Haemophilia is a disorder in which an individual’s blood doesn’t clot
normally because it lacks sufficient blood-clotting proteins (clotting
factors). People, who have haemophilia, may bleed for a longer time after an
injury than they would if their blood clots normally. This constitutes a great
health concern when deep bleeding occurs inside the body, especially in the knees,
ankles and elbows. This could lead to internal bleeding which can damage the
individual’s organs and tissues, and may be life-threatening.
normally because it lacks sufficient blood-clotting proteins (clotting
factors). People, who have haemophilia, may bleed for a longer time after an
injury than they would if their blood clots normally. This constitutes a great
health concern when deep bleeding occurs inside the body, especially in the knees,
ankles and elbows. This could lead to internal bleeding which can damage the
individual’s organs and tissues, and may be life-threatening.
Haemophilia is an inherited (genetic) disorder. There’s no cure yet. But
with proper treatment and self-care, most people with haemophilia can maintain
an active, productive lifestyle.
with proper treatment and self-care, most people with haemophilia can maintain
an active, productive lifestyle.
Signs and symptoms of haemophilia
Signs and
symptoms of haemophilia vary, depending on the level of clotting factors. If an
individual clotting-factor level is mildly reduced, he/she may bleed only after
surgery or trauma. If the deficiency is severe, the individual may experience
spontaneous bleeding.
symptoms of haemophilia vary, depending on the level of clotting factors. If an
individual clotting-factor level is mildly reduced, he/she may bleed only after
surgery or trauma. If the deficiency is severe, the individual may experience
spontaneous bleeding.
Signs and
symptoms of spontaneous bleeding include:
symptoms of spontaneous bleeding include:
- Unexplained and excessive bleeding from cuts or
injuries, or after surgery or dental work - Many large
or deep bruises - Unusual
bleeding after vaccinations - Pain,
swelling or tightness in your joints - Blood in
your urine or stool - Nosebleeds
without a known cause - In infants,
unexplained irritability
Emergency signs and symptoms of haemophilia include:
- Sudden pain, swelling and warmth in large joints,
such as knees, elbows, hips and shoulders, and in the arm and leg muscles - Excessive
bleeding from an injury, especially in a severe form of haemophilia - Painful,
prolonged headache - Repeated
vomiting - Extreme
fatigue - Neck pain
- Double
vision
Causes of haemophilia
Haemophilia is
inherited. However, in some people with haemophilia have no family history of
the disorder. In these people haemophilia is caused by a genetic change
(spontaneous mutation).
inherited. However, in some people with haemophilia have no family history of
the disorder. In these people haemophilia is caused by a genetic change
(spontaneous mutation).
The cause of haemophilia
depends on the type which can be classified according to which clotting factor
is deficient:
depends on the type which can be classified according to which clotting factor
is deficient:
- Haemophilia A, the most common type, is caused by insufficient
clotting factor VIII. - Haemophilia B, the second most common type, is caused by
insufficient clotting factor IX. - Haemophilia C, in which signs and symptoms are often mild, is
caused by insufficient clotting factor XI.
Treatment/management of haemophilia
There’s no cure
for haemophilia, most people with the disease can lead fairly normal lives.
However therapies to stop bleeding depend on the type of haemophilia:
for haemophilia, most people with the disease can lead fairly normal lives.
However therapies to stop bleeding depend on the type of haemophilia:
- Mild haemophilia A: Slow injection of the hormone into a vein can
stimulate a release of more clotting factor to stop bleeding. - Moderate to severe haemophilia A or haemophilia B: Bleeding may stop only after an infusion of
recombinant clotting factor or clotting factor derived from donated human
blood. Repeated infusions may be needed if internal bleeding is severe. - Haemophilia C: Clotting factor XI, the factor missing in this
type of haemophilia, plasma infusions are needed to stop bleeding
episodes.
The role of the family in
the control of haemophilia
the control of haemophilia
Family members should ensure that the haemophilia is well managed by
encouraging family member to:
encouraging family member to:
·
avoid medications or drugs that promote bleeding;
aspirin is one such medication and many prescription drugs have anticoagulant
properties.
avoid medications or drugs that promote bleeding;
aspirin is one such medication and many prescription drugs have anticoagulant
properties.
·
maintain a healthy body weight to limit the stress on
your joints, which can lead to bleeding episodes.
maintain a healthy body weight to limit the stress on
your joints, which can lead to bleeding episodes.
·
Exercise regularly exercise to strengthens the joints
and muscles, which helps prevent bleeding.
Exercise regularly exercise to strengthens the joints
and muscles, which helps prevent bleeding.
·
take steps to avoid injuries, accidents and excessive
strain on the joints.
take steps to avoid injuries, accidents and excessive
strain on the joints.
·
should get immunized against hepatitis A and B.
should get immunized against hepatitis A and B.
The role of the community
on the control of haemophilia:
on the control of haemophilia:
Because it is an inherited genetic disease, haemophilia cannot be
prevented, so community leaders should ensure that people in the community have
a proper knowledge on its management and treatment.
prevented, so community leaders should ensure that people in the community have
a proper knowledge on its management and treatment.
The role of government in the control of haemophilia
The government should ensure that individuals who have a family history of haemophilia
benefit from genetic testing and counselling before deciding to have a
baby. It is important for people whose
immediate family members (parents or siblings) have haemophilia or are carriers
to talk to a health professional who specializes in the study of inherited
disorders before becoming pregnant.
benefit from genetic testing and counselling before deciding to have a
baby. It is important for people whose
immediate family members (parents or siblings) have haemophilia or are carriers
to talk to a health professional who specializes in the study of inherited
disorders before becoming pregnant.
