Haemophilia (2014), 20, 376–381
DOI: 10.1111/hae.12321
ORIGINAL ARTICLE Clinical haemophilia
Social determinants and health-related dimensions of quality of life in adult patients with haemophilia R . D O L A T K H A H , * A . F A K H A R I , † M . Z . P E Z E S H K I , ‡ R . S H A B A N L O U E I , * N . T A V A S S O L I * and M. GHOLCHIN§ *Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran; †Clinical Psychiatry Research Center, Tabriz University of Medical Sciences, Tabriz, Iran; ‡Department of Community Medicine, Program for Estimation of Pretest Probability, Tabriz University of Medical Sciences, Tabriz, Iran; and §Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
Summary. The availability of safe and effective factor replacement therapies, in persons with haemophilia (PWH), has in some countries answered the basic need for treatment of these patients. The findings suggest that adult patients who have always been on prophylaxis reported significantly better physical functioning, and thus better quality of life. This study is designed to evaluate the QoL in adult PWH, by focusing on social determinants of QoL and their relationship with healthrelated dimensions, in Tabriz, Iran. The survey instrument was a self-report 36 items questionnaire, ‘A36 Hemofilia – QoL’, which is a disease-specific questionnaire for the assessment of the health-related QoL in adults living with haemophilia. A total of 100 haemophilia A and B patients, aged over 17 years participated in this study within 1 year. QoL total score
was 71.88 (26.89 SD). Patients who treat in our Hemophilia Treatment Center, had better QoL score (P = 0.000), and education has a significant impact on the social aspects of QoL (P = 0.18). The QoL was very poor in urban area in contrast to patients who lived in the city (54.45 vs. 74.21 respectively). Single patients have a better QoL than married patients (76.56 vs. 68.50 respectively). Our results showed that low education and lack of awareness of the diseases among PWH lead to reduce of QoL and more disease complications. More and wider treatment and psychological care for improving quality of life of these patients are seriously recommended.
Introduction
social functioning of the patient. These factors can also have a destructive effect on patients’ quality of life [9]. Health-related quality of life (HRQoL) is a varying and dynamic structure, which is conducted to determine a wide range of psychological, physical, social and functional dimensions. Thus, its measurement can mean visualization of severity of symptoms of the disease or its treatment complications, assessing perception and experience of patients and manifestation of these in their daily lives [10,11]. Measurement of quality of life in haemophilic patients is important in terms of both clinical decisions, and reported consequences by patients [12]. The inconsistent and ever-at risk state of haemophiliacs cause perceived inability, despair and persistent worrying about the future. These patients are forced to make themselves compatible with a variety of constraints (physical, occupational, educational and financial). Hence, perhaps, the feeling of needing support, and lack of support from the community, could generate frustration and sometimes even suppressed violence
Haemophilia is a genetic haemorrhagic disorder, and in terms of prevalence, Iran ranks second in the Middle East [1–3]. Although modern treatment methods have succeeded in increasing survival rates [4–6], these patients still suffer complications due to haemorrhage. These haemorrhages mainly occur in the joints, often causing arthropathy, severe joint pain, stiffness and reduced range of motions [7,8]. These clinical presentations, not only negatively affect physical functioning of haemophiliacs, but they also threaten psychological and physical well-being and
Correspondence: Ali Fakhari, Clinical Psychiatry Research Center, Tabriz University of Medical Sciences, Razi Educational and Treatment Center, Elgholi Road, PO Box: 51575 Tabriz, Iran. Tel./fax: +98 411 3804486; e-mail: [email protected] Accepted after revision 19 October 2013 376
Keywords: health, haemophilia A, haemophilia B, quality of life
© 2013 John Wiley & Sons Ltd
QUALITY OF LIFE IN HAEMOPHILIA
towards the community. The families of these patients often suffer from the disease themselves, or have had to suffer with the patient, and shoulder the burden of expenses, pains and disabilities of the patient. Concern, fear and despair about the future of the patient are always evident in their behaviour and feelings. The studies conducted in Iran reveal that haemophilic patients are always involved in neurological and psychiatric disorders such as depression, anxiety, paranoid ideation, etc. These conditions have had a significant impact on their quality of life [13]. However, which of the aspects of quality of life is affected in these patients is not clear. Thus, with specialized tools, researchers have studied quality of life of adult haemophiliac, to determine the level of quality of life in haemophiliacs over 17 years of age, and also to specify which aspects are mostly affected, so that, necessary decisions can be taken for improvement of various aspects of quality of life, thereby, reducing sufferings in these patients.
Objectives The aim of this study was to assess quality of life in patients with haemophilia attending haemophilia clinic at Shahid Ghazi-Tabatabaei in Tabriz over the past year. Considering the existing experiences in facing haemophiliacs at this clinic in Tabriz, and on the basis of previous studies, the researcher discovered a variety of neurological disorders in these patients, including depression, anxiety, paranoid ideation, etc. Therefore, it is necessary to pay special attention to these patients and consider all aspects of their lives, and to improve quality of their lives, necessary procedures must be taught, to prevent incidence of psychological, social and economic risk factors, or to reduce them. Because a total of 100 patients with haemophilia regularly attend this centre, all were included in the study. Study subjects were selected from available patients attending Shahid Ghazi-Tabatabaei Clinic. Demographic details in the form of standard questionnaires, A36 Hemofilia-QoL questionnaire were completed and collected for the patients group. Patients’ information was categorized and analysed according to type and severity of the disease, type of treatment, age, marital status, level of education, place of residence and satisfaction with income. All ethical issues were met, and the study was approved by the institute ethical committee of the Tabriz University of Medical Sciences. All of the patients filled the patient consent form. Also patients were assured of anonymity that their responses would be confidential.
Stages of study This was a descriptive, sectional study conducted over 1 year (March 2011–March 2012). A total of 100 © 2013 John Wiley & Sons Ltd
377
haemophilic patients were surveyed according to availability and in order of attendance at the haemophilia clinic. Quality of life questionnaires, A36 Hemofilia-QoL were completed by the patients, and were collected. Patients’ details were categorized and analysed according to type and severity of disease, type of treatment, marital status, level of education, place of residence and satisfaction with income. All patients were in 17 years plus age range. Lack of intention to participate, illiteracy and chronic medical complications were study exclusion criteria.
Design and methodology The study group included 100 patients with one type of haemophilia with any severity (severe, moderate and mild), confirmatory tests including one-stage FVIII and FIX assay have been performed using STA (Standards and Testing Agency) Deficient FVIII and FIX, an immune-depleted plasma intended in plasma by analysers of the STA line suitable with these reagents (Diagnostica Stago, France) in haematology lab of the centre. They were surveyed using the Disease-specific questionnaire for the assessment of the health-related quality of life adults living with haemophilia, A36 Hemofilia-QoL questionnaire [11,14]. All patients were adults (over 17 years of age) undergoing treatment with coagulation factors. This questionnaire is specific to adult haemophiliacs, translated into Persian (available in: http://www.proqolid.org/). The questionnaire contains 36 four-option questions, with response options of 5-point Likert scale, self assessment and is directly associated with complications of the disease and its treatment titled ‘Difficulties with Treatment’. For completion of the questionnaire, 10–15 min time was allocated for each patient. This questionnaire was a multi-purpose tool, evaluating the following criteria in patients. The criteria in the questionnaire were patients’ problem-specific and included Physical health, Daily activities, Joint Damage, Pain, Treatment Difficulties, Treatment satisfaction and Relationships and Social activities. QoL score indicated quality of life of patients, and higher scores showed better quality of life in patients. Moreover, the score obtained was expressed as a percentage of QoL overall score, and evaluated accordingly, with classification in four groups: 1 = good (>75% of maximum score acquired), 2 = moderate to good (50– 75% of maximum score acquired), 3 = poor to moderate (25–50% of maximum score acquired), 4 = poor (
DOI: 10.1111/hae.12321
ORIGINAL ARTICLE Clinical haemophilia
Social determinants and health-related dimensions of quality of life in adult patients with haemophilia R . D O L A T K H A H , * A . F A K H A R I , † M . Z . P E Z E S H K I , ‡ R . S H A B A N L O U E I , * N . T A V A S S O L I * and M. GHOLCHIN§ *Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran; †Clinical Psychiatry Research Center, Tabriz University of Medical Sciences, Tabriz, Iran; ‡Department of Community Medicine, Program for Estimation of Pretest Probability, Tabriz University of Medical Sciences, Tabriz, Iran; and §Hematology and Oncology Research Center, Tabriz University of Medical Sciences, Tabriz, Iran
Summary. The availability of safe and effective factor replacement therapies, in persons with haemophilia (PWH), has in some countries answered the basic need for treatment of these patients. The findings suggest that adult patients who have always been on prophylaxis reported significantly better physical functioning, and thus better quality of life. This study is designed to evaluate the QoL in adult PWH, by focusing on social determinants of QoL and their relationship with healthrelated dimensions, in Tabriz, Iran. The survey instrument was a self-report 36 items questionnaire, ‘A36 Hemofilia – QoL’, which is a disease-specific questionnaire for the assessment of the health-related QoL in adults living with haemophilia. A total of 100 haemophilia A and B patients, aged over 17 years participated in this study within 1 year. QoL total score
was 71.88 (26.89 SD). Patients who treat in our Hemophilia Treatment Center, had better QoL score (P = 0.000), and education has a significant impact on the social aspects of QoL (P = 0.18). The QoL was very poor in urban area in contrast to patients who lived in the city (54.45 vs. 74.21 respectively). Single patients have a better QoL than married patients (76.56 vs. 68.50 respectively). Our results showed that low education and lack of awareness of the diseases among PWH lead to reduce of QoL and more disease complications. More and wider treatment and psychological care for improving quality of life of these patients are seriously recommended.
Introduction
social functioning of the patient. These factors can also have a destructive effect on patients’ quality of life [9]. Health-related quality of life (HRQoL) is a varying and dynamic structure, which is conducted to determine a wide range of psychological, physical, social and functional dimensions. Thus, its measurement can mean visualization of severity of symptoms of the disease or its treatment complications, assessing perception and experience of patients and manifestation of these in their daily lives [10,11]. Measurement of quality of life in haemophilic patients is important in terms of both clinical decisions, and reported consequences by patients [12]. The inconsistent and ever-at risk state of haemophiliacs cause perceived inability, despair and persistent worrying about the future. These patients are forced to make themselves compatible with a variety of constraints (physical, occupational, educational and financial). Hence, perhaps, the feeling of needing support, and lack of support from the community, could generate frustration and sometimes even suppressed violence
Haemophilia is a genetic haemorrhagic disorder, and in terms of prevalence, Iran ranks second in the Middle East [1–3]. Although modern treatment methods have succeeded in increasing survival rates [4–6], these patients still suffer complications due to haemorrhage. These haemorrhages mainly occur in the joints, often causing arthropathy, severe joint pain, stiffness and reduced range of motions [7,8]. These clinical presentations, not only negatively affect physical functioning of haemophiliacs, but they also threaten psychological and physical well-being and
Correspondence: Ali Fakhari, Clinical Psychiatry Research Center, Tabriz University of Medical Sciences, Razi Educational and Treatment Center, Elgholi Road, PO Box: 51575 Tabriz, Iran. Tel./fax: +98 411 3804486; e-mail: [email protected] Accepted after revision 19 October 2013 376
Keywords: health, haemophilia A, haemophilia B, quality of life
© 2013 John Wiley & Sons Ltd
QUALITY OF LIFE IN HAEMOPHILIA
towards the community. The families of these patients often suffer from the disease themselves, or have had to suffer with the patient, and shoulder the burden of expenses, pains and disabilities of the patient. Concern, fear and despair about the future of the patient are always evident in their behaviour and feelings. The studies conducted in Iran reveal that haemophilic patients are always involved in neurological and psychiatric disorders such as depression, anxiety, paranoid ideation, etc. These conditions have had a significant impact on their quality of life [13]. However, which of the aspects of quality of life is affected in these patients is not clear. Thus, with specialized tools, researchers have studied quality of life of adult haemophiliac, to determine the level of quality of life in haemophiliacs over 17 years of age, and also to specify which aspects are mostly affected, so that, necessary decisions can be taken for improvement of various aspects of quality of life, thereby, reducing sufferings in these patients.
Objectives The aim of this study was to assess quality of life in patients with haemophilia attending haemophilia clinic at Shahid Ghazi-Tabatabaei in Tabriz over the past year. Considering the existing experiences in facing haemophiliacs at this clinic in Tabriz, and on the basis of previous studies, the researcher discovered a variety of neurological disorders in these patients, including depression, anxiety, paranoid ideation, etc. Therefore, it is necessary to pay special attention to these patients and consider all aspects of their lives, and to improve quality of their lives, necessary procedures must be taught, to prevent incidence of psychological, social and economic risk factors, or to reduce them. Because a total of 100 patients with haemophilia regularly attend this centre, all were included in the study. Study subjects were selected from available patients attending Shahid Ghazi-Tabatabaei Clinic. Demographic details in the form of standard questionnaires, A36 Hemofilia-QoL questionnaire were completed and collected for the patients group. Patients’ information was categorized and analysed according to type and severity of the disease, type of treatment, age, marital status, level of education, place of residence and satisfaction with income. All ethical issues were met, and the study was approved by the institute ethical committee of the Tabriz University of Medical Sciences. All of the patients filled the patient consent form. Also patients were assured of anonymity that their responses would be confidential.
Stages of study This was a descriptive, sectional study conducted over 1 year (March 2011–March 2012). A total of 100 © 2013 John Wiley & Sons Ltd
377
haemophilic patients were surveyed according to availability and in order of attendance at the haemophilia clinic. Quality of life questionnaires, A36 Hemofilia-QoL were completed by the patients, and were collected. Patients’ details were categorized and analysed according to type and severity of disease, type of treatment, marital status, level of education, place of residence and satisfaction with income. All patients were in 17 years plus age range. Lack of intention to participate, illiteracy and chronic medical complications were study exclusion criteria.
Design and methodology The study group included 100 patients with one type of haemophilia with any severity (severe, moderate and mild), confirmatory tests including one-stage FVIII and FIX assay have been performed using STA (Standards and Testing Agency) Deficient FVIII and FIX, an immune-depleted plasma intended in plasma by analysers of the STA line suitable with these reagents (Diagnostica Stago, France) in haematology lab of the centre. They were surveyed using the Disease-specific questionnaire for the assessment of the health-related quality of life adults living with haemophilia, A36 Hemofilia-QoL questionnaire [11,14]. All patients were adults (over 17 years of age) undergoing treatment with coagulation factors. This questionnaire is specific to adult haemophiliacs, translated into Persian (available in: http://www.proqolid.org/). The questionnaire contains 36 four-option questions, with response options of 5-point Likert scale, self assessment and is directly associated with complications of the disease and its treatment titled ‘Difficulties with Treatment’. For completion of the questionnaire, 10–15 min time was allocated for each patient. This questionnaire was a multi-purpose tool, evaluating the following criteria in patients. The criteria in the questionnaire were patients’ problem-specific and included Physical health, Daily activities, Joint Damage, Pain, Treatment Difficulties, Treatment satisfaction and Relationships and Social activities. QoL score indicated quality of life of patients, and higher scores showed better quality of life in patients. Moreover, the score obtained was expressed as a percentage of QoL overall score, and evaluated accordingly, with classification in four groups: 1 = good (>75% of maximum score acquired), 2 = moderate to good (50– 75% of maximum score acquired), 3 = poor to moderate (25–50% of maximum score acquired), 4 = poor (
