Background: Sanfilippo syndrome and Batten disease are rare genetic groups of progressive, incurable and fatal neurodegenerative disorders. Primarily affecting children, these disorders comprise two of the most  common forms of childhood dementia (CD) and are associated with multiple pain conditions including spasticity, dystonia, and gastrointestinal complications. However, pain self-report is limited due to communication difficulties associated with cognitive and motor decline and as such, the characteristics of pain in individuals with CD remain poorly understood. 

Aim: To explore the characteristics of pain in CD. 

Methods: An observational study was conducted in a sample of non-verbal individuals with a confirmed diagnosis of CD (Batten disease or Sanfilippo syndrome) who were referred to Dementia Support Australia (DSA), a national dementia-specific behaviour support service that provides 24/7 multimodal psychosocial interventions. The sample’s pain was measured by PainChek®, a point of care Class I medical device application (app). PainChek® pain intensity scores are as follows: 0-6 (no pain), 7-11 (mild pain), 12-15 (moderate pain), ≥16 (severe pain). A descriptive account of pain characteristics, such as pain levels was reported. 

Results:  Five DSA referrals (80% female) with CD (Sanfilippo syndrome n = 4, Batten disease n =1) were included in the study. Primary behaviours warranting referral included physical and verbal aggression, agitation, anxiety, aberrant motor behaviours, and irritability. A total of six pain assessments (rest n = 2, movement n = 4) were completed. Mild pain was recorded for three individuals, moderate pain for one individual and two had severe pain. 

Conclusions: Pain may be a prevalent, underrecognised aspect of CD, and may be causally connected to the primary behaviours and psychological symptoms of dementia (BPSD) such as those described here. Access to palliative care services should be offered early to individuals with CD to alleviate pain symptoms and behaviours associated with these disorders.