WHAT IS HEMOPHILIA?
Hemophilia (also spelled “Haemophilia”) is a life-long bleeding disorder of the blood clotting mechanism. It is the deficiency of a particular clotting factor resulting in the excessive bleeding from injury or without obvious reason at all.
CLOTTING FACTOR
People with hemophilia have little or almost no clotting factor. Clotting factor is a protein needed for normal blood clotting. These proteins work with platelets to help the blood clot. When blood vessels are injured, clotting factors help platelets stick together to plug cuts and breaks at the site of the injury and stop bleeding. With deficiency in clotting factor, normal blood clotting can't take place.
85%-90% of people with hemophilia have factor VIII (8) deficiency (Haemophilia A); while 10%-15% of people with haemophilia have factor IX (9) deficiency (Haemophilia B). This factor deficiency is regardless of blood type (A, B, AB, O).
HOW DO PEOPLE GET HEMOPHILIA?
People are born with hemophilia. You cannot “catch” or acquire hemophilia in your lifetime.
Hemophilia is usually inherited from parent to child.
Men with hemophilia have a 50% chance to pass on the hemophilia gene to their daughters, but not their sons.
Women with the hemophilia gene usually do not have signs of hemophilia, though they carry the hemophilia gene and can pass it on to their children. They are called hemophilia carriers. For each child, there is a 50% chance that the son will have hemophilia and a 50% chance that the daughter will carry the gene.
Women with the hemophilia gene usually do not have signs of hemophilia, though they carry the hemophilia gene and can pass it on to their children. They are called hemophilia carriers. For each child, there is a 50% chance that the son will have hemophilia and a 50% chance that the daughter will carry the gene.
Sometimes, hemophilia can also occur when there is no family history of it. This is called sporadic hemophilia. About 30% of people with hemophilia did not get it through their parents’ genes. It was caused by a change in the person’s own genes.
Women can only have hemophilia if their father has hemophilia and their mother is a hemophilia carrier. This is very uncommon.
CLASSIFICATION OF HEMOPHILIA
The normal activity of clotting factor (Factor VIII or IX) in the blood is 50% or much higher. In hemophilia, the clotting factor deficiency varies in 3 severities:
Severe Hemophilia (less than 1%) Prone to “spontaneous” bleeds or bleeds for no clear reason of injury. Bleeds often occur into the muscles or joints (mainly elbows, knees, and ankles) and also occur after undergoing surgery. Recovery from bleeds is very slow.
Moderate Hemophilia (1% - 5%) Bleeds are usually caused by injury or after undergoing surgery. Bleeds might happen about once a month. Recovery from bleeds is slow.
Mild Hemophilia (5% - 30%) Bleeds are only likely to happen when badly injured or after undergoing surgery. Does not bleed often or might never have a bleeding problem. Recovery from bleeds is still slower than the normal clotting.
WHERE IT BLEEDS (SYMPTOMS)
Bleeding can happen inside or outside the body. The most common symptom of hemophilia is bleeding in the joints and muscles. It is seen when there is bruising, pain, swelling, and limited movement especially the elbows, knees, and ankles.
Repeated injury to the joint can eventually damage the joint and can cause chronic pain & arthritis.
Bleeding outside the body is seen after getting a cut, tooth extraction, or surgery. Patients with hemophilia are not advised to undergo surgery as they cause bleeding for a long period of time.
The most dangerous bleeds are called major bleeds: head bleeds, throat bleeds, gut bleeds, and iliopsoas bleeds (muscles surrounding the hip area). These can be life-threatening and should be treated immediately.
WHEN HEMOPHILIACS BLEED
Treatment should be given as soon as the patient with hemophilia thinks that he is bleeding, whether it is caused by accidents or for no clear reason. Delaying treatment can cause more damage to the bleed.
FIRST AID
Whether or not treatment is available, the following first aid “RICE” treatment is advised:
Rest – most injuries should be rested for a while.
Ice – or cold compress can alleviate the pain and can aid in healing bruises and swelling.
Compress – use a splint or bandage to keep the joint still. Do not wrap the joint too tightly.
Elevate – lift the joint or muscle higher than chest level and rest in on something soft.
TREATMENT
The ideal treatment for bleeding episodes is factor concentrate; Factor 8 or 9 concentrate infused to the blood to temporarily increase the factor level and aid recovery from a bleed. However, factor concentrate is very expensive in our country.
Since factor concentrate is very expensive, other blood products that contain factor concentrate (though in lower dosages) along with other blood components may be used as alternative: Fresh-frozen plasma (FFP), Cryo-precipitate (for Hemophilia A), and Cryo-supernate (for Hemophilia B).
In some cases such as chronic joint or muscle injury, physiotherapy may be needed to pair with blood treatment to aid the joint or muscle to function properly again.
WELLNESS & MAINTENANCE
There is no known cure for hemophilia. However, good nutrition and a healthy lifestyle can help cope with hemophilia and also help avoid bleeds.
Regular Exercise
A regular exercise program guided by physiotherapy will ensure patients of strong joints, muscle strength and flexibility. Maintain physical fitness to prevent bleeding. Swimming is specially recommended. Contact sports like basketball, soccer, and boxing are contraindicated because of the danger of injury. Consulting a physician for an appropriate exercise program is advised.
Dental Care
It is important to take good care of teeth to prevent problems and complications. Tooth extractions and dental surgery can cause major bleeding.
LIMITATIONS & PROHIBITIONS
Strenuous Activities
Lifting heavy objects and enduring long periods of physical activity (hiking, sports, etc.) can cause injuries such as muscle sprains and/or muscle strains to anyone. For people with hemophilia, they cause bleeds.
Contact Sports
Sports such as basketball, volleyball, soccer, and boxing expose a person with hemophilia to the risk of getting injured.
Intake of Foods High in Uric Acid
Limit the intake of foods such as nuts, soy, tofu, & preservatives as they have high uric acid content. They can cause joint pains. But since uric acid is also found in meat & other common food groups as well, it is advisable to regulate the uric acid level by eating lots of raw fruits and vegetables.
Aspirin & Other Pain Relievers
Do not take pain relievers that contain ASA (acetylsalicylic acid) or Aspirin as they can cause more bleeding. NSAIDs (Aleve, Motrin, Ibuprofen) also decrease the blood's ability to clot. Pain relievers also merely alleviate the pain; they do not help increase the clotting factor activity for bleeds to heal.
Intake of Alcohol
The liver is responsible for creating the blood clotting factor. Alcohol causes liver damage thereby compromising liver function – this causes the liver production of the clotting factor even lower. Drinking alcohol in itself does not necessarily cause bleeding; it makes people with hemophilia very prone to bleeds.
IF YOU THINK YOUR CHILD MAY BE SHOWING SIGNS OF HEMOPHILIA, CONTACT US
