Table of Contents  

Y Hachim, Essa Al Nuaimi, Abd El Baky, Al Hashmi, Rahman, Mazroei, and Hamouda: Quality of life in patients suffering from thalassaemia in Ras Al Khaimah, United Arab Emirates

Introduction

Thalassaemia major is an inherited form of anaemia resulting from several genetic mutations leading to absent or reduced synthesis of the beta (β)-chain of haemoglobin. The consequences of this syndrome affect multiple organs and are associated with considerable morbidity and mortality.1

Beta-thalassaemia constitutes a major public health problem in the United Arab Emirates (UAE), and during the period 1989–2004 more than 850 patients were registered at the Dubai Genetics and Thalassaemia Centre.

DNA studies performed on more than 400 consecutive UAE national newborns, nearly 2000 adult college students and 800 randomly selected nationals have demonstrated that the frequency of the β-globin gene defect, including the β-thalassaemia βS gene and abnormal haemoglobin, is around 8.5%, which is one of the highest levels in the Gulf Region.2

Although medical advances in the treatment of thalassaemia have led to increased survival, some patients still suffer from disease complications. These complications and chronic treatment with transfusions and chelation can adversely impact patients’ quality of life (QoL).3,4 These patients are now facing newer challenges in obtaining higher education and securing employment as well as raising their own families and are also confronted with other problems such as bone disease, infertility and increased prevalence of psychiatric distress.5

Aim

The aim of this study is to determine the types of thalassaemia in Ras Al Khaimah and to measure the impact of thalassaemia and its complications on the QoL of patients and its correlation with different socioepidemiological factors.

Materials and methods

A cross-sectional study was carried out in children and adolescents with thalassaemia who received treatment at Saqr and Saif Bin Ghabash Hospitals from January to March 2012. The overall number of patients was 34, all of whom were diagnosed with thalassaemia major and four of whom suffered from both thalassaemia major and sickle cell anaemia.

In this study, QoL was assessed using the QoL Short Form questionnaire-36 items (SF-36) in 25 patients and 25 age- and sex-matched control subjects without thalassaemia. A bilingual individual applied the standard forward–backward procedure to translate the SF-36 questionnaire from English to Arabic, with some cultural adaptation. The patients or their parents were asked to fill in the SF-36 questionnaire after providing written informed consent.

The SF-36 questionnaire was chosen for its cability to evaluate both the physical and psychological components of QoL. This questionnaire assesses eight dimensions of health-related QoL (HRQoL), which relate to the physical and mental components of the individual’s health perception. Specifically, the domains ‘physical functioning’ (10 items), ‘role physical’ (which means role limitation owing to physical health problems, four items), ‘bodily pain’ (two items) and ‘general health’ (five items) are more related to the physical component, whereas the domains ‘vitality’ (four items), ‘social functioning’ (two items), ‘role emotional’ (which means role limitations owing to emotional problems, three items) and general ‘mental health’ (five items) are more related to the psychological component. Possible scores for each domain range from 0 (corresponding to the worst possible state) to 100 (corresponding to the best possible state). These eight domains can be grouped into two summary scores: the physical component summary (PCS) evaluates the patients’ perception of limitations or disabilities in self-care, physical, social and role activities, the presence of bodily pain and fatigue, whereas the mental component summary (MCS) evaluates the feelings of psychological distress, social and role disability because of emotional problems. The total score of these eight indices ranges from 0 to 100, with designations of weak (≤ 20), bad (21–40), good (41–60), very good (61–80) and excellent (> 81).6

All the socioepidemiological and clinical data relating to the patients were retrieved from the files of the patients.

The research was approved by the Research and Ethics Committee in Ras Al Khaimah Medical and Health Sciences University.

Data analysis

Analysis of data was carried out using Microsoft Excel version 14 (Microsoft Corporation, Redmond, WA, USA) and Statistical Package for the Social Sciences, version 13.0 (SPSS Inc., Chicago, IL, USA). Patients’ socioepidemiological data are presented in the form of percentages, means and standard deviations (SDs), and the summary scores of the QoL and the different domains are presented as mean and SD. The chi-squared test, analysis of variance and t-tests were used to assess the relationship between QoL and each socioepidemiological factor.

Result

Sociodemographic and clinical data

The overall number of patients was 34, all of whom were diagnosed with thalassaemia major and four of whom suffered from both thalassaemia major and sickle cell anaemia.

The mean age of the patients was 19 ± 9.8 years and the median age was 20 years. There were 18 male patients (52.9%), and most of the patients (76%) had been diagnosed in the first year of life, with only 24% being diagnosed after the first year. More than 50% received a blood transfusion at least 24 times per year, and only 24% received a blood transfusion fewer than 12 times per year.

The incidence of β-thalassaemia major was significantly higher in consanguineous marriage (92.3%) than in unrelated marriage (7.8%). Most patients (96%) had a positive family history of thalassaemia, and only 23% had a positive family history for other blood disorders. The average serum ferritin level of our patients was 1455.167 ± 404.5 ng/ml.

The sociodemographic profile and disease characteristics of patients with thalassaemia are shown in Table 1.

TABLE 1

Sociodemographic profile and disease characteristics of patients with thalassaemia

Criterion n %
Age group (years) (n = 34)
< 17 12 35.3%
17–24 9 26.4
25–33 13 38.2%
Sex (n = 34)
Male 18 53%
Female 16 47%
Nationality (n = 34)
Emirati 14 41.4%
Pakistani 8 23.5%
Omani 4 11.7%
Iraqi 2 5.8%
Jordanian 1 2.9%
Other 5 14.7%
Educational level (n = 29)
Not in school 7 24%
Primary school 9 31%
Secondary school 6 20%
College or more 7 24%
Age (years) at first diagnosis (n = 25)
< 1 19 76%
1–3 1 4%
3–5 4 16%
> 5 1 4%
Consanguinity (n = 25)
Consanguineous 23 92%
Unrelated marriage 2 7.8%
Family history of thalassaemia (n = 25)
Positive 24 96%
Negative 1 4%
Number of blood transfusion per year (n = 34)
< 12 8 23.5%
12–23 9 26.5%
≥ 24 17 50%
Number of times patients take chelation per month (n = 29)
< 5 9 31%
6–29 12 41%
≥ 30 8 27.5%
Splenectomy (n = 29)
Yes 10 34.5%
No 19 65.5%
Pretransfusion haemoglobin level (g/dl) (n = 30)
≥ 9 0 0%
≥ 7.5 and < 9 7 23.3%
< 7.5 23 76.6%
Serum ferritin level (ng/ml) 1455.16 ± 404.5 ng/ml

The SF-36 questionnaire found that most of the patients (53%) were in good health. Compared with 1 year previously, 48% said that their health had remained the same, whereas 36% said their health was now much better.

The mean physical functioning in our patients was 74.2 ± 13.28, role physical was 10 ± 28, bodily pain was 45.6 ± 16, general health was 46.92 ± 24.37, vitality was 53.2 ± 20.35, social functioning was 53 ± 20.19, role emotional was 34.66 ± 46.6 and mental health was 48.32 ± 16.7. The mean PCS was 39.49 ± 5.9 and the mean MCS was 37.22 ± 10.5.

Scores between 80 and 100, which indicate excellent QoL, for each of the eight indexes were achieved by five patients (20%) for physical functioning, by two (8%) for role physical, by one (4%) for bodily pain, by two (8%) for general health, by two (8%) for vitality, three (12%) for social functioning, by eight (32%) for role emotional and by only one patient (4%) for mental health (Table 2).

TABLE 2

The designations of thalassaemia patients according to QoL scores

Score Physical functioning Role physical Bodily pain General health Vitality Social functioning Role emotional Mental health
Excellent (> 81) 5 (20%) 2 (8%) 1 (4%) 2 (8%) 2 (8%) 3 (12%) 8 (32%) 1 (4%)
Very good (61–80) 15 (60%) 0 (0%) 1 (4%) 7 (28%) 8 (32%) 5 (20%) 0 (0%) 5 (20%)
Good (41–60) 5 (20%) 1 (4%) 18 (72%) 3 (12%) 4 (16%) 8 (32%) 0 (0%) 8 (32%)
Bad (21–40) 0 (0%) 0 (0%) 5 (20%) 10 (40%) 11 (44%) 9 (36%) 2 (8%) 11 (44%)
Weak (< 20) 0 (0%) 22 (88%) 0 (0%) 3 (12%) 0 (0%) 0 (0%) 15 (60%) 0 (0%)

Comparison between the QoL in the patients with thalassaemia major and those in the age- and sex-matched control group showed significant differences in all domains, with the highest difference in the role physical domain [which means role limitations due to physical health problems (−77.27)] and the role emotional domain [which means role limitations due to emotional problems (−37.34)] (Table 3).

TABLE 3

Mean and SD of SF-36 domains and summary scores among thalassaemia patients compared with the age- and sex-matched control group

Domain Thalassaemia patients, mean (SD) (n = 25) Control group, mean (SD) (n = 25) Difference P-value
Physical functioning 74.2 ± 13.28 90.11 ± 9.18 −15.91 < 0.001
Role physical 10 ± 28 87 ± 27.11 −77.27 < 0.001
Bodily pain 45.6 ± 16 80.68 ± 21.62 −35.06 < 0.001
General health 46.92 ± 24.37 73.08 ± 12.68 −26.16 < 0.001
Vitality 53.2 ± 20.35 73.66 ± 19.47 −20.46 < 0.001
Social functioning 53 ± 20.19 78.5 ± 20.89 −25.5 < 0.001
Role emotional 34.66 ± 46.6 72.00 ± 34.26 −37.34 < 0.001
Mental health 48.32 ± 16.7 76.76 ± 20.37 −28.44 < 0.001
PCS 39.49 ± 5.9 52.94 ± 3.84 −13.45 < 0.001
MCS 37.22 ± 10.5 49.10 ± 11.41 −11.88 < 0.001

Patients in the age group 17–24 years had a higher score in six of the eight domains and also a higher PCS score than the other age groups; however, there was no significant difference in the QoL according to the different age groups (Table 4).

TABLE 4

Quality of life scores of patients classified by socioepidemiological and clinical factors

Criterion n PF RP BP GH VT SF RE MH PCS MCS
Age group (years)
< 17 9 Mean 69.16 8.33 39.83 40.33 45 54.17 22.21 54 36.71 37.2
SD 15.94 20.41 4.35 15.38 13.41 24.57 40.36 24.49 2.28 13.59
17–24 7 Mean 77.14 28.57 54.29 49.71 54.29 50 42.86 48.57 43 36.77
SD 17.28 48.79 21.51 25.67 20.29 26.02 53.45 15.21 7.33 8.39
25–33 9 Mean 76.67 0 45.22 46.33 53.33 56.94 33.33 39.56 39.92 35.31
SD 9.013 0 17.25 30.32 26.33 16.67 50 6.14 5.23 10.55
P-value 0.525 0.175 0.302 0.805 0.702 0.825 0.752 0.224 0.145 0.938
Sex
Male 14 Mean 73.21 14.28 45.28 43.42 51.07 49.10 38.09 45.71 39.38 36.23
SD 14.75 36.31 18.07 24.57 21.31 16.60 48.66 15.58 6.66 10.94
Female 11 Mean 75.45 4.54 46 51.36 55.90 57.95 30.3 51.63 39.62 38.47
SD 11.71 15.07 14.07 24.50 19.72 23.89 45.83 18.36 5.12 10.50
P-value 0.676 0.375 0.912 0.430 0.562 0.310 0.685 0.403 0.919 0.609
Educational level
Not in school 7 Mean 68.57 0 38.28 35.71 45.71 44.64 19.04 45.14 36.27 33.75
SD 11.07 0 4.99 23.49 19.88 6.68 37.79 19.14 4.20 11.87
Primary 9 Mean 70 8.33 36.66 38.33 45 54.16 22.21 54 36.18 37.26
SD 15.16 20.41 8.38 18.01 13.41 24.57 40.36 24.49 2.88 13.53
Secondary 6 Mean 78.33 0 64.66 60.33 66.66 58.33 66.66 50.66 41.46 43.26
SD 7.63 0 31.00 29.29 23.62 31.45 57.73 12.22 7.01 9.65
More 7 Mean 87 40 48.2 58.8 60 60 40 51.2 46.18 37.74
SD 12.04 54.77 11.62 23.82 17.32 28.50 54.77 11.45 6.20 7.38
P-value 0.085 0.127 0.033 0.226 0.236 0.654 0.457 0.858 0.009 0.681
Blood transfusions per year
< 12 8 Mean 80 0 67 79.5 85 62.5 100 40 44.1 46.95
SD 0 0 18.38 3.53 7.07 0 0 0 2.12 0.070
12–23 9 Mean 67.14 7.14 37 28.28 40 42.85 4.75 38.85 37.17 29.34
SD 9.94 18.89 7.63 19.89 17.32 6.68 12.58 13.80 3.42 5.59
≥ 24 17 Mean 77 13.33 47.73 50.26 55 55.83 42.22 53.86 39.95 39.73
SD 14.85 35.18 16.53 22.21 18.51 24.48 49.54 17.68 6.98 11.28
P-value 0.242 0.799 0.050 0.013 0.015 0.311 0.021 0.122 0.334 0.038
Frequency of iron chelation per month
< 5 5 Mean 74 0 53.8 58 65 60 60 41.6 40.4 40.88
SD 11.93 0 15.75 26.31 27.83 18.54 54.77 6.69 4.04 11.13
6–29 14 Mean 70.71 0 43.28 41.07 44.64 52.67 21.42 46.28 37.67 34.78
SD 13.84 0 18.46 24.95 18.13 22.02 42.58 19.82 5.83 12.03
≥ 30 2 Mean 90 100 48 56 70 37.5 100 62 48 44.5
SD 14.14 0 8.48 1.41 7.07 17.67 0 2.82 4.10 3.81
P-value 0.197 NAN 0.520 0.374 0.092 0.457 0.05 0.380 0.05 0.400
Splenectomy
No 18 Mean 73.61 2.77 47 50 54.16 55.55 31.47 49.55 39.24 37.78
SD 12.81 11.78 17.91 24.99 19.57 20.21 44.97 18.05 5.96 10.59
Yes 7 Mean 75.71 28.57 42 39 50.71 46.42 42.85 45.14 40.12 35.75
SD 15.39 48.79 10.37 22.41 23.70 20.04 53.45 13.60 6.22 11.27
P-value 0.377 0.106 0.198 0.153 0.369 0.164 0.314 0.25 0.376 0.344

BP, bodily pain; GH, general health; MH, mental health; NAN, not a number; PF, physical functioning; RE, role emotional; RP role physical; SF, social functioning; VT, vitality.

Women had a higher score on all domains than men, except for role physical and role emotional. However, there was no significant difference in the QoL according to sex (see Table 4).

Patients with a higher educational level (secondary school or higher) had better QoL than those who had a lower educational level. This occurred in all domains except for mental health, for which the QoL was higher in those educated to a primary school level than in those educated to higher levels. There was significant difference between the QoL and educational level in the domain bodily pain and PCS (P < 0.05) (see Table 4).

Patients receiving fewer blood transfusions (< 12 per year) had a better QoL in six out of the eight domains than patients with more blood transfusions per year. There was a significant statistical difference in the bodily pain, general health, vitality and role emotional domains as well as in the MCS (see Table 4).

Patients who received regular administration of iron chelation (≥ 30 per month) showed a better QoL than the other groups in five domains (physical functioning, role physical, vitality, role emotional and mental health) and higher PCS and MCS. In addition, there was a statistical difference in the role emotional domain and PCS (P < 0.05) (see Table 4).

Splenectomized patients had a better QoL score than non-splenectomized patients in three domains (physical functioning, role physical and role emotional); however, there was no significant difference in the QoL between splenectomized and non-splenectomized patients (see Table 4).

Discussion

Thalassaemia major is usually recognized in childhood, at which time patients begin treatment; however, over the course of their lives, patients and their families are at risk of social and behavioural disorders. The probability of behavioural abnormalities in children with thalassaemia major has been estimated to be 1.6-fold higher than in healthy children.7

Therefore, the preservation of good QoL is one of the major targets in clinical management of patients suffering from thalassaemia and the importance of assessing HRQoL in patients with thalassaemia has been highlighted by different studies.5,8,9 It is believed that these assessments can provide complementary clinical information that can help the haematologist to assess the patient’s health status.10

Most of our patients (53%) were in good health and, compared with 1 year previously, 48% said that their health had remained the same, whereas 36% said their health was now much better. This is similar to the results reported by Dahlui et al.,11 who found that the general well-being of patients suffering from thalassaemia was not affected to any great extent.11

The highest score of all the domains was seen in physical functioning (74.2 ± 13.28), which is similar to the findings reported by Dahlui et al.11 and Safizadeh et al.,12 who explained that the reason for this finding was that these patients had been living with the disease since childhood and, thus, they were not working for a living and, as such, did not have high expectations regarding physical performance.11,12

The lowest score was observed for the role physical domain (10 ± 28), and this was also found by Safizadehet al.,12 who reported lowest scores in the general health and role physical domains.

There was a significant reduction, ranging from 11% to 77%, in all the domains of HRQoL in patients with thalassaemia compared with the age- and sex- matched control group. This was also reported by Ismail et al.,13 who found a reduction in the physical functioning, social functioning and role physical domains of patients with thalassaemia compared with a control group,13 and Garaibeh et al.,14 who found that mean HRQoL scores on all domains were significantly lower in patients with thalassaemia than in their healthy counterparts in Jordan.14

We observed that the QoL is higher in the age group 17–24 years than in older and younger age groups; other studies have also found that QoL decreases as age increases, possibly as a result of patients being able to value health more and realize how the disease limits their function as they get older.11 In addition, older patients suffering from thalassaemia were diagnosed during earlier decades and may have been receiving blood transfusions and chelation therapy for longer. Furthermore, oral chelation is a relatively new therapeutic option that may have a generational effect on HRQoL.15

Women had a better QoL than men, a finding that was also reported by Safizadeh et al.,12 who found women to have a higher score in all domains except for bodily pain.12 Although the problems resulting from thalassaemia and its treatment are similar in men and women, the expectations placed on men by society (such as managing a family and the income) are greater, and this may explain the differences.12

Patients with a higher level of education had a better QoL, and there was a significant difference regarding educational level and QoL in the bodily pain domain and PCS. A higher level of education is not only important in improving patients’ QoL, but also necessary to prevent further births of children with thalassaemia children by increasing patients’, and families’, awareness and knowledge of the disease.16

Quality of life was significantly higher in patients who had undergone fewer blood transfusions (< 12) than in the other groups, as patients who required more regular transfusions tended to suffer from a more severe form of the disease and were more likely to suffer from disease- and transfusion-related complications.15

Another possible explanation for the association between frequency of blood transfusions and lower HRQoL is that patients who received blood transfusions prior to QoL assessment were those with low pretransfusion haemoglobin levels, which is associated with a number of symptoms such as fatigue, general weakness and decreased mental alertness, and may lead to impaired QoL in several domains.

Comparing the patients according to the frequency of chelation showed that those receiving chelation more often (≥ 30 per month) had higher scores than other groups in seven domains, which was similar to the results reported by Dahlui et al.,11 who observed that QoL was higher as the dose of deferoxamine (desferrioxamine) increased. This could be explained by the lower proportion of complications caused by iron overload.11

Conclusion

Quality of life in patients with thalassaemia was much lower than in the age- and sex-matched control group. The most affected domains were the role physical and role emotional domains, meaning that patients felt limited in their physical and emotional ability as a result of the disease. Frequency of blood transfusion, regular administration of iron chelation and the level of education of our patients were the most important factors in determination of the QoL.

All patients with thalassaemia should undergo assessment of QoL so that interventions focusing on the affected domains can be implemented. In addition, patients with thalassaemia and their families often require support in many aspects, especially psychological support to prevent mental disorders.

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