Out Damn Spot!

We left sunny Arizona on Tuesday and tried to take the sunshine with us to Chicago...unfortunately, I picked up quite a nice cold having spent a few nights in a hospital and a couple nights up with Zac, who grew very uncomfortable as the week progressed.  He did not seem to sleep well and would wake up crying and then not want to go back to sleep.  I even skipped his last fill because he seemed to just be at his limit.

530cc of saline AND a new tooth!

What a wonderful trip!  I have to give props to Southwest Airlines who seemed to anticipate my every need even before I did...starting with....I had two bags, a pack n play and a car seat...my Gramma had 1 suitcase so naturally I thought I'd have to lie..."oh yes, two of those suitcases are my Gramma's and the pack n play, suitcase and car seat are mine..."   BUT to my surprise, after my Gramma told them the truth...that she only had one bag...they checked my FOUR ITEMS with NO PROBLEM!  Then, after praying I would sit by some nice, understanding people on the plane (and boy did God answer that one!  Thank you so much for sending me those wonderful people who helped me out SO MUCH!) and hoping Zac would be calm enough for 5 minutes for me to finish a coke - SW served my soft drink...with a lid!  These people are awesome!!

Okay so, on Wednesday, we paid a visit to Dr. Bauer's office to get our pre-op check-up.  While we were sitting in the waiting room, someone said...did you say tissue expander?  Dr. Bauer put his first tissue expander in my son 15 years ago....to fix his webbed fingers."  Holy cow!!  Really??  Another fabulous use for tissue expanders and yet another fantastical thing that Dr. Bauer does to help children!!
This morning, we got to the hospital at 6 a.m....well, really close to 6 a.m....I guess I didn't realize that you need to put an extra 15 minutes in your schedule to scrape the ice off of your windshield here in the Springtime!  We got to Highland Park Hospital and were greeted by some of the same nurses that we saw in January.  What a wonderful group of people they all were.  Dr. Dobryman, the anesthesiologist paid us a visit followed by Dr. Bauer.  This surgery was so different than the first one.  I was...well, I was EXCITED!  I had no hesitation whatsoever when I handed Zac over to them.  I knew he was in the very best hands and that those hands were guided by the grace of God.

I'm too sexy for my nevus...

We waited for about two hours before Zac came out of surgery and Dr. Bauer came out and sat with me while he explained to me that he was able to excise a great deal of nevus from the back, as well as the small satellite on Zac's left arm.  While I was talking to Dr. Bauer, I couldn't help but think about what a wonderful person he was.  Kind, compassionate, sincere how he personally has changed the lives of thousands of children.  He thinks that it may be possible that Zac may only need two full rounds to be able to remove the rest of his trunk nevus!  I thank God for Dr. Bauer and for helping me find my way to him.  And... I am looking forward to changing Zac's dressings tomorrow to see his progress!

Recuperating

Captain Happy Takes On A Nasty Infection!

Daddy and Captain Happy in his Superhero cape!

Wow - this week did not exactly go how I had planned (what else is new around here, huh?).  What was planned...getting ahead at work and packing for our trip to visit Dr. Bauer in Chicago next Tuesday to have our tissue expander removed on Thursday.  What was not planned???  A trip to Phoenix Children's Hospital for two nights for a round of IV antibiotics!!!

It started on Monday night when Zac spiked a fever.  Though he's had 2 fevers during this expansion process, this one felt like it was higher.  The next morning, Zac was still really hot and very cranky.  He was also whimpering like he was in pain with any sort of movement.  I refilled his normal antibiotic and gave him his first half-dose.  The next day, he was still running very hot and acting even more listless...his temp registered 102.5!  We made an appointment with the pediatrician's office - we needed a pre-surgical physical anyway.  The doctor ran a  CBC and found that Zac's White Blood Count was VERY high.  His recommendation was that Zac needed IV antibiotics.  I immediately called Dr. Bauer's office and spoke to nurse Susan thinking we may need to go to Chicago a little earlier than planned if the expander was infected.  Susan said that IV antibiotics were a good idea and it would help him prepare for the surgery.  She said Dr. Bauer did not want Zac poked or prodded any more than he already had been for the first test at the ped's office and that the hospital should call Dr. Bauer as soon as we got to the hospital BEFORE they attempted anything.

We grabbed our superhero cape and headed to the hospital.  Upon arrival, we had the admitting doctor call Dr. Bauer and we proceeded with trying to find a good vein to put the antibiotics in.  When I talked to Dr. Bauer (who I called on his personal cell phone - I LOVE THIS MAN) he also wanted me to see if I could withdraw any fluid from the port area since it was swollen and looked like it could be the source of the infection.....oh, happy, happy, joy, joy...lucky me!  Why me when I am surrounded by a plethora of medical staff??  Dr. Bauer said that I knew the port and expander better than anyone there...(a huge compliment but....)  So...I'll spare you the details but let me tell you that slipping in that needle under these circumstances when Zac was NOT numb from Emla cream and already screaming....with a host of medical professionals hovering over you = the worst stress of my life!

After spending almost 4 hours in a triage room smaller than my closet...after watching two "IV teams" try to find a place to stick my beautiful boy...we decided to put the needle for the antibiotics in his head.  So, for the third time that day...I watched my little precious guy being held down and stuck by nurses...at least I didn't have to do the holding this time.  I sure was ready to do the comforting though.  After getting his little antenna, we were herded to another room of about the same size to wait for a bed.

After his first round of antibiotics - already improving!

So, Zac got his first round of antibiotics in this tiny room.  After that, he hung out with me on a gurney and watched American Idol with me.  ***Lesson learned....when choosing a vein for antibiotics - just choose the one in the head.  It seems painful but it wasn't any more painful than the hands or feet and the bonus was he couldn't see it and therefore didn't mess with it***

It was coming up on 9 p.m. (we'd been at the hospital since 1:30 p.m.) and Daddy had to leave to go get Zoie when we were transferred to our new, shared room.  Oh, yeay for the 16 year old boy we were sharing a room with - I am sure he was thrilled to share the room with a cranky baby LOL.  The sides of the hospital crib were not working so Zac and I slept huddled up on a padded cot/couch type thing which was slightly sloped making it a little challenging to not roll off while I was sleeping.

After the dark comes the dawn!  We woke up from a horrible night of sleep - but Zac was feeling so much better!!  We had to spend the entire day at the hospital much to our surprise since we went in thinking we'd be there for 24 hours...only to find out we may be there until Saturday morning.  After 4 rounds of antibiotics, we got to leave this morning though and we are VERY anxious to go to Chicago and visit our Super Team to get this tissue expander removed.  We are even more excited to have four whole months before we have to do this process again even though we are entirely thankful for it and the fantastic team of doctors and nurses in Chicago we are blessed to have found.

Happy Again!  What a trooper!

What Spots?

You know how you always hear, "it's not what you say...it's how you say it"?  Well, I don't know that that phrase applies in my life right now...  After almost a year of living with a funky, rare disorder that I can barely pronounce...I still haven't come up with a way to answer the million-dollar question "why does he have those spots?" or "what is wrong with him".  Usually these questions come from children because, let's face it, they are the only souls brave enough to ask.  I even had one little girl ask me why he had so many spots and then report it to her mother a moment later as if the mother was the one who really wanted to know.  I had to laugh because after the little girl asked, Zoie said "it's his congenital nevus."  That's it...just that simple...she may as well have said, "my brother has two eyes".
I guess maybe I am over thinking it.  I want to properly explain it so that people are educated.  I don't want to use the words birth defect, disorder or Congenital Melanocytic Nevus.  I can barely even say Melanocytic Nevus and when I do...the deadpan look I get is almost comical.  So...I hesitate.  I stammer and struggle with my words.  I don't know...maybe..."it's a rare skin condition"..."they were having dalmatian awareness week in heaven when he was born"...."what spots?"...  Maybe its because when I look at him I don't even see spots.
When Zac was born, he had a few satellites, but over the past year, satellites seem to have exploded all over his legs.  He looks like he's been splattered with coffee.  Now that summer is here and he is a shorts-wearing little dude, everyone seems to be asking.  Why am I so unprepared to answer what should be a very easy question.  It's a tough situation when you want to act like it's nothing  - he is a healthy, happy little boy and I refuse to give anyone the impression that he is not...yet it is obvious something is different.  And seriously folks...he has 500cc of saline in his side and all you can focus on are some measly little spots!!  I guess I will just have to let my four-year old answer from now on.

1 Month

8 months

What the heck is the amygdala??

Well, this week has been a little tough.  We have gotten news that we have been praying we would not hear since the day Zac was born ..."we found a spot of melanosis in the brain area".  Hearing such a thing could only mean one thing...Zachary has NCM (neurocutaneous melanosis).  Before you go googling NCM to get one step ahead of me and 'try' to educate yourself on NCM - please don't.  The internet is not always your friend and in cases of  NCM, such a rare disorder, it most certainly is NOT your friend.

Let me tell you what I do know about NCM - and tell you in normal people terms what I understand it to be.  When a child is born with nevi - particularly those that are large or in Zac's case, giant, there is a small chance of them having the same melanosis 'inside' the body.  Sometimes it can be found in the brain; sometimes it can be found on the spinal cord.  What can happen if melanosis is found inside the body....well, lots of things.  Seizures, hydrocephalus, leptomeningeal melanosis, even death.  I have heard that mentioning that your child has been diagnosed with NCM to someone who understands the disease gets you one of those sighs...followed by a sympathetic glance.  NCM is something no one wants their child to have.  So...how did we get here?

When Zac was 2 1/2 months old, he had his first MRI to scan his head and back in search of things that don't belong there.  When the scan came back, we were told that though the scan looked clear, there was a spot that was clouded - most likely from Zac moving during the MRI... (hmmm....wasn't he asleep..?).  Our dermatologist did not think there was a cause for concern though.  Bruce and I were a little uneasy that we a) just put our son through an MRI and sedation  b) paid a huge amount of money and c) still didn't feel like we had results that were 100% since there was an unidentifiable portion.  A couple months later while visiting our dermatologist, I asked for a copy of the MRI.  There, in plain English (and trust me, most of the MRI was not in plain English) it talked about the unidentifiable spot and said that we should think about repeating the scan in 6 months because the unidentifiable area was located in the amygdala-the most common area for NCM!  Well...my Mommy alarm really went off then.  I had a gut feeling that we should take a closer look.

The wonderful thing about Nevus Outreach is that we were able to send them a copy of Zac's MRI and they forward it to Dr. James Barkovich.  Dr. Barkovich is an extremely gifted pediatric radiologist who specializes in detecting NCM.  After sending a copy of Zac's MRI, I received Dr. Barkovich's results yesterday to learn that Zac has a 3mm parenchymal melanosis on his right amygdala.

After reading this report, I went through every emotion you can think of...sadness, fear, confusion, panic...but after talking to many people, from my fellow Nevus moms to our pediatric neurologist, what I am finding is that melanosis on the amygdala is actually quite common.  The amygdala is evidently, one part of the brain that doesn't really serve a specific function and most NCM remains asymptomatic.  Zac's is very small (3mm) and this morning, our neurologist even went so far as to say he believes Zac is developmentally advanced.

What does this all mean?  It means we pray.  It means we hope.  It means we choose to stay positive that one day, the initials NCM won't stir up the worst kind of fear in nevi wearers.  Though I am not going to lie... this tiny little spot will always be in the back of my mind.  I am choosing to believe that Zachary's inside spot will remain asymptomatic and put my trust in God that he will hear our prayers and keep our little guy healthy.

Man-Boob

440cc = a D cup

Wow!  440cc and three more fills to go!  3 x 70cc = 650cc!  While it seems like it has been smooth sailing I must say that last night's fill was a little stressful.  First of all, after 45 minutes with the numbing cream on Zac's port, when I went to insert the needle, he pulled away and wimpered...somehow...I have no idea how...he was NOT numb!!  What to do?!?  Do I just go through with the expansion hoping the needle is only a little uncomfortable or do I try numbing him up again and waiting 45 more minutes? I decided on the latter and
numbed him up again.  After 45 more minutes, not only was he numb - he was asleep because it was now 9:00 p.m!  This was definitely the easiest expansion to do.  He didn't wake up at all and we were able to inject 75cc into the expander.

Unfortunately, the challenge did not end there.  Zac woke up and felt a little warm to me so we gave him a little Tylenol.  He slept well but when he woke up this morning, he was still warm so I took his temperature and it was 100.2!  Luckily, he did not have any other symptoms other than being just a little cranky.  During this process I have tried to stay very positive but honestly, with every cough, sneeze or cry I worry that there is a greater illness or infection to follow. The chance of infection is so great with a foreign object in his little body and I feel like the world's biggest germ-a-phobe as I wipe down everything with alcohol gel and Clorox wipes and don't even take him to the nursery at church for fear of random germs.  Last week Zoie got the flu and the first thing that came to my mind is 'how am I going to quarantine Zac?'.  

Well, after talking to our favorite nurses at Dr. Bauer's office, we have decided it is necessary to put him back on the antibiotics - just as a precaution...  ugh.  BUT...FOUR MORE WEEKS!!  Just three more fills and we go back to Chicago.  Then we can rest for four months until we have to do it all again.  (Sigh)

Thank you everyone for your kind words and recipes....keep 'em coming.  I will be compiling them until the end of March.