she saw. The appointment was on a Thursday. On Monday, we were at the pediatric opthamologist. We were there for hours and they dilated his pupils and did an ultrasound on his eye. The poor kid was miserable and we both cried through most of it. At the end, the doctor told us he had bad news. There was some sort of mass in F's eye. He had little to no vision in his left eye. More than likely, it was cancer.

My heart sank. I didn't know what to do. We were overwhelmed enough with our three boys and now we were faced with the possibility of a terrifying diagnosis. 

They wanted us to go to Massachusetts Eye and Ear Infirmary to see Dr. Mukai, one of the country's only pediatric retinal specialists. We went down two days later. F was evaluated under anesthesia. We got more bad news. It was a tumor and a cyst that was taking up most of his eye. Dr. Mukai believed it was either retinoblastoma or an intraocular medulloepithelioma. Either way, his eye needed to be removed.

Let that sink in for a second...they needed to remove HIS EYE. 

If it was retinoblastoma, all the boys would have to be tested. It is sometimes hereditary and has a genetic component. It can sometimes spread and also puts you at higher risk for secondary cancers. If it was the IM, it would be a one in a million diagnosis. Go ahead and google it. By the second page, there are articles about animals with it, telling you how rare it is to find in humans.

A week later, we were back at MEEI for surgery. Enucleation is day surgery. We were there in the morning and left that evening. Seeing my baby bandaged in recovery was the worst experience of my life. The nurses had to pick me up off the floor. I hope that that day is the worst day of all our lives.

We brought him home the next day. The only pain meds they give is Tylenol every few hours. The first few days were awful. On top of F's surgery, my husband had viral conjunctivitis. Then B got bacterial conjunctivitis. It was several days until we were all together again.

F was immediately an entirely new kid. He did not seem in pain and was immediately happier. He had a huge bandage with packing for the first few days.

He started crawling just a few days later. We had to remove the dressing that weekend. I will be forever grateful for my mother doing that. He had a stitch in his eyelid for healing purposes. We were told we could patch it if we wanted but to leave it uncovered as much as possible.

The opthamologist took the stitch out in his office about a week after surgery. F's eye just appeared closed and slightly sunk in.

We got our results after about two weeks of waiting. We found put F had an intraocular medulloepithelioma, as Dr. Mukai had thought. In almost all cases, the tumor is contained in the eye and no follow up care is needed as long as the margins were clear. If he had tried to take a sample, it would have likely broken up and spread. We are so thankful he knew what to do. Several weeks later, we did find that the margins were clear but the tumor had a low level of malignancy.

F is being careful followed by the local Children's Cancer program. For now, we do regular MRIs to ensure no cancerous cells spread. 

His work with the occularist and early intervention will be in different posts.

It has been quite an ordeal for our family, but we are now starting to see the other side.  People frequently compliment us about how positive and strong we have been through this. What other choice do we have? We have three kids who need us.