Mommy I'm Hungry (Again):
A Look At Prader-Willi Syndrome
By
Mia Bolaris-Forget
Staff Writer LIFamilies
Parents have always been
typically been concerned with the health and
nutrition of their children. And, with all the chemicals contained in
?mainstream? foods these days, it would seem that the best option
(though they need careful cleansing as well) are organic alternatives.
But today?s parents are not only conscientious about what they put into
their child?s (children?s) body (bodies), but how much. Obviously you
don?t want your child to be malnutritioned, but with obesity becoming
and increasingly prevalent and growing problem, portion control is
becoming not only important for adults but also for children.
Monitoring food intake is
especially important for children who part of a small group afflicted
with Prader-Willi Syndrome, a condition that affects, among other
things, how much they eat. In fact, this ?rare? (yet serious) syndrome
often leads to an insatiable appetite that can literally result in
gross obesity or the child eating himself (or herself) to death.
Prader-Willi Defined: Prader-Willi syndrome (PWS) is a
condition characterized by short stature, below average mental and
cognitive abilities, incomplete sexual development, behavioral
problems, low muscle tone and a constant urge to eat.
Genetic Predisposition: While this syndrome IS a genetic
abnormality linked to an aberration of chromosome 15, it is typically
NOT considered hereditary, but rather a spontaneous genetic birth
defect that occurs during the time of conception, affecting both sexes
and all races.
PWS Prevalence: With this condition affecting an
estimated 1 in 14,000 in the United States alone, it is considered one
of the 10 most common birth defects identified in genetics clinics and
is the most common genetic cause of obesity identified (to date).
Age Of Onset: While this IS considered a birth
defect often leading to gross obesity, the eating disorder is not
necessarily apparent from birth. In fact, many newborns with this
syndrome tend to be ?floppy?, and are unable to take to the bottle or
nipple well, and are frequently deprived of sufficient nutrients and
many often require being fed through a tube for the first few months
until muscle tone and control improves. As children grow, approaching
preschool age, so does their appetite and their weight gain if calories
and food intake are not carefully controlled and monitored.
Why They Can?t Control Their
Appetite: Those with PWS
have a malfunction in their hypothalamus, a part of the brain that both
stimulates and controls hunger. Since the part of their brain that
tells them when they are full doesn?t work properly, they never feel
full, and thus are always hungry. In addition, these
children/individuals generally need significantly fewer calories than
normal to maintain an appropriate weight, and many are at risk for
serious illness or fatal consequences due to obesity and improper
control of their daily diet.
Controlling The Hunger: Experts assert that other than
conscience and continual monitoring of their calories and food intake,
there is little that can be done to suppress their appetite. In fact,
most with PWS need to be on an extremely low-calorie diet all their
lives and must have their environment adjusted so they have limited
access to food, even if that includes locking cabinets, refrigerators
or taking alternate routes to work that don?t allow for stops at delis,
groceries, or fast food chains.
Differentiating PWS From Basic
Overeating: While it may
seem that all those who are overweight may suffer from some form or
PWS, experts assert that this is by far NOT true. In fact, they note
that there is more to PWS than ?simply? overeating. People with PWS
also have a characteristic appearance and speech quality, significant
learning disabilities or mental disability plus a number of other
problems. Many of these features need to be present for a clinical
diagnosis and specific tests confirming it are usually available.
Behavioral Problems Associated
With The Syndrome: Besides
extreme measures taken to sneak or get food, individuals with PWS are
noted for temper outbursts, stubbornness, rigidity, argumentativeness,
and repetitive thoughts and actions. The best way to deal with a person
with PWS is to structure his or her environment using behavioral
management techniques and sometimes drug therapy.
Living A Normal Life: According to experts, those with
PWS can accomplish just as much and succeed just as well as those
without the affliction. However, they note, they need lots of
discipline and help. Experts note that youngsters with PWS generally
need special education and related services including speech and
occupational therapy. In the ?real world?, community, work, and
residential setting, adolescents and adults with PWS can seek special
assistance to learn how to carry out responsibilities and get along
with others. And, they add, most need round the clock food supervision
and that as adults they tend to fare better in group homes specially
designed for people with PWS, where food access is restricted and
limited and where people with the condition can be around peers with
similar challenges without interfering with those who don?t need such
attention. And while in the past, the condition was considered fatal,
today greater understanding and prevention of obesity can help many
with PWS live a normal life and lifespan.
The Benefits Of Early
Diagnosis: The earlier you diagnosis Prader-Willi Syndrome, the sooner
you can begin to help your child take control of his or her life and
problem, and to avert obesity. Because young children with PWS
generally have developmental delays in a variety of area,
identification of the condition may result in necessary early
intervention and help identify areas of need or risk. Furthermore,
early diagnosis allows for families to do amble research and get the
necessary information, help, and support from professionals and other
families who are dealing with the syndrome.
What To Look For: As with
anything, identifying the problem is the first step is ?solving? or
?controlling? it. The best way to determine whether of not you child
has PWS (if you are concerned about some possible symptoms) is by
contacting your health care provider for and evaluation and formal
diagnostic. Note that many doctors may suggest a medical geneticist
specializing in diagnostic tests for such genetic conditions and after
a thorough evaluation, examination and family history, will arrive at a
conclusion and diagnosis.
For More Information: If you
are interested in acquiring more detailed information on PWS you can
contact the Prader-Willi Syndrome Association at 1-800-926-4797, which
in addition to answering your questions and concerns, provides a
newsletter and other publications (on the syndrome), an annual national
conference, and chapters throughout the country offering family support
and advocacy.