eISSN: 2299-8284
ISSN: 1233-9989
Nursing Problems / Problemy Pielęgniarstwa
Current issue Archive Manuscripts accepted About the journal Editorial board Journal's reviewers Abstracting and indexing Subscription Contact Instructions for authors Ethical standards and procedures
2/2020
vol. 28
 
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abstract:
Case report

Model of nursing management of a child with Crouzon syndrome according to the International Classification for Nursing Practice®

Agata Cieniawa
1

1.
Department of Nephrology, The University Hospital, Krakow, Poland
Nursing Problems 2020; 28 (2): 106-110
Online publish date: 2020/09/09
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Introduction
Crouzon syndrome is a genetic disease causing a set of congenital cranial anomalies. The disorder results from a mutation in the FGFR2 gene. Abnormalities arising from the defect in the gene lead to severe cranial deformities due to the premature fusion of one or more cra-niofacial sutures.

Aim of the study
To present nursing and care of a patient with Crouzon syndrome using the International Classification for Nursing Practice (ICNP®) termi-nology.

Material and methods
The study was based on the research method of a case study using a range of case study tools and techniques, such as interview, obser-vation, and analysis of medical records of a two-year-old boy suffering from Crouzon syndrome. The identification of 12 nursing diag-noses along with nursing interventions was performed on the basis of the ICNP®.

Results and conclusions
Crouzon syndrome is a rare genetic disease that causes severe skull deformities and numerous disorders, as a result of which the treat-ment process is long and difficult for children and their carers. Patients require individual care tailored to their needs. The scope of nurs-ing interventions included monitoring according to ICNP®: the child’s status, signs of worsening pain, identifying deficits in child’s devel-opment, and providing informational and emotional support to child carers. In-depth observation allowed for early diagnosis and elimina-tion of constipation, gas exchange disorders through the tracheotomy tube, and skin integrity disorders in the area of gastrostomy and tracheostomy. Thanks to the ICNP® reference terminology, it was possible to formulate 12 diagnoses and appropriate nursing interven-tions to care for children with Crouzon syndrome.

keywords:

Crouzon syndrome, child, nursing management

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