Professional Documents
Culture Documents
Readings About Hemophilia: Submitted By: Mary Elijah Jane P. Popes
Readings About Hemophilia: Submitted By: Mary Elijah Jane P. Popes
Submitted by:
Mary Elijah Jane P. Popes
BSN 2-2
Submitted to:
Maricel F. Nagamos, RN
Clinical Instructor
Hemophilia
Hemophilia is usually an inherited
bleeding disorder in which the blood
does not clot properly. This can lead
to spontaneous bleeding as well as
bleeding following injuries or
surgery. Blood contains many
proteins called clotting factors that
can help to stop bleeding. 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.
Hemophilia is a rare disorder in which your blood doesn't clot normally because
it lacks sufficient blood-clotting proteins (clotting factors). If you have
hemophilia, you may bleed for a longer time after an injury than you would if
your blood clotted normally. Small cuts usually aren't much of a problem. If you
have a severe deficiency of the clotting factor protein, the greater health
concern is deep bleeding inside your body, especially in your knees, ankles and
elbows. That internal bleeding can damage your organs and tissues, and may be
life-threatening.
Types
There are several different types of hemophilia. The following two are the most
common:
Who is Affected
Hemophilia occurs in about 1 of every 5,000 male births. Based on recent study
that used data collected on patients receiving care in federally funded
hemophilia treatment centers during the period 2012-2018, about 20,000 as
many as 33,000 males in the United States are living with the disorder.
Hemophilia A is about four times as common as hemophilia B, and about half of
those affected have the severe form. Hemophilia affects people from all racial
and ethnic groups.
Inhibitors
Symptoms
A simple bump on the head can cause bleeding into the brain for some people
who have severe hemophilia. This rarely happens, but it's one of the most
serious complications that can occur. Signs and symptoms include:
Repeated vomiting
Sleepiness or lethargy
Double vision
Convulsions or seizures
Causes
There are several types of hemophilia, and most forms are inherited. However,
about 30% of people with hemophilia have no family history of the disorder. In
these people, an unexpected change occurs in one of the genes associated with
hemophilia.
Pregnancy
Autoimmune conditions
Cancer
Multiple sclerosis
Hemophilia inheritance
In the most common types of hemophilia, the faulty gene is located on the X
chromosome. Everyone has two sex chromosomes, one from each parent. A
female inherits an X chromosome from her mother and an X chromosome from
her father. A male inherits an X chromosome from his mother and a Y
chromosome from his father. This means that hemophilia almost always occurs
in boys and is passed from mother to son through one of the mother's genes.
Most women with the defective gene are simply carriers and experience no signs
or symptoms of hemophilia. But some carriers can experience bleeding
symptoms if their clotting factors are moderately decreased.
Risk factors
The biggest risk factor for hemophilia is to have family members who also have
the disorder.
Complications
Damage to joints. Internal bleeding may also put pressure on your joints,
causing severe pain. Left untreated, frequent internal bleeding may cause
arthritis or destruction of the joint.
Diagnosis
For people with a family history of hemophilia, it's possible to determine during
pregnancy if the fetus is affected by hemophilia. However, the testing poses
some risks to the fetus. Discuss the benefits and risks of testing with your
doctor. In children and adults, a blood test can reveal a clotting-factor
deficiency. Depending on the severity of the deficiency, hemophilia symptoms
can first arise at various ages. Severe cases of hemophilia usually are diagnosed
within the first year of life. Mild forms may not be apparent until adulthood.
Some people first learn that they have hemophilia after they bleed excessively
during a surgical procedure.
Treatment
Several different types of clotting factors are associated with different varieties
of hemophilia. The main treatment for severe hemophilia involves receiving
replacement of the specific clotting factor that you need through a tube placed in
a vein. This replacement therapy can be given to combat a bleeding episode
that's in progress. It can also be administered on a regular schedule at home to
help prevent bleeding episodes. Some people receive continuous replacement
therapy. Replacement clotting factor can be made from donated blood. Similar
products, called recombinant clotting factors, are manufactured in a laboratory
and aren't made from human blood.
Physical therapy. It can ease signs and symptoms if internal bleeding has
damaged your joints. If internal bleeding has caused severe damage, you
may need surgery.
First aid for minor cuts. Using pressure and a bandage will generally take
care of the bleeding. For small areas of bleeding beneath the skin, use an
ice pack. Ice pops can be used to slow down minor bleeding in the mouth.
Vaccinations. Although blood products are screened, it's still possible for
people who rely on them to contract diseases. If you have hemophilia,
consider receiving immunization against hepatitis A and B.
Avoid certain pain medications. Drugs that can aggravate bleeding include
aspirin and ibuprofen (Advil, Motrin IB, others). Instead, use
acetaminophen (Tylenol, others), which is a safer alternative for mild pain
relief.
Avoid blood-thinning medications. Medications that prevent blood from
clotting include heparin, warfarin (Coumadin, Jantoven), clopidogrel
(Plavix), prasugrel (Effient), ticagrelor (Brilinta), rivaroxaban (Xarelto),
apixaban (Eliquis), edoxaban (Savaysa) and dabigatran (Pradaxa).
Protect your child from injuries that could cause bleeding. Kneepads,
elbow pads, helmets and safety belts all may help prevent injuries from
falls and other accidents. Keep your home free of furniture with sharp
corners.
Get a medical alert bracelet. This bracelet lets medical personnel know
that you or your child has hemophilia, and the type of clotting factor
that's best in case of an emergency.
Talk with a counselor. You may be concerned about striking the right
balance between keeping your child safe and encouraging as much normal
activity as possible. A social worker or therapist with knowledge of
hemophilia can help you cope with your concerns and identify the
minimum limitations necessary for your child.
Let people know. Be sure to tell anyone who will be taking care of your
child — babysitters, workers at your child care center, relatives, friends
and teachers — about your child's condition. If your child plays
noncontact sports, be sure to tell coaches, too.
References:
Hemophilia treatment has come a long way. Most people lead full, healthy
lives with careful management of their condition. Severe Hemophilia presents at
an early age; therefore, attention to family history and early bleeding episodes is
critical. Prophylactic factor therapy should be started at an early age to prevent
major bleeding complications.