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International Journal of Medicine and Public Health | Oct-Dec 2015 | Vol 5 | Issue 4 322
Bhosale, et al.: Impact of splenectomy on quality of life of children with -thalassemia
323 International Journal of Medicine and Public Health | Oct-Dec 2015 | Vol 5 | Issue 4
Bhosale, et al.: Impact of splenectomy on quality of life of children with -thalassemia
to 40, higher scores indicating higher QOL. The interview time The children are on regular follow-up. They visit the thalassemia
was 10-15 min. Following this initial exercise, the protocol was unit once a month for the 1st year following splenectomy and
critically assessed and validated by our statistician. It has been 2-3 monthly thereafter. Parents are informed about the immune-
found to have a good internal consistency and very low inter-rater
compromised status of these children and are instructed about
variance (variability) of 0.05-0.07. The standardized protocol/
healthcare precautions to be taken. School authorities are
scale [Table 1] has been followed for consequent interviews of
the study population. informed accordingly. Follow-up assessment aims to assess
their health and well-being, documents benefits of splenectomy
All children posted for splenectomy underwent assessment as per and a search is made to pick-up infections occurring because
the standardized protocol [Table 1] at the time of hospitalization for
of hyposplenism. Our longest follow up is to 5 years (mean
splenectomy (pretest) and 2-years following splenectomy (posttest)
follow-up of 2 years) postsplenectomy. Parents are counseled
for comparison of the composite QOL index. The pre- and post-
test values of composite QOL Index of these children were plotted about genetic transmission of the thalassemia trait, and antenatal
graphically [Graph 1], and the results of the same were analyzed counseling is also provided in the subsequent pregnancy
using paired t-test. whenever applicable.
International Journal of Medicine and Public Health | Oct-Dec 2015 | Vol 5 | Issue 4 324
Bhosale, et al.: Impact of splenectomy on quality of life of children with -thalassemia
Graph 1: Graph (Box and Wilson Plot) showing comparison of quality of life index scores pre- and post-operatively
325 International Journal of Medicine and Public Health | Oct-Dec 2015 | Vol 5 | Issue 4
Bhosale, et al.: Impact of splenectomy on quality of life of children with -thalassemia
patients and the benefits of splenectomy have been found to be the less Hb drop leads to less fatigue, increased physical growth and
unequivocal by one and all.[2,7-9] Various series by Targarona et al., improves the overall QOL of these children.[5]
Wolff et al. have detected decreased PRBC transfusion requirement
and less drop in Hb following splenectomy.[10,11] However, none Portal vein thrombosis is a recognized complication after splenectomy
of the previously published studies have taken into account and in b-thalassemia major due to the chronic hypercoagulable state
objectively assessed the impact of splenectomy on overall QOL of that exists in childhood thalassemia.[14] Various authors like Al-
children with -thalassemia. Ours is the first ever study of this kind. Hawsawi et al. have reported portal venous thrombosis in children
undergoing splenectomy especially within the 1st month following
Poddubnyi et al. have used the standard short-form-36 (SF-36) splenectomy.[14,15] We did not have any incidence of portal venous
scale in children with benign hematological disorders including thrombosis. However, we had one child with cerebrovascular
thalassemia. They found improvement in almost all parameters of thromboembolic complication as has been described by DeBaun
life quality after laparoscopic splenectomy.[12] However, we found the et al.[5] He responded well to Warfarin. The postsplenectomy
application of the SF-36 scale, which is a general indicator of health infectious complications noted were upper respiratory tract
status commonly used in adults to be tedious, time-consuming and infections, pulmonary tuberculosis, minor skin abscesses, dental
impractical in children as young as 5-16 years. Ware and Sherbourne, caries and urinary tract infections among girls, similar to the studies.
the inventors of the SF-36 scale themselves have utilized this scale However, we do not have detailed microbiological data of these
in patients above 14 years of age.[13] Based on the concept of SF-36 infectious complications. Wolff et al.[11] have reported mortality. Two
scale, we employed our own modifications and have formulated a deaths attributable to septicemia occurred in our study beyond 2
standardized protocol/scale [Table 1] for assessment of QOL of years following splenectomy, one being a seropositive child.
children with -thalassemia having symptomatic hypersplenism. It
records the rise in QOL and objectively documents the improvement The objective assessment of QOL focuses on what the individual
in overall QOL of these children following splenectomy. can do and is important in defining the degree of health.[16] In our
study, the composite QOL index rose from the preoperative level
The mechanisms by which splenectomy imparts its benefits can be of 13.5 to 31.3, 2 years postoperatively. There were many hurdles in
postulated as follows (Schematic Representation 1). By means of objectively calculating the QOL index, e.g., growth assessment of the
operative removal of large bulk of tissue eating away the blood cells, children 2 years postsplenectomy might be partly influenced by the
it decreases the requirement of PRBC transfusion therapy. Similarly, pubertal growth spurt since at least one-third of our children were
by reducing the number of PRBC transfusions per month, it prevents above 10 years at the time of postoperative assessment and since we
problems associated with transfusional iron overload, which is a major do not have pre and postoperative hormonal data for comparison
concern in patients with thalassemia. In addition, by maintaining of the same. However, growth assessment is definitely one of the
higher baseline Hb value and preventing frequent Hb drops, it major determinants of the overall QOL. Morsy et al. have studied
decreases iron absorption from the gut (chronic anemia produces an echocardiographic changes in splenectomized and nonsplenectomized
increase in iron absorption from the gastro-intestinal tract); thereby individuals.[17] They have described left ventricular diastolic dysfunction
decreasing the need for chelation therapy, which in itself is associated in splenectomized individuals having reserved left ventricular systolic
with long-term sequelae.[5] Removal of mechanical discomfort from dysfunction preoperatively. Also, they (splenectomized individuals) are
the massively enlarged spleen gives a feeling of general well-being and at a higher risk of developing pulmonary hypertension.[17,18] We had
helps in increased physical activity in these children. Furthermore, two-dimensional ECHO done on all children preoperatively. Many of
them had ejection fraction between 50% and 60%. Two-dimensional
ECHO done on follow-up has shown stabilization of cardiac function
in few of our children. However, because of technical problems, we
could not get two-dimensional ECHO in all children postoperatively.
In spite of above limitations simplicity, sensitivity, objectivity, good
internal consistency and minimal inter-rater variability are major
advantages of this particular standardized protocol/scale devised by
us. In addition, it is less time-consuming, requiring <10-15 min to
complete the entire assessment. Majority of our children are on regular
follow-up, and we plan to present an extended comparative study
of composite QOL index on long-term follow-up in near in future.
International Journal of Medicine and Public Health | Oct-Dec 2015 | Vol 5 | Issue 4 326
Bhosale, et al.: Impact of splenectomy on quality of life of children with -thalassemia
transplantation, peripheral blood stem cell transplantation, specific 9. al Hawsawi ZM, Hummaida TI, Ismail GA. Splenectomy in thalassaemia
major: experience at Madina Maternity and Childrens Hospital, Saudi
gene therapy[6] and fetal Hb augmentation[19] in these children to
Arabia. Ann Trop Paediatr 2001;21:155-8.
ease their life in future. 10. Targarona EM, Espert JJ, Cerdn G, Balagu C, Piulachs J, Sugraes
G, et al. Effect of spleen size on splenectomy outcome. A comparison of
ACKNOWLEDGMENT 11.
open and laparoscopic surgery. Surg Endosc 1999;13:559-62.
Wolff JA, Sitarz AL, Vonhofe FH. Effect of splenectomy on thalassemia.
Pediatrics 1960;26:674-8.
We gratefully acknowledge Dr. Nikhil Panse for his suggestions in drafting
12. Poddubnyi IV, Tolstov KN, Isaev AA, Iatsenko EA, Doniush EK, KozlovMI.
and presenting this manuscript. We acknowledge the help rendered by Life quality of children with hematological diseases after laparoscopic
Dr.S.P. Rao, Professor, Preventive and Social Medicine in validating the splenectomy. Khirurgiia (Mosk) 2007;7:41-4.
protocol followed in this study and for providing the graphical representation 13. Ware JE Jr, Sherbourne CD. The MOS 36-item short-form health
survey (SF-36). I. Conceptual framework and item selection. Med Care
of pre- and post-operative values of composite QOL indices.
1992;30:473-83.
14. Al-Hawsawi ZM, Haouimi AS, Hassan RA, Tarawah AM. Portal vein
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Int Surg 2002;87:269-73. Source of Support: Nil, Conflicts of Interest: None declared.
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