Concerns of Silver–Russell syndrome patients extend beyond height
medwireNews: Patients with Silver–Russell syndrome experience issues with their appearance and with social interactions, as well as with their height, show findings from an interview-based study.
Lisa Marie Ballard (University of Southampton, UK) and colleagues interviewed 15 patients with Silver–Russell syndrome – all adults at the time – about their experiences of growing up with the condition.
The concerns identified fell into four categories: appearance-related issues; real and perceived adverse reactions from others; pain and disability; and feeling at a disadvantage when it came to forming romantic relationships.
“However, it should be noted that, at the time of interview, many participants had successful careers, were in romantic relationships, and some had, or were starting to have, families of their own”, the researchers write in the Archives of Disease in Childhood.
Psychosocial issues had a large impact during adolescence. During these years, the patients had problems with feeling different; experiencing staring, negative comments and bullying from peers; and even hearing negative comments from healthcare professionals. Although they reported successful friendships, they struggled with romantic relationships, feeling that their physical differences ruled them out as a romantic partner for the majority of people.
The research team therefore believes that psychosocial support should be implemented early, contrary to previous suggestions that the point of transition from paediatric to adult care would be the ideal time.
“Our findings indicate that a psychologist should be an integral member of a multidisciplinary team managing individuals with [Silver–Russell syndrome] during childhood, adolescence and adulthood, to aid in the development of strategies to manage any appearance-related concerns and other psychosocial issues that arise”, they say.
Short stature was frequently cited as an important physical issue (the participants’ adult height standard deviation scores ranged from –0.49 to –5.52); however, only two participants said this was the issue with the largest impact on them. Asymmetry had the largest impact for three participants; for others it was weight, speech (due to cleft lip/palette), restricted leisure activities and pain.
Pain had an impact both in childhood/adolescence and in adulthood, with the patients saying it was a problem per se, while also affecting employment, daily activities and other people who had to support them.
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