Results of the National Program for Prevention of β-Thalassemia Major in the Iranian Province of Mazandaran

Abstract

Prevention programs are considered to be a top priority in Iran because beta-thalassemia (beta-thal) major (TM) is the most common autosomal disorder in Iran, and in the Mazandaran Province in particular. The main strategies comprise providing appropriate information for the public and professionals, screening and counseling of families at-risk and screening of general population prior to marriage. Providing laboratories for prenatal diagnosis was the most recent step in the program. We report the results of our prevention campaign for the period 1993-2006 in order to assess the effectiveness of all actions in controlling thalassemia major. In 1993, 500 TM patients were registered at the clinic of the Boo Ali Sina Hospital, Sari, Mazandaran, Iran. From 1993 to 1996, on average of 50 new cases were added to the cohort annually, whereas from 1995 to 2005 the number of new cases declined to 35 per year. Furthermore, the patients' average age increased. Overall, 51% of couples at-risk, who received genetic counseling, decided not to marry. All at-risk couples who are married were counseled for prevention of unplanned pregnancies. Currently, 64% are using safe contraceptive methods of family planning, and 14% are no longer at-risk for further pregnancies, the rest remained at-risk for unplanned pregnancies. In conclusion, at the relatively low cost of premarital screening and genetic counseling, we have offered at-risk couples the possibility of preventing the birth of at least 600 undesired TM patients. Thus, a great deal of suffering and an unbearable financial burden has been prevented to patients and their families.

Delay in Diagnosis of Hemoglobulinopathies (Thalassemia, Sickle Cell Anemia): A Need for Management of Thalassemia Programs

“…The first study conducted by Samavat et al (14) in 2004, and another one in 2007 (15) showed a significant success in decreasing the rate of thalassemia newborns, which was further validated by other studies (16)(17)(18). Khorasani et al (16) in a study to evaluate the success of the program concluded that the TPP has significantly reduced the financial burden upon thalassemic patients and their families.…”

Consanguineous marriages

“…Carrier detection and genetic counseling programs have been very successful in reducing the birth prevalence of inherited disorders in some populations, such as in Iran (Khorasani et al 2008;Samavat and Modell 2004). These programs are most successful when they are sensitive to the cultural backgrounds of populations in which they are applied.…”

Carrier screening for Beta-thalassaemia: a review of international practice

“…3 Nevertheless, some countries including Iran, Saudi Arabia, Palestinian Territories and Cyprus have laws in place making premarital screening for haemoglobinopathies mandatory for all couples before they are given approval to get married. 9,[20][21][22][23][24][25][26] This raises ethical questions, as couples are unable to voluntarily decide for themselves whether they would like to undergo genetic screening. Thalassaemia carrier screening has been included as part of an existing mandatory premarital blood test in Iran since 1991.…”

“…Thalassaemia carrier screening has been included as part of an existing mandatory premarital blood test in Iran since 1991. [22][23][24] This form of mandatory premarital thalassaemia screening also began in the Gaza Strip in 2000 9 and in Saudi Arabia in 2003. 20 Carrier couples receive advice on the options available to them, one of which is cancellation of marriage, and they can then decide whether to marry each other.…”