Anxiety and Depression Common in School-Age SMA Patients in China

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School-aged children and adolescents with spinal muscular atrophy (SMA) in China experience high rates of anxiety and depression, a questionnaire-based study suggests.

“Professional psychological care [may be] included in standard of care, ”the researchers wrote, noting that a higher prevalence of depression has been observed in students with ADS who have respiratory and digestive problems and skeletal deformities.

“These results also call for possible targets for intervention such as reducing complications, improving access to drugs, maintaining normal schooling, strengthening academic support and improving the capacity of children. caregivers of SMA patients to aid in the diagnosis and treatment of the disease, thereby improving the mental health of SMA patients, ”the investigators wrote.

The study, “Anxiety and depression in school-aged patients with spinal muscular atrophy: a cross-sectional study, ”Was published in the Orphanet Journal of Rare Diseases.

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Muscle weakness and atrophy (narrowing) are hallmarks of ADS, but patients can also experience digestive, respiratory, cardiovascular, and motor problems, which can seriously reduce their quality of life.

Anxiety, depression, and other psychological disorders often accompany chronic physical disorders and can have a dramatic impact on disease progression. Such psychological symptoms can also complicate the symptoms and increase the difficulty of diagnosis and treatment.

Research shows that depression and anxiety can reduce interest in social activities, limit motivation, and impair cognitive function, further reducing social functioning and quality of life.

In adults with ADS, studies have shown that patients’ anxiety and depression levels are significantly affected by the disease. However, the psychological health of school-aged children and adolescents with ADS has not been fully explored.

“The aim of the present study was to describe the prevalence of anxiety and depression and to study the risk factors for these in school-aged SMA patients,” according to researchers from the School of medicine from Zhejiang University, China. All but one of the researchers are affiliated with the university’s National Clinical Research Center for Child Health, based in Hangzhou.

Two questionnaires, the Screen for Child Anxiety-Related Emotional Disorders (SCARED) and the Depression Self-Rating Scale for Children (DSRSC), were used to assess anxiety and depression. In either case, higher scores indicate worse anxiety and depression. Questionnaires were completed over the phone, or in face-to-face interviews, or via an online platform with patients and their caregivers.

The study involved 155 respondents, diagnosed with SMA, who were aged 8 to 18 (mean 10.28); most of the students (78.7%) were between 8 and 12 years old. Of the students, 71 (45.8%) were men and 84 (54.2%) were women.

Infantile-onset SMA type 1 was diagnosed in 12 of the participants (7.7%), while type 3 SMA, also referred to as juvenile SMA, was confirmed in 42 (27.1%). The majority of students – 101 or 65.2% – had an intermediate type 2 ADS, which typically develops between 7 and 18 months.

Eight participants were treated with Spinraza (nusinersen) and three were diagnosed with anxiety or depression.

A total of 48 patients attended a personalized school, where children can get special academic, emotional or physical support from the school or teachers. The rest were in traditional mainstream schools without support programs for ADS patients and without personalized supports and equipment. For more than half of the respondents, school delays were reported.

Overall, 60 respondents (40.0%) generated a SCARED score of 25 or higher, which met the definition of anxiety, while 39 (25.2%) had a DSRSC score of 15 or higher. , meeting the criteria for depression.

Notably, depression and anxiety were also high in most caregivers. When asked about themselves, most caregivers answered “Yes” to questions about their subjective anxiety (83.9%) and depression (80.0%).

The presence of anxiety in children was significantly correlated with respiratory and digestive problems as well as skeletal deformities, but did not differ significantly in terms of sex, age or type of disease.

“Patients of type [3] and type [1] SMA had the highest and lowest anxiety prevalence rates, respectively, although this difference did not reach statistical significance, ”the team wrote.

“A possible explanation for these sightings is this type [3] patients retain more mobility than the type [1] patients and can therefore maintain normal contacts, but this type [3] patients cannot enjoy the same activity levels as their normal peers, which leads to anxiety, ”they wrote.

There was a significantly lower prevalence of severe anxiety among those who exercised compared to those who did not exercise.

In addition, children with school delays were more likely to be anxious than those without delay. There was also a significant correlation between depression and academic delay as well as higher rates among those who attended traditional and regular schools.

Patients who resided in households with high annual income reported a significantly lower prevalence of depression than those from low-income families. There was also a significant inverse correlation between higher anxiety and a lower household income level, meaning that people with lower family income were much more likely to have greater anxiety.

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Higher anxiety in school-aged SMA children was also positively correlated with subjective anxiety of their caregivers, and subjective depression of caregivers was significantly associated with anxiety in children, “such that patients increased as the subjective depression of their caregivers increased, ”the researchers wrote.

Additionally, patient anxiety was significantly lower when caregivers had high expectations for their diagnosis and treatment than when caregivers had low expectations.

As seen with anxiety, a higher prevalence of depression was also seen in patients with respiratory and digestive problems and skeletal deformities than in those without these problems. On the other hand, no difference was observed concerning the sex and the type of SMA. Depression in patients who did rehabilitation exercise was significantly lower than in those who did not.

“The lack of a significant difference in the prevalence of anxiety and depression between the sexes in our study could be explained by the fact that SMA morbidity is not linked to sex,” the scientists wrote.

Finally, depression in patients was positively correlated with subjective caregiver anxiety, but depression in patients was no different from subjective depression in caregivers. In addition, caregiver expectations significantly affected depression in patients.

“In this study sample of SMA patients in China, depression and anxiety were prevalent, affecting more than a quarter and a third of the study population, respectively,” the researchers wrote.

“Professional psychological care for SMA patients is essential,” they added. “Scaling up mental health services, including personalized education, improved mental health and caregiver expectations, and precise management of multisystem disorders, is also crucial for the prevention of anxiety and depression in patients with SMA. “


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