Thalassemia: Epidemiology, Risk Factors, and Public Health Programs

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Introduction

Thalassemia is a genetic disorder of the blood (autosomal recessive disorder) that leads to the abnormal formation of haemoglobin. The condition creates an imbalance between the oxygen and haemoglobin in the patient’s body. Patients diagnosed with the thalassemia disease cannot produce enough haemoglobin; hence, they can suffer from severe anaemia. The organs of such a patient are deprived of oxygen. Consequently, they cannot function effectively. The disorder is referred to as ‘autosomal recessive’ since parents with major and minor Thalassemia coupled together with the beta Thalassemia genes have either a higher chance of about 25-percent of bearing children having Thalassemia major or 50-percent likelihood of getting a child with Thalassemia minor. The probability of bearing a child without thalassemia stands at 25-percent. This essay provides a critical analysis of the epidemiology of Thalassemia, risk factors, and public health programmes addressing the issue to both the internal and local health agencies in the United Arab Emirates (UAE).

Purpose of the Essay

The purpose of this essay is to analyse the epidemiology of Thalassemia, risk factors, and public health programmes addressing the issue to both the internal and local health agencies in the UAE. Immigration due to jobs and cross-border intermarriages is rampant in the UAE population. This situation has significantly contributed to blood disorders such as Thalassemia. About one in every 12 persons is currently a carrier of the disorder. The prevalence of the malady is currently a national concern. Its prevalence is highest in the UAE as compared to other countries worldwide. The disease is predominant among the adolescents in the UAE. This situation is likely to pose an adverse effect on the social and economic aspects of the UAE community (Vichinsky, 2005). Individuals within the age bracket constitute about 16-percent of the total population. Various researchers have noted an amplified pervasiveness of the mutant alleles responsible for the development of the alpha and beta-Thalassemia disorders in the UAE due to admixture.

The UAE community also practices consanguinity marriages whereby individuals of the same blood (relatives) join in matrimony. This situation is a predisposing factor to the thalassemia disorders. However, the lack of clear reports on the frequencies of the disorders in the UAE significantly affects its maintenance patterns. This set of circumstances increases the risk of bearing a homozygous and heterozygous offspring. Eventually, there can be a higher financial burden to both the individuals and government (Vichinsky, 2005). Furthermore, there is limited information regarding the prevalence of the malady despite the fact that its analysis is mostly based on quantitative studies. This situation results from the small-scale studies that are conducted without clear national information concerning the disorder (Vichinsky, 2005).

Statistical Results and Information about Thalassemia in the United Arab Emirates

Epidemiology of Thalassemia in the UAE

Two types of the disease namely Alpha and Beta Thalassemia exist. The body needs to have both the alpha and beta-globin to manufacture haemoglobin. The Beta Thalassemia occurs when one or both of the required genes for the formation of the beta-globin are non-existent or function ineffectively. When one of the genes is damaged, the individual can suffer from a minor Thalassemia. This condition can occur when a person acquires a normal gene from one parent and a Thalassemia gene from the other. The condition is revealed through signs and symptoms that include loss of appetite, whitish skin, irritation, and retarded growth among others. This symptoms appear in the first two years of infection.

Alpha Thalassemia is a condition that develops in case the patient lacks or has deformed alpha-globin components. This situation leads to a reduction in the size of the red blood cells. Patients experiencing such health conditions do not show any symptoms. As a result, they cannot be treated; hence, they are regarded as carriers. If the two genes are missing or damaged, a mild anaemia that requires treatment occurs. This situation is identified as the alpha Thalassemia trait. When the three genes are missing, the haemoglobin H disease occurs. This condition raises a need for a blood transfusion. In case the four genetic components are absent, the patient develops alpha Thalassemia major. The disease can be treated through a routine administration of blood transfusion alongside other therapies.

Although Thalassemia is infrequent in countries such as the United States, it is often noted in some Asian countries and the Mediterranean regions among others. Recent statistics indicate that about 350,000 births per year come with serious blood conditions that are related to the Thalassemia disorder. An increase of the disorder is exhibited in the United Arab Emirates due to the prevailing immigration patterns among other factors. This disease is also common in regions where there is a higher rate of P. falciparum malarial infections. However, the Alpha Thalassemia is more frequent in the Southern China, Malaysia, and Southeast Asia. The Beta Thalassemia is mostly noted in the Mediterranean regions, Southeast Asia, and Africa (Taher et al., 2010).

A report released by the third and fourth Annual Meetings of the WHO Working Group for the Community Control of Hereditary Anaemia indicated that haemoglobinopathies and Thalassemia are the most prevalent single gene disorders among the UAE population with an increasing frequency of 9.9/1000 births. The leading contributing factors as indicated in the report are both admixture and immigration. Various studies on the disease indicate that the beta-Thalassemia is more prevalent in the Mediterranean countries, the Middle East, Central Asia, Southern China, and India among other countries in Africa and South America. Highest percentages of carriers have been noted in Cyprus (14%), Sardinia (10.3%), and in South Asia. This situation has resulted from the fact that the regions are prone to the Plasmodium falciparum malaria. An estimation of 1 in about 100,000 worldwide is noted to have the disease at birth. A report released by the Thalassemia International Federation (TIF) indicated that about 200,000 individuals are registered patients who are given routine treatment (Vichinsky, 2005).

In the UAE, marriages that are based on consanguinity and immigration are the main contributors to the disorder. It is estimated that about 1 out of 12 people is a carrier of the disorder in the country. Past researches have also indicated about 16.7 percent and 1.7 percent adults in the UAE suffer from the Alpha and Beta Thalassemia respectively (Baysal, 2011). Symptoms of anaemia, G6PD deficiency, sickle cell traits, and beta-Thalassemia are more prominent among the children with pre-school children mostly affected (Baysal, 2011). It is further noted that the prevalence of the disease in this age category in the UAE is highest worldwide (Baysal, 2011).

Risk Factors of Thalassemia in the UAE

Economically, the UAE oil and tourism industries have increased the national revenue significantly. The country is ranked as one of the top twenty world economies in relation to the gross national income per capita. Due to the increased economic development and technology in the region, it has been experiencing an increased immigration of workers in the region. According to the 2005 national statistics, the population stood at about 20.1 percent with the rest coming from immigrants (Colah, Gorakshakar, & Nadkarni, 2010). A change in the lifestyle, environment, and social inclination in the region has increased likelihood of the risk factors to result in the Thalassemia disorder. There are increased social risk factors such as genetic-related predisposition due to marriages within families and influx of immigrants with various health threats such as smoking among others. It has been noted that the consanguineous intermarriages occur due to the culture and religious background that allow the action as one of the social practices valued in the UAE (Colah et al., 2010).

Public Health Programmes addressing the Issue to both the Internal and Local Public Health Agencies in the U.A.E

The healthcare standards in Dubai offer high quality services due to the increased government spending on the sector. The total state expenditure in the UAE was approximately US $436 million between 1996 and 2003. A report released by the World Health Organisation in 2004 indicated that the total expenditure of about 2.9% of the Gross Domestic Product. Furthermore, the World Bank has also indicated that the Dubai and Abu Dhabi Cities are the most prominent in the Middle East with highest medical tourism medication (Matar et al., 2011).

In 2009, the government discovered about 119 genetic disorders among the Emiratis. This figure represented the highest cases of genetic disorders in the region. The autosomal recessive ailments are common amongst haemoglobinopathies. The beta Thalassemia disorder is has also raised a major concern among many policymakers and health practitioner’s in the region. The prevalence of the disease stands at a frequency of 8.5 percent. It is indicated that 1 out of every 12 persons is a Thalassemia carrier. About 10,000 people had been taken for premarital tests in the Genetic and Thalassemia Centre by 2005 (Baysal, 2011). This situation led to a tremendous increase in the health sectors with over 40 public hospitals opening in the seven emirates. These centres include hospitals, medical, and trauma centres. In 2007, a state-of-the-art general hospital was launched in Abu Dhabi. The facility has a bed capacity of 143 patients, a trauma unit, and home-based healthcare programmes. The Dubai health care city is categorised as a hospital free zone that offers international advance standards for private healthcare and academic medical training (Baysal, 2011).

Premarital Screening

In 2006, the UAE government made a number of mandatory premarital tests for legal marriages to ensure that the country is protected from having children suffering from the genetically acquired diseases such as Thalassemia. The screening programme is part of preventive campaign that is developed in the country against Thalassemia (Matar et al., 2011). A prenatal diagnosis is also done to ensure that the genetic makeup of the foetus is detected and diagnosed for any carrier characteristics. The government used a principle of a 25-percent chance of getting a child with Thalassemia by a carrier parent or 50% likelihood of getting an uninfected child to identify 28 positive individuals in over 115 cases at the Genetic Centre in the country. The diagnosis came with various benefits of early detection and management, as people were relieved from the financial constraints that arose from bearing an infected child (Matar et al., 2011). Information on issues related to blood transfusion can also be clarified by ensuring an earlier diagnosis of a foetus. Such practices have been accomplished through the well-established DNA laboratory centre that works together with the high-tech cytogenetic test and analysis laboratory stations in the UAE to further clarify the metabolic conditions (Matar et al., 2011).

Although such programmes have been put in place, the government lays more emphasis on the premarital screening instead of the prenatal tests as a strategy for the prevention and control of the Thalassemia disorder. A mandatory screening before marriage is preferred to ensure that the issues of pregnancy termination that are against the religious beliefs of the society are alleviated (Matar et al., 2011).

New-Born Screening as a National Initiative Programme in the Country

Screening for the Thalassemia disorder is performed through high performance liquid chromatography (HPLC) tests to determine the presence of abnormal haemoglobin in the body after birth. In most cases, the babies normally have higher foetal haemoglobin Hg F with Hgb S and no adult haemoglobin. A confirmatory test is always performed after 90 days in cases where the parents had a blood transfusion. Furthermore, the government also provides genetic counselling programmes to the parents and other patients concerning the conditions to reduce the development of stress. The counselling also has a purpose to protect future pregnancies since the mothers are trained in what to do in such cases. This situation can help in the prevention of terminating pregnancies (Matar et al., 2011).

Blood Donation Initiative and other Campaign Programmes

The government of the UAE in conjunction with the Al Naboodah National Contracting Group LLC has been in the forefront to encourage blood donation. These donations are conducted in the universities, shopping malls, and hospitals among other designated areas. The initiative is conducted to ensure that the anaemic patients are supplied with adequate amounts of blood, especially the ones suffering from the Thalassemia disorder (Matar et al., 2011). Other campaigns include the ‘Future without Thalassemia’ movements conducted during the celebration of the International Thalassemia Day all over the country. This campaign is meant for creating the awareness of the disorder and measures of prevention (Matar et al., 2011). The blood donation programme is also supported by the Emirate Central Cooling Corporation (ECCC) to assist children with the Thalassemia disorder (Matar et al., 2011).

Conclusion

The essay has elaborated the epidemiology, risk factors, and public health programmes that have been put in place to curb the prevalence of the Thalassemia disorder in the UAE. It is noted that the condition has become a public concern. The government of the UAE has put various programmes in place as a step towards the alleviation of the malady. Most of the interventions of the UAE government focus on premarital screening as a sure solution to the problem. Based on the analysis, there is a need to conduct more evidence-based approaches to the implementation of such interventions in the near future.

Reference List

Baysal, E. (2011). Molecular basis of β-thalassemia in the United Arab Emirates. Haemoglobin, 35(5-6), 581-588.

Colah, R., Gorakshakar, A., & Nadkarni, A. (2010). Global burden, distribution and prevention of β-thalassemias and haemoglobin E disorders. Expert Review of Haematology, 3(1), 103-117.

Matar, M., Naveed, M., Salim, S., Hareb, N., Alba, E., Hino, M.,…& Hattori, Y. (2011). Internet‐based approach to population screening for common haemoglobinopathies in United Arab Emirates. Nursing & health sciences, 13(2), 105-113.

Taher, A., Musallam, K., Karimi, M., El-Beshlawy, A., Belhoul, K., Daar, S.,…& Cappellini, M. (2010). Overview on practices in thalassemia intermedia management aiming for lowering complication rates across a region of endemicity: the OPTIMAL CARE study. Blood, 115(10), 1886-1892.

Vichinsky, E. (2005). Changing patterns of thalassemia worldwide. Annals of the New York Academy of Sciences, 1054(1), 18-24.

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