Growth attenuation

When I visited Cloudcroft two summers ago, Ricky was small for his age — just 42 inches tall and 37 pounds, about the size of an average 4-year-old boy. In part this was because of genetics and his various health problems, but it was also partly a result of his having been through a controversial medical intervention known as growth-attenuation therapy. From the time he was 4 until just shy of his 7th birthday, he received doses of estrogen high enough to stimulate the premature closing of the epiphyseal or “growth” plates, the thin wedges of cartilage found at the end of the long bones in children and adolescents. (Long bones include the femurs, tibias and fibulas of the legs and the humeri, radii and ulnas of the arms.) The younger the child when treatment begins — usually between 3 and 6 years of age — the greater the reduction in height…

Then, in 2006, Douglas Diekema, the director of education at the Treuman Katz Center for Pediatric Bioethics at Seattle Children’s Hospital, and Daniel Gunther, a pediatric endocrinologist, announced in the Archives of Pediatrics and Adolescent Medicine that they had resurrected the treatment once known as estrogen therapy for a different population: what Diekema and Gunther described as “nonambulatory children with severe, combined neurologic and cognitive impairment.” In other words, children like Ricky Preslar, expected to rely on caregivers for every basic need for the rest of their lives. When such dependents enter adolescence and adulthood, the doctors posited, the simple tasks of caring for them — dressing, toileting, bathing, holding and carrying — can become prohibitively difficult for parents. Arresting a child’s growth could benefit both child and parent. A smaller person who required no hoisting apparatuses, Diekema and Gunther reasoned, would in all likelihood benefit from more attention and greater inclusion in family activities. And he or she would be more likely than someone fully grown and similarly impaired to be cared for in the home, rather than an institution. “We propose,” the authors concluded, “that in situations in which parents request such an intervention, it is both medically feasible and ethically defensible.”…

After Diekema and a hospital ethics committee gave their approval, Gunther administered the estrogen estradiol, which effectively reduced her future predicted height by approximately 13
inches.  Additionally, the child — unidentified in the original paper but soon known as Ashley, her first name, or “Ashley X” — underwent a hysterectomy to preclude the discomfort of painful menses as well as the uterine bleeding that can accompany estrogen in high doses, and the removal of her breast nodules to forestall the growth of breasts. For the treatment they called “growth-attenuation therapy” to be most effective — resulting in a shorter and lighter child — a careful monitoring of calories was also required…

According to the disability activists’ criticism, growth-attenuation therapy and its associated procedures violated Ashley X’s rights to privacy and freedom from unnecessary bodily manipulation, which are the sorts of things the Americans With Disabilities Act, the Rehabilitation Act and even the 14th Amendment were supposed to protect people from. Her care did not constitute a “treatment” or “therapy” at all, they said, because it did not treat an illness. A statement issued by the American Association on Intellectual and Developmental Disabilities claimed that “this practice, if judged acceptable, will open a doorway leading to great tragedy.”…

Still, the treatments were now a known option for the families of children with profound disabilities. They congregated on Pillowangel.org, a website and private message board maintained by Ashley’s parents, who have never made their names public, identifying themselves only as “Ashley’s Mom” and “Ashley’s Dad,” or AM and AD. (Their site’s name comes from the endearment given to Ashley and other children like her who spend most of their hours propped up by pillows in bed.) The carefully screened message boards became a supportive oasis in an online environment where, one Washington State mother told me, “parents looking for information about this are made to feel like monsters.”…

But then last summer, the Pediatric Endocrine Society published a survey of its members in The Archives of Diseases in Childhood indicating that at least 65 children have received the therapy. (The survey did not break down participants by sex or note whether they had hysterectomies or breast-bud removal.) And by many doctors’ accounts, the demand for it is increasing even as doctors prefer not to talk about the practice publicly…

Kappy initially prescribed 2 milligrams daily of estradiol, but on the private message boards at Pillowangel.org, Cindy learned that there were no regulations on how much estrogen physicians could prescribe off-label; other children were getting much higher dosages than Ricky, and their bone age (measured by the degree to which the growth plates are closed) was increasing more rapidly, which would lead to an overall greater reduction in height. She persuaded Kappy to increase the dosage incrementally over the next three years to 8 milligrams daily. Kappy told me that he closely monitored Ricky “to be sure we weren’t going to do him harm.” The higher doses, he said, were “quite effective in speeding up the rate of skeletal maturation.” Within six months, Ricky’s bone age jumped three years, up to that of a 6-year-old. When he began to develop breast tissue, a side effect of the estrogen, Kappy told Cindy that Ricky could have his breast buds removed for cosmetic purposes….

“My daughter is trapped in a wheelchair all her life, and she’s more comfortable” being smaller, says Nancy, who believes her daughter, Nykkole, may have been the second girl ever to undergo the three interventions that make up the Ashley Treatment…

Like Ashley’s parents, she believed that if her daughter had no breasts, she would be less likely to be a target of sexual abuse. Before undergoing growth-attenuation therapy, Nykkole was given both a hysterectomy and a bilateral mastectomy at the University of Minnesota Masonic Children’s Hospital. When the family then met with the hospital’s ethics committee to discuss Nykkole’s growth attenuation, Nancy recalls, “We brought her out of her chair and into our laps, and said: ‘This is why we want to do this. She needs to be in our laps for our whole life.’ ”…

Ricky Preslar is now 9. Two years after completing growth-attenuation therapy, his height has stabilized around 43 inches. He is still nonverbal (unless you count his easy laugh), but in the past year, Cindy says, he has shown signs of purposeful movement, an important developmental milestone, by using a head-motion-activated assistive-communication device to stop and start videos at his school for the visually impaired. He can move around the kitchen in a gait trainer and goes to hippotherapy (a kind of physical therapy on horseback) once a week. Cindy’s goal this year is to run a marathon while she and her husband push him the whole way. She says that Ricky, who is now 43 pounds, is beginning to seem conspicuously smaller and lighter than many of his peers at school. The Preslars have never regretted their decision to attenuate Ricky’s growth. For Cindy, there are times — like when she recently strained her back — when she is particularly grateful that her boy is, and always will be, small.

http://www.nytimes.com/2016/03/27/magazine/should-parents-of-severely-disabled-children-be-allowed-to-stop-their-growth.html?_r=2

Courtesy of my mother.