Pandemic Affected Mental Health of PWS Patients, German Survey Finds

The COVID-19 pandemic greatly affected the mental health of people with Prader-Willi syndrome (PWS), with worsening of behavioral symptoms such as irritability, temper outbursts, and food‐seeking behaviour, according to results of an online survey conducted by researchers in Germany.

These findings are consistent with those of the Foundation for Prader-Willi Research’s “Impact of COVID” survey, which highlighted the negative impact of the pandemic on PWS patients and their families in the U.S. and the U.K.

The German survey also found that patients living in specialized care facilities appeared to be less vulnerable to the pandemic’s damaging effects than those living in their family home, highlighting the pronounced need for help for these families, the researchers said.

The study, “Behavioural change in Prader–Willi syndrome during COVID‐19 pandemic,” was published in the Journal of Intellectual Disability Research and conducted by researchers at Medical School Hannover, in Germany.

PWS can result in insatiable appetite, temper outbursts, obsessive-compulsive activities such as skin picking, intellectual disability, sleep disturbances, emotional and social difficulties, and resistance to changes in activities or routines.

“Above all, a structured environment seems essential for the well‐being of people with PWS,” the investigators wrote, adding that this stable environment is likely “disturbed by the effects of national responses to the COVID‐19 pandemic.”

To assess the impact of the pandemic on PWS patients’ mental health, the team conducted an online, caregiver-directed survey. It included questions about sociodemographic information — age, sex, body mass index (a measure of body fat), and type of accommodation — COVID-19-related data (test results, effect of restrictions in care and daytime activity), and changes in behavioral symptoms during the pandemic.

A total of 108 caregivers, with a mean age of 50.7 years (range from 24–79 years), completed the questionnaire. Most were women (78.7%) and a parent of the PWS patient (98.1%).

For the analysis of behavioral changes, assessed with a five-level scale, the team included data only from the 89 patients who were 6 years and older, as such changes may be difficult to assess in younger children. More than half of them were males (51.7%) and the mean age was 21.3 years (range from 6–56 years).

Results showed that 21.3% of the patients were tested for COVID-19, with three (2.8%) testing positive. Most caregivers reported pandemic-related restrictions in care (67.3%) and daily activities (82.4%) for these patients.

Most respondents also stated that PWS patients were concerned about the pandemic and its consequences (85.4%), as well as their own health (58%), and were generally able to implement government measures, such as wearing mouth and nose protection or following social distancing rules (77.7%).

Caregivers frequently reported worsening of behavioral symptoms in patients during the lockdown, with the most common being irritability (55%), temper outbursts (51.7%), skin picking (50.6%), conflicts with other people (46.1%), and sadness (43.8%).

More than one-third of patients were also reported to show more daytime sleepiness (40.4%), food-seeking behavior (39.3%), anxiousness (38.2%), and social withdrawal (33.7%).

Psychosis, in which people lose contact with reality, and suicidal thoughts did not change in most cases.

The team then investigated whether symptom worsening was different between patients living in a specialist care facility and those living with their families. Since the youngest patient in a care center was 14 years old, the analysis was limited to the 53 participants of that age or older: 28 lived in their family home (mean age 20.8) and 25 in a specialist care facility (mean age 30.8).

Data showed that worsening in food-seeking behavior and irritability were significantly more common among patients living in their family home than among those in a care center.

An increase in other behavioral symptoms was also less commonly reported for patients in care facilities, but these group differences did not reach statistical significance.

“Our data may suggest that PWS living at home with their families are more vulnerable to the effects of the [COVID-19] pandemic than those living in specialist facilities,” the researchers wrote, adding that “the structured environment in these facilities may have helped people with PWS better overcome the effects of the pandemic.”

“Families providing care to their child with PWS may benefit from additional professional support during such difficult times,” they wrote.

The team noted, however, the results may have been influenced by the 10-year age difference between the two groups and the fact that caregivers in care centers may not have had daily contact with patients to report symptom changes accurately. As such, more studies are needed to confirm these findings.

“The COVID‐19 pandemic has had a significant effect on the mental health of individuals with PWS, evidenced by an increase in behaviors associated with PWS, including temper outbursts, food‐seeking, and irritability, which again underlines their need for specialized care,” the researchers concluded.