When Sarah Dowd was diagnosed with breast cancer in August 2013, the news hit her like a shock wave. Her family has no history of cancer. She was only 48 years old.
For anyone facing a cancer diagnosis, the physical, mental, and emotional challenges are immense, but Dowd had to face an additional hurdle — she has cerebral palsy.
Her treatment, however, did not differ from standard breast cancer protocol. “They were a little more cautious in my chemotherapy because I have weak lungs, but my treatment was typical otherwise,” Dowd says.
Fiercely independent and resilient by nature, Dowd faced her cancer defiantly. Throughout chemotherapy, she spent time with friends, continued working, and kept going to the gym.
It wasn’t until after her chemotherapy treatment was done that she started to have issues. “After the cancer, I gained a lot of weight,” Dowd says. “I started experiencing a lot of my pain in my knee and I started walking differently.”
Like 60 percent of individuals with CP, Dowd can walk — albeit with a slight limp on her left side. Her balance isn’t perfect, but she can usually get around just fine. That changed when she started taking Tamoxifen, a drug to prevent her cancer from recurring. Her balance faltered, her left knee was in constant pain, and she started falling regularly.
Dowd knew she needed to find a specialist — someone who could help her navigate the limitations of CP alongside the complex side effects of cancer treatment. Her search for that person brought Dowd to the Department of Allied Health Sciences at the UNC School of Medicine.
Coming to UNC
Little research exists on the combination of CP and cancer because individuals with CP didn’t use to live into late adulthood. Up until about 25 years ago, children born with cerebral palsy would be institutionalized, and remained that way through their adult lives — they often died in their 30s and 40s.
“But that’s not the case anymore,” says Deborah Thorpe, a UNC physical therapist and researcher who specializes in CP. “People with CP now live a typical life span, to within five or six years of their peers without CP.”
Before she became a physical therapist, Thorpe worked as an elementary education specialist and special educator. She spent the better part of two decades working in schools with children with CP, developing lasting relationships over the years. But once they aged out of the school system, many of these children no longer had access to regular therapy — and, eventually Thorpe started receiving calls from families who didn’t know where to turn.
“When I started to look into it, I realized there was very little research in this country, especially on aging and cerebral palsy,” she says. “About 12 years ago, I decided to devote my career to that.”
Today, the majority of Thorpe’s work includes adults with CP. Moving through the world is a challenge for these individuals, and a constant strain on joints that have already been weakened by the disorder. Many individuals with CP who walk — but don’t receive regular physical therapy — are actually worse off than their peers in wheelchairs because of the stress on their joints.
In one of her studies, Thorpe examined 100 patients and found 84 percent of them experienced musculoskeletal pain. “These are not old arthritic people,” Thorpe says. “The average age of the participants in that study was late 30’s.”
Thorpe was in the midst of recruiting for another research study when she met Dowd at the UNC Carolina Rehabilitation Center.
“I wasn’t even thinking that physical therapy was going to be part of the solution — I thought bracing or something,” Dowd says. “Then the doctor tells me about research, and Debbie happened to be at the clinic that day.”
“Sarah is very unique in a way because she has other things going on besides cerebral palsy,” Thorpe says. “She’s the first adult with CP I’ve met that is also a breast cancer survivor.”
After consulting with her oncologist in April 2016, Dowd stopped taking Tamoxifen. “I was aware of the possible side effects of bone and joint pain, but I didn’t correlate it to impacting my CP in the way that it did,” she says. Since Tamoxifen is a preventive medicine, the oncologist cautioned Dowd about the possibility of her cancer returning. She decided to take her chances in hopes of improving her quality of life.
“It’s kind of like a puzzle when you work with someone with cerebral palsy,” Thorpe says. You address one thing and then something else happens.” Soon after she started seeing Dowd, Thorpe found she had a leg length discrepancy. “We had orthotics made for her shoes, evened her out, but when we did that, it was her good leg that was shorter,” Thorpe says. “Now she’s being shifted onto her impaired side so putting more stress on that side. Her knee is swelling more frequently — we’re trying to address these things.”
Because of her CP, Dowd has low bone density. “So her chance of fracture when she falls — and she falls very often — is high,” Thorpe says. “She came to me because she wanted to gain her strength back and reduce her risk of falls.”
Of all the therapists Dowd has worked with over the years, she says Thorpe has been the most attentive. “The way she emails back and forth. If I had to cancel an appointment, she didn’t just say, ‘Okay, we’ll see you next time.’ She would say: ‘We’ll fit you in tomorrow.’ If I was experiencing pain, she would problem solve it,” Dowd says. “She always tells me, ‘I want you to be at your best.’”
Thorpe is also realistic. When they first started working together in September 2016, she told Dowd there was a good chance she would eventually have to have surgery on her knee, despite all their physical therapy work.
Dowd is scheduled to have her knee operated on in July. But as soon as she’s done with the surgery and rehabilitation, she wants to go back to working with Thorpe.
Falling off the radar
Cerebral palsy most often results from a lack of oxygen to an infant brain during pregnancy or childbirth, and babies born prematurely are most at risk for developing it. “Unbelievably, in this technologically advanced country that we live in, the incidence of CP was increasing up until the last couple years — now it’s leveled off,” Thorpe says. “Because we’re getting better and better at saving younger preemies—and they have the highest risk for a brain bleed. Even though we’re really good at saving them, a percentage of them will have cerebral palsy.”
In the best case scenario, the child is diagnosed early on, and receives intensive services and therapies.
“Between zero and 5 years of age is when the brain has the most neuroplasticity,” Thorpe says. “Intervention can still be effective past that point, but to get the biggest bang for your buck neurologically — to get the brain to rewire itself — you want to intensely intervene very early in the child’s life.”
Approximately 764,000 people in the U.S. manifest one or more symptoms of cerebral palsy, according the Centers for Disease Control. The severity of symptoms varies widely, including muscular weakness and spasticity, trouble with walking and balancing, and trouble multi-tasking. While 40 percent of adults with cerebral palsy have both cognitive and physical impairments, the other 60 percent are not cognitively impaired, and have the potential to be productive, hold a job, and fully participate in their communities.
“Some people, like Sarah, do very well,” Thorpe says. “There are students walking around on this campus that have cerebral palsy and you would never know it, except for possibly a slight limp or a posturing of a hand.”
Until they are 18, most children with cerebral palsy receive regular, intensive therapy in school. Some students, because of cognitive involvement, don’t age out of the school system until they’re 21. “But after that, whether they have cognitive impairment or not, typically all services drop way off,” Thorpe says.
Once they exit the school system, adults with cerebral palsy often fall off the radar, according to Thorpe. “If they don’t have a catastrophic medical event, then they may not get seen by a primary physician or other adult health care providers for several years.”
While advances in medicine and technology have contributed to longer life expectancy for individuals with CP, the training for healthcare practitioners has not caught up.
A typical adult practitioner is not trained to provide specialized care for someone with cerebral palsy, which has traditionally been approached as a “pediatric” disorder. “In the past — about 40 to 50 years ago — they didn’t need to be trained because these kids didn’t live that long,” Thorpe says.
But now they do.
Thorpe often gets referred patients who are losing their motor function. They start falling more or have a fear of falling. “Regardless of whatever else they have going on, almost every single one of them is experiencing significant pain.”
Thorpe is part of a small contingent of researchers both in the U.S. and abroad and whose research focus is studying the development of secondary conditions in adults with cerebral palsy. This country does not currently have a national registry but a group of dedicated researchers (including Thorpe) is working to fill that void with an initiative called the Cerebral Palsy Research Network.
“Many European countries are far ahead of us in research with adults with cerebral palsy because they all have registries and can follow the children longitudinally into adulthood,” Thorpe says. “The United States needs to get up to speed.”
She wants the ability to collect data on children as they transition into adulthood, and specifically follow the services offered to them. She has a grant to look at national Medicare claims to look at the treatments offered to individuals with a diagnosis of CP who have claims for musculoskeletal conditions as compared to similar patients who don’t have CP. Her goal is to determine if adults with CP are a disparate health group.
Presently, in North Carolina, adults with Medicaid (the majority of the adults with CP) are only allowed three therapy visits per year. “I think the policy makers have to get on the ball here and change the Medicaid laws, but to do that, they’re going to look to the research,” Thorpe says. “We need research to show that providing physical therapy to individuals with cerebral palsy after the age of 21 is going to make a difference in their quality of life and their productivity to society.”
“My life revolves around medical stuff,” Dowd says. “For me, it’s like why not be involved in research? I’m 50. We have the resources to fill in the gaps so that other individuals with CP might benefit. It’s a give and take.”