Video: Sam's Story

By Hoda Kotb Correspondent
NBC News
updated 4/12/2009 6:07:25 PM ET 2009-04-12T22:07:25

There’s a little of an adult and a kid in Sam Gladen. Some say he’s one of a kind, precocious and creative.

Kent Gladen, father: He gets people’s attention and then he just starts feeding on it..

Debbie Gladen, mother: We’re always going, "Okay, now, Sam. Is this true or is this some elaborate fairy tale?"

But Sam says he’s just a kid—a Boy Scout who likes hiking, sailing, and playing football with his friends. But all of that changed drastically.

Sam Gladen's video diary: To anybody that’s watching this and doesn’t know what it’s like to have your childhood ripped away, it hurts.

Getting that childhood back would take Sam on a long, frustrating medical trail. And at the end, Sam would have to call on the adult in him, to face a very grown up and terrifying decision.

It began gradually in early 2007. Sam couldn’t figure out why he was limping or why his ankle was giving out on him.

Sam: I couldn’t stand flat or anything. My foot just kept, like, hitting loose gravel when there was no loose gravel.

But he didn’t tell anybody.

Debbie: He doesn’t complain. He buries a lot of things.

And when Sam’s parents Debbie and Kent Gladen finally noticed something, they didn’t think much of it.

Kent: He just was limping. You know? Kids limp for a billion reasons. Video: Sam's advice

They were more annoyed than concerned, they say, thinking Sam was being Sam— just goofing around. Sam's parents' attention was focused elsewhere. Debbie had learned that her mother was terminally ill.

Hoda Kotb, correspondent: During that time, did you ever reach out and say, “Hey, Mom, hey Dad, something’s funky” ?

Sam Gladen, 12 years old: My mom had enough going on with my Nana and that would be another medical problem in her life and she really didn’t need that.

But Debbie’s mother, in a prescient, cautioned her about her son.

Debbie: She looked at me and said "You need to take this serious. He is not goofing around with you. There’s something wrong." And that was the last conversation I had with her.

Her funeral was March 29, 2007.

Sam: I couldn’t even get out of the car, ‘cause like, my leg just wouldn’t work.

Kent: I was furious.

His parents thought Sam was acting out because of his grief. He’d been so close to his grandmother.

Debbie: At that point, it was easier just to say, “Okay. Fine. Sit in the car.”

Kent: Not one of my better moments in life.

Debbie: Not something we’re proud of.

Kent: But I was furious. Video: Being teased

Kotb: Did you miss the funeral?

Sam: I just missed them burying her. I made the limp out to the memorial service, and then I had to limp back to the car.

Kotb: How much pain were you in at that point?

Sam: Immense. It was almost unbearable.

Kotb: How would you describe it? How much did it hurt?

Sam: Go lay down in front of a train and you’ll figure it out.

In the weeks following the funeral, Sam needed crutches more and more. And it was now clear that there was something seriously wrong with his foot.

Kent: His foot’s now turning in, and his toes are curled up like he’d had a stroke.

Frantic now, his parents took him from doctor to doctor. A pediatrician, a podiatrist, and two orthopedic surgeons. Each said something different: It was Sam’s heel, his foot, his knee. Finally, they say, one of the orthopedic surgeons determined that it was all in Sam’s head—that he was still grieving over the loss of his grandmother.

Debbie: There was probably a shred of us that—that said, “Okay, maybe it is. Maybe our kid is crazy.” And that’s probably our biggest regret.

They had Sam in grief counseling, tried physical therapy, even massage therapy. Nothing helped.

And Sam was angry with just about everybody he knew the problem with his foot wasn’t all in his head.

Kotb: How ticked off were you?

Sam: Pretty ticked.

Kotb: How did you take out your anger?

Sam: At first, I chewed my lips to shreds. Then, I went to the brows.

Debbie: He was rubbing his eyebrows off with a pencil eraser

Kent: And he literally had no eyebrows.

And Sam was checking out of his life.

Myles, younger brother: He wasn’t as fun, he became mellow.

It scared his 9 year old brother Myles.

Myles: He’s always been the up and moving and want to meet everyone type of guy and it kind of was creepy because he wasn’t like that anymore.

The Gladens had to do something. They took Sam back to the doctor and demanded an MRI. This time, doctors got it right.

Sam’s problem was in fact in his head—but it was neurological—not psychological. Sam was diagnosed with a little known disease called dystonia.

Sam: I was like, “Not good,” that’s all that was going through my mind. I was like, “Oh, crap.”

Dystonia is a rare neurological disorder in which the brain sends incorrect signals to the muscles causing them to contract or spasm involuntarily.

In Sam’s case it was affecting the muscles in his foot. There is no cure, only treatment.

Kotb: So now you know what you have, did you talk to your parents like, “What are we gonna do?”

Sam: No, I didn’t really talk to them at that point. I was peeved at doctors and at my parents for not believing me that I did have something wrong.

Debbie: We sat him down and we apologized and we said, “You know, we are so sorry that we didn’t know. Because we hurt him so badly by not believing him.”

Kent: Not believing what he was saying to us.

Now it was online searches, and phone calls to find treatment for Sam. In November 2007, they were referred to Cook Children’s Medical Center in Ft. Worth and a neurologist, Dr. Fernando Acosta Jr., who told them just what they wanted to hear.

Dr. Acosta: I said, Give me a chance. Let me try and see if I can straighten it out. I think I can.

Dr. Acosta wanted to inject botox into Sam’s leg. Another doctor had already tried this but it didn’t work. What’s more, it hurt... really hurt.

Kent: Sam laid on his stomach, and I literally had to lay on top of him to hold him down to keep his legs still ‘cause it burned. It hurt so bad as he’s screaming, at the top of his lungs.

But this time the pain paid off. Dr. Acosta straightened out Sam’s foot enough to fit into a brace. That and physical therapy at least had Sam off crutches and back on his feet. But it was still an ordeal, as Sam described in his video diary.

Sam's video diary: This is my brace. It’s very stiff and hard to walk around in. And it hurts a lot.

It was just a temporary fix. Dr. Acosta expected the disease to spread, and the shots would no longer help. His estimate was, within 5 to 10 years Sam’s entire body would be twisted. Sam would be in his 20s facing life in a wheelchair.

Dr. Acosta says he wanted to intervene as soon as possible to prevent that.

So in early 2008, he proposed a different and frightening treatment—one that might have Sam walking without a brace, and stop the progression of the disease.

Dr. Acosta: It involves brain surgery. It’s called deep brain stimulation.

Debbie: I went “no way,” “not on my child.” This is my smart, articulate, creative child. And you’re not gonna mess with his brain.

12-year-old Sam Gladen was offered a treatment that might have him walking without his brace, and stop the dystonia from twisting his entire body.

But the treatment was terrifying. It was brain surgery.

Sam: And I was like, “What? Where are the cameras? You guys punking me or something? I thought I heard brain surgery.” And then when they said, “Yeah, you heard brain surgery.” (Sam makes a face)

Hoda Kotb, correspondent: You shut down.

Sam: Yeah.

The procedure is called deep brain stimulation. It involves drilling holes in the head and implanting electrodes into the part of the brain that controls muscle tone.

Sam’s type of dystonia, which is genetic, made him an ideal candidate for this treatment.

Kotb: So did you say, “What happens if this thing goes wrong?”

Sam: Not as much say it as scream it that night at dinner. I was like, “And what if he gets one of the numbers wrong and goes—“ (Sam makes crashing sound)

Video: The decision to undergo surgery

The risks were frightening: infection, hemorrage, stroke. Doctors would be navigating around parts of the brain that control vision, movement and strength. There was little room for error.

And deep brain stimulation is a new treatment for dystonia. Doctors have been using it for less than 10 years, and only a handful of children have had it. Little is known about the long term effects on their brains. What’s more, the outcome is uncertain.

Dr. Acosta: There’s a possibility that we’ll do all this, and it may not work. And so, it’s a big leap of faith, I mean, to go through with this.

But once mom saw the pain Sam endured during his botox injections, she changed her mind.

Debbie: I said, “I can’t do this to him every 90 days. I just can’t do it.”

Debbie and Kent agreed that surgery was the way to go.

But the final decision would be Sam’s.

Dr. Acosta: In our hospital, at Sam’s age, it’s called "assent." Sam has to agree to the surgery or we can’t do it.

Sam: It took me a good, solid three weeks to finally warm up to the idea. And then another month to actually sign the papers.

Sam's video diary: Just 3 days ago, I signed off on it. I checked the little box and I said “give it to me.” And it wasn’t easy. It was probably one of the hardest things I ever had to do.

Kotb: What made you decide to have brain surgery?

Sam: Basically, it was, “All right, I can keep getting the most painful injections you’ll ever have in your life, every other month, and taking medicines I can barely keep down, no matter how much food I eat with ‘em, or I can get an eight hour surgery, be done with it.”

Kotb: Did you ever look at the risks?

Sam: I chose to ignore them.

Sams' video diary: So in two weeks’ time I will be administered the deep brain stimulation therapy. It will take a total of 8 hours and for 5 of those hours, I will be awake.

Video: Staying awake during brain surgery May 28, 2008, on the day of Sam’s surgery, thoughts of Sam’s grandmother were with the family, as they said their goodbye.

Kotb: Morning of, what are you thinking about?

Sam: “Oh my God. This is so stupid of me. I am gonna regret this.”

Deborah Ferguson, of KXAS TV in Dallas, Ft. Worth, followed Sam’s story for six months. She and her camera crew were there with him that day.

Dr. John Honeycutt was Sam's neurosurgeon.

Dr. Honeycutt, neurosurgeon: It’s a big operation. And it takes a long time to do it.

He would be implanting electrodes in Sam’s brain. The electrodes receive electrical pulses from pacemaker like devices implanted in Sam’s chest. If it works, this stimulation would stop the brain from sending those incorrect signals to his muscles, which caused them to contract.

Sam would be sedated, but conscious during the surgery, so doctors could see how he responded.

Dr. Honeycutt: It’s a difficult operation to go through being awake. And so doing it with kids, that adds a whole new level to it.

He began by literally screwing this headframe into Sam’s skull. That headframe gives doctors the coordinates of Sam’s brain.

Sam: I hated that thing. The worst part was when they were screwing it into my skull. You know, just a whole bunch of pain.

The target was the size of an almond. A team of 3 doctors—Dr. Honeycutt, Dr. Acosta, and neurology medical director Dr. Warren Marx, spent an hour plotting the path the electrodes would take to reach that target.

Dr. Honeycutt: The most intense moment for me is the actually planning making sure we that we have the electrode exactly where we want it.

Outside the operating room, Sam’s family—his parents, aunt and uncle, and grandfather anxiously awaited updates.

At 11 a.m., three hours into the procedure, word came that the surgery itself was about to begin.

Debbie: They called and told us “it’s time” and the walls closed in on us.

Dr. Honeycutt opened Sam’s scalp and began drilling two holes in his head.

Sam's video diary: When they’re cutting my head open, I will be awake. When they’re parting my skull, I will be awake. When they’re probing to find the right spots, I will be awake. When they insert the wires, awake.

Sam would feel little pain, because the brain has no nerve endings. But there’s still the stress of it all.

Dr. Honeycutt: The sound is the really the disturbing thing. And you can’t muffle it. You can’t put a silencer on the drill.

With the two holes drilled in Sam’s head, doctors made one final, remarkable check.

This thin needle has a tiny recording device at it’s tip. Doctors lowered it towards the target in Sam’s brain.

Dr. Acosta: Listening to the brain helps us confirm that we’re going to the right place that the targeting is correct.

After targeting was confirmed, doctors were ready to implant the electrodes. It was the moment of truth.

Four hours since doctors began the deep brain stimulation surgery on Sam, Dr. Honeycutt finally implanted the electrodes. Dr. Acosta was looking to see how Sam would respond.

Video: The Gladens on brain surgery

Dr. Acosta asked Sam to relax his foot, and Sam did more than relax it.

Dr. Acosta: I don’t remember him moving it that easy (before).

It may not have looked like much, but it was monumental. The stimulator worked.

Dr. Acosta: It was relief—excitement—adrenaline rush. It’s the moment.

Dr. Honeycutt: It’s our fix, you know, to see them get better right away.

Walking on air he says, Dr. Acosta delivered the news to the family.

Dr. Acosta to Debbie: It couldn’t have gone better, it couldn’t have. And Sam’s just the best.

Hoda Kotb to Sam: I know you were sedated, but did it register in that moment that, “Oh, my God. I just moved my foot.”

Sam: Basically, I was Tom-Cruise-on-Oprah’s-couch happy.

By 4 p.m., it was all over, and time to see Sam.

Sam: All I could say was like, “Hi.” That’s all I could muster. But, like, in my head I was like, “Party, party.”

Kotb: So, you knew it was a success—

Sam: Oh, definitely.

Two days later, Sam left the hospital.

Sam: From day one I had awesome results.

Awesome is right. Sam’s response to the stimulators was unusually quick, his doctors say. It can take many months, even a year for the them to begin to have an effect.

Debbie: Every day was something new. He could walk a little further without the brace.

Kent: We went from the large brace that went up to his knee down to just one around his ankle.

In late July, two months after his surgery, Sam was riding his bike.

Sam: I can actually put my foot flat now. So it’s a lot easier.

By October, he no longer needed a brace, and was playing football.

Sam: They said football was like a three years mark. And I was like, “Forget that, I’ll go play right now.”

And in December, Sam got a scooter for Christmas and has been riding with his friends ever since.

Debbie: We were in tears because it was something we never thought we would get to see them do again.

For younger brother Myles, it’s back to the good old days.

Myles: I love being able to hang out with my brother. I basically gotten my old brother back.

Not all is perfect with Sam.

Kent: He’s not a graceful runner right now, by any stretch. But I just love when he does run. It’s not for long periods of time.

But compared to what was, and what could have been, no one is troubled at all. And, his gait may still change, says Dr. Acosta.

Dr. Acosta: I think his brain is still getting used to it, so it’s possible that in another year, you wouldn’t notice anything. I want to make him perfect. But I don’t know.

Sam will have the stimulators in him for the rest of his life. That means no contact sports, and staying away from metal detectors. He’ll need new surgery about every five years, to change the batteries in the generators in his chest.

Kotb: So now when you picture his future, what do you see Sam at 20 or 30 or 40?

Debbie: That we gave him a level playing field. We gave him a chance. He’s not gonna have his disability. My hope and my prayer is it’s a forgotten thing for him.

Kotb: For some people surgery does defines them. "I had X," you know what I mean?

Sam: If you start a conversation with, “Hi, my name is bla lah lah, and I had this surgery at—on this date,” you have no life. And you need to find a new hobby, buddy.

Sam says he once saw his future living in a wheelchair and motorized bed. Now he has much brighter plans.

Sam: 15 years old, being a normal kid. 20 years old—out of college, in New York and writing books.

Kotb: Writing books?

Sam: Yeah, I’m gonna get a penthouse that overlooks Central Park.

Sam's doctors stress there are many kinds of dystonia, and deep brain stimulation is not a treatment for all forms of the disorder.

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