|
| |
 |
|
| ORIGINAL ARTICLE |
|
| Year : 2016 | Volume
: 4
| Issue : 1 | Page : 18-25 |
|
Relationship between quality of life, functional independence and joint functions of adult hemophiliacs- A cross sectional study
R Thangamani1, I Chaudhari2, K Jariwala2, J Patel2, N Patel2, S Purani3, S Kumar4, M Patel4
1 Lecturer, Sarvajanik College of Physiotherapy, Surat, Gujarat, India
2 Physiotherapist, Sarvajanik College of Physiotherapy, Surat, Gujarat, India
3 Consultant Physiotherapist, Sparsh Physiotherapy Clinic, Surat, Gujarat, India
4 Lead Clinical Support Therapist, Ann Physiocare Ltd, Swansea, UK
| Date of Web Publication | 29-Aug-2018 |
Correspondence Address:
R Thangamani
Lecturer, Sarvajanik College of Physiotherapy, Surat, Gujarat
India
 Source of Support: None, Conflict of Interest: None  | 2 |
DOI: 10.4103/2347-6486.240039
Background: QOL is a wide based subject and multifaceted topic dependent on culture, beliefs, and social and environmental factors etc., it is expected that a comprehensive evaluation with available tools in an Indian population would give more insight and awareness to their problems and limitations.
Objective: The main aim of the current study was to asses QOL, Functional independence and functional limitations related to joints of adult Hemophilia patients.
Methods: Adult male patients age equal or greater than 18 years with factor viii or ix deficiency and who were willing to participate and given consent were included in the study. The data were collected by getting the self- reported outcome measures filled, the WHOBREF – QOL, and the objective functional instruments Functional independence score of hemophiliacs (FISH) and GILBERT joint score administered by the researcher. The data were analyzed with Pearson correlation analysis, non -parametric test and Regression analysis to find out the variability.
Results: The result showed that the functional components like transfer and locomotion were mainly determined by joint bleeds and total joint score respectively. And the moderate bleed groups were different in terms of pain, total joint score, transfer and total functional independence.
Conclusion: More the bleed in the joints leads to reduced joint function and functional independence of hemophiliac patients, affects their health related quality of life.
Keywords: Hemophilia, QOL, Functional Independence, Joint function
How to cite this article:
Thangamani R, Chaudhari I, Jariwala K, Patel J, Patel N, Purani S, Kumar S, Patel M. Relationship between quality of life, functional independence and joint functions of adult hemophiliacs- A cross sectional study. J Integr Health Sci 2016;4:18-25 |
How to cite this URL:
Thangamani R, Chaudhari I, Jariwala K, Patel J, Patel N, Purani S, Kumar S, Patel M. Relationship between quality of life, functional independence and joint functions of adult hemophiliacs- A cross sectional study. J Integr Health Sci [serial online] 2016 [cited 2023 Jun 9];4:18-25. Available from: https://www.jihs.in/text.asp?2016/4/1/18/240039 |
| Introduction | |  |
Hemophilia is a genetically transmitted disorder of deficiency of circulating blood clotting factor. Hemophilia A and B are rare disorders which result from a deficiency of clotting factor VIII&IX.[1],[2] The deficits of hemophilia individuals lead to repeated spontaneous and post-traumatic internal bleeding episodes and the consequent musculoskeletal implications of haemophilia and immobilization may result in muscle atrophy, synovitis, chronic hemophilic arthropathy, joint and muscle contractures, Limb length inequality and loss of proprioception.[3],[4],[5]
Quality of Life (QoL) is a phrase used to refer to an individual’s total wellbeing. This includes all emotional, social, and physical aspects of the individual’s life. However, the individual’s wellbeing may be impacted over time by a disease, a disability, or a disorder.[6] Hemophilia may have a significant impact on the quality of life (QoL). And older children were reported to have higher impairments on social adaptation to conditions.[7],[8] As patients with haemophilia age, A need for support around employment is necessary for individuals and they are less involved in full time work and suffer more from occupational disability.[9] For example, a
Polish study, found that 38% were unemployed and received some form of social subvention [10]. Adults with haemophilia have financial consequences for themselves and their families as well as feelings of dependency and more likely to take early retirement.[1] One study stressed the importance of career counseling regarding training and work for young adults.[11]
To explain the higher reported quality of life in some studies, a phrase called ‘the disability paradox,' was used to describe the correlation between poor quality health and the development of adaptive coping strategies, which leads to good quality of life.[12] Hence a constant measuring of QOL in changing life situations can help to determine the relationships between QOL and risk factors. Interpretation of these data can help identify the needs for health policies and legislation.[13] Significant differences in quality of life between countries were found. The paper attempts to describe conceptual, methodological and practical foundations of QoL research in adults and children at an international level[14] Use of factor prophylaxis and its cost in both children and adults require objective measures of health, such as joint status and quality of life. There was a highly significant negative correlation between functional independence and quality of life scores. Quality of life in the Turkish patients were more impaired in the subscales of physical health, feeling as well as more impaired joint scores and authors recommended primary factor prophylaxis and encouraging the patients to learn home treatment to improve joint scores and quality of life.[15],[16],[17] The development of functional scores such as the Functional Independence Score and quality-of-life measurement tools, have helped to provide a more comprehensive assessment and management of health in hemophilia.[18]
Purpose of the study
Increasingly more emphasis is placed on issues related to QoL in disorder, disease and disability. Since QoL is a wide based subject and multifaceted topic dependant on culture, beliefs, social and environmental factors etc., it is expected that a comprehensive evaluation with available tools in an Indian population would give more insights into the policies and treatment plan related to haemophilia patients. The main aim behind the current study was to asses and find out the relationships between QOL, Functional independence and functional limitations related to affect in adult Hemophilia patients of Indian population in a small centre like Surat.
| Methodology | | |
Subjects
Adult male patients aged equal or greater than 18 years with factor viii or ix deficiency and who were willing to participate and willing to give consent from Surat chapter of hemophiliacs were included in the study. Patients with disabilities other than hemophilia were excluded from the study. A Non probability sampling method was used.
Procedure
The data were collected from the eligible individuals with hemophilia, who gave consent to participate in the study, by getting the self-reported outcome measures filled and the objective functional instrument administered by the researcher.
Outcome measures
WHOBREF Questionnaire (QoL) – The WHOQOL-BREF consists of 24 facets and provides a profile of scores on four dimensions of quality of life: physical health, psychological, social relationships, and the environment. WHOQOL- BREF is available in both self-administered and interviewer-administered and assisted forms.[19] For patients who did not understand English language were helped with interviewer assisted forms.
Function independence score - The Functional Independence Score in Hemophilia (FISH) was developed as a performance-based assessment tool to objectively measure an individual’s functional ability (self-care, transfer and locomotion) for individuals suffering with Haemophilia. It is intended to measure what the person with disability actually does, not what he ought to be able to do, or might be able to do if circumstances were different, or thinks he can do. It can also be used to evaluate change in functional independence over time, or after a therapeutic intervention. The current version of FISH includes the assessment of eight activities: eating, grooming, dressing, chair transfer, squatting, walking, step climbing, and running. Each activity is graded according to the amount of assistance required to perform it.[18],[21]
Gilbert Score - This tool measures joint health, in the domain of body structure and function (i.e. impairment), of the joints most commonly affected by bleeding in hemophilia – knees, ankles, elbows. It is primarily designed for adults and children with established arthropathy to measure pain, bleeding and joint score (Swelling, Muscle atrophy, Axial deformity, Crepitus on motion, Range of motion, Flexion contracture, Instability). It may be used when there is a need for orthopedic intervention, or as an outcome measure to study the effect of physiotherapy interventions.[22],[23]
Statistical analyses
Data collected were cleaned for paucity before the analysis and the categorical variables presented in percentages and numerical variables in terms of mean and standard deviations for the demographical and clinical outcomes. A Pearson correlation analysis was done among QOL, functional independence and joint scores. Significant outcome variables entered in regression analysis to find out the variance after determining the dependent and independent variables theoretically. A mean comparison between mild and moderate bleeding groups was done using non parametric Mann Whitney U test. A Logistic regression analysis was done to find out the significant personal demographical factors for bleeding groups. Data were analyzed using IBM SPSS Statistics for Windows, Version 20.0. Armonk, NY: IBM Corp. Statistical significance was set at p<0.05 (Two tailed).
| Results | | |
The present study collected data from 59 adult male hemophiliacs with mean age 30.89±9.10, among them 57 with factor VIII defect and 2 with factor IX defect. The personal demographical and clinical data of the subjects are given in [Table 1] and [Table 2].
[Table 3],[Table 4] &[Table 5] show the correlation between the quality of life components, Functional independence measure components and the total joint limitation score of Gilbert joint evaluation of adult haemophiliac individuals. Significantly correlated outcomes were used in regression analysis to find out the proportion of variance between outcomes. | Table 3: Pearson correlation among Qol and, Functional independence score
Click here to view |
 | Table 5: Pearson correlation among Functional independence and Gilbert Joint score
Click here to view |
[Table 6] shows the regression analysis of the significantly related out comes of QOL, functional independence and gilbert joint score.
| Discussion | | |
Despite long term improvement in life expectancy of patients with Haemophilia over the years, QoL measures are a subject of intense discussion and every attempt in research is a part to constantly improve issues related to it. As mentioned previously, since QOL is a multifaceted topic which varies with culture, beliefs, etc., we aimed to study this function comprehensively in small sample of Haemophiliacs Indian population. Based on the results from the baseline indicators, the sample population that we assessed were predominantly of Haemophilia A (Factor level < 1, 86.4%), the most common type in general population. And at the time of study, most of the patients in the sample population studied were of mild bleed around 66% in the past 1 year, who reported of mild and moderate pain in the range of around 27% and 63% respectively. 95% of the patients reported of episodic transfusion implying that at the time of assessment of this study most of the patients would have preventative measures and coping measures in place for the disease and did not have an active bleed. The percentages for the total joint score were moderate and severe of 44% and 39% respectively indicated that the subjects in the sample population have predominantly established deformities from episodes.
Overall, in terms of QOL, the sample population assessed for their Physical, Psychological, Social and Environmental domains espoused fair results (> 65%) as showed from the mean values indicating they exhibited fairly good adjustment in these domains in their daily life, however need to study in depth in relation to functional independence and joint status which is mentioned below.
Analyzing from the results, Psychological health domain positively correlated with pain (p < 0.003) in Gilbert joint score and locomotion (p < 0.01) in Functional independent measure, indicating that Psychometric values of emotions are clearly related to pain and inability to move among hemophiliac patients. And issues related to pain dominate the Psychological health of a patient compared to locomotion. The Environmental domain of QOL showed positive relation to pain, locomotion and total joint score. As observed, pain in major joints (Elbow, Knee and, Ankle) seemed to dominate the mobility and feeling of self satisfaction among the sample population. Compared to other factors commonly affecting hemophilic patients, transfer ability dominated the Social relationships domain and this could be due to improper usage of appropriate walking aids for mobility and it is expected that a proper evaluation of walking aid is warranted.
The social relationships domain was positively related (p = 0.006) only to transfer ability of the hemophiliacs meaning that most of the patients believed that their mobility about a chair and ability to squat was helpful in maintaining relationships. And importantly the functional independence of hemophiliacs was dependent upon the severity of pain, bleeding of joints and tota l joint score which were common and evident from other studies done on hemophiliac patients. The functional components like transfer and locomotion were mainly determined by joint bleeds and total joint score respectively which were observed by the positive correlation between the respective components. Hence more the bleed and joint score, higher the dependence for transfer and locomotion.
The mean comparison of mild and moderate bleed groups of hemophiliacs showed that the moderate bleed groups were different in terms of pain, total joint score, transfer and total functional independence. More the bleed in the joints and involvement in more number of joints could be the factor which leads to reduced joint function and functional independence of hemophiliac patients which in turn affects their health related quality of life. The result of the present study was similar to many authors who pointed out that the psychosocial dimensions are of great importance in determining and to maximize quality of life [1],[11]. Repeated joint bleeds, pain, arthritis and arthropathy may reduce the QOL and lack of independence due to reduced mobility [1],[24]. Nowadays living a better life with better QOL is possible for hemophiliacs due to advancement in medical treatments and psychosocial support [25],[26]. The present study also reported moderately good score in all the dimensions of QOL and the personal demographical factors did not contribute to bleeding of joints. This study is a reinforcement of the existing studies done on QOL on haemophiliacs with respect to outcomes.
The limitation of the study was patients were a small sample size taken predominantly from a single center Surat. And the present study used only generic QOL tool and the result may have less generalizability.
| Conclusion | | |
The present study shows that the adult hemophiliac patients of Surat chapter reported reasonably good quality of life. The moderate bleed group hemophiliac patients have more of pain and lack of independence. A more concerted, timely and aggressive Physiotherapy principles in the prevention of pain and bleeding for hemophiliac patients and improving their independence may be of more importance and advantage for leading a good quality life.
| References | | |
| 1. | Barlow JH, Stapley J, Ellard DR. Living with haemophilia and von Willebrand’s: a descriptive qualitative study. Patient education and counseling. 2007 Nov 30;68(3):235-42.  |
| 2. | Price VE, Hawes SA, Chan AK. A practical approach to hemophilia care in children. Paediatrics & child health. 2007 May;12(5):381. |
| 3. | Genderen FR, Meeteren NL, Bom JG, Heijnen L, Kleijn PD, Berg HM, Helders PJ. Functional consequences of haemophilia in adults: the development of the Haemophilia Activities List. Haemophilia. 2004 Sep 1;10(5):565-71. |
| 4. | Hilberg T, Herbsleb M, Gabriel HH, Jeschke D, Schramm W. Proprioception and isometric muscular strength in haemophilic subjects. Haemophilia. 2001 Nov 12;7(6):582-8. |
| 5. | Ghosh K. Challenges of haemophilia care in India: lest we forget. Indian Journal of Medical Research. 2009 Jul 1;130(1):87-8. |
| 6. | Barr RD, Saleh M, Furlong W, Horsman J, Sek J, Pai M, Walker I. Health status and health-related quality of life associated with hemophilia. American journal of hematology. 2002 Nov 1;71(3):152-60. |
| 7. | Bradley CS, Bullinger M, McCusker PJ, Wakefield CD, Blanchette VS, Young NL. Comparing two measures of quality of life for children with haemophilia: the CHO-KLAT and the Haemo-QoL. Haemophilia. 2006 Nov 1;12(6):643-53. |
| 8. | Gringeri AV, Von Mackensen S, Auerswald G, Bullinger M, Garrido RP, Kellermann E, Khair K, Lenk H, Vicariot M, Villar A, Wermes C. Health status and health-related quality of life of children with haemophilia from six West European countries. Haemophilia. 2004 Mar 1;10(s1):26-33. |
| 9. | Heijnen L. The role of rehabilitation and sports in haemophilia patients with inhibitors. Haemophilia. 2008 Nov 1;14(s6):45-51. |
| 10. | Windyga J, Stefańska E, Lopaciuk S, Juszyński A, Wozniak D, Strzelecki O. [The orthopaedic status of a selected severe haemophilia group]. Polskie Archiwum Medycyny Wewnetrznej. 2005 Jun;113(6):562-9. |
| 11. | Frederica R.M.Y. Cassis. Psychosocial Care for People with Hemophilia. In: Sam Schulman, editor. Treatment of Hemophilia.44:World Federation of Hemophilia ; 2007. |
| 12. | Beeton K. Evaluation of outcome of care in patients with haemophilia. Haemophilia. 2002 May 1;8(3):428-34. |
| 13. | Miners AH, Sabin CA, Tolley KH, Jenkinson C, Kind P, Lee CA. Assessing health-related quality-of-life in individuals with haemophilia. Haemophilia: the official journal of the World Federation of Hemophilia. 1999 Nov;5(6):378- 85. |
| 14. | Bullinger M, Gringeri A, von Mackensen S. Quality of life of young patients with haemophilia in Europe. Bundesgesundheitsblatt, Gesundheitsforschung, Gesundheitsschutz. 2008 Jun;51(6):637-45. |
| 15. | Aledort L, Bullinger M, Von Mackensen S, Wasserman J, Young NL, Globe D. Why should we care about quality of life in persons with haemophilia?. Haemophilia. 2012 May 1;18(3):e154-7. |
| 16. | Hassan TH, Badr MA, EL-GERBY KM. Correlation between musculoskeletal function and radiological joint scores in haemophilia A adolescents. Haemophilia. 2011 Nov 1;17(6):920-5. |
| 17. | Mercan A, Sarper N, Inanır M, Mercan HI, Zengin E, Kılıç SÇ, Gökalp AS. Hemophilia- Specific Quality of Life Index (Haemo-QoL and Haem-A-QoL questionnaires) of children and adults: result of a single center from Turkey. Pediatric hematology and oncology. 2010 Aug 27;27(6):449-61. |
| 18. | Poonnoose PM, Thomas R, Keshava SN, Cherian RS, Padankatti S, Pazani D, Kavitha ML, Devadarasini M, Bhattacharji S, Viswabandya A, John JA. Psychometric analysis of the functional independence score in haemophilia (FISH). Haemophilia. 2007 Sep 1;13(5):620-6. |
| 19. | World Health Organization’s. Quality of Life group: WHOQOL-sBREF Introduction. Administration and Scoring. Field Trial version. 1996. |
| 20. | Group TW. The World Health Organization quality of life assessment (WHOQOL): development and general psychometric properties. Social science & medicine. 1998 Jun 15;46(12):1569-85. |
| 21. | Poonnoose PM, Manigandan C, Thomas R, Shyamkumar NK, Kavitha ML, Bhattacharji S, Srivastava A. Functional Independence Score in Haemophilia: a new performance-based instrument to measure disability. Haemophilia. 2005 Nov 1;11(6):598-602. |
| 22. | Gilbert MS. Prophylaxis: musculoskeletal evaluation. InSeminars in hematology 1993 Jul (Vol. 30, No. 3 Suppl 2, pp. 3-6). |
| 23. | Gilbert MS, Weidel JD. The Treatment of Hemophilia: Current Orthopedic Management; National Hemophilia Foundation 1995. |
| 24. | Beeton K, Neal D, Lee C. An exploration of health-related quality of life in adults with haemophilia–a qualitative perspective. Haemophilia. 2005 Mar 1;11(2):123-32. |
| 25. | Beeton K, Neal D, Watson T, Lee CA. Parents of children with haemophilia–a transforming experience. Haemophilia. 2007 Sep 1;13(5):570- 9. |
| 26. | Gringeri A, Musso R, Mazzucconi MG, Piseddu G, Schiavoni M, Pignoloni P, Mannucci PM. Immune tolerance induction with a high purity von Willebrand factor/VIII complex concentrate in haemophilia A patients with inhibitors at high risk of a poor response. Haemophilia. 2007 Jul 1;13(4):373-9. |
[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6]
|
Search Pubmed for