Hysterectomy and growth attenuation: Therapy for disabled girl sparks debate

Parents hope 'Ashley treatment' will mean comfortable life at home

Much of America heard about the "Ashley treatment" in late 2006, upon publication of an article detailing the growth-attenuation measures taken in 2004 in the case of a then-6-year-old, severely developmentally disabled Seattle-area girl whose parents sought medical help that might ensure that she could be comfortable and that they could care for her as she grew older.

The only trouble, says Doug Diekema, MD, MPH, of the Treuman Katz Center for Pediatric Bioethics at Seattle (WA) Children's Hospital and Regional Medical Center, is that there is no such thing as an "Ashley treatment," but rather three separate measures evaluated individually on the basis of their benefit to the child and approved by a 40-member ethics review team.

But the story of Ashley, reported by Diekema and pediatric endocrinologist Daniel Gunther, MD, MA, in the October issue of Archives of Pediatrics and Adolescent Medicine framed a new debate over old, controversial treatments — high-dose estrogen treatments to intentionally attenuate height, and hysterectomy in disabled females.1

Ashley, known publicly only by her first name, is now 9 years old and cared for by her parents at home, where she is "an integral, and much loved, member of the family," Diekema and Gunther write. However, static encephalopathy with marked global developmental deficits caused her development to never progress beyond infancy. She cannot sit up, walk, or talk; she is fed via gastrostomy tube, and the specialists treating her say there will be no significant improvement in her cognitive and neurologic state.

When she started showing signs of puberty at age 6 (breast buds, pubic hair), her parents became concerned about the effects of growth and maturation on their ability to care for her and keep her comfortable. Lifting an immobile 7 year old is difficult; maneuvering an immobile adult at home would prove very difficult. Fully developed breasts would interfere with Ashley's ability to comfortably wear the harness that allows her to sit up, and menses would make hygiene additionally difficult for her caregivers and uncomfortable for Ashley.

For those reasons, with the support of Ashley's physician (Gunther), the family asked that Ashley undergo a hysterectomy, that growth attenuation be initiated, and that her breast buds be removed.

Individually, these procedures are not unheard of and are even common in very developmentally disabled people, says Diekema. What made this case unusual was the family's and physician's choice to have the multiple procedures vetted by the hospital.

"What made it unique is that it came to the ethics committee [at Children's Hospital] without there being a disagreement between the parents and physicians," says Diekema. "This came to us not as a dispute, but as a physician supporting the parents, saying, 'We need a broader look at what is happening here.'"

What the ethics committee was asked to consider were three separate procedures, any of which could be accepted or rejected independent of the other two. The most commonly performed in developmentally disabled children is a hysterectomy, with preservation of the ovaries. The most uncommon is surgical removal of breast buds, which Diekema says is done on occasion but is rare in the absence of pathology.

"Somewhere in the middle is growth attenuation," a procedure that is both common and historically controversial.

Attenuation of growth

In an response to the report by Diekema and Gunther, physicians Jeffrey P. Brosco, MD, PhD and Chris Feudtner, MD, PhD, MPH, wrote in the same issue of Archives that they opposed the practice of attenuating growth in profoundly impaired children, posing four questions:2

  • Will stopping Ashley's growth successfully make her easier to care for at home?
  • Is such "heavy-handed" manipulation to produce a smaller person acceptable?
  • Can the treatment be abused?
  • Is attenuating growth the correct way to address the problem of caring for impaired children and adults?

Growth attenuation achieved by high-dose estrogen (most commonly, oral ethinyl estradiol) has been used for decades, notably in the 1960s to curb the growth of girls who were expected to grow "too tall" for then-acceptable ideas of normal female height.

"Not everyone would say it is appropriate to attenuate a [healthy] girl who is going to grow to be 6 feet tall, just because she's tall," says Diekema. "But it's still done."

In Ashley's case, her parents have said on the web site they created to tell her story (www.ashleytreatment.spaces.live.com), growing tall vs. remaining small could be the difference between having to live her life in a skilled nursing facility and living at home in the care of her family.

"The main benefit of the height and weight reduction is that Ashley can be moved considerably more often, which is extremely beneficial to her health and well-being. Currently, one person can carry Ashley [who is 4'5" tall and 65 pounds], vs. requiring two people or a hoisting harness and ropes, should she have grown larger," her parents report on their blog.

"As a result, Ashley can continue to delight in being held in our arms and will be moved and taken on trips more frequently and will have more exposure to activities and social gatherings [for example, in the family room, backyard, swing, walks, bathtub, etc.] instead of lying down in her bed staring at TV [or the ceiling] all day long. In addition, the increase in Ashley's movement results in better blood circulation, GI functioning (including digestion, passing gas), stretching, and motion of her joints."

Diekema says that arguments against attenuation in this case that insist the attenuation was merely to convenience Ashley's parents are only seeing one part of the picture.

"We had to weigh the harm that each procedure might have against the benefits that accrue to the patient," Diekema says. "What you have to understand is that not all the benefits that accrue to her accrue because it makes her parents' lives easier.

"But having her light enough to be picked up means she gets picked up."

Ashley's parents say that their being able to lift, move, and position Ashley means she can continue to join her parents and two siblings on family outings, something they say is important to all of them.

"It seems to me that while people will represent this case [as either] the decisions were being made for the patient's good, or for the good of the people or person making decisions for that patient, we must recognize that the two are hardly exclusive," points out John Banja, PhD, of Atlanta's Emory University Center for Ethics. "Indeed, it might well be that what is in Ashley's interest will turn out to be what is in her caretaker's interest, and vice versa. A common rehabilitation goal is reducing caregiver burden, such that both the caregivers and the one cared for can be better off."

Hysterectomy and breast bud removal

Historically, hysterectomy has not been uncommon in severely developmentally disabled women in whom childbearing would never be a consideration. Growth attenuation is considered less risky when done in conjunction with hysterectomy, which eliminates the need for progesterone during the attenuation treatment and reduces the risk of thrombosis.

However, hysterectomy has been used in the past as a means of sterilizing "undesirable" potential mothers — women who were mentally ill, mentally retarded, or deemed morally "deficient." The difference between this eugenic use of hysterectomy — sterilization for the "improvement" of society — and the surgery done in Ashley's case is that it was done for no one's benefit but the patient's, Diekema says.

Hysterectomy eliminates the complications of menses, her physicians and family say, as well as freeing her from the risk of uterine or cervical cancer. Retention of her ovaries offers her some protection from developing osteoporosis. Prophylactic appendectomy was performed at the same time.

There is little in the literature to indicate how often breast bud removal is performed, but Diekema suspects it is not all that rare. Ashley's parents have said that besides making her support halter more comfortable to wear, they hope the lack of developed breasts will prevent Ashley from being "sexualized" by caretakers later in her life. Additionally, she will avoid a family tendency to develop painful fibrocystic growths.

Banja asks whether, because Ashley is not "for herself," as ethicists would say — she cannot direct or plan her life — her body and its organic functions, especially as they define her gender, are ambiguous contributors to her "dignity" as a person.

"So, is she more or less dignified by the fact that it is more or less difficult for her caretakers to provide for her needs? This seems a core question," Banja says. "And it will be on this count, I think, that we will assess how successful the plan has been as the years go on."

There also are legal issues in some states with performing hysterectomies or sterilization on minors without a court order. That was not the case in Ashley's procedure, Diekema says. But that's not to say there weren't concerns on the part of Children's Hospital attorneys and administrators.

"In most hospitals, how does this play out? In most hospitals, barring some legal barrier, like a barrier to hysterectomy in a minor, the child would be brought to a physician, the physician would say, 'It sounds like this should happen,' and if appropriate, they would proceed," Diekema suggests. "In many ways, what made [Ashley's case] unique was that we didn't just proceed. We stopped the train and said, 'All three of these procedures are unique, so we need to review them before we proceed.'

"One of the distortions [in published reports of the case] is that there is such a treatment as an 'Ashley treatment.' We approached this as three separate requests — growth attenuation, breast bud removal, and hysterectomy."

While much innovative therapy that occurs in U.S. hospitals "just happens," Diekema says, at Children's Hospital the medical director is involved in any innovative treatment or therapy, to monitor "reasonableness" and call in the ethics committee when needed.

"But I don't think there was a single member of the ethics committee who didn't realize this was very complex from an ethical standpoint," he says. "For any one of the requests, there were arguments and counter arguments, where we had to figure out how she stood to benefit and any potential harms that could come to her. And there was consensus that all three of them, separately and together, offered benefits to her quality of life."

If there was a bias at the outset of the ethics committee's study of the treatments proposed for Ashley, it was a bias against doing the procedures, Diekema recalls.

"Taking the uterus out of anyone who hasn't given their consent — you walk out of the room saying, 'That was a hard decision,'" he says. "It was difficult to see how [doing the procedures] would benefit the hospital. We knew it could create adverse publicity.

"If you were a risk manager in that room, you'd be saying 'Why risk having people picketing outside your hospital?' So people on the committee had to be convinced it was the right thing for this little girl."

Ultimate success seen later

Banja points out that advocates for the disabled, as well as some in the medical community, likely view what happened to Ashley as a "mutilation" performed for the convenience of others.

"Aside from the symbolism of that mutilation, however, we lack an empirical metric to demonstrate how much burden or benefit has practically been created," Banja suggests. "Ashley will never be able to tell us, so we will have to wait and see whether the interventions that occurred ultimately rebound to the benefit of her parents and, perhaps, to her."

Ashley's parents, along with her treatment team, stress that the therapies used for their daughter are not suited or appropriate for mildly disabled children.

"Some articles fail to clarify up front that our daughter is severely disabled, in a small and extreme category of disability, leaving their audience with the image of a mildly disabled girl — which is what most people have a direct experience with — which would indeed make the Ashley treatment shockingly inappropriate," her parents write on their web site.

Some ethicists would say that given Ashley's level of retardation, her life is, in a sense, not exactly hers — she is not "for herself" — because she cannot decide, plan, or direct her life, Banja adds.

"Ashley's life largely exists for others, especially her parents, on whom she categorically depends," says Banja. "The difficult part, I think, is managing our very uncomfortable feelings that are aroused by the surgical interventions."

References

  1. Gunther DF, Diekema DS. Attenuating growth in children with profound developmental disability: A new approach to an old dilemma. Arch Pediatr Adolesc Med 2006; 160:1,013-1,017.
  2. Brosco JP, Feudtner C. Growth attenuation: A diminutive solution to a daunting problem (Editorial). Arch Pediatr Adolesc 2006; 160:1,077-1,078.

Sources

For more information, contact:

  • John D. Banja, PhD, Assistant Director for Health Sciences & Ethics, Emory University Center for Ethics, Atlanta, GA. Email: jbanja@emory.edu.
  • Douglas Diekema, MD, MPH, Professor, Educational Director, Treuman Katz Center for Pediatric Bioethics, Seattle, WA. Email: diek@u.washington.edu.