Everyone Is Home!

One week ago. That is when Sam had his last surgery. Today, Sam, Nathan and Shelley made it home from Hobe Sound/Miami. Sam had is one-week post op visit with Dr. Tse (pronounced "see") on Monday morning. The Dr is very happy with his results and Sams recovery.

Note: Sam's eye/plastic surgeon's name is Dr. Tse. That is like having a dentist named "Dr. Clean" or a pulmonologist name "Dr. Air." Very appropriate if you ask me!

Anyway, a third surgery is being planned, but not for a while. There is no rush. It will be another cosmetic surgery, to lift the eyelid a bit more and to re-crease the seam where the first surgery took place. My guess is that, especially after reading the surgical report, that the first surgery was very traumatic to the lid. The neurofibroma was rather large and involved.

So for now, Sam is home. He goes back to school tomorrow but cannot participate in PE for a couple of weeks. We have to be careful about the sutures clean and to prevent any infection. No rough-housing with dad or Nate. Bummer.

I cannot say enough about how tough Sam is. Remember, he has been getting poked and proded by doctors, techs, assistants, nurses, mom, dad, you name it, since he was 3 months old. He wears a patch over his good eye every day to help keep the right eye functioning and he's been doing that for years! He never complains about being in any pain or discomfort or anything. It is amazing how tough he is.

Please keep Sam in your thoughts and prayers. We appreciate the support and kind words from everyone so far. Please keep them coming!

Post Op #2

Well, surgery number 2 (for the orbit/eyelid anyway) was a great success. Let's see. We arrived at Bascom-Palmer at 5:50am on Tuesday, October 27, 2009. By the time Sam was admitted and repeatedly checked over, he was in the surgical suite by about 8:30am.

Dr. Tse came out to see us around 10:00 to show us some great digital pictures and to explain what was done:

Sam had a portion of his right eyelid clipped out to remove some excess eyelid. Remember, five months ago Sam underwent a huge tumor debulking surgery. After that surgery was complete and he had healed, the eyelid was very floppy and loose. It was also blocking some of his vision. Tuesday's surgery was planned.

So a small incision was made up the eyelid. The lid was then pulled over to check for redundancy and a measurement was taken. From there, the surgeons removed the measured amount of eyelid and then closed the incision very delicately.

Sam came out of the surgery suite around 10:30 or so, drunk of course!!! I wish us adults could buy some of the stuff they use to knockout patients. Well, it looks like fun but I'm sure it is not, especially the hangover.

He had a rather larger pressure bandage on that gave him a fit. He did not like it and fought it at times; he actually grabbed it and tried to pull it off on the way home.

We were discharged from the hospital by noon. But first we had a great surprise. Daniel Graeff, one of the drivers from the FarnbacherLoles Racing team stopped by to cheer up Sam! Smiles were plentiful to say the least.

Sam met Daniel in July at the Brumos Porsche 250 race in Daytona. Daniel really took to Sam and lead him around, showed him the race car, explained things to Sam, and really got down to his level. So to have Daniel show up at the hospital, to take time out of his day and make Sam smile, that was the best. Those small things (as Judy Mader reminded me today) are what makes life so special.

Anyway, after a long two hour drive back to Hobe Sound, the day was nearing an end. Sam was uncomfortable to say the least. The patch was his nemesis. He did not like it at all.

That night he slept in the bed that Shelley and I typically share. I kept an eye on him through the night to make sure he didn't pull that patch off. In case any of you may not have had the experience of sleeping in the same bed as a five year old, here is a warning: they kick... a lot.

Today Sam was in a great mood. He knew the "bad patch" was coming off. So after being awake for, ah, 20 minutes, he demanded that the patch come off. That was hell! The tape is very strong and there was a lot of screaming, crying and kicking done by Sam. But when the patch was finally off, I showed it to him and everything just stopped. Wow.

Sam's wound looks great (for being a wound). The swelling is minimal and the discoloration is moderate. I'm sure it will change over the next few days but he'll be fine. He was even wearing his glasses later on.

I'm now back in Ocala. Sam has a follow-up appointment on Monday then they will come home on Tuesday, the 3rd. What a couple of weeks this has been.

We are very thankful for the care Sam receives. Dr. David Tse at Bascom-Palmer is just a part of Sam's total care. Sam sees at least three other specialists there and then about six specialists up at UAB. We travel a lot, but when you need the best for your child, money and time is no object. Just do it!

Fund Raising Update 1

Wow! In two weeks time friends and families have come up big as we hit 7% of our fund raising goal.

We are fully aware that these things take time and that times are tough for people to donate anything these days, but think about this:

Beer money

• If you had one beer at a bar, it could cost you $4 or more.


• Add a tip and just round it up to $5 per beer.


• Have three beers per visit and rack up a $15 tab.


• Maybe you really like the bar (or really don't like going home) and go there say three times per week.


• Now figure visits times cost per visit equals $45 per week.


• Over a year's time you'll spend $2,340 at this bar.


• Ten percent of that annual tab is $234.


• One percent of the annual tab is $23.40.

You can afford to donate one percent of an estimated bar tab.

Visit https://www.active.com/donate/racing4research2010/samcouillard and make it happen.

Fuel the Cure!

#85 CTF Porsche 911 - Daytona 2009

Way too cool watching the CTF car go by you at 150mph only 50 feet away.

Television cannot do racing justice.

Sam's Fund Raising Efforts

We will be in touch with some local (and I mean Ocala local) race teams to set up an event out at the Ocala Speedway, all 3/8 mile paved track.

The top car a few years ago was the Flying Tomato. It was an Orange Pinto.

Stay in tune.

2010 NF Heroes Page

Sam's 2010 NF Hero page is up. Please make a small contribution to this reseach fund raising campaign in Sam's honor. Every little bit counts and no donation is too small.

Thank you!
https://www.active.com/donate/racing4research2010/samcouillard

10 Days After...off to the RACES!

The Children's Tumor Foundation called and asked if we would be interested in heading over to Daytona to be a part of the CTF team as they enter the Brumos Porsche 250 Grand Am race. What did we have to do? Show up!

What a great experience for Sam (and not that shabby of one for me and Shelley either!). We heading over to the coast, dropped Nate off at Andrea's house for the day and then headed to the track. We walked... a lot. Then Michelle Marden with the Farbacher Loles team picked us up in a golf cart and off we went, right to the team's garage.

From there the CTF drivers (Daniel Graeff and Ron Yarab, Jr.) pretty much took over. They got down to Sam's level and just showed him everything about the car. Sam is a NF Hero and his name actually appears on the car (under the 8 on the passenger side). He got to sign his name on his name on a race car! How cool is that?!

We also met Lilly Ann Brooks, another NF Hero from Orlando. She is just great and she and Sam warmed up quickly.

After taking care of the car stuff, we headed to the Fan Zone to meet a Nationwide Series Nascar driver Ken Butler III who has a friend with NF. We were able to tour the Fan Zone for a while, scope out some Sprint Series drivers and garages. Sam even scored a lug nut from the #2 Miller Lite car!

The next day we rushed back to the track and spent some time in the Fan Zone again, walking around, seeing sights. It was hot so we headed back to the stands and caught the start of the Grand Am race.

It was really cool to see the CTF car, the one Sam sat in, signed his name, met the drivers, all that stuff the day before, zipping around the track. What a day. What a weekend.

Surgery

I don't think that people really realize how much family members change over a period of time since you are with them all of the time.

Sam is now five years old. Over the past four years we have seen him just grow, both physically and mentally. We know that is what is supposed to happen but to see four years pass by, while a child of yours is growing, that is an amazing experience for us.

We monitored Sam's NF over the years and like most of the research and care guides we have read, watching and monitoring is about the best thing to do for someone of Sam's age. It paid off for Sam a month ago.

During a scheduled visit to UAB one of the team doctors suggested that Sam have a surgery to help debulk the plexiform over his right orbit. The procedure seemed kind of extensive so of course we wanted a second opinion (always get a second opinion). The doctor at UAB gave us a name of a doctor at the Mayo Clinic in Jacksonville. We all know how good of care people receive there, but it is out of network for our insurance so we are talking some serious money out of our pockets.

We turned instead, back to a doctor who saw Sam when he was less than one year old. Dr. Tse at Bascom-Palmer saw Sam and agreed on two, or maybe three, surgeries. The first one would be to debulk the tumor above his orbit. He would make an incision along the crease/fold of Sam's eyelid, retract the skin and then start the debulking.

Plexiformneurofibromas bleed a lot. So as it was explained to us, Dr. Tse would have to cut the tumor, the cauterize it, then cut, the cauterize, and so on.

On May 16th, 2009 Sam had this surgery. This was his second major surgery (the first being a surgery to relieve the pressure in his eye). Sam was in the operating room for just over 2 1/2 hours and came out with a patch over his eye and a smile. WOW. We were all there waiting for him to come out.

Before Sam came out Dr. Tse came out to talk with us and he showed us digital pictures that he took during and after the procedure. We saw the mass he cut out. It was huge! We also saw pictures of Sam during the operation; that was a little too much for Shelley. I was amazed to see the photos. Dr. Tse told us about the procedure and how well Sam did.

After Sam recovered, we were released from the hospital. This was in out-patient procedure and we were back in Hobe Sound by 5:00pm. Wow. 12 hours and what a day.

The next morning we were allowed to take off the bandages. Sam looked like he lost a serious boxing match. His eye area was very swollen and purple.

Over the next few weeks we were amazed. The majority of the swelling has gone away and Sam's eyelid is really opening up. I think that everyday it gets better. The first picture in this post was taken earlier this year. The second picture was taken last week. I can tell you now, it looks even better. The healing is just about over from this surgery. Next week Sam heads back down to see Dr. Tse for a follow up visit. I'll put money on it that Dr. Tse will want to schedule the next surgery (to remove some of the eyelid to tighten it up a bit) sooner than later.

Any takers?

After seeing a great team, a great team, of doctors at UAB we realized that we were in the right place. We were ready to throw in the towel on all of the other doctors and clinics here in Florida.

That is how good we feel about the team approach. All of the books and handouts and articles and web sites we read how important having a good team of doctors to guide your child's care through. At UAB we had that team...and they communicate between themselves rather consistently about the care of the patient.

Before we started going there we tried to assemble our own team, but everyone was working in other locations, other hospitals, perhaps with different missions. Yet at each clinic or location, the small group of eye care physicians spoke amongst their own team. Linking the same types of doctors together under one roof made a big difference and I think we felt more comfortable knowing that what we were doing was the right thing to do.

Sam's Eye

Sam's right eye is proptotic, meaning it protrudes outward a bit. His eye is also lower than his left eye so it is a down and out location relative to the socket and other eye.

We had believed that the tumor mass behind his eye, or on top of the orbit, was pushing his eye out and straining the optic nerve. I am sure this is happening at some level, but not at the level of danger we thought before (at the date of this writing, Sam had his debulking surgery and optic nerve photos were taken, we haven't heard back from the doctors). We learned that Sam had glaucoma. His internal eyeball pressures had always been monitored but it was experts who saw that Sam's eye was deformed by the pressures related to glaucoma.

In children (toddlers) glaucoma is not so easy to figure; toddlers have a hard time of telling someone that their eye hurts. Sam never really (ever) complained about his eye or eye care. Anyway, instead of being able to tell someone about a specific pain or pressure sensation, a toddler's eye may deform from the pressure. If that happens, the shape of the eye will/may not return to the correct shape. Sam had surgery to relieve the eye pressure back in 2006 or 2007. The surgery was a huge success and we have been told that we "are managing Sam's pressures effectively." Good news on that.

Sam's eye is not in the normal eye location and the reshaping of his eyeball leads it to appear further out than it is.

A deep side effect from NF1 is bone deformation. Sam is missing a shpenoid bone behind his right eye. That is a very fine bone which separates the eye from the brain. That is another issue we are aware of and the care for this condition isn't really expected until later in Sam's life, if he really needs it. We pay more attention to the masses, where they have grown, or tea stains that we never noticed before. The sphenoid may be replaced with a bone graft after his teen-related growth spurts; like wait until the bigger growth spurts are over, then do surgeries, etc.

I am constantly reminded about how good of a patient Sam really is. Even with us at home. He can be as difficult as any other 5 year old; not doing what he doesn't want to do, talking back a bit, whining. But when it comes to caring for that eye or discussing surgery/what we are going to do next, Sam is a stud. He listens. He sits still. He lets you poke and prod areas very close to his eye and won't flinch. He'll even come up with crazy stories while you're examining him.

Over the years he as grown from 18" tall to 36" plus tall. He's doubled his size in five years so that is one heck of a growth spurt. Shelley and I feel that we may have made it through an opportunistic period for plexiform growth, but we'll see.

Sam's First Care

Sam's health care was really a wonder to us. NF is not a well known disorder so finding specialists was a struggle. Every book we read told us to develop a team of specialists; neurologists, opthamologists, pediatric neurosurgeons, pediatric occuloplastic specialists and so on. There is no one like that here in Ocala, that is for sure.

So our closest place was Shands @ UF. We found some great eye specialists there. Dr. Kuddus was one of the first to see Sam. From there we saw other doctors at Shands. Eventually that is where we went through some genetic counseling and had blood drawn from Sam which was sent off to UAB. We should have seen that back then that UAB was the place to go.

We were then made aware of the Bascom-Palmer Eye Institute and Jackson Memorial Hospital and Miami Children's Hospital. So our next round of "outings" was down to Miami. We met Dr. Tse there while we were looking for a second opinion about a surgery, one that a doctor at Shands said he "had done a few." We were very skeptical about that surgeon. A "few" of any surgeries to not enough for our son.

We met several doctors in Miami and fell in to the care of a glaucoma specialist. Sam's orbit pressures were followed closely. I think when Sam was 3 he had a surgery to relieve his high eye pressures. We later learned that his deformed eyeball was due to the glaucoma, not any tumors. Personally I think we waited too long for his first surgery but hindsight...blah blah.

I can't remember how we learned about the NF Clinic at UAB. It may have been through Grandma's efforts and tireless letter writing. We eventually made it to Birmingham and fell in love with the team care. When we say team, I mean team.

Some of the worlds leading NF researchers are at UAB. There are nine NF Clinics in the US and UAB is the hub for the southeastern region. For us, it takes about 8 hours to get from Ocala to Birmingham and we go there about every 6 months or so. Their follow up is amazing.

Besides driving through Georgia I don't mind the trip. Sometimes our stay in Birmingham is too short so the driving there and back is a hassle. Cost is another issue but whatever it takes.

A Little History...

Sam was three months old when we first heard the word "tumor" from a local opthamologist. I can remember the day like it was this morning. It was terrible. A routine 90 visit with our pediatrician turned into a day of worry, panic and unknowns.

We got Sam up to Shands at UF to start seeing specialists eventually heard the term "NF" and then "NF1."

The internet can be very helpful and hurtful as you tend to learn a lot that may or may not apply to you. Regardless, we take it all in and then try to throw out what doesn't make sense. Unfortunately, it was all making sense. Sam had some of the primary markers for NF1, but not quite enough for us to know for sure.

Genetic testing proved everything. Sam has NF1.

This is from the Children's Tumor Foudation (www.ctf.org) website and I use it a lot when I describe what NF is:

"Neurofibromatosis 1 (NF1): also known as von Recklinghausen NF or Peripheral NF. Occurring in 1:3,000 births, web characterized by multiple cafe-au-lait spots and neurofibromas on or under the skin. Enlargement and deformation of bones and curvature of the spine (scoliosis) may also occur. Occasionally, tumors may develop in the brain, on cranial nerves, or on the spinal cord. About 50% of people with NF also have learning disabilities. "

Re-read that second sentence... 1 in 3,000 births.

Here is another CTF tidbit:

"The Neurofibromatoses are genetically-determined disorders which affect more than 100,000 Americans; this makes NF more prevalent than Cystic Fibrosis, hereditary Muscular Dystrophy, Huntington's Disease and Tay Sachs combined."

That is a lot of people with NF.

We feel that we were given Sam for a reason. Fortunately we have been able to afford the best healthcare for him. He is seen at Bascom-Palmer Eye Institute in Miami (The #1 Eye Center in the US) and we travel to Birmingham, AL to go to the NF Clinic at UAB. At UAB he sees a team, a real team, of doctors. They communicate with each other about Sam's care and we are frequently surprised to go to an appointment to hear the doctor say "I was just talking with Dr. Soandso about your son." For those of you had been in the care of multiple doctors, you know how good this feels.

That is it for now.

I'd like to journal more about Sam's progression of care, since the diagnosis. There are a lot of notes to read and edit.

I'll try to be shorted winded... right!

Just Ask

It is Neurofibromatosis. My son Sam has NF1.

Neurofibromas, the most common tumors in NF, are benign growths which typically develop on or just underneath the surface of the skin but may also occur in deeper areas of the body. Neurofibromas, which are composed of tissue from the nervous system (neuro) and fibrous tissue (fibroma), usually develop around puberty although they may appear at any age. The tumors are not contagious. Nodule-like surface tumors are known as dermal neurofibromas. Plexiform neurofibromas grow diffusely under the skin surface or in deeper areas of the body.

Sam was diagnosed with NF1 at the age of 9 months. Next week he turns 5 years old.

This blog will be an effort to help us journal his life.