Survey of blood transfusion-induced malaria and other diseases in Thalassemia patients from Solapur District (M.S.) India.

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1 7. CONCLUSION Over 125 patients affected by thalassemia live in Solapur District, Maharashtra, India. Thalassemia is a blood disease and is common in both sexes. Thalassemia was suspected in all these patients with age six months to eighteen years, the prevalence percentage of TM was more than the BTI, BTMi and SCT. The prevalence percentage of thalassemia in specific ethnic groups. In Solapur District the high frequency of thalassemic patients were observed in the area of Solapur City, Pandharpur Taluka and in South Solapur as compared to other Talukas. In our study, only one SCT patient was found, total BTI patients were sixteen, BTMi were four and TM were ninety eight. Hence the prevalence percentage of TM were very high in Solapur District as compared to SCT, BTI and BTMi. Patients present with pallor, poor appetite, poor growth, lethargy and irritability was observed. Only one mentally challenged female patient was observed. As thalassemia has an impact on the different psychosocial life aspects, it is very essential to provide psychosocial support, including progress of the disease in the patients and parents for clear understanding. In the present study, the age of thalassemia patients was in between the range of 6 months to 18 years. The mean age was around 8.75 years. The numbers of patients in the age group 6 months to 3 years are more. There were 19 Male and 15 female with t avarage 34(27.2%) patients in 6 months-3 years age group. Patients between 4-6 years, 12 Male and 6 female with avarage 18(14.4%); patients in 7-10 years, Male 22, female 11, avarage 33(26.4%); in years Male 9 female 11 total 20(16%); in between age Male7, female 3, avarage 10(8%); in years Male 4, female 6, avarage 10(8%), Maximum patients was: 34(27.2%) were in the age group of 6 months 3 years and minimum patients were : 10(8%) in both age group of and years age. The present study comprised of males and females. A higher incidence of thalassemia was observed in male than female. Thalassemia was the serious medical problem. In the present research work, the findings are the failure to thrive after six months of age, stunting in height and weight. It indicates majority thalassemic patients showed poor growth in poly-transfused beta thalassemia patients. The observations showed the consanguinity rate was very high 117

2 (>49%) in male and female. That indicates the need for implementing a comprehensive genetic preventive program for the eradication of thalassemia in Solapur District. As per the pedigree analysis of the patients observed, most of the parents were thalassemia minor. The majority (>22.4%) of thalassemia patients were illiterate with only 1.6% with a higher education. Only one case was reported under psychological burden. It is evident from the findings of our study that besides the clinical burden, thalassemic have to shoulder very low psychosocial impact associated with the disease. There was no any correlation between the psychological impact and the gender. Furthermore, the concept of non-directive genetic counseling is incompatible with a campaign against consanguineous marriage. The prevalence percentage was very high in Hindu and muslin as compared in Jain and Sindhi community. In the present study the non vegetarian thalassemic patients are more than the vegetarian. Rodent face and saddle nose are more in thalassemic patients as compared to maxillary protrusion, maxillary anterior teeth spacing and anterior open bite. Only one case was observed in mucosal discoloration. There was not any relationship between the complications and sex but skull and face deformities can be closely related to the patient's age. In conclusion the hematological parameters vary as compared to normal ranges. The normal WBC was 80.80% and below normal range percentage of WBC was observed nil in all patients as compared to high WBC count (>19%). RBC count above range was nil in both sexes. But in TM patients RBC count below normal was observed in majority patients (>95%). RBC indices showed microcytic anemia. TM is characterized by reduced Hb level (<7 g/dl). Hematological investigations revealed the low Hb levels <8.0 g/dl (falling further with increasing age and severity) with severe microcytosis and hypochromia and increase target cells in the peripheral film. All thalassemic patients hemoglobin, HCT count was very low, not a single patient was observed in normal and with high range. MCV count was very low (>99%) in majority patients. The MCH and MCHC readings was normal in majority patients but nineteen patients MCH and thirteen patients MCHC was observed below range. Overall the normal range platelet count is more than that of the high range observed in twenty four patients and below range was eight in thalassemia patients. 118

3 The pre and post transfusion normal hemoglobin and HCT count was nil in all patients but below range was observed in all patients, which is comparable to studies conducted by other authors. Electrophoresis is a gold standard for the diagnosis of thalassemia but it is not available at all places. Thus several attempts have been made to diagnose the condition by using red cell indices. Only the history of one thalassemic patient was showed Plasmodium vivax infection due to blood transfusion. Recently available kits are useful for testing the positive malarial cases and it helps to prevent transfusion-induced malaria, other diseases and other complications. This extensive and well-executed study concludes that SCT, BTI, BTMi, and TM protect individuals against acquiring malarial infection, They become symptomatic but develop very low parasite densities. However, the study also concludes that the risks of developing severe malaria, especially malarial anaemia, are reduced in people with SCT,BTI, BTMi and TM patients and they reduced risk of malaria-specific mortality. The HIV positive and VDRL positive percentage was nil in all thalassemia patients. The HBsAg positive observed 2.40%. The HCV positive were observed 8.80% due to blood transfusion. The mild liver enlargement was observed in nine patients and moderate enlarge liver was observed in twenty two patients. Liver function and hepatitis serology must be routinely screened in thalassemia patients on chronic transfusion. The hyperthyroidism was not seen in any thalassemic patients, but the alloimmunization was observed in one male and one female. A highly enlarged spleen was observed in only one patient, in sixteen patients spleen was mild enlarge and in thirty two patients spleen was moderately enlarge. Total fifteen patients were done splenectomy due to heavy iron overload by poly-transfusion. Total fifty four patients were given treatment with different drugs by suggestions of the medical expert, the drugs are namely anti TB drugs, Desirox-500mg, Asunra, ayurvedic treatment, Calcium-X; Kelfer, Calpol, Bevo, Dyton, Folvite, Insulin, Loser whichever necessary. For supportive treatment mainly by blood transfusion and medications is freely available in various blood banks at Solapur. 119

4 The age group of 6-10 years the mortality rate in male were 6.84%; female 0.00%, in the age between Years and years: male 2.73%, female 5.76%, avarage 4%. This leads to increased mortality during years of age. As a result, transfusion iron overload can cause increased morbidity and premature mortality in thalassemia patients. In conclusion, blood transfusion therapy and optimal safety of transfused blood are the key concepts in the protocol of treatment of thalassemia. The maintenance of red cell viability and function during storage, to ensure sufficient transport of oxygen, maintainance of appropriate hemoglobin level was essential. Avoidance of adverse reactions, including transmission of infectious agents like malarial parasites, other pathogenic protozoans, HCV, HIV, HBsAg, VDRL, microorganisms and other complications are essential. Intake of Milk, a rich source of calcium, should be at least 500 ml daily, particularly because it helps to prevent osteoporosis. In Thalassemia, although most of the iron overload is due to blood transfusions, a small amount of iron is also absorbed from the diet. The absorption of iron is higher when the hemoglobin is low. Iron chelators such as Desferal and Deferiprone not only bind iron but also some Zinc and excrete it in the urine. The main nutritional sources of Zinc are animal foods (meat and dairy products) and wholemeal cereals. In thalassemia, because of the excess iron in the body, there is a higher risk of oxidative damage. The main antioxidants are vitamin E, vitamin C, carotenoids and flavonoids are to be given to patients. Knowing the results of thalassemia screening can impact decisions for prenatal diagnosis before marriage and well before pregnancy. In conclusion this thesis offers various types of data that may be useful in management and prevention of thalassemia in India and abroad. Future research directions The goal for future research in thalassemia is not only to introduce new strategies of diagnosis and treatment of thalassemia but also to discover ways to prevent transfusioninduced malaria, HCV, HIV, HBsAg, VDRL and other complications in thalassemic patients. Current work deals with specific complications in thalassemia patients such as orofacial complications, consigunity, ethnicity, hyperthyroidism, Alloimmunization, Hb level at pre and post blood transfusions, anthropologic measurements, hematological study and transfusion-induced or transmitted pathogens, complications in liver, spleen, alloimmunization etc. Efforts should be towards promoting awareness of thalassemia 120

5 amongst the public and health professionals, and promoting the establishment and improvement of policies aimed at the prevention and medical treatment of thalassemia. 121