Imagine a life in which you live for a mere 10-12 years and it is full of agony and misery. A life in which you have to stay put in a hospital bed 2-4 days a week for 3-4 hours with blood being injected in your body. It petrifies you to your core, does it not? Well, believe it or not, but this is what the life of a person who has thalassemia (major) looks like.
Thalassemia is a blood disorder that is passed down congenitally (inherited) (Medline Plus, 2020). It is a type of anemia. Thalassemia causes the body to yield an abnormal amount of hemoglobin - an iron-rich protein found in red blood cells whose task is to circulate oxygen. Hemoglobin is composed of 2 proteins namely alpha and beta-globin, and a fault in the production of either of the two proteins leads to thalassemia.
There are 2 types of thalassemia: alpha and beta thalassemia. Alpha and beta thalassemia are further divided into major and minor thalassemia. Thalassemia minor ensues when a person receives a faulty gene from one of the parents. People with thalassemia minor are just a ‘carrier’ of the disease, they don't have visible symptoms (Medline Plus, 2020).
Each year, 50,000 to 100,000 children are deceased due to thalassemia major in low and middle-income countries while about 7% of the world's population is a carrier (Ammad, et al., 2011). Carrier (thalassemia minor) rate in Pakistan is deemed to be between 5-8% whereas, nearly 5,000 new patients are diagnosed with thalassemia major every year (Hashim, et al., 2018). One of the main reasons that thalassemia minor matures into thalassemia major is inter-marriage - mostly amongst second-degree relatives who carry this disease.
Most of the affected are from lower-income backgrounds who barely have any knowledge of the repercussions of inter-marriages especially when this disease runs in the family, i.e. there are a lot of thalassemia minor carriers. When the two minors reproduce, there is a 25% chance that their offspring is infected with thalassemia major as the damaged gene is passed on from both the father and the mother (HealthHub, 2019). In order to avert marriage amongst two thalassemia minors, blood screening is a cardinal test that must be done. This test reveals if both or one of them have thalassemia minor or not (Arif, Fayyaz, & Hamid, 2008).
Family dynamics is also one of the reasons for the escalation of the disease as the elders and extremists in the family do not permit their sons and daughters to be tested and screened which increases the risk of thalassemia major amongst the progeny (Arif, Fayyaz, & Hamid, 2008).
The effects of thalassemia major on children are far complicated than what meets the eye. The fact that a child has to visit the hospital or transfusion centers (such as Afzaal Memorial Thalassemia Foundation) regularly for treatment means that he does not get enough time for other pursuits; such as studying, playing, and socializing, etc. This greatly affects their quality of life and their psyche (Ammad, et al., 2011). They dawdle socially and have a small number of friends due to which they cannot spend a lot of time with children of their own age. They also suffer from depression and the lack of adequate hemoglobin means that their energy levels are crucially low throughout the day. Thalassemia major children find it hard to focus on the any activity that they are doing. Their sleeping and eating patterns also get muddled. Additionally, the parents (of the affected child) also face anxiety and an increased economic burden as they shell out an enormous amount of cash to arrange blood and any necessary medical treatment and also have to worry about a steady source of income so they can cover these expenses (Ammad, at el., 2011).
Continuing the point of heavy economic burden, blood transfusion (which has to be done 2-4 times a week) costs Rs.30,000 per child per year, while, the treatment to clean the organs with leftover iron (called iron chelation) is Rs.150,000 per child per year (Shamsi, 2013).
There are umpteen blood banks (Hussaini blood bank, Fatimid center, and many more) that provide free blood and blood transfusion facilities to those who can't afford them (Ammad, at el., 2011). However, the caveat is that these organizations have limited resources due to which they can't provide high-quality trained staff and other important facilities that should be paired-up with blood transfusion in order to have an optimal impact (Arif, Fayyaz, & Hamid, 2008).
Thalassemia is now mostly reduced to third world countries as it has been curtailed significantly by the first world nations (Weatherall, 2010). If the maximum impact is to be attained, then the best course of action is that the government has to realize the magnitude and the repercussions thalassemia has, and then use its full potential to create awareness through different channels. At the same time, NGOs can also start campaigning so the awareness can reach the widest number of people collectively. One great campaign slogan that I came across was "no test, no nikkah". Secondly, NGOs can connect quality health care professionals with the public so that the public can get first-hand information about the disease from the professionals themselves. Additionally, more and more donations should be encouraged to the blood banks so that those who are affected can have sufficient amounts of blood to be transfused. Monetary help can be given to the affected so that they can have good quality treatment.
Ammad, S. A., Mubeen, S. M., Shah, S. F., & Mansoor, S. (2011, May). Parents’ opinion of quality of life (QOL) in Pakistani thalassaemic children. Retrieved July 22, 2020, from https://jpma.org.pk/article-details/2780
Arif, F., Fayyaz, J., & Hamid, A. (2008, November). Awareness among parents of children with thalassemia major. Retrieved July 22, 2020, from https://jpma.org.pk/article-details/1544
Hashim, S., Sarwar, M., Arsalan, A., Awan, I., & Naseem, S. (2018, January). Frequency of carrier screening and preventive orientation among first degree relatives of Thalassemia patients. Retrieved July 22, 2020, from https://jpma.org.pk/article-details/8520
HealthHub. (2019, August 14). Thalassemia Tests. Retrieved July 26, 2020, from https://www.healthhub.sg/live-healthy/952/pregnancy-thalassemia-tests
Medline Plus. (2020, June 2). Thalassemia: MedlinePlus Medical Encyclopedia. Retrieved July 22, 2020, from https://medlineplus.gov/ency/article/000587.htm
Shamsi, T. (2013, March). Medical management of b-thalassaemia without blood transfusion: A myth or a reality? Retrieved July 22, 2020, from https://jpma.org.pk/article-details/4048
Weatherall, D. (2010). Thalassemia as a global health problem: Recent progress toward its control in the developing countries. Annals of the New York Academy of Sciences, 1202(1), 17-23. doi:10.1111/j.1749-6632.2010.05546.x