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Neurological Sciences

, Volume 39, Issue 11, pp 1961–1964 | Cite as

Intrathecal administration of Nusinersen in type 1 SMA: successful psychological program in a single Italian center

  • Stefania La Foresta
  • Cristina Faraone
  • Maria Sframeli
  • Gian Luca Vita
  • Massimo Russo
  • Claudia Profazio
  • Immacolata Rulli
  • Eloisa Gitto
  • Antonio Versaci
  • Sonia Messina
  • Giuseppe VitaEmail author
Brief Communication

Abstract

Nusinersen is the first approved drug to treat spinal muscular atrophy (SMA). Its periodic intrathecal delivery may cause psychological burden in infants and in their parents. We report our experience during expanded access program (EAP) for type 1 SMA in a single Italian center. Because of the occurrence of stress emotional states, anxious reactions and fear before, during, and after lumbar puncture (LP), a specific psychological intervention was implemented based on regulation of emotions, anticipatory expectations, and post-event attributions. Activities included the use of fairy tales, distraction, music play through listening preferred cartoon themes in the youngest children, and contextual games and solution of fun riddle quizzes in the oldest ones. State anxiety greatly reduced in children and their parents. Treatment of psychological aspects should therefore become an integral part of health care in SMA infants and children during Nusinersen treatment.

Keywords

Spinal muscular atrophy Lumbar puncture Nusinersen Psychological support Contestual games Cognitive-behavioral therapy 

Notes

Acknowledgements

We thank type 1 SMA infants and children and their parents.

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

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

Informed consent

Informed consent was obtained from all individual participants included in the study.

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Copyright information

© Springer-Verlag Italia S.r.l., part of Springer Nature 2018

Authors and Affiliations

  • Stefania La Foresta
    • 1
  • Cristina Faraone
    • 1
  • Maria Sframeli
    • 1
  • Gian Luca Vita
    • 1
  • Massimo Russo
    • 1
  • Claudia Profazio
    • 1
  • Immacolata Rulli
    • 2
  • Eloisa Gitto
    • 2
  • Antonio Versaci
    • 3
  • Sonia Messina
    • 1
    • 4
  • Giuseppe Vita
    • 1
    • 4
    Email author
  1. 1.Centro Clinico Nemo SudMessinaItaly
  2. 2.Department of Human Pathology in Adult and Developmental Age, Neonatal and Paediatric Intensive Care UnitUniversity of MessinaMessinaItaly
  3. 3.Department of Human Pathology in Adult and Developmental Age, Intensive Care UnitUniversity of MessinaMessinaItaly
  4. 4.Department of Clinical and Experimental Medicine, Unit of Neurology and Neuromuscular DiseasesUniversity of MessinaMessinaItaly

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