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Clinical and Health-related Outcome of rFVIIIFc Prophylaxis

Conditions
Hemophilia A
Registration Number
NCT04583930
Lead Sponsor
Prof. Dr. Dr. Thomas Hilberg
Brief Summary

Current standard therapy for patients with haemophilia (PwH) in the prevention of bleeding episodes is a prophylactic intravenous treatment with recombinant coagulation factor (F) VIII (Haemophilia A) or rather FIX (Haemophilia B) two to three times weekly. With the development of recombinant factor VIII Fc fusion protein (rFVIIIFc) the conventional routine prophylaxis regime is complemented by an extended half-life (EHL) factor replacement prophylaxis with the potential of improved bleed prevention and reduced injection frequency at similar factor consumption. Aim of this longitudinal multicentre study is to evaluate the influence of an EHL factor replacement regime with rFVIIIFc on haemophilic specific parameters (annual bleeding rate, bleeding localisation), joint status, pain, functional parameters, treatment adherence and health-related quality of life in PwH A.

Detailed Description

Not available

Recruitment & Eligibility

Status
UNKNOWN
Sex
Male
Target Recruitment
48
Inclusion Criteria
  • Patients suffering from moderate to severe haemophilia A
  • Age ≥ 18-years
  • Treatment with FVIII prophylaxis
  • Submitted written informed consent
Exclusion Criteria

Patients suffering from other bleeding diseases

  • Patients with inhibitors
  • Patients without written informed consent
  • Age < 18-year-old
  • Any surgeries up to 6 months before the examination date
  • Suffering from different rheumatologic diseases like M. Bechterew, Psoriasis or other local or generalized joint infections (Borreliosis, septic arthritis)

Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
total annual bleeding rateone year

number of total bleedings

orthopaedic joint status (Haemophilia health joint score)one year

The clinical joint status will be examined in all patients with haemophilia by the World Federation Joint Examination Score and Haemophilia Joint Health Score. Higher score points imply an increased deficit in the functional and structural joint status as a sign of more pronounced haemophilic arthropathy, with a maximum possible value of 124 (no deficits = 0).

pressure pain thresholdsone year

physiological Parameter in Newton

Secondary Outcome Measures
NameTimeMethod
spontaneous joint ABRone year

number of total joint bleedings

subjective quality of life (SF-36)two years

The standarized questionnaire SF-36 includes a physical and psychological score. The higher the score, the higher the physical or psychological quality of life. A higher score implies a better physical and mental quality of life

subjective physical performance (HEP-Test-Q)two years

The final version of the standarized questionnaire consists of 25 items pertaining to the domains 'mobility', 'strength \& coordination', 'endurance' and 'body perception'. The response options were a five-point Likert scale (ranging from 1 = never to 5 = always). Some of the items had to be re-coded; subscales and the total score were transformed to a scale of 0-100 with high scores indicating better physical performance

self-perceived functional abilities (Haemophilia Activities List)two years

The Haemophilia Activities List measures the impact of hemophilia on self-perceived functional abilities in adults. It contains 42 multiple choice questions in seven domains: Lying/sitting/kneeling/standing (8 items), Functions of the legs (9 items), Functions of the arms (4 items), Use of transportation (3 items), Self-care (5 items), Household tasks (6 items), Leisure activities and sports (7 items)

Trial Locations

Locations (2)

Department of Sports Medicine

🇩🇪

Wuppertal, Northwest, Germany

Department of Sports Medicine, University of Wuppertal

🇩🇪

Wuppertal, North Rhine-Westphalia, Germany

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