Rare disease care pathways in the EU: from odysseys and labyrinths towards highways

Birutė Tumienė1, Holm Graeßner2
1Institute of Biomedical Sciences, Faculty of Medicine, Vilnius University, Vilnius, Lithuania
2Institute for Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany

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Bachoud-Lévi AC, Ferreira J, Massart R et al (2019) International guidelines for the treatment of Huntington’s disease. Front Neurol 10:710. https://doi.org/10.3389/fneur.2019.00710

Rare Barometer (2017) Juggling care and daily life: the balancing act of the rare disease community. Available at: http://download2.eurordis.org.s3.amazonaws.com/rbv/2017_05_09_Social%20survey%20leaflet%20final.pdf

Bolz-Johnson M, Meek J, Hoogerbrugge N (2020) “Patient Journeys”: improving care by patient involvement. Eur J Hum Genet 28(2):141–143. https://doi.org/10.1038/s41431-019-0555-6

Castro R, Senecat J, de Chalendar M et al (2017) Bridging the gap between health and social care for rare diseases: key issues and innovative solutions. Adv Exp Med Biol 1031:605–627. https://doi.org/10.1007/978-3-319-67144-4_32

Cialone J, Augustine EF, Newhouse N, Vierhile A, Marshall FJ, Mink JW (2011) Quantitative telemedicine ratings in Batten disease: implications for rare disease research. Neurology. 77(20):1808–1811. https://doi.org/10.1212/WNL.0b013e3182377e29

Commission Implementing Decision (2014) of 10 March setting out criteria for establishing and evaluating European Reference Networks and their Members and for facilitating the exchange of information and expertise on establishing and evaluating such Networks. 2014. Available at: https://eur-lex.europa.eu/legal-content/EN/TXT/?uri=OJ%3AJOL_2014_147_R_0007

Commission Implementing Decision (2019) 2014/287/EU of 26 July amending Implementing Decision 2014/287/EU setting out criteria for establishing and evaluating European Reference Networks and their Members and for facilitating the exchange of information and expertise on establishing and evaluating such Networks. 2019. Available at: https://eur-lex.europa.eu/legal-content/EN/TXT/PDF/?uri=CELEX:32019D1269&from=EN

Coster S, Li Y, Norman IJ (2020) Cochrane reviews of educational and self-management interventions to guide nursing practice: a review [published online ahead of print, 2020 Jul 2]. Int J Nurs Stud 110:103698. https://doi.org/10.1016/j.ijnurstu.2020.103698

Council of the European Union. Consolidated versions of the Treaty on European Union and the Treaty on the Functioning of the European Union (2012) Consolidated versions of the Treaty on European Union and the Treaty on the Functioning of the European Union. Official Journal C 326 1–12. Available at: https://eur-lex.europa.eu/legal-content/EN/TXT/PDF/?uri=CELEX:12012E/TXT&from=EN

De Silva R, Greenfield J, Cook A et al (2019) Guidelines on the diagnosis and management of the progressive ataxias. Orphanet J Rare Dis 14(1):51. https://doi.org/10.1186/s13023-019-1013-9

Derbel O, Heudel PE, Cropet C et al (2017) Survival impact of centralization and clinical guidelines for soft tissue sarcoma (A prospective and exhaustive population-based cohort). PLoS One 12(2):e0158406. https://doi.org/10.1371/journal.pone.0158406

Donald A, Cizer H, Finnegan N, Collin-Histed T, Hughes DA, Davies EH (2019) Measuring disease activity and patient experience remotely using wearable technology and a mobile phone app: outcomes from a pilot study in Gaucher disease. Orphanet J Rare Dis 14(1):212. https://doi.org/10.1186/s13023-019-1182-6

ERN Board of Member States (2019) Statement of the ERN Board of Member States on Integration of the European Reference Networks to the healthcare systems of Member States. Available at: https://ec.europa.eu/health/sites/health/files/ern/docs/integration_healthcaresystems_en.pdf;https://ec.europa.eu/health/sites/health/files/ern/docs/integration_healthcaresystems_annex_en.pdf

European Commission (2016) Report of the Expert Panel on effective ways of investing in Health (EXPH) on Disruptive Innovation. Considerations for health and health care in Europe Available at: https://ec.europa.eu/health/sites/health/files/expert_panel/docs/012_disruptive_innovation_en.pdf

European Commission (2017) State of Health in the EU: Companion Report 2017. Publications Office of the European Union, Luxembourg Available at: https://ec.europa.eu/health/sites/health/files/state/docs/2017_companion_en.pdf

European Commission (2018) Health system performance assessment – Integrated Care Assessment (20157303 HSPA). Publications Office of the European Union, Luxembourg Available at: https://ec.europa.eu/health/sites/health/files/systems_performance_assessment/docs/2018_integratedcareassessment_en.pdf

European Commission (2019) ERN Assessment Manual for Applicants. Description and Procedures Available at: https://ec.europa.eu/health/sites/health/files/ern/docs/call2019_assesmanual_en.pdf

European Commission website (n.d.) European Reference Networks. Board of Member States. Available at: https://ec.europa.eu/health/ern/board_member_states_en

European Court of Auditors (2019) EU actions for cross-border healthcare: significant ambitions but improved management required. Available at: https://www.eca.europa.eu/Lists/ECADocuments/SR19_07/SR_HEALTH_CARE_EN.pdf

European Joint Programme for Rare Diseases (n.d.) website. Available at: https://www.ejprarediseases.org/

European Parliament and the Council of the European Union (2011) Directive 2011/24/EUof the European Parliament and of the Council of 9 March on the application of patients’ rights in cross-border healthcare. Available at: https://eur-lex.europa.eu/legal-content/EN/TXT/?uri=celex%3A32011L0083

European Reference Network Neurological Diseases (ERN-RND) (n.d.) Available at: http://www.ern-rnd.eu/

Gatta G, Trama A, Capocaccia R (2019) RARECARENet Working Group. Epidemiology of rare cancers and inequalities in oncologic outcomes. Eur J Surg Oncol 45(1):3–11. https://doi.org/10.1016/j.ejso.2017.08.018

Greer SL, Fahy N, Rozenblum S, et al. Everything you always wanted to know about European Union health policies but were afraid to ask. 2nd ed., 2019. European Observatory on Health Systems and Policies. Available at: https://apps.who.int/iris/bitstream/handle/10665/328267/9789289051767-eng.pdf?sequence=1&isAllowed=y.

Harrison M, Birch S, Eden M, Ramsden S, Farragher T, Payne K, Hall G, Black GCM (2015) Variation in healthcare services for specialist genetic testing and implications for planning genetic services: the example of inherited retinal dystrophy in the English NHS. J Community Genet 6(2):157–165. https://doi.org/10.1007/s12687-014-0210-4

Hoogeveen IJ, Peeks F, De Boer F, Lubout CMA, de Koning TJ, te Boekhorst S, Zandvoort RJ, Burghard R, van Spronsen FJ, Derks TGJ (2018) A preliminary study of telemedicine for patients with hepatic glycogen storage disease and their healthcare providers: from bedside to home site monitoring. J Inherit Metab Dis 41(6):929–936. https://doi.org/10.1007/s10545-018-0167-2

Kalaiselvan R, Malik AK, Rao R, Wong K, Ali N, Griffin M, Chandrasekar CR, Fenwick SF, Poston GJ, Malik H (2019) Impact of centralization of services on outcomes in a rare tumour: retroperitoneal sarcomas. Eur J Surg Oncol 45(2):249–253. https://doi.org/10.1016/j.ejso.2018.06.032

Keusch S, Turk A, Saxer S et al (2017) Rehabilitation in patients with pulmonary arterial hypertension. Swiss Med Wkly 147:w14462. https://doi.org/10.4414/smw.2017.14462

Kyrklund K, Sloots CEJ, De Blaauw I et al (2020) ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease. Orphanet J Rare Dis 15(1):164. https://doi.org/10.1186/s13023-020-01362-3

Lawal AK, Rotter T, Kinsman L et al (2016) What is a clinical pathway? Refinement of an operational definition to identify clinical pathway studies for a Cochrane systematic review. BMC Med 14:35. https://doi.org/10.1186/s12916-016-0580-z

Le Cam Y, Bolz-Johnson M (2019) Expert by Experience: valuing patient engagement in healthcare. In: M.-P. Pomey et al. (eds.), Patient engagement, organizational behaviour in healthcare, Springer Nature Switzerland AG. doi:10.1007/978-3-030-14101-1_9

McDonald KM, Sundaram V, Bravata DM, Lewis R, Lin N, Kraft S, McKinnon M, Paguntalan H, Owens DK (2007) Care Coordination. Vol 7 of. In: Shojania KG, KM MD, Wachter RM, Owens DK (eds) Closing the quality gap: a critical analysis of quality improvement strategies. Technical Review 9 (Prepared by the Stanford University-UCSF Evidence-based Practice Center under contract 290-02-0017). AHRQ Publication No. 04(07)-0051-7. Agency for Healthcare Research and Quality, Rockville, MD

National Transitions of Care Coalition (n.d.) Available at: www.ntocc.org

Nuti S, Seghieri C, Niccolai F, Vasta F, Grazzini G (2017) Comparing regional models of congenital bleeding disorders: preliminary steps in the Italian context. BMC Res Notes 10(1):229. https://doi.org/10.1186/s13104-017-2552-6

Overill S (2003) The development, role, and integration of integrated care pathways in modern day health care. In: Ellershaw J, Wilkinson S (eds.). Care for the dying: A pathway to excellence. Oxford University Press

Pavan S, Rommel K, Mateo Marquina ME, Höhn S, Lanneau V, Rath A (2017) Clinical practice guidelines for rare diseases: the Orphanet Database. PLoS One 12(1):e0170365. https://doi.org/10.1371/journal.pone.0170365

Perrier L, Rascle P, Morelle M et al (2018) The cost-saving effect of centralized histological reviews with soft tissue and visceral sarcomas, GIST, and desmoid tumors: the experiences of the pathologists of the French Sarcoma Group. PLoS One 13(4):e0193330. https://doi.org/10.1371/journal.pone.0193330

Peycelon M, Faraj S, Leclair MD, Bonnard A (2017) French Connection between specialized and routine pediatric surgical care. Eur J Pediatr Surg 27(5):410–415. https://doi.org/10.1055/s-0037-1606636

Plishka C, Rotter T, Kinsman L et al (2016) Effects of clinical pathways for chronic obstructive pulmonary disease (COPD) on patient, professional and systems outcomes: protocol for a systematic review. Syst Rev 5(1):135. https://doi.org/10.1186/s13643-016-0311-8

Portaro S, Calabrò RS, Bramanti P, Silvestri G, Torrisi M, Conti-Nibali V, Caliri S, Lunetta C, Alagna B, Naro A, Bramanti A (2018) Telemedicine for facio-scapulo-humeral muscular dystrophy: a multidisciplinary approach to improve quality of life and reduce hospitalization rate? Disabil Health J 11(2):306–309. https://doi.org/10.1016/j.dhjo.2017.09.003

Posset R, Garbade SF, Boy N, Burlina AB, Dionisi-Vici C, Dobbelaere D, Garcia-Cazorla A, de Lonlay P, Teles EL, Vara R, Mew NA, Batshaw ML, Baumgartner MR, McCandless SE, Seminara J, Summar M, Hoffmann GF, Kölker S, Burgard P, Additional individual contributors of the UCDC and the E‐IMD consortium, Berry SA, Burrage L, Coughlin C, Diaz GA, Gallagher RC, Gropman A, Harding CO, Lee B, le Mons C, Lawrence Merritt J II, Nagamani SCS, Schulze A, Stricker T, Tuchman M, Waisbren S, WeisfeldAdams J, Wong D, Yudkoff M, Arnoux JB, Bari&cacute I, Bosch AM, Chabrol B, Chakrapani A, CortèsSaladefont E, Couce ML, Eyskens F, Laet C, Meirleir L, Freisinger P, Gleich F, Grünewald S, Häberle J, Hwu WL, Jalan A, Karall D, Lindner M, Lund AM, Martinelli D, Murphy E, Mühlhausen C, Olivieri G, Ottolenghi C, Rodrigues E, Rubert L, Sarajlija A, Schiff M, Sokal E, SykutCegielska J, Walter JH, Williams M, Zeman J (2019) Transatlantic combined and comparative data analysis of 1095 patients with urea cycle disorders-a successful strategy for clinical research of rare diseases. J Inherit Metab Dis 42(1):93–106. https://doi.org/10.1002/jimd.12031

RD-ACTION: Data and policies for rare diseases (n.d.) Workpackage 5: Steering, maintaining and promoting the adoption of Orphacodes across MS. Available at: http://www.rd-action.eu/workpackage/workpackage-5/

Rotter T, Kinsman L, James E, Machotta A, Steyerberg EW (2012) The quality of the evidence base for clinical pathway effectiveness: room for improvement in the design of evaluation trials. BMC Med Res Methodol 12:80. https://doi.org/10.1186/1471-2288-12-80

Stordeur S, Vrijens F, Leroy R (2016) Reference centres for adults with rare and complex cancers - policy recommendations to improve the organisation of care in Belgium. Rev Epidemiol Sante Publique 64(1):1–6. https://doi.org/10.1016/j.respe.2015.11.006

Taruscio D, Floridia G, Salvatore M, Groft SC, Gahl WA (2017) Undiagnosed diseases: Italy-US collaboration and international efforts to tackle rare and common diseases lacking a diagnosis. Adv Exp Med Biol 1031:25–38. https://doi.org/10.1007/978-3-319-67144-4_2

The European Rare Kidney Disease Network (ERKNet) (n.d.) Available at: https://www.erknet.org/index.php?id=home

Tumienė B, Maver A, Writzl K, Hodžić A, Čuturilo G, Kuzmanić-Šamija R, Čulić V, Peterlin B (2018) Diagnostic exome sequencing of syndromic epilepsy patients in clinical practice. Clin Genet 93(5):1057–1062. https://doi.org/10.1111/cge.13203

Vandeborne L, van Overbeeke E, Dooms M, De Beleyr B, Huys I (2019) Information needs of physicians regarding the diagnosis of rare diseases: a questionnaire-based study in Belgium. Orphanet J Rare Dis 14(1):99. https://doi.org/10.1186/s13023-019-1075-8

Vos JR, Giepmans L, Röhl C, Geverink N, Hoogerbrugge N (2019) ERN GENTURIS. Boosting care and knowledge about hereditary cancer: European Reference Network on Genetic Tumour Risk Syndromes. Familial Cancer 18(2):281–284. https://doi.org/10.1007/s10689-018-0110-6

Webb-Robertson BM, Stratton KG, Kyle JE et al (2020) Statistically driven metabolite and lipid profiling of patients from the undiagnosed diseases network. Anal Chem 92(2):1796–1803. https://doi.org/10.1021/acs.analchem.9b03522