title: Adherence to prophylaxis and quality of life in children and adolescents with severe haemophilia A.
abstract: Treatment adherence in adolescents with chronic diseases is around 50%, and failure is more common in preventive therapy. In haemophilia, contradictory results are reported by the published studies. The objective of this study was to evaluate adherence with factor VIII (FVIII) prophylaxis in Spanish patients with severe haemophilia A between age 6 and 20 years. Data were collected retrosp-ectively in the previous 2 years. The primary endpoint was the absolute adherence index (AAI), and the endpoints were related to clinical status, age, prophylaxis regimen, responsibility for factor administration and quality of life (QoL), assessed by the Haemo-QoL questionnaires. A total of 78 patients from 14 Spanish hospitals were recruited. Adherence ranged between -64.4 and 66.7 (mean -3.08). No differences were observed between children and adolescents (7.11 vs. 6.39; P = 0.809). A statistically significant association (P < 0.010) between infra adherent group and target joint was found, as was a statistically significant difference (P < 0.010) between the number of bleeding episodes experienced by the adherent group (mean 1.4) and by infra adherents (mean 4.5). There was no significant difference between AAI and prophylactic regimen (6.35 vs. 6.96, P = 0.848), neither between AAI and the person responsible for factor administration (5.57 vs. 8.79, P = 0.326). The Haemo-QoL scores (8-12 years) were related to adherence level (P < 0.05). Adherence was approximately ideal and patients perceived a high QoL. Because of the repercussions for compliance, it is essential to work during puberty on emotional and self-acceptance aspects of the disease, as well as coping, and the patient's family, school and health team relationships.
authors: Hospital Universitario y Politécnico La Fe, Valencia, Spain.
authors: M
authors: M
authors: García-Dasí
authors: Hospital Universitario y Politécnico La Fe, Valencia, Spain.
authors: J A
authors: JA
authors: Aznar
authors: Hospital La Paz, Madrid, Spain.
authors: V
authors: V
authors: Jiménez-Yuste
authors: Hospital Vall d'Hebron, Barcelona, Spain.
authors: C
authors: C
authors: Altisent
authors: Hospital Universitario y Politécnico La Fe, Valencia, Spain.
authors: S
authors: S
authors: Bonanad
authors: Hospital Carlos Haya, Málaga, Spain.
authors: E
authors: E
authors: Mingot
authors: Hospital Miguel Servet, Zaragoza, Spain.
authors: F
authors: F
authors: Lucía
authors: Hospital Torrecárdenas, Almería, Spain.
authors: F
authors: F
authors: Giménez
authors: Hospital Juan Canalejo, A Coruña, Spain.
authors: M Fernanda
authors: MF
authors: López
authors: Hospital General de Alicante, Alicante, Spain.
authors: P
authors: P
authors: Marco
authors: Hospital Virgen del Rocío, Sevilla, Spain.
authors: R
authors: R
authors: Pérez
authors: Hospital Central de Asturias, Asturias, Spain.
authors: M Á
authors: MÁ
authors: Fernández
authors: Hospital Virgen del Camino, Pamplona, Spain.
authors: M J
authors: MJ
authors: Paloma
authors: Hospital Son Espases, Mallorca, Spain.
authors: B
authors: B
authors: Galmes
authors: Hospital General de Guadalajara, Guadalajara, Spain.
authors: S
authors: S
authors: Herrero
authors: Hospital de la Candelaria, Tenerife, Spain.
authors: J A
authors: JA
authors: García-Talavera
mesh_terms: D000293:Adolescent; D002648:Child; D003430:Cross-Sectional Studies; D005169:Factor VIII / Q000627:therapeutic use; D006467:Hemophilia A / Q000188:drug therapy / Q000473:pathology / Q000523:psychology*; D006801:Humans; D008297:Male; D010290:Parents / Q000523:psychology; D010349:Patient Compliance; D011788:Quality of Life; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D055815:Young Adult
chemical_list: D005169:Factor VIII
keywords: adherence; adolescents; children; haemophilia; prophylaxis; quality of life