Dr. Daniel Gunther and Dr. Douglas Diekema, who first revealed the details of "The Ashley Case" in the Archives of Pediatric and Adolescent Medicine, think that many of their critics don't understand the nature of this case. Talk to them, and you confront every modern challenge in weighing what medicine can do, versus what it should.
The case: Ashley is a brain-damaged girl whose parents feared that as she got bigger, it would be much harder to care for her; so they set out to keep her small. Through high-dose estrogen treatment over the past two years, her growth plates were closed and her prospective height reduced by about 13 inches, to 4'5". "Ashley's smaller and lighter size," her parents write on their blog "makes it more possible to include her in the typical family life and activities that provide her with needed comfort, closeness, security and love: meal time, car trips, touch, snuggles, etc." They stress that the treatment's goal was "to improve our daughter's quality of life and not to convenience her caregivers."
But the treatment went further: doctors removed her uterus to prevent potential discomfort from menstrual cramps or pregnancy in the event of rape; and also her breast tissue, because of a family history of cancer and fibrocystic disease. Not having breasts would also make the harness straps that hold her upright more comfortable. "Ashley has no need for developed breasts since she will not breast feed," her parents argue, "and their presence would only be a source of discomfort to her."
The parents say that the decision to proceed with "The Ashley Treatment" was not a hard one for them, but the same cannot be said for the doctors. "This was something people hadn't thought about being a possibility, much less being done," says Diekema, who chairs the bioethics committee of the American Academy of Pediatrics and was brought in to consult on this case. For the ethics committee of Seattle Children's Hospital, which reviewed the proposed treatment, "it took time to get past the initial response'wow, this is bizarre' and think seriously about the reasons for the parents' request," says Diekema.
First they had to be sure there would be no medical harm: removing breast buds, Gunther says, is a much less invasive procedure than a mastectomy. The hormone treatment was commonly used 40 years ago on lanky teenage girls who didn't want to get any taller. "The main risk," Gunther says, "is of thrombosis or blood clot, which is a risk in anybody taking estrogen. It's hard to assess in a young child because no one this young has been treated with estrogen." There were very few reports of thrombosis among the teenage patients, he says, "So I suspect the risk is fairly low. After treatment is finished, I don't see any long-term risk, and we've eliminated the risk of uterine and breast cancer."
The ethics committee essentially did a cost-benefit analysis and concluded that the rewards outweighed the risks. Keeping Ashley smaller and more portable, the doctors argue, has medical as well as emotional benefits: more movement means better circulation, digestion and muscle condition, and fewer sores and infections. "If you're going to be against this," Gunther says, "you have to argue why the benefits are not worth pursuing."
They knew that the treatment would be controversial, though they did not quite foresee the media storm that would erupt when they decided to publish the case and invite their peers to weigh in. "I felt we were doing the right thing for this little girlbut that didn't keep me from feeling a bit of unease," admits Diekema. "And that's as it should be. Humility is important in a case like this."
Gunther also understands why the case has inspired such intense feelingsbut notes that "visceral reactions are not an argument for or against." This was not a girl who was ever going to grow up, he says. She was only going to grow bigger. "Some disability advocates have suggested that this course of treatment is an abuse of Ashley's ‘rights' and an affront to her ‘dignity.' This is a mystery to me. Is there more dignity in having to hoist a full-grown body in harness and chains from bed to bath to wheelchair? Ashley will always have the mind of an infant, and now she will able to stay where she belongsin the arms of the family that loves her."
But how far would Drs. Gunther and Diekema take this argument? Would they agree to amputate a child's legs to keep her lighter and more portable? Hormone treatment is nowhere near as risky and disfiguring as amputation, Diekema retorts; it just accelerates a natural process by which the body stops growing. Parents of short children give them growth hormones for social more than medical reasons, he notes. How can it be O.K. to make someone "unnaturally" taller but not smaller? To warnings of a slippery slope, Gunther tilts the logic the other way: "The argument that a beneficial treatment should not be used because it might be misused is itself a slippery slope," he says. "If we did not use therapies available because they could be misused, we'd be practicing very little medicine."
On Tuesday, Part 2, critics of Ashley's parents and her doctors have their say.