Subcutaneous immunoglobulin 16.5 % given by the rapid push method in patients with inborn errors of immunity: a prospective multicenter study of quality of life
- Authors: Avedova A.Y.1, Deripapa E.V.1, Rodina Y.A.1, Mukhina A.A.1, Latysheva E.A.2, Yukhacheva D.V.1, Burlakov V.I.1, Kan N.Y.1, Bogdanova D.V.1, Moiseeva A.A.1, Kuzmenko N.B.1, Nesterenko Z.A.1, Deordieva E.A.1, Ogneva A.L.1, Bludova V.O.1, Roppelt A.A.3, Fomina D.S.3, Zinovieva N.V.4, Sevostyanova Y.N.4, Kalmeteva L.R.5, Prolygina D.D.5, Barycheva L.Y.6, Selezneva O.S.7, Shakhova N.V.8, Laba O.M.7, Vlasova E.V.7, Gorenkova A.V.9, Timofeeva E.V.10, Trusova O.V.11, Guseva M.N.12, Yudina N.B.13, Grevtseva E.A.13, Ibisheva A.K.14, Bambaeva Z.V.7, Mashkovskaya D.V.5, Isakova S.N.15, Shakirova A.R.16, Selina E.Y.16, Shilova T.V.17, Zubova E.S.18, Khabaeva I.U.19, Kitova E.V.20, Mandzhieva A.G.21, Starikova S.Y.22, Pavlova T.B.23, Tyulyakova E.N.24, Grachev N.S.1, Shcherbina A.Y.1
-
Affiliations:
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
- National Research Center Institute of Immunology
- Clinical City Hospital No. 52
- Children’s City Clinical Hospital No. 9 named after G.N. Speransky
- Republican Children’s Clinical Hospital
- Stavropol State Medical University
- Regional Children’s Clinical Hospital
- Altai State Medical University
- Arkhangelsk Regional Children’s Clinical Hospital named after P.G. Vyzhletsova
- Nizhny Novgorod Regional Children’s Clinical Hospital
- First Saint Petersburg State Medical University named after I.P. Pavlov
- First Saint Petersburg State Medical University named after I.P. Pavlov, Saint Petersburg
- Voronezh Regional Children’s Clinical Hospital No. 1
- Republican Children’s Clinical Hospital named after E.P. Glinka
- Research Institute of Fundamental and Clinical Immunology
- Children’s Republican Clinical Hospital
- Chelyabinsk Regional Children’s Clinical Hospital
- Children’s Regional Hospital
- Regional Children’s Clinical Hospital named after N.N. Silishcheva
- Republican Children’s Clinical HospitalИжевск
- Regional Clinical Hospital No. 1
- City Children’s Clinical Hospital No. 2 named after V.P. Bisyarina
- Irkutsk State Regional Children’s Clinical Hospital
- Kaluga Regional Children’s Clinical Hospital
- Pages: 451-468
- Section: Original studies
- Submitted: 11.09.2024
- Accepted: 08.11.2024
- Published: 20.12.2024
- URL: https://rusalljournal.ru/raj/article/view/16969
- DOI: https://doi.org/10.36691/RJA16969
- ID: 16969
Cite item
Abstract
BACKGROUND: Replacement therapy with immunoglobulin preparations is the basic therapy for patients with impaired antibody formation in the context of inborn errors of immunity. The introduction of replacement therapy with subcutaneous immunoglobulin (SCIG) into clinical immunological practice has allowed patients with inborn errors of immunity, regardless of age, to carry out therapy independently at home. This circumstance has played a leading role in improving the quality of life of patients.
AIM: To assess the quality of life of patients and adherence to therapy during the transition from intravenous immunoglobulin replacement therapy to SCIG therapy.
MATERIALS AND METHODS: A total of 233 patients of different ages with inborn errors of immunity were enrolled in the study. All patients were switched from intravenous immunoglobulin to SCIG 16.5 % therapy. SCIG was administered every 7–10 days for at least 12 months via the rapid push method. Assessment of quality of life and adherence to SCIG therapy was carried out 6 and 12 months after the start of therapy using a questionnaire.
RESULTS: Based on the results of the assessment of the quality of life of patients using a questionnaire, patients of all age groups under 18 years and their parents demonstrated improvement in the social sphere, academic performance at school, and physical activity against the background of switching to SCIG. According to the survey, 84 % of patients did not experience technical difficulties and 83 % wanted to continue the SCIG infusion.
CONCLUSION: Performing manual infusion of SCIG at home has a positive effect on the quality of life of patients and helps improve adherence to therapy.
Full Text
About the authors
Asmik Ya. Avedova
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Author for correspondence.
Email: asya.avedova95@mail.ru
ORCID iD: 0000-0002-9399-6534
Россия, Moscow
Elena V. Deripapa
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: deripapa@mail.ru
ORCID iD: 0000-0002-9083-4783
Cand. Sci. (Medicine)
Россия, MoscowYulia A. Rodina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: rodina.julija@rambler.ru
ORCID iD: 0000-0001-9857-4456
MD, Cand. Sci. (Medicine)
Россия, MoscowAnna A. Mukhina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: ffmanya@yandex.ru
ORCID iD: 0000-0002-3305-1694
SPIN-code: 3925-3328
MD, Cand. Sci. (Medicine)
Россия, MoscowElena A. Latysheva
National Research Center Institute of Immunology
Email: ealat@mail.ru
ORCID iD: 0000-0002-1606-205X
SPIN-code: 2063-7973
MD, Dr. Sci. (Medicine)
Россия, MoscowDaria V. Yukhacheva
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: yukhachevadv@gmail.com
ORCID iD: 0000-0001-9078-8206
SPIN-code: 3052-1661
MD, Cand. Sci. (Medicine)
Россия, MoscowVasily I. Burlakov
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: sarovas@yandex.ru
ORCID iD: 0000-0003-1267-9957
Россия, Moscow
Nellie Yu. Kan
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: nkan123@yandex.ru
ORCID iD: 0000-0003-3564-6496
SPIN-code: 2163-8349
Cand. Sci. (Medicine)
Россия, MoscowDaria V. Bogdanova
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: miss.daryabogdanova@yandex.ru
ORCID iD: 0000-0003-2897-5208
SPIN-code: 4123-9308
Россия, Moscow
Anna A. Moiseeva
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: lira_belaqua@mail.ru
ORCID iD: 0000-0002-6134-3811
SPIN-code: 2482-8500
Россия, Moscow
Natalia B. Kuzmenko
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: plunge@list.ru
ORCID iD: 0000-0002-1669-8621
SPIN-code: 6281-2729
MD, Cand. Sci. (Medicine)
Россия, MoscowZoya A. Nesterenko
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: nesterenkozoya7@gmail.com
ORCID iD: 0000-0003-4427-054X
SPIN-code: 5957-1752
Россия, Moscow
Ekaterina A. Deordieva
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: deor2005@yandex.ru
ORCID iD: 0000-0002-8208-2075
MD, Cand. Sci. (Medicine)
Россия, MoscowAnna L. Ogneva
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: ania.tulip@mail.ru
ORCID iD: 0000-0002-8697-4206
MD, Cand. Sci. (Medicine)
Россия, MoscowViktoria O. Bludova
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: voevodina_1996@list.ru
ORCID iD: 0000-0003-0960-3089
Россия, Moscow
Anna A. Roppelt
Clinical City Hospital No. 52
Email: roppelt_anna@mail.ru
ORCID iD: 0000-0001-5132-1267
SPIN-code: 7249-4423
Moscow Center of Allergy and Immunology, MD, Cand. Sci. (Medicine)
Россия, MoscowDaria S. Fomina
Clinical City Hospital No. 52
Email: daria_fomina@mail.ru
ORCID iD: 0000-0002-5083-6637
SPIN-code: 3023-4538
Moscow Center of Allergy and Immunology, MD, Cand. Sci. (Medicine), Assistant Professor
Россия, MoscowNatalia V. Zinovieva
Children’s City Clinical Hospital No. 9 named after G.N. Speransky
Email: nvzinov@gmail.com
ORCID iD: 0000-0002-6926-2055
MD, Cand. Sci. (Medicine)
Россия, MoscowYulia N. Sevostyanova
Children’s City Clinical Hospital No. 9 named after G.N. Speransky
Email: mayuliya@yandex.ru
ORCID iD: 0009-0004-4328-7803
Россия, Moscow
Linara R. Kalmeteva
Republican Children’s Clinical Hospital
Email: l_kalmetieva@mail.ru
ORCID iD: 0000-0003-1866-0640
Россия, Ufa
Dilara D. Prolygina
Republican Children’s Clinical Hospital
Email: drkb.gem.immun@mail.ru
ORCID iD: 0000-0002-5797-4370
Россия, Ufa
Ludmila Yu. Barycheva
Stavropol State Medical University
Email: for_ludmila@inbox.ru
ORCID iD: 0000-0002-4069-0566
SPIN-code: 8946-3393
MD, Dr. Sci. (Medicine)
Россия, StavropolOlga S. Selezneva
Regional Children’s Clinical Hospital
Email: selezneva-doc@mail.ru
ORCID iD: 0000-0003-1907-4168
Россия, Rostov-on-Don
Natalia V. Shakhova
Altai State Medical University
Email: Natalia.shakhova@mail.ru
ORCID iD: 0000-0002-7143-8259
SPIN-code: 3290-8520
MD, Dr. Sci. (Medicine)
Россия, BarnaulOlga M. Laba
Regional Children’s Clinical Hospital
Email: labaom@mail.ru
ORCID iD: 0000-0001-5784-1263
Россия, Yaroslavl
Elena V. Vlasova
Regional Children’s Clinical Hospital
Email: evvlasova@mail.ru
ORCID iD: 0000-0002-6678-5360
Россия, Yekaterinburg
Alla V. Gorenkova
Arkhangelsk Regional Children’s Clinical Hospital named after P.G. Vyzhletsova
Email: domgav63@rambler.ru
ORCID iD: 0009-0008-2514-1594
Россия, Arkhangelsk
Elena V. Timofeeva
Nizhny Novgorod Regional Children’s Clinical Hospital
Email: elenatim-nn@mail.ru
ORCID iD: 0000-0003-3908-1947
Россия, Nizhny Novgorod
Olga V. Trusova
First Saint Petersburg State Medical University named after I.P. Pavlov
Email: o-tru@mail.ru
ORCID iD: 0000-0002-0854-1536
SPIN-code: 3938-4377
MD, Dr. Sci. (Medicine)
Россия, Saint PetersburgMarina N. Guseva
First Saint Petersburg State Medical University named after I.P. Pavlov, Saint Petersburg
Email: gusevamarina@mail.ru
ORCID iD: 0000-0002-3997-3390
SPIN-code: 3535-9896
Россия, Saint Petersburg
Natalia B. Yudina
Voronezh Regional Children’s Clinical Hospital No. 1
Email: n.yudina@list.ru
ORCID iD: 0009-0001-7498-0836
SPIN-code: 3908-7166
Россия, Voronezh
Evgenia A. Grevtseva
Voronezh Regional Children’s Clinical Hospital No. 1
Email: grievtsieva85@mail.ru
ORCID iD: 0009-0004-9437-4851
SPIN-code: 3717-6562
Россия, Voronezh
Asya Kh. Ibisheva
Republican Children’s Clinical Hospital named after E.P. Glinka
Email: ibisheva18@mail.ru
ORCID iD: 0000-0003-4732-4515
Россия, Grozny
Zema V. Bambaeva
Regional Children’s Clinical Hospital
Email: z.bambaeva@yandex.ru
ORCID iD: 0000-0002-7541-2793
Россия, Ulan-Ude
Dina V. Mashkovskaya
Republican Children’s Clinical Hospital
Email: mashkovskaya@child-hosp.ru
ORCID iD: 0009-0000-4995-0427
Россия, Simferopol
Svetlana N. Isakova
Research Institute of Fundamental and Clinical Immunology
Email: hell_paradise@mail.ru
ORCID iD: 0009-0007-8368-5250
Россия, Novosibirsk
Almazia R. Shakirova
Children’s Republican Clinical Hospital
Email: almaziaysh@mail.ru
ORCID iD: 0000-0001-9975-3632
SPIN-code: 2666-7175
Россия, Kazan
Ekaterina Yu. Selina
Children’s Republican Clinical Hospital
Email: selina-katya1998@yandex.ru
ORCID iD: 0009-0003-6545-1435
Россия, Kazan
Tatiana V. Shilova
Chelyabinsk Regional Children’s Clinical Hospital
Email: tanya920477@mail.ru
ORCID iD: 0000-0001-9826-9654
SPIN-code: 2028-9392
MD, Cand. Sci. (Medicine)
Россия, ChelyabinskElena S. Zubova
Children’s Regional Hospital
Email: zubovaes@mail.ru
ORCID iD: 0000-0002-9755-8929
SPIN-code: 4140-9615
Россия, Perm
Imani U. Khabaeva
Regional Children’s Clinical Hospital named after N.N. Silishcheva
Email: el25kv@mail.ru
ORCID iD: 0009-0008-7161-4540
Россия, Astrakhan
Ekaterina V. Kitova
Republican Children’s Clinical HospitalИжевск
Email: Ek.Burova@yundex.ru
ORCID iD: 0009-0001-6004-2558
Россия, Izhevsk
Anastasia G. Mandzhieva
Regional Clinical Hospital No. 1
Email: mandzhieva81@bk.ru
ORCID iD: 0009-0002-7068-2601
Россия, Tyumen
Svetlana Yu. Starikova
City Children’s Clinical Hospital No. 2 named after V.P. Bisyarina
Email: Starikowa17@mail.ru
ORCID iD: 0000-0002-4321-7301
Россия, Omsk
Tatiana B. Pavlova
Irkutsk State Regional Children’s Clinical Hospital
Email: tabopav@rambler.ru
ORCID iD: 0000-0003-3403-4447
SPIN-code: 4893-7250
MD, Cand. Sci. (Medicine)
Россия, IrkutskElvira N. Tyulyakova
Kaluga Regional Children’s Clinical Hospital
Email: tabopav@rambler.ru
ORCID iD: 0009-0005-1642-4398
Россия, Kaluga
Nikolai S. Grachev
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: nick-grachev@yandex.ru
ORCID iD: 0000-0002-4451-3233
SPIN-code: 2836-2349
MD, Dr. Sci. (Medicine), Professor
Россия, MoscowAnna Yu. Shcherbina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology
Email: shcher26@hotmail.com
ORCID iD: 0000-0002-3113-4939
SPIN-code: 6759-0031
MD, Dr. Sci. (Medicine), Professor
Россия, MoscowReferences
- Cunningham-Rundles C. Key aspects for successful immunoglobulin therapy of primary immunodeficiencies. Clin Exp Immunol. 2011;164(2):16–19. doi: 10.1111/j.1365-2249.2011.04390.x
- Ochs HD, Hitzig WH. History of primary immunodeficiency diseases. Curr Opin Allergy Clin Immunol. 2012;12(6):577–587. doi: 10.1097/ACI.0b013e32835923a6
- Hinman J, Tullis JL, Saravis CA, Pennell RB. Intravenous use of plasmin treated immunoglobulin G. I. Preliminary report on tolerance by immunologically deficient patients. Vox Sang. 1967;13(1):85–90.
- Latysheva E, Rodina Y, Sizyakina L, et al. Efficacy and safety of octanorm (cutaquig®) in adults with primary immunodeficiencies with predominant antibody deficiency: aprospective, open-label study. Immunotherapy. 2020;12(5):299–309. doi: 10.2217/imt-2020-0012
- Jolles S, Bernatowska E, de Gracia J, et al. Efficacy and safety of Hizentra® in patients with primary immunodeficiency after a dose-equivalent switch from intravenous or subcutaneous replacement therapy. Clin Immunol. 2011;141(1):90–102. doi: 10.1016/j.clim.2011.06.002
- Borte M, Kriván G, Derfalvi B, et al. Efficacy, safety, tolerability and pharmacokinetics of a novel human immune globulin subcutaneous, 20 %: a phase 2/3 study in Europe in patients with primary immunodeficiencies. Clin Exp Immunol. 2017;187(1):146–159. doi: 10.1111/cei.12866
- Kobayashi RH, Gupta S, Melamed I, et al. Clinical efficacy, safety and tolerability of a new subcutaneous immunoglobulin 16.5 % (Octanorm [Cutaquig®]) in the treatment of patients with primary immunodeficiencies. Front Immunol. 2019;10:10–40. doi: 10.3389/fimmu.2019.00040
- Ochs HD, Gupta S, Kiessling P, et al. Safety and efficacy of self-administered subcutaneous immunoglobulin in patients with primary immunodeficiency diseases. J Clin Immunol. 2006;26(3):265–273. doi: 10.1007/s10875-006-9021-7
- Smirnova IN, Rodina YuA, Deripapa EV, et al. Pharmacoeconomic analysis of intravenous immunoglobulin replacement therapy in patients with primary humoral immunodeficiencies. Pediatric Hematology/Oncology and Immunopathology. 2016;15(1):66–71. (In Russ). doi: 10.24287/1726-1708-2016-15-1-66-71
- Mukhina AA, Abramova IN, Kuzmenko NV, et al. Immunoglobulin replacement therapy in patients with primary immunodeficiency diseases in Russia. Pediatric Hematology/Oncology and Immunopathology. 2020;19(4):18–29. (In Russ). doi: 10.24287/1726-1708-2020-19-4suppl-18-29
- Parker M. The ethics of evidence-based patient choice. Health Expect. 2001;4(2):87–91. doi: 10.1046/j.1369-6513.2001.00137.x
- Elwyn G, Edwards A, Thompson R. Shared decision making in health care: achieving evidence-based patient choice. 3rd ed. Oxford: Oxford University Press, 2016. 336 p.
- Asekretova TV, Anderzhanova LH, Leontyeva ME, et al. Clinical and laboratory characteristics of a group of patients with ataxia-telangiectasia syndrome. Pediatric Hematology/Oncology and Immunopathology. 2022;21(3):47–55. (In Russ). doi: 10.24287/1726-1708-2022-21-3-47-55
- Anterasian C, Duong R, Gruenemeier P, et al. Quality of life differences for primary immunodeficiency patients on home SCIG versus IVIG. J Clin Immunol. 2019;39(8):814–822. doi: 10.1007/s10875-019-00705-5
- Al-Saud B, AlRumayyan N, Alfattani A, et al. Quality of life evaluation in Saudi Arabian pediatric patients with primary immunodeficiency diseases receiving 20 % subcutaneous IgG infusions at home. J Clin Immunol. 2023;43(6):1360–1366. doi: 10.1007/s10875-023-01507-6
- Avedova АYa, Deripapa EV, Rodina YuA, et al. Evaluation of the effectiveness and safety of subcutaneous immunoglobulin 16.5 % replacement therapy using manual infusion in patients with inborn errors of immunity: results of a multicenter prospective study. Pediatric Hematology/Oncology and Immunopathology. 2024;23(4). (In Russ). [In print].
- Warnatz K, Jolles S, Agostini C, et al. Subcutaneous Gammanorm® by pump or rapid push infusion: Impact of the device on quality of life in adult patients with primary immunodeficiencies. Clin Immunol. 2022;(236):108938. doi: 10.1016/j.clim.2022.108938
- Bienvenu B, Cozon G, Mataix Y, et al. Rapid push vs pump-infused subcutaneous immunoglobulin treatment: a randomized crossover study of quality of life in primary immunodeficiency patients. J Clin Immunol. 2018;38(4):503–512. doi: 10.1007/s10875-018-0507-x