THALASSAEMIA SITUATUION IN MALDIVES
Thalassaemia is a major socio – medical problem in Maldives.
Beta Thalassaemia is the type most common in Maldives. But with the improvement of laboratory techniques, homozygous and heterozygous states of alpha thalassaemia and abnormal haemoglobin disorders such as HbE, HbS, HbC and HbD cases are frequently seen.
It is not known when the first thalassaemia case was diagnosed in Maldives. The first few cases were diagnosed in the nearby country Sri Lanka approximately 20 years back. Awareness regarding thalassaemia was nil at the time. Many children die at the age of 5 to 7 years due to intercurrent infections, severe anemia and ultimately heart failure without being diagnosed or treated. A minority of severely anemic children who seek medical help were transfused without knowing proper diagnosis.
Diagnostic facilities were introduced around 1984. The government hospital in Maldives, the only one at the time, started diagnosing beta thalassaemia majors with HbA2 and HbF estimation by elution technique and macro column chromatography along with Hb estimation and peripheral smear cytology.
Two leading physicians in the work of thalassaemia presented two country reports based on the existing situation of thalassaemia in Maldives.
Dr.Bernadette Modell (UK), in 1988 estimated that 40 nre patients would be born every year in Maldives.
Professor Calogero Vullo (Italy), in 1992 estimated that 1 out of every 5 to 6 persons in Maldives is a carrier for Beta Thalassaemia.
Society For Health Education a local Non Governmental Organisation, conducted a survey in 1992 on 496 students (age group 14-20 yrs.) Based on this survey the beta thalassaemia carrier rate was estimated as 18.1%. One 120 births is expected to be a case of beta thalassaemia major. 1 in 30 marriages is expected to be between two beta thalassaemia carriers.
Based on the prevalence data of carriers, and if the birth rate does not change, approximately 60 to 70 new cases are expected each year.
The spread of thalassaemia may be due to the considerable in – breeding within the island communities although there is no convention of consanguineous marriages in Maldives. This may be due to confinement of the people to one’s island due to the difficult geographical situation.
Registration of thalassaemia cases at the beginning of 1992. Number of cases registered (as of September 15th 1998) are 326 and total number of deaths are 57. It is estimated that this number of registered cases is only a quarter of all the thalassaemia cases in Maldives.
Till the end of 1994, the diagnoses cases were managed in the OPD and later in a separate clinic in the government hospital ( Central Hospital) in Male’.
Due to the constant increase in the number of thalassaemia cases, the need arose to open a separate centre for their management.
Thus on 29th December 1994, the paediatric wing of the Central Hospital was converted to National Thalassaemia Centre (since a new hospital was opened the same year).
All the expences for blood transfusion and desferal therapy are covered by the government.
Cost of treatment per child per annum is $6000 (need to revise the figure)
In 1997, a Thalassaemia Advisory Committee was formed to give advice on different issues related to thalassaemia and its control.
NATIONAL THALASSAEMIA CENTRE (N.T.C)
National Thalassaemia Centre is a day care centre open for 12 hrs during 5 week days and 7 hrs on Saturdays.
When the centre started, it was manned by 19 staff members including 1 doctor, 2 staff nurse and 3 laboratory technologists. Today there are 46 staff members including 4 doctors