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Pain experience and perception in individuals with Snijders Blok-Campeau syndrome
Don Daniel Ocay1,2, Philippe M Campeau3, Charles B Berde1,2,4
1Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Boston, MA, United States.
Insights
Caregivers reported that individuals with Snijders Blok-Campeau Syndrome (SNIBCPS) may have decreased pain sensation. This highlights the need for careful observation to ensure pain and injuries are not missed in these patients.
Area of Science:
- Neurodevelopmental Disorders
- Pain Perception Research
- Pediatric Neurology
Background:
- Snijders Blok-Campeau Syndrome (SNIBCPS) is a neurodevelopmental disorder with limited understanding of pain perception.
- Anecdotal reports suggest altered pain sensation in individuals with SNIBCPS.
Purpose of the Study:
- To investigate pain perception and response in individuals with SNIBCPS.
- To understand the experiences of individuals with SNIBCPS regarding pain through proxy reports.
Main Methods:
- A mixed-methods anonymous survey study involving 15 caregivers of individuals with SNIBCPS.
- Utilized validated pain assessment tools including the Pediatric Pain Profile and Non-Communicative Children's Pain Checklist-Revised.
Main Results:
- Nearly a quarter of caregivers reported insensitivity to hard impacts or pressure in individuals with SNIBCPS.
- Findings indicate a range of pain expression behaviors and highlight past painful experiences.
Conclusions:
- Increased awareness is needed for the proper examination of individuals with SNIBCPS.
- Pain and injuries may be underreported in SNIBCPS, emphasizing the value of individualized parental observational scales for healthcare providers and caregivers.
Introduction:
Snijders Blok-Campeau Syndrome (SNIBCPS) is a neurodevelopmental disorder characterized by intellectual disability, developmental delays, speech impairment, hypotonia, and distinctive facial features. Little is known about pain perception in children with cognitive impairments, such as patients with SNIBCPS. Although it has been noted that some individuals with SNIBCPS have decreased pain sensation and response to painful stimuli, these reports are anecdotal. Therefore, the objective was to better understand this syndrome and the affected individual's perception and response to pain through proxy-reported observational assessments.
Methods:
Fifteen caregivers of individuals with a diagnosis of SNIBCPS participated in this mixed-methods anonymous survey study between July and September 2024. The survey questionnaires included the Pediatric Pain Profile, a Pain Sensory Questionnaire, the Non-Communicative Children's Pain Checklist-Revised, and the Individualized Numerical Rating Scale.
Results:
Almost a quarter of our respondents reported insensitivity in the affected individual to hard impacts or pressure. Our findings highlight early and past painful experiences in individuals with SNIBCPS who have a range of behaviors to express their pain.
Discussion:
Our findings bring awareness about the proper examination of individuals with SNIBCPS. Despite the small sample size, our findings suggest that pain and injuries may go unreported in individuals with SNIBCPS, and individualized parental observational scales may be beneficial for their healthcare providers and their caregivers.
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