Hospital offers hope for teens with mysterious, excruciating pain
At Stanford University’s Lucile Packard Children’s Hospital, Elliot J. Krane, a leading professor of anesthesiology and pediatric pain, insists the best way to think of amplified pain syndrome is as a catch-all phrase, not a diagnosis.
(MCT) KANSAS CITY, Mo. — One moment, Albiera Camaiora — Albi — is a stellar free-diver, a 13-year-old from Lima, Peru, with the lung capacity to hold her breath for more than two minutes and the daring to plunge deep beneath the ocean surface, up to 70 feet, unaided by scuba gear.
She is a driven athlete. Then, in February, everything changes.
"One day, she falls down in the shower," her mother, Claudia Calderon, explains at Children's Mercy Hospital.
The injury is nothing, a trifling hip bruise.
But soon after, while on a skiing trip to Colorado, Albi is struck by crushing headaches from the back of her neck to her eyebrows. Her father and mother think it is the altitude, or maybe a sinus infection, until the pain spreads to her shoulders and back. On the return flight to Peru, it grows worse.
"That's when we start a nightmare," her mother says.
Over the next weeks and months, Albi sees more than 40 physicians from around the world — neurologists, cardiologists, rheumatologists, psychologists, psychiatrists, orthopedists, endocrinologists, experts in tropical diseases. They draw blood and analyze urine. They give her X-rays and MRIs. They prescribe pain pills. They can find nothing medically wrong.
By the time she flies in August to a children's hospital in Miami, the pain has spread to her entire body. Unable to walk without horrendous difficulty, she uses a wheelchair. She can't lift her arms. Her mother must spoon-feed her and brush her teeth. Touching her skin sends her into excruciating paroxysms.
Albi is incapacitated.
"We are desperate. We are afraid," her mother says. "... You stay awake at night. You don't know what's going on. It's horrible."
Then comes a call from a relative, a journalist in Los Angeles who has heard of a handful of children's hospitals nationwide that see kids with symptoms like Albi's. Within a day, in mid-September, the seventh-grader and her parents are on a plane to Kansas City.
"As of today, we are done with the wheelchair," rheumatologist Cara Hoffart, 34, tells the family at Children's Mercy.
Get up. Put it aside. Walk.
"As of today, we are done with the medications."
Hoffart knows what is wrong.
In some medical circles, a diagnosis of Amplified Musculoskeletal Pain Syndrome is as controversial as its symptoms are odd.
At Stanford University's Lucile Packard Children's Hospital, Elliot J. Krane, a leading professor of anesthesiology and pediatric pain, insists the best way to think of amplified pain syndrome is as a catch-all phrase, not a diagnosis.
"It's a term to describe kids with widespread pain when they can't figure out what's causing it," he said.
Other names include Widespread Pain Syndrome, Pain Associated Disability Syndrome and Diffuse Idiopathic Pain Syndrome, with "diffuse" referring to its body-wide nature and "idiopathic" going directly to its mystery, meaning "of unknown origin."
While some experts argue that amplified pain is just another name for a juvenile form of fibromyalgia — itself a chronic, all-encompassing pain with no set cause — others argue they are different in two ways.
First, while specific medications sometimes ease fibromyalgia's symptoms, they don't work with amplified pain. Second, there's longevity.
"I think when you read about fibromyalgia, there is a sense that it is something you will live with the rest of your life," Hoffart says. "I don't accept that (with amplified pain). I see our kids get better."
What no one argues is the reality or intensity of symptoms that physicians suspect may have plagued some teenagers for years, but may have been misdiagnosed.
It's unclear how many children are affected by this kind of body-wide chronic pain. But in the last decade, several hospitals have started programs to treat them. Children's Mercy opened Rehabilitation for Amplified Pain Syndrome in March, basing it on a similar program at the Children's Hospital of Philadelphia.
Other programs exist at the Mayo Clinic in Rochester, Minn.; Boston Children's Hospital; Stanford in California; the Cleveland Clinic Children's Hospital; and a few other hospitals.
Their patients, like Albi, tend to be driven, type-A, perfectionist teens. Girls typically are more affected than boys.
After a minor injury, or even a sudden perceived failure, they develop symptoms that doctors think have as much to do with psychology as physiology. The condition appears to exist at the nexus of illness and culture, suggesting that today's stressful, high-expectation teen environment plays a noxious role.
"I'll tell you about the pain I see and the kids I see," says Barbara Bruce, director of the Mayo Clinic's pediatric chronic pain program, which opened in 2010. "There's a lot of pressures on the kids we see. They're driven."
Even before they get ill, these kids tend to worry, Bruce said, about school and the future. Pressure can arise from themselves, parents, peers, culture.
Often it's a small hurt that temporarily hobbles them but causes high-functioning teens suddenly to feel imperfect or vulnerable. Stress kicks in, triggering a cascade of events in the nervous system. Hormones flood the body and, figuratively, set the nerves on fire. What began as a small pain grows and spreads.
Soon more doctors are called in. Tests show nothing physically wrong. That's often when parents and teens are told the pain is purely psychological.
"But the pain itself is real," Bruce said.
Hoffart likens it to bleeding ulcers. They, too, can be induced by stress, but that doesn't mean the sore eating away at the gut isn't real.
The more doctors these children see, the thinking goes, the more their stress mounts. They miss school and fall behind. They see parents worry and medical bills mount.
The nerves in the body, already on high alert, become even more sensitive. What started out as an insignificant hurt amplifies to incapacitate the entire body.
"I have kids who are in so much pain they can't wear clothes," Hoffart said.
For Emily Lowe of De Soto, Kan., as for Albi, symptoms developed quickly.
Emily's mother, Shannon Lowe, 41, says her daughter had been treated by doctors on and off since her appendix burst when she was 9 months old. She also was diagnosed with Kawasaki disease, a rare but treatable inflammation of the blood vessels that can affect the skin and cause heart problems.
"She's been dealing with pain her whole life," Emily's mother says.
But what happened during the last school year, when Emily was 14 and in middle school, was something else, she says.
Her daughter had had an extensive surgery and had already missed close to 30 days of school. Too long afterward, she was still in pain. Her principal could see it on the girl's face. The family, which had toyed with home-schooling anyway, pulled Emily out of school last November.
"After we pulled her out, it just went on and on with the pain," Lowe says. "Hers was diffuse, from head to toe. Her muscles would spasm. You couldn't get it to stop."
Headaches hit her like clenched fists.
"The light would just kill her eyes. We had towels over the windows. It (the pain) wouldn't turn off. She couldn't sleep — no sleeping for 24 hours. Her hair began dropping out. Her eyelashes dropped out. We went to all kinds of doctors."
One misdiagnosed her with lupus, an autoimmune disease.
"I had a whole chart of 17 pills a day she was on: oxycodone, hydrocodone, lupus medicine, everything. And it wasn't working. I slept on the floor next to her bed for months because she couldn't sleep," Lowe recalls. "I would have to take her to the bathroom; she would cry the whole way."
Then her daughter ceased walking and, at times, seeing.
"She would go blind," her mother says. "It (the pain) would turn off her eyes. It would turn off her ears. It was horrifying."
To both mother and daughter.
"It was scary," says Emily, now 15. "I didn't know how this was going to end. I didn't know what I had."
On the Internet, Lowe found a research paper on amplified pain. She called Children's Mercy.
"OK, Miss Albi, back in the pool," says Children's Mercy occupational therapist Misty Wilson. "Get down there and crab walk."
The former competitive diver and swimmer turns her body in the shallows of a therapy pool and walks backward like a crab on her hands and feet.
She says nothing, but winces in pain.
In the pool shower, she hunches her shoulders and grimaces. The water hits her back like bees stinging. The therapist's goal, over the next few weeks, is to get Albi to stay under the shower continuously for eight minutes.
Water splashes her head and back. Albi shies away.
"That was 15 seconds," Wilson says. "Shoulders down. Back in."
This day is the start of what will be close to six weeks of intense physical and mental work.
Five days a week, from 7:30 a.m. to 4:30 p.m., Albi will undergo a regimen of occupational therapy, physical therapy, psychological counseling, yoga and relaxation exercises while her mother takes part in family counseling.
The hospital's program treats children with less severe symptoms at an outpatient clinic, but it has three spots in its most concentrated daylong program for severe cases like Albi's.
The cost is considerable, although Children's Mercy has been successful at getting most insurance companies to cover the bills, which easily run in the tens of thousands of dollars.
Only blocks from the hospital, Albi's mother is spending thousands of dollars to room for six weeks with her daughter at the Westin Kansas City at Crown Center. Albi's father, who must stay in Peru to care for her 15-year-old sister, owns a construction company. Less affluent out-of-town families often stay at the Ronald McDonald House.
Besides kids like Albi, there are also those like Makayla Clark, of Marion, Iowa, who was 12 when she entered the program in late May. An A-student and athlete, she played basketball, softball and volleyball and danced nearly every night. Then one knee began to hurt. She began to limp. Her mother, Karen Dowler, took her to doctors in Iowa who put her in a brace.
"We went from a limp to a brace to crutches to, within a few weeks, not being able to walk at all," Dowler said.
Instead of the widespread pain that Albi has, Makayla was diagnosed with a more localized condition called Complex Regional Pain Syndrome.
"I was thinking I probably wouldn't walk again," said Makayla, now 13.
For both girls, the program at Children's Mercy was grueling. It attacks the pain on three fronts: Exercise; "desensitization" therapies; and cognitive and family therapies to identify and address the psychological roots of stress.
"I tell our kids they have weird pain. We do weird things," Hoffart said.
Their methods are specific, slow and controlled. But the last thing they do is steer clear of the pain.
"If it hurts to do something, that's what we tell them you should do," Hoffart said, which is one of the prime reasons they tell kids on day one to get up from their wheelchairs.
Hoffart also takes kids off their pain medications. Nor does the program use pain medications or nerve blocks during therapies, an aspect of the program that makes it more controversial than others that do use medicines. It also tends to reflect differences in philosophy in programs run by rheumatologists, like Hoffart, and those run by anesthesiologists.
At Stanford, the anesthesiologist Krane, for example, thinks "there is a role for medications from time to time."
"For some kids, physical therapy is just too painful, and you can't do it," he said. "I think it is cruel to subject a child to painful physical therapy without a nerve block."
Hoffart maintains that for most kids, these medicines have already failed to work.
For Albi, just attempting to walk is excruciating. Her steps were mincing, like a child walking on shards of glass.
Most of the day's therapy takes place in the hospital's Don Chisholm Building, an annex about one-third of a mile from the main hospital. The kids are forced to walk there.
On her first day, it took the once-athletic Albi more than 70 minutes.
Using balls and baskets, cones and other equipment, the kids are asked to walk up and down stairs, do pushups and knee bends, walk on treadmills.
"We don't coddle them," says Wilson, the occupational therapist.
Desensitization therapies — massages, touching, rubbing, showers, hair brushing — help the kids develop a tolerance to pain that, gradually, causes the pain to subside.
"They make me do a million things a day," Albi says during a brief break. "When I get out of here, I'm so tired and exhausted and frustrated, I'm in a bad mood and a lot of pain. But inside of me, I know this is good for me."
Meanwhile, the yoga, music therapy, art therapy and personal counseling are geared to find ways to reveal and deal with pressures and psychological stress.
When many of the teens first arrive at Children's Mercy, they deny that they are feeling undue stress, said psychologist Dustin Wallace.
But it often doesn't take long before kids are talking about the high expectations they face. In counseling, Wallace said, kids reveal their most fundamental fears: that they're not living up to their potential, that they're not worth the sacrifices their parents have made for them.
"They may get a bad grade on a test," Wallace said, "and then think, 'If I get a bad grade, I'm never going to pass this class. If I don't pass this class, I can't get into college. If I can't get into college, I won't be able to get the job I want.'"
Less than six weeks after she entered the program in a wheelchair, Albi left able to shower, dress, brush her teeth and even jog. The walk to the annex took eight minutes instead of 70.
The program is so stressful that pain sometimes actually increases in the weeks they're there, Hoffart said. But the experience at other programs and Children's Mercy's own follow-ups show that the pain tends to subside to manageable levels over the following months.
When pain flairs, the teens have the tools to reduce it without medications. Time will tell if it ever completely goes away.
At Albi's "graduation" in October, her mother told Hoffart and her team, "You made a miracle."
"I can't explain the emotion to come home with Albiera again as a normal person," she said. "I don't have the words. Thank you from my heart."
Albi said that when she got home, she had a plan.
"I'm going diving again," she said.
©2013 The Kansas City Star (Kansas City, Mo.)
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