Author(s): E. Schaefer, Created: 2010/07/20, Reviewed by: I. Astigarraga, J. Donadieu, R. Haupt, M. Minkov, R. Price, last update: 2013/03/24
A reference network for Langerhans Cell Histiocytosis and associated syndromes.
A project for Langerhans cell histiocytosis and associated syndromes has received European funding and is supported by 30 project partners: physicians and representatives of patient associations involved in histiocytosis. Histiocytoses are very rare diseases affecting patients of all ages. Heterogeneous clinical presentations and unpredictable courses reach from involvement limited to skin or bone, with an excellent prognosis, to severe multi-system involvement associated with spontaneous poor outcome, requiring intensive cares. The heterogeneity of the disease necessitates a very particular competence from several medical specialists, whereas time and financial restrictions lead to difficulties in sharing acquired knowledge with patients and non-specialised treating physicians.
The associated project partners are physicians who have already a long commitment, both in the care of the patients and in the collaboration with patients’ associations. Based on their experience, the contents of the four core work packages were defined: medical guidelines and recommendations, online expert support, a web based data base, and a forum for patients’ exchange. These elements will be accessible through the Histio Net web portal as main dissemination instrument which will be a multilingual, secure, web-based portal. It will be an important contact point for everyone who is interested in LCH and associated syndromes. It will provide access to information, knowledge bases, and applications, for histiocytosis specialists, attending doctors, patients, and other people concerned with LCH and associated syndromes. The contents will be created in English and validated by the project partners. After this, they will be translated to many different languages. At present, translators for Arabic, Bulgarian, Dutch, French, German, Greek, Italian, Polish, Portuguese, Russian, Spanish, and Swedish have agreed to contribute. The existence of the web portal will be published in many medical societies in the different countries, including pediatricians and general practitioners – as the first contact point for each patient – as well as specialists with regard to the numerous involvements: dermatologists, endocrinologists, ENT-specialists, hematologists, hepatologists, internists, neurologists, neuro surgeons, oncologists, orthopedists, pneumologists, radiologists, and surgeons.
A common challenge in the field of rare diseases is the problem of knowledge acquisition and knowledge exchange. Histio Net wants to help solving this dilemma for LCH and related histiocytic syndromes. The web portal can be an operative instrument of exchange for reaching three important goals:
- A better care for isolated patients in EU: The availability of validated guidelines for physicians can improve diagnosis, treatment, and follow-up of patients concerned by LCH and associated syndromes.
- A better knowledge about the disease: The constitution of larger cohorts of patients at EU scale can improve the knowledge of the disease (e.g. natural history, risk factors for complications).
- A better exchange of newly acquired knowledge: The availability of communication tools can improve communication about histiocytoses at different international levels: among centers of reference of the different countries, between non-specialized physicians and centers of reference, between patients and centers of reference, among the patient associations of the different countries, and between patients and patient associations dealing in the patients’ language.