Promoción de la Salud para Pacientes con Hemofilia: Una Mirada Biopsicosocial
Health Promotion for Patients with Hemophilia: A Biopsychosocial Perspective
DOI:
https://doi.org/10.56712/latam.v6i3.4022Palabras clave:
hemofilia, diagnóstico, tratamiento, promoción de la salud, estrategiasResumen
La hemofilia es un trastorno genético de la coagulación causado por alteraciones cualitativas y cuantitativas en los factores de coagulación VIII (hemofilia A) o IX (hemofilia B). En los últimos años, los avances biotecnológicos han mejorado considerablemente el diagnóstico y tratamiento de esta enfermedad. Sin embargo, los pacientes aún enfrentan desafíos en su acceso a la atención sanitaria, debido a la disponibilidad existente por región y a sus altos costos; además, las limitaciones del sistema de salud al que pertenecen que a menudo carecen de recursos materiales y personal especializado. Asimismo, este trastorno raro conlleva una significativa carga física, psicoemocional y social, lo que agrava aún más su impacto en la calidad de vida. En este sentido, en esta investigación documental analizamos el contexto general de personas con hemofilia A y B, desde la esfera biomédica, psicoemocional y socioeconómica; con el objetivo de identificar los problemas potenciales de estos grupos vulnerables, y proponer estrategias basadas en promoción de la salud que ayuden a la mejora de su bienestar.
Descargas
Citas
Ahmed, S., Waseem, S., Shaikh, T., y Hasan, M. (2023). Advancing haemophilia care in Pakistan: Progress and future directions. Haemophilia, 29. https://doi.org/10.1111/hae.14789
Alvarado-Ibarra, M. (2021). Consensus on hemophilia in Mexico. Gac Med Mex, 157(Supl 1), S1-S35.
Arbab Siyar, N., Amiri Majd, M., y Baghdassarians, A. (2021). The effectiveness of cognitive‐behavioural therapy on emotional regulation and quality of life in children with Haemophilia. Haemophilia, 27(4), 599-605.
Batran, R., Kamel, M., Bahr, A., Khalil, A., y Elsokary, M. (2024). Hemophilia A: Economic burden, therapeutic advances, and future forecasts in the Middle East and North Africa region. Thrombosis research, 243, 109175. https://doi.org/10.1016/j.thromres.2024.109175
Batty, P., y Lillicrap, D. (2019). Advances and challenges for hemophilia gene therapy. Human molecular genetics, 28(R1), R95-R101.
Bertamino, M., Riccardi, F., Banov, L., Svahn, J., y Molinari, A. C. (2017). Hemophilia care in the pediatric age. Journal of clinical medicine, 6(5), 54.
Callahan, D. (1973). The WHO definition of 'health'. Studies - Hastings Center, 1 3, 77-88. https://doi.org/10.2307/3527467
Cassis, F. (2007). Atención psicosocial para personas con hemofilia. Recuperado de http://www1. wfh. org/publication/files/pdf-1199. pdf.
Cassis, F., Emiliani, F., Pasi, J., Palareti, L., y Iorio, A. (2012). Psychological interventions for people with hemophilia. Cochrane Database of Systematic Reviews, 3. https://doi.org/10.1002/14651858.CD010215
Castillo-González, D. (2012). Hemofilia: aspectos históricos y genéticos. Revista Cubana de Hematología, Inmunología y Hemoterapia, 28(1).
Cavazza, M., Kodra, Y., Armeni, P., De Santis, M., López‐Bastida, J., Linertová, R.,…Network, B.-R. R. (2016). Social/economic costs and quality of life in patients with haemophilia in Europe. The European Journal of Health Economics, 17, 53-65. https://doi.org/10.1007/s10198-016-0785-2
Chen, C., Baker, J., y Nichol, M. (2017). Economic Burden of Illness among Persons with Hemophilia B from HUGS Vb: Examining the Association of Severity and Treatment Regimens with Costs and Annual Bleed Rates. Value in health : the journal of the International Society for Pharmacoeconomics and Outcomes Research, 20 8, 1074-1082. https://doi.org/10.1016/j.jval.2017.04.017
Chen, S.-L. (2016). Economic costs of hemophilia and the impact of prophylactic treatment on patient management. The American journal of managed care, 22, 126-133. PMID: 27266809
Chen, Y., Cheng, S., Thornhill, T., Solari, P., y Sullivan, S. (2023). Health care costs and resource use of managing hemophilia A: A targeted literature review. Journal of Managed Care & Specialty Pharmacy, 29. https://doi.org/10.18553/jmcp.2023.29.6.647
Coban, A., Bornberg-Bauer, E., y Kemena, C. (2022). Domain evolution of vertebrate blood coagulation cascade proteins. Journal of Molecular Evolution, 90(6), 418-428.
D'Angiolella, L., Cortesi, P., Rocino, A., Coppola, A., Hassan, H., Giampaolo, A.,…Mantovani, L. (2018). The socioeconomic burden of patients affected by hemophilia with inhibitors. European Journal of Haematology, 101, 435-456. https://doi.org/10.1111/ejh.13108
García-Dasí, M., Torres-Ortuño, A., Cid‐Sabatel, R., y Barbero, J. (2016). Practical aspects of psychological support to the patient with haemophilia from diagnosis in infancy through childhood and adolescence. Haemophilia, 22. https://doi.org/10.1111/hae.13018
Godlee, F. (2011). What is health? BMJ : British Medical Journal, 343. https://doi.org/10.1136/BMJ.D4817
Hernández-Girón, C., y Cabrales-Arreola, J. L. (2024). Trastornos de la coagulación: factor V Leiden, panorama biológico, clínico y epidemiológico. Revista de la Facultad de Medicina (México), 67(2), 7-18.
Hoffman, M. (2003). A cell-based model of coagulation and the role of factor VIIa. Blood reviews, 17 Suppl 1. https://doi.org/10.1016/S0268-960X(03)90000-2
Hoffman, M., y Monroe, D. (2001). A Cell-based Model of Hemostasis. Thrombosis and Haemostasis, 85, 958-965. https://doi.org/10.1055/s-0037-1615947
Huber, M., Knottnerus, J., Green, L., Horst, H., Jadad, A., Kromhout, D.,…Smid, H. (2011). How should we define health? BMJ : British Medical Journal, 343. https://doi.org/10.1136/bmj.d4163
Iorio, A., Stonebraker, J., Chambost, H., Makris, M., Coffin, D., Herr, C., y Germini, F. (2019). Establishing the Prevalence and Prevalence at Birth of Hemophilia in Males. Annals of Internal Medicine, 171, 540-546. https://doi.org/10.7326/M19-1208
Kadir, Economides, Sabin, Pollard, y Lee. (1999). Assessment of menstrual blood loss and gynaecological problems in patients with inherited bleeding disorders. Haemophilia, 5. https://doi.org/10.1046/j.1365-2516.1999.00285.x
Kang, P., Fang, Y.-H., Cheng, Y., Zhang, X.-Q., Wang, J., Li, H., y Zhang, X. (2019). Analysis of haemophilia case information of Shandong Province in China: Data from 2010 to 2017. Haemophilia, 25. https://doi.org/10.1111/hae.13724
Lassandro, G., Accettura, D., y Giordano, P. (2021). Promoting sports practice in persons with hemophilia: a survey of clinicians’ perspective. International Journal of Environmental Research and Public Health, 18(22), 11841.
Lazăr, A. C., Ilea, A., Moldovan, B., Ionel, A., Pop, A. S., Păcurar, M., y Campian, R. S. (2019). State of the art regarding anticoagulant and thrombolytic therapy in dental procedures. Rom J Morphol Embryol, 60(2), 403-410.
Leebeek, F., Duvekot, J., y Kruip, M. (2020). How I manage pregnancy in carriers of hemophilia and patients with von Willebrand disease. Blood. https://doi.org/10.1182/blood.2019000964
Leonardi, F. (2018). The Definition of Health: Towards New Perspectives. International Journal of Health Services, 48, 735-748. https://doi.org/10.1177/0020731418782653
López-Arroyo, J. L., Pérez-Zúñiga, J. M., Merino-Pasaye, L. E., Saavedra-González, A., Alcivar-Cedeño, L. M., Álvarez-Vera, J. L.,…Bates-Martín, R. A. (2021). Consenso de hemofilia en México. Gaceta médica de México, 157, S1-S37.
López-Fernández, L. A., y Solar Hormazábal, O. (2017). Repensar la Carta de Ottawa 30 años después. In (Vol. 31, pp. 443-445): SciELO Public Health.
López-Otín, C., y Kroemer, G. (2021). Hallmarks of health. Cell, 184(1), 33-63.
Luna, S. C., Roldán, M. D. T., y Martínez, L. (2024). Hemofilia en urgencias. Revista Repertorio de Medicina y Cirugía. https://doi.org/10.31260/repertmedcir.01217372.1433
Malhan, S., Öksüz, E., Antmen, B., Ar, M., Balkan, C., y Kavaklı, K. (2021). Cost of hemophilia A in Turkey: an economic disease burden analysis. Journal of Medical Economics, 24, 1052-1059. https://doi.org/10.1080/13696998.2021.1965388
Mannucci, P. M. (2020). Hemophilia therapy: the future has begun. Haematologica, 105(3), 545.
Martínez-Sánchez, L. M., Álvarez-Hernández, L. F., Ruiz-Mejía, C., Jaramillo-Jaramillo, L. I., Builes-Restrepo, L. N., y Villegas-Álzate, J. D. (2018). Hemofilia: abordaje diagnóstico y terapéutico. Revisión bibliográfica. Revista Facultad Nacional de Salud Pública, 36(2), 85-93.
Martinuzzo, M. (2017). Blood Coagulation System Physiology. Sociedad Argentina de Hematología, 21, 31-42.
Monreal, L. A., Arillo-Santillán, E., Betanzos-Reyes, A. F., Jiménez-Aguilar, A., Márquez-Serrano, M., Rangel-Flores, H.,…Villanueva-Borbolla, M. A. (2018). Promoción de la salud. Juan Rivera Dommarco Tonatiuh Barrientos Gutiérrez Carlos Oropeza, 194.
Monroe, D., y Hoffman, M. (2006). What does it take to make the perfect clot? Arteriosclerosis, thrombosis, and vascular biology, 26 1, 41-48. https://doi.org/10.1161/01.ATV.0000193624.28251.83
Mustafa, A. (2019). Evaluation of Nutritional Status among Adult Hemophilia patients enrolled in European Gaza hospital in Gaza strip, Palestine. Journal of Clinical and Medical Research. https://doi.org/10.37191/mapsci-2582-4333-2(2)-028
Navarro-Rodríguez, D. C., Guevara-Valtier, M. C., y Paz-Morales, M. d. l. Á. (2023). Análisis y Evaluación del Modelo de Promoción de la Salud. Temperamentvm, 19.
Nieva, N. R. (2023). Obesity and physical exercise in hemophilia. Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis, 34 S1. https://doi.org/10.1097/MBC.0000000000001216
O’Hara, J., Hughes, D., Camp, C., Burke, T., Carroll, L., y Diego, D. (2017). The cost of severe haemophilia in Europe: the CHESS study. Orphanet Journal of Rare Diseases, 12. https://doi.org/10.1186/s13023-017-0660-y
OMS. (1986). Carta de Ottawa para la Promoción de la Salud. Promoción de la Salud, Ginebra: OMS, 559-568.
Pacheco, L., Saade, G., y James, A. (2023). Von Willebrand Disease, Hemophilia, and Other Inherited Bleeding Disorders in Pregnancy. Obstetrics & Gynecology, 141, 493-504. https://doi.org/10.1097/AOG.0000000000005083
Palareti, L., Melotti, G., Cassis, F., Nevitt, S. J., y Iorio, A. (2020). Psychological interventions for people with hemophilia. The Cochrane database of systematic reviews, 3. https://doi.org/https://doi.org/10.1002/14651858.CD010215.pub2
Paredes, A., Costa, P., Fernandes, S., Lopes, M., Carvalho, M., Almeida, A., y Pinto, P. (2019). Effectiveness of hypnosis for pain management and promotion of health-related quality-of-life among people with haemophilia: a randomised controlled pilot trial. Scientific Reports, 9. https://doi.org/10.1038/s41598-019-49827-1
Perolla, A., y Kalaja, B. (2024). Improving Hemophilia Care in Low-and Middle-Income Countries: Addressing Challenges and Enhancing Quality of Life. Cureus, 16(6).
Pinto, P., Paredes, A., Costa, P., Carvalho, M., Lopes, M., Fernandes, S.,…Almeida, A. (2017). Effectiveness of two psychological interventions for pain management, emotional regulation and promotion of quality of life among adult Portuguese men with haemophilia (PSY-HaEMOPEQ): study protocol for a single-centre prospective randomised controlled trial. BMJ Open, 7. https://doi.org/10.1136/bmjopen-2017-016973
Quintana Paris, L. (2023). Foundations of hemophilia and epidemiology. Blood Coagulation & Fibrinolysis: an international journal in haemostasis and thrombosis, 34, S35-S36. https://doi.org/10.1097/MBC.0000000000001222
Ragni, M. (2021). Hemophilia as a blueprint for gene therapy. Science, 374, 40-41. https://doi.org/10.1126/science.abg0856
Ramos-Petersen, L., Rodríguez-Sánchez, J., Cortés-Martín, J., Reinoso-Cobo, A., Sánchez-García, J., Rodríguez-Blanque, R., y Coca, J. (2023). A Qualitative Study Exploring the Experiences and Perceptions of Patients with Hemophilia Regarding Their Health-Related Well-Being, in Salamanca. Journal of clinical medicine, 12. https://doi.org/10.3390/jcm12165417
Rodríguez-Merchán, E. C., De Pablo-Moreno, J. A., y Liras, A. (2021). Gene therapy in hemophilia: recent advances. International Journal of Molecular Sciences, 22(14), 7647.
Rosner, F. (1994). Hemophilia A. The New England journal of medicine, 330 22, 1617. https://consensus.app/papers/hemophilia-a-rosner/8a74dc3f28a85e6d805f4f1c8c6da091/
Salinas-Escudero, G., Castillo-Martínez, I. D., Martínez-Valverde, S., Garduño-Espinosa, J., y Carrillo-Vega, M. F. (2020). Análisis de minimización de costos entre dos tratamientos a demanda de hemofilia A en niños en México.¿ Son los crioprecipitados una opción más económica? Revista de Hematología, 21(2), 92-102.
Sanigorska, A., Chaplin, S., Holland, M., Khair, K., y Pollard, D. (2022). The lived experience of women with a bleeding disorder: a systematic review. Research and Practice in Thrombosis and Haemostasis, 6(1), e12652.
Schmitt, F., Maier, L. A., Albisetti, M., Trinchero, A., Graf, L., Hengartner, H.,…Kartal-Kaess, M. (2023). “Hermes” - Hemophilia Related Mental Illness: A Cross-Sectional, Multicenter Study in Switzerland. Blood. https://doi.org/10.1182/blood-2023-189771
Schramm, W. (2014). The history of haemophilia–a short review. Thrombosis research, 134, S4-S9.
Segre, M., y Ferraz, F. (1997). The concept of health. Revista De Saude Publica, 31(5), 538-542.
Setoodeh, G., Karami, A., Edraki, M., y Nick, N. (2023). Investigating the effect of psychological empowerment on using coping strategies, stress, anxiety and depression in adolescents with haemophilia: a randomized controlled trial. International Journal of Adolescence and Youth, 28. https://doi.org/10.1080/02673843.2023.2270036
Smith, L. (2019). Hemophilia. The American Society for Clinical Laboratory Science, 32, 21-26. https://doi.org/10.29074/ascls.2018001289
Smith, S., y Dacvim. (2009). The cell-based model of coagulation. Journal of veterinary emergency and critical care, 19 1, 3-10. https://doi.org/10.1111/j.1476-4431.2009.00389.x
Srivastava, A., Santagostino, E., Dougall, A., Kitchen, S., Sutherland, M., Pipe, S. W.,…Windyga, J. (2020). WFH guidelines for the management of hemophilia. Haemophilia, 26, 1-158.
Steen Carlsson, K., Winding, B., Astermark, J., Baghaei, F., Brodin, E., Funding, E.,…Andersson, E. (2022). Pain, depression and anxiety in people with haemophilia from three Nordic countries: cross‐sectional survey data from the MIND study. Haemophilia, 28(4), 557-567.
Strandberg-Larsen, M., Gater, A., Marlow, E., y Thomson, A. (2010). Hemophilia A: Patient Impact and Economic Burden of the Disease. Blood, 116, 4763-4763. https://doi.org/10.1182/BLOOD.V116.21.4763.4763
Tovar Arroyo, J. A. (2022). Calidad de vida y representaciones sociales en las personas con Hemofilia: Un análisis cualitativo Ambato: Universidad Tecnològica Indoamèrica].
Toy, L., Williams, T., y Young, E. (1981). Nutritional status of patients with hemophilia. Journal of the American Dietetic Association, 78 1, 47-51. https://doi.org/10.1016/s0002-8223(21)04737-4
Tulloch, A. (2005). What do we mean by health? The British journal of general practice : the journal of the Royal College of General Practitioners, 55 513, 320-323. https://consensus.app/papers/what-do-we-mean-by-health-tulloch/34de9925cef05b949774e4897c1a03dc/
Velou, M., y Ahila, E. (2020). Changing concepts of clotting mechanism of blood: From cascade model to cell-based model. https://consensus.app/papers/changing-concepts-of-clotting-mechanism-of-blood-from-velou-ahila/ef86dff43d885118b07647c20be09e0a/
Von Mackensen, S., Gallmann, L., Siebert, M., y Halimeh, S. (2023). Assessment of Haemophilia Patients' and Other Bleeding Disorders Needs of Psychosocial Support and Health Care Needs and Their Utilization in Haemophilia Treatment Centres (ILLUSTRATE Study). Blood. https://doi.org/10.1182/blood-2023-187236
Weyand, A. C., y Pipe, S. W. (2019). New therapies for hemophilia. Blood, The Journal of the American Society of Hematology, 133(5), 389-398.
Wilson, J. (2022). Nola J. Pender: modelo de promoción de la salud. M. Raile, & EH Sciences (Red.), Modelos y teorias en enfermeria, 320-336.
Witkop, M., Guelcher, C., Forsyth, A., Quon, D., Hawk, S., Curtis, R.,…Cooper, D. (2015). Challenges in transition to adulthood for young adult patients with hemophilia: Quantifying the psychosocial issues and developing solutions. American Journal of Hematology, 90. https://doi.org/10.1002/ajh.24217
Wong, T., Majumdar, S., Adams, E., Bergman, S., Damiano, M., Deutsche, J., y Recht, M. (2011). Overweight and obesity in hemophilia: a systematic review of the literature. American journal of preventive medicine, 41 6 Suppl 4. https://doi.org/10.1016/j.amepre.2011.09.008
Wysocki, D. (1998). But Women Can't Have ‘Hemophilia’!: A Look at the Lives of Women with Bleeding Disorder. Sociological Research Online, 3, 14-25. https://doi.org/10.5153/sro.194
Yong, J., Abrams, S., Wang, G., y Toh, C. (2023). Cell-free histones and the cell-based model of coagulation. Journal of thrombosis and haemostasis : JTH. https://doi.org/10.1016/j.jtha.2023.04.018














