Skip to main content

Advertisement

SpringerLink
Log in

Pain Experience and Expression in Rett Syndrome: Subjective and Objective Measurement Approaches

  • ORIGINAL ARTICLE
  • Published:
Journal of Developmental and Physical Disabilities Aims and scope Submit manuscript

Abstract

Rett syndrome (RTT) is associated with myriad debilitating health issues and significant motor and communicative impairments. Because of the former there is concern about the possibility of recurrent and chronic pain but because of the latter it remains difficult to determine what pain ‘looks like’ in RTT. This study investigated pain experience and expression using multiple complementary subjective and objective approaches among a clinical RTT sample. Following informed consent, 18 participants (all female) with RTT (mean age = 12.8 years, SD = 6.32) were characterized in terms of pain experience and interference, typical pain expression, and elicited pain behavior during a passive range of motion-like examination procedure. Parents completed the Dalhousie Pain Interview (DPI; pain type, frequency, duration, intensity), the Brief Pain Inventory (BPI; pain interference), and the Non-Communicating Children’s Pain Checklist – Revised (NCCPC-R; typical pain expression). A Pain Examination Procedure (PEP) was conducted and scored using the Pain and Discomfort Scale (PADS). The majority of the sample (89 %) were reported to experience pain in the previous week which presented as gastrointestinal (n = 8), musculoskeletal (n = 5), and seizure related pain (n = 5) that was intense (scored 0–10; M = 5.67, SD = 3.09) and long in duration (M = 25.22 h, SD = 53.52). Numerous pain-expressive behaviors were inventoried (e.g., vocal, facial, mood/interaction changes) when parents reported their child’s typical pain behaviors and based on independent direct observation during a reliably coded pain exam. This study provides subjective and objective evidence that individuals with RTT experience recurring and chronic pain for which pain expression appears intact.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Barney, C. C., Krach, L., Rivard, P., Belew, J., & Symons, F. J. (2013). Motor function predicts parent-reported spasticity pain in children with cerebral palsy. Pain Research and Management, 18(6), 323–327.

    PubMed Central  PubMed  Google Scholar 

  • Belew, J., Barney, C. C., Schwantes, S., Tibboel, D., Valkenburg, A. J., & Symons, F. J. (2013). Pain in children with intellectual or developmental disabilities. In P. McGrath, B. Stevens, S. Walker, & W. Zempsky (Eds.), The oxford textbook of pediatric pain (pp. 147–156). Oxford: Oxford University Press.

    Google Scholar 

  • Bodfish, J. W., Harper, V. N., Deacon, J. M., Deacon, J. R., & Symons, F. J. (2006). Issues in pain assessment for adults with severe to profound mental retardation: from research to practice. In T. F. Oberlander & F. J. Symons (Eds.), Pain in children & adults with developmental disabilities (pp. 173–192). Baltimore: Paul Brookes Publishing Co.

    Google Scholar 

  • Breau, L. M., Camfield, C., McGrath, P. J., Rosmus, C., & Finley, G. A. (2001). Measuring pain accurately in children with cognitive impairments: refinement of a caregiver scale. Journal of Pediatrics, 138, 721–727.

    Article  PubMed  Google Scholar 

  • Breau, L. M., McGrath, P. J., Camfield, C. S., & Finley, G. A. (2002). Psychometric properties of the non-communicating children’s pain checklist-revised. Pain, 99(1–2), 349–357.

    Article  PubMed  Google Scholar 

  • Breau, L. M., Camfield, C. S., McGrath, P. J., & Finley, G. A. (2003). The incidence of pain in children with severe cognitive impairments. Archives of Pediatrics and Adolescent Medicine, 157(12), 1219–1226.

    Article  PubMed  Google Scholar 

  • Christianson, J. A., & Davis, B. M. (2010). The role of visceral afferents in disease. In L. Kruger & A. R. Light (Eds.), Translational pain research from mouse to man (pp. 51–76). New York: CRC Press, Taylor & Francis Group.

    Google Scholar 

  • Devarakonda, K. M., Lowthian, D., & Raghavendra, T. (2009). A case of Rett syndrome with reduced pain sensitivity. Paediatric Anesthesia, 19(6), 625–627.

    Article  Google Scholar 

  • Downs, J., Geranton, S. M., Bebbington, A., Jacoby, P., Bahi-Buisson, N., Ravine, D., et al. (2010). Linking MECP2 and pain sensitivity: the example of Rett syndrome. American Journal of Medical Genetics, 152A(5), 1197–1205.

    Article  PubMed  Google Scholar 

  • Geranton, S. M., Morenilla-Palao, C., & Hunt, S. P. (2007). A role for transcriptional repressor methl-CpG-binding protein 2 and plasticity-related gene serum- and glucocorticoid-inducible kinase 1 in the induction of inflammatory pain states. The Journal of Neuroscience, 27, 6163–6173.

    Article  PubMed  Google Scholar 

  • Hadjistavropoulos, T., & Craig, K. D. (2002). A theoretical framework for understanding self-report and observational measures of pain: a communications model. Behavior Research and Therapy, 40, 551–570.

    Article  Google Scholar 

  • Hagberg, B. (2002). Clinical manifestations and stages of Rett syndrome. Mental Retardation and Developmental Disabilities Research Reviews, 8, 61–65.

    Article  PubMed  Google Scholar 

  • Hagberg, B., Hanefeld, F., Percy, A., & Skjeldal, O. (2002). An update on clinically applicable diagnostic criteria in Rett syndrome: comments to the Rett syndrome clinical criteria consensus panel satellite to European paediatric neurology society meeting Baden Baden, Germany, 11 september, 2001. European Journal of Paediatric Neurology, 6, 293–297.

    Article  PubMed  Google Scholar 

  • Hennequin, M., Faulks, D., & Roux, D. (2000). Accuracy of estimation of dental treatment need in special care patients. Journal of Dentistry, 28, 131–136.

    Article  PubMed  Google Scholar 

  • Hwang, C. K., Song, K. Y., Kim, C. S., Choi, H. S., Guo, X. H., Law, P. Y., et al. (2009). Epigenetic programming of μ-opioid receptor gene in mouse brain is regulated by MeCP2 and brg1 chromatin remodeling factor. Journal of Cellular and Molecular Medicine, 13(9B), 3591–3615.

    Article  PubMed  Google Scholar 

  • Khasabov, S. G., Rogers, S. D., Ghilardi, J. R., Peters, C. M., Mantyh, P. W., & Simone, D. A. (2002). Spinal neurons that possess the substance P receptor are required for the development of central sensitization. The Journal of Neuroscience, 22, 9086–9098.

    PubMed  Google Scholar 

  • Konen, A. A., Joshi, G. P., & Kelly, C. K. (1999). Epidural analgesia for pain relief after scoliosis surgery in a patient with Rett’s syndrome. Anesthesia and Analgesia, 89, 451–452.

    PubMed  Google Scholar 

  • Nader, R., Oberlander, T. F., Chambers, C. T., & Craig, K. D. (2004). Expression of pain in children with autism. Clinical Journal of Pain, 20, 88–97.

    Article  PubMed  Google Scholar 

  • Neul, J. L., Kaufmann, W. E., Glaze, D. G., Christodoulou, J., Clarke, A. J., Bahi-Buisson, N., Leonard, H., Bailey, M. E. S., Schanen, N. C., Zappella, M., Renieri, A., Huppke, P., & Percy, A. K. (2010). Rett syndrome: revised diagnostic criteria and nomenclature. Annals of Neurology, 68(6), 944–950.

    Article  PubMed Central  PubMed  Google Scholar 

  • Osborne, T. L., Raichle, K. A., Jensen, M. P., Ehde, D. M., & Kraft, G. (2006). The reliability and validity of pain interference measures in persons with multiple sclerosis. The Journal of Pain and Symptom Management, 32(3), 217–229.

    Article  Google Scholar 

  • Phan, A., Edwards, C. L., & Robinson, E. L. (2005). The assessment of pain and discomfort in individuals with mental retardation. Research in Developmental Disabilities, 26(5), 433–439.

    Article  PubMed  Google Scholar 

  • Ramstad, K., Jahnsen, R., Skjeldal, O. H., & Diseth, T. H. (2011). Characteristics of recurrent musculoskeletal pain in children with cerebral palsy aged 8 to 18 years. Developmental Medicine and Child Neurology, 53, 1013–1018.

  • Samaco, R. C., Fryer, J. D., Ren, J., Fyffe, S., Chao, H. T., Sun, Y., et al. (2008). A partial loss of function allele of methyl-CpG-binding protein 2 predicts a human neurodevelopmental syndrome. Human Molecular Genetics, 17(12), 1718–1727.

    Article  PubMed Central  PubMed  Google Scholar 

  • Schiavenato, M., & Craig, K. D. (2010). Pain assessment as a social transaction: beyond the “gold standard”. Clinical Journal of Pain, 26(8), 667–676.

    PubMed  Google Scholar 

  • Symons, F. J., Byiers, B., Tervo, R., & Beisang, A. (2013). Parent-reported pain in Rett syndrome. Clinical Journal of Pain, 29(8), 744–746.

    Article  PubMed Central  PubMed  Google Scholar 

  • Tyler, E. J., Jensen, M. P., Engel, J. M., & Schwartz, L. (2002). The reliability and validity of pain interference in persons with cerebral palsy. Archives of Physical Medicine and Rehabilitation, 83(2), 236–239.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors wish to acknowledge the support of the Observational Methods Lab in the Dept. of Educational Psychology at the University of Minnesota and specifically Alyssa Merbler for her technical assistance. This study was supported, in part, by NICHD Grant No: 73126, 44763, and a Coffman Professorship (FS) from the College of Education & Human Development at the University of Minnesota. We are indebted to the families and their daughters for their participation and their patience.

Ethical Approval

All procedures performed in this study involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Author information

Authors and Affiliations

  1. Gillette Children’s Specialty Healthcare, 200 University Ave, Saint Paul, MN, 55101, USA

    Chantel C. Barney, Timothy Feyma & Arthur Beisang

  2. Department of Educational Psychology, University of Minnesota, 56 East River Road, Minneapolis, MN, 55455, USA

    Chantel C. Barney & Frank J. Symons

Authors
  1. Chantel C. Barney
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Timothy Feyma
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Arthur Beisang
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Frank J. Symons
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Chantel C. Barney.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Barney, C.C., Feyma, T., Beisang, A. et al. Pain Experience and Expression in Rett Syndrome: Subjective and Objective Measurement Approaches. J Dev Phys Disabil 27, 417–429 (2015). https://doi.org/10.1007/s10882-015-9427-3

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10882-015-9427-3

Keywords

Search

Navigation