Fragile Beginnings

by Adam Wolfberg, MD

  When Larissa was about two, a pediatric orthopedist noticed that Larissa’s right leg had some hypertonicity. Lacking the governance of a complete set of neurons from the left side of her brain, the muscles had a tendency to fire unopposed, which led to a generalized tightness. (Corticospinal tract neurons not only stimulate muscle contractions but also regulate reflex motion, and when they are injured the result is spasticity or hypertonicity. This is why children with profound cerebral palsy are contorted by muscles contracting without the brain’s regulation.) It was subtle—we had not noticed—but it was clear that the calf muscles of her right leg were pulling her foot down, and the muscles behind her thigh were pulling her leg into a bent position.

  “Let’s start with an AFO,” the orthopedist said, using the acronym for a thin plastic brace that keeps the foot flexed at the ankle. “Depending on how she develops, there are other things we can do.”

  “Like what?” I asked.

  “We’ve seen a lot of success with Botox,” he said, referring to botulinum toxin, the same thing that is used to make wrinkles go away temporarily. In kids with hypertonicity, the Botox is injected into the muscles that are overfiring, causing a partial paralysis that allows the other muscles to compensate. “The downside is that, with Botox, we aren’t really training the brain to compensate for this hypertonicity; we’re just shutting down the ability of those muscles to contract.”

  “What are our other options?”

  “Well, we can always do a tendon-release surgery.” This procedure lengthens the tendon connecting the muscle to the bone, compensating for the uncontrolled contraction of the muscle that has shortened its length and caused the foot to tip downward.

  We went down to the brace shop in the basement of Children’s Hospital where Larissa’s foot and leg were measured, and when we returned three weeks later, Larissa was fitted with a thin plastic device, the AFO, that hugged the back of her calf, the back of her ankle, and the underside of her foot, with straps coming across the front; it was decorated with pictures of Tweety Bird. Her foot still fit inside her sneaker, and its rigidity prevented her foot from tipping down past the flattened position. This would be the first-line therapy for her hypertonicity.

  Over time, Larissa clarified for us the enormously ill-defined prediction of the neurologists during her first week of life. Her major disability would be her right hand. Once a few months of development had refined her volitional movement, it became clear that “Righty,” as her hand was known to her occupational therapists and to us, would open and close only slowly, without precision or strength.

  Even now I try to imagine what it must feel like to have to think consciously about opening one’s right hand. I have wondered if it’s like the sensation I get when I sleep in an awkward position and wake up to find my arm has “fallen asleep” due to a temporary loss of blood flow that prevents me from moving my hand normally. Once I asked Larissa what it felt like, and she gave me a quizzical look. “It feels like Righty,” she said.

  “It will be an assist,” her neurologist said. “But I don’t think she will get more than that out of her right hand.”

  I held Larissa’s right hand for moral support while the left arm was wrapped first in cotton and then in dampened fiberglass strips that quickly hardened. Given a choice of turquoise, green, or pink, Larissa, now five, had chosen pink, and a layer of stretchy adherent material was wrapped around the cast, making her left arm extremely bright. Larissa’s gaze showed both fascination and trepidation.

  Then we were off to our furnished apartment in a comfortable but nondescript apartment complex in Birmingham, Alabama, where we were to stay for a month while Larissa went through a special therapy program. We climbed the steps, already beginning to sweat in the heat of the Birmingham summer. Air-conditioning units buzzed around us, working overtime to keep the apartments cool.

  Reggi Lutenbacher, who would be Larissa’s occupational therapist for the next month, was close behind us. We helped her bring large bins filled with toys up from her minivan: Connect Four, Yahtzee, Trouble, and other games requiring fine-motor manipulative skills, along with a child-size table and a chair. The living room became a fine-motor therapy space.

  Each morning at seven, Reggi would knock on the door. Thirty-two weeks pregnant with her first baby, Reggi had charm, enthusiasm, and an unselfconscious way about her. Smiling and ebullient, always upbeat, Reggi immediately got down to business. “Good morning, Larissa. Would you like to get dressed first or have breakfast first?” The choice didn’t matter, because both activities would be converted into a fine-motor-skill-building task with real-world utility.

  “Remember, Larissa, elbow down by your side. I want to see thumb and pointer holding on to that knob. Now pull, Larissa. Squeeze hard and pull. I’ll be your left hand over here on the other knob.” She compensated for Larissa’s left hand, which was casted and inaccessible.

  “Good job, Larissa” was a constant refrain, heard at least a dozen times each hour.

  Tasks were discrete—dressing, brushing teeth, playing games—and never lasted more than twenty minutes; the motions were repetitive, designed to improve range of motion, strength, and dexterity of Larissa’s right hand.

  “Good, Larissa,” Reggi cooed. “Now with the next grape, remember, elbow at your side, get the grape between thumb and pointer, and then turn your hand over. I want you to see that dot each time before you put the grape in your mouth.” Reggi had painted a dot on Larissa’s wrist just up from her pinkie finger, and the dot could only be seen when Larissa completely rotated her forearm, against the muscle tightness caused by her brain injury, so that her palm faced the sky.

  Our prior experience with physical and occupational therapy were the sixty-minute sessions twice a week at Children’s Hospital. Longer sessions weren’t considered—no one really thought a five-year-old could tolerate more than an hour at a time without becoming restless.

  “Have you had enough of Connect Four, Larissa?” Reggi asked when Larissa started to fidget and lose focus.

  “Yes, Reggi. Can we do something else?”

  “Let’s do our weight-bearing exercises, Larissa. But first, let’s clean up Connect Four.” As Larissa and Reggi put the pieces back in the box, Reggi gently cued her. “Remember, Larissa, elbow by your side.”

  We were in Birmingham for Larissa to participate in the Constraint-Induced Therapy program at the University of Alabama at Birmingham, a seriously intense and uniquely effective therapy program that was the result of research conducted by iconoclastic psychologist Edward Taub.

  Taub’s original research in macaque monkeys grew out of a surprising observation: If the afferent, or sensory, nerves of a monkey’s arms are cut, leaving the monkey without sensation in both arms, the monkey learns—over a period of days—to cope. Movements are somewhat more clumsy than they were prior to the loss of sensation, or deafferentation, but the monkey quickly relearns how to do everything, from swinging on branches to feeding and grooming. However, if the afferent nerves are cut on one side only, the monkey stops using that arm entirely.

  Scientists were puzzled. Neuroplasticity was obviously working for these monkeys—they quickly compensated for the loss of sensory stimuli when both arms lost sensation and they had no choice. They used other sources of information—particularly their vision—to help their arms accomplish necessary tasks. So why didn’t neuroplasticity help a single deafferented limb regain function?

  Taub’s theory was that a process of learned nonuse was at work. After one-sided deafferentation, it was easier for the monkey to learn to accomplish tasks with its unscathed arm then it was for it to learn how to use the arm lacking sensation. In essence, the monkey learned not to use the damaged arm.

  Working in his laboratory in Silver Spring, Maryland, Taub took an adult monkey who had had a one-sided deafferentation and was getting along nicely with its o
ther hand, entirely ignoring the deafferented limb, and he put the good limb in a cast so the monkey couldn’t use it at all.

  Nearly immediately, the monkey began to use its deafferented arm, presumably because it had no choice. Neuroplasticity kicked in, overcame the learned nonuse, and within hours, the monkey was accomplishing routine tasks with the deafferented arm. In subsequent experiments, Taub showed that if the cast was removed after a couple of days, the monkey went back to using the normal limb, and the deafferented limb was once again neglected. However, if the cast was left on for five or six days, the monkey regained function of its deafferented limb and continued to use it even after the cast was removed from its normal arm. The deafferented limb never became normal, and was always slightly more clumsy than the unscathed limb, but the regained function was significantly impressive nonetheless. The monkeys’ ages didn’t seem to matter, although Taub wondered whether younger monkeys would demonstrate even more plasticity than the older ones.

  Taub thought he had a treatment that would work with stroke victims, but just as he was about to move his research into humans, politics intervened. Alex Pacheco, a graduate student at nearby George Washington University, volunteered to work in Taub’s lab caring for the monkeys. Unbeknownst to Taub, Pacheco had recently helped to found People for the Ethical Treatment of Animals (PETA), a militant animal rights group. Pacheco was a spy.

  Pacheco took photos of the animals and secretly invited sympathetic veterinarians to visit in the dead of night. Finally, he called the state police to report cruelty to animals. The police raided the monkey facility, impounded the animals, and arrested Taub, charging him with 119 counts of cruelty to animals. A media storm erupted, the National Institutes of Health canceled Taub’s grants, and the scientific community split, with loud advocates of both animal rights and scientific research publishing letters to the editor and making speeches.

  At trial, it came out that Pacheco had probably created the unsanitary conditions he reported by not cleaning the cages and not caring for the animals during a two-week period when Taub was on vacation; it was also found that some of the photos he had submitted to the police and subsequently leaked to the media were doctored. Of the original 119 counts, 113 were dismissed, and the other 6 were dismissed on appeal. Eventually, the NIH reinstated Taub’s grants, but the damage to the research had been done.

  In an interesting coda to the story, the monkeys became the subject of a multi-party custody battle, and most of them ended up being euthanized. At autopsy, pathology studies of the brains demonstrated significant evidence of the neuroplasticity that Taub had anticipated, providing important evidence that plasticity exists in adult mammals.

  In 1986, five years after the police raid, Taub was offered a grant and a position at the University of Alabama at Birmingham, and he reopened his research there, this time focusing on adult stroke victims.

  The protocol was similar to the monkey experiment. Each stroke victim signed a contract stating he would keep his unaffected arm in a sling for greater than 90 percent of his waking hours. For six hours each day, the stroke victims participated in shaping activities, a special type of therapy designed to increase a limb’s function by breaking down the desired activity into its component parts and then building the activity up, one step at a time.

  Taub’s stroke victims developed connections too, although the anatomy of the connections (was it spinal cord or cortex?) was not entirely understood.

  His papers were groundbreaking. Practitioners in the field of neurology had believed that function lost to stroke was gone forever, but here were Taub’s research subjects regaining significant function. (Taub compared his subjects with a group of stroke victims who’d received conventional therapy and demonstrated what was well known: traditional therapy did little for these disabled men and women.)

  Applying the same principles of constraining the adapted behavior and working on the lost function, Taub demonstrated the effectiveness of his program for stroke victims, victims of traumatic brain injury, patients with multiple sclerosis, and even those with a loss of speech. For these last individuals, he insisted that they not use the mechanisms they had developed to compensate for their inability to speak: signing, motioning, writing notes. Then he developed a set of playing cards with a series of pictures that required increasingly complex speech to articulate and had the research subjects play Go Fish.

  In 1995, he published a paper suggesting that his constraint-induced therapy program might be useful for children with cerebral palsy or very early stroke. He wondered whether the significant neuroplasticity present in children might make the program even more successful than it had been in adults.

  A doctoral research assistant, Stephanie DeLuca, took on the project, and they set about designing a research protocol. However, the children’s hospital at UAB would not permit the children to have their unaffected arms restrained around the clock, and the two sides were at an impasse; the hospital called full-time restraint unethical, and Taub refused to run the program with only part-time restraint for fear that doing so would prevent the children from unlearning their learned nonuse that was a foundational principle of his work.

  Eventually, a respected therapist from an outpatient therapy program nearby offered to supervise the constraint, and the program began: four weeks of constraint, with intensive therapy using the shaping methodology for six hours each day and a constant focus on useful tasks.

  “Parents will say to us, ‘There is no way my child can do six hours of therapy a day,’ ” said Dr. DeLuca, who now runs the program that Larissa was in. “We don’t accept that.” What she found was that parents had more trouble watching their children struggle—even if the children ultimately succeeded—than the children had doing it.

  The results were relatively profound: compared to children who participated in traditional therapy, children who participated in the CI program showed a greater gain in function that lasted for a longer period of time. Most meaningful to Taub and his associate was that the children in the program and their parents reported that they were able to do more things that improved their quality of life than they had been able to accomplish previously.

  Hour after hour, in our temporary Birmingham living room, Larissa and Reggi worked on the same exercises, strengthening muscles, trying again and again to accomplish the pinching, grasping, reaching, and turning that would recruit neurons to the task of expanding the strength, range, and dexterity of her right hand.

  We quickly adopted Reggi’s phrases: “Whoa, Joe” when Larissa did something particularly impressive, like carrying a cup of water across the room. Or “Now, Larissa, you’re all catywhompus” when Larissa took a disorganized approach to opening the refrigerator door instead of doing as Reggi had shown her: lining herself squarely up in front of the door, bringing her elbow to her side, rotating her arm, and grasping the door handle.

  By far the most skilled occupational therapist Kelly or I had ever met, Reggi never tired, never lost her patience, and never had a situation she couldn’t redirect into an activity that enhanced the function of Larissa’s right hand.

  Each day, after six hours without a break, during which time Larissa dressed, made breakfast and lunch, played countless games, and did strengthening exercises, Reggi said good-bye until the next morning.

  A month later the cast came off, and the effect of neuroplasticity was apparent: Larissa’s right hand could rotate further, lift more, and operate with more precision than it had when we’d arrived in the stifling heat of Birmingham.

  “The way that neuroplasticity occurs is with repetition,” explained Dr. DeLuca. “And through repetition, the activity becomes permanent. That’s how the brain works.”

  Sarah Habib, the New Hampshire preemie who had survived against all odds, also went home, a hundred days after her precipitous birth. In many ways she was like any other newborn. But in her case, she did
n’t grow. At a year, she was barely ten pounds, and while most children at that age are thinking about walking or trying to, Sarah had difficulty sitting up.

  Sounds came too—all babies play with their voices—but most babies’ sounds turn into imitations and then coalesce into repetition and words; Sarah’s didn’t change. Sometimes it seemed like she was trying but just couldn’t form the words. Was it a motor problem? Did the muscles that control her voice lack coordination due to her brain injury? Or was it a cortical abnormality in one of the speech centers of the brain? It was so difficult to sort out at such an early age.

  By the time Sarah was two, Kim and David were beginning to understand the consequences of her injuries: cerebral palsy affected movement on both sides of her body, her speech, and her ability to eat (at about that time she had a feeding tube surgically placed in her stomach). Sarah’s vision was meager, and she had only a couple of words.

  Sarah received the same set of services that Larissa did—occupational therapy, physical therapy—and she had innumerable visits with doctors, from orthopedists to neurologists. Kim also had confidence in alternative therapies. While Sarah was still in the NICU, a good friend who was a Reiki master came and performed a Reiki ceremony on Sarah to channel good energy to the tiny child in her incubator.

  Once home, Sarah continued to receive alternative therapy. For a while Kim took her regularly to western Massachusetts to visit a practitioner there who performed craniosacral therapy to get energy to flow between the brain and the rest of Sarah’s body. Closer to home, she had visits with another practitioner who worked with her fascia and viscera to help “realign” her. “We were just trying to see if there was anything we could do,” Kim said.