By: Syeda Sundeela Fayyaz
Every year on 17 April, World Haemophilia Day is celebrated. This day is observed to increase the awareness of haemophilia, disease, and other inherited bleeding disorders.
This day has got great importance since the observance of the haemophilia day has increased awareness and with that better diagnosis and access to care for the millions who remain without treatment has evolved.
History
The history of World Haemophilia Day dates back to 1989 by the World Federation of Haemophilia (WFH).
17 April is selected to bring the community together in honour of WFH founder Frank Schnabel’s birthday.
Theme
Every year the day has got some inspirational theme to encourage people to pay attention to a serious problem and to work for its betterment.
This year’s theme for the world hemophilia day is “Adapting to Change”.
What is haemophilia?
Haemophilia is a blood disorder and an inherited condition that results in the deficiency of certain blood factors. This disorder is largely seen in men but many women got affected too. The main problem of this disorder is that it leads to an inability for blood to clot in the person. The reason behind this is the body doesn’t make a protein called clotting factor.
Haemophiliacs continue to bleed for a LONGER TIME than usual if they are injured, or start bleeding for other SIMPLE reasons. As a result of the severity of this disorder their spontaneous bleeding, joint disorders and loss of joint function, and brain bleed.
Haemophilia is caused by a mutation in the gene for factor VIII or factor IX. This problem mainly takes place on the X chromosome, which is the chromosome inherited from the mother.
As Haemophilia is an inherited genetic disorder where the detective gene for clotting factor is in the father and carried over to the son by the mother, thus the female is the carrier and the male is the sufferer.
This disorder isn’t curable, but with the aid of proper treatment, we can control the symptoms and the intensity of the disease can also be reduced to prevent severe health complications.
What causes prolonged bleeding?
In a normal person, there is a complex natural reaction that starts in the body soon after an injury is detected. The blood clot soon or we can say within a few seconds, platelets, proteins and other clotting factors found in the blood assemble around the wound and form a clot. It acts like a plug over the injury preventing further bleeding. In haemophiliacs, clotting factors are either missing or don’t work appropriately which results in improper clotting of blood at the site of the wound and bleeding for a longer time than the normal one.
Different forms of haemophilia
There are three different forms of haemophilia:
• Haemophilia A- It is the most common type and is caused by a deficiency in factor VIII or classic haemophilia.
• Haemophilia B- It is caused by a deficiency of factor IX. It is also called Christmas disease, named for Stephen Christmas, the first person diagnosed with the factor IX deficiency.
• Haemophilia C- It is a mild form of the disease that’s caused by a deficiency of factor XI. It doesn’t cause spontaneous or major bleeding. It is also known as Rosenthal syndrome.
Severity of hhaemophili
Haemophilia can be mild, moderate, or severe depending on how much of the clotting factor is in a person’s blood. 50% and 150% is the normal range of factor VIII and factor IX.
Mild Haemophilia
During mild haemophilia bleeding is infrequent and typically occurs only after injury, trauma, or surgery, including dental procedures. About 25% of people with haemophilia have a mild form of the condition.
Moderate Haemophilia
During moderate haemophilia, bleeding occurs after minor injuries; incidents of spontaneous bleeding without an obvious cause may occur, but it is rare. About 15% of people with haemophilia have a moderate form of the condition.
Severe Haemophilia
During severe haemophilia, you may experience bleeding after injury, trauma, or surgery; however, you may also experience spontaneous bleeding into joints and muscles. Repeated bleeding into joints can cause a joint condition known as haemophilicarthroplasty which can result in physical disability. About 60% of people with haemophilia have a severe form of the condition.
Symptoms of haemophilia can cause the following problems to occur:
• Deep, large, unexplained bruises
• Frequent nosebleeds
• Bleeding gums
• Blood in the urine
• Severe pain in the abdomen
• Irritability (in children)
• Severe unexplained headache
Bleeding in a joint, followed by swelling, tenderness, stiffness, and trouble using the joint.
Bleeding in a muscle, which causes pain, swelling, warmth, and a bruise over the area.
Bleeding in the digestive system causes black or bloody poop or blood in throw-up (vomit).
Bleeding in the brain causes headache, vomiting, sleepiness, or seizures.
Diagnosis of haemophilia
To diagnose haemophilia, doctors can do the following blood tests:
• Complete blood count (CBC)
• Prothrombin time (PT)
• Activated partial thromboplastin time (PTT)
• Factor VIII activity test
• Factor IX activity test
Treatment Options for haemophilia
There are many different types of therapies for haemophilia and many new ones are in development.
The current and most-used treatment for haemophilia is factor replacement therapy. This is done by infusing an FVIII product into the affected person. When you infuse, FVIII concentrate, the FVIII is immediately available in the bloodstream, and your body can use it to stop or prevent the bleeding.
People with haemophilia can expect to have a lifespan and lifestyle that is relatively normal, as long as they are educated about their condition and have adequate treatment.
Preventions
People with haemophilia must get some prevention to avoid the severity of the disorder, they are some things you can do on your own;
Always treat your bleeding quickly.
Exercise at least three times a week.
It is advisable to brush and floss your teeth at least once a day.
Eat regular, healthy meals.
Try to maintain an average body weight.
Get a good night’s sleep.
Take all the vaccines that you need.
Follow a normal healthy daily routine.
As per estimation, about 400,000 people around the world have haemophilia and the sad part is that around 75% of these people are unable to access adequate treatment.
World Haemophilia Day is about recognizing those living with bleeding disorders around the world. On this day of world haemophilia, I would like our government, concerned health officers, and the authorities to bring haemophilia and other inherited bleeding disorders into consideration because together we can increase sustainable and equitable access to care and treatment globally. Everyone can and should play a part.
