Thalassemia incidence spreads sorrow

Popularity of consanguineous marriages, stigmatization of premarital testing snowballed cases of congenital diseases

PESHAWAR:

For tribal families merrily celebrating the union of two cousins in intracaste matrimony, the sheer excitement of deepening kindred ties camouflages the grievous repercussions of their decision, which haunt not only the new couple but also the congenitally diseased children born out of the contentious wedlock.

While some cultures in the world frown upon consanguineous marriages for their close association with incest, a large majority exhibits skepticism towards the customary practice in light of scientific studies which establish a clear link between blood-related spouses and the development of multiple hereditary diseases among their future offspring like thalassemia.

Thalassemia, an inherited blood disorder that usually arises in a child when both the biological parents are carriers of the recessive or mutated gene, compromises the body’s natural ability to manufacture the oxygen-carrying pigment, haemoglobin present inside red blood cells, leading a suffering child to develop severe anemia, which can prove fatal if left untreated.

Where countries like Cyprus, Iran, Saudi Arabia, and the United Arab Emirates strictly carry out genetic testing for thalassemia on all couples before marriage, in the hopes of curtailing the incidence of intergenerational disease, Pakistan’s maladroit enforcement of premarital screening through poor implementation of laws like the Khyber Pakhtunkhwa Preventative Health Act (2009) has only escalated the number of toddling patients requiring regular transfusions or bone marrow transplants for survival.

Haider Ali, a father from Mardan, brings his little daughter Gulalay, a thalassemia patient, every month to a private center in Peshawar for a critical blood transfusion. “Begging people for blood to save my beloved daughter’s life makes me feel extremely helpless. If only I had known that marrying my cousin would lead to this, I would never have agreed to it,” sorrowfully confessed Ali, who alongside his wife was a thalassemia carrier.

Read more: Thalassemia’s troubling rise haunts K-P

According to a report by the Pakistan Demographic and Health Survey in 2018, thalassemia patients comprise 11 per cent of Pakistan’s population, including 10 million carriers and 5000 newborn babies annually, with an average life expectancy of only 10 years, and a significant proportion of this number is reported from K-P.

“Every year thousands of thalassemia patients are reported from KP, since cousin marriages are especially common in northern areas. Despite the existence of the 2009 law requiring couples to test for genetic abnormalities before marriage, implementation continues to be a challenge,” said Dr. Tariq Khan, Medical Director at a non-governmental organization (NGO) working for the relief of thalassemia patients.

Dr. Khan’s statement holds value given the fact that in 2009, the K-P assembly had passed the Khyber Pakhtunkhwa Health Act, according to which a Nikah registrar was mandated to obtain the carrier test reports for thalassemia and hepatitis C of both the man and the woman before solemnizing their marriage and a violation of the above could result in a cancellation of the license of the registrar or the imposition of a fine of Rs10,000.

“Apart from the cooperation of Nikah clerics and registrars, implementation of the law also requires destigmatizing genetic screening among couples, who are generally reluctant to undergo such tests before marriage,” highlighted Dr. Qazi Shahbaz Mohyuddin, Deputy Medical Superintendent at Nishtarabad Hospital Peshawar, who further highlighted the need to raise awareness among the local population on the pertinence of congenital diseases like thalassemia.

Expanding on the same, Saba Saeed, a psychologist, underscored the importance of raising awareness on the pitfalls of institutionalized practices like cousin marriages. “Such discourse must be included in the provincial school curriculum in order to holistically tackle the growing incidence of thalassemia,” opined Saeed.

Speaking to the Express Tribune on the matter, Dr. Tariq Hayat, Project Director Integrated program for HIV, Hepatitis and Thalassemia said,” Special seminars in schools and colleges are spreading awareness on thalassemia. Once the new government is elected, we will work towards a successful implementation of the 2009 K-P Preventative Health Act,” assured Dr. Hayat.