Quality of Life and the Degree of Disease Acceptance In Patients with Spinocerebellar Ataxia
DOI:
https://doi.org/10.15225/PNN.2018.7.1.2Keywords
spinocerebellar ataxia, quality of life, acceptance of disease, patient’s self–assessmentAbstract
Introduction. Spinocerebellar ataxias (SCA) belong to a group of rare genetic diseases. They are characterized by the multiplicity of symptoms, of which the most characteristic is limb ataxia and dysarthria. The disease progresses over time, leading to severe disability. So far an effective method of treating SCA has not been invented.
Aim. The main purpose was to analyze the quality of life and the level of disease acceptance in patients with SCA.
Material and Methods. The study included 70 patients with the average duration of SCA of approximately 13 years. The age of patients ranged from 26 to 81 years (mean 50±10 years). All participants were examined using the Acceptance Illness Scale (AIS) and the Health Survey (SF-36). Statistically significant differences in the degree of disease acceptance were observed in patients who differed in terms of education level and gender.
Results. In the physical sphere, women assessed their life quality more favorably than men (p=0.040), however, men declared better scores in bodily pain (p=0.017). It was observed that, in the sphere of mental health, men were more peaceful, and women were believed in deterioration of their health condition (p=0.039). Patients with SCA exhibit a very low level of both, disease acceptance and the quality of life. Women worse than men accept the disease, and they feel useless more often. The higher level of disease acceptance is shown by patients with higher education than those with vocational education. An interpersonal relationships get worse, especially among men, however, women often feel useless. Patients with SCA rate their life quality very low.
Conclusions. It is necessary to educate patients, their caregivers, medical staff in order to develop desirable health behaviors. Understanding this rare disease will help improve the quality of life and therefore will improve the level of disease acceptance. (JNNN 2018;7(1):12–21)
References
Jayadev S., Bird T.D. Hereditary ataxias: overview. Genet Med. 2013;15(9):673–683.
van Gaalen J., Giunti P., van de Warrenburg B.P. Movement disorders in spinocerebellar ataxias. Mov Disord. 2011;26(5):792–800.
Rüb U., Schöls L., Paulson H. et al. Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7. Prog Neurobiol. 2013;104:38–66.
Mallaret M., Synofzik M., Lee J. et al. The tumour suppressor gene WWOX is mutated in autosomal recessive cerebellar ataxia with epilepsy and mental retardation. Brain. 2014;137(Pt 2):411–419.
Cruz-Mariño T., Vázquez-Mojena Y., Velázquez-Pérez L. et al. SCA2 predictive testing in Cuba: challenging concepts and protocol evolution. J Community Genet. 2015;6(3):265–273.
Ogun S.A., Martins S., Adebayo P.B., Dawodu C.O., Sequeiros J., Finkel M.F. Machado-Joseph disease in a Nigerian family: mutational origin and review of the literature. Eur J Hum Genet. 2015;23(2):271–273.
Sułek-Piątkowska A., Zdzienicka E., Rakowicz M. et al. The occurrence of spinocerebellar ataxias caused by dynamic mutations in Polish patients. Neurol Neurochir Pol. 2010;44(3):238-245.
Rossi M., Perez-Lloret S., Doldan L. et al. Autosomal dominant cerebellar ataxias: a systematic review of clinical features. Eur J Neurol. 2014;21(4):607–615.
Ruano L., Melo C., Silva M.C., Coutinho P. The global epidemiology of hereditary ataxia and spastic paraplegia: a systematic review of prevalence studies. Neuroepidemiology. 2014;42(3):174–183.
Shakkottai V.G., Fogel B.L. Clinical neurogenetics: autosomal dominant spinocerebellar ataxia. Neurol Clin. 2013;31(4):987–1007.
Ashizawa T., Figueroa K.P., Perlman S.L. et al. Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study. Orphanet J Rare Dis. 2013;8:177.
Martins C.P., de Carvalho Rodrigues E., de Oliveira L.A.S. Physical therapy approach to spinocerebellar ataxia: a systematic review. Fisioter Pesqui. 2013;20(3):293–298.
Marszałek L. Cultural Influence on the role of woman in contemporary society. Semin Sci Search. 2008;25:267–279.
Janowski K., Kurpas D., Kusz J., Mroczek B., Jedynak T. Health-related behavior, profile of health locus of control and acceptance of illness in patients suffering from chronic somatic diseases. PLoS One. 2013;8(5):e63920.
Kaźmierczak M., Kubiak I., Gebuza G., Gierszewska M., Mieczkowska E. Factors that determine the acceptance of illness in women who are treated for lesions of the cervix. Gen Med Health Sci. 2015;21(2):181–186.
Vogel A.P., Folker J., Poole M.L. Treatment for speech disorder in Friedreich ataxia and other hereditary ataxia syndromes. Cochrane Database Syst Rev. 2014;(10):CD008953.
Fonteyn E.M., Keus S.H., Verstappen C.C., Schöls L., de Groot I.J., van de Warrenburg B.P. The effectiveness of allied health care in patients with ataxia: a systematic rev
Downloads
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution-NoDerivatives 4.0 International License.
Stats
Number of views and downloads: 187
Number of citations: 0