Cultural Preferences and Limited Public Resources Influence the Spectrum of Thalassemia in Egypt Amira A. M. Adly, MD and Fatma Soliman El Sayed Ebeid, MD Background: Cultural beliefs of Egyptians with respect to the origin of thalassemia and its prevention, as well as national resources available for care, often diﬀer from those of Western countries. Objectives: To assess the impact of cultural attitudes and the eﬀect of limited medical and ﬁnancial resources that could aﬀect the management of Egyptian thalassemic patients. Subjects: A cross sectional study included 205 Egyptians b-tha- lassemia major (b-TM) patients, with a mean age of 149 ± 87.90 months and a male to female ratio of 94:111. Methods: Demographic data stressing on order of birth, con- sanguineous marriage, and family history of b-TM, transfusion, and chelation therapy, were reported. HCV-Ab, HBV-Ag, and complete blood count were recorded with calculation of mean pretransfusional hemoglobin. Results: The age distribution was relatively nonhomogenous, with 39% of patients between 10 and 20 years of age and 16% were younger than 5. There were high family birth rates and 35% of patients were third or more in order of birth and a marked cultural preference for consanguineous marriage, representing 61% of all the parents’ marriages, as well as a high rate (59.5%) of a positive family history of b-TM. Patients transfused on low pretransfusion hemoglobin levels around 8 g/dL, and those receiving blood transfusion before the establishment of National Blood Trans- fusion Services showed a statistically signiﬁcant higher rate of positive hepatitis B and C viral infections. Chelation therapy tended to start at late age, mean age was around 4 years. Before 2000, subcutaneous deferoxamine was the most widely used che- lation, and since then a considerable number of patients (50%) had started to use oral iron chelators. Conclusions: The strong cultural preferences for consanguineous marriage and limited preventive programmes and resources have had a negative impact on the management of Egyptians thalasse- mic patients. Key Words: Egypt, thalassemia, cultural preferences (J Pediatr Hematol Oncol 2015;37:281–284) T halassemias are the most common monogenetic dis- orders worldwide and represent a signiﬁcant public health concern for developing countries. 1 The epidemiology of the disease is changing due to a fall in total birth rates, prevention programs, recent patterns of population move- ment, and migration. 2 Patients with b-thalassemia major (b-TM) present in the ﬁrst year of life with profound anemia and subsequently require regular blood transfusions for survival, as well as iron chelation therapy to treat transfusional iron overload and prevent end-organ damage. 1 This modern management strategy generates high costs to society but is eﬃcacious in clinical terms. Eﬀorts must be made to improve patient compliance to improve the eﬀectiveness of treatment; hence, this would allow a more appropriate use of available resources. 3 Consanguinity is a deeply rooted social trend among one ﬁfth of the world’s population and it is estimated that 1 billion of the current global population live in communities with a preference for consanguineous marriage. 4 Con- sanguineous marriage is a respected tradition observed in most communities of North Africa, the Middle East, and West Asia, where intrafamilial unions collectively account for approximately 20% to 50% of all marriages. 5 For example, ﬁrst cousin marriage of the parents was found in 56% of couples and 19.8% were relatives among Pakistani thalassemic patients. 6 Mounting public awareness con- cerning the prevention of genetic disorders in oﬀspring is driving an increasing number of couples contemplating marriage and reproduction in highly consanguineous communities to seek counseling on consanguinity. 7 Cultural and religious beliefs, the organization and characteristics of the health care system, knowledge, and previous experience with the disease, all inﬂuence attitudes toward reproductive and child bearing choices. 8 We aim to address the eﬀect of Egyptian cultural attitudes about consanguineous marriage and family planning, as well as how limited resources aﬀect the management of thalasse- mia, especially the availability of blood transfusions and iron chelation therapy. SUBJECTS This is a cross-sectional study, which recruited 205 patients with b-TM from regular attendees of the Pediatric Hematology Clinic, Ain Shams University, Cairo. Their diagnoses were based on clinical and laboratory hemato- logic parameters as well as hemoglobin electrophoresis. The study was approved from the Medical Ethical Committee of Human Experimentation of Ain Shams University. Informed consent was obtained from parents or legal guardians. METHODS Retrospective reviews of the ﬁling system with self- reported history during the regular follow-up visits stressed on the following: Received for publication August 7, 2014; accepted February 19, 2015. From the Thalasemia Centre, Pediatric Hematology/Oncology Department, Ain Shams University, Cairo, Egypt. The authors declare no conﬂict of interest. Reprints: Fatma Soliman El Sayed Ebeid, MD, Pediatric Hematology/ Oncology Department, Children’s Hospital, Faculty of Medicine, Ain Shams University, 21 Fakry Mohamed Ahmed, Elzawia Elhamra, Cairo, 99110, Egypt (e-mail: dr.fatma_ebeid@ yahoo.com). Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved. ORIGINAL ARTICLE J Pediatr Hematol Oncol  Volume 37, Number 4, May 2015 www.jpho-online.com | 281