After 40 hospitalizations, four transfusions, two operations, pneumonia, inexplicable fevers and a life-threatening loss of blood from his spleen, 5- year-old Kyle Smith has entered a new phase of sickle cell disease.
His red blood cells, distorted into rigid half-moon shapes, sometimes jam the tiny vessels of his blood system and starve the muscles and bones of oxygen, creating a pain deeper and more severe than most people will ever experience.
Such pain – unpredictable, intermittent and lifelong – is a hallmark of sickle cell disease. Narcotics have been the only available treatment – morphine in the hospital and codeine at home are standard – yet even these powerful painkillers often fall short.
So Kyle Smith, who is just starting to read, will soon embark on learning another skill as well: self-hypnosis.
Kyle is part of a continuing study at the Institute of Pennsylvania Hospital, where researchers have found that self-hypnosis can eliminate mild and moderate pain in patients with sickle cell disease.
The average patient is spared the equivalent of a month of pain per year, according to preliminary results of the study, presented in March at the annual meeting of the National Sickle Cell Disease Program. The length of time between painful episodes is more than doubled, and the need for pain medicine is reduced.
Though hypnosis is considered an “alternative” form of medicine, it’s been used regularly, if not widely, for the last 30 years to treat pain. Studies have shown its effects with burn victims who must undergo acutely painful dressing and redressing of their wounds, and with leukemia patients who must have their bone marrow aspirated by a needle.
In rare cases, patients under hypnosis have undergone major surgery without anesthesia, including appendectomies, tonsillectomies and even operations of the heart, lung and brain.
Although the physiological mechanisms of hypnosis are not well understood, researchers believe it relieves pain in several ways. It reduces the anxiety that can make pain worse. It allows a person to separate, or dissociate, from the pain by focusing attention elsewhere. In some people – those who can withstand surgery without anesthesia, for instance – it actually blocks the sensation of pain.
Most importantly for people with sickle cell disease, hypnosis dilates the vessels and increases the blood flow. This breaks up the occlusions and restores the flow of oxygen, doctors believe.
Sickle cell is an inherited disorder that affects ethnic groups from areas where malaria is endemic, including Africa and the Mediterranean. It is the most common serious genetic disorder among African Americans, affecting one in 600.
The illness, for which there’s no proven cure, can cause strokes and other life-threatening complications, even among the very young. Its long-term effects include painful erosion of the hip and shoulder joints and gradual damage to any number of organs, including the heart, liver, lungs, kidney and spleen.
The problem lies in the hemoglobin that floats in the red blood cells, carrying oxygen to all parts of the body. When the hemoglobin gives up its oxygen, it can crystallize into long fibers, distorting the once-pliable cell into one that is stiff and sickle-shaped.
Cells that are moving through very small blood vessels and capillaries can become stuck, depriving the muscle or bone downstream of necessary oxygen and triggering a release of pain-causing chemicals.
“It’s like if you put my hand in a vise and continued . . . screwing it,” explains Walter Brandon, 54, the oldest participant in the self-hypnosis study and chairman of the Sickle Cell Genetic Disease Council of Southeastern Pennsylvania.
The most severe pain usually begins in his abdomen and radiates throughout his entire body, said Brandon. “It’s almost unbelievable.”
Children, who are less able to describe their pain, are often asked to rate the intensity numerically. Given a range of zero to five, Kyle Smith told his mother, “It hurts a hundred million.”
In the self-hypnosis study, psychiatrist Peter B. Bloom does much of the training of the patients. He describes a hypnotic state as the relaxed self- absorption that occurs in certain everyday experiences.
“You get absorbed in a book and you forget about what’s happening in the next room,” said Bloom. “A child screams . . . (and) you can put your book aside. So it is with hypnosis.
“It’s also like driving in a car when you’re daydreaming. You go street after street thinking about something else. You’re driving safely, but your mind’s somewhere else. Hypnosis is the purposeful way of putting your mind somewhere else.”
Initially, Bloom will demonstrate to patients the power of the imagination, how the mind can affect the body in unexpected ways. At a recent group session, he pulled out a gold ball suspended from a length of chain. Handing it to one woman, he asked her to imagine the ball moving back and forth, then side to side. And as she did, the ball moved.
“If you can do that,” said Bloom, “then we know that imagination can affect how we feel. . . . The image can make something happen, and our body will follow along.”
Other times, Bloom will induce a trance, then ask the patients to imagine that a helium-filled balloon is pushing their left arm into the air. Most will believe the arm feels more comfortable in this raised position – “supported” by the imaginary balloon – than it does resting normally at their side.
In the same way, Bloom tells them, they can alter their perception of pain. ”Their conscious mind doesn’t have to be in charge of everything that happens,” he said.
Most sickle cell patients experience at least one or two episodes of pain each year, and 15 percent to 20 percent endure more frequent bouts.
At its most intense, physicians say, the pain of sickle cell disease is worse than that associated with cancer. Its ferocity is captured by African tribal names for the disease, which translate as “body chewing,” “body biting” and “beaten up.”
Patients in severe pain must be hospitalized so they can be given painkillers intravenously or by injection.
Despite advances in pain management, however, many of these patients are undertreated. According to pain specialists, many doctors, nurses and family members administer painkillers in insufficient doses out of fear that the patient will become addicted.
“I’m always fond of telling medical audiences that the overall lifetime prevalence of substance abuse in physicians and nurses is much higher than it is in patients with sickle cell disease,” says pediatrician Barbara S. Shapiro, associate director of the pain service at Children’s Hospital of Philadelphia.
Even when narcotics are administered properly, the most they can do is make pain tolerable, Shapiro says. “Pain is such a complex human experience. It partakes of emotion and memory and cognition, as well as sensation.
“When one gets medication, it helps to dull the way in which the brain perceives the pain. But it doesn’t . . . address the inchoate fears that patients bring. . . . The fear that the pain is going to kill them. The feeling that they cannot do anything.”
What makes the pain of sickle cell disease so insidious is how unpredictable and uncontrollable it is. It comes and goes without warning, lasting anywhere from minutes to weeks.
“It’s not like (when) you’re going to the dentist (and) you’re getting your wisdom tooth yanked out,” explained Carlton Dampier, a pediatric hematologist/oncologist and medical director of the sickle cell center at St. Christopher’s Hospital for Children.
“These people don’t know when it’s going to start, they don’t know where’s it going to happen, they don’t know how bad it’s going to be, and they don’t know how long it’s going to last.”
In time, such pain can have enormous consequences for nearly every aspect of life – disrupting work, destroying relationships, altering mood and personality.
As a fellow in hematology and oncology, Shapiro has treated many children with sickle cell disease. “What I kept hearing from patients was their helplessness,” she says.
At the Institute of Pennsylvania Hospital, Shapiro persuaded a group of researchers internationally recognized for their work in hypnosis to apply the technique to sickle cell pain.
With funding from the National Institute of Mental Health and several private foundations, the Unit for Experimental Psychiatry set out, initially, to assess just how much pain these patients were having and how much it interfered with their lives.
Previous studies had measured the pain primarily by how often the patient had been hospitalized. But in this study, patients were asked to keep track of their pain at home. They were given a weekly diary and asked to evaluate their pain every morning and night, also recording any pain medications they took and whether they were able to go to work or school.
Eventually, the researchers collected 34,000 days of entries from 78 patients. The patients, referred from 12 area hospitals, ranged in age from 5 to 51.
“There was a lot more pain out there than anybody realized,” said psychologist David F. Dinges, co-director of the Unit for Experimental Psychiatry.
Nearly nine out of 10 painful episodes occurred without any contact with a doctor or hospital, the researchers found.
The average patient spent 26 percent of the time in pain – about one day in four.
And when children and adolescents had sickle cell pain, about half the time they were likely to sleep poorly, miss school and have to take a narcotic.
Many variables can trigger the painful episodes, including stress, cold weather, fatigue and infection. The researchers identified one group of patients who seem especially susceptible at night.
For them, sleep itself may be a cause, Dinges and others theorized. The normal drop in oxygen that occurs during sleep may trigger their pain.
Once patients know what it’s like to be hypnotized, they learn fairly quickly how to induce their own trances, says Bloom. He teaches them to stare at their thumbnail or a spot on the wall.
All hypnosis is essentially self-hypnosis, he says, because the ability rests with the person who is hypnotized. It is estimated that 90 percent to 95 percent of the population can experience at least a moderate trance.
New patients meet weekly, in a group, with either Bloom or the director of the research unit, psychiatrist Martin T. Orne. The hypnotist induces a trance in the patients and, for 10 to 15 minutes, guides them through peaceful, relaxing imagery.
They are encouraged to practice on their own, so they can become adept at entering a trance quickly – before the pain gets too distracting. To do that, they have to find their own image of peace and comfort.
For Walter Brandon, who spends much of his time at the Sickle Cell Genetic Disease Council helping others, that image is a dark, empty room. “I go to the corner of the room and I sit in the dark,” he said. “. . . There’s nothing there but me.”
After 15 minutes or so, he comes out of the trance and the pain is gone, he said. But it returns in time, as he again becomes aware of his body.
For Tiffany Gonzalez, 11, the image is much different. She is playing in the park or having fun with her Barbies. “It makes me feel comfortable,” she said.
Together, hypnosis and Demerol control some of Tiffany’s pain. “But when she’s in the hospital,” said her mother, Sarah Abrams, “when the pain is like that, forget it. She is in too much pain to concentrate.”
Others report that hypnosis allows them to get along with less medication.
But not everyone finds relief. Roashia Willis, 19, said neither hypnosis nor medication helps with her pain, which has become constant. She continues to attend the research program, however, because the hypnosis “was working at one point.”
After five years, the program has exhausted its original research funding, but doctors are trying to keep it running by seeking new government sponsors and foundation support.
In May, seven new patients were enrolled, including 5-year-old Kyle Smith.
Though typically a child must be 7 to participate in the study, Juanita Smith lobbied hard to get her son Kyle enrolled. Like many parents, she feels tremendous conflict about the narcotics that ease her son’s pain.
“I don’t want to not give him any pain medicine, but . . . Kyle has a chronic illness. He’s going to spend a lot of his time being ill, and we have to find a way to manage it.
“If there is an alternative,” she said, “let’s explore it.”