Thalassemia and Its Problems in the Adolescent Age

I. Wahidiyat; Maria Abdulsalam; A.H. Markum; Muslichan Mz.

doi:10.14238/pi23.5-6.1983.85-94

I. Wahidiyat Department of Child Health, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta
Maria Abdulsalam Department of Child Health, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta
A.H. Markum Department of Child Health, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta
Muslichan Mz. Department of Child Health, Universitas Indonesia/Cipto Mangunkusumo Hospital, Jakarta

DOI: https://doi.org/10.14238/pi23.5-6.1983.85-94

Keywords: thalassemia, physical, emotional, social, face deformity, growth disturbance, skin discoloration

Abstract

Due to better facilities and management in facing thalassemic children it is expected that there will be more surviving patients reaching the adult age. But on the other hand there appears many new problems, such as physical, emotional and social problems. Forty cases of thalassemia major and thalassemia Hb E disease who reached the adult age were discussed in this paper. Face deformity, growth disturbance and skin discoloration were seen in 82,5.%, 95% and 85% of the cases respectively.

Inferiority complexes and physical disability were found in about 50% of them. Frequent mild to moderate infections were seen in 25% of the patients. No one suffered from severe or overwhelming infections. Twenty seven (27) out of 40 cases had been splenectomized at the age of more than 5 years. Only 4 cases (10%) showed signs of puberty. The growth disturbance might be due to 2 factors a) failure in maintaining the hemoglobin content at about IO g%, and b) disturbance of gonadotropin hormone due to iron overload. It is therefore highly recommended to put the child at high transfusion regime as early as possible and administer to the child regularly iron chelating agents to prevent the iron overload. Every infection should be promptly eradicated especially in those whose spleen have been removed.

Depression which is common among them should be overcome by giving equal opportunities in educations, vocational training and employment. They should be helped to become integrated into adult society.

References

1. BAKER, D.H. : Roentgent malliifestations of Cooley's anemia. Ann. N.Y. Acad. Sci. 119 : 641 (1964).
2. BETKE, K.; MARTI, H.R.; SCHLICHT, I. : Estimation of small percentages of foetal haemoglobin. Nature, Lond. 184:1877 (1959).
3. CAFFEY, J. : Pediatnic XÂ·Ray diagnosis; 3rd ed., pp. 77-81 (Year Book Pubis. Chicago 1957).
4. DJUMADIAS, A.N.; PRA WIRANEGARA, D.; LUBIS, B.A. : Heights and weights of healthy Indonesian School Children and youth. Eleventh Science Pacifie Congress, Tokyo (1969).
5. ELLIS, J.T.; SCHULMAN, 1.; SMITH, C.H. : Generalized siderosis with fibrosis of liver and pancreas in Cooley's (Mediterranean) anemia: with observations on the pathogenesis of the siderosis and fibrosis. Am. J. Path. 30 : 287 (1954).
6. JOHNSTON, F.E.; KROGMAN, W.M.; Patterns of growth in children with thalassemia major. Ann. N.Y. Acad. Sci. 119 : 667 (1964).
7. JOHNSTON, F.E.; HERTZOG, K.P.; MALINA, R.M. : Longitudinal growth in thalassemia major Am. J. Dis. Child. 112 : 396 (1966).
8. KANAKOUDI-TSAKALIDIS, F.; SPYROGLOU, K.; TZAFI, R.; CASSIMOS, C. : Effect of blood transfusion on the immune response of children with thalassemia. Acta haemat. 57 : 65 (1977).
9. KLETSKY, O.A.; COSTIN, G.; MARRS, R.P.; BERNSTEIN, G.; MARCH, C.M.; MISHELL, D.Jr. : Gonadotropin insufficiency in patients with thalassemia major. J. clin. En doer. Meta b. 48 : 901 Â· 905 (1979).
10. LOGOTHETIS, J.; ECONOMIDOU, J.; CONST ANTOULAKIS, M.; AUGOUSTAKI, 0.; LOEWENSON, R.B.; BILEK, M. : Cephalofacial deformities in thalassemia major (Cooeley's anemia): A correlative study among 138 cases. Am. J. Dis. Child. 121 : 300 (1971).
11. LOGOTHETIS, J.; LOEWENSON, R.B.; AUGOUSTAKI, 0.; ECONOMIDOU, J.; CONSTANTOULAKIS, M.: Body growth in Coolley's anemia (homozygous fHhalassemia) with a correlative study as to other aspects of the illness in 138 cases. Pediatrics 50 : 92 (1972).
12. SMITH, C.H.; ERLANDSON, M.E.; STERN, G.; SCHULMAN, I.: The role of splenectomy in the management of thalassemia. Blood 15 : 197 (1960).
13. SMITH, C.H.; ERLANDSON, M.E.; STERN, G.; HILGARTNER, M.W. : Post splenectomy infection in Cooley's anemia. An appraisal of the problem in this and other blood disorders, with 11. consideration of prophylaxis. New Engl. J. Med. 266 : 737 (1962).
14. SMITH, C.H.; ERLANDSON, M.E.; STERN, G.; HILGARTNER, M.W. : Post splenectomy infections in Cooley's anemia. Ann. N.Y. Acad. Sci. 119 : 748 (1964).
15. VALASSI-ADAM, H.: Immunoglobulin [evels in children with thalassemia. Arch. Dis. Childh. 48 : 828 (1973).
16. WAHIDIYAT, I.: Penelitian Thalassemia di Jakarta; Tesis, Jakarta (1979).
17. WEATHERALL, E.D.; CLEGG, J.B.: The thalassemia syndromes; 2nd ed., p, 278 (Blackwell Scient. Pubis. Oxford/ London/Edinburgh/Melbourne 1972).
18. WEINBERG, E.D.: Iron and susceptibility to infectious disease. Science 184 : 952 (1974).
19. WHO WORKING GROUP. Hereditary anaemias: genetic basis, clinical features, diagnosis and treatment. Bull. WHO 60: 043- 660 (1982).
20. WOLMAN, I.J. : Transfusion therapy in Cooley s anemia: Growth and health as related to long-range hemoglobin levels. A progress report. Ann. N.Y. Acad Sci. 119 : 736 (1964).
21. WOLMAN, I.J.; ORTOLANI, M.: Some clinical features of Cooley's anemia patients as related to transfusion schedules. Ann. N.Y. Acad. Sci. 165 : 407 (1969).
22 . ZAINO, E.C.; KUO, B.; ROGINSKY, M.S. : Growth retardation in thalassemia major. Ann. N.Y. Acad. Sci. 165 : 394 (1969).