Prader-Willi Syndrome: What It Is, Causative Behavior and Control

Among the different types of diseases and disorders that people suffer with, there are particular diseases that are being studied at the genetic level. One such disease is the Prader-Willi Syndrome.

This is a disorder caused by a deformation of Chromosome 15. Around 1:12,000- 15,000 people have this disorder, and the disorder is not limited to particular sex or sexes. The notable symptoms of the disorder include: hypotonia, hypogonadism, hyperphagia, cognitive impairment, difficult behaviors and also obesity that one has to live with for the rest of his or her life.

Chromosome Deficiency

Around 70% people with this disorder (PWS) do not have chromosome 15, and this means that there would automatically be some kind of disturbance in proper functioning of their bodies. This is because of the fact that each and every chromosome has some function in the body.

In the absence of any of them, there is bound to be some deficiency. The 15th chromosome has bands 15q11.2-q13, which denotes specific importance regarding their functions.
The result of a chromosome missing from the genetic make up means that the space left in between has top filled up.

There is a different type of arrangement in the chromosome because of the absence of chromosome 15. Around 1% of all patients diagnosed with PWS have a balanced configuration of chromosomes.

Those who suffer from this disorder quite naturally include all age groups. This is because of the fact that the same children that are born with the deficiency grow up into adults demonstrating the symptoms.

Age Group & The Related Problems

From birth until the age of two, children have Hypotonia and also poor suck. From two years till the age of six, Hypotonia co-exists with a history of poor suck, and there is also an overall developmental problem.

From six years till the age of twelve, children have a history of hypotonia with poor suck, slow development, and overeating accompanied by central obesity. Obesity usually results from the uncontrolled eating.

From 13 years of age till adulthood, there are cognitive problems, often mild retardation of the mind, overeating, obesity, and Hypothalamic hypogonadism problems.

One has to be careful when a person has not been confirmed as a PWS individual. This is because one may think that the individual is a case simply because s/he he eats a lot. However, in order for a person to be kept away from excess food, it is better to have a diagnosis done. In addition to this, one may also take note of characteristics such as appearance, speech, learning problems, etc.

Behavior Problems

This includes struggles to get food, which may be expressed in the form of outcries for food, being obstinate, rigid, and thinking about food all the time.

In view of these emotions and efforts to obtain food it is no wonder why they end up getting their way most of the time and putting on an excess amount of weight. In order to take care of this type of behavior strategies are usually developed and implemented. These may consist of setting up the patient’s environment, enforcing behavior management, and even drug therapy in some cases.

Aside from using an environment to curtail the patient’s eating behavior there is no means of inhibiting the patient’s eating through appetite suppressants.

So, alternative methods of controlling one’s weight are placed in action. The alternative that is used is a low-calorie diet in tandem with vigilant supervision to make sure that the patients do not have access to food.

For children especially, it is suggested that cabinets containing food supplies should be locked.