Beta-Thalassemia Prevention & Control Program in Zahedan University of Medical Sciences, 2017

Thalassemia is a Greek word that is derived from the two words “Thalassa” meaning the sea and “Emia” means blood and is called Mediterranean anemia or Cooley’s anemia. The disease is spread across the Mediterranean region, Africa, the Middle East, Asia, Europe and United States. Between 1.5% and 3% of the worlds population is the carrier of β-thalassemia gene. In South-East Asia, the prevalence of thalassemia carriers is 10% to 20%. It is estimated that there are about 80 to 90 million beta thalassemia carriers in the world. It is also estimated that at least 60,000 new beta-thalassemia cases are born in the world every year. Most of the cases occur in developing countries. Thalassemia has an autosomal recessive inheritance pattern. When parents are carriers of the β-thalassemia gene, one in four (25%) pregnancies will be affected.

Beta-Thalassemia Prevention and Control Program in Zahedan University of Medical Sciences, 2017

Reportfrom: ZAUMS Non-Communicable Diseases Control Department
Published on: 16 April 2018

Thalassemia is a Greek word that is derived from the two words "Thalassa" meaning the sea and "Emia" means blood and is called Mediterranean anemia or Cooley"s anemia.
The disease is spread across the Mediterranean region, Africa, the Middle East, Asia, Europe and United States. Between 1.5% and 3% of the world"s population is the carrier of β-thalassemia gene. In South-East Asia, the prevalence of thalassemia carriers is 10% to 20%. It is estimated that there are about 80 to 90 million beta thalassemia carriers in the world. It is also estimated that at least 60,000 new beta-thalassemia cases are born in the world every year. Most of the cases occur in developing countries.
Thalassemia has an autosomal recessive inheritance pattern. When parents are carriers of the β-thalassemia gene, one in four (25%) pregnancies will be affected.

About two hundred types of genetic defects have been identified for β-globin gene that 20% of these defects account for most of clinical cases of β-thalassemia. To implement disease prevention and control programs, target population mutations should be investigated. Consanguineous marriage increases the chance of homozygosity by doubling the number of mutated genes compared to the general population.
Beta thalassemia is the most common hereditary disease in Iran, and it is mostly scattered in the southern and northern regions of the country. It is estimated that, on average, between 4-5% of the population in Iran are carriers of β-thalassemia gene. According to the registration of cases in blood transfusion centers throughout the country, the number of patients in Iran is estimated to be 18,000. The expected incidence of beta thalassemia in Iran on average is one in 1,000 live births, annually. As a result of quality services and good management of cases, the average age of patients with beta thalassemia in Iran has increased from 15 years in 1997 to about 35 years in 2018.
The program for the prevention of beta-thalassemia was developed in late 1996, and was implemented in 1997. Since 1999, the new technological methods for the diagnosis of the affected embryos was used for all people, both in rural and urban areas.

The goals of Beta-Thalassemia Prevention and Control Program
In the Beta-Thalassemia Prevention and Control program, with the aim of preventing the occurrence of β thalassemia major, two specific objectives have been stipulated:
1- Identifying 98% of the couples who are carriers of Beta-Thalassemia in the country
2- Offering healthcare services to 98% of the identified carrier couples

Three strategies have been followed to achieve those goals:
1- Screening of couples for Beta-Thalassemia carrier status at the time of marriage
2- Identifying couples who have a child with Beta-Thalassemia and implementing a special care program
3- Identifying couples who have not been screened for thalassemia at any time during the marriage.

The implementation of the above strategies is done with the following operational aspects:
- Education (general, target group, healthcare workers)
- screening tests
- Genetic diagnostic test
- Genetic counseling
- Genetic care
- Applied genetics research

Beta-Thalassemia in Sistan and Baluchistan Province and ZAUMS
According to the Blood Transfusion Organization, there are currently 2600 Beta-Thalassemia patients in the province who have been receiving blood transfusion services from the blood transfusion centers of the province. According to the Blood Transfusion Organization, 60% of the blood in Sistan and Baluchistan is currently used for Thalassemia patients. The number of major thalassemia cases in the population covered by Zahedan University of Medical Sciences was 1562 cases by the end of 2017. From 2014 to the end of the year 2016, a total of 114 affected pregnancies with Beta-Thalassemia major had been identified and referred in a timely manner, of which 102 cases (89.5%) have resulted in abortion.
Among the most important measures taken in ZAUMS in the Beta-Thalassemia Prevention and Control Program, was a campaign for identification of carrier couples who had no history of Beta-Thalassemia screening tests in the rural and suburban areas, which was launched with the participation of Community Health Volunteers (CHVs) in June 2016.
Free of cost services such as PND tests, abortion services, and free accommodation for poor couples in Zahedan, for performing diagnostic tests, performing a campaign to identify couples carrying Beta-Thalassemia, and holding Incident Cases Committees in cities covered by ZAUMS.

Figure 1- Prevalence of Beta-Thalassemia gene by province

Figure 2- The frequency of identified cases of Beta-Thalassemia in ZAUMS by district, 2018

Figure 3- The trend of incident cases of Beta-Thalassemia in ZAUMS, 2004-2016

Figure 4- The proportion of Prenatal Diagnosis stage 1 & 2 tests performed in the identified couples who are carriers of Beta-Thalassemia in ZAUMS, 2013-2016

References:
1- Report on the results of the National Program for Preventing the Occurrence of β thalassemia major, 2016, Ministry of Health and Medical Education, I.R. of Iran
2- Comprehensive instruction book on the National Guidelines on the Prevention of β thalassemia major, 2015, Ministry of Health and Medical Education

News Group : News,Latest News
News Code : 17873

Keywords