LETTERS TO THE EDITORS
inhibitor development subsequent to factor exposure [10,11]. Our case report clearly shows that EVAR can safely be conducted in haemophilic patients by means of perioperative factor replacement. The choice of this procedure has allowed us to significantly reduce both the haemorrhagic risk associated with the procedure, as well as the cost related to clotting factor replacement therapy. In our opinion, EVAR should be considered as first-line therapy in patients with clinically relevant haemorrhagic disorders. Intraoperative anticoagulant treatment and long-term antiplatelet
References 1 Franchini M, Mannucci PM. Past, present and future of hemophilia: a narrative review. Orphanet J Rare Dis. 2012; 7: 24. 2 Jacquemin M, Vantomme V, Buhot C et al. CD4+ T-Cell clones specific for wild-type factor VIII: a molecular machanism responsible for higher incidence of inhibitor formation in mild/moderate hemophilia A. Blood. 2003;101: 1351–8. 3 Maleux G, Koolen M, Heye S. Complications after endovascular aneurysm repair. Semin Intervent Radiol 2009; 26: 3–9. 4 Marrocco-Trischitta MM, Melissano G, Castellano R et al. Endovascular abdominal aortic aneurysm repair in a patient with
5
6
7
8
agents are population.
not
contraindicated
in
this
e235
patient
Acknowledgement The authors gratefully acknowledge Dr. Hasan Catovic from CSL Behring for his assistance in the preparation of the manuscript.
Disclosures The authors stated that they had no interests which might be perceived as posing a conflict or bias.
severe hemophilia B. J Endovasc Ther. 2009; 16: 120–3. Kobayashi M, Matsushita M, Nishikimi N et al. Treatment for abdominal aortic aneurysm in a patient with hemophilia A: a case report and review of the literature. J Vasc Surg 1997; 25: 945–8. Brown B, Steed DL, Webster MW et al. General surgery in adult hemophiliacs. Surgery 1986; 99: 154–9. Bergqvist D, Thimberg L, Bergentz SE et al. Abdominal aortic aneurysm surgery in a hemophiliac. Vasa 1985; 14: 394–7. Gula G, Frezza G. Successful surgery in a patient with haemophilia B: report of case with abdominal aneurysm. Vasc Surg. 1973; 7: 183–7.
9 Maurel B, Bartoli MA, Jean-Baptiste E et al. Perioperative evaluation of iliac ZBIS branch devices: a French multicenter study. Ann Vasc Surg 2013; 2: 131–8. 10 Darby SC, Keeling DM, Spooner RJ et al. The incidence of factor VIII and factor IX inhibitors in the hemophilia population of the UK and their effect on subsequent mortality, 1977-99. J Thromb Haemost 2004; 2: 1047–54. 11 Eckhardt CL, Menke LA, van Ommen CH et al. Intensive peri-operative use of factor VIII and the Arg593–>Cys mutation are risk factors for inhibitor development in mild/moderate hemophilia A. J Thromb Haemost 2009; 7(Suppl 6): 930– 7.
Comment on: Khair K. Compliance, concordance and adherence: what are we talking about? Haemophilia Sept 2014;20(5):601–3. N . A . D U N C A N , C . P . R O B E R S O N and A . D . S H A P I R O Indiana Hemophilia and Thrombosis Center, Inc, Indianapolis, Indiana
“I have been struck again and again by how important measurement is to improving the human condition.” - Bill Gates Kate Khair recently published an interesting commentary proposing a shift from measuring treatment adherence to encouraging concordance, defined in the article as “an agreement between the patient and the provider about how best the patient can manage their treatment.” Khair suggests that if tools designed to Correspondence: Natalie A Duncan, Indiana Hemophilia and Thrombosis Center, Inc, 8326 Naab Road, Indianapolis, IN 46260. Tel.: 317 871 0011; fax: 317 871 0010; e-mail: [email protected] Accepted after revision 16 January 2015 © 2015 John Wiley & Sons Ltd
measure adherence such as the VERITAS-Pro do not actually improve adherence, we should rather focus our efforts on supporting persons with haemophilia (PWH) in their lifelong need to self-administer intravenous therapy. As the authors of the Validated Hemophilia Regimen Treatment Adherence Scales (VERITAS-Pro and VERITAS-PRN) [1,2], we would like to respond to Dr. Khair’s article. We agree and believe that the prevailing approach to care for PWH is in agreement with Khair’s sentiment regarding the involvement of patients in making treatment decisions, individualizing tailored treatment regimens, asking patients for their input on treatment adherence and encouraging age and condition-matched mentorship among patients. Khair offers the specific example of a patient engaged Haemophilia (2015), 21, e223--e259
e236
LETTERS TO THE EDITORS
in evening shift work, for whom the common recommendation to infuse prophylactically in the morning [3–5] would be inappropriate, to demonstrate the shortcomings of measuring adherence. This, however, is predicated on the notion that adherence is singularly focused on the administration of clotting factor at a specific time that is universally applicable to all patients. Although the VERITAS-Pro asks if infusions were completed in the morning, this could be interpreted as at the agreed upon time between patient and provider without compromise of the scale’s validation. In fact, we believe that the prevailing paradigm of prescribing treatment for PWH is based on treatment concordance. It is only after an agreed upon treatment plan has been established that it becomes important to monitor adherence objectively. The inability to follow this agreed upon treatment plan is what can accurately be labelled as non-adherence. Khair is correct, if we as clinicians have non-adherent PWH, we are also to blame. With validated tools such as the VERITASPro and VERITAS-PRN, clinicians are able to identify specific challenges and behaviours contributing to a patient’s non-adherence (such as difficultly remembering, planning or communicating with his haemophilia centre) and are compelled to discuss them and support patients in mutually agreed upon terms to overcome them. Describing a PWH as “non-adherent” should not be viewed as a pejorative stigma; it should be considered an opportunity to support patients in self-management. Khair’s article appears to create a dichotomy between promoting adherence and its measurement, and providing patient-centred care in which the patient’s needs, preferences and experiences are taken into account. Khair states that physicians interested in adherence tend to label patients who deviate from treatment recommendations as “non-adherent” and often admonish them for not doing as they have been asked. We would hesitate to characterize the assessment of adherence as causative of this behaviour,
References 1 Duncan NA, Kronenberger WG, Roberson CP, Shapiro AD. VERITAS-PRN: a new measure of adherence to episodic treatment regimens in haemophilia. Haemophilia 2010; 16: 47–53. 2 Duncan NA, Kronenberger WG, Roberson CP, Shapiro AD. VERITAS-Pro: a new measure of adherence to prophylactic regimens in haemophilia. Haemophilia 2010; 16: 247–55. 3 Collins PW, Bjorkman S, Fischer K et al. Factor VIII requirement to maintain a target plasma level in the prophylactic treatment of
Haemophilia (2015), 21, e223--e259
rather we believe the VERITAS tools support communication between providers and patients and assist both parties in understanding adherence and identification of specific modifiable variables [1,2]. Khair poses the questions, “Does the VERITAS-Pro actually improve adherence or is it ‘just’ a tool for measuring adherence? If it does not improve adherence, what can we do to support persons with haemophilia in their lifelong need to self-administer intravenous therapy?”[6] The VERITAS tools alone do not, and were never designed to in and of themselves, improve adherence. The VERITAS scales are instruments of measurement; they are not interventions. Interventions come after use of an assessment tool to address identified areas where behaviour may be modified. Interventions are developed by knowledgeable staff and vary based on the identified area(s). These interventions are likely highly individualized for each patient. The VERITAS tools can then be used to assess specific interventions. We only know what works to support PWH when we have a means to measure pre- and post-intervention adherence. The possible uses for the tools going forward, both in clinic and in research, are wide ranging. Publications have utilized these scales to evaluate adherence and quality of life [7] as well as pain levels [8]. In addition to their increasing use in research, several haemophilia centres have incorporated the VERITAS tools into routine clinical practice. It is our hope that clinicians using these tools will continue to recognize the importance of practicing patient-centred care as described by Khair while also emphasizing the importance of measuring adherence to the agreed upon treatment plan.
Disclosures The authors state that they had no interests which might be perceived as posing a conflict or bias.
severe hemophilia A: influences of variance in pharmacokinetics and treatment regimens. J Thromb Haemost 2010; 8: 269–75. € 4 Fischer K, Collins P, BjORkman S et al. Trends in bleeding patterns during prophylaxis for severe haemophilia: observations from a series of prospective clinical trials. Haemophilia 2011; 17: 433–8. 5 Yee TT, Beeton K, Griffioen A et al. Experience of prophylaxis treatment in children with severe haemophilia. Haemophilia 2002; 8: 76–82. 6 Khair K. Compliance, concordance and adherence: what are we talking about? Haemophilia 2014; 20: 601–3.
7 Duncan N, Shapiro A, Ye X, Epstein J, Luo MP. Treatment patterns, health-related quality of life and adherence to prophylaxis among haemophilia A patients in the United States. Haemophilia 2012; 18: 760– 5. 8 McLaughlin JM, Witkop ML, Lambing A, Anderson TL, Munn J, Tortella B. Better adherence to prescribed treatment regimen is related to less chronic pain among adolescents and young adults with moderate or severe haemophilia. Haemophilia 2014; 20: 506–12.
© 2015 John Wiley & Sons Ltd
Copyright of Haemophilia is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
inhibitor development subsequent to factor exposure [10,11]. Our case report clearly shows that EVAR can safely be conducted in haemophilic patients by means of perioperative factor replacement. The choice of this procedure has allowed us to significantly reduce both the haemorrhagic risk associated with the procedure, as well as the cost related to clotting factor replacement therapy. In our opinion, EVAR should be considered as first-line therapy in patients with clinically relevant haemorrhagic disorders. Intraoperative anticoagulant treatment and long-term antiplatelet
References 1 Franchini M, Mannucci PM. Past, present and future of hemophilia: a narrative review. Orphanet J Rare Dis. 2012; 7: 24. 2 Jacquemin M, Vantomme V, Buhot C et al. CD4+ T-Cell clones specific for wild-type factor VIII: a molecular machanism responsible for higher incidence of inhibitor formation in mild/moderate hemophilia A. Blood. 2003;101: 1351–8. 3 Maleux G, Koolen M, Heye S. Complications after endovascular aneurysm repair. Semin Intervent Radiol 2009; 26: 3–9. 4 Marrocco-Trischitta MM, Melissano G, Castellano R et al. Endovascular abdominal aortic aneurysm repair in a patient with
5
6
7
8
agents are population.
not
contraindicated
in
this
e235
patient
Acknowledgement The authors gratefully acknowledge Dr. Hasan Catovic from CSL Behring for his assistance in the preparation of the manuscript.
Disclosures The authors stated that they had no interests which might be perceived as posing a conflict or bias.
severe hemophilia B. J Endovasc Ther. 2009; 16: 120–3. Kobayashi M, Matsushita M, Nishikimi N et al. Treatment for abdominal aortic aneurysm in a patient with hemophilia A: a case report and review of the literature. J Vasc Surg 1997; 25: 945–8. Brown B, Steed DL, Webster MW et al. General surgery in adult hemophiliacs. Surgery 1986; 99: 154–9. Bergqvist D, Thimberg L, Bergentz SE et al. Abdominal aortic aneurysm surgery in a hemophiliac. Vasa 1985; 14: 394–7. Gula G, Frezza G. Successful surgery in a patient with haemophilia B: report of case with abdominal aneurysm. Vasc Surg. 1973; 7: 183–7.
9 Maurel B, Bartoli MA, Jean-Baptiste E et al. Perioperative evaluation of iliac ZBIS branch devices: a French multicenter study. Ann Vasc Surg 2013; 2: 131–8. 10 Darby SC, Keeling DM, Spooner RJ et al. The incidence of factor VIII and factor IX inhibitors in the hemophilia population of the UK and their effect on subsequent mortality, 1977-99. J Thromb Haemost 2004; 2: 1047–54. 11 Eckhardt CL, Menke LA, van Ommen CH et al. Intensive peri-operative use of factor VIII and the Arg593–>Cys mutation are risk factors for inhibitor development in mild/moderate hemophilia A. J Thromb Haemost 2009; 7(Suppl 6): 930– 7.
Comment on: Khair K. Compliance, concordance and adherence: what are we talking about? Haemophilia Sept 2014;20(5):601–3. N . A . D U N C A N , C . P . R O B E R S O N and A . D . S H A P I R O Indiana Hemophilia and Thrombosis Center, Inc, Indianapolis, Indiana
“I have been struck again and again by how important measurement is to improving the human condition.” - Bill Gates Kate Khair recently published an interesting commentary proposing a shift from measuring treatment adherence to encouraging concordance, defined in the article as “an agreement between the patient and the provider about how best the patient can manage their treatment.” Khair suggests that if tools designed to Correspondence: Natalie A Duncan, Indiana Hemophilia and Thrombosis Center, Inc, 8326 Naab Road, Indianapolis, IN 46260. Tel.: 317 871 0011; fax: 317 871 0010; e-mail: [email protected] Accepted after revision 16 January 2015 © 2015 John Wiley & Sons Ltd
measure adherence such as the VERITAS-Pro do not actually improve adherence, we should rather focus our efforts on supporting persons with haemophilia (PWH) in their lifelong need to self-administer intravenous therapy. As the authors of the Validated Hemophilia Regimen Treatment Adherence Scales (VERITAS-Pro and VERITAS-PRN) [1,2], we would like to respond to Dr. Khair’s article. We agree and believe that the prevailing approach to care for PWH is in agreement with Khair’s sentiment regarding the involvement of patients in making treatment decisions, individualizing tailored treatment regimens, asking patients for their input on treatment adherence and encouraging age and condition-matched mentorship among patients. Khair offers the specific example of a patient engaged Haemophilia (2015), 21, e223--e259
e236
LETTERS TO THE EDITORS
in evening shift work, for whom the common recommendation to infuse prophylactically in the morning [3–5] would be inappropriate, to demonstrate the shortcomings of measuring adherence. This, however, is predicated on the notion that adherence is singularly focused on the administration of clotting factor at a specific time that is universally applicable to all patients. Although the VERITAS-Pro asks if infusions were completed in the morning, this could be interpreted as at the agreed upon time between patient and provider without compromise of the scale’s validation. In fact, we believe that the prevailing paradigm of prescribing treatment for PWH is based on treatment concordance. It is only after an agreed upon treatment plan has been established that it becomes important to monitor adherence objectively. The inability to follow this agreed upon treatment plan is what can accurately be labelled as non-adherence. Khair is correct, if we as clinicians have non-adherent PWH, we are also to blame. With validated tools such as the VERITASPro and VERITAS-PRN, clinicians are able to identify specific challenges and behaviours contributing to a patient’s non-adherence (such as difficultly remembering, planning or communicating with his haemophilia centre) and are compelled to discuss them and support patients in mutually agreed upon terms to overcome them. Describing a PWH as “non-adherent” should not be viewed as a pejorative stigma; it should be considered an opportunity to support patients in self-management. Khair’s article appears to create a dichotomy between promoting adherence and its measurement, and providing patient-centred care in which the patient’s needs, preferences and experiences are taken into account. Khair states that physicians interested in adherence tend to label patients who deviate from treatment recommendations as “non-adherent” and often admonish them for not doing as they have been asked. We would hesitate to characterize the assessment of adherence as causative of this behaviour,
References 1 Duncan NA, Kronenberger WG, Roberson CP, Shapiro AD. VERITAS-PRN: a new measure of adherence to episodic treatment regimens in haemophilia. Haemophilia 2010; 16: 47–53. 2 Duncan NA, Kronenberger WG, Roberson CP, Shapiro AD. VERITAS-Pro: a new measure of adherence to prophylactic regimens in haemophilia. Haemophilia 2010; 16: 247–55. 3 Collins PW, Bjorkman S, Fischer K et al. Factor VIII requirement to maintain a target plasma level in the prophylactic treatment of
Haemophilia (2015), 21, e223--e259
rather we believe the VERITAS tools support communication between providers and patients and assist both parties in understanding adherence and identification of specific modifiable variables [1,2]. Khair poses the questions, “Does the VERITAS-Pro actually improve adherence or is it ‘just’ a tool for measuring adherence? If it does not improve adherence, what can we do to support persons with haemophilia in their lifelong need to self-administer intravenous therapy?”[6] The VERITAS tools alone do not, and were never designed to in and of themselves, improve adherence. The VERITAS scales are instruments of measurement; they are not interventions. Interventions come after use of an assessment tool to address identified areas where behaviour may be modified. Interventions are developed by knowledgeable staff and vary based on the identified area(s). These interventions are likely highly individualized for each patient. The VERITAS tools can then be used to assess specific interventions. We only know what works to support PWH when we have a means to measure pre- and post-intervention adherence. The possible uses for the tools going forward, both in clinic and in research, are wide ranging. Publications have utilized these scales to evaluate adherence and quality of life [7] as well as pain levels [8]. In addition to their increasing use in research, several haemophilia centres have incorporated the VERITAS tools into routine clinical practice. It is our hope that clinicians using these tools will continue to recognize the importance of practicing patient-centred care as described by Khair while also emphasizing the importance of measuring adherence to the agreed upon treatment plan.
Disclosures The authors state that they had no interests which might be perceived as posing a conflict or bias.
severe hemophilia A: influences of variance in pharmacokinetics and treatment regimens. J Thromb Haemost 2010; 8: 269–75. € 4 Fischer K, Collins P, BjORkman S et al. Trends in bleeding patterns during prophylaxis for severe haemophilia: observations from a series of prospective clinical trials. Haemophilia 2011; 17: 433–8. 5 Yee TT, Beeton K, Griffioen A et al. Experience of prophylaxis treatment in children with severe haemophilia. Haemophilia 2002; 8: 76–82. 6 Khair K. Compliance, concordance and adherence: what are we talking about? Haemophilia 2014; 20: 601–3.
7 Duncan N, Shapiro A, Ye X, Epstein J, Luo MP. Treatment patterns, health-related quality of life and adherence to prophylaxis among haemophilia A patients in the United States. Haemophilia 2012; 18: 760– 5. 8 McLaughlin JM, Witkop ML, Lambing A, Anderson TL, Munn J, Tortella B. Better adherence to prescribed treatment regimen is related to less chronic pain among adolescents and young adults with moderate or severe haemophilia. Haemophilia 2014; 20: 506–12.
© 2015 John Wiley & Sons Ltd
Copyright of Haemophilia is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
