What is Hemophilia?
Hemophilia is a lifelong bleeding disorder that prevents blood from clotting properly. People with hemophilia do not have enough clotting factor, a 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, pain, severe joint damage, disability, and very rarely death, if the treatment is out of reach.
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. 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 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 people suffering from hemophilia
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.
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 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.
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 the person can be the victim of HIV/AIDS or Hepatitis or other unknown viruses.
ALL THIS IS FORTUNATELY PREVENTABLE.
And How: It is possible that a person with hemophilia can lead a near normal life. If the following issues and limitations are taken care of:
Diagnosis: A child, having symptoms need to visit the well known hospital in his state for further investigation and blood coagulation tests. At present the diagnostic facilities are very limited. The parents have to travel hundreds of kilometers to have the access to a hematologist or the laboratory.
Awareness: In India there are still 40% people illiterate and even more when it comes to knowing about health. Government of India spends 0.9% 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.
Disability: 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 life long 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.2000/-. 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%.
Gender Bias: 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. Counseling to the family plays a very important role.
Financial Burden: Hemophilia treatment is very expensive even for a 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 become a school drop out for fear of bleeds.
These issues are mainly due to the inability of our society or Government not being positive about health. 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-free life.
HIV / AIDS
Human Immunodeficiency Virus infection (HIV) is a viral disorder that progressively destroys a certain type of white blood cell (lymphocyte) and causes Acquired Immunodeficiency Syndrome (AIDS).
Infection by the human immunodeficiency virus eventually results in progressive deterioration of the body’s immune system, allowing opportunistic infections and certain cancers to develop. AIDS is the late stage of HIV infection.
AIDS has reached epidemic proportions, with more than 750,000 cases and 400,000 deaths reported in the US. The World Health Organization estimates that 30 to 40 million people worldwide are infected today.
It is estimated that currently 5% to 8% of persons with hemophilia are infected with HIV in India. This is because such people receive blood transfusions instead of the standard therapy with Anti Hemophilia Factor Concentrates.
Nowhere in the world, treatment with FFP or Cryo is advised in view of window period.
The AIDS epidemic has placed great health, economic, ethical and emotional burdens on such affected families and the wider bleeding disorders community.
No case of HIV/AIDS has been found in people who have been using only Factor Concentrates since 1986 due to viral inactivation (viral killing) methods that are used to treat such people. These viral inactivation processes include heat treatment, solvent-detergent cleansing and monoclonal purification.
The latest therapy to treat hemophilia is recombinant factors which are free from any risk of blood contamination but are prohibitively expensive for people in developing countries.
In order to prevent any HIV/AIDS infection in people with hemophilia Factor Concentrates are the treatment of the day. This is the reason that this medicine has been placed in the List of Essential Drugs by WHO and Govt. of India.
All people suffering from hemophilia should be treated by Factor Concentrates only as a rule.
The transmission of HIV requires contact with a body fluid that contains infected cells or virus particles, including blood, semen, vaginal secretions, cerebrospinal fluid and breast milk. HIV is also present in tears, urine and saliva, but in much lower concentrations. The virus is transmitted in the following ways:
⦁ Injection or infusion of contaminated blood, as occurs with blood transfusions, the sharing of needles or an accidental prick from an HIV-contaminated needle.
⦁ Sexual relations with an infected person, during which the mucous membrane lining the mouth, vagina or rectum is exposed to contaminated body fluids.
⦁ Transfer of the virus from an infected mother to a child before or during birth or through the mother’s milk.
Prevention
⦁ Uninfected people should abstain from sex or engage only in safe (protected) sex.
⦁ HIV-positive people should abstain from sex or have safe (protected) sex; they should not give blood or donate organs, avoid pregnancy and notify previous and prospective sexual partners.
⦁ Drug abusers should halt the practice of sharing or reusing needles and enter a drug treatment program.
⦁ Medical and dental professionals should wear latex gloves whenever there is a possibility of contact with body fluids, and properly use and dispose of hollow needles.
There is no current vaccination for HIV, but several new studies hold promise for the future.
Treatment
Many drugs are now available to treat HIV infection. All prevent the virus from reproducing and slow the progression of the disease. The trouble is HIV usually develops resistance to these drugs when they are used alone.
Treatment seems to be most effective when at least two to three of the drugs are given in combination. Drug combinations may delay the onset of AIDS in HIV-positive people and extend one’s life compared with the use of single drugs. Recent studies suggest that these drug combinations can reduce the virus to undetectable levels and restore the immune system to near normal. Doctors aren’t certain how soon after infection these drugs should be started, but people with high levels of HIV in their blood should be treated.
People with AIDS are usually prescribed drugs to prevent infections, such as pneumonia, as well.
