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16 April, 2019 00:00 00 AM

Haemophilia and World Haemophilia Day

Haemophilia is a bleeding disorder that affects approximately 1 in 10,000 people. People with haemophilia do not have enough clotting factor VIII or IX in their blood. As a result, they can bleed for longer than normal.
The World Federation of Haemophilia
Haemophilia and World Haemophilia Day

World Haemophilia Day is an international awareness day for haemophilia and other bleeding disorders. It is held annually on April 17, date of the birthday of Frank Schnabel, founder of the World Federation of Haemophilia.

Since 1989, World Haemophilia Day is the day the whole bleeding disorders community comes together to celebrate the continuous advances in treatment while raising awareness and bringing understanding and attention to the issues related to proper care to the wider public.

This year, the World Federation of Haemophilia (WFH) is focusing on reaching out and identifying new members of the bleeding disorders community. For many years, the WFH has supported outreach projects in different countries and conducted educational events for both lay and health care professionals in conjunction with our national member organizations (NMOs).

The WHD2019 campaign will feature examples of WFH outreach initiatives such as organizing regional workshops, raising awareness of bleeding through media coverage or training health professionals and collecting data. It will combine these examples with submissions from our community about outreach efforts in their own regions or ideas about what would be helpful for future initiatives.

This landmark day also helps to illustrate the importance of the WFH Humanitarian Aid Program—an important endeavour for the WFH that provides a range of integrated care development training programs to ensure the local infrastructure and medical expertise are available to optimize and appropriately use donated products.

What are bleeding disorders?

In people with bleeding disorders, the blood clotting process doesn’t work properly. As a result, people with bleeding disorders can bleed for longer than normal, and some may experience spontaneous bleeding into joints, muscles, or other parts of their bodies.

Haemophilia is a bleeding disorder that affects approximately 1 in 10,000 people. People with haemophilia do not have enough clotting factor VIII or IX in their blood. As a result, they can bleed for longer than normal.

The most common bleeding disorder is von Willebrand disease (VWD). It is generally less severe than other bleeding disorders. Many people with VWD may not know that they have the disorder because their bleeding symptoms are very mild.

Rare clotting factor deficiencies are disorders in which one of several clotting factors is missing or not working properly. Less is known about these disorders because they are diagnosed so rarely. In fact, many have only been discovered in the last 40 years.

Finally, inherited platelet disorders are conditions in which platelets don’t work the way they should, resulting in a tendency to bleed or bruise.

The clotting process

Clotting factors are proteins in the blood that control bleeding. When a blood vessel is injured, the walls of the blood vessel contract to limit the flow of blood to the damaged area. Then, small blood cells called platelets stick to the site of injury and spread along the surface of the blood vessel to stop the bleeding.

At the same time, chemical signals are released from small sacs inside the platelets that attract other cells to the area and make them clump together to form what is called a platelet plug.

On the surface of these activated platelets, many different clotting factors work together in a series of complex chemical reactions (known as the coagulation cascade) to form a fibrin clot. The clot acts like a mesh to stop the bleeding.

Coagulation factors circulate in the blood in an inactive form.

initiated and each coagulation factor is activated in a specific order to lead to the formation of the blood clot. Coagulation factors are identified with Roman numerals (e.g. factor I or FI).

Drugs that can cause bleeding

People with bleeding disorders should avoid aspirin and other drugs that can cause bleeding. This list of drugs that can cause bleeding does not include drugs that have to be given as injections or intravenous infusions. The generic or “chemical” names are given since the brand names or marketing names differ between countries.

While every effort has been made to include as many drugs as possible, some may be missing. In addition, many non-prescription medications, such as cold remedies, contain aspirin. People with bleeding disorders should check with their haemophilia centre or physician, or consult the pharmaceutical company’s printed instructions before taking any new medication.

What is haemophilia?

Haemophilia is a bleeding problem. People with haemophilia do not bleed any faster than normal, but they can bleed for a longer time. Their blood does not have enough clotting factor. Clotting factor is a protein in blood that controls bleeding.

Haemophilia is quite rare. About 1 in 10,000 people are born with it.

Types of haemophilia

The most common type of haemophilia is called haemophilia A. This means the person does not have enough clotting factor VIII (factor eight).

Haemophilia B is less common. A person with haemophilia B does not have enough factor IX (factor nine). The result is the same for people with haemophilia A and B; that is, they bleed for a longer time than normal.

How do you get haemophilia?

People are born with haemophilia. They cannot catch it from someone like a cold.

Haemophilia is usually inherited, meaning that it is passed on through a parent’s genes. Genes carry messages about the way the body’s cells will develop as a baby grows into an adult. They determine a person’s hair and eye colour, for example.

Sometimes haemophilia can occur when there is no family history of it. This is called sporadichaemophilia. About 30% of people with haemophilia did not get it through their parent’s genes. It was caused by a change in the person’s own genes.

How is haemophilia inherited?

The haemophilia gene is passed down from parent to a child. The genes for haemophilia A and B are on the X chromosome. For this reason, haemophilia is called an X-linked (or sex-linked) disorder.

The figure below explains how the haemophilia gene is inherited. When the father has haemophilia but the mother does not, none of the sons will have haemophilia. All the daughters will carry the haemophilia gene.

If a woman inherits a copy of the altered gene from either of her parents, she is said to "carry" the haemophilia gene and is therefore called a "carrier." In other words, she has one normal and one altered copy of the gene.

She can pass either gene onto her children. For each child, there is a 50% chance that a son will have haemophilia and a 50% chance that a daughter will carry the gene. On average, carriers of haemophilia will have about 50 per cent of the normal amount of clotting factor, but some carriers have far lower levels of clotting factor.

Acquired haemophilia

In rare cases, a person can develop haemophilia later in life. The majority of cases involve middle-aged or elderly people, or young women who have recently given birth or are in the later stages of pregnancy.

This condition often resolves with appropriate treatment.

 

 People with severe haemophilia usually bleed frequently into their muscles or joints. They may bleed one to two times per week. Bleeding is often spontaneous, which means it happens for no obvious reason.

People with moderate haemophilia bleed less frequently, about once a month. They may bleed for a long time after surgery, a bad injury, or dental work. A person with moderate haemophilia will rarely experience spontaneous bleeding.

People with mild haemophilia usually bleed as a result of surgery or major injury. They do not bleed often and, in fact, some may never have a bleeding problem.

Symptoms and diagnosis

The signs of haemophilia A and B are the same:

Big bruises

Bleeding into muscles and joints

Spontaneous bleeding (sudden bleeding inside the body for no clear reason)

Prolonged bleeding after getting a cut, removing a tooth, or having surgery.

Bleeding for a long time after an accident, especially after an injury to the head.

Bleeding into a joint or muscle causes:

an ache or “funny feeling”

swelling

pain and stiffness

difficulty using a joint or muscle

Where does bleeding occur?

People with haemophilia can bleed inside or outside the body. Most bleeding in haemophilia occurs internally, into the muscles or joints. The most common muscle bleeds occur in the muscles of the upper arm and forearm, the iliopsoas muscle (the front of the groin area), the thigh, and the calf. The joints that are most often affected are the knee, ankle, and elbow.

If bleeding occurs many times into the same joint, the joint can become damaged and painful.

Repeated bleeding can cause other health problems like arthritis. This can make it difficult to walk or do simple activities. However, the joints of the hands are not usually affected in haemophilia (unlike some kinds of arthritis).

How is haemophilia diagnosed?

Haemophilia is diagnosed by taking a blood sample and measuring the level of factor activity in the blood. Haemophilia A is diagnosed by testing the level of factor VIII activity. Haemophilia B is diagnosed by measuring the level of factor IX activity.

If the mother is a known carrier of haemophilia, testing can be done before a baby is born. Prenatal diagnosis can be done at 9 to 11 weeks by chorionic villus sampling (CVS) or by fetal blood sampling at a later stage (18 or more weeks).

These tests can be done at a haemophilia treatment centre.

Treatment

Treatment for haemophilia today is very effective. The missing clotting factor is injected into the bloodstream using a needle. Bleeding stops when enough clotting factor reaches the spot that is bleeding.

Bleeding should be treated as quickly as possible. Quick treatment will help reduce pain and damage to the joints, muscles, and organs. If bleeding is treated quickly, less blood product is needed to stop the bleeding.

With an adequate quantity of treatment products and proper care, people with haemophilia can live perfectly healthy lives. Without treatment, most children with severe haemophilia will die young. An estimated 400,000 people worldwide are living with haemophilia and only 25% receive adequate treatment. The World Federation of Haemophilia is striving to close this gap.

Treatment products

Factor concentrates are the treatment of choice for haemophilia. They can be made from human blood (called plasma-derived products) or manufactured using genetically engineered cells that carry a human factor gene (called recombinant products).

Factor concentrates are made in sophisticated manufacturing facilities. All commercially prepared factor concentrates are treated to remove or inactivate blood-borne viruses.

Cryoprecipitate is derived from blood and contains a moderately high concentration of clotting factor VIII (but not IX). It is effective for joint and muscle bleeds, but is less safe from viral contamination than concentrates and is harder to store and administer. Cryoprecipitate can be made at local blood collection facilities.

In fresh frozen plasma (FFP) the red cells have been removed, leaving the blood proteins including clotting factors VIII and IX. It is less effective than cryoprecipitate for the treatment of haemophilia A because the factor VIII is less concentrated. Large volumes of plasma must be transfused, which can lead to a complication called circulatory overload. FFP is still the only product available for treatment of haemophilia A and B in some countries.

People with mild haemophilia A may sometimes use desmopressin (also called DDAVP) to treat minor bleeding. DDAVP is a synthetic hormone that stimulates the release of factor VIII.

New panel discussion: New Developments in Treatment for Bleeding Disorders – Novel Therapies, is a cure for haemophilia around the corner?

While clotting factor concentrates remain, by far, the mainstay of the management of haemophilia, a number of novel therapies under development have the potential to dramatically change the treatment landscape. Leading hematologists and patient advocates from around the world debate the potential of factor mimetics, antithrombin therapies, and gene therapy to change the lives of all people living with bleeding disorders.

Carriers and women with haemophilia

For many years, people believed that only men could have symptoms of haemophilia and that women who "carry" the haemophilia gene do not experience symptoms themselves.

We now know that many carriers do experience symptoms of haemophilia. As our knowledge about the disorder has increased, so has our understanding of why and how women can be affected. Some women live with their symptoms for years without being diagnosed or even suspecting they have a bleeding disorder. Through education and awareness-raising, the World Federation of Haemophilia is working to close this gap in care.

Quality of life

Being a carrier of haemophilia can have a significant impact on a woman’s health and her academic, professional, and social life.

Excessive or prolonged menstrual bleeding can be especially difficult for young girls, who may isolate themselves from family and friends, miss days from school, or avoid social events due to pain, discomfort, or the fear of staining clothing. A girl’s self-image and confidence can be negatively affected if she experiences shame or embarrassment because of heavy menstrual bleeding.

Many carriers are not aware that their symptoms are abnormal and do not seek medical advice. Even when they do, caregivers are not always well informed about bleeding disorders and the right diagnosis may be overlooked. Furthermore, medical care for women is lacking in many countries around the world. There may be cultural taboos and obstacles preventing women from seeking help, particularly for menstrual problems.

Heavy and prolonged menstrual bleeding and pain can affect a woman’s sexuality and may cause problems in her marriage. Women may also need to take time off work each month because of heavy bleeding, which can impact their career choices or professional success.

Many carriers of haemophilia, like others at risk of passing on a genetic disease, also experience guilt. They may feel as though they should not have children because of the possibility of passing on a bleeding disorder, or having a daughter who must face this possibility in turn.

The prospect of marriage may be affected because men, or their families, may not accept the risk of having an affected child. If they do have children with haemophilia, the needs of that child can put pressure on all family members, including siblings.

Many haemophilia treatment centres can provide carriers with skilled and sensitive counselling. The professionals there can provide information and support to work through these complex feelings and to empower women to take charge of their condition and advocate for proper treatment.

Building a support network of other women who are facing the same issues, through the haemophilia treatment centre or local patient organization, can be a great source of comfort.

 

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Editor : M. Shamsur Rahman

Published by the Editor on behalf of Independent Publications Limited at Media Printers, 446/H, Tejgaon I/A, Dhaka-1215.
Editorial, News & Commercial Offices : Beximco Media Complex, 149-150 Tejgaon I/A, Dhaka-1208, Bangladesh. GPO Box No. 934, Dhaka-1000.

Editor : M. Shamsur Rahman
Published by the Editor on behalf of Independent Publications Limited at Media Printers, 446/H, Tejgaon I/A, Dhaka-1215.
Editorial, News & Commercial Offices : Beximco Media Complex, 149-150 Tejgaon I/A, Dhaka-1208, Bangladesh. GPO Box No. 934, Dhaka-1000.

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