SOMETHING about Ashley, the severely disabled 9-year-old American girl, has touched a nerve in the US and beyond. For more than two years she has been receiving high doses of hormones to stunt her growth, and she has had radical surgery to remove her breast buds and uterus to ensure she does not mature sexually.
Ashley’s parents insist these measures are for her benefit – that they will enhance her quality of life while they continue to care for her in their own home. Others accuse them of subjecting their daughter to potentially dangerous treatments for their own convenience, and denying her what they say is her right to grow up. There is unlikely to be a definitive answer, but the debate has shed unexpected light on the dilemmas facing those responsible for the welfare of severely disabled children.
Ashley’s case is unprecedented in that the precise combination of hormones and surgical interventions she was given have never before been documented in medical literature (see “An infant forever”), although doctors have previously used hormones to limit the growth of particularly tall teenage girls. This means the decision to intervene had to be taken without clear evidence of exactly how this package would affect her prospects. “There really is no solid evidence to suggest what the right thing to do is, or to confirm that it has had the desired outcome,” says Robert Sade of the Medical University of South Carolina in Charleston.
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This, however, is a frequent problem. “It is probably true of about 90 per cent of what we do in medicine,” says Sade.
The urgency and complexity of many surgical procedures mean they are often carried out without evidence to inform the decision whether to go ahead. A study published last month found just eight prospective randomised controlled studies – the gold standard for most clinical trials – out of a total of 135 studies published in the journals Archives of Surgery, Surgery, and Annals of Surgery over three months (Journal of the American College of Surgeons, vol 203, p 827).
In other respects, too, Ashley’s case may not be unique. Alan Rogol, a paediatric endocrinologist at the University of Virginia, Charlottesville, says he knows of at least two other instances in which high doses of oestrogen have been used to halt a disabled child’s growth. There are also precedents for the hysterectomy performed on Ashley. Bioethicist and paediatrician Douglas Diekema of the Children’s Hospital and Regional Medical Center in Seattle, Washington, where Ashley was treated, says it is not uncommon for families to seek hysterectomies for girls in Ashley’s position, though in many states this cannot be done without court approval.
“In at least two other instances, high doses of oestrogen have been used to halt a disabled child’s growth”
Less severe interventions to improve the quality of life of disabled children are relatively common. For children with a cleft palate, surgery can improve their speech. Cosmetic repairs to a cleft lip are also routine. Parents of children with Down’s syndrome or Beckwith-Wiedemann syndrome sometimes have them undergo tongue reduction, for functional and cosmetic reasons. “It is uncontroversial to improve quality of life,” says paediatrician Chris Feudtner of The Children’s Hospital of Philadelphia. Operations on disabled children to correct excessive drool are another example.
Ashley’s case attracted only limited public attention in October 2006, when the doctors involved published details of her treatment and the decision-making process behind it in the journal Archives of Pediatrics & Adolescent Medicine (vol 160, p 1013). It was not until Ashley’s parents went public and posted their experiences on a blog () on 2 January that the wider world became fully aware of it and began to debate their decision.
In most cases like Ashley’s, the decisions are rarely held up for public inspection because in the US they typically involve only doctors and parents, though things can be different elsewhere (see “When caring costs too much”). Where the intervention is considered to be research, federal law requires it to be overseen by the review board of the hospital or university concerned. Most cases involving individual children are not classified as research, however, and ethics committees such as the one at Seattle Children’s Hospital that discussed Ashley’s treatment vary in terms of how often they are used and what authority they have.
“Very few ethical committees actually have a power of decision-making,” Sade says, and they usually try to keep the decisions in the hands of the parents. “We really only take the rights away when what the parents want to do constitutes child abuse,” says David Magnus, a bioethicist at Stanford University in California. It is often a judgement call as to whether such a committee should be consulted. “Any surgeon at any time can decide to do a procedure on somebody,” depending on an institution’s guidelines for novel procedures, Diekema says.
In Ashley’s case, the review process and the circumstances that led those caring for her to decide she was an appropriate candidate for the treatment she received have been laid out for all to see. “It’s admirable they were so public,” says Feudtner, even though he expressed reservations over the experimental nature of her treatment in an editorial accompanying the publication of her case (Archives of Pediatrics & Adolescent Medicine, vol 160, p 1077). “You want the debate to be in public.”
When caring costs too much
“Why are loving parents being put in the position where they have to consider this type of treatment?” asks paediatrician Chris Feudtner of The Children’s Hospital of Philadelphia. In some cases, it comes down to the resources the family can call on.
In the US, public healthcare provision rests largely in the hands of individual states. When families need special equipment – such as a hospital-type bed, bathing equipment, or a lifting harness – to care for someone like Ashley, they face a scramble to find resources. “The system isn’t rational,” Feudtner says. “You’ve got to know Dave at the Department of Whatever, and if you don’t know Dave, you’re screwed.”
Even tougher is finding qualified caregivers to offer relief to the families in their own homes. “We have a home-care crisis in this country,” Feudtner says. In their blog, Ashley’s parents write: “We tried hard and found it impossible to find qualified, trustworthy and affordable care providers.”
Parents of profoundly disabled children like Ashley see the future bearing down on them “like a slow-moving freight train”, says Feudtner. “The child is getting older, the parents are getting older. You see it coming down the track.” When parents can no longer cope, their child is placed in a state or private long-term care facility. While state and federal funding through Medicaid, and any health insurance, may help, the costs can still reduce a family to poverty.
The situation is likely to be better in the UK, where free healthcare, social support and educational provision for disabled children are guaranteed by law. How much better depends on which part of the country you live in, says David Congdon, head of policy at the learning-disability charity Mencap, since access to social service provision varies from region to region. “It’s a real battle to get what you want,” he says.
In the UK, looking after children with learning disabilities costs the state around £4.5 billion a year – excluding the costs of special education. The most expensive outcome of all is if the child has to go into permanent care, which costs up to £200,000 a year. Whether cost considerations would ever be used to justify giving a British child similar treatment to Ashley’s remains an open question. “The whole concept would be contingent on the best interests of the child,” says Richard Parnell of SCOPE, a charity supporting people with cerebral palsy. Under UK law, the welfare of the child is paramount, so parents would have to have a strong case for the intervention to avoid a legal challenge by the state on the child’s behalf.
Additional reporting by Andy Coghlan
An infant forever
Ashley has been severely disabled since shortly after birth and is thought unlikely ever to be able to function beyond the level of an infant. Aged 9, she can’t sit up or hold a toy, or even recognise her family. The source of her disability is a condition called static encephalopathy, or unchanging brain damage, the cause of which is unknown.
Yet Ashley is expected to have a normal lifespan, and for this reason her parents argue that she will be better off if she stays small. Because this will make her easier to move, she will be moved more often and taken along on more family outings, they say. She will be less likely to develop bedsores and other health problems. They also suggest that a small body may be a more appropriate match for her mental age.
Whatever the rights or wrongs of Ashley’s medical treatment, it is already over. For two-and-a-half years she has worn contraceptive skin patches that delivered high doses of oestrogen. This triggered early puberty and the fusion of growth plates in her bones, so they stopped growing. At the end of the treatment a few weeks ago, X-rays showed that she had the bone age of a 15-year-old. At 135 centimetres tall, she is estimated to be some 33 centimetres shorter than she would otherwise have been as an adult, and is about 23 kilograms lighter.
The removal of Ashley’s uterus will prevent the bleeding and potential discomfort of menstruation, and remove the risk of pregnancy were Ashley to be sexually assaulted by a future caregiver – as has happened to some severely disabled women in care. The removal of her breast buds – the beginnings of breast growth – was justified on the grounds that many women in her family have large breasts, which might have caused Ashley discomfort and made it difficult to strap her into a wheelchair. These procedures also mean she will not suffer the high risk of breast or uterine cancer that the hormone treatment would normally cause. Ashley’s ovaries remain in her body and the oestrogen they produce will protect against osteoporosis, though her inability to bear weight as a result of her disability may still weaken her bones.
