Ohad Oren, a 3rd year medical student at Bruce Rappaport Faculty of Medicine, in Haifa, Israel considers an ethical dilemma.

A profoundly disabled child whose annual growth rate is subnormal approaches your care. The parents ask you to test growth hormone function and to consider an active intervention to help their child attain a larger stature. How should you proceed? Increased growth will surely not benefit a severely brain impaired child, with no trace of body image whatsoever. A larger stature, moreover, will impose a higher burden on his caregivers, hampering their continuous care of their child.

This paradoxical scenario - whether to use medical treatments to increase the final stature of a developmentally-disabled child - is not uncommonly encountered in pediatric endocrine clinics. Rejecting the GH hormonal therapy may portray abandoning an effective medical therapy on the grounds of little or no benefit to the patient. But, is offering parents of severely disabled children proactive measures to attenuate growth an ethical thing to do? The Ashley X case, presented in this article, sparked an intensive public discussion concerning the ethical justification of irreversibly altering the body of a profoundly disabled and nonconsenting child.

Ashley X, the core of the ensuing debate, suffers from static encephalopathy. According to her physicians, she would never progress beyond the developmental stage of a 3 month-old infant. (1),(2) At the age of 6, Ashley is completely dependent in all daily activities, being unable to walk, communicate, eat, sit up, or roll over. Her parents were concerned about her growth, which would make it “untenable for them to care for their daughter at home, despite their strong desire to do so”.(1) The parents and the physicians thoroughly deliberated the condition, and devised a treatment program using high-dose estrogen to attenuate final adult height (box). To reduce long-term complications of puberty, removal of her uterus and breast-buds was considered. An ethics committee carefully analysed the family’s reasons for their requests, and concluded that all were ethically appropriate (table 1).

Table 1

Potential benefits and harms inherent in each procedure of the Ashley Treatment (5), (6), (7), (8)

* DVT - Deep Vein Thrombosis.

** These adverse effects were demonstrated in constitutionally tall adolescent girls that were given high-dose estrogen, in trials done in the 1950’s. (9)

Risks not fully determined

The case of Ashley X was the first to present the question of whether hormone therapy should be used to make children with profound impairments smaller. We may still gain knowledge and perspective if we think about other medical treatments whose risk-benefit calculation may seem comparable to the case we discuss (fig 1). On the one hand, there are risky treatments that are taken prophylactically, in an attempt to avoid a life-threatening condition (i.e. mastectomy in BRCA carriers). Genetically-unfortunate women can weigh the risks and benefits, and reach a decision based on their values and beliefs. On the other side, prophylactic interventions, like immunization, carry high benefits, with risks that are extremely low. Which category could describe Ashley’s condition? You could argue that the risks involved in Ashley’s treatment are substantial, and not in proportion to the possible gains she might get. Or you could say, as her physician did, that “these risks do not seem to be unreasonable and are not out of line with the risks of other medical interventions these children undergo”.(5)

How, then, could risks be quantified, or at least estimated, when there were no children like Ashley undergoing growth attenuation therapy combined with hysterectomy and breast-bud removal? The only similar trials involving high-dose estrogen to stunt growth were treatments of constitutionally tall adolescent girls, wishing to minimize any further gain in height. (10) Ashley’s physicians extrapolated data from these 1950’s studies to support the efficacy and safety of the treatment. But Evidence-Based Medicine principles oblige the medical profession to seek conclusive scientific evidence prior to using a presumably efficient therapy. It was not yet shown that children with significant developmental disabilities who are shorter stay at home longer. In fact, we don’t even know whether keeping Ashley at home has any beneficial effect on her social or health status. Observational studies should test these issues, among others, to gain a better understanding of the therapy’s potential risks.

Parental motives

In most cases, parents are in the best position to make medical decisions for their child. It is believed that they understand and attempt to achieve the “best interests” of their child better than anyone else. Therefore, parents are given the legal and moral responsibility to care for their children. (11) Being Ashley’s legal guardians, her parents asked the doctors to perform an intervention that was neither a medical necessity nor a hitherto-recognized therapeutic option.

Now, try scrutinizing the Ashley case appropriateness through less shiny lenses, such as those of a developmental disabilities professional. It has been claimed that what we have here is an invasive, risky treatment, whose goals are essentially quality of parental life, and with the patient “good” defined by someone other than her.

The American College of Obstetrics and Gynecology (ACOG), in their paper “Sterilization of women, including those with mental disabilities” , notes that involuntary and nonvoluntary medical interventions and surgical procedures in general should only be provided when there are clear health benefits.(8) Ashley’s parents wish to take care of her, and stunting her growth, they argue, will make it easier for them to extend the time they can care for her at home, and enhance their child’s mobility, social interaction and physical contact with them (table 2). (5), (13)

This reasoning of the parents led some to claim that their own convenience was the primary rationale for attenuating Ashley’s size. They could have hired people to help them, or go to the gym, or purchase special devices for their home, instead of surgically modifying their child’s height. But, stating Judith Jarvis Thomson, “nobody is morally required to make large sacrifices, of health, and all other interest and concerns… in order to keep another person alive”. (12) In my humble opinion, as long as motives of convenience form only part of the parents’ justifications, with the child’s benefits as the center of gravity (in choosing the requested treatment), they should not be considered as distinct from those of the child. Consider, for example, a parent bringing a child with a toothache to the dentist, hoping that the child will undergo a lengthy procedure, so that he will earn a few hours of sleep. Do these latent motives make the procedure unethical? Of course not. (13) Further emphasized in a child with developmental disabilities is the complete dependency upon his parents for all his daily care. In circumstances such as this, the child’s interests and needs are intertwined with those of the parents. Defragmenting the family to pieces, to isolate and analyze the needs of the disabled child only, is analogous to prescribing medication based upon mere lab results, without seeing the patient.

Table 2

The pros and cons of the “Ashley Treatment” (5), (13)

Society’s role

In a perfect world, with unlimited social support resources, Ashley could be nursed and cared for in a normal size. In view of the options open to them, Ashley’s parents sought after the possibility that would enable them to improve their child’s (current and future) medical as well as social state.

If we target a fundamental change in the harrowing predicament that parents of such children face, medication is a possible adjunct, but solving the problem requires more funds for community-based living programs. If the collective community response proscribes the Ashley treatment, society must be prepared to provide the caregivers with enough assistance that they would not have to resort to these means. (13) As long as social support is lacking, however, the medical profession may offer its interventions, in cases like Ashley’s, taking into account her natural and social circumstances.

As I view it, what the Ashley Case showed was that there was much more needed discussion about the value of disabled children in our society. Many disciplines in medicine may help translate such challenging dilemmas into reasoned and balanced solutions. Specialists in pediatric endocrinology, neurology, surgery, development, and ethics contribute their perspectives on highly debatable issues such as Ashley’s. An interdisciplinary group of experts of that type may only further emphasize the pivotal discussion we must engage in regarding social attitudes toward people, in particular children, with neurologic and cognitive impairments.

Acknowledgements: I am deeply grateful for the critical and insightful review provided by Zeev Hochberg, MD PhD (Director, Pediatric Endocrinology Unit, Meyer Children’s Hospital, RAMBAM Health Care Campus, Haifa, Israel) as well as for his fascinating perspectives and ideas with regard to the ethical issue at hand.

Ohad Oren, 3rd year medical student, Bruce Rappaport Faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel. ohadoren@gmail.com

References 1-13:

1. The “Ashley Treatment“: Toward a better quality of life for “Pillow Angels”.
2. Coombes R. Ashley X: A difficult moral choice. BMJ 2007;334;72-73.
3. Fancher TK. Some observations on anterior lobe hyperpituitarism. Endocrinology. 1932; 16:611.
4. Kirklin OL, Wilder RM. Follicular hormone administration in acromegaly. Proc Staff Meet Mayo Clin. 1936; 11:121-125.
5. Gunther DF, Diekema DS. Attenuating growth in children with profound developmental disability. Arch Pediatr Adolesc Med 2006: 1013-7.
6. Bladbjerg EM, Skouby SO, Andersen LF et al. Effects of different progestin regimens in hormone replacement therapy on blood coagulation factor VII and tissue pathway inhibitor. Hum Reprod. 2002;17:3235-3241.
7. Girolami A, Spiezia L, Rossi F, Zanon E. Oral contraceptive and venous thromboembolism: which are the safest preparations available? Clin Appl Hemost. 2002;8:157-162.
8. Committe on Ethics, American College of Obstetrics and Gynecology: Sterilization of Women, including those with Mental Disabilities.
9. Lee J, Howell J. Tall Girls; The Social Shaping of a Medical Therapy. Arch Pediatr Adolesc Med. 2006;160:1035-1039.
10. Goldzieher MA. Treatment of excessive growth in the adolescent female. J Clin Endocrinol Metab. 1956; 249-252.
11. Kopelman LM: The best-interests standard as threshold, ideal, and standard of reasonableness. J Med Phil 1997;22:271-289.
12. J. J. Thomson, “A defense of Abortion” in Ethics in practice, ed. by H. Lafollette (Oxford, UK: Blackwell, 1997), 69-78, at 77.
13. S. Matthew Liao, J. Savulescu, M. Sheehan. The Ashley Treatment: Best Interests, Convenience, and Parental Desicion-Making. Hasting Center Report 37, no. 2 (2007): 16-20.

 Matthew Kirkman discusses the influence of the pharmaceutical industry on drug prescription practices. 

Of the total NHS budget in England (£78 billion), over £7 billion is spent on drugs alone. (1) 80% is spent on branded drugs, which make up the minority of prescriptions by volume. (2)  General practitioners (GPs) in the UK write approximately 650 million of the total 678 million prescriptions every year and, as such, are a big target for pharmaceutical companies hoping to push their products onto the market. (2) (3) At a time when minimising cost has become a national priority for the NHS (and indeed other healthcare systems across the world), it is necessary to ask what exactly can be done to reduce the expenditure on drugs, which account for the second largest chunk of  NHS expenditure after staff costs. Whilst the primary focus of this article is the UK, many of the ideas have a wider relevance. Since generic drugs are in most cases significantly cheaper than their branded counterparts, this is primarily an issue of increasing generic prescribing. So, what exactly are generic drugs? (more…)

The pharmaceutical industry and the medical profession are uneasy bedfellows as Tom Jaconelli discusses

It would be hard to imagine a functioning healthcare system without medicines.  Even outside the system many of us frequently take over-the-counter drugs for common ailments: in the USA 50 billion aspirin tablets are consumed each year. (1)  Also, it is difficult to think of any kind of human experience that doesn’t come with a health warning. (2) This medicalisation of society explains why pharmaceutical companies such as GlaxoSmithKline and AstraZeneca rank in the top 10 share prices in the UK economy.  However, the nature of pharmaceutical companies as profit-driven businesses in contrast to their role as health providers heralds a seemingly intrinsic problem: is their priority health or wealth? (more…)

  Johnny Boylan discusses what is happening in the UK and beyond

There is clear evidence that, throughout the world, people from ethnic and cultural minorities experience inferior health care compared to the majority, dominant population. In the United States this applies to African-Americans,  the Hispanic population and American Indians; in France to people of North African descent; in Germany to Turkish migrant workers; in Turkey to the Kurds; in Israel to Jews from Ethiopian, North African and Russian backgrounds; and in the United Kingdom to people of Indian, Pakistani and West Indian origin.(1)

This international healthcare issue has led to an increased awareness of the need to educate medical undergraduates in equality and diversity issues (2): for example in Britain in 2007 when the BMA produced their guidelines on Equality and Diversity Education. (3) In addition there is evidence that such educational programmes have positive outcomes and that participant attitudes changed over the period of the teaching. (2) (more…)

Whether you are a new medical student or not, this letter from Daniel Sokol hits the mark

Congratulations!  How many others would love to be in your shoes, tracing the footsteps of Hippocrates, Jenner, Lister, Osler, Fleming and other greats of medicine. (1) The path ahead is indeed long, but was it not Confucius who reminded us that even a journey of a thousand miles begins with a single step? (2) (3)Why this letter?  I have some advice which you may find helpful.  A secret?  Not really.  The talking fox, in The Little Prince, had a real secret: “It is only with the heart that one can see rightly; what is essential is invisible to the eye”. (4)

My simple message concerns the moral dimension of medicine.  Medicine is fundamentally about human beings and, whenever humanity is involved, so too is morality.  Why?  Because, as social creatures, we have duties to each other.  I have a duty to treat you in a particular way.  I shouldn’t lie to you, or steal from you, or insult you for no good reason.  And we also have duties to ourselves.  We must treat ourselves with respect and dignity.  As a medical student, and later as a doctor, you will be dealing with patients, relatives and colleagues.  More specifically, you will strive to help patients who are by nature sick and vulnerable.  For the patient, the awkward shift from health to illness is not the only change.  The clinical environment itself can be impersonal, unfamiliar and often confusing. (5)

The task is all the more complicated because whenever we try to help patients, whether through words, drugs or procedures, we risk harming them.  An aspirin tablet can trigger an anaphylactic reaction; a caesarian uncontrollable bleeding.  The sharp sword of Damocles hangs precariously over doctors and their patients. (6)   As time is limited and resources scarce, you may also deprive others of needed assistance.  If you decide to drain Mr Smith’s abscess now, the injured Mr Jones will have to wait in pain a while longer.  More dramatically, giving a heart to a patient with severe cardiomyopathy (an abnormality of the heart muscle) may entail the death of another patient in desperate need of the organ.  For all these reasons, medicine is a deeply moral endeavour, often involving conflicting moral principles. 

Throughout your training, you will be exposed to the scientific and technical components of medicine.  You will wonder at, and on occasion curse, the sheer volume of medical and biochemical knowledge acquired over the centuries.  We have come a long way from the days of supernatural explanations of disease, and Galen’s long-standing belief that illness was caused by an imbalance of four humours.  The ethical aspect will not feature as much as the technical and the temptation will be to dismiss ethics as irrelevant, unimportant or inconvenient to the immediate task of helping the patient. 

My message is this: do not yield to this temptation, however strong, but take the ethical issues in medicine as seriously as you do the technical ones.  This doesn’t mean devouring textbooks on medical ethics.  It means simply seeing ethics as integral to the proper care of your patients.  Just as you want to increase your understanding of the factual aspects of medicine, so should you want to deepen your moral understanding.  Your ability to perceive moral issues, to reason through ethical problems in search of a solution, and to act upon your decision is inextricably linked to your future success as a doctor.   

I have on occasion heard your peers say that ethics is merely a matter of law.  You should of course consider the law when deciding how to act, but the law is no moral panacea.  While morals may form the basis of law, there is much that the law permits but that morality forbids.  A student who laughs at the misfortune of a dying patient is not acting unlawfully, but may still be morally at fault.  Law often represents the lowest acceptable measure of morality.  As a member of the medical community, you should be striving for a higher standard.  Sometimes, the law is silent, or permits several options.  Should you breach a patient’s confidentiality if you believe your silence may endanger someone else?  The law offers no easy solution.

You will be faced with many diagnostic and therapeutic mysteries in years to come.  Medical journals are replete with case studies involving rare cases of tetanus, typhoid or other diseases whose unusual symptoms stumped the medical team.  Biomedicine is a young discipline and much remains to be found.  You will also encounter ethical puzzles.  What should you do or say if you made a medical error that no one else noticed?  How should you deal with patients’ cultural or religious beliefs at odds with your own?  How should you handle racist or abusive patients?  How should you evaluate a patient’s quality of life or the futility of a given treatment?  Like the medical ones, these problems will need to be diagnosed and resolved, and may require skill, creativity, humility, wisdom and courage.  In ancient times, whether in Greece or China, doctors were philosophers.  Today, a good doctor - and a good medical student - remains a practical philosopher.

So here endeth the lesson.  I’m aware that I haven’t discussed how to resolve moral problems.  This will, I hope, be taught to you in the coming months.  My intention here is more modest: to remind you, at the outset of this lifelong journey, of the profoundly ethical nature of medicine which in this technical age is too easily overlooked.  The ethical aspects are neglected because, unlike physical abnormalities, they are difficult to see.  The fox was right: what is essential is invisible to the eye.

Daniel K. Sokol L
Lecturer in Medical Ethics and Law
St George’s, University of London
Cranmer Terrace
London SW17 0RE
daniel.sokol@talk21.com

 Endnotes

(1) An excellent introduction to the history of medicine is Roy Porter’s Blood and Guts: A Short History of Medicine  (2002), published by W.W. Norton and Co.

(2) Confucius was a Chinese thinker living in the 6^th century BC.  For a concise summary of his life and philosophy, see http://plato.stanford.edu/entries/confucius/

(3) Sir William Osler (1849-1919), while Regius Professor of Medicine at Oxford, addressed medical students as “fellow students”.  For Osler, all doctors, however experienced, were students of medicine, always learning more about the many facets of medicine.

(4) For more on this inspiring little book, see http://en.wikipedia.org/wiki/The_Little_Prince

(5) I have written frankly about my own experience as a patient in the British Medical Journal (2004, 328:471. http://www.bmj.com/cgi/content/full/328/7437/471).

(6) The Roman writer Cicero recounts the story of Damocles.  In the story, king Dionysius allows the envious Damocles to experience, for a short time, the life of a powerful ruler.  As Damocles is enjoying a lavish banquet, he notices, hanging directly above him, a sharp sword suspended by a single horse’s hair.  This was meant to represent the illusory appearance of comfort and the ever-present danger that Dionysius faced as a king.

Would you ever blow the whistle? Elizabeth Leyland explains more about this potentially career wrecking activity

From childish taunting in the playground to kiss-and-tell exposés, whistleblowing affects everybody at some stage of their lives. It raises awareness of ‘naughty’ behaviour, but is almost invariably damaging and very difficult to reverse. In healthcare there are particularly high stakes: lives, jobs and money are potentially at risk if inappropriate practice goes unchecked. If whistleblowing is used too freely the repercussions can be especially severe. I want to tune in to some of the difficulties associated with whistleblowing in a healthcare setting. With these in mind, I will consider the balance between moral responsibility and personal cost of ‘whistling while you work’.

(more…)