Can Hemophilia, the Genetic Blood Disorder, Lead to Death?

Mr. Jagdish Sharma, VP of the Hemophilia Federation of India, speaks to Medindia about the ill effects of hemophilia, the rare genetic and life-threatening blood disorder.

Highlights:

Blood doesn’t clot in hemophilic patients due to the lack of clotting proteins
Anti-Hemophilic Factor (AHF) injection is the only possible treatment, but its costly and neither developed nor readily available in India
Excessive bleeding sometimes leads to death

New Hemophilia Drug Launched in India
In India, more than 1,36,000 people have hemophilia, which causes lifelong bleeding. However, Takeda Pharmaceutical Company has launched a new hemophilia drug.

‘You can die of hemophilia without even knowing about it.’

What is Hemophilia?

Hemophilia is a genetic and life-threatening bleeding disorder. Due to the lack of clotting proteins even with the slightest cut, patients’ blood does not clot normally. This ailment needs to be taken special care or else it leads to recurrent and prolonged bleeding into joints and muscles sometimes leading to permanent disability and even worse, to death at times.

Infusion of Anti-Hemophilic Factors (AHF), seems to be the only possible treatment that is quite expensive. (One unit of Factor costs approximately Rs. 10-12, and during any bleeding episode, a person requires 500 to 2,000 IUs in one shot, resulting in an average expenditure of Rs. 5,000 to Rs. 20,000.)

Hemophilia – Beating the ‘Bloody’ Disorder
Meet the ‘patient’ leader who reaches out to fellow hemophiliacs on World Hemophilia Day 2011 with the message that it is possible to lead a successful life with hemophilia, the bleeding disorder.

About Hemophilia Federation (India)

Hemophilia Federation (India) is a non-profit, organization working for the welfare of people suffering from hemophilia. They have a network of almost 90 Chapters spread across the country, aiming to reach out to more and more people suffering from hemophilia.

The organization also aims to provide quality care, affordable treatment, educational and psycho-social support, and economic rehabilitation. The Federation has been voicing about hemophilia for the last 39 years.

International Workshop on Hemoglobinopathies and Hemophilia at Bangalore
An international workshop was held in Bangalore in February 2018 to discuss about the current status of prevalent blood and bleeding disorders in India.

World Hemophilia Day

Hemophilia is a rare disorder that often goes unnoticed because of a lack of awareness. Massive sensitization drives are needed to make people aware of this rare genetic disorder. World Hemophilia Day is one of the main initiatives that is observed globally in 140 countries to stimulate the same.

World Hemophilia Day 2021: “Adapting to Change & Sustaining Care in a New World”
World Hemophilia Day is celebrated on 17th April every year. It aims to generate awareness about hemophilia. This year’s focus is on adapting to change and sustaining care in a new world in the wake of COVID-19.

Main Concerns

Healthcare practitioners are not aware of the ailment.
Huge lack of procurement of AHF.
Uneven distribution of AHF.
No good hemophilia policy at the state and central levels.
Women can also have hemophilia but it is of a much rarer concern.

Government Working on the Issue

The most important component of human existence is blood. Considering the importance, and future projection and prevention and proving effective management, the Government of India has prepared comprehensive guidelines for prevention and control of hemoglobinopathies (Thalassemia, Sickle cell anemia and other variant anemia).

Under the National Health Mission, the Government of India provides financial support to the States to strengthen their health care systems, including a system for handling blood disorders with the following tasks.

Vision for Future

Ensuring the infrastructure required for networking of services at different levels of health care.
Strengthened to ensure screening of children and the antenatal mothers.
Training of the required staff involved in the program for blood disorders.
Providing optimal care to all patients of thalassemia, sickle cell disease, and hemophilia by establishing daycare facilities for transfusion and infusion, monitoring with the help of state health departments.
Provide financial support for obtaining medicines like iron chelation, AHF, and other necessary drugs in coordination with the state health department.
Awareness programs for hemoglobinopathies and hemophilia must be organized.
A national registry is being created which would be worthwhile and would be an important tool for planning future patient services.
Health fraternity and doctors must be made consciously aware for the same.

Source-Medindia