KNOWLEDGE AND SKILLS OF NURSES REGARDING ORPHAN DISEASES

Abstract

Introduction. The role of the nurse in providing comprehensive and individualized care to patients with rare diseases is one ofthe key roles. Nurses work closely with physicians and patients and play an educational role for patients and their families, facilitating coordination, monitoring and continuity of care. Therefore, nurses should have a certain range of knowledge and skills regarding the most common rare diseases.The aim of research was to determine the knowledge and skills of nurses regarding some specifi c rare diseases.Material and methods. An anonymous and voluntary survey of nurses was conducted at the regional children’s clinical hospitalfrom April to May 2023. A total of 63 nurses from the hospital participated in the survey. The questionnaire consisted of 27 questions related to the sociodemographic data of the respondents and rare diseases. The most common rare diseases encountered in the practice of nurses at the hospital were primary immunodefi ciencies (PID), spina bifi da (SB), cystic fi brosis, and juvenile idiopathic arthritis.The research results are presented in the form of descriptive statistics. Comparisons of frequency parameters were made using the Chi-square test. Diff erences between parameters were considered statistically signifi cant at p<0.05. Statistical analysis of the survey results was performed using the statistical package STATISTICA 10.0 and Microsoft Excel 2003 spreadsheet editor.The research and the publication of the materials were approved by the Bioethics Committee of the I. Horbachevsky Ternopil National Medical University (protocol № 73 dated 03.04.2023).The study was conducted within the framework of the research project of the Department of Children’s Diseases with PediatricSurgery of the I. Horbachevsky Ternopil National Medical University “Improvement of methods of diagnostics, treatment and rehabilitation of the most common children’s diseases”, registration number 0122U000039.Results. The majority of respondents were female (96.8%), between the ages of 31 and 50 (63.5%), and employed in the hospitalsetting (90.5%). Most were experienced workers with more than 20 years of experience (52.4%). Overall, the majority of respondents (71.0%) correctly identifi ed which diseases are rare. The percentage of correct answers regarding knowledge of rare diseases ranged from 47.9% for PID to 77.5% for SB, while correct answers regarding skills ranged from 41.3% for SB to 63.0% for PID. Overall, nurses demonstrated better knowledge than skills regarding rare diseases (p=0.0003). The most problematic issues were that HIV/AIDS was classifi ed as PID (31.7%), lack of knowledge about standards of neurogenic bladder control in children with SB (49.2%), unfamiliarity with the technique of subcutaneous immunoglobulin administration (87.3%), and bowel cleansing procedures in children with SB (95.2%), as well as infection control in patients with cystic fi brosis (66.1%).Conclusion. The study revealed a lack of awareness among nurses regarding certain rare diseases. The lowest level of knowledge was found for primary immunodefi ciencies. There is a need to improve nurses’ knowledge and skills regarding rare diseases, which can improve the quality of care for rare disease patients and their families. The development of educational interventions for nurses can help to increase their awareness of rare diseases.