Prader Willi Syndrome

Characteristics of Prader Willi Syndrome

PWS occurs when children are born without the paternal Chromosome 15. A genetic DNA test can be taken to determine how specifically the chromosome was affected and to determine the risk of passing the syndrome on to future children. There are several symptoms that affect the daily lives of those who have the syndrome.

The main symptoms include food intake and the great risk of morbid obesity because of it. Between the ages of 1 and 6, children develop a form of obsessive-compulsive disorder involving overpowering cravings for food. The child’s diet has to be strictly monitored and maintained continuing into adulthood. It is very difficult to control the obsession and there is no known medication to help curb it. Daily exercise and a consistent diet plan are strongly recommended.

Although behaviors are not always triggered this way, the behavioral problems usually begin after the food obsessions do during adolescence. OCD symptoms, perseveration, and moods swings are typical while depression is not unheard of in adults. It is a rare even to experience psychotic episodes with PWS.

Developmentally, there is a strong delay in the development of motor skills of about 1-2 years. Strength, coordination, and balance suffer due to lack of muscle tone, hypotonia, but should improve with age as long as the proper physical therapies are followed, and the child should remain actively engaged in physical activities.

Hypotonia may affect speech, but severe cases are rare. Feeding problems also occurs,s as well as a delay in speech and oral motor skills.

With cognition, the average IQ is about 70. Those with PWS have an excellent long-term memory, and reading abilities, although having a normal IQ is usually accompanied by learning disabilities.a Attention, short-term auditory memory, and abstract thinking are typical weaknesses.

Growth hormone therapy is usually taken due to the severe growth affects. Infants usually have a small stature, and puberty is delayed later into adolescence.

Although sex hormone levels are typically low, hormone treatments are usually successful and PWS patients can have children, although genetic counseling should be taken seriously as the risk of passing the syndrome on is significant.

Those with PWS typically have no problems with general health, or social concerns as long as the strict diet plans are adhered to. Maintaining the plan however is extremely stressful for both the patient, and those close to him/her.

Strategies for Teachers in the Classroom

In the classroom, the child should always be well informed about procedures, changes, and assignments. All given directions should be simple, clear, and concise as possible. Gradually increase the work load over time instead of bombarding the child all at once. Confrontations with the student should be avoided. Maintain a positive attitude and there should be plenty of positive reinforcement of positive behaviors.
Food should never be used as a reward. Lunches should be held by the bus driver, teacher, or aid until it is allowed to be eaten. During these lunch and snack times, the child should be monitored to make sure he or she eats only what should be eaten. Lunch should not be delayed, and food should be kept out of sight until the proper time. The nurse should make sure to take weekly measurements to monitor weight gain.

Resources for People with Prader Willi Syndrome

When teaching a child with PWS, it is important for the teacher to have many connections in order to best serve the student. In school support teams include the nurse, guidance counsellor, lunch personnel, and recess attendants. The nurse should be an active participant, checking in with the child's pediatrician and other doctors, and should be required to take weekly measurements so his or her weight can be monitored accordingly. The lunch personnel need to know that the child has PWS and prepare a proper lunch menu to suit the child's needs. They should also keep an eye on him or her to make sure no food is traded, stolen, stolen, or altered. The guidance councellor will be able to help the child with any frustrations related to the classwork and behavior while helping him or her voice his or her needs so the teacher can accomodate. Espescially with young children, other teachers should be aware that the child has PWS. Recess attendents should watch to make sure he or she does not eat any food found or given by another student.

http://www.pwsausa.org/index.html This is an exellent link to support systems, knowledge, and other resources that will benefit the child.

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"Basic Facts About PWS." Prader Willi Syndrome Association. 7 November 2007. 26 November 2007. <http://www.pwsausa.org/index.html>