Advertisement

Diaphragm Pacers in Pediatric Patients with Cervical Spinal Cord Injury: a Review and Implications for Inpatient Rehabilitation

  • Janet M. DeanEmail author
  • Raymond P. Onders
  • Mary Jo Elmo
Spinal Cord Injury Rehabilitation (CL Sadowsky, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Spinal Cord Injury Rehabilitation

Abstract

Purpose of review

Pediatric spinal cord injury (SCI) at C3 level and above results in tetraplegia and profound respiratory insufficiency requiring long-term positive pressure ventilation (PPV) and is associated with significant complications and morbidity. Diaphragm pacing (DP), a form of functional electrical stimulation (FES) of the diaphragm, provides natural negative pressure breathing which can replace or decrease PPV need. There are numerous reported benefits of DP including improved speech, reduced caregiver burden, and decrease in secretions. The purposes of this paper are to review the limited literature on the safety and effectiveness of DP in pediatric SCI and describe collaboration between two unaffiliated, geographically separated institutions to facilitate implantation of DP for children with ventilator-assisted tetraplegia during inpatient rehabilitation. We further describe the process for ventilator weaning/diaphragm conditioning during inpatient rehabilitation.

Recent findings

Two multicenter studies in adult patients with SCI demonstrate safety of the laparoscopic implantation procedure and effectiveness of the DPS system. The first pediatric patient was implanted in 2009, and since then, a total of 22 children with SCI had the system implanted. Among them, six children have now benefited from medically supervised multidisciplinary ventilator weaning/diaphragm conditioning supported by intensive activity-based restorative therapies (ABRT).

Summary

For the pediatric patients reviewed, implantation of DP was a safe and effective alternative to long-term PPV. Using DP in lieu of PPV provided freedom from ventilator tubing and fear of disconnection, simplifying and facilitating ABRT. We have found that the benefits of DP far surpass the significant effort needed to facilitate implantation during inpatient rehabilitation.

Keywords

Tetraplegia Diaphragm pacing Ventilator weaning Pediatric Respiratory insufficiency Rehabilitation 

Notes

Acknowledgments

Pam Kramer, nurse care manager for her contribution to this paper.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no competing interests.

Case Western Reserve University, Dr. Raymond Onders, and University Hospitals Case Medical Center have intellectual property rights involved with the diaphragm pacing system and equity in Synapse Biomedical that manufactures the device. Janet M. Dean and Mary Jo Elmo reported no biomedical financial interests or potential conflicts of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

  1. 1.
    National Spinal Cord Injury Statistical Center, University of Alabama at Birmingham, 2017 Annual Statistical Report – Complete Public Version.Google Scholar
  2. 2.
    Kokoska ER, Keller MS, Rallo MC, Weber TR. Characteristics of pediatric cervical spine injuries. J Pediatr Surg. 2001;36:100–5.  https://doi.org/10.1053/jpsu.2001.20022.CrossRefGoogle Scholar
  3. 3.
    DeVivo MJ, lvie CS. Life expectancy of ventilator-dependent persons with spinal cord injuries. Chest 1995;1 08:226–23 2.CrossRefGoogle Scholar
  4. 4.
    Shavelle RM, DeVivo MJ, Strauss DJ, et al. Long-term survival of person ventilator dependent after spinal cord injury. J Spinal Cord Med. 2006;29:511–9.CrossRefGoogle Scholar
  5. 5.
    Krause JS, Devivo MJ, Jackson AB. Health status, community integration, and economic risk factors for mortality after spinal cord injury. Arch Phys Med Rehabil. 2004;85:1764–73.CrossRefGoogle Scholar
  6. 6.
    Porth C. Recognition and management of respiratory dysfunction in children with tetraplegia. J Spinal Cord Med. 2004;27(Suppl 1):S75–9.CrossRefGoogle Scholar
  7. 7.
    Edwards JD, Kun SS, Keens TG. Outcomes and causes of death in children on home mechanical ventilation via tracheostomy: an institutional and literature review. J Pediatr. 2010;157:955–959.e2.CrossRefGoogle Scholar
  8. 8.
    Onders RP, Elmo MJ, Ignagni AR, et al. Diaphragm pacing stimulation system for tetraplegia in individuals injured during childhood or adolescence. J Spinal Cord Med. 2007;30(Suppl 1):S25–9.CrossRefGoogle Scholar
  9. 9.
    Dalal K, DiMarco AF. Diaphragmatic pacing in spinal cord injury. Phys Med Rehabil Clin N Am. 2014;25(3):619–29.  https://doi.org/10.1016/j.pmr.2014.04.004.CrossRefGoogle Scholar
  10. 10.
    Adler D, Gonzalez-Bermejo J, Duguet A, Demoule A, Le Pimpec-Barthes F, Hurbault A, et al. Diaphragm pacing restore olfaction in tetraplegia. Eur Respir J. 2009;34:365–70.CrossRefGoogle Scholar
  11. 11.
    Jarosz R, Littlepage MM, Creasey G, et al. Functional electrical stimulation in spinal cord injury respiratory care. Top Spinal Cord Inj Rehavil. 2012;18:315–21.CrossRefGoogle Scholar
  12. 12.
    Dolbow DR, Gorgey AS, Recio AC, Stiens SA, Curry AC, Sadowsky CL, et al. Activity-based restorative therapies after spinal cord injury: Inter-institutional conceptions and perceptions. Aging Dis. 2015;6(4):254–61.  https://doi.org/10.14336/AD20141105.CrossRefGoogle Scholar
  13. 13.
    Sadowsky C, McDonald J. Activity based restorative therapies (ABRT); concepts and applications in spinal cord injury-related neurorehabilitation. Dev Disabil Res Rev. 2009;15:112–6.  https://doi.org/10.1002/ddrr.61.CrossRefGoogle Scholar
  14. 14.
    Onders RP, Elmo M, Khansarinia S, Bowman B, Yee J, Road J, et al. Complete worldwide operative experience in laparoscopic diaphragm pacing: results and differences in spinal cord injured patients and amyotrophic lateral sclerosis patients. Surg Endosc. 2009;23(7):1433–40.  https://doi.org/10.1007/s00464-008-0223-3.CrossRefGoogle Scholar
  15. 15.
    Posluszny JA Jr, Onders R, Kerwin AJ, Weinstein MS, Stein DM, Knight J, et al. Multicenter review of diaphragm pacing in spinal cord injury: successful not only in weaning from ventilators but also in bridging to independent respiration. J Trauma Acute Care Surg. 2014;76(2):303–9; discussion 309-10.  https://doi.org/10.1097/TA.0000000000000112.CrossRefGoogle Scholar
  16. 16.
    Onders RP, Ponsky TA, Elmo M, Lidsky K, Barksdale E. First reported experience with intramuscular diaphragm pacing in replacing positive pressure ventilators in children. J Pediatr Surg. 2011;46(1):72–6.  https://doi.org/10.1016/j.jpedsurg.2010.09.071.CrossRefGoogle Scholar
  17. 17.
    Off-Label Use of Medical Devices in Children. Pediatrics. 2017;139(1). pii: e20163439. doi:  https://doi.org/10.1542/peds.2016-3439. Section On Cardiology And Cardiac Surgery; Section On Orthopaedics.
  18. 18.
    Onders RP, Elmo M, Kaplan C, Katirji B, Schilz R. Extended use of diaphragm pacing in patients with unilateral or bilateral diaphragm dysfunction: a new therapeutic option. Surgery. 2014;156(4):776–84.  https://doi.org/10.1016/j.surg.2014.07.021.CrossRefGoogle Scholar
  19. 19.
    Jubran A, Grant B, Duffner L, Collins E, Lanuza D, Hoffman L, et al. Effect of pressure support vs unassisted breathing through a tracheostomy collar on weaning duration in patients requiring prolonged mechanical ventilation. A randomized trial. J Am Med Assoc. 2013;309(7):671–7.CrossRefGoogle Scholar
  20. 20.
    Hall AM, Watt JWH. The use of tracheal stoma stents in high spinal cord injury: a patient-friendly alternative to long-term tracheostomy tubes. Spinal Cord. 2008;46:753–5.CrossRefGoogle Scholar
  21. 21.
    Massery M. Multisystem clinical implications of impaired breathing mechanics and postural control. In: Frownfelter D, Dean E, editors. Cardiovascular and pulmonary physical therapy: evidence to practice. 5th ed. St. Louis, MO: Elsevier-Mosby; 2012. p. 633–53.Google Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Janet M. Dean
    • 1
    Email author
  • Raymond P. Onders
    • 2
  • Mary Jo Elmo
    • 2
  1. 1.International Center for Spinal Cord InjuryKennedy Krieger InstituteBaltimoreUSA
  2. 2.Division of General SurgeryUniversity Hospitals Case Medical CenterClevelandUSA

Personalised recommendations