Assessment of the Quality of Life in Korean Hemophiliacs: Impact of Disease-Related Factors, Social Status and Treatment Factors on the Quality of Life of Korean Hemophiliacs

Article information

Clin Exp Thromb Hemost. 2014;1(1):10-16
Publication date (electronic) : 2014 May 10
doi : https://doi.org/10.14345/ceth.14005
Department of Pediatrics, Eulji University School of Medicine, Daejeon, Korea
*Corresponding author: Chur Woo You, MD, PhD Department of Pediatrics, Eulji University Hospital, 95 Dunsanseo-ro, Seo-gu, Daejeon 302-799, Korea Tel: +82-42-611-3352 Fax: +82-42-259-1111 E-mail: YCW1@eulji.ac.kr
Received 2014 January 17; Revised 2014 March 19; Accepted 2014 March 25.

Trans Abstract

Purpose

The identification of disease-related factors, treatment factors and the social status of hemophiliac patients which may have an impact on their QoL in each country, is vital in establishing potential intervention strategies for these patients. For this reason, we evaluated the impact of these factors on the QoL of Korean hemophiliac patients.

Methods

QoL was evaluated using the SF36 standardized questionnaires. Disease-related factors were obtained from the patients’ medical records. Socio-demographic data, such as marital status, occupational status, education years, as well as a treatment data, self-injection ability were obtained using questionnaires. Correlation analysis and multiple regression analysis were conducted to elucidate the impact of the observed data on the QoL of these patients.

Results

The disease-related factors were correlated with physical health of the patients. Marital status was the only social status shown to have a correlation with the QoL of the hemophiliac patients; marital status was shown to only affect patients’ physical health. However, self-injection ability, the only treatment factor surveyed in this study, was significantly associated with both the physical and mental health of the patients. Mental health was revealed to be poorer in Korean hemophiliac patients when compared with that of patients residing in countries with well-organized hemophilia treatment systems.

Conclusion

One social factor (marital status) and the disease-related factors correlated with the physical health of the Korean hemophiliac patients. Self-injection ability was associated with both the physical and mental health of the patients. The differences in treatment factors across different countries may contribute to the difference in the physical and mental health of hemophiliac patients.

Introduction

The morbidity and mortality of hemophiliac patients has markedly decreased since the introduction of viral inactivation technologies, gene recombinant products, and prophylactic treatment.[1] The success in the treatment of hemophilia requires a shift from the narrow focus on morbidity and mortality to the enhancement of quality of life and well being of patients. Therefore, health related quality of life (HRQoL) has been increasingly used as an outcome parameter in hemophilia treatment.[2-6] Most of the quality of life (QoL) assessments for hemophiliac patients have mainly focused on the impact of the disease-related factors such as the severity of disease, inhibitor presence, arthropathy and human immunodeficiency virus (HIV) infection status of the patients.[7-14] However, the QoL may differ depending on the social status of the patients.[15] Epidemiologic and clinical research studies have indicated that social and cultural factors exert an influence on patients’ QoL, and the impact of these factors may differ among countries.[16,17] Despite the plausible impact of social status on the QoL of hemophiliac patients, few researchers have studied the impact of social status such as marital status and occupational status on the QoL of hemophiliac patients. Furthermore, it is important to identify factors affecting the QoL of hemophiliac patients, such as social status and treatment, in order to establish potential intervention strategies across different countries. Therefore, the impact of social status, disease-related factors and treatment factors on the QoL were evaluated in Korean hemophiliac patients.

Methods

The data were collected from questionnaires, physical examinations and medical records of Korean hemophiliac patients over 17 years of age, in cooperation with the Korean Hemophilia Patient Association (KOHEM, Korea Hemophilia Association). The data were collected during summer camp for hemophiliac patients in the year 2010. All of the patients consented to the study after a detailed explanation of the study was provided. The IRB at Eulji University Hospital approved the study.

Disease-related factors and a treatment factor

Disease-related factors, such as the type and severity of disease, the presence of an inhibitor, and hepatitis C virus (HCV) and HIV infection history were obtained from the medical records of the study patients. Unlike other studies, patients’ hemarthropathy status was evaluated by the degree of a patient’s perception of involved major joints including knees, ankles and elbow joints, to assess hemarthropathy from a patient’s point of view. This is because medical records were not available for hemarthropathy evaluation. If the patient felt that the involved joint was very severely affected, a score of 3.0 was allocated. If the patient felt that the joint was less severely affected or not severely affected, a score of 2.0 or 1.0 was allocated, respectively. Subjective joint scores were obtained for each patient after adding together the scores for each affected joint. The analgesic use scores were calculated from the questionnaires. If the patient used analgesics greater than 3 times a week on average, a score of 2.0 was allocated. If the patient used analgesics less than 2 times a week or had no history of analgesic use, the scores of 1.0 and 0.0 were allocated, respectively. Patient disability levels were obtained from physical examinations during the summer camp period by physicians based on the disability level evaluation guidelines in Korea.[18] A lower score meant more severe disability. As a treatment factor, self-injection score was calculated from the questionnaires for each patient. The self-injection score was 3.0 if a patient was able to selfinject coagulation factor concentrates (CFCs) in 80-100% of all moderate bleeding episodes, 2.0 if it was possible in 50-80% of all moderate bleeding episodes, and 1.0 if it was possible in less than 50% of the bleeding episodes. If a patient was not able to self-inject CFCs, a score of 0.0 was allocated. The impact of prophylactic treatment could not be evaluated, because only on–demand treatment had been provided to the patients who were involved in the study, due to national health insurance restrictions on the administration of prophylactic treatment in Korea.

Socio-demographic data

Socio-demographic data, such as marital status, occupational status, and education years, were obtained by questionnaires. The number of work or school day absences per year was also evaluated by questionnaires.

Quality of Life assessment

SF36 (standardized questionnaires of the medical outcomes study short form 36 health survey) were used to evaluate QoL of the hemophiliac patients.[19,20] SF36 is composed of 36 questions and eight scales to assess the general health state of each study participant. The eight scales represent physical functioning (PF), role-physical (RP), body pain (BP), general health (GH), vitality (VT), social functioning (SF), role-emotion (RE) and mental health (MH) of each subject, and each scale can have a value ranging from 0 (worst) to 100 (best). The physical component summary (PCS) represents four scales of physical states (physical functioning, role-physical, body pain, general health), and the mental component summary (MCS) represents the other four scales.[20]

Comparisons and analysis

Observed disease-related factors, a treatment factor and socio-demographic data were compared among the patient groups that were divided by the severity of hemophilia and the presence of inhibitor. The QoL determined using SF36 was compared among the patient groups, and was compared with the published data for the QoL of osteoarthritic patients and normal healthy control subjects in Korea.[21] Paired t-test was used for comparisons, and a P-value less than 0.05 was defined as significant. In addition, correlation analysis and stepwise multiple regression analysis were conducted between the factors (disease-related factors, the treatment factor and socio-demographic data) and each scale of the SF36 using SPSS version 12.0 (SPSS Inc., Chicago, IL, USA). Because the QoL generally declines with age[22] and hemarthropathy also worsens with age,[23] age-adjusted analysis was conducted.

Results

Severity of disease and the presence of inhibitor

Initially, 108 patients participated in this study. However, only 55 hemophiliac patients completed all of the questionnaires and these patients’ clinical data was collected by reviewing their medical records. There were 35 severe hemophiliac patients (64%), 5 moderate hemophiliac patients (9%) and 15 inhibitor patients (27%). There was no mild hemophiliac patient. Most (80%) of the inhibitor patients were high responding inhibitor patients (Table 1).

Social status, disease-related and treatment-related data in moderate/severe hemophiliac patients, as well as in patients with inhibitors

Disease-related factors, a treatment factor and socio-demographic data of Korean hemophiliac patients

Seventy one percent of patients were already disabled, and 19% of patients were regularly using analgesics. The average subjective joint score was 6.76, which meant that on average, the study patients felt very severe joint abnormalities in at least two major joints. There were no HIV infected patients, but 36.3% of the patients were infected with HCV. Ninety percent of the patients could self-inject CFCs for over 50% of all moderate bleeding episodes, and the average self-injection score was 1.71±0.63. The average age and years of education of the patients were 33.2±12.9 years and 13.06±2.2 years, respectively. No subject under 24 years of age was married or employed. Fifty percent of the patients over 24 years of age were married, and 44% of the patients over 24 years of age were employed. The average loss in work or school was 6.6 days in a year (Table 1).

Comparison of the disease-related factors, treatment factor, socio-demographic data and the QoL, depending on the severity of disease and the presence of an inhibitor

There was no difference in the average age among the following three groups- severe hemophiliac, moderate hemophiliac, and inhibitor patient groups. Compared with the severe hemophiliac patient group, the subjective joint scores were nearly twice (1.8 times) as high, analgesic scores were 3.2 times higher and work- day loss was 1.6 times higher in inhibitor patients. In contrast, disability scores, self-injection scores and socio-demographic data were not significantly different between the severe hemophiliac and inhibitor groups. The number of work days lost and analgesic use were lower and the percentage of patients who were actively working was higher in the moderate hemophiliac patient group compared with the severe hemophiliac and inhibitor patient groups (Table 1). Statistically the QoL of inhibitor patients were significantly lower in the BP, PCS and MH scales compared with severe hemophilia patients (Fig. 1, ★ P<0.05). The QoL was not different between the severe and moderate hemophiliac patients, except for BP (Fig. 1, ◆ P<0.05).

Fig. 1.

The QoL of severe, moderate hemophiliac patients and patients with inhibitors. ★P < 0.05 compared with the patients with inhibitors, ◆P < 0.05 compared with moderate hemophiliac patients. PF, physical function; RP, role physical; BP, bodily pain; GH, general health; VT, vitality; SF, social function; RE, role emotional; MH, mental health; PCS, physical component summary; MCS, mental component summary.

Comparisons of the QoL based on the socio-demographic status and disease-related factors of Korean hemophiliac patients

When the QoL was compared according to differences in the sociodemographic state, there were no significant differences in the QoL between married and unmarried patients and patients with a job and without a job. There were significant differences in some scales of SF 36 above a certain value of age, subjective joint score, disability score, and analgesic score of those patients. In patients older than 24 years of age, the PF, GH, VT, SF, MH, and PCS, MCS scores were significantly lower when compared with those younger than 24 years of age. In patients having the subjective joint score ≥ 4.0, the BP, GH, VT, SF, MH, and PCS scores were significantly lower when compared with those having the subjective joint score<4.0. In patients with the disability score≥ 3.0, only PF score was significantly higher when compared with those having the disability score<3.0. In patients with the analgesic use score≥ 1.0, the PF, BP, GH, VT, SF, MH, and PCS scores were significantly lower when compared with those having the analgesic use score<1.0 (Table 2).

QoL comparisons based on social status and disease-related factors in Korean hemophiliac patients

Correlation analysis for disease-related factors, treatment factor, socio-demographic data and the QoL

The age-adjusted partial correlation analysis showed that the severity of disease and inhibitor presence was negatively correlated with BP, PCS, SF, and PF. The analgesic use score and the disability level were correlated with several scales of SF36. However, neither the subjective joint score nor the presence of HCV infection showed any significant correlation with any of the SF36 scales (Table 3). The analgesic use score was negatively correlated with PCS, BP, and VT. The disability score was correlated with PF and GH (Table 3). However, when stepwise multiple regression analyses were conducted for the SF36 scales, which correlated with the disease- related factors, severity of disease and inhibitor presence were the significant variables only for BP (β=0.752, P=0.048), the analgesic use score was only a significant variable for PCS (β=-0.695, P=0.002), and the disability level was not a significant variable for any of the correlated SF36 scales (Table 4). In the socio-demographic data, only marital status was correlated with the SF36 scales; it was correlated with PCS, GH, PF, BP, VT, and SF when age-adjusted correlation analysis was conducted (Table 3). However, stepwise multiple regression analysis for these correlated SF36 scales revealed that marital status was a significant variable only for the physical SF36 scale, BP (β=0.638, P=0.02) (Table 4). The self-injection score is the only treatment factor that could be surveyed in the study and significantly correlated with GH, SF, and MH (Table 3). The stepwise multiple regression analysis for these SF36 scales showed that self-injection score was a significant variable for all these SF36 correlated scales [GH (β=0.895, P=0.011), SF (β= 0.489, P= 0.046) and the MH domain (β=0.611, P=0.040)] (Table 4)

Age-adjusted partial correlations between social status, disease-related factors, the treatment factor and each SF36 scale

Stepwise multiple regression analysis between social status, disease-related factors, treatment factor and each SF36 scale

Comparisons of social status and the QoL of hemophiliac patients with normal healthy control subjects and osteoarthritic patients

The marital status and years of education completed by the Korean patients with hemophilia were not different from those of normal healthy subjects of a similar age group (Table 5). However a significantly lower proportion of hemophiliac patients were in the active work force (44% of hemophiliac patients vs. 100% of healthy individuals). The QoL of hemophiliac patients was much lower than that of normal control subjects in all SF36 scales, including the mental health scales; the average scores for each scale ranged from 44.1% (RP)-74.8% (VT), compared to those of the control subjects (Fig. 2). The hemophiliac patients had similar QoL to osteoarthritic patients in Korea, especially in the physical health scales. However, the average age of the osteoarthritic patients was nearly twice that of the hemophiliac patients, and the SE36 mental health scales (SF, RE, MH and MCS) of the osteoarthritic patients was better than that of the hemophiliac patients (Fig. 2).

Socio-demographic data between normal healthy controls, osteoarthritic, and hemophiliac patients in Korea

Fig. 2.

The QoL of normal healthy persons, osteoarthritic patients and hemophiliac patients in Korea. Control: normal healthy persons. OA, osteoarthritic patients; PF, physical function; RP, role physical; BP, bodily pain; GH, general health; VT, vitality; SF, social function; RE, role emotional; MH, mental health; PCS, physical component summary; MCS, mental component summary.

Discussion

The impact of disease-related factors, the treatment factor, and social status on the QoL of Korean hemophiliac patients

The age-adjusted partial correlation analysis showed that the severity of disease, presence of inhibitor, analgesic use and disability were associated with the SF36 physical and mental health scales, in the present study (Table 3). However, the stepwise multiple regression analysis showed that all of these factors were significant variables only for a few physical health domains of SF36 (Table 4). This was very similar to the results observed in other studies; the severity of disease,[7-9] inhibitor presence,[24,25] and arthritis[9,11-13] correlated only with the SF36 physical health scales. Unlike other studies, our study patients’ subjective report of their joint state was shown to not correlate with their QoL. This result may be derived from the limitations of arthropathy evaluation in this study because most of the medical records were not available to appropriately evaluate hemarthropathy. Consequently, we could not objectively determine the state of arthropathy. Instead, analgesic use and disability, which may represent a state of more severe hemarthropathy, were associated with QoL. According to the study conducted by Fischer et al.[13], the impact of hemarthropathy on the QoL in hemophiliac patients may not be significant unless the patient is severely affected. Therefore, the subjectively evaluated joint state may have had an impact on the QoL only after the hemarthropathy was severe enough to necessitate the use of analgesics, or which would have resulted in disability. This speculation was supported by the observation of significantly lower values of several physical and mental health scales of those patients whose subjective joint scores were above 4.0 points when compared to patients with subjective joint scores below 4.0 points (Table 2). In addition to the impact of disease-related factors on the QoL, social status (marital status) and treatment factor (self-injection score) were correlated with the SF36 physical and mental health scales (Table 3). Marriage may have a positive impact on the QoL of hemophiliac patients; a study conducted in Austrian patients showed that marriage may be the cause of nearly normal emotional and mental health scores in Austrian hemophiliac patients.[26] However, there were no differences in emotional and mental health scores between the married and unmarried patients in the present study (Table 2), and marital status was only a significant variable for the physical domain (BP: β= 0.638, P= 0.02) of SF36, based on stepwise multiple regression analysis (Table 4). Therefore, marital status may have little impact on the mental health of these Korean patients. In contrast, the ability of these patients to self-inject was the only factor that had an impact on the mental health of hemophiliac patients. The self-injection score was the only treatment factor that could be recorded in the study. The mental health scale (MH) and two other scales (GH and SF) that represent physical and mental health of patients[20] were correlated with self-injection ability when the age-related partial correlation analysis was conducted (Table 3). Furthermore, the self- injection score was shown to be significantly correlated with the SF36 scales when stepwise multiple regression analysis was conducted (Table 4). Few studies have been conducted about the impact of self-injection on the QoL of hemophiliac patients. The ability to self inject may have a significant impact on the physical, social and mental health status of hemophiliac patients because these patients can rapidly control their bleeding, and feel that they can independently control their disease and actively participate in social activities. As a result, the ability to self- inject may have an impact on both the physical and mental health of hemophiliac patients.

Speculations regarding the cause of poor mental health in Korean hemophiliac patients

Mental health has been shown to be poorer in Korean hemophiliac patients compared with age-matched normal healthy subjects and osteoarthritic patients in Korea (Fig. 2). This result was different from that of other studies, most of which were conducted in developed countries that have a long history of well-organized hemophilia care systems.[6-8] Results of these studies showed that the mental health scale scores were similar to the normative means.[6-8] Social status was not different between hemophiliac patients and normal healthy subjects of similar ages in the present study except for employment rate, which was higher in normal healthy subjects (44% of hemophiliac patients vs. 100% of healthy individuals) (Table 5). Markova et al.[27] showed that unemployed hemophiliac patients appeared to be less adjusted to life and suffered most from psychosomatic symptoms. However, the lower employment rate could not alone explain the lower mental health among Korean hemophiliac patients. The mental health scales score for hemophiliac patients was similar to that of normal control subjects, although the employment rate in Austrian patients[26] was also significantly lower than normal control patients (64% of hemophiliac patients vs. 90% of healthy individuals). The proportion of patients with different hemophilia severities in this study were different from other studies.[7,8] There was no mild hemophiliac patient, and only 9% of the patients had moderate hemophilia. 64% of the patients had severe hemophilia, and 27% of the patients had inhibitors present, most of whom were high responding inhibitor patients (Table 1). The difference in the proportion of hemophilia severities may explain the different scores of mental health scales and the lower scores in all SF36 scales compared with other studies.[7-11,24,25] However, mental health was not different between patients with various disease severities in these studies. Therefore, the poorer mental health in Korean hemophiliac patients could not be explained by the different proportion of disease severities in hemophiliac patients. The social, cultural, physical and educational status may all affect a person’s mental health.[28] However, according to the result of a study conducted in Chinese hemophiliac patients,[29] mental well being was not correlated with hemarthropathy, salary, or cost of replacement therapy. Similar results were also observed in Korean hemophiliac patients. Neither disease-related factors nor social status were significantly associated with the mental health of these patients when multiple regression analysis was conducted (Table 4). Only the self-injection score, which was the only treatment factor that could be surveyed in this study, had a significant impact on the mental health of Korean hemophiliac patients (Table 4). Few researchers have studied the impact of treatment factors on the mental health of hemophiliac patients. A comparative study showed no difference in the QoL between patients treated with prophylaxis and patients treated with on-demand treatment.[6] However, there are many treatment factors that may affect the QoL of hemophiliac patients, including the ability to self- inject, as well as the presence or absence of well-organized hemophilia treatment centers, resource constraints and the cost of hemophiliac therapy, which vary in different countries. Therefore the differences in treatment factors across countries may contribute to poorer mental health in hemophiliac patients in Korea and China, although the etiology remains to be elucidated.

In conclusion, disease-related factors and social status only had an impact on the physical health of Korean hemophiliac patients. However, the ability to self-inject had an impact on both the physical and mental health of Korean hemophiliac patients. In contrast to the results found by studies carried out in developed countries, mental health was significantly poorer in Korean hemophiliac patients. Differences in the treatment factors across countries may have contributed to the poorer mental health of Korean hemophiliac patients.

Acknowledgements

We would like to thank the Korean Hemophilia Association (KOHEM) for their sincere cooperation in data collection.

Notes

This work was sponsored by a grant from the Novo Nordisk Region A/S.

References

1. Ingram GI. The history of haemophilia. J Clin Pathol 1976;29:469–79.
2. Leidy NK, Revicki DA, Geneste B. Recommendations for evaluating the validity of quality of life claims for labeling and promotion. Value Health 1999;2:113–27.
3. Schramm W, Royal S, Kroner B, Berntorp E, Giangrande P, Ludlam C, et al, ; European haemophilia economic study group. Clinical outcomes and resource utilization associated with haemophilia care in Europe. Haemophilia 2002;8:33–43.
4. Association of Hemophilia Clinic Directors of Canada. Hemophilia and von Willebrand`s disease: 1. Diagnosis, comprehensive care and assessment. CMAJ 1995;153:19–25.
5. De Kleijn P, Heijnen L, Van Meeteren NL. Clinimetric instruments to assess functional health status in patients with haemophilia: a literature review. Haemophilia 2002;8:419–27.
6. Royal S, Schramm W, Berntorp E, Giangrande P, Gringeri A, Ludlam C, et al, ; European haemophilia economic study group. Quality-of-life differences between prophylactic and on-demand factor replacement therapy in European haemophilia patients. Haemophilia 2002;8:44–50.
7. Miners AH, Sabin CA, Tolley KH, Jenkinson C, Kind P, Lee CA. Assessing health-related quality-of-life in individuals with haemophilia. Haemophilia 1999;5:378–85.
8. Trippoli S, Vaiani M, Linari S, Longo G, Morfini M, Messori A. Multivariate analysis of factors influencing quality of life and utility in patients with haemophilia. Haematologica 2001;86:722–8.
9. Solovieva S. Clinical severity of disease, functional disability and health-related quality of life. Three-year follow-up study of 150 Finnish patients with coagulation disorders. Haemophilia 2001;7:53–63.
10. Barr RD, Saleh M, Furlong W, Horsman J, Sek J, Pai M, et al. Health status and health-related quality of life associated with hemophilia. Am J Hematol 2002;71:152–60.
11. Aznar JA, Magallon M, Querol F, Gorina E, Tusell JM. The orthopaedic status of severe haemophiliacs in Spain. Haemophilia 2000;6:170–6.
12. Molho P, Rolland N, Lebrun T, Dirat G, Courpied JP, Croughs T, et al. Epidemiological survey of the orthopaedic status of severe haemophilia A and B patients in France. Haemophilia 2000;6:23–32.
13. Fischer K, Van der Bom JG, Mauser-Bunschoten EP, Roosendaal G, Van den Berg HM. Effects of haemophilic arthropathy on health-related quality of life and socio-economic parameters. Haemophilia 2005;11:43–48.
14. Schick M, Stucki G, Rodriguez M, Meili EO, Huber E, Michel BA, et al. Haemophilic; arthropathy: assessment of quality of life after total knee arthroplasty. Clin Rheumatol 1999;18:468–72.
15. Stucki G, Sigl T. Assessment of the impact of disease on the individual. Best Pract Res Clin Rheumatol 2003;17:451–73.
16. Schmidt S, Bullinger M. Cross-cultural quality of life assessment: approaches and experiences from the health care field. In : Gough I, McGregor JA, eds. Wellbeing in developing countries: from theory to research 1st ed New York, NY: Cambridge University Press, New York; 2007. p. 219–41.
17. Bullinger M, von Mackensen S. Psycho-social determinants of quality of life in children and adolescents with haemophilia - a cross-cultural approach. Clin Psychol Psychother 2008;15:164–72.
18. Ministry health and welfare. Korean guideline for deciding disability levels http://www.mw.go.kr/front_new/jb/sjb0402ls.jsp. Accessed April 10, 2014.
19. Ware JE Jr, Sherbourne CD. The MOS 36-item shortform health survey (SF-36). I. Conceptual framework and item selection. Med Care 1992;30:473–83.
20. McHorney CA, Ware JE Jr, Raczek AE. The MOS 36- item Short-Form Health Survey (SF-36): II. Med Care 1993;31:247–63.
21. Lee SW, Oh KT, Chung WT, Bae SC. Health-related quality of life in Korean patients with osteoarthritis. J Korean Rheum Assoc 2002;9(Suppl):S73–83.
22. Aaronson NK, Muller M, Cohen PD, Essink-Bot ML, Fekkes M, Sanderman R, et al. Translation, validation, and norming of the Dutch language version of the SF- 36 Health Survey in community and chronic disease populations. J Clin Epidemiol 1998;51:1055–68.
23. Fischer K, Van Hout BA, Van der Bom JG, Grobbee DE, Van den Berg HM. Association between joint bleeds and Pettersson scores in severe haemophilia. Acta Radiol 2002;43:528–32.
24. Gringeri A, Mantovani LG, Scalone L, Mannucci PM. COCIS Study Group. Cost of care and quality of life for patients with hemophilia complicated by inhibitors: the COCIS Study Group. Blood 2003;102:2358–63.
25. Brown TM, Lee WC, Joshi AV, Pashos CL. Health-related quality of life and productivity impact in haemophilia patients with inhibitors. Haemophilia 2009;15:911–7.
26. Hartl HK, Reitter S, Eidher U, Ramschak H, Ay C, Pabinger I. The impact of severe haemophilia on the social status and quality of life among Austrian haemophiliacs. Haemophilia 2008;14:703–8.
27. Markova I, Lockyer R, Forbes C. Self-perception of employed and unemployed haemophiliacs. Psychol Med 1980;10:559–65.
28. Kitchener BA, Jorm AF. Mental health first aid manual. Canberra: Centre for mental health research. 2002:5.
29. Wang T, Zhang L, Li H, Zhao H, Yang R. Assessing health-related quality-of-life in individuals with haemophilia in China. Haemophilia 2004;10:370–5.

Article information Continued

Fig. 1.

The QoL of severe, moderate hemophiliac patients and patients with inhibitors. ★P < 0.05 compared with the patients with inhibitors, ◆P < 0.05 compared with moderate hemophiliac patients. PF, physical function; RP, role physical; BP, bodily pain; GH, general health; VT, vitality; SF, social function; RE, role emotional; MH, mental health; PCS, physical component summary; MCS, mental component summary.

Fig. 2.

The QoL of normal healthy persons, osteoarthritic patients and hemophiliac patients in Korea. Control: normal healthy persons. OA, osteoarthritic patients; PF, physical function; RP, role physical; BP, bodily pain; GH, general health; VT, vitality; SF, social function; RE, role emotional; MH, mental health; PCS, physical component summary; MCS, mental component summary.

Table 1.

Social status, disease-related and treatment-related data in moderate/severe hemophiliac patients, as well as in patients with inhibitors

Severe hemophilia Moderate hemophilia Inhibitor patient Total
Number of patients (%) 35 (64) 5 (9) 15 (27) 55 (100)
Age, years 31.8±1.9 33.8±16.3 35.6±15.1 33.2±12.9
Socio-demographic data
 Job>24 years (%) 10/22 (45) 2/3 (67)* 4/11 (36) 16/36 (44)
 Marriage>24 years (%) 11/22 (50) 2/3 (67) 11/5 (45) 18/36 (50)
 Years of education, years 13.40±1.99 11.00±1.73 12.9±2.55 13.06±2.2
Disease-related data
 Subjective joint score 5.66±5.28 4.20±4.49 10.20±7.85 6.76±6.30
 Disability (%) 24/35 (69) 4/5 (80) 11/15 (73) 39/55 (71)
 Disability score 3.00±1.35 3.25±1.26 2.91±1.58 3.00±1.38
 Analgesic use (%) 5/34 (15) 0/4(0)* 5/14 (36) 10/52 (19)
 Analgesic use score 0.18±0.46 0.00* 0.57±0.85 0.27±0.59
 Work (school) day loss 5.94±6.64 2.50±3.53* 9.29±7.87 6.60±6.88
 Hepatitis C infected (%) 13/35 (37.1) 2/5 (40) 5/15 (33.3) 20/55 (36.3)
Treatment-related data
 Self-injection (>50%)] (%) 33/35 (94) 3/5 (60) 14/15 (93) 50/55 (90)
 Self-injection score 1.89±0.47 1.20±1.09 1.47±0.64 1.71±0.63

Some values are expressed as mean ± standard deviation.

*

P < 0.05 compared with severe hemophiliac patients and inhibitor patients;

P < 0.05 compared with severe hemophiliac patients;

Self-injection was possible in > 50% of bleeding episodes.

Table 2.

QoL comparisons based on social status and disease-related factors in Korean hemophiliac patients

PF RP BP GH VT SF RE MH PCS MCS
Age ≥24 33.04* 49.83 39.95 34.10* 42.80* 36.77* 9.95 39.83* 36.27* 38.75*
n=36 (10.24) (89.67) (10.03) (10.53) (10.69) (11.11) (11.27) (12.80) (8.16) (12.83)
<24 48.61 43.86 42.84 45.04 51.26 47.65 41.2 48.97 45.61 47.29
n = 19 (7.72) (9.55) (12.94) (10.71) (11.19) (7.04) (10.52) (10.26) (7.26) (10.39)
Marriage (>24 years) (+) 34.53 63.88 37.18 32.26 40.98 36.23 41.25 39.82 35.19 38.64
n = 18 (10.87) (126.69) (10.19) (11.51) (11.51) (12.61) (13.9) (14.96) (14.96) (14.83)
(-) 31.55 35.78 42.72 35.94 44.62 37.30 39.05 39.82 37.34 38.85
n = 18 (9.63) (9.29) (9.33) (9.42) (9.92) (9.73) (12.5) (10.64) (6.18) (10.90)
Occupation (>24 years) (+) 33.10 38.55 41.87 34.59 41.36 36.57 39.20 39.78 38.11 37.67
n = 16 (10.89) (7.95) (8.71) (9.08) (10.85) (9.45) (9.80) (9.45) (6.81) (11.75)
(-) 32.99 58.77 38.40 33.71 43.95 36.92 38.50 39.86 34.79 39.60
n = 20 (9.97) (120.72) (10.93) (11.79) (10.68) (12.53) (10.01) (13.26) (8.99) (13.87)
Subjective joint score ≥4 38.32 53.39 37.33 36.83 43.11 38.69 37.02 41.98 37.92 40.39
n=32 (11.73) (94.89) (11.62) (12.50) (10.74) (11.67) (15.05) (12.38) (9.64) (12.47)
<4 38.54 39.95 45.97 39.33 49.37 43.07 42.01 44.37 41.69 43.52
n = 23 (12.59) (10.12) (8.11) (10.68) (11.76) (9.98) (12.56) (13.22) (7.68) (12.91)
Disability score ≥3 42.19* 52.75 41.97 40.38 47.63 42.59 37.52 47.07 41.71 43.46
n=40 (10.68) (84.51) (10.93) (12.00) (11.65) (10.48) (9.06) (13.47) (8.31) (12.98)
<3 28.35 34.50 38.21 31.22 40.64 35.01 41.05 37.41 33.59 37.05
n = 15 (9.30) (9.97) (11.41) (8.02) (9.69) (11.28) (11.02) (8.23) (8.25) (10.65)
Analgesic use score ≥1 30.62§ 30.67 31.17§ 30.03§ 36.48§ 31.19§ 36.02 36.76§ 30.69§ 35.56
n = 10 (9.81) (9.59) (6.42) (6.62) (6.40) (12.06) (10.60) (10.80) (6.51) (11.34)
<1 39.69 51.83 42.60 39.61 47.02 42.42 40.12 43.69 41.12 42.38
n=42 (11.86) (82.48) (10.92) (12.13) (11.11) (10.13) (12.93) (12.47) (8.41) (12.39)

All the values were expressed as mean (standard deviation).

*P< 0.05, †P< 0.05, ‡P< 0.05, and §P< 0.05 compared with the age < 24 years, subjective joint score < 4, disability score < 3, and the analgesic score < 1, respectively. PF, physical function; RP, role physical; BP, bodily pain; GH, general health; VT, vitality; SF, social function; RE, role emotional; MH, mental health; PCS, physical component summary; MCS, mental component summary.

Table 3.

Age-adjusted partial correlations between social status, disease-related factors, the treatment factor and each SF36 scale

PF RP BP GH VT SF RE MH PCS MCS
Marriage 0.395* -0.095 0.356* 0.461 0.359* 0.339* 0.217 0.207 0.521 0.203
Job 0.060 -0.107 0.176 0.064 -0.113 0.005 0.024 0.014 0.259 -0.063
Severity & inhibitor -0.274* -0.056 -0.502 -0.171 -0.145 -0.312* -0.103 -0.124 -0.380 -0.121
Education years -0.056 0.066 0.022 -0.181 -0.192 -0.154 -0.046 -0.060 -0.068 -0.055
Self-injection score -0.102 -0.079 -0.018 0.314* 0.021 0.282* 0.031 0.319* -0.111 0.234
Analgesic score -0.223 -0.070 -0.415 -0.227 -0.322* -0.267 -0.116 -0.177 -0.418 -0.103
Workday loss 0.052 0.038 -0.230 -0.280 0.193 -0.016 -0.025 -0.055 -0.164 0.020
Disability score 0.332* 0.171 -0.067 0.278* 0.142 0.266 0.154 0.170 0.160 0.160
Subjective joint score -0.187 -0.061 -0.221 -0.056 -0.159 -0.056 0.078 -0.045 -0.231 0.022
Hepatitis C -0.186 -0.097 -0.152 -0.204 -0.193 -0.005 -0.149 -0.226 -0.182 -0.122

All the values listed above are Pearson correlation coefficients with 95% confidence intervals.

*

P< 0.05,

P< 0.01.

PF, physical function; RP, role physical; BP, bodily pain; GH, general health; VT, vitality; SF, social function; RE, role emotional; MH, mental health; PCS, physical component summary; MCS, mental component summary.

Table 4.

Stepwise multiple regression analysis between social status, disease-related factors, treatment factor and each SF36 scale

PF RP BP GH VT SF RE MH PCS MCS
Marriage NS NS β=0.638
P=0.002
NS NS NS NS NS NS NS
Severity & inhibitor NS NS β=0.752
P=0.048
NS NS NS NS NS NS NS
Self-injection score NS NS NS β=0.895
P=0.011
NS β=0.489
P=0.046
NS β=0.611
P=0.040
NS NS
Analgesic score NS NS NS NS NS NS NS NS β=0.695
P=0.002
NS
Disability score NS NS NS NS NS NS NS NS NS NS

All of the values listed above are beta coefficients and P-values of the respective beta coefficients.

β, beta coefficient; NS, Not significant; PF, physical function; RP, role physical; BP, bodily pain; GH, general health; VT, vitality; SF, social function; RE, role emotional; MH, mental health; PCS, physical component summary; MCS, mental component summary.

Table 5.

Socio-demographic data between normal healthy controls, osteoarthritic, and hemophiliac patients in Korea

Age, mean±SD Married, % Occupation rate, % Education years, mean±SD
Control (n=228) 37.4±11.5 52.6 100 14.6±4.6
Hemophilia (n=55) 33.2±12.9 50 44 13.1±2.2
OA (n=103) 61.1±6.7 100 NS 6.0±3.4

Control: normal healthy persons.

OA, Osteoarthritic patients; NS, not studied; SD, standard deviation.