Clinical assessment of quality of life of the patients with chronic blood transfusion and chelation therapy

Background: The patient with hemoglobinopathy like thalassemia major (TM), bone marrow dysfunction, and sickle cell disease are requiring chronic blood transfusion for their life time to survive. Among these, TM is one of the most common hemoglobinopathy worldwide. Objective: This study is done to assess the quality of life (QOL) of the patients with chronic blood transfusion and chelation therapy. Materials and Methods: The QOL scores were obtained through the selfadministered SF-36 questionnaire. The study subjects were asked to answer the SF-36 questionnaire once every 3 months. Results: After 6 months, the SF 36 general health mean score was 63.58 ± 12.98 (P < 0.05). The highest mean score was 69.37 ± 11.61. The mean difference after the fi nal reviews was statistically signifi cant. Conclusion: The preponderance of scores used to assess QOL suggests that there is a direct and independent effect on QOL when treated with transfusion and iron-chelation therapy.


INTRODUCTION
The patient with hemoglobinopathy like Thalassemia major (TM), Bone marrow dysfunction, and Sickle cell disease are requiring chronic blood transfusion in their life time to survive.Among these, TM is one of the most common hemoglopinopathy in world wide.The purpose of the blood transfusion is to restore the blood hemoglobin (Hb) level and to eliminate the complication of TM.State that patient are transfused at an early age may develop complications of iron overload in tissues, blood transfused infections, heart failure, diabetes mellitus, liver fi brosis, delayed puberty and growth retardation. [1,2]Iron chelators like desferrioxamine and defriprone are used to eliminate the toxic iron load and reduce the complications of iron overload in tissues since introduction of iron chelators, the mortality and morbidity rate associated with thalassemia have been signifi cantly reduced. [3]The quality of life (QOL) is one of the important indexes of effective treatment.QOL is a focused study on the individual's views on their well-being and aspect of life.A model of QOL is proposed that integrates objective and subjective indicators, a broad range of life domains, and individual values.It takes account of concerns that externally derived norms should not be applied without reference to individual differences.It also allows for objective comparisons to be made between the situations of particular groups and what is normative.Considerable agreement exists that QOL is multidimensional.Coverage may be categorized within fi ve dimensions: Physical well-being, material well-being, social well-being, emotional well-being, and development and activity. [4]e SF-36 questionnaire yields an eight-scale profi le of functional health and well-being scores as well as psychometrically-based physical and mental health summary measures and a preference-based health utility index.7][8]

MATERIALS AND METHODS
This prospective study was performed on transfusion dependent patient with hemoglobinopathy in the Department of Transfusion Medicine at Government General Hospital, Guntur, Andhra Pradesh India.The study was approved by Institutional Review Board.The inclusion criteria were both genders with the age more than 5 years, a diagnosis of hemoglobinopathy having more than 20 blood transfusions so far.Patient with bone marrow transplantation and the history of chronic disease like chronic obstructive pulmonary disease (COPD) and acquired immunodefi ciency syndrome (AIDS) were excluded from the study.In total 147 patients were interviewed, 142 qualifi ed, and 137 completed the study.The QOL scores were obtained through the self-administered questionnaire.These patients were assessed once in every 3 months with SF-36 questionnaire.Patient who were unable to answer the questionnaire were assisted by their parents or guardians.The subject's medical record was reviewed to assess the morbidity associated with chronic blood transfusion.Hb threshold of 6.0-7.0 g/dl was the baseline.

Outcome measures
The SF-36 questionnaire is a self-administered health status measure that is widely accepted and validated. [9]It consists of 36 questions in eight dimensions such as physical function, physical health, role emotional, energy/fatigue, emotional well-being, social functioning, pain, and general health.All questions are scored on a scale from 0 to 100, with 100 representing the highest level of functioning possible.The mean scores of SF 36 for each patient at 0 month are underlined as a baseline score.This baseline score is used to evaluate the difference in mean scores after every 3 months for a period of 6 months.

Statistical analysis
Documented data was analyzed by using analysis of variance (ANOVA).Statistical signifi cance was taken at 95% level (P < 0.05).Results were expressed as mean ± standard deviation (SD).

Primary variables
Out of 137 patients, most of them belong to the age group between 10 and 15 years (42.3%).Male (67.1%) patients were greater than the female (32.9%) patients.Most of the patients have the family history of hemoglobinopathy (66.4%) [Table 1].

Physical function
The mean baseline score of SF-36 physical function was 62.87.87 ± 23.58.The mean score difference was not statistical signifi cant after 3 months (63.14 ± 21.02).However, at fi nal review mean score (73.72 ± 21.98) showed statistical signifi cant from the base line mean score (P < 0.05).

Role physical health
The mean baseline score of SF-36 role physical health was 43.

DISCUSSION
In developing countries TM has become a major health problem. [10]M is usually recognized in childhood, at which time patient begin treatment with blood transfusion to maintain Hb level and chelation therapy to overcome iron overload in tissues later.This study results support the other studies on the QOL of TM patent with other chronic disease like hepatitis, hypothyroidism, ulcerative colitis, cystic fi brosis, congenital heart disease, epilepsy, and diabetes. [11]Very good scores were obtained for the physical, emotional, role, and social function domains.In the current study, we found that patients rated their overall health signifi cantly better with transfusion and ironchelation therapy.The differences were mainly found in perceived physical health that they considered themselves less dependent on other and medical aids, having good energy and less fatigue in their daily activity.The regimen of iron chelation probably has signifi cant infl uence on the QOL of conventionally treated patients.The current study has demonstrated that patient on chronic blood transfusion with chelation therapy had signifi cantly better self-rated QOL in physical mental and social domains.

CONCLUSION
The measurement of health-related QOL is fraught with methodological difficulties, and comparison across is often impossible.But the preponderance of scores used to assess QOL suggests that there is a direct and independent effect on QOL when treated with transfusion and iron-chelation therapy.We often do not have the capacity to cure disease, but we can focus on improving symptom complexes, such as fatigue, lack of energy, and functional capacity.

Limitations
Hb threshold of 6.0-7.0 g/dL was the baseline.This recommendation, however, may not take into account the range of potentially normal Hb concentrations.For example, it assumes that a man with a baseline Hb concentration of 18 g/dL and a woman with a baseline Hb concentration of 12 g/dL will have a similar tolerance to an acute decrease in Hb to 7.0 g/dL. [12] International Journal of Medicine and Public Health | Jan-Mar 2014 | Vol 4 | Issue 1 75 ± 11.57.The mean score difference was statistical signifi cant after International Journal of Medicine and Public Health | Jan-Mar 2014 | Vol 4 | Issue 1