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An Assistive Device for Congenital Central Hypoventilation Syndrome Outpatients During Sleep

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Abstract

Congenital Central Hypoventilation Syndrome is a genetic disease characterized by alveolar hypoventilation and autonomic dysregulation. Patients have hypoventilations, especially during sleep, conditioning hypercapnia which can lead to neurological damage and death. They therefore need mechanical ventilators, that provide sufficient gas exchange, and pulse-oximeters that monitor oxy-hemoglobin blood concentration. Due to the restrictions regarding domiciliary assistive devices, the presence of a caregiver is required all night long. Currently, the only alarm systems available are the ones integrated in the ventilators and monitoring systems. During the night, multiple false alarms may occur, interrupting the sleep and causing anxiety. In this work we describe an assistive device that acquires real-time data from a pulse-oximeter, provides a multisensory stimulation if oxygen saturation falls under a certain threshold, and wakes up the patient if the hypoxia is severe. Tests on healthy subjects have shown that the device guarantees rapid awakenings, with a stimulator-dependent efficacy, and that it does not affect sleep efficiency. The purpose of the device is to determine a gentle awakening if mild hypoxia conditions persist, and to assure rapid awakening when a severe hypoxia occurs, reducing false alarms, improving the quality of sleep and increasing the self-sufficiency of the patients.

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References

  1. Amiel, J., B. Laudier, T. Attie-Bitach, H. Trang, L. de Pontual, B. Gener, D. Trochet, H. Etchevers, P. Ray, M. Simonneau, M. Vekemans, A. Munnich, C. Gaultier, and S. Lyonnet. Polyalanine expansion and frameshift mutations of the paired-like homeobox gene PHOX2B in congenital central hypoventilation syndrome. Nat. Genet. 33:459–461, 2003.

    Article  CAS  PubMed  Google Scholar 

  2. Ancoli-Israel, S., R. Cole, C. Alessi, M. Chambers, W. Moorcroft, and C. P. Pollak. The role of actigraphy in the study of sleep and circadian rhythms. Sleep 26:342–392, 2003.

    PubMed  Google Scholar 

  3. Balfour-Lynn, I. M., D. J. Field, P. Gringras, B. Hicks, E. Jardine, R. C. Jones, A. G. Magee, R. A. Primhak, M. P. Samuels, N. J. Shaw, S. Stevens, C. Sullivan, J. A. Taylor, C. Wallis, and Paediatric Section of the Home Oxygen Guideline Development Group of the BTS Standards of Care Committee. BTS guidelines for home oxygen in children. Thorax 64(Suppl 2):ii1–ii26, 2009.

    Article  PubMed  Google Scholar 

  4. Bass, J. L., M. Corwin, D. Gozal, C. Moore, H. Nishida, S. Parker, A. Schonwald, R. E. Wilker, S. Stehle, and T. B. Kinane. The effect of chronic or intermittent hypoxia on cognition in childhood: a review of the evidence. Pediatrics 114:805–816, 2004.

    Article  PubMed  Google Scholar 

  5. Cavalleri, M., A. Carcano, F. Morandi, C. Piazza, E. Maggioni, and G. Reni. A new device for the care of Congenital Central Hypoventilation Syndrome patients during sleep. Conf. Proc. IEEE Eng. Med. Biol. Soc. 2013:2445–2448, 2013.

    CAS  PubMed  Google Scholar 

  6. Gozal, D., C. L. Marcus, S. L. Ward, and T. G. Keens. Ventilatory responses to passive leg motion in children with congenital central hypoventilation syndrome. Am. J. Respir. Crit. Care Med. 153:761–768, 1996.

    Article  CAS  PubMed  Google Scholar 

  7. Gozal, D., and N. Simakajornboon. Passive motion of the extremities modifies alveolar ventilation during sleep in patients with congenital central hypoventilation syndrome. Am. J. Respir. Crit. Care Med. 162:1747–1751, 2000.

    Article  CAS  PubMed  Google Scholar 

  8. Hager, A., W. Koch, H. Stenzel, J. Hess, and J. Schober. Effects of movement and work load in patients with congenital central hypoventilation syndrome. Eur. J. Cardiovasc. Prev. Rehabil. 14:294–298, 2007.

    Article  PubMed  Google Scholar 

  9. Healy, F., and C. L. Marcus. Congenital central hypoventilation syndrome in children. Paediatr. Respir. Rev. 12:253–263, 2011.

    Article  CAS  PubMed  Google Scholar 

  10. Huang, J., I. M. Colrain, H. B. Panitch, I. E. Tapia, M. S. Schwartz, J. Samuel, M. Pepe, P. Bandla, R. Bradford, Y. P. Mosse, J. M. Maris, and C. L. Marcus. Effect of sleep stage on breathing in children with central hypoventilation. J. Appl. Physiol. 105:44–53, 2008.

    Article  PubMed Central  PubMed  Google Scholar 

  11. Hyde, M., D. M. O’Driscoll, S. Binette, C. Galang, S. K. Tan, N. Verginis, M. J. Davey, and R. S. Horne. Validation of actigraphy for determining sleep and wake in children with sleep disordered breathing. J. Sleep Res. 16:213–216, 2007.

    Article  PubMed  Google Scholar 

  12. Jean-Louis, G., D. F. Kripke, R. J. Cole, J. D. Assmus, and R. D. Langer. Sleep detection with an accelerometer actigraph: comparisons with polysomnography. Physiol. Behav. 72:21–28, 2001.

    Article  CAS  PubMed  Google Scholar 

  13. Kam, K., C. Bjornson, and I. Mitchell. Congenital central hypoventilation syndrome; safety of early transition to non-invasive ventilation. Pediatr. Pulmonol. 49:410–413, 2013.

  14. Kushida, C. A., A. Chang, C. Gadkary, C. Guilleminault, O. Carrillo, and W. C. Dement. Comparison of actigraphic, polysomnographic, and subjective assessment of sleep parameters in sleep-disordered patients. Sleep Med. 2:389–396, 2001.

    Article  CAS  PubMed  Google Scholar 

  15. Ottonello, G., I. Ferrari, I. M. Pirroddi, M. C. Diana, G. Villa, L. Nahum, P. Tuo, A. Moscatelli, and G. Silvestri. Home mechanical ventilation in children: retrospective survey of a pediatric population. Pediatr. Int. 49:801–805, 2007.

    Article  PubMed  Google Scholar 

  16. Ross, P. A., C. J. Newth, and R. G. Khemani. Accuracy of pulse oximetry in children. Pediatrics 133:22–29, 2014.

    Article  PubMed  Google Scholar 

  17. Shea, S. A., L. P. Andres, D. C. Shannon, and R. B. Banzett. Ventilatory responses to exercise in humans lacking ventilatory chemosensitivity. J. Physiol. 468:623–640, 1993.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  18. Vanderlaan, M., C. R. Holbrook, M. Wang, A. Tuell, and D. Gozal. Epidemiologic survey of 196 patients with congenital central hypoventilation syndrome. Pediatr. Pulmonol. 37:217–229, 2004.

    Article  PubMed  Google Scholar 

  19. Weese-Mayer, D. E., E. M. Berry-Kravis, I. Ceccherini, T. G. Keens, D. A. Loghmanee, H. Trang, and ATS Congenital Central Hypoventilation Syndrome Subcommittee. An official ATS clinical policy statement: congenital central hypoventilation syndrome: genetic basis, diagnosis, and management. Am. J. Respir. Crit. Care Med. 181:626–644, 2010.

    Article  CAS  PubMed  Google Scholar 

  20. Weese-Mayer, D. E., E. M. Berry-Kravis, L. Zhou, B. S. Maher, J. M. Silvestri, M. E. Curran, and M. L. Marazita. Idiopathic congenital central hypoventilation syndrome: analysis of genes pertinent to early autonomic nervous system embryologic development and identification of mutations in PHOX2b. Am. J. Med. Genet. A 123A:267–278, 2003.

    Article  PubMed  Google Scholar 

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Acknowledgments

We thank Francesco Brenna, who was scholarship holder at the IRCCS E. Medea Institute, for the great contribution to this work both at the design stage and in the phase of experimentation. We thank Dr. Giulia Biffi for her support in the manuscript proofreading. Finally, we would like to thank also all those subjects that underwent the experimentation.

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Authors and Affiliations

  1. Bioengineering Lab, Scientific Institute, IRCCS E. Medea, Via don Luigi Monza 20, 23842, Bosisio Parini, LC, Italy

    Emilia Biffi, Caterina Piazza, Matteo Cavalleri, Peter Taddeo & Gianluigi Reni

  2. Electronics, Information and Bioengineering Department (DEIB), Politecnico di Milano, Milan, Italy

    Caterina Piazza

  3. Mechanical Engineering and Mechanics Department, Drexel University, Philadelphia, PA, USA

    Peter Taddeo

  4. AISICC, Italian Association for Congenital Central Hypoventilation Syndrome (CCHS), Florence, Italy

    Alessandro Carcano

  5. Pediatric Unit, Sacra Famiglia Hospital, Erba, CO, Italy

    Francesco Morandi

Authors
  1. Emilia Biffi
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  2. Caterina Piazza
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  3. Matteo Cavalleri
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  4. Peter Taddeo
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  5. Alessandro Carcano
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  6. Francesco Morandi
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  7. Gianluigi Reni
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Corresponding author

Correspondence to Emilia Biffi.

Additional information

Associate Editor Zahra Moussavi oversaw the review of this article.

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Biffi, E., Piazza, C., Cavalleri, M. et al. An Assistive Device for Congenital Central Hypoventilation Syndrome Outpatients During Sleep. Ann Biomed Eng 42, 2106–2116 (2014). https://doi.org/10.1007/s10439-014-1068-7

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  • DOI: https://doi.org/10.1007/s10439-014-1068-7

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