Quality of life in people with hemophilia:

bibliographic review

Authors

  • Luis Horacio Aguiar-Palacios Universidad Autónoma del Estado de México. Facultad de Ciencias de la Conducta; México. Universidad Autónoma de Baja California. Facultad de Ciencias de la Salud; México.
  • Norma Ivonne González-Arratia López-Fuentes Universidad Autónoma del Estado de México. Facultad de Ciencias de la Conducta; México.
  • Ana Olivia Ruíz Martínez Universidad Autónoma del Estado de México. Facultad de Ciencias de la Conducta; México.
  • Alejandra del Carmen Domínguez Espinosa Universidad Iberoamericana. Departamento de Psicología; México.
  • Denisse Ariana Osuna Encinas Universidad Autónoma de Baja California. Facultad de Ciencias de la Salud; México.
  • Yadira Pabón-Varela Universidad Cooperativa de Colombia Sede Santa Marta-Colombia. Programa de Enfermería; Colombia.

Keywords:

Quality of life, Hemophilia, Systematic review, Health, Caregivers

Abstract

Hemophilia is a chronic disease that causes bleeding and severe pain and it has a negative impact on health-related quality of life (HRQoL of those who have this disease. This phenomenon has not been sufficiently studied in many countries. The objective of this study was to analyze and systematize the theoretical and empirical information about the determinants of HRQoL in people with hemophilia (PWH) and their caregivers. The search was carried out in the following databases: Google Scholar, EBSCO and Pubmed, between 2015 and 2020. 57 studies were included in which the most attended population was the adult. The most affected areas were painful joints, disability and the emotional imbalance that this disease causes. High levels of worry and stress are reported in caregivers. The cost of treatment, the effectiveness of medical care, and comparisons with healthy controls were the least studied subjects. 36% of the studies propose applying prophylactic treatment to improve HRQoL. Areas of opportunity are identified that will allow further understanding, measurement and interventions aimed to improve HRQOL in PWH.

References

Allhaidan, M. A., Almashaan, A. M., Alduaij, A. A., Altuwaijri, H. A., Alotaibi, L. A., & Almomen, A. M. (2018). Health-related quality of life in adult patients with hemophilia, Riyadh, Saudi Arabia. J Appl Hematol, 9, 5-10. https://doi.org/10.4103/joah.joah_4_18

Álzate, J. D. V., Sánchez, L. M. M., & Jaramillo, L. I. J. (2018). Calidad de vida: un aspecto olvidado en el paciente con hemofilia. Archivos de Medicina (Manizales), 18(1), 172-180. https://doi.org/10.30554/archmed.18.1.2584.2018

Arteaga-Rubiano, B., & García-Valencia, J. (2018). Calidad de vida en adultos con hemofilia afiliados a un programa de salud en Medellín, Colombia. Iatreia.

Baek, H. J., Park, Y. S., Yoo, K. Y., Cha, J. H., Kim, Y. J., & Lee, K. S. (2020). Health-related quality of life of moderate and severe haemophilia patients: Results of the haemophilia-specific quality of life index in Korea. Plos one, 15(9), e0238686. https://doi.org/10.1371/journal.pone.0238686

Bernal Villada, L., Munar Fonseca, V. C., & Fajardo Granados, N. K. (2020). Impacto de la actividad física en la calidad de vida de pacientes con hemofilia en Bogotá, Colombia (Doctoral dissertation, Universidad del Rosario).

Booth, J., Oladapo, A., Walsh, S., O'Hara, J., Carroll, L., Garcia Diego, D. A., & O'Mahony, B. (2018). Real‐world comparative analysis of bleeding complications and health‐related quality of life in patients with haemophilia A and haemophilia B. Haemophilia, 24(5), e322-e327. https://doi.org/10.1111/hae.13596

Carcao, M., Zunino, L., Young, N. L., Dover, S., Bouskill, V., Hilliard, P., ... & Blanchette, V. S. (2020). Measuring the impact of changing from standard half‐life (SHL) to extended half‐life (EHL) FVIII prophylaxis on health‐related quality of life (HRQoL) in boys with moderate/severe haemophilia A: Lessons learned with the CHO‐KLAT tool. Haemophilia, 26(1), 73-78. https://doi.org/10.1111/hae.13905

Carcao, M., Kearney, S., Santagostino, E., Oyesiku, J. O. O., Young, N. L., Meunier, J., ... & Blanchette, V. S. (2017). Insight into health-related quality of life of young children with haemophilia B treated with long-acting nonacog beta pegol recombinant factor IX. Haemophilia: the official journal of the World Federation of Hemophilia, 23(3), e222-e224. https://doi.org/10.1111/hae.13195

Casas, C. P., Hernández, S. M., Solano, M. H., Castiblanco, R. A., & Carrillo, A. J. (2018). Experiences of women in the care of their children diagnosed with severe and/or moderate symptomatic haemophilia. Iatreia, 31(2), 145-154. https://doi.org/10.17533/udea.iatreia.v31n2a03

Castaño, A. F., Restrepo, M. J., & Durán, F. S. (2017). Quality of life in a population with haemophilia: A cross-sectional study from a single haemophilia treatment center. Revista Colombiana de Reumatología (English Edition), 24(1), 18-24. https://doi.org/10.1016/j.rcreue.2017.04.002

Chowdary, P., Kearney, S., Regnault, A., Hoxer, C. S., & Yee, D. L. (2016). Improvement in health‐related quality of life in patients with haemophilia B treated with nonacog beta pegol, a new extended half‐life recombinant FIX product. Haemophilia, 22(4), e267-e274. https://doi.org/10.1111/hae.12995

Córdova Sánchez, D. A. (2020). Efectos de la hemofilia en la amplitud articular y en la fuerza muscular en pacientes registrados en la provincia de Tungurahua (Bachelor's thesis, Universidad Técnica de Ambato/Facultad de Ciencias de la Salud/Carrera de Terapia Física).

Devlin, N. J., & Brooks, R. (2017). EQ-5D and the EuroQol group: past, present and future. Applied health economics and health policy, 15(2), 127-137. https://doi.org/10.1007/s40258-017-0310-5

Elander, J. (2019). 'Haemophilia'. In C.D. Llewellyn, S. Ayers, C. McManus, S. Newman, K.J. Petrie, T.A. Revenson, & J. Weinman (Eds.). Cambridge Handbook of Psychology, Health and Medicine (3rd ed.), pp. 507-508. Cambridge: Cambridge University Press.

Espinosa-Maldonado, E. G., Torres-González, R., & Gibraltar-Conde, A. (2020). Evaluación de la calidad de vida relacionada con la salud en adolescentes y adultos con hemofilia atendidos en una unidad de rehabilitación. Revista de Hematología, 21(3), 136-141.

Fernández, C. S., & Uribe Rodríguez, A. F. (2016). Comparación de la conducta de enfermedad en pacientes colombianos con hemofilia A, en una muestra con dolor crónico y sin dolor crónico. Nova, 14(26), 63-75. https://doi.org/10.22490/24629448.1752

Fuenmayor Castaño, Alexandra, Jaramillo Restrepo, Mauricio, & Salinas Durán, Fabio. (2017). Calidad de vida en una población con hemofilia: estudio de corte transversal en un centro de tratamiento de hemofilia. Revista Colombiana de Reumatología, 24(1), 18-24. https://doi.org/10.1016/j.rcreu.2016.10.006

García‐Dasí, M., Torres‐Ortuno, A., Cid‐Sabatel, R., & Barbero, J. (2016). Practical aspects of psychological support to the patient with haemophilia from diagnosis in infancy through childhood and adolescence. Haemophilia, 22(5), e349-e358. https://doi.org/10.1111/hae.13018

Girabent-Farrés, M., Monné-Guasch, L., Bagur-Calafat, C., & Fagoaga, J. (2018). Traducción y validación al español del módulo neuromuscular de la escala Pediatric Quality of Life Inventory (PedsQL): Evaluación de la calidad de vida percibida por padres de niños de 2-4 años con enfermedades neuromusculares. Rev Neurol, 66, 81-8. https://doi.org/10.33588/rn.6603.2017323

Guzmán, M. O., Trejo, L. E. L., & Romero, C. P. (2020). Repercusiones biopsicosociales de la hemofilia en la familia diferencias entre padres y madres. Revista Electrónica de Psicología Iztacala, 23(3), 961.

Iorio, A., Iserman, E., Blanchette, V., Dolan, G., Escuriola Ettingshausen, C., Hermans, C., ... & Gringeri, A. (2017). Target plasma factor levels for personalized treatment in haemophilia: a Delphi consensus statement. Haemophilia, 23(3), e170-e179. https://doi.org/10.1111/hae.13215

Iorio, A., Stonebraker, J. S., Chambost, H., Makris, M., Coffin, D., … Herr, C. (2019). Establishing the Prevalence and Prevalence at Birth of Hemophilia in Males. Annals of Internal Medicine, 171(8), 540. https://doi.org/10.7326/M19-1208

Khair, K., Bladen, M. y Holland, M. (2018). Función física y calidad de vida en adolescentes con hemofilia (estudio SO-FIT). Revista de práctica de la hemofilia, 1(2), 11-14.

Limperg, P. F., Haverman, L., Beijlevelt, M., van der Pot, M., Zaal, G., de Boer, W. A., ... & Grootenhuis, M. A. (2017). Psychosocial care for children with haemophilia and their parents in the Netherlands. Haemophilia, 23(3), 362-369. https://doi.org/10.1111/hae.13186

Limperg, P. F., Terwee, C. B., Young, N. L., Price, V. E., Gouw, S. C., Peters, M., ... & Haverman, L. (2017). Health‐related quality of life questionnaires in individuals with haemophilia: a systematic review of their measurement properties. Haemophilia, 23(4), 497-510. https://doi.org/10.1111/hae.13197

Mackensen, S., & Harrigton, C. (2016). The impact of sport on health status, psychological well-being and physical performance of adults with haemophilia. Haemophilia, 1-10. https://doi.org/10.1111/hae.12912

Mahlangu, J., Oldenburg, J., Callaghan, M. U., Shima, M., Mancuso, M. E., Trask, P., ... & von Mackensen, S. (2019). Health‐related quality of life and health status in persons with haemophilia A with inhibitors: A prospective, multicentre, non‐ interventional study (NIS). Haemophilia, 25(3), 382-391. https://doi.org/10.1111/hae.13731

Mancuso, M. E., Mahlangu, J., Sidonio Jr, R., Trask, P., Uguen, M., Chang, T., ... & von Mackensen, S. (2020). Health‐related quality of life and caregiver burden of emicizumab in children with haemophilia A and factor VIII inhibitors-Results from the HAVEN 2 study. Haemophilia.

Muñoz Grass, L. F., & Palacios-Espinosa, X. (2015). Calidad de vida y hemofilia: Una revisión de la literatura. Revista Ces Psicología, 8(1).

Naous, E., De Moerloose, P., Sleilaty, G., Casini, A., & Djambas Khayat, C. (2019). The impact of haemophilia on the social status and the health‐related quality of life in adult Lebanese persons with haemophilia. Haemophilia, 25(2), 264-269. https://doi.org/10.1111/hae.13694

O'Hara, J., Hughes, D., Camp, C., Burke, T., Carroll, L., & Diego, D. A. G. (2017). The cost of severe haemophilia in Europe: the CHESS study. Orphanet journal of rare diseases, 12(1), 1-8. https://doi.org/10.1186/s13023-017-0660-y

O'Hara, J., Walsh, S., Camp, C., Mazza, G., Carroll, L., Hoxer, C., & Wilkinson, L. (2018). The impact of severe haemophilia and the presence of target joints on health-related quality-of-life. Health and Quality of Life Outcomes, 16(84), 2-8. https://doi.org/10.1186/s12955-018-0908-9

Oldenburg, J., Mahlangu, J. N., Bujan, W., Trask, P., Callaghan, M. U., Young, G., ... & von Mackensen, S. (2019). The effect of emicizumab prophylaxis on health‐ related outcomes in persons with haemophilia A with inhibitors: HAVEN 1 Study. Haemophilia, 25(1), 33-44. https://doi.org/10.1111/hae.13618

Guzmán, M. O., Moreno, Z. D. S., Eugenia, G., & Riverón, B. (2017). Repercusiones psicosociales en la salud de portadoras, cuidadoras primarias de pacientes pediátricos con hemofilia. Revista Electrónica de Psicología Iztacala, 20(4), 1233.

Osorio, M. (2016). Repercusiones biopsicosociales en los padres que tienen un hijo con hemofilia. Revista de investigación en psicología, 19(2), 111-121. https://doi.org/10.15381/rinvp.v19i2.12893

Osorio-Guzmán, M., Olvera-González, S., Bazán-Riverón, G. E., & Gaitán- Fitch, R. C. (2015). Calidad de vida relacionada con la salud percibida por pacientes pediátricos con hemofilia y por sus padres. Salud Pública de México, 57, 112-114.

Osorio-Guzmán, M., & Vitale, S. G. (2017). Apoyo psicológico a la familia: efecto en la estructura familiar de pacientes con hemofilia. Revista Digital Internacional de Psicología y Ciencia Social, 3(2), 212-225. https://doi.org/10.22402/j.rdipycs.unam.3.2.2017.142.212-225

Osorio-Guzmán, M., Gutiérrez-González, G., Bazán-Riverón, G. E., Núñez- Villegas, N. N., & Fernández-Castilloc, G. J. (2017). Percepción de la calidad de vida relacionada con la salud y la depresión en pacientes con hemofilia. Revista Médica del Instituto Mexicano del Seguro Social, 55(4), 416-422.

Pai, M., Key, N. S., Skinner, M., Curtis, R., Feinstein, M., Kessler, C., ... & Schünemann, H. J. (2016). NHF‐McMaster guideline on care models for haemophilia management. Haemophilia, 22, 6-16. https://doi.org/10.1111/hae.13008

Pedra, G., Christoffersen, P., Khair, K., Lee, X. Y., O'Hara, S., O'Hara, J., & Pasi, J. (2020). The impact of factor infusion frequency on health-related quality of life in people with haemophilia. The Journal of Haemophilia Practice, 7(1), 102-109. https://doi.org/10.17225/jhp00158

Peyvandi, F., Tavakkoli, F., Frame, D., Quinn, J., Kim, B., Lawal, A., ... & Wong, W. Y. (2019). Burden of mild haemophilia A: Systematic literature review. Haemophilia, 25(5), 755-763. https://doi.org/10.1111/hae.13777

Phadnis, S., & Kar, A. (2017). The impact of a haemophilia education intervention on the knowledge and health related quality of life of parents of Indian children with haemophilia. Haemophilia, 23(1), 82-88. https://doi.org/10.1111/hae.13070

Robles-Espinoza, A. I., Rubio-Jurado, B., De la Rosa-Galván, E. V., & Nava-Zavala, A. H. (2016). Generalidades y conceptos de calidad de vida en relación con los cuidados de salud. El residente, 11(3), 120-125.

Runkel, B., Von Mackensen, S., & Hilberg, T. (2017). RCT-subjective physical performance and quality of life after a 6‐month programmed sports therapy (PST) in patients with haemophilia. Haemophilia, 23(1), 144-151. https://doi.org/10.1111/hae.13079

Salimam, M., Brodin, E., Spaals-Abrahamsson, Y., Berntorp, E., & Zetterberg, E. (2016). The effect of Nordic Walking on joint status, quality of life, physical ability, exercise capacity and pain in adult persons with haemophilia. Short Communication, 27, 467-472. https://doi.org/10.1097/MBC.0000000000000554

Salomon, T., Chaves, D. G., Brener, S., Martins, P. R. J., Mambrini, J. V. M., & Peixoto, S. V. (2017). Determining the health‐related quality of life in individuals with haemophilia in developing economies: results from the Brazilian population. Haemophilia, 23(1), 42-49. https://doi.org/10.1111/hae.13130

Shah, R. M. (2016). Measuring And Understanding Health-Related Quality Of Life Among Adult Patients With Hemophilia.

Soucie, J. M., Grosse, S. D., Siddiqi, A. E. A., Byams, V., Thierry, J., Zack, M. M., ... & Hemophilia Treatment Centers Network. (2017). The effects of joint disease, inhibitors and other complications on health‐related quality of life among males with severe haemophilia A in the United States. Haemophilia, 23(4), e287-e293. https://doi.org/10.1111/hae.13275

Stemberger, M., Kallenbach, F., Schmit, E., McEneny-King, A., Germini, F., Yeung, C. H. & Iorio, A. (2019). Impact of adopting population pharmacokinetics for tailoring prophylaxis in haemophilia A patients: a historically controlled observational study. Thrombosis and haemostasis, 119(03), 368-376. https://doi.org/10.1055/s-0039-1677700

Tang, L., Xu, W., Li, C. G., Hou, F., Feng, X. Q., Wang, H., ... & Wu, R. (2018). Describing the quality of life of boys with haemophilia in China: Results of a multicentre study using the CHO‐KLAT. Haemophilia, 24(1), 113-119. https://doi.org/10.1111/hae.13349

Trindade, G. C., Viggiano, L. G. D. L., Brant, E. R., Lopes, C. A. D. O., Faria, M. L. D., Ribeiro, P. H. N. D. S., ... & Pinheiro, M. D. B. (2019). Evaluation of quality of life in hemophilia patients using the WHOQOL-bref and Haemo-A-Qol questionnaires. Hematology, transfusion and cell therapy, 41(4), 335-341. https://doi.org/10.1016/j.htct.2019.03.010

Ventura-León, J. L. (2017). Propiedades psicométricas del FACES-III. Revista Peruana de Medicina Experimental y Salud Pública, 34(1), 149-150. https://doi.org/10.17843/rpmesp.2017.341.2778

Von Mackensen, S., Harrington, C., Tuddenham, E., Littley, A., Will, A., Fareh, M., ... & Khair, K. (2016). The impact of sport on health status, psychological well‐being and physical performance of adults with haemophilia. Haemophilia, 22(4), 521-530. https://doi.org/10.1111/hae.12912

Von Mackensen, S., Eldar‐Lissai, A., Auguste, P., Krishnan, S., von Maltzahn, R., Yu, R., & Wyrwich, K. W. (2017). Measurement properties of the Haem‐A‐QoL in haemophilia clinical trials. Haemophilia, 23(3), 383-391. https://doi.org/10.1111/hae.13140

Von Mackensen, S., Schleicher, C., Heine, S., Graf, N., & Eichler, H. (2020). Health-related quality of life, treatment satisfaction and adherence outcomes of haemophilia patients living in a german rural region. Hämostaseologie, 40(05), 631-641. https://doi.org/10.1055/a-1141-1175

Von Mackensen, S., Catalani, O., Asikanius, E., Paz‐Priel, I., Lehle, M., & Trask, P. (2020). Determining meaningful health‐related quality‐of‐life improvement in persons with haemophilia A using the Haemophilia Quality of Life Questionnaire for Adults (Haem‐A‐QoL). Haemophilia. https://doi.org/10.1111/hae.14184

Von Mackensen, S., Shah, J., Seifert, W., & Kenet, G. (2019). Health‐related quality of life in paediatric haemophilia B patients treated with rIX‐FP. Haemophilia, 25(1), 45-53. https://doi.org/10.1111/hae.13624

Wellington Criollo, K. K. (2018). Tratamiento profilactico en hemofilia: impacto en calidad de vida y en la economía institucional (Doctoral dissertation, Universidad de Guayaquil. Facultad de Ciencias Médicas. Escuela de Graduados).

Williams, V. K., Antoniou, G., Jackson, A., & Atkins, A. (2016). Parents' perception of quality of life in their sons with haemophilia. Journal of paediatrics and child health, 52(12), 1095-1098. https://doi.org/10.1111/jpc.13340

World Health Organization. (2016). World health statistics 2016: monitoring health for the SDGs sustainable development goals. World Health Organization.

Wyrwich, K. W., Krishnan, S., Auguste, P., Poon, J. L., von Maltzahn, R., Yu, R., ... & von Mackensen, S. (2016). Changes in health‐related quality of life with treatment of longer‐acting clotting factors: results in the A‐LONG and B‐LONG clinical studies. Haemophilia, 22(6), 866-872. https://doi.org/10.1111/hae.12987

Yazicioglu, I., Deveci, C., Çiftçi, V., Antmen, B., & Doğan, M. C. (2019). Parent's report on oral health‐related quality of life of children with haemophilia. Haemophilia, 25(2), 229-235. https://doi.org/10.1111/hae.13678

Zhang, H., Huang, J., Kong, X., Ma, G., & Fang, Y. (2019). Health-related quality of life in children with haemophilia in China: a 4-year follow-up prospective cohort study. Health and quality of life outcomes, 17(1), 1-8. https://doi.org/10.1186/s12955-019-1083-3

Published

2022-07-05

How to Cite

Aguiar-Palacios, L. H., González-Arratia López-Fuentes, N. I., Ruíz Martínez, A. O., Domínguez Espinosa, . A. del C., Osuna Encinas, D. A., & Pabón-Varela, Y. (2022). Quality of life in people with hemophilia:: bibliographic review. Calidad De Vida Y Salud Journal, 15(1), 15-29. Retrieved from http://revistacdvs.uflo.edu.ar/index.php/CdVUFLO/article/view/341

Issue

Section

Artículos

Most read articles by the same author(s)