A CHILD'S PAIN

By Denise Grady

Looking back, trying to pinpoint the moment in 1990 when she first knew something was wrong, Julia Uihlein recalls a September afternoon, a soccer game and the ashen face of Alex, her 11-year-old son. Alex was not injured, but he had suddenly become so pale and ill looking that his coach pulled him out of the game. What had at first seemed to be the flu soon took a frightening turn. Constant, burning pain in his legs kept Alex awake at night. The slightest touch, even by a bedsheet, hurt unbearably. He began to have trouble walking.

The doctors were stumped. "My worst fear was that it was a tumor on his spinal cord," his mother says. The Uihleins (pronounced E-lines), who live in Milwaukee, Wis., took Alex to several respected medical centers in the Midwest, but none had an answer. One doctor even accused Alex of faking. "He said, 'You deserve an Academy Award,' " Alex says bitterly. For a day or two, even Julia began to doubt her son. Then, she remembers, "I said, 'Let's get out of here.' "

By November, Alex needed a wheelchair. Julia kept calling doctors. Finally, a neurologist suggested that Alex might have an unusual nerve disorder known as reflex sympathetic dystrophy. He urged Julia and her husband David to take their son to Children's Hospital in Boston to see a doctor named Charles Berde.

A pediatrician and anesthesiologist, and an associate professor at Harvard Medical School, Berde, 46, is co-founder and director of the pain-treatment service at Children's Hospital. That service, established in 1986, was among the first in the U.S. to specialize in children's pain. Fewer than a dozen American hospitals provide such departments for children.

The staff includes doctors, nurses, psychologists, physical therapists and an acupuncturist. They treat children in the hospital and in an outpatient clinic for both acute and chronic pain--due to surgery, injuries, cancer, cystic fibrosis, AIDS, sickle-cell anemia, migraine headaches, hemophilia and nerve disorders. The team is nationally recognized for its expertise in treating the reflex sympathetic dystrophy that Alex Uihlein's doctor suspected. Berde is also a leading researcher in pediatric anesthesiology, and he has written more than 70 scientific papers and 50 book chapters dealing with ways to improve the prevention and treatment of pain in children. No child in his care is ever accused of faking. "They're not crazy, they're not faking, they're not making it up, they're not lying," he says. "Pain is real."

Berde likes and respects children, and he has known he wanted to work with kids and families ever since his medical-school days at Stanford, where he also earned a Ph.D. in biophysics. He is calm, soft-spoken, easy to talk to and genuine: not given to calling his young patients "Pal" or "Dude" or demanding high-fives. Pictures of his two children abound in his cluttered office. His beard gives him a slightly impish look, and he is not tall enough to tower over his patients.

Morning rounds on the surgical wards with Berde's team are a pleasant surprise to visitors. No one is crying or moaning. Children who had major operations only a day or two earlier seem comfortable. Some are provided with pumps they can activate as needed to inject pain killers through intravenous lines. Others have epidural catheters inserted in their backs, delivering medication into the space around the spinal cord to numb the lower part of the body. Such treatment provides steady control of pain, Berde says, and eliminates the need for the repeated shots most children dread.

The pain-treatment service came about because Berde and colleague Navil Sethna, faced with patients who had seemingly intractable problems, devised novel solutions, and because other doctors began to seek them out. One of their first cases was an 18-year-old boy with cancer. Suddenly, the boy's pain had spun out of control. "In three days he went from no morphine to 400 milligrams an hour, which is a pretty industrial dose," says Berde. "A normal amount might be 3 milligrams an hour."

Berde and his colleagues inserted a spinal catheter and gave the boy a local anesthetic and an opiate. The patient had been screaming; now he became comfortable and alert and was able to go home. Although that treatment had been used to control pain in adults, Berde says, "I don't know if it had been used much in kids. We had no protocol for it. But he clearly was terminal and not relieved by massive amounts of morphine."

Other difficult cases followed, among them children with chronic pain. Some of the toughest cases, like that of the boy with cancer, involved neuropathic pain caused by damage to major nerves. Such pain can result from amputations, injuries, cancer and other diseases that affect the nerves, and it often does not respond to standard therapy. "I was making it up by extrapolating what had been done for adults and knowing the pharmacological differences between children and adults," Berde says. "I began thinking there was a need for better ways of managing pain, and a need to have it be multidisciplinary." In some cases, he knew, particularly the chronic ones, psychologists and physical therapists would be essential.

Berde and Sethna told the head of pediatric anesthesiology that they wanted to work on pain management. "He wasn't opposed, but he didn't see a need for it," Berde says. But when 90 patients were treated in the first eight months, the boss changed his mind. Together with psychologist Bruce Masek, Berde and Sethna formally opened the service in 1986.

When it comes to treating pain in children, the medical profession has a checkered history. Until the 1970s, the mistaken idea that babies do not feel pain was widely accepted, and infants undergoing major surgery were often given little or no anesthesia, just drugs to paralyze them temporarily. "The reluctance to use anesthesia was not due to doctors' being mean and nasty," Berde says. "There were real risks. It was an era when some babies did die from anesthesia, especially the ones who were very sick. So if you didn't know how to anesthetize them safely, it was easier to believe they didn't feel pain."

But of course they do. In fact, Berde says, research has shown that babies actually feel more pain than older patients--longer-lasting, more widespread pain that is likely to affect their behavior later in life. Pain unleashes a destructive cascade of stress hormones that can weaken the immune system and make the heart rate and blood pressure soar. Studies in the 1970s and '80s showed that babies deprived of anesthesia during surgery were more likely to develop infections, brain hemorrhages, muscle wasting and difficulties in healing.

Those findings, combined with advances that have greatly reduced the risks from anesthesia during the past 10 to 15 years, have brought about some substantial changes. "Now no newborn is too sick to get pain medication," Berde says. In general, there seems to be more effort to reduce kids' pain from all medical procedures, including bone-marrow biopsies, spinal taps and repeated blood drawings. Says Berde: "I think most major children's hospitals are changing. There is less willingness than there used to be to hold kids down and brutalize them."

There is still room for improvement, though. Families with children who need surgery are traveling long distances to Children's, specifically because they have had bad experiences with pain control in other hospitals. In addition, not enough research is being done on pain medication for children because, Berde believes, drug companies do not think children are a large enough market. Too many babies are still being circumcised without anesthesia, in Berde's opinion; he thinks that at the very least a numbing cream should be used but that general anesthetics and nerve blocks are more effective. "You couldn't go into an animal lab and do a procedure like that without anesthesia," he says.

One of Berde's research interests is developing local anesthetics that will work for days or a week after surgery instead of for six hours, as existing drugs do. Prolonged pain after chest or abdominal surgery is not just unpleasant; it can be harmful as well, keeping a patient from taking deep breaths or coughing--things they need to do. Pain can also keep people bedridden, impeding their recovery. "Our major aim is to get people up quickly," Berde says. "They're less likely to develop pneumonia, lose muscle mass and have trouble sleeping." Ambulatory adults are also less prone to blood clots, heart attacks and mental confusion.

But pain killers now in use have drawbacks. Morphine, codeine and related drugs, given by mouth or intravenously, can cause such side effects as nausea, constipation and itching. Epidural blocks can lead to similar problems. In addition, they must usually be removed before the patient goes home, even though he or she may still be in pain. Berde has found that many people are reluctant to take pain medication at home, or give it to their children, in the mistaken fear that they will become addicts.

The problem might be solved by long-acting local anesthetics. Berde and his colleagues at Harvard and the Massachusetts Institute of Technology have patented time-release beads that contain a commonly used drug, bupivacaine. Injected into a surgical incision, the microscopic beads may block pain for a week, possibly reducing or even eliminating the need for opiates. The hope is that they will enable patients to recover sooner. The beads are being tried on patients overseas, and Berde expects testing in the U.S. to begin soon.

He is also working with other researchers to develop long-acting local anesthetics from toxins found in some fish, shellfish and algae--the same toxins that cause poisoning victims to feel numb and weak all over. Berde is pursuing the toxins because they work for two or three days and seem free of the side effects of existing drugs, which occasionally cause convulsions or disturbances in heart rhythm.

Important as research is, Berde does not let it keep him from taking care of patients. "One of the best things he does is care for kids who are dying," says Pauline Scopton, a nurse who has known Berde for 17 years. "He is a master of the chemistry, of mixing the drugs to keep them comfortable." For families who wish to keep a dying child at home, she has known him to spend hours on the telephone with pharmacists and home-care nurses to come up with the right pain medicines.

Berde questions the widely held belief that doctors and nurses become inured to their patients' suffering. "You don't distance yourself," he says. "It's not realistic, the notion that you don't develop a connection. Do I get sad? Yes. It's sad when a kid dies. But feeling that I can do something for them helps. At times it's hard, but that doesn't make me not want to do it. Having my own kids makes me understand the impact of illness even more, and I admire the courage of these families even more."

It is not unusual, says Scopton, for Berde to go home to say good night to his children--David, 12, and Anna, 9--and then return to the hospital to take care of a child who needs help, particularly one who is dying of cancer and in great pain. It is also not rare for him to get a 3 a.m. phone call from, say, India for a consultation about some young patient in pain. "He has worked almost every day of the week almost since I've known him," says his wife Evelyn.

Seven years have passed since Alex Uihlein was treated at Children's Hospital, but Berde remembers him well. "He arrived in severe pain, essentially confined to a wheelchair, and if anyone moved his legs or touched them, he would cry and scream," Berde says. "He was withdrawn and just in very, very bad shape." Alex viewed Berde warily. "I was sick of dealing with doctors who didn't understand," Alex says now. But he found Berde different. For one thing, Berde listened. "He did understand," Alex says. "He believed in me, so I believed in him."

Berde determined that Alex did indeed have reflex sympathetic dystrophy, a condition in which pain originates from an abnormality in the nerves. In Alex's case, it was due to hyperactivation of nerves running from the spinal cord to the limbs. Alex's legs became hypersensitive to the slightest touch, and they turned blue with cold, for no apparent reason. The cause of the disorder cannot always be determined. It often follows an injury, but Alex's case might have been triggered by a mysterious viral illness. Untreated, the condition can lead to loss of muscle and bone and even permanent disability.

Because Alex's pain was so severe, Berde began by giving him an epidural that numbed his legs for several days, freeing him of pain for the first time in months. Next Alex began an intensive program of physical therapy and counseling. He learned self-hypnosis and imagery to help him cope with the pain, and Berde prescribed antidepressant medications--not because Alex was depressed but because the drugs have been found to quiet the nerve activity that causes neuropathic pain.

Most important was to get Alex moving, to reassure him that physical therapy would not harm him even though it would hurt. Not only would exercise help restore his strength, but Berde had found that it seemed also to help reprogram and quiet the misfiring nerves. Alex spent two months in the hospital. By March, six months after he first became ill, he had begun to walk with a cane. He recovered steadily, though he still needed physical therapy and took several years to regain his strength.

Today Alex, at 18, backpacks, skis, plays tennis and kayaks. But Julia Uihlein thinks that if they had not found their way to Boston, he might never have recovered. She has met other patients with his condition who went untreated for years, and they have not fared well. "Listening to patients," she says. "That's where Dr. Berde started. It seems elementary, but it's really so profound." The greatest tribute, however, is that Alex is thinking about a career in medicine. He spent this summer working for a Milwaukee anesthesiologist who trained under Charles Berde.