Flexibility and Freedom for Paxton
Paxton Williams is a happy, outspoken 10-year-old girl who likes playing games with her siblings Eli, Isaac, and Aila. She loves music, painting, beading, sparkles, and the color pink. Paxton also has spastic quadriplegic cerebral palsy, which affects all four of her limbs and her speech.
"Paxton had difficulty moving her arms and legs, not because she's weak but because her muscles are too tight and she can't control her movements very well," explains Dartmouth-Hitchcock pediatric neurosurgeon David F. Bauer M.D. Paxton's parents, Andrea and Jesse Williams, had been referred to Bauer to explore options for Paxton's treatment; possibilities included surgery or medication.
"[Surgery] involves cutting nerves in the lower spine that are responsible for muscle rigidity in the lower body," says Bauer. "But Paxton had spasticity in her tongue and vocal cords—which affected her speech—as well as in her arms and legs. I knew that she'd do really great with the baclofen pump."
The Baclofen Pump
Baclofen is a muscle relaxer and anti-spasticity medication that helps relieve tightness and muscle spasms caused by cerebral palsy and other medical conditions. When administered through an implanted pump, baclofen acts directly on the central nervous system.
The pump itself resembles a hockey puck, and is implanted in the abdominal cavity. Through a catheter the time-release pump infuses a highly concentrated baclofen solution directly into the spinal column.
Paxton was already on the pill form of baclofen, "but we weren't noticing any huge benefits from it," says Andrea Williams. Adds Bauer: "When you take the pill orally, very little of it goes to the brain, and a lot of people can't take high doses because of side effects. With the pump, we can bathe the nerve roots and spinal cord in baclofen; that's when patients can get great improvement in spasticity."
But between Paxton and her dad against insertion of the pump, and Andrea not knowing if Paxton would obtain any benefit from the surgery, Dr. Bauer had a suggestion. "I offered her a baclofen test dose, which involves injection of baclofen directly into the dural sac where the nerve roots and spinal cord are floating in spinal fluid. That way, they could all see what kind of benefit Paxton would get, and Paxton could make the decision whether she wanted the permanent implantable pump or not."
Andrea, who knew the frustration her daughter felt because of her spasticity, and the difficulties she and Jesse sometimes experienced while caring for her, made a decision. "We have always included Paxton's opinion, but I kind of overstepped her on this one. I said, 'Dr. Bauer has a point here. Let's at least do the trial.'"
On an early Thursday morning the Williams family went in for Paxton's trial dosing. After a quick 15-minute procedure, the results were clearly evident: "We've struggled her whole life to get her braces on because her feet were always extremely tight even when she was sleeping. But Paxton's foot was relaxed. "It was soft," Andrea recalls. "It didn't kick at me."
Within minutes Paxton was awake and ready for breakfast. "The first thing she did was to pick up the carton of milk and pour it into the cup." Andrea says. "She's never in her life been able to pour into a cup because her arm would always twitch out. Now, not only could she pour it, but she had control. So she would stop and start up again, stop and start up again. It was incredible." But it wasn't just her arms and legs that benefited from the baclofen dose. "She didn't stop talking that whole day because her tongue and jaw weren't tight, and the words were just flowing."
Home and Back Again
The effects of the medication from the trial dose usually last about eight hours, but for Paxton they lasted closer to 10. She went to bed that evening still feeling "high on how the day had gone," remembers Andrea. But the next morning Andrea found Paxton sobbing in her bed. "She said her legs were tight and she was so hoping it wouldn't come back. That was hard. But from that moment on we were on a countdown to the pump."
Five days later Andrea and Jesse were tracking the stages of Paxton's on-going surgery via a computer monitor in the Dartmouth-Hitchcock waiting area.
"The next morning Paxton was in a great mood and she was feeling really good. We had wonderful nurses in the PICU, and we went home four days after surgery," recalls Andrea. "When we went for her two-week follow up with Dr. Bauer, she was running down the hallway with her walker. It was amazing."
Since Paxton has recovered from her surgery, she is working on rehabilitation. As she achieves greater flexibility her muscle strength needs to increase to improve her coordination and control.
The pump also has to be refilled every three-to-four months, with a needle inserted through the skin directly into the pump. The refill procedure takes about 15 minutes and is done in the clinic. Andrea says she loves the pump, not only for what it's done for Paxton, but because recently when the family went on vacation "it was just pack the kids up and go." She didn't have to pack up medications, or worry about refrigeration. She didn't have to "remember or forget to give Paxton pills." She finds the quarterly refills are much more manageable.
Bauer agrees: "We are always trying to achieve the best result for the patient, and we can keep adjusting the flow at each clinic visit until we find the correct dose, the 'sweet spot' for that particular patient. Paxton is in the conditioning phase, so she'll notice more and more benefits the longer she has the pump because she's able to do more as she gets stronger." The hope, says Bauer, is that over time "we'll be able to get rid of as much spasticity as possible, so that Paxton can be as functional as she can be. I'd be happy if she can walk on her own and do all those activities of daily living that her parents are helping her with. And if she could do more than that I would be thrilled."
Andrea and Jesse believe Paxton's future is filled with possibilities. "Right now Paxton is determined to stop using her walker and use just her canes—her pink canes. Years down the road she's going to need to be as independent as possible, live on her own someday, get married and have children—the whole nine yards," Andrea says. "I want Paxton to live her dreams…and to just be happy."
For additional information about Pediatric Neurosurgery, visit the Pediatric Neurosurgery web site.