Prader Willi syndrome 411

• paranoia and distrust

• an unwillingness to discuss the trauma

• persistent, intense fear and anxiety

• irritation or agitation

• concentration problems

• feelings of numbness or detachment

• no longer finding pleasure in previously enjoyable activities

• helplessness

• survivor guilt

• preoccupation with the event

• physical symptoms (headache, stomach distress, or dizziness)

• suicidal thoughts, plans, or gestures

Treatment

Some children can recover from PTSD without treatment, with symptoms fading away within six months. Otherwise, cognitive-behavioral therapy can help, particularly if the trauma was unusually severe or life-threatening. This type of therapy helps a child adopt new thoughts and behaviors to replace negative ones. Temporary medications may be helpful to treat depression and anxiety and can help a child cope with school and other daily living activities. Group therapy or support groups also may sometimes help.

Parents should let a child talk about the traumatic event but should not force a discussion. The child should be reassured that her feelings are normal and that she is not crazy. Parents should let a child with PTSD make simple decisions whenever appropriate, which will help her see that she has control over certain aspects of her life. Children also must be assured that the traumatic event was not their fault and should be encouraged to discuss feelings of guilt. Regressive behavior is common and should not be criticized; sleeping with the lights on or cuddling a stuffed animal is normal and soothing. (See also anxiety disorders.)

Prader-Willi syndrome A complex genetic disorder that includes short stature, mental retardation or learning disability, incomplete sexual development, characteristic behavior problems, low muscle tone, and an involuntary urge to eat constantly. This, coupled with a reduced need for calories, leads to obesity. The disorder is found in children of both sexes and all races and occurs in about one in 14,000 people in the United States. It is one of the 10 most common conditions seen in genetics clinics and is the most common genetic cause of obesity that has been identified.

Although Prader-Willi syndrome (PWS) is associated with an abnormality of chromosome 15, it is generally considered not to be an inherited condition but rather a spontaneous genetic birth defect that occurs at or near the time of conception. The faulty chromosome affects functioning of the hypothalamus.

Newborns with the condition have poor muscle tone and cannot suck well enough to get sufficient nutrients. Often they must be fed through a tube for several months after birth until muscle control improves. Sometime in the following years, usually by preschool age, children with PWS develop an increased interest in food and quickly gain excessive weight if calories are not restricted.

In addition to sometimes extreme attempts to obtain food, children with PWS are prone to temper outbursts, stubbornness, rigidity, argumenta-tiveness, and repetitive thoughts and behaviors. Strategies to deal with these problems usually include structuring the person's environment, implementing behavioral management techniques, and occasionally using drug therapy.

Children with PWS can attend school, enjoy community activities, get jobs, and even move away from home, although they need a lot of help. Schoolchildren with PWS are likely to need special education and related services, such as speech and occupational therapy. In community, work, and residential settings, adolescents often need special assistance to learn and carry out responsibilities and to get along with others.

Children with PWS need around-the-clock food supervision. When they grow up, most affected individuals do best in a special group home for people with PWS, where food access can be restricted without interfering with those who do not need such restriction. Although in the past many patients died in adolescence or young adulthood, preventing obesity will allow a person with PWS to live a normal lifespan.

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