What you need to know
- Behavioral and psychiatric problems may interfere with quality of life for adolescents and in adulthood.
- These behaviors can be persistent. They may escalate in severity and intensity with age (particularly from adolescent to adulthood).
- This escalation is independent of intellectual, language, and motor abilities.
- People with PWS have a characteristic behavior profile which includes: temper tantrums, stubbornness, emotional lability, exaggerated emotional response, controlling and manipulative behaviors, compulsivity, and difficulty with changes in routine.
- Many behavioral challenges are similar to those found in people diagnosed with autism or autism spectrum disorders.
- Attention Deficit Hyperactivity Disorder (ADHD) may be seen.
- Perseveration and repetitive behaviors are common.
- Students with PWS can be demanding and prone to rage episodes when frustrated.
- Psychosis is reported in young adulthood in 10-20% of people who have PWS.
- By late adolescence, about 15% will have a diagnosed mood disorder.
- Delusions and paranoid ideation are more common in people with PWS than in the general population.
- They may have obsessive compulsive tendencies:
- These behaviors can be a source of strain in families, and in living and work environments.
- The constant need to monitor food intake can be very stressful for families.
- This is due to an abnormally increased appetite and the eating of unusual food related items (i.e. sticks of butter, used cooking grease, decaying food, garbage, and food flavored items like shampoo).
- Hoarding food when it is accessible is common.
- Social judgment can be poor. Interpretations of social information by students with PWS are similar to some children who have a pervasive development delay.
- They may have an inability to read other’s facial expressions, decode emotion, and have difficulty interpreting visually presented social information.
- decreased sensitivity to pain
- a tendency to not run a fever even when significantly ill
- increased skin picking and picking at other body areas
What you can do
- Consider proactive behavioral support strategies with behavioral or mental health professionals.
- Parents may consider a referral for medication for a child.
- Selective serotonin reuptake inhibitors (e.g., fluoxetine and sertroline) have been helpful in many patients.
- Be firm with directions, rules, and discipline.
- Behavioral and environmental support for weight management is needed.
- Consider:
- Restricted access to food in all environments
- Locks on refrigerators and food storage areas (including other student’s lunches and snacks)
- Constant supervision
- Calorie restrictive diets
- Consistent and scheduled meals and snacks
- Programs that help teach behavioral and weight management strategies
- Many children and young adults with PWS have difficulty regulating emotions and behavior. This is especially true when handling unplanned changes.
- They usually thrive with consistency and routine. They can be easily upset with disruption.
- Prepare students for any change in schedule.
- Provide a “safe” area for students to share emotions.
- Teach and model use of words and/or pictures in sharing emotions.
- Teach and emphasize behaviors you want.
- Make sure they have an effective communication system.
Skin picking helps them decrease anxiety and boredom.
- Eliminate opportunities to skin pick.
- Distraction may be helpful.
- Decrease anxiety and eliminate boredom.
- Provide social cues and coaching.
- Provide information and discuss differences with the child’s peers.
- Help develop confidence and focus on strengths.Provide positive reinforcement.
- Teach child appropriate social behaviors/skills (how to ask a friend to play, role model, small groups of friends).
- Teach how to recognize facial expressions, body language, and moods.