Operational description of rare diseases: a reference to improve the recognition and visibility of rare diseases

被引:3
作者
Wang, Chiuhui Mary [1 ]
Whiting, Amy Heagle [1 ]
Rath, Ana [2 ]
Anido, Roberta [3 ]
Ardigo, Diego [4 ]
Baynam, Gareth [5 ]
Dawkins, Hugh [6 ]
Hamosh, Ada [7 ]
Le Cam, Yann [8 ]
Malherbe, Helen [9 ,16 ]
Molster, Caron M. [10 ]
Monaco, Lucia [11 ]
Padilla, Carmencita D. [12 ]
Pariser, Anne R. [11 ]
Robinson, Peter N. [13 ]
Rodwell, Charlotte [2 ]
Schaefer, Franz [14 ]
Weber, Stefanie [15 ]
Macchia, Flaminia [1 ]
机构
[1] Rare Dis Int, Plateforme Malad Rares, 96 Rue Didot, F-75014 Paris, France
[2] INSERM, US14 Orphanet Plateforme Malad Rares, 96 Rue Didot, F-75014 Paris, France
[3] Federac Argentina Enfermedades Poco Frecuentes, Antonino Ferrari 1044, RA-1424 Buenos Aires, Argentina
[4] Chiesi Farmaceut SpA, Via Paradigna 131-A, Parma, Italy
[5] King Edward Mem Hosp, Perth Childrens Hosp & Western Australian Register, Rare Care Ctr, Western Australian Dept Hlth, Subiaco, WA, Australia
[6] Univ Notre Dame Australia UNDA, Sch Med, 160 Oxford St, Darlinghurst, NSW, Australia
[7] Johns Hopkins Univ, Sch Med, 600 N Wolfe St,Blalock 1008, Baltimore, MD 21287 USA
[8] EURORDIS Rare Dis Europe, Plateforme Malad Rares, 96 Rue Didot, F-75014 Paris, France
[9] Rare Dis South Africa, 63 Peter Pl, Johannesburg, South Africa
[10] West Australia Dept Hlth, Off Populat Hlth Genom, Level 3,C Block,189 Royal St, East Perth, WA 6004, Australia
[11] Int Rare Dis Res Consortium IRDiRC, Paris, France
[12] Univ Philippines Manila, Coll Med, Dept Pediat, Pedro Gil St,Taft Ave, Manila 1000, Philippines
[13] Jackson Lab Genom Med, 10 Discovery Dr, Farmington, CT 06032 USA
[14] Heidelberg Univ, Med Fac Heidelberg, European Rare Kidney Dis Reference Network ERKNet, Neuenheimer Feld 130-3, D-69120 Heidelberg, Germany
[15] Fed Inst Drugs & Med Devices, Dept K Code Syst & Registers, Kurt Georg Kiesinger Allee 3, D-53175 Bonn, Germany
[16] North West Univ, Ctr Human Metabol, Potchefstroom, South Africa
关键词
Rare diseases; Visibility; Definition; Coding; Healthcare systems; Prevalence; Diagnosis; Social care; Research and development; Universal health coverage;
D O I
10.1186/s13023-024-03322-7
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Improving health and social equity for persons living with a rare disease (PLWRD) is increasingly recognized as a global policy priority. However, there is currently no international alignment on how to define and describe rare diseases. A global reference is needed to establish a mutual understanding to inform a wide range of stakeholders for actions. A multi-stakeholder, global panel of rare disease experts, came together and developed an Operational Description of Rare Diseases. This reference describes which diseases are considered rare, how many persons are affected and why the rare disease population demands specific attention. The operational description of rare diseases is framed in two parts: a core definition of rare diseases, complemented by a descriptive framework of rare diseases. The core definition includes parameters that permit the identification of which diseases are considered rare, and how many persons are affected. The descriptive framework elaborates on the impact and burden of rare diseases on patients, their caregivers and families, healthcare systems, and society overall. The Operational Description of Rare Diseases establishes a common point of reference for decision-makers across the world who strive to understand and address the unmet needs of persons living with a rare disease. Adoption of this reference is essential to improving the visibility of rare conditions in health systems across the world. Greater recognition of the burden of rare diseases will motivate new actions and policies to address the unmet needs of the rare disease community.
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