Note: This story was updated July 24, 2020, to note that Rett affects mostly girls and women, but can also occur in males.
About 40% of parents whose daughters have Rett syndrome show clinically high levels of stress, an Italian study found. Stress levels correlate with both disease severity and the amount of time spent caring for the child.
The study, “Parenting girls with Rett syndrome: An investigation on self‐perceived levels of stress,” was published in the Journal of Applied Research in Intellectual Disabilities.
Rett syndrome mostly affects women and girls, although it can also occur in males, with neurodevelopmental symptoms typically starting between 6 and 18 months of age. Among other manifestations, Rett children often develop epilepsy and scoliosis, and have difficulties with walking and communicating.
Prior research found that anxiety, depression and stress common among parents of Rett syndrome patients. Some are able to show resiliency, especially those who are pre-disposed to a demanding style of care and who share responsibilities with their partner.
Most of these studies addressed psychological symptoms of mothers due to their predominant role in caring.
A research team with the Developmental Neurology and Psychiatry Unit at Don Carlo Gnocchi Foundation in Milan sought to evaluate the effects of caring for a daughter with Rett syndrome on both parents. Fathers, the scientists said, may also offer useful information regarding their perspectives on family life, and in terms of the rehabilitation treatments.
Researchers contacted 79 couples whose daughters had Rett syndrome. A total of 70 fathers (mean age, 46.4) and 71 mothers (mean age, 43.7) responded to the questionnaire, with at least one parent responding for each of the 79 patients (mean age, 12).
Parents were asked to fill out the short form of the Parenting Stress Index, a 36-item questionnaire that categorizes the stress of parenting based on four subscales — the Parental Distress (PD) scale, the Parent-Child Dysfunctional Interaction (P-CDI) scale, the Difficult Child (DC) scale, and the Defense Levels (DL) scale.
A total score above the 85th percentile according to the child’s age range (up to age 2, 3 to 6, 6 to 9, and older than 9) was considered clinically relevant.
Parents and their daughters also had a one-day personal evaluation, meeting with a team that included a pediatric neurologist, psychologist, neurodevelopmental therapist, and a music therapist.
Results showed that 27 fathers (38.6%) and 31 mothers (43.6%) had clinical levels of stress.
As for the subscales, 24.3% of fathers had clinical levels on the PD scale, 44.3% on P-CDI, 32.8% on DC, and 21.4% on the DL scale. Clinical levels of stress were found in 38% of mothers on the PD scale, 46.5% on P-CDI, 36.6% on DC, and 39.4% on the DL scale.
Scores on the PD and DL scales scores were significantly lower in fathers than in mothers, a comparison showed, but other metrics, including total stress, were not significantly different.
The study then examined Rett severity in the 79 girls, looking at scoliosis, difficult walking, and epilepsy as indicators.
Twenty of these 79 (28%) had no scoliosis, 30 (38%) had mild scoliosis, 17 (21%) had moderate scoliosis, and 10 (13%) had severe scoliosis. Exams also found that 66 girls (83.5%) required assistance with walking and were prescribed a wheelchair, and that 18 used wheelchair or stroller to move.
In turn, 27 girls (34%) did not have epilepsy, 28 (35%) had epilepsy controlled with medication, and 24 (30%) had epilepsy resistant to treatment.
Overall severity of Rett was measured by the Rett Assessment Rating Scale (RARS), a 31-item survey for parents that incorporates cognition, sensory and motor abilities, emotions, daily life autonomy, and typical Rett features. Each question is assessed on a 1 to 4 point scale. A score of 55 or less indicates mild impairment, 56 to 81 indicates moderate impairment, and 82 to 124 marks severely impaired.
Researchers then looked at correlations between disease severity and parental stress. In fathers, greater Rett severity was associated with higher total stress, as well as higher scores on the P-CDI and DC subscales. In mothers, greater disease severity correlated with a higher P-CDI score.
Stress levels were also higher in parents — particularly mothers — of older girls, indicating the cumulative effect of caring contributes to clinical stress.
No links were found between having scoliosis or epilepsy and higher levels of stress.
“This study demonstrated that, while in general fathers and mothers can deal with the stress of caring for a girl with RTT [Rett], a certain proportion of them show high (and clinical) levels of stress,” the researchers wrote. “Moreover, disease severity is a crucial predictor of this outcome.”
However, a “substantial portion” — about 61% of the fathers and 56% of the mothers — were able to cope with this disease without clinical stress levels, they added, possibly due to “resilience mechanisms” or “the processes of reward and emotional enrichment” that come with being a caregiver.
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