Policy Statement:
Adults With Prader-Willi Syndrome and Decisions Regarding Least Restrictive Environment
and the Right To Eat
The quality of life for persons with mental retardation has improved significantly over
the past two decades. This can be attributed, at least in part, to changes in societal
attitudes and standards of care, often accompanied by legislative supports. Thus the
concepts "inclusion," "least restrictive environment," and "the
dignity of risk" in the context of community-based "supported living
arrangements" have opened many new avenues of opportunity previously denied these
adults. When applying the concept of "rights" to persons with developmental
disabilities, however, it must be remembered that current disability rights laws were
formulated primarily in consideration of those with physical disabilities. Uncritical
application of these "rights" to persons with cognitive disabilities without
regard to possible consequences may lead to tragic outcomes. While specific medical
etiologies for any given disability may not always be of overriding importance in
determining services and supports, there are, nonetheless, specific genetic syndromes and
recognizable neurobehavioral patterns that present serious considerations that must be
addressed in the development of a service plan. Thus, for instance, the type and level of
support services for persons with Prader-Willi syndrome are relatively independent of
their level of mental retardation and are much more related to the medical and
neurobehavioral implications of the specific syndrome.
Developing an appropriate social milieu for individuals with Prader-Willi syndrome
means creating an environment where the least restraints are present; however,
environments of least restraint do not maximize freedom in an unbridled sense but are
designed to help individuals achieve their fullest possible potential. Environments must
become more restrictive when lesser restraints fail to protect the physical or emotional
well-being of the person or to protect the person from doing avoidable harm to themselves
or to others.
The issue of adults with Prader-Willi syndrome deciding whether they "want to diet
or not" is just such an issue. The dialog that raises this issue is framed by the
concept "least restrictive environment" or "client rights." The
argument generally is that strict dietary management is "too restrictive" or
that locking food abrogates "rights." A second issue is whether restricting
spending money (to limit ability to buy food) violates the personal rights of adults with
Prader-Willi syndrome. In many states, the agencies and group homes that specialize in
Prader-Willi syndrome are increasingly criticized as being too restrictive, and as
violating consumer rights. Many of these programs have been ordered to increase client
access to food, to move clients into less restrictive settings, and to give clients
control of their diets (Cormier, 1995; Goff, 1995; Greenswag et al., 1995). Although
easier access to food may be a stong desire for individuals with Prader-Willi syndrome, it
is a dangerous and medically neglectful practice. In too many cases, such practices have
led to medical emergencies or to premature deaths related to complications of obesity.
This growing trend is both alarming and tragic.
Failure to restrict access to food is tantamount to medical neglect. To illustrate, let
us draw a parallel with diabetes. Diabetes results from a failure of the pancreas to
produce adequate insulin. Thus, the person with diabetes must maintain a calorie- and
carbohydrate-restricted diet while taking supplemental insulin. Failure to rigidly follow
this regimen leads to elevated blood sugars and, ultimately, death. No caregiver home
would think of telling diabetics that their diet was "too restrictive" or that
restricting access was an abrogation of rights.
The management of the eating behaviors in persons with Prader-Willi syndrome is based
on similar physiologic failures and is equally medically critical. In this instance, there
is a genetically based inability to sense satiety, combined with a decreased utilization
of calories, resulting in an elevated production of fat tissue. A failure to experience
satiety leads the person to continue eating far beyond physiologic or nutritional needs.
This overeating combined with elevated fat tissue production leads to rapid and morbid
obesity. This rapid obesity overtaxes the heart and leads to complications that can
include sleep apnea, diabetes, hypertension, and cardiopulmonary compromise. This
physiologically driven eating behavior is no more under cognitive control, nor amenable to
cognitive remediation, than is the failure of the pancreas to produce insulin in diabetes.
Further, there are, to date, no medical, pharmacologic, or behavioral treatments that fix
or cure this biological malfunction. Bioethicists dictate that informed consent requires
the capacity to consider, and fully understand, the pros and cons of both sides of a
decision issue prior to making a decision. Since by their own physiology, persons with
Prader-Willi syndrome cannot decide "not to eat," therefore they cannot
responsibly decide the converse: "to eat, or not to diet." Thus, to allow such
decisions under the guise of "restriction of rights" is both medically and
ethically unsound. Failure of the care-giving environment to maintain a rigidly managed
diet or to supervise food access leads to the previously described rapid weight gain and
can easily result in cardiopulmonary compromise and death. Such a failure in a medical
setting would lead to charges of malpractice. Such a failure in a certified living
environment may arguably lead to equally serious legal consequences.
Nonetheless, in the past three years, several persons with Prader-Willi syndrome have
been placed in less restrictive environments under the argument of "rights." To
date, several have died and the rest have been placed in more restrictive settings or
rushed to critical care due to cardiopulmonary crises. Most have gained over 100 pounds in
less than six months with the attendant acute medical complications. Clearly, restricting
food is not an abrogation of rights; it is the standard of care for a person with
Prader-Willi syndrome. Failure to restrict food and allowing a person to eat themselves to
death is, in fact, a removal of "rights" to a protected environment.
Is it not a paradox that we would allow someone with Prader-Willi syndrome the
"right" to eat themselves to death, but if someone without such cognitive
limitations were to threaten suicide, the caregiver that failed to provide suicide
restrictions would be found guilty of lack of protection? So we will protect those who are
cognitively normal from their own self-destructive impulses, but argue that someone who
has cognitive limitations and has physiologically driven eating behaviors has the right to
eat themselves to death.
In planning the care-giving environment for persons with Prader-Willi syndrome, some
contradictions are evident. While persons with Prader-Willi syndrome need extensive food
support, they show fewer needs for support in other aspects of their lives. Indeed, many
persons with Prader-Willi syndrome show competencies and decision-making abilities outside
the food arena. Nonetheless, until there are medical or pharmacologic interventions for
this physiologically driven eating behavior, structured environments with restricted
access to, and intake of, food must be standard care for persons with Prader-Willi
syndrome.
Some will argue that these recommendations conflict with concerns with choice, personal
rights, and least restrictive environment. We do not take issue with these philosophical
goals. Instead we assert that the appropriate frame of reference is the "least
restrictive environment," given that the individual has Prader-Willi syndrome. Indeed
the concept "least restrictive environment" is meant to imply "as normal a
life as possible within the framework of a given disability." Too often it is
translated as: "Despite your disability, you will live as though you are
normal". Society's efforts to undo a previously created "social disability"
may ultimately lead to a completely restricted environment when appropriate limit setting
is insufficient. Persons with Prader-Willi syndrome must be uniquely considered as least
restrictive goals are put into practice in order to prevent further deaths and to promote
a fuller quality of life.
Approved by the PWSA (USA) Board of Directors
January 1998
