Assessment of Visual Problems on Patients with Thalassemia (Major) and it impact on their Quality of Life: Case control study

: Background: Major thalassemia is the most inherited hemoglobinopathy that caused severe and progressive anemia. Iron overload due to repeated blood transfusion cause organ damage such as heart, liver, endocrine gland and skeletal system


Abstract:
Background: Major thalassemia is the most inherited hemoglobinopathy that caused severe and progressive anemia.Iron overload due to repeated blood transfusion cause organ damage such as heart, liver, endocrine gland and skeletal system.Desferal, as an iron chelator is used to eliminate iron from the blood that despite its side effects is few, visual problems due to it is exactly known.Objectives: 1-Assessment of visual problems among thlassemia major patients 2-To findout the impact of the visual problems on patients quality of life 3-To compare between thalassemic major patients quality of life for case and control study.Methodology: A descriptive design study was carried out in Ibn-Al-baldi hospital and AL -Karama hospital, starting from 1 th November2013to the 1 st Jun 2014.A non-probability (purposive) sample of 70 adults thalassemia major patients who have visual complication (Case) were attending to the hospitals, and 70 thalassemia major patients not have visual complication as a control study sample.The study instrument was composed of three parts and page of patents agreement to participate in the present study, which as sociodemographic information was included; age group, gender, marital status, level of education, occupational, economic, part two consist of medical information was comprised of (15 ) items, which deals(The past and present medical history), and the Indian Vision Function questionnaire.(IND -VFQ) which consist of (33-

INTRODUCTION
Thalassemia is the most common hemoglobin disorder in the world and thalassemia major and intermedia stand among the most severe forms(1),β-thalassemia is one of the most common heterogeneous inherited single gene disorders.The disease results from one or more of 380 different mutations in the β-globin gene (2), thalassemia major is the most severe form of beta-thalassemia leading to severe anemia and patients are in need of blood transfusion since the young age; this type may end in heart failure, due to iron overload, or early death in childhood in case of no transfusion.(1), Iron is essential for all living organisms.Under normal conditions there is no regulatory and rapid iron excretion in humans and body iron levels are mainly regulated from the absorption of iron from the gut.Regular blood transfusions in thalassaemia can result in excessive iron deposition in tissues and organs.This excess iron is toxic, resulting in tissue and organ damage and unless it is removed it can be fatal to those chronically transfused(3), Desferrioxamine is used for the treatment of chronic iron overload, acute iron poisoning, and certain anemia's.Ocular toxicity secondary to prolonged treatment with desferrioxamine may result in night blindness, visual field constriction, cataract, pigmentary retinopathy and optic neuropathy (4).several complicationswith DFO have been reported, which were due to pharmacological activity and high dose toxicity with regard to both acoustic and visual effects, (2), like other chronic diseasesthalassemiaand their associated complications is affects different aspects and quality of patient life.Sothequality of life should now be considered an important index of effective health care.The prolonged survival of patients with thalassemia major as a result of the novel therapeutic strategies introduced in the last decade makes patient quality of life an important issue (5) Based on the World Health Organization's definition of health, quality of life is an integral part of health and well-being and the WHO recently recommended that vision related quality of life form part of assessments and evaluations of interventions in eye health, the relationship between impaired vision and thalassemia was developed as a results of chelation therapy in thalassemic patients.(6) Reduced visual acuity significantly reduces participation in social or religious activities, mobility, activities of daily living, and visually intensive tasks (7), and vision loss is impact on one's functional ability and quality of life .Assessing the impact of visual impairment on QOL can provide a comprehensive picture of the burden of visual impairment beyond clinical evaluation.(8)Our study investigated the changes occurring in overall quality of life in patients with thalassemia major as a results of visual defects association chelation therapy.

Objectives of the study: The study aims to
1-Assessment of visual problems of Patients with thlassemia major.2-To find out the impact of the visual problems on patients quality of life 3-To compare between quality of life for case and control study.

METHODOLOGY:
Design of the study: A descriptive design study was carried out inIbn-Al-baldi hospital and AL -Karama hospital, starting from 1 th November 2013 to the 1 st Jun 2014.The administrative agreement was obtained from the hospitals and the patients who participated in the study.
The study Sample: A non-probability (purposive) sample of 70 adults thalassemia major patients who have visual complication (Case) were attending to the hospitals, and 70 thalassemia major patients not have visual complication as a control study sample, the patients was diagnosed with visual problems The study Instrument: The study instrument was composed of three parts which associodemographic information was included; age group, gender, marital status, level of education, occupational, economic, part two consist of medical information was comprised of (15) items, which deals (The past and present medical history), and the Indian Vision Function questionnaire.(IND -VFQ) which consist of (33-items) to measures the quality of life.

Validity and Reliability:
The content validity of the instrument was established through a panel of (18) experts, the reliability of the items was based on the internal consistency of the questionnaire was assessed by calculating Cronbach s' Coefficient alpha which as= 0.73

Data collection:
The data collected by revision the patients chart to obtain information concerning a definitive diagnosis with thalassemia major and complain from visual problems for case study, and self report and structured interview technique used for clients who cannot read and write.

Statistical Analysis:
The researchers used the appropriate statistical methods for data analysis which include the descriptive data analysis, and inferential data analysis, by using SPSS version 19 RESULTS:  Table 2 revealed that the 40% of the case family was a mother and cousins is a carrier disease, 55.7% of the control study was a mother is a carrier disease in their family, 68.6% and 64.3% of the case and control groups respectively have regular desferral, 65.7%, 64.3% have both blood transfusion and desferral as a treatment respectively, and 41.4% of the case group have splenomegaly and 35.7% of control group have splenomegaly as a complication of disease.Table 3 presented that 47.1% of the study sample have visual problems since 5-20 years, 45.9%, 23% of these problems was cataract and myopia respectively, most of the study sample have defect in both eyes and 47.1% conduct eye surgery, 61.4% of them wears glasses, 54.3% feel batter after operation, and 61.4% of the patients returned to vision problems after short period from operation as a results of complication of disease and treatment.Table 4 presented that the physical function of control group batter quality than case group through the mean of means which as 3.8 for control than 1.2 for case group, the psychosocial status of control group batter than psychosocial status for case group through the mean of means which as 3.8 for control, and 1.5 for case, and the mean of means of visual symptoms for control group was 3.8 than 1.6 for case group that means the control group high quality than case group in visual status.Table 6 shows that there were highly significant between return of visual problems with psychological status of patients, significant relationship was appear between eye problems and patients wear class, and there were highly significant between patients feel batter after operation and their problems in both eyes at p≥ 0.05 value.

DISCUSSION:
The socio-demographic characteristics of present study for 70 thalassemia major patients (case) indicated that the 51.4% was males, the mean of score for age was ±28.5, 47.3% of them were single, 28.4% of them at higher education graduated, 28.4% was unemployed, 43.2% of the study with high income, and the characteristics of 70 thalassemia major (control) was 48.6% females, at ± 28.5 mean of age, 45.9% were continuous married, 18.9% of them was primary education graduated, 35.1% were unemployed, and 48.6% were high income (table 1).The present findings compatible with (9) results during determine the prevalence of ocular manifestation in their study on 80 thalassemia major patients, they revealed that (46males, 34 females) with age ranging between 6 to 16 years, have ocular involvements which as 85% of cases in the form of lens opacity, decreased visual acuity(45%), retinal pigment epithelium (RPE) mottling (25%), disc hyperemia(12.5%)and increased cup/disc ratio (37.5%) and these involvements were observed to be more in younger age.The lower levels of formal education were independently associated with poorer vision-specific mobility and more visual symptoms in thalassemic patients who have visual defect, these opinion agree with the findings of present study (10) Forty percent of the case family was a mother and cousins is a carrier disease, 55.7% of the control study was a mother is a carrier disease in their family, 68.6% and 64.3% of the case and control groups respectively have regular desferral, 65.7%, 64.3% have both blood transfusion and desferral as a treatment respectively, and 41.4% of the case group have splenomegaly and 35.7% of control group have splenomegaly as a complication of disease (table2) The splenectomy was performed in 17(33.3%)female and 15 (42.9%) male patients.Cardiac complications like dilated cardiomyopathy, congestive heart failure and arrhythmia were in 15 female patients (29.4%) and 9 male patients (25.7%).Also, diabetes mellitus found in 4(7.8%) female patients and 2 male patients (5.7%).(11) The presented findings presented that 47.1% of the study sample have visual problems since 5-20 years, 45.9%, 23% of these problems was cataract and myopia respectively, most of the study sample have defect in both eyes and 47.1% conduct eye surgery, 61.4% of them wears glasses, 54.3% feel batter after operation, and 61.4% of the patients returned to vision problems after short period from operation as a results of complication of disease and treatment.the ocular manifestation in multiple transfused betathalassemia major patients and assess the ocular side-effect of iron chelating agent, they assess that ocular involvement was observed in 58% of patients, Lenticular opacities were the most common ocular finding (44%), followed by decreased visual acuity (33%) (12).the prevalence of ocular abnormalities in multi-transfused patients with β-thalassaemia receiving desferrioxamine and to determine the association of abnormalities with the patients' age, serum ferritin level, haemoglobin concentration, and dosage and duration of treatment with desferrioxamine on Twenty-five thalassaemic patients receiving desferrioxamine and attending the day-care centre of a tertiary care hospital in Delhi, India, and 25 healthy age-matched controls were examined to determine the prevalence and pattern of ocular abnormalities.(13) physical function of control group in present study shows a batter quality than case group through the mean of means which as 3.8 for control than 1.2 for case group, the psychosocial status of control group batter than psychosocial status for case group through the mean of means which as 3.8 for control, and 1.5 for case, and the mean of means of visual symptoms for control group was 3.8 than 1.6 for case group that means the control group high quality than case group in visual status (table4 and 5) Sarah, et al., (2007) they funded in their study on 196 cases visually impaired from cataract and 128 population-based controls without visually impairing and assess their health related quality of life, they revealed that the cases had significantly poorer general functioning, psychosocial, overall eyesight and pain/discomfort scores than controls.(14) reduced visual acuity significantly reduces participation in social or religious activities, mobility, activities of daily living, and bilateral vision loss reduces the quality and length of life( 10) There were highly significant in present finding of the study between return of visual problems with psychological status of patients, significant relationship was appear between eye problems and patients wear class, and there were highly significant between patients feel batter after operation and their problems in both eyes at p≥ 0.05 value (table6).A bilateral vision loss reduces the quality and length of life.However, it has not been widely shown whether unilateral vision loss affects any aspects of quality of life.In comparison with people with normal vision, those with unilateral vision loss correctable by refraction did not have significantly poorer mean general health scores while those with non-correctable unilateral vision loss did have significantly poorer mean general health scores in the dimension of role limitation due to physical problems.Furthermore, people with moderate to severe non-correctable unilateral vision loss had significantly poorer mean physical and emotional health scores in three dimensions: "role limitation due to physical problems," "social functioning," and "role limitation due to emotional problems.(4)

CONCLUSION
Most of the study and control groups have regular desferral, high percent of them have both blood transfusion and desferral, most of the study and control groups have splenomegaly as a complication of disease, High percent of the study sample have visual complication for 5-20 years ago, most of them have cataract, most of the study sample have defect in both eyes and high percent of them was conducted eye surgery, the physical function of control group batter quality of life than case group, the psychosocial status of control group batter than psychosocial status for case group, and the visual symptoms for control group was better than case group RECOMMENDATION 1-Screening and genetic counseling for hemoglobin disorders should be an intrinsic part of health care in country, as recommended by the WHO

Table 1 : Distribution of Thalassemia Major Patients by Socio-Demographic Characteristics for Case and Control Study (NO=70)
graduated, 35.1% were unemployed, and 48.6% were high income.

Table 5
revealed that the quality of life for control group batter than quality of life for case groups by total mean of means of Indian Vision Function domains which as 42.04, and 15.14 for case.