UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Experiences and concerns of patients with recurrent attacks of acute hepatic porphyria: A qualitative study

Naik, H; Stoecker, M; Sanderson, SC; Balwani, M; Desnick, RJ; (2016) Experiences and concerns of patients with recurrent attacks of acute hepatic porphyria: A qualitative study. Molecular Genetics and Metabolism , 119 (3) pp. 278-283. 10.1016/j.ymgme.2016.08.006. Green open access

[img]
Preview
Text
Sanderson_Naik_Acutes Focus Group Manuscript- MGM Revised 17AUG2016-clean.pdf - Accepted version

Download (326kB) | Preview

Abstract

Background: The acute hepatic porphyrias (AHPs) are rare inborn errors of heme biosynthesis, characterized clinically by life-threatening acute neurovisceral attacks. Patients with recurrent attacks have a decreased quality of life (QoL); however, no interactive assessment of these patients' views has been reported. We conducted guided discussions regarding specific topics, to explore patients' disease experience and its impact on their lives. / Methods: Sixteen AHP patients experiencing acute attacks were recruited to moderator-led online focus groups. Five groups (3–4 patients each) were conducted and thematic analyses to identify, examine, and categorize patterns in the data was performed. / Results: All patients identified prodromal symptoms that began days prior to acute severe pain; the most common included confusion (“brain fog"), irritability, and fatigue. Patients avoided hospitalization due to prior poor experiences with physician knowledge of AHPs or their treatment. All patients used complementary and alternative medicine treatments to avoid hospitalization or manage chronic pain and 81% reported varying degrees of effectiveness. All patients indicated their disease impacted personal relationships due to feelings of isolation and difficulty adjusting to the disease's limitations. / Conclusion: Patients with recurrent attacks recognize prodromal warning symptoms, attempt to avoid hospitalization, turn to alternative treatments, and have markedly impaired QoL. Counseling and individualized support is crucial for AHP patients with recurrent attacks.

Type: Article
Title: Experiences and concerns of patients with recurrent attacks of acute hepatic porphyria: A qualitative study
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.ymgme.2016.08.006
Publisher version: http://dx.doi.org/10.1016/j.ymgme.2016.08.006
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Acute hepatic porphyria, Acute intermittent porphyria, Focus groups, Psychosocial experiences, Quality of life
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Epidemiology and Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Epidemiology and Health > Epidemiology and Public Health
URI: https://discovery.ucl.ac.uk/id/eprint/10044293
Downloads since deposit
62Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item