Attenuating Growth in Children With Profound Developmental ...



Attenuating Growth in Children With Profound Developmental Disability - New Approach to an Old Dilemma

Daniel F. Gunther, MD, MA; Douglas S. Diekema, MD, MPH

CASE REPORT

The patient is a 6-year-7-month-old white female initially referred to the pediatric endocrine service for early pubertal development. She had been the full-term product of an unremarkable pregnancy followed by an uncomplicated birth. After the first month of life, she began to display symptoms of hypotonia, feeding difficulties, choreoathetoid movements, and developmental delay. Extensive subsequent evaluation by specialists in neurology, medical genetics, and developmental pediatrics failed to uncover a specific cause. Static encephalopathy with marked global developmental deficits eventually was diagnosed. In the ensuing years, her development never progressed beyond that of an infant. At the age of 6 years, she cannot sit up, ambulate, or use language. She is gastrostomy-tube dependent for nutrition. However, she clearly responds to others—vocalizing and smiling in response to care and affection. The combined opinion of the specialists involved in her care is that there will be no significant future improvement in her cognitive or neurologic baseline.

Since birth, the patient has been cared for in the home by her parents, both of whom are college-educated professionals. She has 2 healthy siblings. Despite her severe disability, she clearly is an integral, and much loved, member of the family.

…When deciding whether it is ethically appropriate to attenuate growth in these children, there are 2 primary considerations. First, does growth attenuation offer the patient benefit? And second, does growth attenuation do any harm to the patient? The question here, then, is whether there is a reasonable expectation of improved quality of life for a nonambulatory child with profound developmental disability, and at what risk?

The primary benefit offered by growth attenuation is the potential to make caring for the child less burdensome and therefore more accessible. A smaller person is not as difficult to move and transfer from place to place. Although this may seem to be an advantage that accrues to the caretakers rather than the child, it offers several distinct benefits to the child as well.

…Can one imagine harm to this population from simply being small? Height and normal stature clearly have social value for most individuals. Being taller has been associated with enhanced social stature, greater pay, greater success in attracting a mate, and other social benefits.39 However, a nonambulatory, severely impaired child is not someone who will experience these benefits of tall stature and therefore will not suffer their loss if kept short. For an individual who will never be capable of holding a job, establishing a romantic relationship, or interacting as an adult, it is hard to imagine how being smaller would be socially disadvantageous.

One might argue that being smaller might alter the way others interact with an older disabled person, perhaps tending to treat that person as a child instead of an adult. Whereas this might be an important issue for a short-statured adult who is capable of normal adult interactions, it is unlikely that such "infantilization" harms a person whose mental capacity will always remain that of a young child. In fact, for a person with a developmental age of an infant, smaller stature may actually constitute an advantage because others probably would be more likely to interact in ways that are more appropriate to that person's developmental age.



David is a young man with severe cerebral palsy. He can't walk, he can't talk, he can't sit up by himself, but he can blog. This week, David blogged about Ashley.

"Ashley's parents have committed the ultimate betrayal," he writes. "They have treated their daughter as less than human, not worthy of dignity.... What strikes me about 'the Ashley treatment' and has brought me to tears is that the very people in all of society whom this child should trust have betrayed her."

Ashley's parents speak publicly only through their blog[pic]. On it, they explain why they did what they did: Ashley will be more comfortable at a smaller size; large breasts would have made lying down difficult; it will be easier to include her in family gatherings if she is lighter and easier to carry around. …Ashley, 9, has a condition called static encephalopathy, which means an unchanging brain injury of unknown origin. She's in a permanent infant-like state -- can't hold her head up, speak or roll over on her own.

…Like the Terri Schaivo story before her, Ashley and her story have a lot to say about what it means to be disabled, what it means to be different and what it means to be human.

Some of the most vociferous critics of what Ashley's parents call "the Ashley treatment" have been other parents of disabled children.

"My son is 11....doesn't walk, doesn't talk, etc., etc." writes one mom[pic]. "He's hard to carry. He's not going to get easier to carry.... And still, I don't understand. I don't understand removing healthy tissue and functioning organs.... Growing is not a sin or a disease -- it's what kids' bodies do, even disabled bodies."

Many others have been supportive of Ashley's doctors and parents. On , for example, one person wrote, "No one objects to surgery to remove a cancer or hormone treatments to treat a variety of ailments. No one objects to pain medication for palliative care. Why would anyone object to surgery or hormones to improve the quality of life and care for a severely mentally disabled child?"

In the end, as in the Schiavo case, it comes down to who speaks for Ashley, a girl who cannot speak for herself. A reader who is supportive of Ashley's parents wrote: "Nobody is stopping to think how a little girl with the mentality of an infant would treat bodily processes such as puberty and menstruation -- a process that scares even the most normal of our children."

But others think they're in a position to understand what Ashley wants. "Ashley is human. I am human," writes David on his blog. "Ashley is me. I am Ashley. And you are Ashley, too."

Ashley’s Story -

Our daughter Ashley had a normal birth, but her mental and motor faculties did not develop. Over the years, neurologists, geneticists, and other specialists conducted every known traditional and experimental test, but still could not determine a diagnosis or a cause...Now nine years old, Ashley cannot keep her head up, roll or change her sleeping position, hold a toy, or sit up by herself, let alone walk or talk. She is tube fed and depends on her caregivers in every way. We call her our Pillow Angel since she is so sweet and stays right where we place her—usually on a pillow. Ashley is a beautiful girl whose body is developing normally with no external deformities. She is expected to live a full life and was expected to attain a normal adult height and weight.

Ashley brings a lot of love to our family and is a bonding factor in our relationship; we can’t imagine life without her. She has a sweet demeanor and often smiles and expresses delight when we visit with her, we think she recognizes us but can’t be sure. She has a younger healthy sister and brother. We constantly feel the desire to visit her room (her favorite place with special lights and colorful displays) or have her with us wanting to be in her aura of positive energy. We’re often gathered around her holding her hand, thus sensing a powerful connection with her pure, innocent and angelic spirit. As often as we can we give her position changes and back rubs, sweet talk her, move her to social and engaging places, and manage her entertainment setting (music or TV). In return she inspires abundant love in our hearts, so effortlessly; she is such a blessing in our life!

Faced with Ashley’s medical reality, as her deeply loving parents, we worked with her doctors to do all we could to provide Ashley with the best possible quality of life. The result is the “Ashley Treatment.”

…A fundamental and universal misconception about the treatment is that it is intended to convenience the caregiver; rather, the central purpose is to improve Ashley’s quality of life. Ashley’s biggest challenges are discomfort and boredom; all other considerations in this discussion take a back seat to these central challenges. The Ashley Treatment goes right to the heart of these challenges and we strongly believe that it will mitigate them in a significant way and provide Ashley with lifelong benefits.

Ashley’s smaller and lighter size 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. Typically, when awake, babies are in the same room as other family members, the sights and sounds of family life engaging the baby’s attention, entertaining the baby. Likewise, Ashley has all of a baby’s needs, including being entertained and engaged, and she calms at the sounds of family voices. Furthermore, given Ashley’s mental age, a nine and a half year old body is more appropriate and provides her more dignity and integrity than a fully grown female body.

If people have concerns about Ashley’s dignity, she will retain more dignity in a body that is healthier, more of a comfort to her, and more suited to her state of development as George Dvorsky, a member of the Board of Directors for the Institute for Ethics and Emerging Technologies, alludes to in a related article4: “If the concern has something to do with the girl’s dignity being violated, then I have to protest by arguing that the girl lacks the cognitive capacity to experience any sense of indignity. Nor do I believe this is somehow demeaning or undignified to humanity in general; the treatments will endow her with a body that more closely matches her cognitive state – both in terms of her physical size and bodily functioning. The estrogen treatment is not what is grotesque here. Rather, it is the prospect of having a full-grown and fertile woman endowed with the mind of a baby.”

• Break up into 4 groups

o Health professionals

▪ Outline the arguments that the physicians in this case lay out

▪ What are the pros and cons they stated?

▪ Can you think of medical pros and cons that aren’t stated that would assist in the argument?

o Parents

▪ Layout the arguments that the parents provide

▪ Can you think of any pros and cons they did not state that would assist in their argument?

o Community members

▪ As community members what is your stake in this case?

▪ What might you be concerned with in this case? (Think here maybe about the cost to society)

o Disability members

▪ After listening to the previous videos, what do you think the woman in the video would say about this case?

▪ What would her arguments be (outline them)?

• In ethic committee groups

o Begin with the parents stating their case for the treatment

o Each “member” of the committee (health professional, community, and disability members) have the opportunity to provide their argument for the pros and cons of the treatment from their point of view

o Then open it up for discussion but remember to stay in your “role”!

o Questions

▪ Anything interesting arise?

▪ Did people’s views change?

▪ Ultimately who do you think should make a decision like this one (whether it be growth attenuation, sterilization, etc.)?

▪ Do we need to be concerned with the welfare of persons w/ cognitive disabilities? How do we ensure that? Who makes those choices?

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