HEMOPHILIA IN CHILDREN - ENGLISH
Nursing Notesclick here to subscribe My Student Support System
HEMOPHILIA-
•
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.
•
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.
CAUSES
OF HEMOPHILIA-
•
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.
•
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.
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.
SIGN
AND SYMPTOMS-
•
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
•
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.
DIAGNOSTIC
INBESTIGATIONS-
•
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.
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
•
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.
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.
Thankyou sir
ReplyDelete