Casey B.

This story recounts the experience of one caregiver who has a family member receiving Medtronic DBS Therapy to reduce the symptoms associated with dystonia.* Please bear in mind that the experiences are specific to this particular person.

Casey B. was a single mother running her own business when doctors diagnosed her daughter, Kamri, with primary dystonia.

The diagnosis was life-changing for Kamri, who soon couldn't sit, lie down, and eat on her own, but also for Casey. She instantly became a full-time caregiver. Except for the half days when Kamri attended school, she never strayed from her daughter’s side, forgoing breaks and vacations.

"I didn’t want to get away," says Casey. "I was with her, always."

Casey had been familiar with the symptoms of dystonia years before Kamri's diagnosis. Four of Kamri's cousins were living with severe dystonia, and other family members had demonstrated mild symptoms. Casey knew well how the disorder can affect people’s lives.

Medtronic DBS Therapy (deep brain stimulation) had been introduced as a possible treatment option for her nephew Riley, who also has dystonia. After several years of considering the benefits and risks, Riley decided to go forward with Medtronic DBS Therapy and received it about 1 month after Kamri's diagnosis.

His experience was a positive one, and it persuaded Casey to consider Medtronic DBS Therapy for Kamri as well.

Casey conducted her own research, and talked to several people who were receiving DBS Therapy or whose children were receiving DBS Therapy for Dystonia. Casey’s mother discovered an online support group and bulletin board, where Casey was able to further connect with other DBS Therapy recipients.

Many of them offered reassurance and advice.

"While I was very scared of the decision to have my child get brain surgery, I knew the potential it had to give her back her life," says Casey. "I felt in my heart that it was the right—and only—choice."

Kamri's neurologist also strongly suggested DBS Therapy, since Kamri's oral medications weren't sufficiently managing her symptoms. Though beneficial at first, Kamri's medications came with unpleasant side effects, and eventually they lost much of their effectiveness.

"We couldn’t increase the medication fast enough to catch up with her symptoms," says Casey.

During her surgery to place DBS Therapy, a medical team inserted leads in Kamri's brain.

Unfortunately, Kamri experienced some complications. Twelve hours after the procedure and while still recovering in the hospital, Kamri had a "dystonic crisis," during which her dystonia symptoms worsened. For a day and a half her body twisted like a pretzel, she was in severe pain, and she screamed out. Doctors attributed the crisis to the trauma of lead placement and the cessation of her medications the day of her surgery.

Kamri was sedated for 9 days to get her condition under control, but even then she would occasionally awaken and convulse.

During that time—about 5 days after her leads were placed—a surgical team placed a Medtronic neurostimulator below her collarbone.

Kamri experienced additional complications 6 weeks later, in August 2008, when she developed an infection, prompting the removal of the lead and neurostimulator from the left side of her body. Kamri's dystonia symptoms temporarily worsened until the leads were replaced in November.

Like Kamri, recipients of DBS Therapy may experience adverse events or side effects. Implanting a Medtronic neurostimulator to deliver DBS Therapy requires brain surgery. Risks of brain surgery may include serious complications such as coma, bleeding inside the brain, seizures, and infection. Some of these may be fatal.

Once implanted, the system may become infected, parts may wear through the skin, and the lead or lead/extension connector may move. DBS Therapy could stop suddenly because of mechanical or electrical problems. Any of these situations may require additional surgery or cause symptoms to return.

Stimulation may cause worsening of some motor symptoms associated with dystonia, and may cause speech and language impairments. Programming parameters can generally be adjusted to minimize side effects and attain maximum symptom control. In patients receiving Medtronic DBS Therapy, depression, suicidal thoughts and suicide have been reported.

Casey saw an improvement in Kamri 2 weeks after the neurostimulator was placed.

"I just saw signs," says Casey. "She was able to sit a little bit; she was able to lie down a little bit. Her mouth wasn't lopping open as much. You could slowly see little improvements."

After 4 separate adjustments, Kamri's programming settings were optimized to give her the greatest relief.

March 2009 was the turning point, says Casey.

"All of a sudden it was like a switch; it was like day and night," she says. "Once they got the settings right, it was like they turned the dystonia off."

Recently Kamri broke a contact in one of her leads on her left side. She knew something was wrong when she felt little electrical shocks in her chest, near the area where her neurostimulator was implanted. Her neurologist confirmed the breakage during an examination by doing a diagnostic check on Kamri's device. Since Kamri's symptoms haven’t returned, Casey doesn't plan to have the lead replaced, and the neurologist simply turned the lead off. Kamri continues to receive stimulation on her right side.

Throughout it all, Casey stayed positive. She found strength in her faith, and in her strong belief that everything she and Kamri were going through was temporary, she says.

Her family, who empathized with what she was going through, was a constant source of moral support, as was Kamri's neurologist.

"He's amazing," she says. "He's become a very good friend throughout all of this. He was always there."

Casey has kept a blog to chronicle her experience, and continues to be in contact with Internet support groups for dystonia and DBS Therapy. Through them, she and Kamri have met people across the country whose lives have been touched by the condition and the treatment.

To parents of children with dystonia, Casey says not to feel alone. "Even though they may feel like this is the worst thing, there are lots of children and parents that are going through this."

Now that the most challenging part of their experience is behind them, Casey says she can’t even begin to tell what it feels like. "To this day I still cry when I try to talk about it."

She is, however, very happy with having chosen DBS Therapy for Kamri. "I would do every bit of it all over again."

*Medtronic DBS Therapy received a Humanitarian Device Exemption from the US Food and Drug Administration (FDA) in April 2003 for primary dystonia including generalized and segmental dystonia, hemidystonia, and cervical dystonia. It is indicated for individuals 7 years of age or older as an aid in the management of chronic dystonia when symptoms of the disease are not adequately controlled by medications. The effectiveness of this device for this use has not been demonstrated.

Medtronic invited this caregiver to share her story candidly. Not everyone who receives Medtronic DBS Therapy will receive the same results as the patient in this story; some people may experience significant symptom relief from DBS Therapy, and others may experience minimal symptom relief. Talk to your doctor to determine if Medtronic DBS Therapy is right for you or someone in your care.

This therapy is not for everyone. Please consult your physician. A prescription is required. For a complete list of adverse events that have been associated with the therapy, please refer to Important Safety Information.

*Humanitarian Device: The effectiveness of this device for the treatment of dystonia has not been demonstrated.