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.