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1. By – SURESH KUMAR ( Nursing Tutor )
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2. Haemophilia
•Hemophilia is a rare disorder in which blood
doesn't clot normally because it lacks sufficient
blood-clotting proteins (clotting factors). If
someone have hemophilia, he/she may bleed
for a longer time after an injury than a normal
person would if his blood clotted normally.

3. Haemophilia
• People with hemophilia have low levels of either
factor VIII (8) or factor IX (9). The severity of
hemophilia that a person has is determined by the
amount of factor in the blood. The lower the amount
of the factor, the more likely it is that bleeding will
occur which can lead to serious health problems.

4. Causes of
Haemophilia
• Hemophilia is caused by a mutation or change, in one of the genes, that
provides instructions for making the clotting factor proteins needed for
blood clotting. This change or mutation can prevent the clotting protein
from working properly. These genes are located on the X chromosome.
Males have one X and one Y chromosome (XY) and females have two X
chromosomes (XX). Males inherit the X chromosome from their mothers
and the Y chromosome from their fathers. Females inherit one X
chromosome from each parent.

5. Causes of
Haemophilia
• The X chromosome may contain defective genes that are not present on
the Y chromosome. This means that males only have one copy of most of
the genes needed for proper blood clotting and that is missing. Thus, males
can have a disease like hemophilia if they inherit an affected X
chromosome that has a mutation in either the factor VIII or factor IX
gene. Females can also have hemophilia, but this is much rarer. In such
cases both X chromosomes are affected or one is affected and the other is
missing or inactive. Mostly females are carriers of haemophilia as they have
one affected x chromosome and one healthy x chromosome.

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10. Classification of
Hemophilia
• There are several different types of hemophilia. The following
two are the most common:
• Hemophilia A (Classic Hemophilia)
This type is caused by a lack or decrease of clotting factor VIII.
• Hemophilia B (Christmas Disease)
This type is caused by a lack or decrease of clotting factor IX.

11. Sign and symptoms
of Hemophilia
• Common signs of hemophilia include:
• Bleeding into the joints. This can cause swelling and pain or tightness in the
joints; it often affects the knees, elbows, and ankles.
• Bleeding into the skin (which is bruising) or muscle and soft tissue causing
a build-up of blood in the area (called a hematoma).
• Bleeding of the mouth and gums, and bleeding that is hard to stop after
losing a tooth

12. Sign and symptoms
of Hemophilia
• Bleeding after having injections, such as vaccinations.
• Bleeding in the head of an infant after a difficult delivery.
• Blood in the urine or stool.
• Frequent and hard-to-stop nosebleeds.

13. Diagnostic
investigations
• Diagnosis includes screening tests and clotting
factor tests. Screening tests are blood tests that
show if the blood is clotting properly. Clotting
factor tests, also called factor assays, are required
to diagnose a bleeding disorder. This blood test
shows the type of hemophilia and the severity.

14. Treatment
• The best way to treat hemophilia is to replace the missing
blood clotting factor so that the blood can clot properly.
This is typically done by injecting treatment products,
called clotting factor concentrates, into a person’s vein. If
clotting factor VIII is missing than factor VIII
concentrates are injected and If clotting factor IX is
missing than factor IX concentrates are injected

15. Treatment
• Some patients will need regular replacement therapy in
order to prevent bleeding. This is called prophylactic
therapy.
• Others receive demand therapy, a treatment that is given
only after bleeding begins and remains uncontrollable.

16. Living with
hemophilia
• There is no way to cure hemophilia, but there are ways to reduce the risk
of excessive bleeding and to protect joints. These include:
• Regular exercise
• Avoiding certain medications, such as aspirin, nonsteroidal anti-
inflammatory drugs, and heparin, which are blood thinners
• Practicing good dental hygiene, preventing injury during shaving or other
outdoor activities.

17. By – SURESH KUMAR ( Nursing Tutor )