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BETA-THALASSAEMIA CONTROL
SIR,-
Bianco et all have reported their results with beta-thalassaemia prevention in Latium,
Italy, achieving a 67% reduction in homozygote births.
Latium is a low-frequency area for thalassaemia, and better control has been
achieved in Ferrara (90%), Cyprus (77%), and in a Cypriot community in London (7701o),
where the condition is more common. In
Sardinia, an island with 1600 000 people, the frequency of ß-thalassaemia gene is about 1 heterozygote every 8 people.
In
the past ten years thalassaemia has been widely debated in Sardinia, with information in
newspapers and on television and radio, scientific meetings, the activities of a parents'
association, some mass-screening campaigns in limited areas, the possibility of voluntary
screening by some centres and the possibility of antenatal diagnosis at the Ospedale
Regionale per le Microcitemie, Cagliari. As a
result the incidence of homozygous thalassaemia at birth fell from 4 per 1 000 in 1975 to
2. 7 per 1 000 in 1983, for the island as a whole. In
southern Sardinia - i.e. in the more populated provinces of Cagliari and Oristano - the
reduction was greater. Since 1975 Prof A. Cao and his group have been running a big
programme of carrier screening and antenatal diagnosis.
In the southern provinces the birth-rate of homozygotes has fallen from 4.8
per 1000 in 1975 to 2.5 per 1 000 in 1983 and to about 1 per 1 000 in 1984.
Unfortunately,
many affected babies are still being born in some areas, because Sardinia lacks a
coordinated programme that might achieve success uniformly over the whole island. We have been working since 1977 in
Sulcis-Iglesiente, an area of southern Sardinia with about 138000 people, and we treat
most thalassaemic patients who live there. The
frequency of the ß- thalassaemia gene is about 0.065 in Sulcis-Iglesiente so 4.2 affected
babies for every 1000 born would be expected. There
are 14.7 couples at risk for every 1000 marriages.
We
began our prevention programme by a media information campaign complete with meetings with
physicians, teachers, community leaders, students, workers, and ordinary people. We tried to transmit simple, accurate information
about thalassaemia and its transmission, and prevention.
Many public figures helped us in this programme, the object being to make
people aware of the consequences of the disease so that they would ask for screening and
change their reproductive plans when a possible risk was found. One of us (G.Z.) wrote two
booklets, one addressed to educators Talassemia:
Educare per prevenire), the other to identified heterozygotes (Io sono microcitemico ... e tu?). However,
during this first phase we could not offer screening locally and this worried us, even
though we had excellent collaboration from the specialist hospital in Cagliari, 60 km
away. |
