AN EXPERIENCE OF HEALTHCARE MANAGEMENT FOR PATIENTS WITH PRIMARY IMMUNODEFICIENCY SIMILAR TO PATIENTS WITH ORPHAN DISEASES IN SVERDLOVSK REGION
https://doi.org/10.18093/0869-0189-2014-0-2-116-121
Abstract
The aim of this study was to analyze specialized healthcare for patients with orphan diseases and activity of a regional clinical immunology center in term of patients with primary immunodeficiency.
Methods. Common problems of patients with orphan diseases were identified using a model of a regional clinical immunology and primary immunodeficiency center. An experience of the center has been analyzed.
Results. A register of patients with orphan diseases living at the Sverdlovsk region includes 483 patients, of them, 255 patients with primary immunodeficiency. This register is not full. Medication therapy is needed for 108 patients. The average cost of orphan drugs is about 204,167.1 RUB.
Conclusion. Management of patients with primary immunodeficiency encounters several difficulties, such as: this disease is not included in the list of orphan disease; late diagnosis because of insufficient knowledge of this disease by a general practitioner; inadequate therapy leading to disability; high drug cost; high burden of this disease due to disease progression, disability, complications, days-off work.
Methods. Common problems of patients with orphan diseases were identified using a model of a regional clinical immunology and primary immunodeficiency center. An experience of the center has been analyzed.
Results. A register of patients with orphan diseases living at the Sverdlovsk region includes 483 patients, of them, 255 patients with primary immunodeficiency. This register is not full. Medication therapy is needed for 108 patients. The average cost of orphan drugs is about 204,167.1 RUB.
Conclusion. Management of patients with primary immunodeficiency encounters several difficulties, such as: this disease is not included in the list of orphan disease; late diagnosis because of insufficient knowledge of this disease by a general practitioner; inadequate therapy leading to disability; high drug cost; high burden of this disease due to disease progression, disability, complications, days-off work.
About the Authors
I. A. Tuzankina
ФГБУН "Институт иммунологии и физиологии" Уральского отделения РАН: 620049, Екатеринбург, ул. Первомайская, 106
ГОБУЗ Свердловской области "Областная детская клиническая больница № 1": 620149, Екатеринбург, ул. С.Дерябиной, 32
Russian Federation
Russian Federation
M. L. Karakina
ФГБУН "Институт иммунологии и физиологии" Уральского отделения РАН: 620049, Екатеринбург, ул. Первомайская, 106
ГОБУЗ Свердловской области "Областная детская клиническая больница № 1": 620149, Екатеринбург, ул. С.Дерябиной, 32
ГОБУЗ Свердловской области "Свердловская областная клиническая больница №1": 620102, Екатеринбург, ул. Волгоградская, 185
Russian Federation
Russian Federation
E. V. Vlasova
ГОБУЗ Свердловской области "Областная детская клиническая больница № 1": 620149, Екатеринбург, ул. С.Дерябиной, 32
Russian Federation
Russian Federation
T. V. Chernova
ГБОУ ДПО "Уралмедсоцэкономпроблем": 620175, Екатеринбург, ул. К.Либкнехта, 8б
Russian Federation
Russian Federation
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Review
For citations:
Tuzankina I.A., Karakina M.L., Vlasova E.V., Chernova T.V. AN EXPERIENCE OF HEALTHCARE MANAGEMENT FOR PATIENTS WITH PRIMARY IMMUNODEFICIENCY SIMILAR TO PATIENTS WITH ORPHAN DISEASES IN SVERDLOVSK REGION. PULMONOLOGIYA. 2014;(2):116-121. (In Russ.) https://doi.org/10.18093/0869-0189-2014-0-2-116-121
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