I n f o r m a t i o n b o o k l e t o n
Mild hemophilia can be very serious and
even life threatening if injuries or bleeds are
not treated promptly and adequately.
This booklet was made possible
by an unrestricted educational grant from
Ta b l e o f
C o n t e n t s
Introduction to Mild Hemophilia 1
Inheritance of Hemophilia 5
Hemophilia Treatment Centre (HTC) 7
Recognizing a bleed 8
Treatment options 17
First Aid 18
Healthy lifestyle 22
Fast facts for managing hemophilia 24
The Canadian Association of Nurses in Hemophilia Care (CANHC) recognizes the need
to ensure a high standard of nursing practice, education and research. In order to achieve
quality nursing care for people affected by hemophilia and other bleeding disorders, we
strive to enhance professionalism through partnerships collegiality
A c k n ow l e d g m e n t s
Dorine Belliveau, BScN., Moncton, New Brunswick
Annette Flanders, BN., Halifax, Nova Scotia
Marilyn Harvey, BN., St. John’s, Newfoundland
Sue Ann Hawes, RN., Halifax, Nova Scotia
Carol Mayes, RN., Saint John, New Brunswick
Lynn Payne, RN., Halifax, Nova Scotia
Charlotte Sheppard, BN., St. John’s, Newfoundland
The Atlantic Hemophilia Nurses Group gratefully acknowledges the Canadian
Hemophilia Society and Bayer HealthCare for their continued support and
commitment to the Canadian Association of Nurses in Hemophilia Care.
We express our gratitude to the nurses, physicians, and individuals with
bleeding disorders who reviewed this booklet and provided us feedback.
A special thanks for the contributions from the following physiotherapists:
Carolyn Jarock, BSc. P.T., Halifax, Nova Scotia
Patty Patstone, BSc. P.T., Saint John, New Brunswick
Annette Vautour, BSc. P.T., Moncton, New Brunswick
Copyright © 2007
First Edition, October 2007
I n t r o d u c t i o n t o
M i l d H e m o p h i l i a
Mild hemophilia can be very serious and even life threatening if
injuries or bleeds are not treated promptly and adequately.
The danger is that a person with mild Hemophilia, having so few
bleeds will not recognize one when it occurs or will not know what
to do. It is important to contact your Hemophilia Treatment Centre
after injury and before surgery or dental extraction. Learn how to
W h a t i s h e m o p h i l i a ?
Hemophilia is a genetic disorder characterized by a missing or a decreased amount
of one of the clotting proteins in the blood.
Some people with hemophilia lack a protein called Factor VIII (8).This is hemophilia A
(Classic Hemophilia) and is the most common type. Other people lack a protein
called factor IX (9).This is hemophilia B (Christmas Disease).
It is a myth that people with hemophilia bleed profusely from minor cuts.The reality
is that external wounds are usually not serious. Far more important is internal
bleeding.This occurs in joints, especially knees, ankles and elbows; and into tissues
and muscles. When bleeding occurs in a vital organ, especially the brain, the person’s
life is in danger.
W h o i s a f fe c t e d by h e m o p h i l i a ?
Hemophilia is found all around the world and affects all races equally. Hemophilia
usually affects males, but in rare situations females can also have hemophilia. Women
who are carriers for hemophilia may or may not have bleeding symptoms.
H ow c o m m o n i s h e m o p h i l i a ?
Hemophilia A and Hemophilia B are very rare. Hemophilia A affects 1 in 10,000
people. Hemophilia B is less common, affecting 1 in 35,000 people.
H ow s e r i o u s i s h e m o p h i l i a ?
There are 3 levels of hemophilia, mild, moderate and severe, depending on the
amount of factor VIII or factor IX present in the blood.The normal range of factor
VIII and IX is 50-200%.
L e ve l o f f a c t o r V I I I o r I X i n t h e bl o o d
Less than 1%
People with mild hemophilia usually only bleed during or after significant injury,
surgery or dental extraction. A person with mild hemophilia may experience very
few bleeding episodes in their lifetime. Some people with mild hemophilia are not
diagnosed until they reach adult age.
H ow d o e s h e m o p h i l i a a f fe c t b l o o d ?
Blood is carried through the body within a network of blood vessels. When tissues
are injured, damage to a blood vessel may occur and result in leakage of blood
through holes in the vessel wall.The vessel can break deeper inside the body,
making a bruise or an internal hemorrhage.
The blood needs to form a clot (plug) to stop bleeding. Proteins in the blood, work
together through a series of steps to form a clot. When one of the proteins such as
factor VIII or IX, is missing or decreased, the chain reaction will not work properly.
Clotting does not happen or is delayed causing the clot to be soft and easily
dislodged.This can cause bleeding for prolonged periods of time. Bleeding may
re-occur hours or days later.
A c t i o n o f c l o t t i n g f a c t o r s
N o r m a l
Clotting factors are activated when a vessel breaks
One factor activates the next - a clot is formed
H e m o p h i l i a Factor VIII or IX
If factor VIII or IX is defective
activation stops - no clot is formed
* symbolic representation
I n h e r i t a n c e o f H e m o p h i l i a
Father with hemophilia
Carrier mother and
father with hemophilia
I n h e r i t a n c e o f
H e m o p h i l i a
Hemophilia is an inherited disorder.This means it is passed from
generation to generation, from the parent to the child. People with
mild hemophilia pass on mild hemophilia. Family members should
be tested if there is someone in the family who has hemophilia.
Hemophilia is caused by a gene on the “X” chromosome that does not work
normally. Genes are found in the body and contain information that makes a person
unique. Females have two” X” genes and males have an “X” gene and a “Y” gene.
Females who have the “X” gene with the hemophilia trait are called carriers.They also
have a normal “X” and do not usually have bleeding problems. Carriers of hemophilia
have a 50% chance of passing on the hemophilia X gene. Sons who receive this gene
will have Hemophilia, daughters who receive this gene will be carriers (see diagram).
Daughters of males who have the Hemophilia X gene will automatically be carriers
this is called an “obligate carrier”. Males with Hemophilia will not pass on hemophilia
to their sons.
In rare situations, a child is born with hemophilia when there is no family history.
This is called a spontaneous occurrence. When this child grows up and decides to
begin a family, s/he will start his/her own generation of hemophilia and possibly
pass it on to his/her children.