Family caregivers’ needs of people with orphan diseases: closing arrows through nursing care
DOI:
https://doi.org/10.17162/rccs.v13i1.1341Keywords:
Caregivers, Rare Diseases, Nursing Care, Needs Assessment, Theoretical Model.Abstract
Objective: establish the needs of family caregivers of people with orphan diseases considering the Adventist Nursing Model. Methodology: Literature review covering quantitative and qualitative articles found in databases and journals indexed in Spanish, English and Portuguese languages, between 2014 and 2019, whose methodological rigor met STROBE, COREQ, PRISMA, and JADAD criteria. The information obtained was related to the Adventist Nursing Model, NOC, and NIC. Results: 28 articles were selected that analyzed physical, psychological, social, educational, spiritual, health care and economic needs in caregivers of people with orphan diseases, which required multidisciplinary and integral support, including nurse spiritual support through interventions proposed by the NIC, and the Adventist Nursing model (care, connection with God and the other, and empowerment of the caregiver in their own health). Conclusion: the needs of caregivers cover different areas of being, which can be approached independently and interdependently, identified by theoretical models.
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References
Alba, R. (2016). Síndrome de Mowat-Wilson: Historia
de vida de la fortaleza de una madre. Revista
Científica de la Sociedad Española de Enfermería
Neurológica, 44, 25-30. Recuperado de: https://
doi.org/10.1016/j.sedene.2016.06.002
Avellaneda, A., Izquierdo, M., Torrent, J., y Ramón,
J. R. (2007). Enfermedades raras: Enfermedades
crónicas que requieren un nuevo enfoque
sociosanitario. Anales del Sistema Sanitario
de Navarra, 30(2). Recuperado de: https://doi.
org/10.4321/S1137-66272007000300002
Baumbusch, J., Mayer, S. y Sloan-Yip, I. (2019).
Alone in a Crowd? Parents of Children with
Rare Diseases’ Experiences of Navigating the
Healthcare System: XXXX. Journal of Genetic
Counseling, 28(1), 80-90. Recuperado de :
https://doi.org/10.1007/s10897-018-0294-9
Boele, F. W., van Uden-Kraan, C. F., Hilverda,
K., Weimer, J., Donovan, H. S., Drappatz, J.,
Lieberman, F. S., Verdonck-de Leeuw, I., &
Sherwood, P. R. (2017). Neuro-oncology family
caregivers’ view on keeping track of care issues
using eHealth systems: It’s a question of time.
Journal of Neuro-Oncology, 134(1), 157-167.
Recuperado de: https://doi.org/10.1007/s11060-
-2504-y
Bose, M., Mahadevan, M., Schules, D. R., Coleman,
R. K., Gawron, K. M., Gamble, M. B., Roullet,
J.-B., Gibson, K. M., & Rizzo, W. B. (2019).
Emotional experience in parents of children with
Zellweger spectrum disorders: A qualitative study.
Molecular Genetics and Metabolism Reports, 19,
Recuperado de: https://doi.org/10.1016/j.
ymgmr.2019.100459
Cañedo, M., Rice, D. B., Levis, B., Carrier, M.-E.,
Cumin, J., Malcarne, V. L., Hagedoorn, M.,
Thombs, B. D., & the Scleroderma Caregiver
Advisory Committee. (2018). Factors associated
with symptoms of depression among informal
caregivers of people with systemic sclerosis:
A cross-sectional study. Disability and
Rehabilitation, 1-6. Recuperado de: https://doi.or
g/10.1080/09638288.2018.1500647
Egerod, I., Andersson, A. E., Fagerdahl, A.-M. y
Knudsen, V. E. (2017). Images of suffering
depicted in diariea IJMK,Ñ- s of family caregivers
in the acute stage of necrotising soft tissue
infection: A content analysis. Intensive and Critical
Care Nursing, 41, 57-62. Recuperado de: https://
doi.org/10.1016/j.iccn.2017.02.004
Enfermedades huérfanas. (s. f.). Recuperado de
https://www.minsalud.gov.co/salud/publica/
PENT/Paginas/enfermedades-huerfanas.aspx
Fundación Universitaria de Ciencias de la Salud.
Hospital de San José. Bogotá, Colombia., Casas,
C., Hernández, S., Fundación Universitaria de
Ciencias de la Salud. Hospital de San José, Helena
Solano, M., Fundación Universitaria de Ciencias
de la Salud. Hospital de San José, Castiblanco, R.,
Fundación Universitaria de Ciencias de la Salud.
Hospital de San José, Carrillo, A., & Fundación
Universitaria de Ciencias de la Salud. Hospital de
San José. (2018). Experiencias de mujeres frente
al cuidado de hijos diagnosticados con hemofilia.
IATREIA, 31(2), 145-154. Recuperado de: https://
doi.org/10.17533/udea.iatreia.v31n2a03
Harrington, M., Whalley, D., Twiss, J., Rushton,
R., Martin, S., Huynh, L., y Yang, H. (2019).
Insights into the natural history of metachromatic
leukodystrophy from interviews with caregivers.
Orphanet Journal of Rare Diseases, 14(1), 89.
https://doi.org/10.1186/s13023-019-1060-2JAE_
v79n5.pdf. (s. f.). Recuperado de:http://circle.
adventist.org/files/jae/JAE_v79n5.pdf
Kavanaugh, M. S., Cho, C. C., & Howard, M. (2019).
“I Just Learned by Observation and Trial and
Error”: Exploration of Young Caregiver Training
and Knowledge in Families Living with Rare
Neurological Disorders. Child & Youth Care
Forum, 48(4), 479-492. Recuperado de: https://
doi.org/10.1007/s10566-019-09490-z
Kesselheim, A. S., McGraw, S., Thompson, L.,
O’Keefe, K., & Gagne, J. J. (2015). Development
and Use of New Therapeutics for Rare Diseases:
Views from Patients, Caregivers, and Advocates.
The Patient - Patient-Centered Outcomes
Research, 8(1), 75-84. Recuperado de: https://
doi.org/10.1007/s40271-014-0096-6
Landfeldt, E., Edström, J., Buccella, F., Kirschner,
J., y Lochmüller, H. (2018). Duchenne muscular
dystrophy and caregiver burden: A systematic
review. Developmental Medicine y Child
Neurology, 60(10), 987-996. Recuperado de:
https://doi.org/10.1111/dmcn.13934
Landfeldt, E., Lindgren, P., Bell, C. F., Guglieri, M.,
Straub, V., Lochmüller, H., y Bushby, K. (2016a).
Health-related quality of life in patients with
Duchenne muscular dystrophy: A multinational,
cross-sectional study. Developmental Medicine y Child Neurology, 58(5), 508-515. Recuperado de:
https://doi.org/10.1111/dmcn.12938
Landfeldt, E., Lindgren, P., Bell, C. F., Guglieri, M.,
Straub, V., Lochmüller, H., & Bushby, K. (2016b).
Quantifying the burden of caregiving in Duchenne
muscular dystrophy. Journal of Neurology, 263(5),
-915. Recuperado de:https://doi.org/10.1007/
s00415-016-8080-9
Lauder, B., Sinclair, P. M., & Maguire, J. (2018).
Mothers’ experience of caring for a child with
early onset scoliosis: A qualitative descriptive
study. Journal of Clinical Nursing, 27(7-8),
e1549-e1560. Recuperado de: https://doi.
org/10.1111/jocn.14301
Linertová, R., González-Guadarrama, J., Serrano-
Aguilar, P., Posada-De-la-Paz, M., Péntek,
M., Iskrov, G., & Ballester, M. (2019).
Schooling of Children with Rare Diseases
and Disability in Europe. International Journal
of Disability, Development and Education,
(4), 362-373. Recuperado de: https://doi.
org/10.1080/1034912X.2018.1562159
Magliano, L., Patalano, M., Sagliocchi, A., Scutifero,
M., Zaccaro, A., D’Angelo, M. G., Civati, F.,
Brighina, E., Vita, G., Vita, G. L., Messina,
S., Sframeli, M., Pane, M., Lombardo, M. E.,
Scalise, R., D’Amico, A., Colia, G., Catteruccia,
M., Balottin, U., … Politano, L. (2014). “I have
got something positive out of this situation”:
Psychological benefits of caregiving in relatives
of young people with muscular dystrophy. Journal
of Neurology, 261(1), 188-195. Recuperado
de:https://doi.org/10.1007/s00415-013-7176-8
Mori, Y., Downs, J., Wong, K., Anderson, B., Epstein,
A., y Leonard, H. (2017). Impacts of caring for
a child with the CDKL5 disorder on parental
wellbeing and family quality of life. Orphanet
Journal of Rare Diseases, 12(1), 16. Recuperado
de: https://doi.org/10.1186/s13023-016-0563-3
NIC.pdf. (s. f.). Recuperado de http://www.
consultadelsiglo21.com.mx/documentos/NIC.pdf
NOC.pdf. (s. f.). Recuperado de http://www.
consultadelsiglo21.com.mx/documentos/NOC.
OMS | Unidos para combatir las enfermedades raras.
(s. f.). WHO. Recuperado de https://www.who.int/
bulletin/volumes/90/6/12-020612/es/
Palacios-Ceña, D., Famoso-Pérez, P., Salom-
Moreno, J., Carrasco-Garrido, P., Pérez-Corrales,
J., Paras-Bravo, P., y Güeita-Rodriguez, J.
(2018). “Living an Obstacle Course”: A Qualitative
Study Examining the Experiences of Caregivers
of Children with Rett Syndrome. International
Journal of Environmental Research and Public Health, 16(1), 41. Recuperado de: https://doi.
org/10.3390/ijerph16010041
Pelentsov, L. J., Fielder, A. L., y Esterman, A. J.
(2016). The Supportive Care Needs of Parents
With a Child With a Rare Disease: A Qualitative
Descriptive Study. Journal of Pediatric Nursing,
(3), e207-e218. Recuperado de: https://doi.
org/10.1016/j.pedn.2015.10.022
Pelentsov, L. J., Laws, T. A., & Esterman, A. J.
(2015). The supportive care needs of parents
caring for a child with a rare disease: A scoping
review. Disability and Health Journal, 8(4), 475-
Recuperado de: https://doi.org/10.1016/j.
dhjo.2015.03.009
RESOLUCION-8430-DE-1993.pdf. (s. f.). Recuperado
de https://www.minsalud.gov.co/sites/rid/Lists/
BibliotecaDigital/RIDE/DE/DIJ/RESOLUCION-
-DE-1993.PDF
Rice, D. B., Cañedo, M., Carboni, A., Carrier, M.-
E., Cumin, J., Malcarne, V. L., Hagedoorn, M.,
Thombs, B. D., y Scleroderma Caregiver Advisory
Team. (2019). Challenges and support service
preferences of informal caregivers of people
with systemic sclerosis: A cross-sectional survey.
Disability and Rehabilitation, 1-7. Recuperado de:
https://doi.org/10.1080/09638288.2018.1557268
Schwartz, C. E., Powell, V. E., & Eldar-Lissai, A.
(2017). Measuring hemophilia caregiver burden:
Validation of the Hemophilia Caregiver Impact
measure. Quality of Life Research, 26(9), 2551-
Recuperado de:https://doi.org/10.1007/
s11136-017-1572-y
Soares, J. de L., Araújo, L. F. S. de, Bellato, R.,
Universidade Federal do Mato Grosso, Brazil,
Universidade Federal do Mato Grosso, Brazil,
y Universidade Federal do Mato Grosso, Brazil.
(2016). Cuidar na situação de adoecimento raro:
Vivência da família e sua busca por amparo
dos serviços de saúde. Saúde e Sociedade,
(4), 1017-1030. Recuperado de: https://doi.
org/10.1590/s0104-12902016162301
Somanadhan, S., y Larkin, P. J. (2016). Parents’
experiences of living with, and caring for
children, adolescents and young adults with
Mucopolysaccharidosis (MPS). Orphanet Journal
of Rare Diseases, 11. Recuperado de: https://doi.
org/10.1186/s13023-016-0521-0
Stewart, M., Shaffer, S., Murphy, B., Loftus, J., Alvir,
J., Cicchetti, M., & Lenderking, W. R. (2018).
Characterizing the High Disease Burden of
Transthyretin Amyloidosis for Patients and
Caregivers. Neurology and Therapy, 7(2), 349-
Recuperado de:https://doi.org/10.1007/
s40120-018-0106-z
Tejada, E. M., Flores-Rojas, K., Moreno-Quintana, L.,
Muñoz-Villanueva, M. C., Pérez-Navero, J. L., y
Gil-Campos, M. (2019). Necesidades sanitarias
y socioeducativas de niños con enfermedades
raras de tipo metabólico y sus familias: Estudio
cualitativo en un hospital de tercer nivel. Anales
de Pediatría, 90(1), 42-50. Recuperado de:
https://doi.org/10.1016/j.anpedi.2018.03.003
Uttley, L., Carlton, J., Woods, H. B., & Brazier, J. (2018).
A review of quality of life themes in Duchenne
muscular dystrophy for patients and carers.
Health and Quality of Life Outcomes, 16(1), 237.
Recuperado de: https://doi.org/10.1186/s12955-
-1062-0
Yoo, J., Halley, M. C., Lown, E. A., Yank, V., Ort, K.,
Cowan, M. J., Dorsey, M. J., Smith, H., Iyengar,
S., Scalchunes, C., y Mangurian, C. (2019).
Supporting caregivers during hematopoietic
cell transplantation for children with primary
immunodeficiency disorders. Journal of Allergy
and Clinical Immunology, 143(6), 2271-2278.
Recuperado de:https://doi.org/10.1016/j.
jaci.2018.10.017
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