Ageing in Rett syndrome.
Expect steady skills but rising mood, dental, and gut problems as your Rett clients grow older.
01Research in Context
What this study did
Erickson et al. (2016) followed people with Rett syndrome as they aged. They tracked adaptive skills, repetitive behaviors, mood, teeth, and stomach health over time.
The team used parent reports and medical charts. They wanted to see which problems stay the same and which get worse.
What they found
Adaptive living skills and hand stereotypies stayed flat. Mood and interest in activities dipped. Dental and gut troubles grew worse.
In short: behavior held steady, but health and happiness slipped.
How this fits with other research
Waller et al. (2010) saw the opposite in Down syndrome. When adults there lost thinking skills, behavior also slid. Rett adults kept behavior stable even while health sank.
Tsao et al. (2015) found wide swings in aging Down adults. Some stayed sharp, others dropped fast. Rett showed less person-to-person spread.
Oppewal et al. (2018) warn that older adults with ID often die from lung infections. R et al. add dental and gut issues to the watch list for Rett clients.
Why it matters
You can relax about sudden behavior crashes in aging Rett clients, but you must stay alert for low mood, tooth pain, and constipation. Build dental checks, GI screens, and mood rating scales into yearly plans. Share the list with physicians and dentists so small problems are caught before they snowball.
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02At a glance
03Original abstract
BACKGROUND: The aim was to gain a UK national sample of people with Rett syndrome across the age range and (1) conduct a cross-sectional comparison of age groups and (2) undertake a longitudinal follow-up. METHODS: From 308 potential participants approached to take part, a sample of 91 girls and women was achieved (29.5%). Their ages ranged from 4 to 47 years, and 71 were known to have a mutation in the methyl-CpG binding protein-2 (MECP2) gene. Seventy-two of the initial sample were followed up 16 months later, and 50 returned completed assessments (69.4%). Their ages ranged from 7 to 48 years, and 42 were MECP2 positive. Parental questionnaire measures of Rett syndrome specific characteristics, impulsivity, overactivity, mood, interest and pleasure, repetitive behaviour and self-injury were administered. RESULTS: Adaptive behaviour and behavioural characteristics of Rett syndrome were similar across age groups and, where assessed, stable over time, as were repetitive behaviours generally and self-injury. There was some suggestion of deterioration in health arising with ageing, principally contributed to by deteriorations in dental and gastro-intestinal problems both with moderate effect sizes. Indicators of mood, interest and pleasure differed significantly across age groups. The total scale score significantly deteriorated over time, with a moderate effect size. CONCLUSIONS: This study provides further evidence for the post-regression stability that characterises Rett syndrome. Emergent low mood in Rett syndrome requires further research.
Journal of intellectual disability research : JIDR, 2016 · doi:10.1111/jir.12228