Person with Hemophilia
What is Hemophilia?
Hemophilia is a lifelong genetic bleeding disorder that prevents blood from clotting properly. People with hemophilia do not have enough clotting factor- a vital protein in blood that controls bleeding. The severity of a person's hemophilia depends on the amount of Clotting Factor that is missing. A person with hemophilia does not bleed faster than anyone else, but bleeding may last longer. The main danger is uncontrolled internal bleeding that starts spontaneously or results from injury. Bleeding into joints and muscles can cause stiffness, terrible pain, severe joint damage, disability, and very rarely death, if the treatment is out of reach for any reason. It is estimated that over 80% of the persons with hemophilia are physically challenged as they cannot afford the treatment cost. Many of them have also contracted fatal viral infections due to frequent transfusions of unscreened blood components at Govt. as well as Private Hospitals where no safety measures or quality controls and screening tests are done on a blood donor before he donates blood. There is also the risk of window period during which the fatal virus cannot be detected by ordinary tests.
How do people get hemophilia?
Hemophilia is usually inherited and about one in every 10,000 population is born with the disorder. About one third of new cases are caused by a new mutation of the gene in the mother or the child. In these cases, there is no previous history of hemophilia in the family. Women normally do not suffer from hemophilia. When the father has hemophilia but the mother does not, none of the sons will inherit hemophilia, but all of the daughters will carry the defective gene. Women who have the hemophilia gene are called Carriers and they can pass it on to their children. When the mother is a Carrier and the father does not have hemophilia, for each child there is a 50% chance that a son will have hemophilia and a 50% chance that a daughter will carry the gene. India has over 1 lakh males suffering from hemophilia. There should also be more than 2 lakh potential hemophilia Carrier women who are either not diagnosed or keep their status hidden because of social stigma.
What are the signs of hemophilia?
- Big bruises;
- Children with hemophilia often do have reddish blue patches and swellings.
- Small cuts anywhere on the body bleed for longer time.
- Nose bleeds
- Bleeding into muscles and joints, especially the knees, elbows, and ankles
- Sudden bleeding inside the body for no clear reason;
- Prolonged bleeding after a cut, tooth removal, surgery, or an accident.
- Serious internal bleeding into vital organs, most commonly after a serious trauma.
List of Hospitals having hemophilia diagnosis facility is here
How is hemophilia treated?
On finding any of the signs above the immediate step is to get the child diagnosed. If the laboratory tests confirm hemophilia and its severity, following steps would help:-
i) Keeping the reports & records properly in a file.
ii) Knowing how to manage hemophilia care by discussing it in the families affected by it, doctors, social workers and psycho-social consultants. Reading more and more educative material, watching visuals, presentations & films on it will sort out half the problem.
iii) Consulting a qualified and trained medical specialist for medical treatment. Effective treatment for hemophilia is very much available, but as yet there is no cure. Hemophilia is treated by making good the missing clotting factor in the blood. This is done by injecting a product that contains the needed factor into a vein. Bleeding stops when enough clotting factor reaches the affected area. With proper treatment, people with hemophilia live relatively normal lives.
For more information please read our publication "Hemophilia Aur Aap". Also know about a Hemophilia Treatment Centre near you here.
iv) Enrolling the child as a member with the nearest Hemophilia Society and SHC India. List here
Hemophilia & its Gravity:
As per WHO prevalence rate there should be over 1 Lakh persons having Hemophilia in India. In addition at least double this number (mother and daughter/s and sometimes sister/s as well of the affected male) are the carriers of this genetic bleeding disorder.
Moreover it is a life long condition requiring adequate timely attention to stop any bleeds. Having no access to medical treatment leads to physical disabilities. This limits the life activities and sometimes the affected child does not attend the school. More expensive treatment means the collapse of the family budget and priorities, social stigmatization for the child as well as his mother and may be family. And the affected child thus becomes dependent for the rest of his life. Moreover, if he resorts to unconventional treatment like unscreened blood transfusion like FFP and Cryoprecipitate the person can be the victim of HIV/AIDS or Hepatitis or other unknown viruses.
ALL THIS IS FORTUNATELY PREVENTABLE.
It is possible that a person with hemophilia can lead a near normal life. If the following issues and limitations are taken care of:
A child, having symptoms needs to be taken to a well known hospital in his city for further investigation and blood coagulation tests. At present the diagnostic facilities are very limited. The parents have to travel hundreds of kilometres to have the access to a hematologist or the laboratory.
In India there are still 40% people illiterate and even more when it comes to knowing about health. Government of India spends around 1% of GDP on health. Therefore public awareness by NGOs like SHC India about hemophilia and information about the medical facilities available in the country is very important.
: It is the single most debilitating impact of hemophilia. Lack of diagnosis in the beginning and prohibitively expensive treatment lead to frequent bleeds in joints which get damaged and a child is disabled even in his childhood. This disability continues increasing because of little or no affordable treatment. This disability leads to lifelong dependency.
Exposure to fatal viral infections:
The cost of the medicine called Anti Hemophilia Factor – AHF is very high. A vial of AHF for a small child would cost Rs.3000/-. And the need for it is quite frequent. This, being ill- affordable, parents resort to other unsafe modes of treatment like not-properly tested blood. It is estimated that at least 8% of such persons are HIV positive. HBV and HCV infections are estimated to be over 30%.
Due to higher ignorance level in India, especially in rural areas, the mother of the affected child is blamed for giving hemophilia to her son. She faces a lot of gender bias, sometimes torture and taunts of the rest of the family. Empowerment & Psycho-social support to the family plays a very important role.
Hemophilia treatment is very expensive even for a very well-to-do family. With no support from the Government a family has no option but to let their child bleed, suffer unbearable pain or get disabled in his childhood itself. He may also drop out of school for fear of bleeds.
These issues exist mainly due to the inability of our society and the Government not being positive about health of such children. If the health budget is raised to 4% with its equitable distribution every person with hemophilia would lead a near normal life and contribute equally to the progress of the country. They too have "Right to Live" as pronounced in our constitution.
Hemophilia Care is the most neglected health issue and the Government has to come forward and include it in the National Health Programs so that every person with hemophilia can lead a healthy, disable-free and HIV/Hepatitis-free life.
"Nobody should die or become disabled when treatment is available"