By The Hindu Bureau
Copyright thehindu
The Health department has started administering Emicizumab prophylaxis treatment to prevent bleeding episodes in a 32-year-old woman from Thrissur, who is suffering from congenital haemophilia A.
She is being treated at Thrissur Government Medical College, following detailed assessment of her condition and after counselling by a medical team.
Haemophilia is a rare inherited bleeding disorder in which the blood does not clot properly due to the absence of or low levels of clotting proteins known as factors. This can lead to prolonged bleeding after injuries or surgeries and repeated bleeding in joints can lead to joint damage.
In almost 80-85% of the cases, the bleeding is due to the deficiency of Factor VIII, known as Haemophilia A.
The disease is caused by mutations on the X chromosome. Males are the ones commonly affected, while females are usually the carriers, Rarely, females can also be affected if there are mutations on both of their X chromosomes.
The woman from Thrissur was suffering from severe haemophilia with her Factor VIII levels lower than one percent. Initially, she was being treated as a patient with Von Willebrand disease or VMD in which the bleeding disorder is a result of the defect in the Von Willebrand factor in blood, But several blood tests later, the doctors had diagnosed her to be a Haemophilia A patient.
She had had to remove her ovaries and uterus because of uncontrolled bleeding and she had been on hormone treatment. She had been under severe pain following repeated bleeding episodes and bleeding around the joints leading to joint disabilities
Pyrophylaxis strategies are said to be the standard of care in Haemophilia A as it reduces joint damage and the frequency of bleeding episodes. Emicizumab is a non-factor therapy and said to be the gamechanger as far as Haemophilia A is concerned as it can be administered subcutaneously and the dosing schedule is also more relaxed.
Ashadhara scheme
The State is already giving Emicizumab free of cost to 500-odd patients below 18 years in the State under the Ashadhara scheme. This has been helpful in reducing bleeding episodes and hospital visits among haemophilia patients and the changes has been dramatic, according to the Health department
In a statement here, Health Minister Veena George claimed that Kerala was the first State to offer Emicizumab prophylaxis free of cost to patients. The State was also way ahead in drafting dedicated treatment guidelines for managing bleeding disorders in women.
