2. DEFINITION
Hemophilia is an inherited bleeding disorder.
Children with hemophilia can’t stop bleeding
because they don’t have enough clotting factor in
their blood. Clotting factors are needed for blood to
clot. Blood clots to prevent excessive bleeding.
There are many blood clotting factors involved in the
forming of clots to stop bleeding. There are 2 common
factors that affect blood clotting. These are factor VIII
and factor IX.
3. TYPES OF HEMOPHILIA
The 3 main forms of hemophilia include:
•Hemophilia A. This is caused by a lack of the blood clotting factor VIII. About
9 out of 10 people with hemophilia have type A disease. This is also referred to as
classic hemophilia or factor VIII deficiency.
•Hemophilia B. This is caused by a deficiency of factor IX. This is also called
Christmas disease or factor IX deficiency.
•Hemophilia C. Some doctors use this term to refer to a lack of clotting factor
XI.
4. CAUSES
Hemophilia types A and B are inherited diseases.
They are passed on from parents to children through a gene on the X
chromosome.
Females have 2 X chromosomes, while males have 1 X and 1 Y chromosome.
A female carrier has the hemophilia gene on 1 of her X chromosomes. When a
hemophilia carrier female is pregnant, there is a 50/50 chance that the hemophilia
gene will be passed on to the baby.
If the gene is passed on to a son, he will have the disease.
If the gene is passed on to a daughter, she will be a carrier.
If the father has hemophilia but the mother does not carry the hemophilia gene,
then none of the sons will have hemophilia disease, but all of the daughters will be
carriers.
In about 1/3 of the children with hemophilia, there is no family history of the
disorder. In these cases, it’s believed that the disorder could be related to a new
gene flaw.
5.
6. CLINICAL MENIFESTSATION
Symptoms may include:
Bruising
Bleeding in
the brain
Bleeding into
the muscles
Bleeding
into a joint
Easy
bleeding
Other
bleeding
8. TREATMENT
Treatment will depend on symptoms, age, and general health.
It will also depend on how severe the condition is.
Treatment for hemophilia is aimed at preventing bleeding complications
(mainly head and joint bleeds).
Treatment may include:
Bleeding in the joint may need surgery or immobilization. Your child
may need rehab of the affected joint. This may include physical therapy
and exercise to strengthen the muscles around the area.
Blood transfusions may be needed if major blood loss has occurred.
This is when your child gets donated blood.
Self-infused factor VIII or IX can allow a child with hemophilia to lead
a near normal lifestyle.
9. COMPLICATIONS
Complications of hemophilia can include:
•Bleeding in the joints or muscles
•Inflammation of the joint lining
•Long-term joint problems
•Very serious tumor-like enlargements, of the muscle and bone
•Development of antibodies against clotting factors
•Infections from transfusions
10. CARE OF A HEMMOPHILIC CHILD
Managing your child's hemophilia may include:
•Letting your child take part in activities and exercise, but not ones that may cause injury.
These include soccer, rugby, wrestling, motocross, and skiing.
•Preventing dental and gum problems with good dental hygiene.
•Getting vaccines under the skin instead of in the muscle to prevent bleeding in the
muscle.
•Not giving your child aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs).
•Wearing medical identification (ID) in the case of an emergency.
11. Key points about hemophilia in children
•Hemophilia is an inherited bleeding disorder. It causes
an affected child to have low levels of blood clotting
factors.
•The most common symptom of hemophilia is increased,
uncontrollable bleeding.
•Giving factor VIII or IX can allow a child with hemophilia
to lead a near normal lifestyle.
