Haemophilia (2014), 20, 219–225
DOI: 10.1111/hae.12297
ORIGINAL ARTICLE Clinical haemophilia
What should men living with haemophilia need to know? The perspectives of Canadian men with haemophilia E. ARNOLD,* S. LANE,* K. E. WEBERT,†‡ A. CHAN,§ I. WALKER,* J. TUFTS,* S. RUBIN,¶ M - C . P O O N * * and N . M . H E D D L E * † † *Department of Medicine, McMaster University; †Canadian Blood Services; ‡Department of Pathology and Molecular Medicine, McMaster University; §Department of Pediatrics, McMaster University, Hamilton, ON; ¶Department of Medicine, Horizon Health Network, Moncton, NB; **Department of Medicine, Pediatrics and Oncology, University of Calgary, Calgary, AB; and ††Canadian Blood Services, Research and Development, Hamilton, ON, Canada
Summary. Haemophilia is an inherited bleeding disorder affecting approximately 3000 Canadian men (Walker 2012). To manage their disease effectively individuals must be knowledgeable about the disease, bleed prevention strategies, treatment approaches, and complications. Data on individuals’ knowledge levels are scarce. The availability of such data could lead to better educational strategies for disease management. The aim of this study was to determine current knowledge levels, needs and gaps among Canadian individuals with haemophilia to facilitate optimal disease management. A survey was disseminated to adult males with haemophilia at three Haemophilia Treatment Centres (HTCs) in Canada. Self-reported current knowledge levels and knowledge seeking were measured. Survey respondents reported highest levels of knowledge in the following areas: identifying and treating a bleed, haemophilia and physical activity, travel, career issues and genetics. Lower levels of
knowledge were reported in the areas of sexual activity, product safety, information about factor, haemophilia and ageing, advocacy, timing of prophylactic infusions, and new or alternative therapies. Treating a bleed was the most commonly sought information, followed by information about factor, product safety, identifying a bleed and other health care issues. There was a positive correlation between knowledge seeking and severity of disease. HTC attendance was associated with knowledge seeking, and HTCs were the most frequented knowledge source, followed by the Canadian Haemophilia Society website. Canadian men were well informed; the HTC’s role in knowledge sharing was recognized. Timing of infusions, sexual activity and ageing are areas which should be targeted in knowledge sharing.
Introduction
knowledge levels and understanding of haemophilia have been published [2–6] and Canadian data on this topic address only mild haemophilia [7]. The availability of such data could lead to better educational strategies and more optimal disease management. This article reports the results from phase 2 of a mixed methods study which was undertaken to explore and assess knowledge of disease and knowledge gaps among Canadian men living with haemophilia. In phase 1, we explored the perspectives of health care providers regarding areas of knowledge important for care of severe haemophilia [6]. In this study (phase 2) we surveyed individuals (≥18 years) with all severities of haemophilia to identify their existing knowledge levels and gaps and compare how these are similar or different according to disease severity.
Haemophilia is an x-linked, inherited bleeding disorder that affects approximately 3000 Canadian men [1]. Many adults with haemophilia actively manage this chronic disease, through self-infusion at home with support from a Hemophilia Treatment Centre (HTC), of which there are 25 across Canada. Selfmanagement places responsibility on individuals to be knowledgeable about the disease, treatment approaches, complications and injury prevention strategies. Only five studies that assess individuals’ Correspondence: Nancy Heddle, McMaster University, HSC 3H50, 1280 Main Street West, Hamilton, ON L8S 4K1, Canada. Tel.: +(905) 525 9140; ext. 22126; fax: +(905) 524 2983; e-mail: [email protected] Accepted after revision 7 October 2013 © 2013 John Wiley & Sons Ltd
Keywords: haemophilia A and B, knowledge, males, survey
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Methods The knowledge areas identified as important by health care providers in phase 1 of the study [6] were used to develop a survey (online Appendix A) consisting of 18 questions covering the following areas: demographic information, HTC-related questions (attendance, distance from HTC), factor use, technology preferences, current knowledge, knowledge seeking, information sources and formats and knowledge gaps. Respondents were asked to rate their current level of knowledge for each area using a six-point scale with the following anchors: no knowledge (0), low (1), medium (3) and high (5). Survey questions asked whether the responder had sought knowledge in the past 12 months and if so in what areas, from what sources and in what formats (written, verbal, website, social media, video/DVD or other source). Finally, respondents were asked if they had looked but were unable to find information in the past 12 months, and to identify other useful information that was not included in the survey. The survey was pilot tested by six individuals with haemophilia to identify problem questions and/or clarifications. Three HTCs participated in the study (from Eastern, Central and Western parts of Canada), by mailing the survey to all individuals with haemophilia over the age of 18 receiving care at their centre. This process ensured participant confidentiality as the research team at the Coordinating Centre did not know the identity of the individuals to whom the survey was sent. The initial mail-out was followed 1 month later by a second mail-out to non-responders. All completed surveys were returned directly to the Coordinating Centre for data entry and analysis. A formal sample size was not calculated but was dictated by the number of HTCs able to participate and the eligible subjects registered at each site (a convenience sample). Research ethics approval for the study was obtained from the Research Ethics Boards at participating sites.
Analysis Response frequencies were tabulated for all survey questions with denominators based on the number responding. Data from the pilot surveys were included; however, the pilot survey did not ask about information seeking in the past 12 months; hence, responses to this question were not available from these six individuals. Knowledge seeking in specific areas, information sources and information formats, were analysed as percentages of the number who sought any information in the past 12 months. For each knowledge area, the mean current self-assessed knowledge level was calculated for all respondents and by disease severity. Spearman rank correlations were used to evaluate the relationship between current knowledge level and Haemophilia (2014), 20, 219--225
severity of haemophilia. Chi-squared tests were used to compare results between categorical variables. Interval valued predictors of knowledge seeking were tested using simple logistic regression.
Results There were 280 surveys sent and 104 were completed and returned, (overall response rate of 37%). Response rates from each site were as follows: Site A = 33% (42/128); Site B = 44% (37/85); Site C = 34% (23/67). Seventy-five percent of respondents had haemophilia A and 25% had haemophilia B, which is similar to the Canadian national distribution [1]. There were 49% (50/103) of respondents who had mild haemophilia, 13% (13/103) with moderate haemophilia, and 39% (40/103) had severe haemophilia, which again was similar to the Canadian distribution of mild (56%), moderate (14%), and severe (30%) [1]. The age distribution of respondents is shown in Fig. 1. Compared with the age distribution of individuals with haemophilia in the Canadian Hemophilia Registry, our sample was representative of the national distribution in the mid age and oldest age categories (35 through 64, 75 to 85+), under representative in the young age category of 25–34, (16% vs. 29%) and overly representative in the 65–74 age category (15% vs. 9%). 72% of respondents had attended their HTC once in the past 12 months. The majority had attended for their annual review, and a few for advice on treating an acute bleed. Living distance from the HTC was not significantly associated with HTC attendance (P = 0.9). Routine prophylaxis use was highest in the 25 and under age group, (71%), 60% in the 25–35 age group and 20% in those 45–75 years of age. Of those using prophylaxis routinely, 94% had severe haemophilia and the remaining 6% had moderate haemophilia. Computer and internet use were most common used forms of technology (84%), followed by smart phone (44%) and tablet technology (26%). Forty-eight per cent of individuals reported using social media (e.g. Twitter, Facebook, Myspace). Respondents were asked to rate their existing knowledge of haemophilia in 19 areas. The mean current knowledge levels for each knowledge area are summarized in Fig. 2. Overall, respondents reported the highest levels of knowledge in the areas of recognizing and treating a bleed, followed by knowledge of the genetics of haemophilia, physical activity selections and knowledge of haemophilia and other health care issues. This was followed by knowledge of preventing a bleed and identifying the type of bleed (muscle, joint, tissue). Reported knowledge levels of travel issues, prevention of joint damage, career decisions and overall disease were lower, with the lowest levels of knowledge reported in the areas of sexual activity © 2013 John Wiley & Sons Ltd
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Fig. 1. Age distribution of respondents.
Fig. 2. Current knowledge levels.
and bleed risk, product information and safety ageing with hemophilia advocacy, timing of infusions, new developments in haemophilia treatment and alternative therapies (e.g. supplements and herbal medicines). Seventy-nine per cent of routine users of prophylaxis reported medium to high (25%) and high (54%) levels of knowledge regarding timing of prophylactic infusions. In several areas, a significant proportion of respondents reported no knowledge. These included the following: timing – what time of the day to infuse (30%); alternative therapies (30%); information about factor (20%); information about sexual activity (19%); product safety (18%); and advocacy (16%). Areas in which a large proportion of respondents reported high levels of knowledge included the following: recognizing a bleed (39%); treating a bleed (40%); other health care issues (26%); genetics of haemophilia (31%); prevention of bleeds (30%); and physical activity selections (30%). Current knowledge © 2013 John Wiley & Sons Ltd
in all areas was positively correlated with an increased severity of haemophilia (Fig. 3). This correlation was statistically significant for all areas of knowledge except genetics, and the relationship between haemophilia and other health care issues. Respondents were asked if they had sought information about haemophilia in the past 12 months, (four individuals did not answer this question). The majority of respondents (78; 73/94) reported not seeking information during that period. Of those who did seek knowledge, the percentages by specific area are presented in Fig. 4, with the most common being information on treating a bleed (71%; 15/21). This was followed by information on the impact of haemophilia on other health care issues, information on identifying bleeds, factor information, product safety, timing of infusions and ageing, all of which were sought by 55– 65% of respondents seeking information. Only 13% of users of routine prophylaxis reported looking for Haemophilia (2014), 20, 219--225
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Fig. 3. Current knowledge by disease severity.
Fig. 4. Knowledge sought by respondents.
information about the timing of infusions. Between 38% and 52% of respondents sought information on preventing and recognizing bleeds, preventing joint damage, issues related to travel, new treatments and general disease knowledge. Fewer than 33 percent of respondents sought information on genetics, physical activity selections, alternative therapies, sexual activity, career considerations and advocacy. The proportion seeking knowledge was similar when respondents were categorized by: distance from an HTC clinic; severity of haemophilia; inhibitor history; HTC attendance; family with haemophilia; and technology use. The rate of knowledge seeking over varying ages was also compared. HTC attendance within the past 12 months emerged as the only variable associated with a significant difference in the rate of knowledge seeking (P = 0.0019). When the Haemophilia (2014), 20, 219--225
overall percentages of those seeking knowledge were broken down by reported levels of existing knowledge, people who sought knowledge tended on average to report higher current knowledge levels (Fig. 5). There was no association between mean current knowledge levels and information seeking or severity of haemophilia and knowledge seeking. For information seeking in all areas except two (advocacy and alternative therapies), HTCs were the most frequently chosen source (75%). For advocacy and alternative therapies, respondents chose the Canadian Hemophilia Society (CHS), which was also the second most frequented source for all other areas of information (40%). Manufacturers, parents or carers and others with haemophilia were also reported as information sources, but by
DOI: 10.1111/hae.12297
ORIGINAL ARTICLE Clinical haemophilia
What should men living with haemophilia need to know? The perspectives of Canadian men with haemophilia E. ARNOLD,* S. LANE,* K. E. WEBERT,†‡ A. CHAN,§ I. WALKER,* J. TUFTS,* S. RUBIN,¶ M - C . P O O N * * and N . M . H E D D L E * † † *Department of Medicine, McMaster University; †Canadian Blood Services; ‡Department of Pathology and Molecular Medicine, McMaster University; §Department of Pediatrics, McMaster University, Hamilton, ON; ¶Department of Medicine, Horizon Health Network, Moncton, NB; **Department of Medicine, Pediatrics and Oncology, University of Calgary, Calgary, AB; and ††Canadian Blood Services, Research and Development, Hamilton, ON, Canada
Summary. Haemophilia is an inherited bleeding disorder affecting approximately 3000 Canadian men (Walker 2012). To manage their disease effectively individuals must be knowledgeable about the disease, bleed prevention strategies, treatment approaches, and complications. Data on individuals’ knowledge levels are scarce. The availability of such data could lead to better educational strategies for disease management. The aim of this study was to determine current knowledge levels, needs and gaps among Canadian individuals with haemophilia to facilitate optimal disease management. A survey was disseminated to adult males with haemophilia at three Haemophilia Treatment Centres (HTCs) in Canada. Self-reported current knowledge levels and knowledge seeking were measured. Survey respondents reported highest levels of knowledge in the following areas: identifying and treating a bleed, haemophilia and physical activity, travel, career issues and genetics. Lower levels of
knowledge were reported in the areas of sexual activity, product safety, information about factor, haemophilia and ageing, advocacy, timing of prophylactic infusions, and new or alternative therapies. Treating a bleed was the most commonly sought information, followed by information about factor, product safety, identifying a bleed and other health care issues. There was a positive correlation between knowledge seeking and severity of disease. HTC attendance was associated with knowledge seeking, and HTCs were the most frequented knowledge source, followed by the Canadian Haemophilia Society website. Canadian men were well informed; the HTC’s role in knowledge sharing was recognized. Timing of infusions, sexual activity and ageing are areas which should be targeted in knowledge sharing.
Introduction
knowledge levels and understanding of haemophilia have been published [2–6] and Canadian data on this topic address only mild haemophilia [7]. The availability of such data could lead to better educational strategies and more optimal disease management. This article reports the results from phase 2 of a mixed methods study which was undertaken to explore and assess knowledge of disease and knowledge gaps among Canadian men living with haemophilia. In phase 1, we explored the perspectives of health care providers regarding areas of knowledge important for care of severe haemophilia [6]. In this study (phase 2) we surveyed individuals (≥18 years) with all severities of haemophilia to identify their existing knowledge levels and gaps and compare how these are similar or different according to disease severity.
Haemophilia is an x-linked, inherited bleeding disorder that affects approximately 3000 Canadian men [1]. Many adults with haemophilia actively manage this chronic disease, through self-infusion at home with support from a Hemophilia Treatment Centre (HTC), of which there are 25 across Canada. Selfmanagement places responsibility on individuals to be knowledgeable about the disease, treatment approaches, complications and injury prevention strategies. Only five studies that assess individuals’ Correspondence: Nancy Heddle, McMaster University, HSC 3H50, 1280 Main Street West, Hamilton, ON L8S 4K1, Canada. Tel.: +(905) 525 9140; ext. 22126; fax: +(905) 524 2983; e-mail: [email protected] Accepted after revision 7 October 2013 © 2013 John Wiley & Sons Ltd
Keywords: haemophilia A and B, knowledge, males, survey
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E. ARNOLD et al.
Methods The knowledge areas identified as important by health care providers in phase 1 of the study [6] were used to develop a survey (online Appendix A) consisting of 18 questions covering the following areas: demographic information, HTC-related questions (attendance, distance from HTC), factor use, technology preferences, current knowledge, knowledge seeking, information sources and formats and knowledge gaps. Respondents were asked to rate their current level of knowledge for each area using a six-point scale with the following anchors: no knowledge (0), low (1), medium (3) and high (5). Survey questions asked whether the responder had sought knowledge in the past 12 months and if so in what areas, from what sources and in what formats (written, verbal, website, social media, video/DVD or other source). Finally, respondents were asked if they had looked but were unable to find information in the past 12 months, and to identify other useful information that was not included in the survey. The survey was pilot tested by six individuals with haemophilia to identify problem questions and/or clarifications. Three HTCs participated in the study (from Eastern, Central and Western parts of Canada), by mailing the survey to all individuals with haemophilia over the age of 18 receiving care at their centre. This process ensured participant confidentiality as the research team at the Coordinating Centre did not know the identity of the individuals to whom the survey was sent. The initial mail-out was followed 1 month later by a second mail-out to non-responders. All completed surveys were returned directly to the Coordinating Centre for data entry and analysis. A formal sample size was not calculated but was dictated by the number of HTCs able to participate and the eligible subjects registered at each site (a convenience sample). Research ethics approval for the study was obtained from the Research Ethics Boards at participating sites.
Analysis Response frequencies were tabulated for all survey questions with denominators based on the number responding. Data from the pilot surveys were included; however, the pilot survey did not ask about information seeking in the past 12 months; hence, responses to this question were not available from these six individuals. Knowledge seeking in specific areas, information sources and information formats, were analysed as percentages of the number who sought any information in the past 12 months. For each knowledge area, the mean current self-assessed knowledge level was calculated for all respondents and by disease severity. Spearman rank correlations were used to evaluate the relationship between current knowledge level and Haemophilia (2014), 20, 219--225
severity of haemophilia. Chi-squared tests were used to compare results between categorical variables. Interval valued predictors of knowledge seeking were tested using simple logistic regression.
Results There were 280 surveys sent and 104 were completed and returned, (overall response rate of 37%). Response rates from each site were as follows: Site A = 33% (42/128); Site B = 44% (37/85); Site C = 34% (23/67). Seventy-five percent of respondents had haemophilia A and 25% had haemophilia B, which is similar to the Canadian national distribution [1]. There were 49% (50/103) of respondents who had mild haemophilia, 13% (13/103) with moderate haemophilia, and 39% (40/103) had severe haemophilia, which again was similar to the Canadian distribution of mild (56%), moderate (14%), and severe (30%) [1]. The age distribution of respondents is shown in Fig. 1. Compared with the age distribution of individuals with haemophilia in the Canadian Hemophilia Registry, our sample was representative of the national distribution in the mid age and oldest age categories (35 through 64, 75 to 85+), under representative in the young age category of 25–34, (16% vs. 29%) and overly representative in the 65–74 age category (15% vs. 9%). 72% of respondents had attended their HTC once in the past 12 months. The majority had attended for their annual review, and a few for advice on treating an acute bleed. Living distance from the HTC was not significantly associated with HTC attendance (P = 0.9). Routine prophylaxis use was highest in the 25 and under age group, (71%), 60% in the 25–35 age group and 20% in those 45–75 years of age. Of those using prophylaxis routinely, 94% had severe haemophilia and the remaining 6% had moderate haemophilia. Computer and internet use were most common used forms of technology (84%), followed by smart phone (44%) and tablet technology (26%). Forty-eight per cent of individuals reported using social media (e.g. Twitter, Facebook, Myspace). Respondents were asked to rate their existing knowledge of haemophilia in 19 areas. The mean current knowledge levels for each knowledge area are summarized in Fig. 2. Overall, respondents reported the highest levels of knowledge in the areas of recognizing and treating a bleed, followed by knowledge of the genetics of haemophilia, physical activity selections and knowledge of haemophilia and other health care issues. This was followed by knowledge of preventing a bleed and identifying the type of bleed (muscle, joint, tissue). Reported knowledge levels of travel issues, prevention of joint damage, career decisions and overall disease were lower, with the lowest levels of knowledge reported in the areas of sexual activity © 2013 John Wiley & Sons Ltd
WHAT SHOULD MEN LIVING WITH HAEMOPHILIA NEED TO KNOW?
221
Fig. 1. Age distribution of respondents.
Fig. 2. Current knowledge levels.
and bleed risk, product information and safety ageing with hemophilia advocacy, timing of infusions, new developments in haemophilia treatment and alternative therapies (e.g. supplements and herbal medicines). Seventy-nine per cent of routine users of prophylaxis reported medium to high (25%) and high (54%) levels of knowledge regarding timing of prophylactic infusions. In several areas, a significant proportion of respondents reported no knowledge. These included the following: timing – what time of the day to infuse (30%); alternative therapies (30%); information about factor (20%); information about sexual activity (19%); product safety (18%); and advocacy (16%). Areas in which a large proportion of respondents reported high levels of knowledge included the following: recognizing a bleed (39%); treating a bleed (40%); other health care issues (26%); genetics of haemophilia (31%); prevention of bleeds (30%); and physical activity selections (30%). Current knowledge © 2013 John Wiley & Sons Ltd
in all areas was positively correlated with an increased severity of haemophilia (Fig. 3). This correlation was statistically significant for all areas of knowledge except genetics, and the relationship between haemophilia and other health care issues. Respondents were asked if they had sought information about haemophilia in the past 12 months, (four individuals did not answer this question). The majority of respondents (78; 73/94) reported not seeking information during that period. Of those who did seek knowledge, the percentages by specific area are presented in Fig. 4, with the most common being information on treating a bleed (71%; 15/21). This was followed by information on the impact of haemophilia on other health care issues, information on identifying bleeds, factor information, product safety, timing of infusions and ageing, all of which were sought by 55– 65% of respondents seeking information. Only 13% of users of routine prophylaxis reported looking for Haemophilia (2014), 20, 219--225
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E. ARNOLD et al.
Fig. 3. Current knowledge by disease severity.
Fig. 4. Knowledge sought by respondents.
information about the timing of infusions. Between 38% and 52% of respondents sought information on preventing and recognizing bleeds, preventing joint damage, issues related to travel, new treatments and general disease knowledge. Fewer than 33 percent of respondents sought information on genetics, physical activity selections, alternative therapies, sexual activity, career considerations and advocacy. The proportion seeking knowledge was similar when respondents were categorized by: distance from an HTC clinic; severity of haemophilia; inhibitor history; HTC attendance; family with haemophilia; and technology use. The rate of knowledge seeking over varying ages was also compared. HTC attendance within the past 12 months emerged as the only variable associated with a significant difference in the rate of knowledge seeking (P = 0.0019). When the Haemophilia (2014), 20, 219--225
overall percentages of those seeking knowledge were broken down by reported levels of existing knowledge, people who sought knowledge tended on average to report higher current knowledge levels (Fig. 5). There was no association between mean current knowledge levels and information seeking or severity of haemophilia and knowledge seeking. For information seeking in all areas except two (advocacy and alternative therapies), HTCs were the most frequently chosen source (75%). For advocacy and alternative therapies, respondents chose the Canadian Hemophilia Society (CHS), which was also the second most frequented source for all other areas of information (40%). Manufacturers, parents or carers and others with haemophilia were also reported as information sources, but by
