Journal of Nursing Theory and Practice

[Healthcare and social aspects of nursing in relation to the rare Pompe disease]

TÓTH Mónika1, VÁRDI Katalin Borbála1

AUGUST 28, 2014

Journal of Nursing Theory and Practice - 2014;27(04)

[Aim of the study: Pompe disease is a rare mitochondrial disease, which is treated with enzyme replacement therapy. The authors examined the lifestyles of patients diagnosed with Pompe disease, and their knowledge regarding the illness. They compared this with the results of the regular checkups performed during the care process, in order to assess the patients’ compliance. Sample and methods: The research was conducted among patients suffering from Pompe disease, treated at the Rehabilitation Department of the Törökbálint Institute of Pulmonary Medicine (N=14). The survey of the patients’ knowledge took place in the form of personal interviews. The findings related to the regular enzyme replacement therapy were recorded and collated in the course of interviews conducted by telephone with the nurses of the centres administering the treatment. The data was processed using Microsoft Excel software. Results: In 2012 in Hungary the number of patients diagnosed with Pompe disease was 14 (12 adults, 2 children). The time elapsed from emergence of the first symptoms to the precise diagnosis in the case of all known patients (except in the case of one screened child) was an average of 13.4 years. Ten of the patients were receiving enzyme replacement therapy. The survey revealed that the conditions of the patients receiving the enzyme replacement therapy did not deteriorate. In the case of the female child, the decrease in CK levels was accompanied by growth and movement consistent with her age, and an improvement in vocalisation. Conclusions: For patients diagnosed with the once fatal Pompe disease, enzyme replacement therapy now offers the opportunity of a full life; and complementary treatments not only boost the effectiveness of the enzyme replacement, but also improve the patients’ subjective quality of life.]

AFFILIATIONS

  1. Törökbálinti Tüdőgyógyintézet Rehabilitációs Osztály

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