Advanced Centre For Hemophilia at Fortis Hospital Bangalore

Hemophilia is an inherited bleeding disorder that is caused by an altered gene in the body. It requires extreme care in the hands of specialists to treat this disorder which is a result of low levels of the Factors that cause clots. Hence, the bleeding is not just external from wounds but also is most commonly internal. Since the bleeding is not just because of any injury or trauma and can occur without any obvious cause, specialised treatment is needed instantly to help the blood clot normally to stop the bleeding.
 
The extremely proficient and qualified team of hematologists at Fortis Hospitals Bannerghatta pinpoint the exact type of Hemophilia to administer the best mode of treatment to patients. After the precise tests are conducted to diagnose the type, and to check if the bleeding is also internal, the precise medication is given to stem the bleeding and let the blood clot as normally as possible. Most often, specialised treatment includes infusion or injecting of the blood clotting agent into the vein.
 
The excellent team of hematologists at Fortis Hospitals Bannerghatta understand that hemophilia is incurable and if not treated in time, can be life-threatening. This is why they are quick to respond to hemophiliacs to determine if they require genetic engineered clotting factor medication to treat this rare disease, especially in women. This is because they know perfectly well that in women, unrestrained bleeding during menstruation can lead to severe anaemia. In women who have delivered a baby, post-partum bleeding needs to be stemmed and at Fortis Hospitals Bannerghatta know this perfectly to handle it with extreme care and caution.

What is Hemophilia?

Our body functions like a well-oiled machinery when all the organs and tissues work in perfect synchrony with one another. As human beings it is also natural to fall ill or get injured in the course of life. When we get injured, our body’s mechanism fights to control the bleeding by clotting. However, in some people, due to some gene malfunction, the blood does not clot and the bleeding cannot be controlled by the body naturally. This bleeding disorder is very rare and is called Hemophilia. This same gene is then passed down to the family. That is the reason Hemophilia is an inherited disorder.

In hemophiliacs, or those who suffer from hemophilia, the clotting factor is absent from birth and passed down in families. This clotting agent is a protein present in the blood that controls bleeding. Hemophilia afflicts both males and females. Males having hemophilia pass the altered gene on to their daughters, but not their sons. However, sons with the gene will have hemophilia at birth. Some women with the gene can experience severe bleeding problems, and can also pass on the altered gene to their sons and daughters.
 
While hemophilia is found in families with a history of the disorder, there have been cases where it appears in families with no previous history of the blood disorder. This is known as acquired hemophilia and this rare form of the condition develops when the body’s immune system wrongly attacks the clotting factors within the body itself.

Hemophilia Symptoms

This rare genetic bleeding disorder is often not diagnosed in its early stages because there are no visible signs on the surface. It is only when an external injury causing bleeding refuses to stop and the doctors notice that there is something amiss in the clotting process, that they diagnose the condition.
 
In fact, in mild hemophilia, there may not be any noticeable signs until you go for a surgery and tend to bleed heavily.
 
Since most commonly the bleeding is internal, the symptoms are hidden. The low levels of the clotting factors can cause the bleeding into the joints or muscles including the brain. Long term bleeding into the joints can cause joint deformity. Sometimes, the bleeding can occur in the throat, kidneys and brain.

Some of the symptoms include:

• Excessive bruising and bruising easily

• Abnormally excessive bleeding following injuries from accidents, after surgery or even tooth extractions

• Heavy bleeding during menstruation, leading to anaemia or low iron levels in the body
  Prolonged bleeding after childbirth

• In females having very low clotting factor levels there may be bleeding episodes in the muscles and joints. Prolonged episodes can cause scarring of the joints leading to loss of mobility and prone to more bleeding. The joints most susceptible to bleeding are the elbow, knee and ankle joints

• Occasionally there may be blood in the urine caused due to the bleeding in the kidneys

• Sometimes there could be bleeding in the throat causing swelling which can interfere with the normal breathing

• In very severe cases of hemophilia, there may be instances of bleeding in the brain causing permanent brain damage, disability or even death

Causes of Hemophilia

Hemophilia is a rare blood disorder that does not allow the blood to clot naturally and leads to excessive bleeding, both externally and internally.
 
This condition is mostly genetic, meaning handed down through generations of faulty genes or chromosomes. Chromosomes are the cells of life and come in pairs. While women have two X chromosomes, men have one X and one Y chromosome. Hemophilia is linked to a defect or abnormality in the X chromosome.
 
While it has been researched that about 50 per cent of those who have hemophilia have inherited it from their family genetics, the rest of them diagnosed with it have had no hereditary factor. This means hemophilia can be caused due to a change or mutation in the Factors 8 or 9 which cause the blood to clot naturally. In these non-inherited cases, the hemophiliacs can transmit the defective gene to their children.
 
Acquired hemophilia happens when there is no known hereditary cause and the body begins to attack its own blood clotting factors for no known reason.

Diagnosing Hemophilia

Hemophilia is often diagnosed by doctors in the first year of a baby’s life when they spot severe bruising and prolonged bleeding.
 
Whenever there is an injury that causes bleeding the body’s natural clotting process takes over to stem the flow of blood and thickens it. This is called a fibrin clot.
 
At Fortis Hospitals Bannerghatta, doctors determine if you have hemophilia by recommending stringent tests to determine if it is due to a hereditary factor or if it is an acquired condition. The tests examine to see how well or poorly your blood clots. These specific tests are conducted under stringent norms in the best of our hematology labs. In the lab, a sample of your blood is drawn and mixed with specific chemicals to induce clots. If the tests are found abnormal, other specific tests are conducted to determine the levels of the clot inducting Factors 8 and 9. Based on the outcomes of these tests, our highly efficient team of hematologists draw up the next line of effective treatment.

Types of Hemophilia

Hemophilia the rare bleeding disorder of the blood is of two types and is classified into Hemophilia A and Hemophilia B to make it easier to diagnose and treat precisely.
 
Hemophilia A is the most common form of the bleeding disorder which is caused when the person has reduced levels of the Factor 8. The other form of Hemophilia B is a result of having low levels of the Factor 9.
 
Factors 8 and 9 are the clotting agents present in normal blood which naturally stem the flow of blood that could cause excessive bleeding. In other words, when there is bleeding due to injury or trauma, the clotting Factors 8 and 9 thicken the blood and stop it from bleeding.
 
When there is something wrong with this process due to an inherited faulty gene, it causes bleeding disorders divided into two prominent types – Hemophilia A and Hemophilia B.
 
Through precise diagnosis, expert specialists at Fortis Hospitals Bannerghatta are able to treat patients with astuteness and compassion.

Hemophilia Treatment Techniques

Expert hematologists at Fortis Hospitals Bannerghatta have the perfect and precise set of skills and proficiency to handle the most perplexing cases of hemophilia with a great deal of care and kindness.
 
If you or your loved ones suffer from hemophilia, you can rest assured that you and they are in the safest of hands.
 
The best mode of treatment you need and the frequency is determined by the doctors depending on the type, whether A or B, and the severity of the disorder.
 
If your diagnosis reveals that it is a mild form, you may need treatment only when you are injured and bleeding, or before you undergo any form of surgery including a dental implant or extraction.
But, if you have a severe form of hemophilia, the doctors will put you onto a regular treatment regimen to prevent bleeding into your joints and other organs.
 
The Hematologists administer the latest time-tested Factor Replacement Therapy in the treatment of hemophilia of both types.

Treatment for Hemophilia A :

This bleeding disorder is caused by a deficient clotting Factor 8 in the blood. The hematology specialists will inject a medication, a clotting Factor 8 called desmopressin which can temporarily increase the concentration of Factor 8 in your blood.

Treatment for Hemophilia B:

The absence or defective clotting Factor 9 causes this type of bleeding disorder in patients. The best form of treatment is replacement therapy which helps increase the level of Factor 9 in the blood. The other mode is infusing clotting agents which help protect blood clots and keep them in place to stop bleeding. Both may be administered together depending on the severity of the bleeding.