Ahmed was in grade 6 when an incident at school led to him excessively bleeding from a fall. He began to have nose bleeds, sometimes in the middle of a regular school day which made him feel embarrassed and different from other children. At other times, his joints would hurt and swell. He began missing school more frequently and consequently, even though he was studious and bright, led him to fall behind in his studies. His parents were always worried. He was the cause of their stress, and yet in a way it had nothing to do with him. Because Ahmed had hemophilia - a rare inherited disease with no cure.
Essentially, hemophilia is a serious bleeding disorder where a person’s blood doesn't clot properly, leading to uncontrolled bleeding. In a healthy person, proteins called clotting factors work together to form a blood clot and help stop bleeding. People with hemophilia A either lack or do not have enough of a clotting factor called Factor VIII which leads to their blood not being able to clot properly. Hemophilia A is the most common form of hemophilia with 75% of those affected being children.
Symptoms of hemophilia include bruising; bleeding into muscles and joints, which if not treated can lead to long-term pain, swelling and stiffness; controlling bleeding associated with menstruation or childbirth for women, and excessive bleeding associated with menstruation or childbirth for women. Severe hemophilia can potentially be life-threatening and joint bleeding is more frequent and severe. People with severe hemophilia A also experience spontaneous bleeding. On a psycho-social level, it significantly reduces the quality of life of people not just of those affected, but their family, friends and caregivers as well.
The goal for management of hemophilia A is to eliminate all preventable bleeds regardless of disease severity. Prophylaxis is a preventative, regular treatment with the aim to prevent bleeds and allow people with hemophilia to lead active lives and achieve a quality of life comparable to non-hemophilic individuals.
Pakistan is a country facing a host of dire challenges in its healthcare ecosystem. And one such challenge is access to the diagnosis and treatment of hemophilia. Although there are roughly 20,000* Hemophilia A patients in Pakistan, only 3,500** are registered sufferers. There is also a severe lack of hemophilia management in both the public and private sectors and full-dose prophylaxis remains almost negligible. Treatment and care comes in the form of private Patient Welfare Societies, small-scale private NGOs in major cities, who provide services from diagnosis to patient counseling to hemophilia patients. These organisations have resiliently supported hemophilia patients for over 20 years. But despite all their efforts, hemophilia remains a neglected disease - on the outskirts of an already fragile health landscape. A lot more needs to be done so both adults, and children like Ahmed, can get access to the right care because the number of people affected shouldn’t affect how a disease is treated.
*WFH Guidelines for the Management of Hemophilia, 3rd Edition, 2020
**based on available data and acquired insights
Bilal Ahmed is the CEO of SPEAK Trust, a non-profit working to address public health inequities, gender based violence, human rights and HIV issues of migrants. All facts and information are the sole responsibility of the writer