|
PRADERS WILLIE
SYNDROME
THE DISORDER
OF THE APPETITE
A brain dysfunction in the level of the hypothalamus is the reason of this disorder in the
center of the appetite and satiety, which is typical of PWS.
To eat compulsively and
the obsession with the food normally begins before 6 years old.
The impulse of eating
is physiological and very big, it is very difficult to control and requires constant
alertness.
A balanced diet, low in
calories with vitamin supplementation and calcium supplement is advisable. To be weighed regularly and a periodic review of the diet is necessary.
The education to eat
small morsels, morsel by morsel and healthy NON FAT food is the plan to apply
routinely and in a consistent form.
The weight control
depends on the restriction of the food out of home or out of alertness and
it may be needed to close with key the kitchen and areas of food storage.
The daily exercise (at
least 30 minutes) also is essential for the control of weight and health.
Up to now no medication or
surgical intervention has been defined that could helps to eliminate the need
of the strict control of the nourishment and the alertness about the food.
The treatment with
growth hormone, which increases the muscular mass, can allow a level of higher
caloric daily ingestion.
BEHAVIOR PROBLEMS
Infants and young
children with PWS are typically satisfied and loving, and exhibit few problems
of conduct.
The majority of
older children and adult with PWS, nevertheless, have difficulties with their
behavior. The assault of behavioral symptoms normally coincides with the
assault of hyperphagia (though not all the conducts of the problem are related
to the food), and the difficulties reach the maximum in the adolescence.
Daily routines and a
rigid structure, firm rules and well marked limits, as well as the positive
prizes are the best attribute for the direction of the conduct.
PHYSICAL DEVELOPMENT PROBLEMS
The physical and
professional therapies help promoting the development of skills and the
appropriate functions.
Feet may need
orthopedic devices.
The treatment with the growth
hormone, increasing the mass of the muscle, can improve the motion skills.
Exercise and physical activities are necessary.
The need must be
evaluated for language therapy in the childhood. The products to
increase the saliva can help to the problems of joining words when speaking. The
skills trained socially can improve the use of the pragmatic language.
Even with the delay,
the verbal skill becomes often an area of strenght for the children with PWS.
The IQs goes of 40 to
105, with an average of 70.
Those with normal IQs
have the typical troubles of learning. The areas of problem can
include attention, short term auditory memory, and the abstract
thought. The common strengths include the memory in the long term, while reading
the skill, and the receptive language.
Early stimulation should be done and the need for special education is necessary.
SEXUAL
DEVELOPMENT
The sexual hormones
(the testosterone and the estrogen) are typically low. Both sexes can have a
good response to the treatment for the deficiencies of the hormone, although there exist reports of colateral effects. Early pubic hair is common, but the
puberty comes normally late. The fertility
has not been documented in any sex.
PROBLEMS IN THE QUALITY OF LIFE
Health normally is good
in the individuals with PWS. If the weight is controlled, the life expectancy
can be normal, and the health of the individual can increase to the maximum.
The constant need for the restriction of food and direction of conduct can be
the most stressful and difficult matter for the relatives.
CONSULT YOUR DOCTOR AND
A PSYCHOLOGIST.
CONTINUES:
BARDET-BIEDL'S SYNDROME
|
