Parental Caregiving Experiences in Epidermolysis Bullosa: A Phenomenological Study

Document Type : Original Article

Authors

1 Department of Medical-Surgical Nursing, School of Nursing and Midwifery, Mashhad University of Medical Sciences, Mashhad, Iran;

2 Nursing and Midwifery Care Research Center, Mashhad University of Medical Sciences, Mashhad, Iran;

3 Department of Pediatrics, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran

Abstract

Background: Epidermolysis bullosa (EB) is a rare, inherited disease characterized by mucocutaneous
fragility. It requires continuous family support and caregiving. However, insights into the complex
realities of daily caregiving roles for parents remain limited. This study aimed to explore the lived
experiences of Iranian family caregivers caring for their children with EB.
Methods: This phenomenological study was carried out from February to November 2023 at Mashhad
Akbar Hospital or participants’ homes. Purposeful maximum variation sampling was used to recruit
10 parents (3 fathers, 7 mothers) who met the inclusion criteria. Data were gathered through in-person,
semi-structured interviews and analyzed using Van Manen method in MAXQDA software version 2020.
Results: Caregivers’ experiences elucidate the profound adversity endured by parents raising children
with EB. Four themes emerged from the data: “lost and confused about care and treatment”; “trapped in
endless cycle of hardship caring”; “inevitable self-isolation”; and “bitter and deteriorating family life”.
Conclusion: Family caregivers who provide care for children with EB face numerous difficulties,
leading to significant stress and heavy responsibilities. Their lived experiences provide a comprehensive
picture of the physical, psychological, and social difficulties related to this rare disease.

Highlights

Hassan Khalili (Google Scholar) (PubMed)

Zahra Sadat Manzari (Google Scholar) (PubMed)

Keywords

References

1.    Plewig G, French L, Ruzicka T, et al. Braun-Falco ́s Dermatology. Berlin: Springer Berlin Heidelberg; 2022.
2.    Mauritz PJ, Bolling M, Duipmans JC, et al. Patients’ and parents’ experiences during wound care of epidermolysis bullosa from a dyadic perspective: a survey study. Orphanet Journal of Rare Diseases. 2022;17:313.
3.    Pfendner EG, Lucky AW. Dystrophic epidermolysis bullosa. In: Adam MP, Feldman J, Mirzaa GM, et al (eds). GeneReviews. Seattle (WA): University of Washington; 2018.
4.    Watkins J. Diagnosis, treatment and management of epidermolysis bullosa. British Journal of Nursing. 2016;25:428-31.
5.    Prodinger C, Diem A, Bauer JW, Laimer M. [Mucosal manifestations of epidermolysis bullosa: Clinical presentation and management]. Hautarzt. 2016;67:806-15.
6.    Prodinger C, Reichelt J, Bauer JW, 
et al. Epidermolysis bullosa: Advances in research and treatment. Experimental Dermatology. 2019;28:1176-89.
7.    Pelentsov LJ, Laws TA, Esterman AJ. The supportive care needs of parents caring for a child with a rare disease: a scoping review. Disability and Health Journal. 2015;8:475-91.
8.    Chateau AV, Blackbeard D, Aldous C. The impact of epidermolysis bullosa on the family and healthcare practitioners: a scoping review. International Journal of Dermatology. 2023;62:459-75.
9.    Wu YH, Sun FK, Lee PY. Family caregivers’ lived experiences of caring for epidermolysis bullosa patients: A phenomenological study. Journal of Clinical Nursing. 2020;29:1552-60.
10.    Stefano SDE, Grassi FS, Lalatta F, et al. Family burden of children suffering from epidermolysis bullosa. Italian Journal of Dermatology and Venereology. 2021;156:580-7.
11.    Holliday AM, Quinlan CM, Schwartz AW. The hidden patient: The CARE framework to care for caregivers. Journal of Family Medicine and Primary Care. 2022;11:5-9.
12.    Martínez-Ripoll JM, García-Domingo M, de la Fuente Robles YM. Epidermolysis Bullosa in Spain: A Qualitative Analysis of Its Social Impact on Families With Diagnosed Minors. Health Expectations. 2024;27:e14128.
13.    Chogani F, Parvizi MM, Murrell DF, 
et al. Assessing the quality of life in the families of patients with epidermolysis bullosa: The mothers as main caregivers. International Journal of Women’s Dermatology. 2021;7:721-6.
14.    Forghani SF, Jahangiri R, Ghasemi F, et al. Economic Burden of Epidermolysis Bullosa Disease in Iran. Medical Journal of the Islamic Republic of Iran. 2021;35:146.
15.    Eghtedar S, Jasemi M, Habibzadeh H. The role of beliefs and psychological factors in caring of patients with cancer: the lived experiences of informal caregivers. Holistic Nursing Practice. 2021;35:248-56.
16.    Rahnama M, Khoshknab MF, Maddah SSB, et al. Religion as an alleviating factor in Iranian cancer patients: a qualitative study. Asian Pacific Journal of Cancer Prevention. 2015;16:8519-24.
17.    Ahmadi F, Khodayarifard M, Zandi S, et al. Religion, culture and illness: A sociological study on religious coping in Iran. Mental Health, Religion & Culture. 2018;21:721-36.
18.    Davari M, Haycox A, Walley T. The Iranian health insurance system; past experiences, present challenges and future strategies. Iranian Journal of Public Health. 2012;41:1-9.
19.    Van ManenM. Phenomenology of Practice. Meaning-Giving Methods in Phenomenological Research and Writing. London/New York: Routledge; 2016.
20.    Van Manen M. Researching lived experience: Human science for an action sensitive pedagogy. 2nd ed. New York: Routledge; 2016.
21.    Kish AM, Newcombe PA, Haslam DM. Working and caring for a child with chronic illness: A review of current literature. Child Care Health and Development. 2018;44:343-54.
22.    Kearney S, Donohoe A, McAuliffe E. Living with epidermolysis bullosa: Daily challenges and health-care needs. Health Expectations. 2020;23:368-76.
23.    Martin K, Geuens S, Asche JK, et al. Psychosocial recommendations for the care of children and adults with epidermolysis bullosa and their family: evidence based guidelines. Orphanet Journal of Rare Diseases. 2019;14:133.
24.    Chateau AV, Aldous C, Dlova N, et al. 
‘It breaks my heart’: Healthcare practitioners’ caring for families with epidermolysis bullosa. Health SA. 2023;28:2355.
25.    Ireland CJ, Pelentsov LJ, Kopecki Z. Caring for a child with Epidermolysis Bullosa: a scoping review on the family impacts and support needs. Wound Practice & Research. 2021;29:86-97.
26.    Bruckner AL, Losow M, Wisk J, et al. The challenges of living with and managing epidermolysis bullosa: insights from patients and caregivers. Orphanet Journal of Rare Diseases. 2020;15:1.
27.    Mącik D, Kowalska-Dąbrowska M. The need of social support, life attitudes and life satisfaction among parents of children suffering from epidermolysis bullosa. Dermatology Review. 2015;102:211-20.
International Journal of Community Based Nursing & Midwifery
Volume 13, Issue 2
April 2025
Pages 149-160

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