Identifying pain in children with CHARGE syndrome

Background and aims The objective was to conduct the first investigation to identify the frequency and intensity of pain experiences for individuals with CHARGE syndrome and to review the use of two established non-vocal pain assessments with children with CHARGE, the NCCPC-R (Non-Communicating Children's Pain Checklist-Revised) and the PPP (Pediatrics Pain Profile). Methods Parents of children with CHARGE were enrolled. Participants completed a pain questionnaire and the NCCPC-R and PPP twice, once for a baseline measure and second during a painful experience for their child. Results A moderate negative correlation between the mean intensity of pain and the mean duration of pain among individuals with CHARGE was found, ρ=-0.34. There was a tendency for intensity of pain to increase for sources of pain that were of shorter duration. The NCCPC-R and PPP were found to identify pain when compared to baseline performance (no pain) with a large effect, d=1.3. For the NCCPC-R, the difference between these ratings was significant beyond the 0.05 level, t (40)=8.15, p=0.000, 95% CI [16.93, 28.10]. Similarly, for the PPP, the mean pain ratings were significantly greater than the mean ratings for no pain, with significance beyond the 0.05 level, t (51)=9.59, p=0.000, CI 95% [11.74, 17.96]. Conclusions Evidence exists that children with CHARGE experience pain. While the NCCPC-R and PPP were found to identify pain; future research should consider the development of a pain assessment individualized to pain behaviors present in CHARGE syndrome, given this population's unique expression of pain.