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Dual Guidance Structure for Evaluation of Patients With Unclear Diagnosis in Centers for Rare Diseases

Not Applicable
Completed
Conditions
Rare Diseases
Orphan Diseases
Interventions
Other: dual expert guidance structure
Registration Number
NCT03563677
Lead Sponsor
Wuerzburg University Hospital
Brief Summary

In people suffering from a rare disease the diagnostic process and the confirmation of a final diagnosis is often ongoing for many years. Factors contributing to delayed diagnosis include the limited knowledge of health care professionals about rare diseases and their symptoms but also a psychiatric or psychosomatic (co-)morbidity obscuring the symptoms of the rare disease. The project ZSE-DUO will evaluate whether a combination of an expert in somatic medicine and a psychiatric/psychosomatic specialist will increase the rate of assured diagnoses in patients approaching a center of rare diseases (primary outcome), accelerate the process until a diagnosis is made, reduce the costs of diagnosing a patient, and lead to a higher satisfaction of patients and health care professionals. Furthermore, the project will evaluate whether the use of psychosomatic screening tools at registration of a patient in a center for rare diseases will help to guide the diagnostic process. Two cohorts of 682 patients each will be sequentially recruited over 9 plus 9 months: the Control group cohort (CG based on somatic expertise) and the Experimental group cohort (EG combined psychosomatic/somatic expertise Included will be persons from the age of at least 12 years presenting with symptoms and signs which are not explained by current diagnoses (as judged by the patient's primary care physician and a specialized physician at the center for rare diseases ZSE evaluating the medical records). Patients will be recruited from 11 German Centers for Rare Diseases associated with University hospitals in the cities of Aachen, Bochum, Frankfurt, Hannover, Magdeburg, Mainz, Münster, Regensburg, Tübingen, Ulm and Würzburg. Recruitment will be supported by a collaboration with the German patient organization representing many rare disease organizations ACHSE e.V. and a collaboration with the insurance companies Techniker Krankenkasse, IKK gesund plus and AOK Hessen who also provide data on costs of care. Data collection and analysis will be coordinated and performed by the Institute for Clinical Epidemiology and Biometry at the University of Würzburg, the Institute for Epidemiology, Social Medicine and Science of Health Care Systems in Hannover, and the Department of Medical Psychology in Hamburg. The project is funded by the Innovationsfond of the Federal Joint Committee in Germany.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
1379
Inclusion Criteria
  • first contact with the Center for Rare Diseases for unclear diagnosis
  • suspicion of a rare disease but no established diagnosis
  • attending the Center for Rare Diseases as an outpatient
  • written informed consent
Exclusion Criteria
  • age <12 years
  • incomplete medical records including summary letters, imaging studies, blood tests etc.
  • pre-diagnosed disease(s) explaining all symptoms

Study & Design

Study Type
INTERVENTIONAL
Study Design
SEQUENTIAL
Arm && Interventions
GroupInterventionDescription
New Innovative Caredual expert guidance structureThe innovative evaluation process includes the additional involvement of a psychiatrists/psychosomatic expert in all of the processes described for the usual care arm plus the option to use telemedicine in the process of evaluation in addition to outpatient and inpatient visits and to transfer the patient back into standard care (i.e., primary care physician, rehabilitation, psychological/psychosomatic specialized care, etc.)
Primary Outcome Measures
NameTimeMethod
Diagnoses made12 months after signing the consent form

The number of diagnoses explaining the symptomatology of the patient made during the evaluation process

Secondary Outcome Measures
NameTimeMethod
Time to diagnosis12 months after signing the consent form

The time it takes to make a diagnosis

Costs of the diagnostic processup to 12 months after signing the consent from

estimated costs from first contact with the center for rare diseases until a diagnosis explaining the symptoms has been established

Value of screening instruments for psychiatric-psychosomatic (co-)morbidities30 months after the project start (end of intervention period)

Performance of screening tools to identify patients with psychiatric-psychosomatic comorbidities against the judgement of a psychiatrist/psychosomatic expert seeing the patient (intervention group only)

Patient satisfaction with diagnostic process using ZUF-812 months after signing the consent form

The patients' satisfaction with the diagnostic process is assessed in the total sample with the questionnaire ZUF-8 (Fragebogen zur Patientenzufriedenheit - patient satisfaction questionnaire) and in a randomly selected subsample of about 40 patients by structured telephone interviews.

Patients' quality of life using EQ-5D and SF12 (or KIDSCREEN-10 for children)12 months after signing the consent from

Change in the patients' quality of life as assessed with the Quality of life questionnaires EQ-5D from the EuroQoL Group in all patients and Short Form 12 (SF-12) in patients 16 years and older. In patients younger than 16 years, the health-related quality of life questionnaire for children and adolescents KIDSCREEN-10 is used.

Physician satisfaction with new form of care using new questionnaire30 months after the project start (end of the intervention period)

The satisfaction of physicians working in the Centers fro Rare Diseases with the new form of care compared to standard care will be assessed by a newly developed questionnaire administered to all physicians involved in patient care in the 11 centers. For the development of the questionnaire, 3 focus groups of 10 physicians each will be questioned.

Trial Locations

Locations (10)

LWL-Universitätsklinikum der Ruhr-Universität

🇩🇪

Bochum, Germany

Center for Rare Diseases, University Hospital

🇩🇪

Ulm, Germany

Center for Rare Diseases ZESE, University Hospital

🇩🇪

Würzburg, Bavaria, Germany

Center for Rare Diseases ZSEA, University Hospital

🇩🇪

Aachen, Germany

Center for Rare Diseases, Hannover Medical School

🇩🇪

Hannover, Germany

Center for Rare Diseases MKSE, Otto von Guericke University

🇩🇪

Magdeburg, Germany

Center for Rare Diseases CeSER, St. Josef Hospital

🇩🇪

Bochum, Germany

Center for Rare Diseases FRZSE, UNiversity Hospital

🇩🇪

Frankfurt, Germany

Center for Rare Diseases, Medical Center

🇩🇪

Mainz, Germany

Center for Rare Diseases ZSER, University Hospital

🇩🇪

Regensburg, Germany

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