An international patient-centred study of retroperitoneal fibrosis.


Journal

QJM : monthly journal of the Association of Physicians
ISSN: 1460-2393
Titre abrégé: QJM
Pays: England
ID NLM: 9438285

Informations de publication

Date de publication:
22 Mar 2022
Historique:
received: 22 10 2020
revised: 17 11 2020
pubmed: 31 12 2020
medline: 29 3 2022
entrez: 30 12 2020
Statut: ppublish

Résumé

The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient's health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient's life as well as allowing healthcare providers to compare and improve performance. To understand the physical and psychosocial impact that RPF has upon peoples' lives. An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences. An international online questionnaire comprising 62 questions/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6 months. A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically. This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient's health.

Sections du résumé

BACKGROUND BACKGROUND
The impact that rare chronic disorders, such as retroperitoneal fibrosis (RPF), can have on the physical and psychological aspects of a patient's health is poorly understood. Patient-related outcome measures and experiences provide a unique opportunity to understand the impact rare chronic disorders have on a patient's life as well as allowing healthcare providers to compare and improve performance.
AIM OBJECTIVE
To understand the physical and psychosocial impact that RPF has upon peoples' lives.
DESIGN METHODS
An international online questionnaire was therefore created to gain insights into how patients with RPF, a rare fibro-inflammatory condition, viewed their health and experiences.
METHODS METHODS
An international online questionnaire comprising 62 questions/free text options, was designed in collaboration with two patient advocates and the multi-disciplinary Renal Association Rare Disease Registry (RaDaR) RPF Group the questionnaire was anonymous and freely accessible on a GOOGLE Form online platform for 6 months.
RESULTS RESULTS
A total of 229 patients from 30 countries across 5 continents responded. Four key issues were identified; (i) pain; (ii) therapy-related side effects; (iii) lack of informed doctors/information about their condition and its management; and (iv) psychological burden. Variations in diagnosis and management are highlighted with 55% undergoing a biopsy to reach a diagnosis of RPF; 75% of patients underwent a further interventional procedure with 60% concurrently treated medically.
CONCLUSION CONCLUSIONS
This study will guide further development of clinical and academic multi-disciplinary activity and shows the importance of trying to understand the impact of rare chronic disorders on the physical and psychological aspects of a patient's health.

Identifiants

pubmed: 33377941
pii: 6055561
doi: 10.1093/qjmed/hcaa327
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

148-154

Subventions

Organisme : National Institute for Health Research Academic Clinical Fellowship
Organisme : Ken and Mary Minton Chair of Renal Medicine
Organisme : National Institute for Health Research (NIHR) Biomedical Research Centre
Organisme : Imperial College Healthcare NHS Trust and Imperial College London

Informations de copyright

© The Author(s) 2020. Published by Oxford University Press on behalf of the Association of Physicians. All rights reserved. For permissions, please email: journals.permissions@oup.com.

Auteurs

R Dattani (R)

West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK.

T D Barwick (TD)

Department of Surgery and Cancer, Imperial College London, London, UK.

G El Wardany (G)

Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK.

N Gibbons (N)

Department of Urology, Imperial College Healthcare NHS Trust, London, UK.

J C Mason (JC)

Imperial College London, National Heart and Lung Institute London, London, UK.

P Morgan (P)

Retroperitoneal Fibrosis (RPF) Rare Disease Group, Patient Representative London, London, UK.

C D Pusey (CD)

West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK.
Imperial College London, Centre for inflammatory Disease London, London, UK.

F W K Tam (FWK)

West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK.
Imperial College London, Centre for inflammatory Disease London, London, UK.

J A P Tomlinson (JAP)

West London Renal and Transplant Centre, Renal Medicine, Du Cane Road, London W12 0HS, UK.

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