Seattle Children’s has the right mix of expertise to offer kidswith spastic cerebral palsy a path toward independence.
Since play is a child’s most important occupation, all of the post-surgery rehabilitation is done in the context of play – whether that’s learning to standup with straight legs, get dressed, or develop core muscle strength. Christine Laddusaw chose Seattle Children’s because of the quality and intensity of the inpatient rehabilitation offered here. “[Arabelle’s weakness after the surgery] didn’t last long, but it was hard. I would have been at a loss if we’d just been sent home afterthe surgery.”
More than anything else, 3-year-oldArabelle Laddusaw wants to run.
Her big brother, Richard, 15,promised that when Arabelle can run,he’ll teach her to play soccer. If it werejust a matter of will, she’d already beout on the field.
Arabelle is “very stubborn and verydetermined,” says her mom, ChristineLaddusaw. That’s a good thing, becausebefore she can run, or even stand up,Arabelle has to relearn how to movenow that her body’s perpetually rigidmuscles have finally relaxed, thanksto a complex surgical procedure atSeattle Children’s.
Arabelle was born with spastic cerebral palsy (CP), a condition thatmade the muscles of her hips and legsinflexible and hard to control. It’s asif the message center between herbrain and muscles was stuck in the “on” position.
Looking for better options
Spasticity makes it hard to isolatemovements, but Arabelle wasdetermined not to be left behind byher siblings. She learned to crawlby “bunny hopping,” drawing bothlegs up together. When she used herwalker, her feet would cross, trippingeach other up.
Although CP itself doesn’t worsenover time, persistent spasticity will stressand deform the joints, thus limitingrange of motion. It can also causechronic pain and fatigue – diminishingmobility even more. Laddusaw got herdaughter into physical therapy as aninfant and began to explore options.
2 in every 1,000 children are born with cerebral palsy
When Dr. Susan Apkon, a Children’srehabilitation specialist, began seeingArabelle in Billings, Montana (wherethe Laddusaws live), she gave her botulinum toxin and phenol injectionsto relieve the muscle tightness. But theshots were only a temporary fix. Oncethe medicine wore off, the spasticitywould return and Arabelle would needanother injection.
Apkon suggested the Laddusawscome to Seattle Children’s for apermanent solution that combinessurgery (selective dorsal rhizotomy, or SDR) with weeks of intense inpatientoccupational and physical therapyfollowed by months of outpatienttherapy at home.
60% of abnormal sensory nerves are typically disconnected during a selective dorsal rhizotomy
For the right child, SDR is transformative,says Apkon. “But it demands an extraordinary commitment fromthe child and the family, who bothhave to be active participants in rehab.Fortunately, Arabelle has a huge driveto walk, she’s willing to follow instructionsand tolerate hands-on manipulation bythe therapists, and her mom is completelycommitted – plus, there are greatphysical therapists in Billings whocan continue her rehab programafter discharge.”
“There’s a whole host of kids who can benefit from this procedure,” says Dr. Samuel Browd. “Giventhe plasticity of young kids’ brains, you can alter the way the nervous system functions and retrainit. With a patient like Arabelle, not only are we reducing the complications that typically come withlong-term spasticity, we’re also making it possible for her to walk independently.”
Neurosurgeon Dr. Samuel Browd likens SDR to the process of fixingmalfunctioning wires that carryelectricity into a house – it’s amatter of identifying which of those “wires” are acting up and leavingthe others alone.
The team makes a small cut inthe low back and exposes the nervebundles beneath one vertebra. ThenBrowd and the electrophysiologistpainstakingly test each bundleto accurately identify the nervesthat control motion and the nervesthat provide sensation. It’s themalfunctioning sensory nerves thatcause spasticity, so Browd then usestiny electrode hooks to stimulate thesensory nerves and determine whichones are responding abnormally.
“We go through a decision-makingprocess 100 to 120 times during eachsurgery, discussing the result we getfrom testing each nerve and decidingwhich to cut and which to keep,”says Browd.
There’s no room for error – thenerves don’t grow back. Cut motornerves instead of sensory nerves,and the corresponding musclesare paralyzed.
Stretching and strengthening are two majorgoals of Arabelle’s rehabilitation. This “stander”provides a hamstring stretch – and thecustomized iPad provides the necessarydistraction to allow Arabelle to spend enoughtime in the device to get the benefits.
It’s 2 p.m. and Arabelle is on the treadmillagain, supported by a harness thatholds part of her weight. Physicaltherapist Rachelle Steijn cajoles andcoaxes, guiding the preschooler’s legs.
Arabelle is adjusting to what mustfeel like a new body, says Steijn, asher brain and spinal cord learn torespond to new input. “Once thespasticity is gone, the old ways don’twork anymore. She’ll need to take athousand steps using this brand-newmovement pattern before it becomeshabit.”
It’s hard, tiring work. Arabelle alsohas to build strength. Now that hermisfiring nerves have been cut, theweakness of the muscles is revealed – rigidity had been masquerading asstrength. But after nearly three weeksof intense physical and occupationaltherapy, twice a day, six days a week,Laddusaw sees profound and excitingdifferences in her daughter.
“She’s already able to stand up onstraight legs and to sit cross-legged – she could never have done thatbefore – it’s amazing,” says Laddusaw. “I know we have a ton of work still. It’sa good thing we both have patience. I think she’ll be able to walk on herown within a year, I really do.”
Arabelle’s big brother Richard willbe waiting, soccer ball at the ready.
Though he is much older than the typical candidate for selective dorsal rhizotomy, 15-year-old MarkSimoneau successfully lobbied his care team, including Dr. Susan Apkon, for the procedure. “Now,”Mark says, “I’m lighter, more in control of myself.” He’s also free of the leg pain that used to keephim up at night.
Mark Simoneau doesn’t need a newpair of shoes this month. He didn’tneed a new pair last month, and hewon’t need a new pair next month.
Mark, now 15, used to blow throughshoes every few weeks because hecouldn’t lift his feet when walking, evenwhen using crutches or a walker. “Onepair only lasted five days!” Mark recallswith a rueful laugh. He was prone tofalling, and endured painful leg spasms.
Thanks to a successful SDRprocedure in August 2011, weeks of inpatient rehab and months of PTwith therapists in Bellingham, Washington,Mark has a brand-new level of controlover his body – and the independenceany teenager craves.
“I had always been dependenton Mom to put on my shoes and legbraces,” says Mark. “Now I can finallytie my own shoes. Plus, the pair Iwore home from Children’s lastednearly a year!”
“Rely on myself”
Mark had tried other spasticitytreatments over the years – PT, Botoxinjections, and the Baclofen pump, ahockey-puck-sized machine embeddedin his abdomen to deliver medicinedirectly to his spinal cord.
Mark was much older than thetypical SDR candidate, who is usuallybetween 3 and 5 years old. Apkonworried that his muscles wouldn’t haveenough underlying strength to regainhis pre-surgery mobility even thoughthe tightness and pain would berelieved by SDR.
“I really did not want that pumpback in. I wanted to be able to rely onmyself instead of a piece of equipment,”explains Mark, who experienced someserious complications with the pump.He wrote an essay about why hebelieved SDR was right for him, andconvinced his parents and his careteam that he was committed to whatwould surely be an arduous rehabilitation.
“I have alwaysbeen dependenton Mom to put onmy shoes andleg braces. NowI can finally tiemy own shoes.”
Eighteen months later, Mark saysit’s been worth the effort. It’s notjust about the shoes, no longer fallingall the time, or the hard-won abilityto keep up with his friends as theywalk around the mall. “This is abouthelping me to function in a normallife,” says Mark.
His dream is a career in sports,preferably football. “I know I won’t beable to play, but to be at the stadiumas a coach or a broadcaster? Thatwould be great.”
He’s on his way. A few months afterleaving Children’s, Mark was on thefield as the water boy for his brother’sfootball team. Closer to the action,closer to his dream.
Published in Connection magazine, April 2013