Year 2020,
Volume: 5 Issue: 3, 194 - 205, 25.12.2020
Zühre Kaya
,
Serap Kirkiz
,
Meryem Albayrak
,
Esra Güney
,
Selin Aytac
Namık Yaşar Özbek
Supporting Institution
Gazi Üniversitesi Tıp Fakültesi
Thanks
Emekleri için Prof Dr Türkiz Gürsel e teşekkürler...
References
- References
[1]. Coppola, A., et al., Prophylaxis in children with hemophilia: evidence based achievements, old and new challenges. Semin Thromb Hemost. 38,79-94, 2012.
- [2]. Schrijvers, L.H., Schuurmans, M.J., Fischer, K. Promoting self-management and adherence during prophylaxis: evidence-based recommendations for haemophilia professionals. Haemophilia. 22,499-506, 2016.
- [3].Garcia-Dasi, M., et al., Adherence to prophylaxis and quality of life in children and adolescents with severe hemophilia A. Haemophilia. 21,458-464, 2015.
- [4].Krishnan, S., et al., Adherence to prophylaxis is associated with better outcomes in moderate and severe hemophilia: results of a patient survey. Haemophilia. 21,64-70, 2015.
- [5].Lock, J., et al., Adherence to treatment in a Western European paediatric population with hemophilia:reliability and validity of the VERITAS-Pro scale. Haemophilia. 20,616-623, 2014.
- [6]. Megias-Vericat, J.E., et al., Bayesian pharmacokinetic guided prophylaxis with recombinant factor VIII in severe or moderate hemophilia A. Thromb Res. 174,151-162, 2019.
- [7].Nagao, A., et al., Clinical outcome in hemophilia A patients undergoing tailoring of prophylaxis based on population based pharmacokinetic dosing. Thromb Res. 173,79-84, 2019.
- [8].Pasca, S., Milan, M., Sarolo, L., Zanon, E. PK driven prophylaxis versus standard prophylaxis: When a tailored treatment may be a real and achievable cost saving approach in children with severe hemophilia A. Thromb Res. 157,58-63, 2017.
- [9].von der Lippe, C., et al. Treatment of hemophilia: A qualitative study of mothers’ perspectives. Pediatr Blood Cancer. 64,121-127, 2017.
- [10].Bullinger, M., et al., Challenges of patient-reported outcome assessment in hemophilia care-a state of the art review. Value Health. 12,808-820, 2009.
- [11].Varaklioti, A., et al., Health-related quality of life and association with arthropathy in Greek patients with hemophilia. Clin Appl Thromb Hemost. 24,815-821, 2018.
- [12].Mercan, A., et al., Hemophilia-Specific Quality of Life Index (Haemo-QoL and Haem-A-QoL questionnaires) of children and adults: result of a single center from Turkey. Pediatr Hematol Oncol. 27,449-461,2010.
- [13].Abali, O., et al., An examination of the symptoms of anxiety and parental attitude in children with hemophilia. Turk J Med Sci. 44,1087-1090, 2014.
- [14].Evans, M., Cottrell, D., Shiach, C. Emotional and behavioural problems and family functioning in children with haemophilia: a cross sectional survey. Haemophilia. 6,682-687, 2000.
- [15].Pinto, P.R., et al., Emotional distress in haemophilia: Factors associated with the presence of anxiety and depression symptoms among adults. Haemophilia. 24,e344-e353, 2018.
- [16].Ware, J.E., Jr, Sherbourne, C.D. The MOS 36-item short-form health survey (SF 36). I. Conceptual framework and item selection. Med Care. 30:473-483, 1992.
- [17].Julian, L.J., Measures of anxiety. State-Trait Anxiety Inventory (STAI), Beck Anxiety Inventory (BAI), and Hospital Anxiety and Depression Scale-Anxiety (HADS-A). Arthritis Care Res. 63,S467-472, 2011.
- [18].Schrijvers, L.H., et al., Unraveling adherence to prophylaxis in haemophilia: a patients’ perspective. Haemophilia 21,612-621, 2015.
- [19].Kaya, Z., et al., Succesful total hip replacement with sequential administration of bypassing agents in an adolescent boy with hemophilia A and high titer inhibitors. Blood Coag Fibrinolysis 28,419-422, 2017.
- [20].Lehmeier, A., et al., Participation in physical and sportive activities among adult Turkish people with hemophilia A: A single center experience. Turk J Haematol. 35,81-82, 2018.
[21].Hanley, J., et al., Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia. Haemophilia 23,511-520, 2017.
- [22]. Geerts, E., van de Wiel, H., Tamminga, R. A pilot study on the effects of the transition of pediatric to adult health care in patients with haemophilia and in their parents: patient and parent worries, parental illness-related distress and health related quality of life. Haemophilia. 14,1007-1013, 2008.
- [23].Ugur, MC., Kavakli, K. Prospective evaluation of non-compliant severe haemophilia. patients. Turk J Hematol. 36,137-138, 2019.
- [24].Mannucci, P.M., et al., How I treat age-related morbitidies in elderly persons with hemophilia. Blood. 114:5256-5263, 2009.
- [25].Gringeri, A., von Mackensen, S. Quality of life in haemophilia. Haemophilia. 14 Suppl 3:19-25, 2008.
- [26].Lins, L., Carvalho, F.M. SF-36 total score as a single measure of health related quality of life: Scoping review. SAGE Open Med. 4: 2050312116671725, 2016.
ADHERENCE TO PROPHYLAXIS IN RELATION TO QUALITY OF LIFE AND ANXIETY LEVEL IN TURKISH PATIENTS WITH SEVERE HAEMOPHILIA A
Year 2020,
Volume: 5 Issue: 3, 194 - 205, 25.12.2020
Zühre Kaya
,
Serap Kirkiz
,
Meryem Albayrak
,
Esra Güney
,
Selin Aytac
Namık Yaşar Özbek
Abstract
Background: Bleeding increases anxiety and reduces quality of life (QoL) for patients with severe haemophilia A. This study investigated adherence to prophylaxis in relation to QoL and anxiety level in haemophilia A patients.
Methods: Forty-three patients with severe haemophilia A were stratified into three groups by age: 12 children aged 2-13 years, 17 adolescents aged 14-21 years, and 14 adults aged 21-65 years. Quality of life and anxiety level were assessed using the 36-item Short Form Health Survey (SF-36) and the State-Trait Anxiety Inventory (STAI), respectively. Regular prophylaxis (RP) and pharmacokinetic (PK)-guided prophylaxis were prescribed for 30 and 13 patients, respectively.
Results: All the children (n=12; nine RP, three PK-guided prophylaxis) and 10 (58.8%, PK-guided prophylaxis) of the adolescents were completely adherent to prophylaxis. Seven (41.2%) of the adolescents and all 14 adults (100%) were non-adherent to prophylaxis. Compared to findings for the adolescents, anxiety level was higher among the children’s parents and among the adults (p<0.05 for both). The QoL level was lower for the adults than for the parents of children and for the adolescents (p<0.05 for both).
Conclusion: Pharmacokinetic-guided prophylaxis for adults and home treatment for children in haemophilia A would enhance adherence to prophylaxis, increase QoL and reduce anxiety levels.
References
- References
[1]. Coppola, A., et al., Prophylaxis in children with hemophilia: evidence based achievements, old and new challenges. Semin Thromb Hemost. 38,79-94, 2012.
- [2]. Schrijvers, L.H., Schuurmans, M.J., Fischer, K. Promoting self-management and adherence during prophylaxis: evidence-based recommendations for haemophilia professionals. Haemophilia. 22,499-506, 2016.
- [3].Garcia-Dasi, M., et al., Adherence to prophylaxis and quality of life in children and adolescents with severe hemophilia A. Haemophilia. 21,458-464, 2015.
- [4].Krishnan, S., et al., Adherence to prophylaxis is associated with better outcomes in moderate and severe hemophilia: results of a patient survey. Haemophilia. 21,64-70, 2015.
- [5].Lock, J., et al., Adherence to treatment in a Western European paediatric population with hemophilia:reliability and validity of the VERITAS-Pro scale. Haemophilia. 20,616-623, 2014.
- [6]. Megias-Vericat, J.E., et al., Bayesian pharmacokinetic guided prophylaxis with recombinant factor VIII in severe or moderate hemophilia A. Thromb Res. 174,151-162, 2019.
- [7].Nagao, A., et al., Clinical outcome in hemophilia A patients undergoing tailoring of prophylaxis based on population based pharmacokinetic dosing. Thromb Res. 173,79-84, 2019.
- [8].Pasca, S., Milan, M., Sarolo, L., Zanon, E. PK driven prophylaxis versus standard prophylaxis: When a tailored treatment may be a real and achievable cost saving approach in children with severe hemophilia A. Thromb Res. 157,58-63, 2017.
- [9].von der Lippe, C., et al. Treatment of hemophilia: A qualitative study of mothers’ perspectives. Pediatr Blood Cancer. 64,121-127, 2017.
- [10].Bullinger, M., et al., Challenges of patient-reported outcome assessment in hemophilia care-a state of the art review. Value Health. 12,808-820, 2009.
- [11].Varaklioti, A., et al., Health-related quality of life and association with arthropathy in Greek patients with hemophilia. Clin Appl Thromb Hemost. 24,815-821, 2018.
- [12].Mercan, A., et al., Hemophilia-Specific Quality of Life Index (Haemo-QoL and Haem-A-QoL questionnaires) of children and adults: result of a single center from Turkey. Pediatr Hematol Oncol. 27,449-461,2010.
- [13].Abali, O., et al., An examination of the symptoms of anxiety and parental attitude in children with hemophilia. Turk J Med Sci. 44,1087-1090, 2014.
- [14].Evans, M., Cottrell, D., Shiach, C. Emotional and behavioural problems and family functioning in children with haemophilia: a cross sectional survey. Haemophilia. 6,682-687, 2000.
- [15].Pinto, P.R., et al., Emotional distress in haemophilia: Factors associated with the presence of anxiety and depression symptoms among adults. Haemophilia. 24,e344-e353, 2018.
- [16].Ware, J.E., Jr, Sherbourne, C.D. The MOS 36-item short-form health survey (SF 36). I. Conceptual framework and item selection. Med Care. 30:473-483, 1992.
- [17].Julian, L.J., Measures of anxiety. State-Trait Anxiety Inventory (STAI), Beck Anxiety Inventory (BAI), and Hospital Anxiety and Depression Scale-Anxiety (HADS-A). Arthritis Care Res. 63,S467-472, 2011.
- [18].Schrijvers, L.H., et al., Unraveling adherence to prophylaxis in haemophilia: a patients’ perspective. Haemophilia 21,612-621, 2015.
- [19].Kaya, Z., et al., Succesful total hip replacement with sequential administration of bypassing agents in an adolescent boy with hemophilia A and high titer inhibitors. Blood Coag Fibrinolysis 28,419-422, 2017.
- [20].Lehmeier, A., et al., Participation in physical and sportive activities among adult Turkish people with hemophilia A: A single center experience. Turk J Haematol. 35,81-82, 2018.
[21].Hanley, J., et al., Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia. Haemophilia 23,511-520, 2017.
- [22]. Geerts, E., van de Wiel, H., Tamminga, R. A pilot study on the effects of the transition of pediatric to adult health care in patients with haemophilia and in their parents: patient and parent worries, parental illness-related distress and health related quality of life. Haemophilia. 14,1007-1013, 2008.
- [23].Ugur, MC., Kavakli, K. Prospective evaluation of non-compliant severe haemophilia. patients. Turk J Hematol. 36,137-138, 2019.
- [24].Mannucci, P.M., et al., How I treat age-related morbitidies in elderly persons with hemophilia. Blood. 114:5256-5263, 2009.
- [25].Gringeri, A., von Mackensen, S. Quality of life in haemophilia. Haemophilia. 14 Suppl 3:19-25, 2008.
- [26].Lins, L., Carvalho, F.M. SF-36 total score as a single measure of health related quality of life: Scoping review. SAGE Open Med. 4: 2050312116671725, 2016.