This is the story of what it's like when a blissful Luddite, a man who has proudly never owned a cellphone, gets a smartphone that is connected wirelessly to tiny wires that go deep into his brain.
It's about the day Ed Bieber's brain went Bluetooth.
Bieber has spent his life teaching generations of Rockland's kids to love the outdoors, but Parkinson's disease forced him off the trail, to step aside from his Nature Place summer camp in Chestnut Ridge.
Thirteen years after being diagnosed with Parkinson's, Bieber just couldn't do what he used to.
He wanted the Parkinson's tremors to end. He wanted to stop losing his balance, stop falling.
He didn't want his head to shake uncontrollably. He hadn't realized, until watching a video of himself, how much his head shook when his medications began to wear off.
When he first met his neurologist, Dr. Fiona Gupta of North Jersey Brain and Spine Center, at a support-group meeting more than seven years ago, she had told him that there would come a time when surgery might be advisable to do what the medications could no longer do: keep those symptoms at bay.
On Columbus Day, that time came.
Surgery, surgery, followup
Bieber underwent a six-hour surgery, Deep Brain Stimulation, during which surgeon Dr. Hooman Azmi — using live images as Bieber lay inside an MRI — targeted an area of the brain where he later implanted two wires, about the diameter of angel-hair pasta, 3 inches into his brain.
The first signs will be slight, but there will also be one clear sign that the surgery has done nothing to diminish Bieber's spirit and sense of humor.
'It might be time'
Sitting in the waiting room before his appointment, Bieber says he knew that surgery was on the radar. Gupta had put it there, gently.
“She didn’t rush it, didn’t push it. She’d mention it each time I came," he says. "Last fall, she said it might be time.”
By the time of the surgery, the dopamine — which helps nerve cells make connections that have been lost to Parkinson's — was having less and less effect. He was taking 200 mg of dopamine every three hours and noticed that his "off" times were worse at night.
Parkinson's patients live with symptoms that change minute to minute, hour to hour. There are hours when they feel "on" — like they are firing on all cylinders — and hours when they feel "off" — rigid, stiff and in a bit of a fog. They are constantly gauging how well their medication is working.
Deep Brain Stimulation (known as DBS) is an option — for some of the 7 to 10 million people worldwide who have the progressive disease — when the medication's effects begin to diminish.
An important morning
Weeks after the surgery, Bieber has a long scar across the top of his head. His usually thick white hair, close-cropped for the surgery, is already starting to grow back.
In the exam room, sitting beside Jill, he taps his foot. There's no way to know if that's the Parkinson's or the tension of the moment. He says he didn't sleep much the night before.
He knows this is an important morning.
When Gupta arrives, she seeks to temper expectations. The GPI target doesn't produce the lightswitch-fast results seen when surgeons target the subthalamic nucleus, another part of the brain.
“Remember, this is a process," she tells the Biebers. "Today, we’re going to turn on the device to see how you do.”
Jill Bieber wonders how long it will be before it will kick in.
“It could be 24 to 72 hours,” Gupta says. “Some people take longer, some shorter.”
Gupta puts Bieber through a series of small tasks. She holds his hands out in front of him. Has him tap his thumb and index finger on his right hand, open and close his eyes. Stand up. Sit down.
She notes he hasn’t taken his medications this morning, but his tremors don’t seem to be too severe.
Gupta explains that the control device works wirelessly, via Bluetooth. She’ll program it using an iPad and Bieber will control it with an iPhone, which she’ll give him this morning.
“I’ve never had a cellphone before,” he says with a hint of resignation. “Now I can’t say that.”
Gupta primes the device, taking a magnet and placing it against the control device just under the skin. This is a one-time-only procedure, although the battery inside the controller will need replacing in a few years, requiring another surgery.
Then it’s time to turn it on, in a process that will send milliamps — thousandths of an amp — down those wires, to several contacts that will send electricity out into the basal ganglia.
Ed being Ed
Jill watches Bieber intently, eagerly, hoping to see a change, even though she’s been told not to expect fireworks.
Ok, I’m going to turn it on,” Gupta says.
The doctor touches his hands. She puts him through his paces again. Has him touch his thumb and index finger together, put his hands, palm-down, in his lap, then flip them over, open and close his hands.
“Feel any different?” she asks.
“No,” Bieber says.
Everyone is watching. Closely.
Gupta and Jill watch. A photographer and a reporter watch.
Everyone’s leaning in, hoping to detect the slightest change.
All of a sudden, Bieber raises his hands over his head wildly and gasps.
He’s joking. It’s Ed being Ed. It cuts through the tension of the moment.
“I had to,” he says, as his onlookers laugh.
Now that everyone can breathe, Gupta wants to know if he feels pins and needles, lightheadedness, asks him to tap his fingers together again.
No, he reports.
“Beautiful,” she says.
“It’s very subtle, and I don’t know if you saw when it was on, there was much more fluidity,” she says.
Before she turns on the right probe, which will affect Bieber's left side, she tests the fingers again. She then repeats the exam, looking for more subtle signs of change.
“There are about 20,000-plus parameters that can be configured into a DBS device,” Gupta says. "For the future, we have lots of settings stored and, in Mr. Bieber’s case, rather than increasing medication over time, we have the ability to further adjust the stimulator if we need to.”
“Parkinson’s is a disorder of circuits in the brain, one area of the brain is not connecting to another," Gupta says. "In his case, we’re trying to drive that circuit to a more normal setting.”
Today is about initial levels, about ensuring that the probes Azmi inserted into Bieber’s brain, were properly placed.
“In some cases, if the lede is not in the right place, as you amplify these settings, you’ll capture unintended elements of the brain," Gupta says. "You’ll see facial pulling and a lot of sensory stuff, but his ledes are perfectly placed.”
The Biebers laugh, another release of tension in a room full of the unknown.
Gupta asks him to repeat after her: “Today is a sunny day.”
“Today is a sunny day,” he echoes.
And it appears to be.
'You're on'
Gupta looks at Bieber’s hand, which appears to be injured; the fingers splay.
“It’s from falling and catching myself on that side,” Bieber says.
“He keeps falling,” Jill says. “He keeps getting reinjured.”
The doctor turns the signals higher, then notes that, when he taps thumb to index finger, the contact is more precise. She then turns both sides off and notes the contact is just a bit off.
She then turns both sides back on and watches Bieber walk out of the room and down the hall, his gait slow, catching a little mid-stride.
Bieber then takes his medications, which will take 30 minutes to kick in, during which the doctor shows him how the application works on the iPhone, how it shows his name and connects wirelessly to his implanted controller.
It shows where the stimulation is being sent, and its power (amplitude).
“We have lots of room to adjust, as we see how it affects your need for medication,” Gupta says. “This basically shows you that you’re on.”
When DBS is at work, Gupta says, patients like Bieber will notice that their medications last longer, they have more “on” time, they’re not wearing down or having “episodes where they just can’t move.”
The doctor immediately adjusts his medications. Instead of five doses every three hours, he'll take four doses every four hours.
MRI-guided surgery
In 2011, Azmi was the first on the East Coast to perform DBS surgery on a patient lying inside an MRI. He guides non-metallic ceramic stylettes to the precise area where the metal wires are later placed. (Ceramic stylettes aren't affected by the MRI's powerful magnet.)
The surgery has benefits over the more traditional approach, in which surgeons map the brain by listening to each area's unique electrical signature to find where to place the ledes.
In essence, it's the difference between listening and looking, but both approaches have their value, Azmi says.
In the mapping approach, the patient remains awake; during the MRI-guided procedure, the patient is under anesthesia.
When he first began doing the MRI-guided surgeries, Azmi says he would do one for every four traditional approach. Now, he says, patients are given the option, and most of his patients choose the MRI.
Azmi said patients are good candidates for the surgery within five years from the onset of Parkinson's.
Gupta says they shouldn’t wait too long.
"It’s heartbreaking when you see a patient being referred for DBS and they’re no longer a candidate."
Azmi and Gupta say good candidates are those for whom medication still works, but not as long as it had in the past. The electrical impulses replace the chemical ones, but if those connections are no longer working — if the Parkinson's has progressed too far — surgery won't be as effective.
"In Mr. Bieber’s case, he has Parkinson’s disease, which is responsive to medication, but since I’ve known him, over many years, the medication has become less effective," Gupta says.
"The effect when he’s on and functional and able to move better is becoming more limited. He has also developed dyskinesia, which are abnormal involuntary movements, and dystonia, severe neck spasm and pain and pulling. DBS is a good option to open up that window."
Azmi, the surgeon, says the goal of DBS is "to address the motor symptoms of Parkinson’s: tremors, the stiffness and rigidity that develops, the slowness, and get rid of the ups and downs that patients can feel when they’re on medication. The surgery can even that out."
But he is clear that there's only so much DBS can do.
"What we need for Parkinson’s is a cure," Azmi says. "This is excellent therapy, but still not a cure."
"On" days
For Bieber, an “on” day means he’s feeling normal, something he said he feels most often when he’s outdoors, a place of grace for him.
"Nature holds herself out, her treasures, and we walk about with our heads down, wondering why it’s so dark," he says, adding that he'll know an on-day when he feels it.
His wife, Jill agrees. “I guess so. It’s like good art. You know it when you see it.”
A lifelong hiker, Bieber has seen his gait change. He tends to shuffle his left foot along, a condition that has worsened over 13 years.
Gupta has noticed Parkinson's progression.
“I’ve seen him become a little more withdrawn over the past couple of years, maybe not going out as much in the summer," she says. "He’s busy, but I’d love to see him more active. I want him hiking and still very involved with the camp. I want him to be more comfortable.”
Part of the fine-tuning process is learning how to program just the right level of impulse to send, and to which contact on the wire in the patient’s brain.
“We’ve come a long way,” Gupta says. “We test different contacts at different amplitudes, and then we figure out the initial settings.”
Bieber's gait responds to medication and Gupta said she is hopeful that symptoms that respond to medication will likewise respond to DBS, a surgery that has been performed in the U.S. since 1997.
Azmi and Gupta have been doing DBS since 2008 and now perform about 50 to 60 cases per year.
'Therapy is on'
For Jill Bieber, the progression of her husband's Parkinson's has felt "like quite a laborious process.”
“It’s been quite a journey. It’s hard to see someone you love that’s so changed,” she says. It started with stiffness and very quickly after that it affected his walking.
"I’d say his major difficulty now is his legs stop working. Literally. He falls forward. That’s not Ed."
Bieber puts on his glasses to watch Gupta work the iPad.
This first visit, Gupta sets the level at 2 milliamps, two one-thousandths of an amp.
But Gupta doesn’t want Bieber to get caught up in numbers. It’s more about how the current is affecting his symptoms.
The app is called Patient Control and, on the smartphone controller, it gives Bieber limited control over the circuitry. Gupta’s programmer is more detailed. She can regulate the pulse width and frequencies.
The screen reads “therapy is on.”
Eventually, Gupta says, after he has learned how the device works and with her consultation, if Bieber is having a down day, he could raise the amps on the device.
She says the Biebers need to work as a team, to watch for symptoms and side effects. She suggests they keep a diary.
The doctor is optimistic that Bieber will be back hiking his beloved Harriman State Park before too long.
“He was a very good candidate going in, the surgery was done very well and now it’s about the programming and the meds."
"We’ll get him out there,” she says.
For more photos and video :
http://www.lohud.com/story/news/local/rockland/ramapo/2017/12/15/parkinsons-patient-deep-brain-stimulation/922440001/
No comments:
Post a Comment