title: The social burden and quality of life of patients with haemophilia in Italy.
abstract: BACKGROUND
  In Italy, the project on the social burden and quality of life (QoL) of patients with haemophilia investigates costs from a society perspective and provides an overview of their quality of life. Moreover, as life expectancy increased in recent years along with new treatment strategies implemented in the last decades, it analyses trends of costs other than drugs simulating impacts during patient whole life.
  
  
  METHODS
  We ran a web-based cross-sectional survey supported by the Italian Federation of Haemophilia Societies in recruiting patients with haemophilia and their caregivers. We developed a questionnaire to collect information on demographic characteristics, healthcare and social services consumption, formal and informal care utilisation, productivity loss and quality of life. In particular, quality of life was assessed through the EuroQoL tool. Last, we applied the illness cost method from a society perspective.
  
  
  RESULTS
  On average, quality of life is worse in adult patients compared to child and caregivers: more than 75% of adult patients declare physical problems, 43% of adult patients and 54% of their parents have anxiety problems. Assuming a society perspective, the estimated mean annual total cost per patient in 2012 is 117,732 €. Drugs represent 92% of total costs. Focusing on costs other than drugs, each additional point of EuroQoL tool implies a costs' reduction of 279 €. The impact of age varies across age groups: each added year implies a total decrease of costs up to 46.6 years old. Afterwards, every additional year increases costs.
  
  
  CONCLUSIONS
  Quality of life of patients with haemophilia and their caregivers improved and it influences positively on consumed resources and on their contribution to the social-economic system. Costs other than drugs for patients with haemophilia follow the same trends of general population.
authors.affiliation: Italian National Centre for Rare Diseases, National Institute of Health, Rome, Italy.
authors.forename: Yllka
authors.initials: Y
authors.lastname: Kodra
authors.affiliation: Centre for Research on Health and Social Care Management (CERGAS) Bocconi University, Milan, Italy.
authors.forename: Marianna
authors.initials: M
authors.lastname: Cavazza
authors.affiliation: Clinical Research Centre for Rare Disease, Mario Negri Institute, Ranica and Department of Medicine, Rare Disease Unit, Papa Giovanni XXIII Hospital, Bergamo, Italy.
authors.forename: Arrigo
authors.initials: A
authors.lastname: Schieppati
authors.affiliation: Italian National Centre for Rare Diseases, National Institute of Health, Rome, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: De Santis
authors.affiliation: Centre for Research on Health and Social Care Management (CERGAS) Bocconi University, Milan, Italy.
authors.forename: Patrizio
authors.initials: P
authors.lastname: Armeni
authors.affiliation: Italian Federation of Haemophilia Societies, Rome, Italy.
authors.forename: Romano
authors.initials: R
authors.lastname: Arcieri
authors.affiliation: National Blood Centre, National Institute of Health, Rome, Italy.
authors.forename: Gabriele
authors.initials: G
authors.lastname: Calizzani
authors.affiliation: Centre for Research on Health and Social Care Management (CERGAS) Bocconi University, Milan, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Fattore
authors.affiliation: Italian Federation of Haemophilia Societies, Medical Council, Rome, Italy.
authors.forename: Lamberto
authors.initials: L
authors.lastname: Manzoli
authors.affiliation: Italian Federation of Haemophilia Societies, Medical Council, Rome, Italy.
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Mantovani
authors.affiliation: Italian National Centre for Rare Diseases, National Institute of Health, Rome, Italy.
authors.forename: Domenica
authors.initials: D
authors.lastname: Taruscio
mesh_terms: D000328:Adult; D001007:Anxiety / Q000191:economics / Q000209:etiology / Q000628:therapy; D017281:Cost of Illness; D003365:Costs and Cost Analysis; D003430:Cross-Sectional Studies; D005260:Female; D006467:Hemophilia A / Q000150:complications / Q000191:economics* / Q000628:therapy*; D006801:Humans; D007558:Italy; D008297:Male; D011788:Quality of Life; D012959:Socioeconomic Factors