A child diagnosed with rigid spine syndrome complicated by ventilatory disorders: a nursing case report

Rigid spine syndrome is a rare myopathy in children and has a poor prognosis because of its lack of treatment and eventual ventilatory failure. We report the case of a 10-year-old child with RSS and ventilatory disorders. We provided care to the child using bilevel positive airway pressure (BiPAP) n...

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Main Authors: Hua Lu, Zuojia Liu, Biru Li
Format: Article
Language:English
Published: SAGE Publishing 2019-02-01
Series:Journal of International Medical Research
Online Access:https://doi.org/10.1177/0300060518815358
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author Hua Lu
Zuojia Liu
Biru Li
author_facet Hua Lu
Zuojia Liu
Biru Li
author_sort Hua Lu
collection DOAJ
description Rigid spine syndrome is a rare myopathy in children and has a poor prognosis because of its lack of treatment and eventual ventilatory failure. We report the case of a 10-year-old child with RSS and ventilatory disorders. We provided care to the child using bilevel positive airway pressure (BiPAP) non-invasive mechanical ventilation and continuous monitoring of transcutaneous carbon dioxide pressure. A 10-year-old Han Chinese girl presented (6 April 6 2016) to the Shanghai Children’s Medical Center with ventilatory disorders, including hypoxia and hypercapnia. Transcutaneous oxygen saturation with mask oxygen inspiration was 90%. BiPAP non-invasive ventilator-assisted ventilation was continuously used. Through continuous non-invasive ventilation and carbon dioxide monitoring, the symptoms of dyspnea in this child were effectively controlled and improved. She was discharged on April 19 with instructions to continue using BiPAP at home and transcutaneous oxygen saturation was maintained at 94% to 98%. This case highlights that nursing of patients with rigid spine syndrome and ventilatory disorders should focus on evaluating the effect of non-invasive mechanical ventilation, prevention of complications, and continuous nursing after discharge. Additionally, continuous monitoring of transcutaneous carbon dioxide pressure is feasible for evaluating the effect of BiPAP.
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spelling doaj.art-159ece8d028e4e5a9a661115938432fb2022-12-21T19:12:15ZengSAGE PublishingJournal of International Medical Research0300-06051473-23002019-02-014710.1177/0300060518815358A child diagnosed with rigid spine syndrome complicated by ventilatory disorders: a nursing case reportHua LuZuojia LiuBiru LiRigid spine syndrome is a rare myopathy in children and has a poor prognosis because of its lack of treatment and eventual ventilatory failure. We report the case of a 10-year-old child with RSS and ventilatory disorders. We provided care to the child using bilevel positive airway pressure (BiPAP) non-invasive mechanical ventilation and continuous monitoring of transcutaneous carbon dioxide pressure. A 10-year-old Han Chinese girl presented (6 April 6 2016) to the Shanghai Children’s Medical Center with ventilatory disorders, including hypoxia and hypercapnia. Transcutaneous oxygen saturation with mask oxygen inspiration was 90%. BiPAP non-invasive ventilator-assisted ventilation was continuously used. Through continuous non-invasive ventilation and carbon dioxide monitoring, the symptoms of dyspnea in this child were effectively controlled and improved. She was discharged on April 19 with instructions to continue using BiPAP at home and transcutaneous oxygen saturation was maintained at 94% to 98%. This case highlights that nursing of patients with rigid spine syndrome and ventilatory disorders should focus on evaluating the effect of non-invasive mechanical ventilation, prevention of complications, and continuous nursing after discharge. Additionally, continuous monitoring of transcutaneous carbon dioxide pressure is feasible for evaluating the effect of BiPAP.https://doi.org/10.1177/0300060518815358
spellingShingle Hua Lu
Zuojia Liu
Biru Li
A child diagnosed with rigid spine syndrome complicated by ventilatory disorders: a nursing case report
Journal of International Medical Research
title A child diagnosed with rigid spine syndrome complicated by ventilatory disorders: a nursing case report
title_full A child diagnosed with rigid spine syndrome complicated by ventilatory disorders: a nursing case report
title_fullStr A child diagnosed with rigid spine syndrome complicated by ventilatory disorders: a nursing case report
title_full_unstemmed A child diagnosed with rigid spine syndrome complicated by ventilatory disorders: a nursing case report
title_short A child diagnosed with rigid spine syndrome complicated by ventilatory disorders: a nursing case report
title_sort child diagnosed with rigid spine syndrome complicated by ventilatory disorders a nursing case report
url https://doi.org/10.1177/0300060518815358
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