Children with SMA face higher emotional and behavioral challenges
Study highlights need for integrated mental health support in care plan
- Children with SMA experience higher rates of emotional and behavioral difficulties, including anxiety and hyperactivity.
- These challenges often persist despite modern treatments and can significantly impact family life.
- Integrated mental health support is essential for optimal SMA patient care.
Children with spinal muscular atrophy (SMA) have higher rates of emotional and behavioral challenges than children in the general population, even with today’s modern treatments, a new study reports.
“In our treated SMA cohort, mental health challenges occurred across a variety of severities of motor function and created an adverse, often severe and longstanding impact on family life,” researchers wrote in the study, “Behavioural and emotional challenges in children with spinal muscular atrophy,” published in Pediatric Neurology.
“These findings highlight the importance of including mental health in optimal care, from routine screening to early diagnosis, the provision of early intervention and ongoing psychological support that is integrated into multidisciplinary neuromuscular care,” the researchers added, writing that such psychological care “can optimise a child’s capacity to live and thrive within their environments.”
Understanding SMA and its impact beyond motor function
SMA is a genetic disorder that causes progressive muscle weakness and wasting. In recent years, several disease-modifying therapies have become widely available. These treatments can address the underlying cause of SMA and can slow disease progression, helping preserve muscle strength and mobility.
In addition to challenges with mobility and muscle function, there’s evidence that SMA may also affect behavior and emotional development. However, this part of the disease is still not well understood. To better understand how SMA affects behavior and emotions, researchers in Australia surveyed 48 children, ages 4-17. All children were receiving disease-modifying treatments, and there was substantial variety in SMA severity — some could not sit unassisted, while others were able to walk.
All children, or the parents of the younger ones, completed the Strengths and Difficulties Questionnaire (SDQ), a standardized tool used to screen for behavioral and emotional difficulties. In the general population, about 10% of children have SDQ scores that indicate difficulties. Among these SMA children, more than a third (35.4%) met the SDQ threshold for difficulties overall, and more than half (60.4%) had difficulties on at least one subscale.
The most commonly reported issues were anxiety, hyperactivity, and temper tantrums. These were more common in the SMA children than population norms, but similar to rates seen in children with other neurological conditions such as cerebral palsy. However, the researchers said the children with SMA scored similarly to the general population on subscales measuring their ability to form relationships and interact with peers.
“Despite challenges, many children with SMA displayed strengths, particularly in prosocial behaviours, suggesting areas of preserved social functioning amid broader emotional difficulties,” the investigators wrote.
Parents report lasting challenges and emotional strain
A similar percentage of parents (33%) reported that their child had notable emotional or behavioral difficulties. Of the 16 parents who identified concerns, more than half said the problems had lasted for more than a year and were a notable burden on the family.
“The use of parent- and child-reported outcomes enrich insights gained into these complex cognitive processes from different perspectives, highlighting how they directly impact the way the individuals’ function and adapt to living with a complex neuromuscular condition,” the researchers wrote.
The children and parents also completed surveys assessing health-related quality of life in the context of a neuromuscular disease, including items about neuromuscular disease, communication, and family resources.
Parents reported issues such as limited mobility, difficulties with self-care (including use of the toilet or showering), and breathing challenges. The children consistently rated their own quality of life higher than the scores given by their parents.
“Children/young persons may perceive their quality of life more optimistically than their parents, possibly reflecting generational differences in emotional processing, expectations, or positive adaptation to chronic illness,” the researchers wrote, adding that these findings “reinforce the value of incorporating proxy- and self-reports in assessing outcomes, to fully capture the lived experiences of children with SMA.”
Although this study was limited to a single center, the researchers said the findings highlight the need for improved mental health support for people with SMA in the modern era. They called for efforts to integrate mental health into routine SMA care and noted that support programs and educational resources may help families struggling with these challenges.
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