World Hemophilia Day is observed on April 17th every year. All around the world individuals and groups work together to ensure better diagnosis and access to care for the millions of men, women and children who have a bleeding disorder, yet remain without treatment.
Although the prevalence of Haemophilia is approximately 0.01 per cent of total annual births in India, it is important to educate and spread awareness about it as preventive measures are the best way to handle it.
Having haemophilia means your blood cannot clot properly. Contrary to popular belief, this doesn’t mean you are liable to bleed to death from a minor cut, neither would you bleed faster than other people if you get injured. You can, however, bleed for longer. Although bleeding can be from cuts or grazes on the skin, most bleeding that occurs is internal, into muscles and joints.
How does Haemophilia affect clotting ?
The body protects itself from bleeding by producing sticky blood cells called platelets. In people without haemophilia these sticky cells plug any cuts or grazes and then release chemicals that activate biological molecules in the body, which enable the blood to form a clot.
These clotting proteins are known as factors and there are many clotting factors needed for the blood to coagulate (clot) properly and enable bleeding to stop. They do this via a step-by-step process so, if any of the clotting factors are missing, the process doesn’t work properly. The factors are numbered with Roman numerals from I to XIII.
In someone with haemophilia one of the clotting factors required for the blood to clot properly is missing. People with haemophilia A (about 80% of all cases) are lacking in factor VIII (8) and people with haemophilia B (the remaining 20%) lack factor IX (9).
Most people with haemophilia find out that they are affected in early childhood, although milder cases sometimes do not come to light until late childhood or even early adulthood.
How to recognise a bleed?
If you have haemophilia, or you look after someone with the condition, it is important that you are able to recognise different sorts of bleed and understand the best way to manage them.
External bleeding from minor cuts and grazes does not normally cause a problem.
The main difficulty for people with haemophilia is internal bleeding into the joints, muscles and soft tissues, causing them to become inflamed, swollen and painful. Repeated bleeds can lead to long-term damage, particularly to the joints.
In people with severe haemophilia, internal bleeding can happen after a relatively trivial injury or strain, and it can sometimes happen spontaneously – that is, without any obvious reason such as a bump or a fall.
Bleeds should be treated quickly to prevent long-term damage. If you are used to home infusion, follow the doctor’s instructions on treating a bleed and then consider contacting your Haemophilia Centre for further advice. If there is swelling or discomfort, wrap a bag of frozen vegetables or some ice cubes in a towel and apply to the area. Never give anyone with haemophilia any medicine that contains aspirin, as aspirin slows down the blood clotting.
Head bleeds should always be taken seriously and checked by a doctor. Also, any bleeding from the mouth is harder to deal with and should be treated at the Haemophilia Centre. A bleed around the face, neck or throat must be treated as an emergency and treated immediately by either parents or the Haemophilia Centre.
Internal bleeds may not be obvious, but you will learn to recognise the signs:
- A joint bleed often begins with pain or a “funny feeling” in the affected area
- The area around the joint/muscle may feel tight, warm or swollen and the limb is often painful, stiff or difficult to extend
- The limbs may be unequal in appearance
- In a young child look out for crying for no apparent reason and hampered mobility, or in older children look out for uncharacteristic quietness
- Red or brown urine may be a sign of urinary tract bleeds
- Bloody or black tar like motions (except in new born babies) may be a sign of gastrointestinal bleeding
The commonest sites for joint bleeds are: Shoulder, Elbow, Wrist, Hip, Knee and Ankle.
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SOURCES
http://www.cdc.gov/features/hemophiliaday/index.html
http://www.haemophiliacare.co.uk/how-to-recognise-a-bleed.html
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4181156/
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