Families raising a child with Prader-Willi syndrome know how difficult temper outbursts can be. In the study “The Characteristics of Temper Outbursts in Prader-Willi Syndrome,” Drs. Rice, Einfeld, and Woodcock, experts in the field of PWS, took a closer look at these behaviors to better understand them. They examined when temper outbursts typically begin, how often they happen, how long they last, and the types of situations that may trigger them. The researchers also looked at whether factors such as age, gender, genetic subtype, and the onset of hyperphagia may influence temper outbursts.
For this study, Dr. Rice and her colleagues used interviews with 22 families of individuals with PWS to create a survey on temper outbursts.
They then used this survey with a larger group of parents to rate how often outbursts occurred during the past month, how intense they were, and how long they lasted. For the purposes of the study, an outburst was defined as becoming very angry or upset in a way that seemed excessive for the situation and beyond the person’s control.
In total, 101 primary caregivers completed the survey, reporting on individuals with PWS ranging in age from 1 to 64 years. The mean age of the person with PWS was 16.14 years (SD=10.94).
The number of participants are shown in the tables below, broken down by age, gender, and genetic subtype (for those who had genetic subtype confirmation):
| Children (1-10 yrs.) | ||
| Male | Female | |
| Deletion | 11 | 8 |
| UPD | 9 | 6 |
| Imprinting / Other | 2 | 1 |
| TOTALS | 22 | 15 |
| Adolescents (11-18 yrs.) | ||
| Male | Female | |
| Deletion | 12 | 3 |
| UPD | 9 | 5 |
| Imprinting / Other | 1 | 0 |
| TOTALS | 22 | 8 |
| Adults (19 yrs. +) | ||
| Male | Female | |
| Deletion | 7 | 15 |
| UPD | 4 | 3 |
| Imprinting / Other | 1 | 0 |
| TOTALS | 9 | 18 |
Interestingly, in this study, no differences were found in temper outbursts across gender or genetic subtype, suggesting that if the high level of outbursts in people with PWS is due to a genetic aspect, this genetic issue is likely present in both subtypes.
When Do Temper Outbursts Start?
The study found that temper outbursts in PWS tend to begin later than in typically developing children. In the general population, about 91% of children show temper outbursts by age 3. In this study, only 41% of children with PWS were reported to have outbursts by that age.
Researchers also looked at how often outbursts occur at different ages:
- Children: about 2–3 outbursts per week
- Adolescents: about 2 outbursts per week
- Adults: about 1 per week or less
Adults tended to have fewer outbursts, but there was an important difference: outbursts often lasted longer in adolescents and adults.
- Children: 0–15 minutes
- Adolescents and adults: 10–30 minutes
What Triggers Temper Outbursts?
Across all age groups, caregivers reported several common triggers. The most frequent were:
- Being told “no” to something they asked for (non-food related)
- Being asked to do something they don’t want to do (for example, taking a shower)
- Changes in plans or expectations
- Feeling accused or blamed for something
- Being denied food
Caregivers reported that these situations occurred before 85–100% of outbursts.
When researchers looked more closely, most triggers fit into three broader themes:
- Being prevented from getting something they want
- Feeling something is unfair or unjust
- Difficulty coping with changes or transitions
What Helps During an Outburst?
Caregivers reported that the most helpful strategies were:
- Giving the person space to calm down
(either removing them from the situation or removing the trigger) - Providing a distraction
to help shift their attention away from the problem
Having a behavior plan in place was rated as somewhat helpful.
Other approaches—such as family therapy or anger management programs—were generally not rated as effective for reducing temper outbursts in PWS. Parenting programs were reported as somewhat helpful, but very few families had tried them.
What About Medications?
A small number of individuals in the study (n=4) were given medication during an outburst to help calm them. Caregivers reported mixed results:
- Lorazepam and quetiapine were reported as helpful
- Valium was reported as less effective
These differences may be related to how quickly different medications work.
32 people with PWS in the study were prescribed daily medication to help manage aggression or temper outbursts. The most common were:
- Risperidone
- SSRIs (antidepressants)
Caregivers generally rated these medications as somewhat effective, but not a complete solution.
Are Temper Outbursts Related to Hyperphagia?
An important question the investigators were interested in was whether temper outbursts begin around the same time as hyperphagia.
This is difficult to determine, but given the results of the present study and those of a 2001 study by Dimitropoulos et al., it appears that temper outbursts may start before the onset of hyperphagia.
However, the two may still be connected in some way. The biological changes that lead to hyperphagia may begin before families notice clear changes in eating behavior (for example, weight gain may appear before intense food seeking).
Thus, while outbursts may not be triggered by hyperphagia, the mechanism driving these two behaviors may be interrelated in some way
Key Takeaways
Temper outbursts are common in PWS and often continue across the lifespan. While some medications may help reduce their frequency or intensity, their effects are usually modest.
The study highlights several strategies that families report can help:
- Creating space from the trigger
- Allowing time to calm down
- Using distraction to shift attention
These approaches may work because many people with PWS have challenges with emotional regulation and self-control.
Research in both typically developing children and individuals with autism has shown that difficulties with emotion regulation are strongly linked to outbursts and aggressive behavior (Roben, et al.) (Samson, et al.). Because of this, researchers believe that a better understanding of emotional regulation in PWS may be an important step toward developing more effective interventions.
Future studies may help identify new behavioral strategies and non-medication approaches that could make temper outbursts easier to manage for individuals with PWS and their families.
Learn More
Several areas of research are currently exploring new ways to support emotional regulation and behavior in PWS.
Mindfulness - A number of studies suggest that regular mindfulness practices can benefit both individuals with special needs and their caregivers. Learn more about mindfulness for caregivers and people with PWS
Vagus Nerve Stimulation (VNS) - Early research suggests that vagus nerve stimulation may help reduce temper outbursts and improve emotional regulation in some individuals with PWS. Study Shows Vagus Nerve Stimulation Could Help Treat Behavior in PWS
More expert advice on PWS-related behavior from the FPWR blog:
