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In Depth Tutorials and Information
CHAPTER
41
Ethical Implications of Osteogenesis
Imperf
ecta across the Li
fespan
Marilyn E. Coors
University of Colorado Anschutz Medical Campus, Denver, CO, USA
pertinent principles,
3
duties
4
and/or professional vir-
tues.
5
Often questions related to medical care choices
require examination from the different ethical perspec-
tives mentioned above to gain sufficient insight and
provide adequate guidance to tackle complex ques-
tions.
6
Among those questions are: What are the psy-
chosocial circumstances surrounding the case? What
are the professional obligations of the clinicians? What
are the patient's goals of care, the clinicians' goals? Are
those goals aligned and, if not, can they align? What
principles and values apply to this particular situation?
This chapter will make use of clinical cases to ana-
lyze common ethical issues that arise in the treatment
of patients with OI. Even though the range of clinical
severity in OI is a continuum, for the purpose of this
chapter we will focus the ethical discussion on cases
in three categories of OI: lethal perinatal OI (type II),
moderately severe OI (types III-VIII) in children and
moderately severe OI in adults.
7
For the purpose of
our discussion, the term clinicians will include health
care professionals (physicians, physician assistants,
physical therapists, mental health professionals, genetic
counselors, nurses and social workers) who share a
common professional obligation to “maximize function,
minimize deformity and disability, maintain comfort,
achieve relative independence in daily life, and enhance
social integration” for patients with OI.
8
When difficult questions arise regarding the care of
patients with osteogenesis imperfecta (OI), the first con-
sideration is what
can or cannot
be done through treat-
ment options such as physical therapy, bracing and
splinting, pharmacologic therapy and orthopedic sur-
gical management.
1
Medical questions regarding care
for children with OI can be particularly challenging
because the condition is rare enough that there are few
well-established guidelines addressing patient selection
for treatment, duration of treatment, management of
patients who do not respond and more.
2
As increased
attention is focused on the pathogenesis, diagnosis and
treatment of OI, greater interest has also developed
regarding the ethical issues presented by caring for OI
patients. And just as medical options are complex, there
are weighty ethical questions concerning what a clini-
cian
should
do either in developing policies or in caring
for a specific patient.
Should
questions that arise in health care are best
answered by a systematic approach. The purpose of
bioethical analysis is to provide a framework to iden-
tify values in tension around management decisions, to
foster decision-making and to affirm the scientific and
psychosocial aspects of those decisions. Ethics is by its
very nature interdisciplinary; ethical actions occur in
the complex web of the patient (including family and
friends) and clinical team. This web is not strictly bio-
medical; it is impacted by culture, values, beliefs and
differences of all concerned.
The first step in an ethical analysis is to gather the
medical facts and understand the science that applies
to the situation at hand; good ethics relies upon good
facts. Commonly, ethical analysis then proceeds to
examine the ethical question through the lens of
ETHICAL ISSUES: LETHAL PERINATAL,
TYPE II OI
Type II OI is a potentially lethal genetic condition
that is associated with defective maturation of collagen
