Expansion & Setbacks

It’s been a few weeks longer than I would have liked to get everyone an update on how Emily is doing.   The short story is that she’s been amazing and completely unfazed by the whole expansion process.  But it hasn’t been without complication.  A pretty sizable hole has opened up in one of the incisions and this has prevented us from expanding on that side.  Without the ability to use both expanders we are pretty much guaranteed to have to go through another round of expansion.  I always knew that this was a possibility so I never let myself think that we would be able to get through it in one round.  Still, knowing that we have to do this all over again is a pretty hard pill to swallow. 

All said, we still have a lot to be thankful for.  First and foremost is Emily and her great personality.  She’s amazingly calm and just rolls with the punches. That makes it so much easier on us as parents.  The second is Emily’s plastic surgeon Dr. Bauer.  There’s no one else in the world with more experience with nevus removal, so we know with complete confidence that she is getting the best care possible.  The third is the other nevus families we have met.  They’ve provided wonderful support and perspective; especially now with the setback we’ve run into.  And last but not least we are thankful for all the support from our friends and family.  We are blessed with a great support system.

The expansion process itself has been pretty easy.  We’ve gone through five expansions total; one with Dr. Bauer's nurses and four at home, on our own.  We start with lidocaine over the expander port at least an hour before we plan to do the expansion; that numbs the skin so Emily doesn’t feel the needle.  An hour later we are ready to do the actual expansion.  First, we fill a 60 ml and 30 ml syringe with saline.  Then, clean off any excess lidocaine and clean & sterilize the port site before inserting the needle.  The lidocaine is wonderful stuff because she has yet to show any sign that she feels the needle. My job during this whole process is to hold Emily still and keep her occupied while RG does the actual expansion. After inserting the needle we fill the expander so that when you press and release the skin over the expander it returns to its normal color in under 3 seconds.  The most that we’ve been able to fill in a week is 65 ml, but we average 60 ml.  We are currently at 360 ml in the right expander.  The goal was 700ml, but there is a good chance that we won’t get there. Because of the hole, and the fact that it continues to grow, we will most likely not make it our scheduled January 18^th removal date.  We should hear from Dr. Bauer in the next couple of days as to how we will proceed from here. 

Below are some pictures of how things are progressing. 



Tools of the trade



200 ml





360 ml  Halfway to the goal.



Left expander hole.  We haven't expanded this since a week after surgery. 

Expanders

A happy presurgery Emily

This will be a short post for those of you interested in what the expanders looks like.  I tried to get the best camera angle so that the outlines of the expanders are visible.  Since they only have around 70 ml of saline in them they are tough to see in a photo.  That will change fairly quickly as we start the weekly fills.

As Amy mentioned before she has two 500 ml expanders.  One is on the left side of her back and the other is on the upper right side that angles to the middle of her side.  The positioning is important because during the expansion and removal process features may be pulled or pushed out of their original location creating a new visual difference.  In Emily’s case if an expander was placed on her front there may be a situation that during the process one of her breasts gets pulled down.  Then she would have to endure another surgery to put it back where it was supposed to be. 

There are also 2 drains that you will see that are there to remove the fluid that the body is sending to the area.  These will hopefully be removed by the end of the week when they are to longer needed. 

The pictures are below.  They aren’t bad, but you will see the sutures and drainage tubes coming out near the expanders.



 

 

Here you see both expanders on her back.  They are small now but will get very large. 
Especially relative to her small body

 

Right expander.  This one will start to restrict her arm after a couple weeks.

You can see a little more definition of the expander in this photo.
 

We also had a smaller satelite removed now because it only required an excision. 
This is the only satelite we are removing. 

One down...

I was right, those last six weeks went by way too quickly!  It wasn't until Tuesday of this week that we were positive that we would be cleared for surgery.  RG & Ryleigh had been sick with a cold and Emily had picked up the sniffles so we were unsure whether we would be able to proceed.  The pediatrician gave us the green light and we were good to go for surgery on Thursday.

Wednesday was Halloween and we had to meet with Dr. Bauer and his nurse at 3:00.  Since the appointment and the surgery were both on the far north side of Chicago we would be spending the night at RG's cousin's house in Northbrook.  Ryleigh would stay home with my parents to go trick-or-treating around our neighborhood.  To celebrate the holiday Emily wore her puppy costume into the Doctor’s office and they thought that she was just the cutest; I’d have to agree :) 

Dr. Bauer came in to have a look at Emily and discuss his plans.  He would be inserting two large tissue expanders, either 500 or 750 milliliters.  He would also try to excise the larger satellite on her left forearm.  It is currently the size of a nickel but would increase in size as she continues to grow.  He said that removal would be dependent on where they would have to put her IV, so it may or may not happen this time around. After Dr. Bauer left we sat with his nurse for about an hour to talk about the procedure and how we would need to care for her after the surgery. 

After leaving Dr. Bauer's office we decided to 'treat' ourselves to a nice dinner at Ruth's Chris, after all Thursday would be our 4th Anniversary.  I was a little apprehensive taking a 6 month old into such a nice restaurant, but she did great!  She slept the entire time.  While at dinner we got the call from the hospital to tell us that we would be the first surgery of the day and that we needed to be there at 6 am.

That night Emily did not want to go to sleep.  I don't know if it was being in a new place or if subconsciously she knew that something was coming.  I finally got her to sleep around 10.  Since she had gone to sleep so late I was hesitant to wake her up to give her a bottle at midnight, but since this would be her last bottle until after surgery I had to do it.  Good thing that I did, she took a full 8 ounces!  And fortunately she went right back to sleep. 

I tossed and turned the rest of the night and 5 am came all too quickly.  We left Emily asleep while we got ready and packed up our things.  By 5:30 we were in the car and on our way to the hospital. Once we got to the hospital there wasn't much to do but sit and wait.  A few nurses came in and took Emily's vitals, had us sign some forms and changed her into the smallest hospital gown that they had, that was still way too big for her.  Dr. Bauer and the anesthesiologist came in around 7:15 just to talk through the plans once more.  The anesthesiologist explained that they would first put Emily to sleep with sleeping gas and then they would insert and IV into her foot and do a caudal block in her lower back.  This is similar to an epidural and would numb her from the waist down.  This type of anesthesia would minimize the need for narcotics.

Around 7:30 the nurse came back to get us to take us to a pre-op holding area.  We waited here with Emily until close to 8:00.  Emily was bumped from the first spot of the day by a little girl who had a cleft pallet corrected the week before.  They were doing a quick procedure to remove her stitches.  Emily was in pretty good spirits this whole time.  I figured that once she was awake she would want a bottle and start to fuss.  Fortunately for us she never really did. The OR nurse came to get her around 8:00.  I had a knot in my stomach watching the nurse walk away with her, but I knew that she was in the best hands. 

 

RG and I decided to head to the Cafeteria for a bite to eat while we waited. I really wasn't in the mood to eat, but knew that it could be awhile before our next chance to eat.  After we ate we headed to the surgical waiting room where we passed the time by playing on our phones.  It wasn't long before the phone rang and it was for us.  RG spoke to the nurse and they said that she was in recovery and that everything had gone well.  Now we just had to wait for her to wake up before we would be allowed to go back and see her. This seemed to take FOREVER!  After 45 minutes Dr. Bauer came in and met with us to tell us that everything had gone well.  The only issue was the IV.  He said that half the time she was in the OR was spent trying to get the IV in.  They tried in both feet, both hands and finally ended up with it in her head :(  He said that he had inserted two 500 milliliter tissue expanders and had filled one to 65 milliliters and the other to 70.  Our first fill won't be until Friday the 9th back at Dr. Bauer's office. 

Not long after Dr. Bauer left they called and said that she was awake and we could come back.  We had to walk down a long corridor to get to the recovery room.  I could hear her screaming from the end of the hall.  When we entered the recovery room the nurse was sitting with her in a rocking chair and she was just screaming at the tops of her lungs.  The nurse quickly got up so that I could sit down and hold her to try and calm her down.  They covered the two of us with a few layers of blankets to try and warm her up. We tried giving her some milk, but she didn't want anything to do with it. After about 10 minutes of rocking I was able to calm her down and she was back to sleep. Those 10 minutes were probably the worst so far. It's so hard knowing that there is nothing that you can do to make them feel better. It wasn't long before the nurse told us that they would be moving us to a room. They brought a wheel chair for me to ride in with Emily and as soon as I stood up Emily started crying again.  Any slight movement would bring on a crying spell.  For the next hour and a half RG and I alternated sitting with her in the rocking chair trying to comfort her.  We were finally able to get her to take some milk; she had to be starving at this point since it had been at least 12 hours since she last ate. 

At this point we were still unsure if we were going to try and go home or if we would stay the night.  All we had done was sit in a rocking chair with her and feed her.  I was afraid to even move her, none the less change her diaper, any of her dressings and not to mention trying to put her into a carseat!  The nurse told us that we only had 2 hours, after which they would have to admit us. RG went to grab some lunch and the nurse came in again to check on us.  I ask her to help me change Emily’s diaper.  Emily was not happy about this at all!  We decided to change the dressing while we were at it.  It looked like one of the drains was draining into the drainage tube, but the other was just leaking out onto the dressings.  The nurse said that this can happen and it makes a mess, but isn’t anything to worry about.  Over the next hour we were able to feed her 6 more ounces so they were able to take her IV out.  Around this time RG and I decided that we should go home.  We would be much more comfortable there and there really wasn’t anything that the nurses were going to do for her that we couldn’t do ourselves.

We have been home for three days now and Emily has been improving a little more each day.  She has been sleeping for 5 – 6 hour stretches at night and napping regularly throughout the day.  We are staying on top of her medications alternating infant’s Tylenol and infant’s Motrin.  She is definitely sore and we have to be careful as to how we pick her up, but we are learning.  Our biggest issue so far has been that the antibiotic they have her on has given her some very explosive diapers!

Surgery ain't got nothing on me! - 2 days post surgery

 

A Long Time Coming

Well, here we are almost two months later and I have yet to update the blog. I knew that this would happen!

The last week of July I returned to work at SXC; except now it’s called Catamaran and is double the size it was when I left on maternity leave back in May. While I was gone they acquired a company named Catalyst; another similar sized PBM.  Since my return things have been incredibly busy.  On one hand it is great because it makes the days go by fast, but on the other hand I am worn out by the time I get home and get to see the girls.

I can’t believe how quickly those 12 weeks flew by!  And returning to work was extremely difficult for me; more so this time than it was when I came back after Ryleigh.  Don’t get me wrong there were definitely those moments while I was at home that I longed for some adult interaction or to run screaming in the other direction at the thought of another one of Ryleigh’s tantrums.  But all in all I loved being home with both of the girls.  It’s just amazing to me to watch them learn and grow before your eyes.  Can you believe that Emily is already 4 months old?!

Let me catch you up on some things that have happened in the last few months.  Over the 4^th of July weekend RG, Emily and I flew to Dallas, TX for the Nevus Outreach Conference. This is a conference that the Nevus Outreach Organization holds every two years for nevus owners and nevus families to come together to meet and to learn more about the condition.  RG and I went back and forth for about a month before committing to going.  Once we got there we were really happy that we made the decision to go.  We met so many great families; most of which had young children that were also born with CMN.  A lot of the kids that we met were closer in age to Ryleigh than they were to Emily but that didn't matter; no one can resist a baby!  She was a hit!

The organizers made sure to bring in some experts in the field.  There was a dermatologist, a neurologist and a sugeon. We took advantage of this and scheduled some time to meet with Dr. Bruce Bauer. He is well known doctor in the field of pediatric plastic & reconstructive surgery. Lucky for us he is right here in Chicago!  A lot of the families that choose to have their child's nevus removed travel from around the country to come to Dr. Bauer. The method of removal that Dr. Bauer uses is tissue expansion.  This is done by placing tissue expanders, think durable water balloons, under the skin near the nevus. Each week the balloons are exanded a little bit more by filling them with saline.  At the end of the expansion, 10 - 12 weeks later, the expanders are removed and the new, expanded skin is sewn together to replace the area where the nevus was. There are a few other options for removal as well. There is the option of laser removal and dermabrasion. Both of these options are used to remove layers of skin, lightening the nevus as they go. Since neither of these procedures are actually removing the nevus skin there is always a chance for the nevus to return and both would require multiple sessions. This would happen if any melanocytes that were missed during the procedures started to darken.  A third option is skin grafting. This option takes skin from another area on the body to replace the skin that is removed at the location of the nevus.  The downside to this is that the color and texture of the skin may not match, and the graft may not 'take' or may not be accepted by the new location. The location and size of the nevus will determine which procedure is best.

When we met with Dr. Bauer he took a look at Emily's large nevus and said that she was definitely a candidate for the expansion surgery.  He said that for the size and location of her nevus he hoped that they could remove the entire thing with one round.  A round includes two surgerys; the first to insert the tissue expanders under the skin and the second 10 to 12 weeks later to remove the nevus and bring the new skin together.  After meeting with Dr. Bauer and talking with all of the families at the conference we decided that this is the route that we wanted to go.

This wasn't a hard decision for me.  I just tried to put myself in her shoes as she gets older and thought that if it were me I would prefer a scar over the giant nevus. Some people may say that we are changing her by removing her nevus, but RG and I think that we are doing the best thing for her.

About a month after the conference I called Dr. Bauer's office to make the appointments for her surgeries.  The first will be Thursday, November 1st. I wanted to schedule the appointment as early as we could so that we could get it over with.  They won't do the surgery until she is 6 months old; the biggest concern being the anesthesia.  Dr. Bauer prefers to do these sugeries when they are younger for a couple of reasons, they are less likely to remember any of it and their skin is much more elastic at this age.  Her second surgery will be January 18th. 

Up until this point I hadn't thought twice about having her nevus removed, but once I scheduled the surgeries it all became very real.  I instantly started second guessing myself.  Are we really doing the right thing? Could I put her through this?  Would she make the same decision if it were up to her?  For now I'm trying not to think too much about it.  But I fear that these next six weeks are going to go by entirely to fast!  In the mean time we are taking full advantage of our weekends and spending lots of time with family and friends.

Happy Fall!!

Introduction

Hello everyone! For those of you who know me you are probably shocked at the fact that I am writing a blog. Believe me, I'm a little shocked myself! This is something that I never pictured myself doing. The reason that I'm writing a blog is to introduce you to our two beautiful girls and tell you Emily's story.

Some of your have already had the pleasure of meeting our newest addition, Emily Paige. For those of you who haven't I hope that you get to meet her soon or at least get to know her through this blog and our pictures. Emily was born on May 5th, 2012. (woohoo cinco de mayo!)

When Emily was born we were very shocked to see a LARGE dark spot on her right side (16 x 9cm). The doctor and the nurses were very good. They didn't act too surprised or whisk her away. They kept her in the room with us and cleaned her up while taking all of her vitals; just as they had done with Ryleigh when she was born. The doctor mentioned something about a 'small' birthmark. I knew that it was something more and was scared stiff.  It was obvious that they had never seen anything like this. I didn't say a word and just sat there with eyes wide open, squeezing RG's hand as tightly as I could. RG had to let go when they ask if we wanted to take a picture of her on the scale. Shortly after that they brought her over to me and layed her on my chest, skin to skin. I sat there, holding her, crying. I couldn't bring myself to look at the large spot. As I held her I began to notice more smaller spots all over her body. We would later find out that there were 30 smaller 'satellite' spots.

The doctor suggessted that a neonatologist come look at her. He was there within 15 minutes. He knew what it was, but through his broken English and doctor speak we weren't able to decipher what he was saying. All I heard was brain damage and seizures and that we would need to see a specialist at the Children's Hospital. That didn't really help me feel any better.

My mom and dad were in the waiting room awaiting Emily's arrival and to this point they knew nothing.  RG decided to go out and let them know that Emily had a pretty sizeable birthmark so I was a little out of sorts.  They came back shortly after and met our bundled up bundle of joy.  I was reluctant to show them, or anyone for that matter, her large spot until we knew exactly what it was.

Shortly after their visit we were moved from labor and delivery to our room. Once we were settled in our room I told RG to start 'Googling' giant birth marks. It wasn't long before he found the Nevus Outreach page (www.nevus.org) with a picture of a baby that had a birthmark almost identical to Emily's. It wasn't until the next day that we got confirmation from a dermatologist that Emily has a condition called Congenital Melanacytic Nevus. This basically means a mole or birthmark that is present at birth, similar to a mole that you or I might have, just super-sized. The dermatologist referred us to a pediatric dermatologist at Children's Hospital in Chicago. She also said that Emily would need an MRI to determine whether or not she had NCM (neuroceutaneous melanocytosis). This is where Melanocytes, the same cells that make up the moles on her skin, are present in the central nervous system. This could cause a whole host of issues such as hydrocephalus, unconsciousness, vomiting, and certain reflex and eyesight issues. And to think, up to this point I had just been concerned with her physical appearance. All of those things that the Neonatologist had said the day before were possible.  While this was incredibly frightening I tried not to think too much about it.  There was no reason to get all worked up until we knew for sure if she had NCM.    

Around the same time that the dermatologist stopped by so did RG's parents and my parents with Ryleigh.  Ryleigh was very excited to meet her baby sister.  I couldn't believe how big Ryleigh was next to Emily!  Up to this point she had been my baby.  Now she was a BIG girl and a big sister.  We had been preparing her for this day for months.  It took a week or two to get her to call Emily by her name instead of 'little sister.'  We didn't reveal the name to anyone prior to Emily's birth and since Ryleigh can't keep a secret we couldn't tell her either, so little sister it was.

So fast forward to day two and we are taking Emily to the NICU for sedation.  It was heart breaking seeing all of the tiny babies in their incubators. This made us feel very lucky knowing that we were able to hold our baby and that she would be coming home with us the next day. The nurse took her for the MRI and she was gone for almost 3 hours. RG & I used this time to read up on CMN and learned that it was not caused by anything that I did or didn't do during my pregnancy.  PHEW! This was a load off my shoulders since the thought had been running through my head since she was born. All I could think was that it was my fault and was caused by something that I had done. My pregnancy with Emily wasn't an easy one due to the fact that I wasn't able to eat many things such as whole grains, dairy and certain fruits and vegetables.  This made getting the necessary nutrients very difficult! I lived on peanut butter & banana sandwiches and graham crackers.  What I would have given for a glass of milk, cottage cheese and ice cream!   

After Emily awoke from sedation and was able to eat a little bit we were allowed to take her back to our room.  The nurse that was with Emily during the MRI let it slip that everything in the report looked clear, which was great news! We were given a CD with the MRI images to take to the dermatologist at Children's Hospital for further review.

By now it was about 6 pm and we were still scheduled to go home, but we had to wait for the hospital pediatrician to release Emily.  We were finally on the road to head home around 8:30 pm.  It was so nice to finally be headed home; both RG and I were getting stir crazy being in that tiny hospital room!  We were told that someone from the dermatologist's office at Children's Hospital would be contacting us within the next few days to set up an appointment. 

There are so many things about this condition that we have yet to learn but we are taking it day by day and are just happy to have our precious Emily home with us!  Since she doesn't seem to have CNM and is completely healthy other than her spots we seem to be pretty lucky.  I will do my best to update this blog as often as possible so that you are able to follow along with her progress.  Stay tuned!