“Adaptive skills as measured in the study
worsen relative to typical peers,” said
Dr. Berry-Kravis. “They do not actually
get worse in that the children with
Fragile X syndrome are not regressing
or losing skills.”An article on SFARI.org entitled “In Fragile X syndrome, coping skills decline with age” has been making rounds with the Fragile X community on social media. In the article, author Jessica Wright draws conclusions from a Stanford study published in Pediatrics, saying that “in people with Fragile X syndrome, these adaptive skills may worsen as they get older, just when they need them the most.” (Editor’s Note: The original article has since been amended to reflect Dr. Klaiman’s comments on the study.)
Wright’s article drew criticism from parents whose children live with Fragile X syndrome (FXS), who responded that her conclusions are much different from what they experienced at home.
To clear up the confusion, we reached out to a lead author on the study, Dr. Cheryl Klaiman.
In no way were we intending to imply that individuals with FXS lose skills as they age. We don’t believe that this is the case in the least. What we were stating was that individuals with FXS are not keeping pace with typically developing individuals. As they age, this gap widens, creating even greater discrepancies between individuals with FXS and their typically developing peers.
In light of these findings, we were suggesting that educational and other therapeutic programs should consider the inclusion of adaptive behavior skills into the curriculum so that individuals can live as independent lives as possible. It is believed that with a focus on adaptive living skills, individuals with FXS can certainly grow and attain skills to hopefully narrow this gap.
Thus, in response to your questions, we believe that individuals with FXS slow in their acquisition of skills as they age. This may be because the skills required as one ages become more complex and thus require more intensive intervention in order to learn.
I am sorry for any confusion or upset that this press may have caused any families. This was in no way our intent. Instead, our intent was to bring attention to the fact that there are strengths and weaknesses in adaptive behavior in individuals with FXS and we need to, as a community, pay attention to these skills and times of decrease so as to know when and where to focus our intervention efforts.
Dr. Elizabeth Berry-Kravis of Rush University also agreed. “[Individuals with FXS] do not actually get worse in that they are not regressing or losing skills. They just fail to learn and develop new skills as fast as typical children. So their relative scores get worse, just like their IQ scores (which are also relative to typically developing children) get worse as the patient with FXS gets older during childhood, and then the IQ score stays the same in adulthood.”
She also commented on the study itself: “This study is a very important study that helps us understand the long-term trajectory of FXS,” said Dr. Berry-Kravis. “It will be critical as a natural history comparison, to which we can compare the long-term effects of new targeted medication and behavioral interventions on the natural history of adaptive behavior in FXS.”