DEPARTMENT OF PUBLIC HEALTH OFFICER

THESIS PROPOSAL

PREVALENCE OF HAEMOPHILIA IN
ETHIOPIA AT BLACK LION HOSPITAL

prepared by Abdilaahi Abdi and Mohamed Abdi Hassen

May 05,2023.

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 Abstract
Hemophilia is a disease that causes very sluggish blood clotting and, in extreme cases,
spontaneous bleeding into muscles and joints. It is a hereditary condition largely brought on by a
lack of crucial coagulation or clotting components. This thesis proposal set out to ascertain the
frequency of hemophilia cases among Black Lion Hospital patients over the last ten years. All of
the secondary data that is accessible from the patient records at Black Lion Hospital is going
used to conduct a retrospective study. In order to calculate the relative frequencies of hemophilia
A and hemophilia B during a ten-year period, descriptive statistics is planned to be used. In order
to examine the pertinent data, SPSS program window16 will be used. A total of 123 patients'
medical records over the previous ten years are going to be involved, and they were all for male
patients.

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 Table of Contents

Abstract .......................................................................................................................................................... i
ACRONIMS ................................................................................................................................................... iii
CHAPTER ONE ............................................................................................................................................... 1
1.1 INTRODUCTION ................................................................................................................................... 1
1.1.1BACKGROUND ................................................................................................................................... 1
1.2 STATEMENT OF THE PROBLEM ........................................................................................................... 5
1.3 Research Hypotheses /Questions ....................................................................................................... 6
1.4 OBJECTIVES ......................................................................................................................................... 6
1.4.1GENERAL OBJECTIVE ..................................................................................................................... 6
1.4.2 Specific Objectives ....................................................................................................................... 6
1.5 SIGNIFICANCE...................................................................................................................................... 7
1.6 SCOPE/DELIMITATION OF THE STUDY ................................................................................................ 7
1.7 Organization Of The Study .................................................................................................................. 7
CHAPTER TWO .............................................................................................................................................. 8
LITERATURE REVIEW ................................................................................................................................. 8
CHAPTER THREE .......................................................................................................................................... 14
RESEARCH METHODOLOGY .................................................................................................................... 14
3.1 RESEARCH DESIGN ............................................................................................................................ 14
3.2 POPULATION OF THE STUDY ............................................................................................................. 14
3.3 SAMPLE SIZE AND SAMPLING TECHNIQUE ....................................................................................... 14
3.4 Data Type and Source ....................................................................................................................... 15
3.5 METHOD OF DATA ANALYSIS ............................................................................................................ 15
REFERENCE .................................................................................................................................................. 16

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 ACRONIMS
APPT Activated Partial Thromboplastic time

BLH Black Lion Hospital

CDC Center for Disease Control

CVS Chorionic villi sampling

CF Clotting factor

FBS Fetal Blood Sampling

HA Hemophilia A

HB Hemophilia B

Is Inferential statistics

MOH Ministry of Health

RICE Rest, Ice, Compression and elevation

WFH World Federation of Hemophilia

WHO World Health Organization

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 CHAPTER ONE

1.1 INTRODUCTION

1.1.1BACKGROUND
Hemophilia is a rare disorder in which the blood doesn't clot in the typical way because it doesn't
have enough blood-clotting proteins (clotting factors). If you have hemophilia, you might bleed
for a longer time after an injury than you would if your blood clotted properly. Small cuts usually
aren't much of a problem. If you have a severe form of the condition, the main concern is
bleeding inside your body, especially in your knees, ankles and elbows. Internal bleeding can
damage your organs and tissues and be life-threatening. Hemophilia is almost always a genetic
disorder. Treatment includes regular replacement of the specific clotting factor that is reduced.
Newer therapies that don't contain clotting factors also are being used.

What is Hemophilia?
The word hemophilia derives from two Greek words: haima, meaning blood, and philia, meaning
affection. The blood of a person with hemophilia does not clot normally. He does not bleed more
profusely or more quickly than other people; however, he bleeds for a longer time. Such bleeds
are also called hemorrhages. His blood is lacking a protein that is needed for normal clotting.
Some people with hemophilia lack a protein called factor VIII (pronounced “factor eight”). This
is hemophilia A. Others lack a protein called factor IX (pronounced “factor nine”). Their disease
is called hemophilia B. Many people believe that people with hemophilia bleed a lot from minor
cuts. This is a myth. 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.

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 When was hemophilia first recognized?
Hemophilia was recognized, though not named, in ancient times. The Talmud, a collection of
Jewish Rabbinical writings from the 2nd century AD, stated that male babies did not have to be
circumcised if two brothers had already died from the procedure. The Arab physician Albucasis,
who lived in the 12th century, wrote of a family whose males died of bleeding after minor
injuries. In 1803, a Philadelphia physician, Dr. John Conrad Otto, wrote an account of “a
hemorrhagic disposition existing in certain families.” He recognized that the condition was
hereditaryand affected males. He traced the disease back through three generations to a woman
who had settled near Plymouth, New Hampshire, in 1720. The word “hemophilia” first appears
in a description of the condition written in 1828 by Friedrich Hopff, a German medical student at
the University of Zurich.
What is the history of hemophilia in the 20th century?
In the 20th century doctors looked for the cause of hemophilia. Until then, they had believed
that the blood vessels of people with hemophilia were simply more fragile. Then, in 1937,
researchers found that they could correct the clotting problem by adding a substance that came
from the plasma in blood. This was called anti hemophilic globulin. In 1944, a lab test revealed
that blood from one person with hemophilia was able to correct the clotting problem in a second
person with hemophilia, and vice versa. Each person was found to have a deficiency of a
different protein—factor VIII and factor IX. This led to the recognition in 1952 of hemophilia A
and hemophilia B as two distinct disorders. In the 1960s the clotting factors were identified and
named. An article in the prominent scientific journal Nature, in 1964, described the clotting
process in detail. The interaction of the different factors in blood clotting was named the
coagulation cascade. In the 1950s and early 1960s, people with hemophilia were treated
with whole blood or fresh frozen plasma, a major component of blood.
Unfortunately, the factor VIII and IX proteins were not concentrated enough in these blood
products to stop serious internal bleeding. The body’s circulatory system would be overloaded
before a sufficient quantity of clotting factor concentrate was administered. Many people with
severe hemophilia and some people with mild and moderate hemophilia died in childhood or
early adulthood. The most common causes of death were bleeding in vital organs, especially the
brain, and bleeding after minor surgery or after an injury. Those who survived childhood were
usually crippled by the long term effects of repeated hemorrhages into their joints. The

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complications of prolonged and recurrent bleeding into joints and muscles made hemophilia one
of the most painful diseases known to medicine.
Then, in the 1960s, cryoprecipitate was discovered. The sludge at the bottom of a bag of thawing
plasma was found to be rich in factor VIII. For the first time, enough clotting factor VIII could
be infused to control serious bleeding. Even surgery became possible.
Finally, in the early 1990s, genetically engineered factor called recombinant factor
concentrates came on the market. Recombinant factor concentrates are not made from plasma
and contain little or no human proteins. As a result of these advances, children born with
hemophilia in Canada today can look forward to long, healthy, active and productive lives.
Symptoms

Signs and symptoms of hemophilia vary, depending on your level of clotting factors. If your
clotting-factor level is mildly reduced, you might bleed only after surgery or trauma. If your
deficiency is severe, you can bleed easily for seemingly no reason.

Signs and symptoms of spontaneous bleeding include:

 Unexplained and excessive bleeding from cuts or injuries, or after surgery or dental work

 Many large or deep bruises

 Unusual bleeding after vaccinations

 Pain, swelling or tightness in your joints

 Blood in your urine or stool

 Nosebleeds without a known cause

 In infants, unexplained irritability

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 What causes hemophilia?
Hemophilia is a genetic disorder. This means that it is caused by a gene that does not work
normally. Like other genetic health problems, hemophilia can be passed from generation to
generation. In almost all cases, the gene responsible for hemophilia is passed from a parent to the
child at the time of conception. However, in about 3 out of 10 cases, a son is born to a family
that has no history of hemophilia. There are 3 reasons why this might happen:

1. It could be that hemophilia has been in the family for generations. Because no male showed
signs of bleeding problems, no one knew hemophilia was present. The family may have had
females who were hemophilia carriers. But if none of these women had sons, or none of their
sons had hemophilia, no one would know that hemophilia was being passed on—until a boy was
born with hemophilia.

2. It could be that the boy’s mother developed the gene with the hemophilia mutation when
she was conceived. The mother would be the first person in the family to carry hemophilia.Her
daughters could be carriers; her sons could have hemophilia.

3. It could be that the mutation that causes hemophilia happened when the boy was conceived. In
this case, the egg from his mother developed a mutation and as a result, the boy has hemophilia.
In such a case, the mother is not a carrier but some of her other eggs could also develop the
mutation. (1)

What are other names for hemophilia A and B?

Hemophilia A is called by two other names:
classical hemophilia, because it is the most common of the factor deficiencies; and
factor VIII deficiency hemophilia, because it is the lack of the
 factor VIII protein in the blood that causes the clotting problem.
Hemophilia B also goes by two other names:
• Christmas Disease, named after Steven Christmas, a Canadian who in 1952 was the first person to be
diagnosed with this distinct form of hemophilia; and
• factor IX deficiency hemophilia, because factor IX is the blood protein that is lacking and whose
absence slows down the normal clotting process.

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 Risk factors:
• Genetics.
• Gender; It affects males more than females.
• Pregnancy.
• Cancer
• Auto immune diseases (such as: multiple sclerosis).

Who is affected by hemophilia?

Hemophilia affects people of all races, colors and ethnic origins around the world. The most
severe forms of hemophilia affect almost only males. Females can be seriously affected only if…
• the father has hemophilia and the mother is a carrier.
• the female experiences lyonization, also called X-inactivation.
In this case, she has one abnormal X chromosome with the hemophilia gene and one normal
X chromosome; however, the normal X chromosome is inactive in the production of
the clotting protein. Both these situations are extremely rare. However, many females who are
carriers have symptoms of mild hemophilia. We are only now fully recognizing that carriers can
have bleeding problems and that this can affect their quality of life.(2)

1.2 STATEMENT OF THE PROBLEM

This thesis proposal set out to ascertain the frequency of hemophilia cases among Black
Lion Hospital patients over the last ten years. The study is expected to determine and investigate
the prevalence of hemophilia in the case of black lion hospital. Finally the thesis is expected to
determine prevalence of hemophilia and generally provide enough information regarding the
prevalence of hemophilia in Ethiopia.
Hemophilia A is called by two other names:
• classical hemophilia, because it is the most common of the
factor deficiencies; and
• factor VIII deficiency hemophilia, because it is the lack of the
factor VIII protein in the blood that causes the clotting
problem.
Hemophilia B also goes by two other names:
• Christmas Disease, named after Steven Christmas, a Canadian
who in 1952 was the first person to be diagnosed with this
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 distinct form of hemophilia; and
• factor IX deficiency hemophilia, because factor IX is the blood
protein that is lacking and whose absence slows down the
normal clotting process.
■ How common is hemophilia?
Both hemophilia A and B are very rare disorders. Hemophilia A
affects fewer than 1 in 10,000 people, or about 2,500 Canadians.
Hemophilia B is even less common, affecting approximately 1 in
50,000 people, or about 600 Canadians.

1.3 Research Hypotheses /Questions

 What is the frequency of hemophilia case among Black Lion Hospital over the past 10 years?
 What is the prevalence of hemophilia in the cases of Black Lion hospital?
 What are the trends of hemophilia cases in Black lion hospital in the last ten years?
 What is the persistence of hemophilia cases at black lion hospital?

1.4 OBJECTIVES

1.4.1GENERAL OBJECTIVE
The general objective of this project based study was to determine the prevalence of
hemophilia cases among patients diagnosed at Black Lion Specialized Teaching Hospital,
Addis Ababa, for the past ten consecutive years (between 2005 and 2014).

1.4.2 Specific Objectives

 To determine the frequency of hemophilia A and hemophilia B among the patients
visiting Black Lion Specialized Teaching Hospital between 2005 and 2014.
 To compare the relative frequency of the two common types of hemophilia among
the patients visiting Black Lion Specialized Teaching Hospital between 2005 and
2014.
 To analyze the trends of hemophilia cases for the last ten years within Black Lion
Specialized Teaching Hospital.

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 1.5 SIGNIFICANCE
 To determine the frequency of hemophilia A and hemophilia B among the patients visiting
Black Lion Specialized Teaching Hospital between 2005 and 2014.
 To compare the relative frequency of the two common types of hemophilia among the
patients visiting Black Lion Specialized Teaching Hospital between 2005 and 2014.
 To analyze the trends of hemophilia cases for the last ten years within Black Lion
Specialized Teaching Hospital.
Generally this study will contribute to the existing literature on the effects of hemophilia in
Ethiopia and Ethiopia by using the latest available data. Finally, this study will point out
areas for further study in a broader scope by other researchers.

1.6 SCOPE/DELIMITATION OF THE STUDY

The study is planned to be conducted in One of the largest Hospitals in Ethiopia At Black lion Hospital,
Addis Ababa. This study investigates the effects of hemophilia in Ethiopia. Accordingly, the data
employed in this study is limited to the period between 2005 and 2014.

1.7 Organization Of The Study

This study consists of three chapters. The first chapter is an introductory part that provides
relevant background about the study. The theoretical and empirical reviews are discussed in
chapter two. Chapter three focuses on the research methodology which includes the
research design, theoretical framework, model specification, definition and measurement of
variables and data source.

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 CHAPTER TWO

LITERATURE REVIEW
Hemophilia is a bleeding disorder that affects people. Hemophiliacs can bleed for a longer
period of time but do not bleed any quicker than healthy people. Their blood is insufficient.
clotting elements. Blood contains a protein called clotting factor that regulates bleeding (3).

According to Shirmiati et al. (2012), hemophilia in most cases is inherited, meaning that it
is passed on through a parent’s gene; Genes carry messages about the way the body's cells will
develop as a baby grows in to an adult. The disorder continues to their grandsons through their
obligate carrier daughters (William et al., 2004). Hemophilia sometimes can occur when there is
no family history of it. This is called sporadic hemophilia. About 30% of people with hemophilia
did not get it through their parents genes, instead it was caused by a change in the persons own
genes. Hemophilia always affects male because, it is inherited with the X chromosomes and
males have only one X, while females have two of the X chromosomes (Francine et al., 2008).
The transmission pattern of hemophilia is also consistent with the condition for an X-linked
recessive disorder (Lewis, 2007).

The blood disorder manifest itself clinically in two major forms, hemophilia A and hemophilia B
based upon the deficiency of either of the two important coagulation factors (Yoshitake et al.,
1983, Stephal et al., 2003). According to the report of World Hemophilia Federation (WHF
2012), clinical symptoms of hemophilia A and B are more or less similar. The common
symptoms include: big bruises, bleeding into muscles and joints, spontaneous bleeding after a cut
and bleeding for a long time after an accident.

Approximately one in 5,000 males are born with hemophilia A, and one in 30,000 males are born
with hemophilia B (Gitschier et al., 1984, Mannucci et al., 2004). Hemophilia affects people of
all races and ethnic orgins globally (Yoshitake et al., 1985, Peake et al., 1993). The genetic
disorder of hemophilia is caused by an inherited X- linked recessive trait, with the defective gene
located on the X chromosomes. Female have two copies of the X chromosomes, so if the factor
VIII gene on one chromosome doesn’t work, the gene on the other chromosome can do the job of
making enough factor VIII. Males however, have only one X chromosome. So if the factor VIII
gene on that chromosome is broken, they will have hemophilia A. Thus, most people with
hemophilia A are male. Which means that, hemophilia is sex linked character almost exclusively
8
restricted to males, women can carry the gene and pass it on to their sons without being affected
themselves (Oxford, 2010, Jaguru, 2014). If a woman has adequate active factor VIII gene, she is
considered a carrier. This means the defective gene can be passed down to her children. In a
woman who carries the defective gene, any of her male children will have a 50% chance of
hemophilia A, while any of her female children will have a 50% chance of being carriers all
female children of men with hemophilia carry the defective gene (Stephal et al., 2003, Lewis,
2007)

According to journal of management of hemophilia in developing counties by Kanjaksha Ghosh

,There are significant challenges in managing haemophilia patients in developing countries.
These challenges are

 Lack of proper health care infrastructure and human resources suitable for haemophilia
care
 Competing health care priorities of the government.
 Lack of penetrance of medical insurance in the population.
 Lesser visibility of the haemophilia patients in health care system
 Low awareness across the medical profession, population and the policy makers about
the condition
 Non availability of factor concentrates
 Inadequate utilization of knowledge for reducing factor concentrate use.
 Inadequate pain relief
 Challenges due to inhibitor developing
 Viral hepatitis &
 Lack of research publications relevant to the country are some of the challenges faced by
PWH for their management in developing country.

The solutions are not easy but development of a strong patient organization with linkages
with World Federation of Hemophilia is an important initial step. Following that internal and
international twinning, use of internal sources, strong advocacy program targeting government,
doctors, opinion makers will solve many of the challenges in the time to come.

Hemophilia is a sex-related hereditary bleeding illness brought on by a total or partial lack of

clotting factor VIII that is passed down through the X chromosomes (Rosendaal et al., 2006).

9
Mother to son transmission is the typical pattern. The mother, who has two X chromosomes as
part of her 46 chromosomes, gives one of them to her son after conception. His father is the
source of his Y-chromosome. Hemophilia can result from a male inheriting a faulty copy of the
X-chromosome from his mother (Francine et al., 2008; Manuel, 2008).

Hemophilia is typically hereditary, which means that a parent's gene causes it to be passed down.
Genes transmit information on how a person's body cells will change as they mature from a baby
to an adult. Sometimes even if there is no family history of hemophilia, it might nonetheless
happen. We refer to this as sporadic hemophilia.

About 30% of hemophiliacs did not inherit their condition from their parents. As stated by
Stephal et al. (2003), Lewis (2007), Shrimati et al. (2012), it was brought on by a change in the
individual's own gene.

Hemophilia is always a male disease, according to Franchini et al. (2008), because men have
only one x chromosome whereas women have twoRare examples of persons contracting the
illness without being born with it also exist, as do rare cases of females inheriting the illness.

The hemophilia-A gene is found at Xq28, while the hemophilia-B gene is found at Xq27.1-
q27.2. The Xq28 region is linked to roughly thirty other illnesses, including favism and manic
depression. This must mean that many distinct genes that are part of Xq28 but have not yet been
identified (Stephal et al., 2003; Lewis, 2007).

Cytogenetic studies have revealed that, changes in the F8 gene are responsible for hemophilia A
(OMIM 306700), while mutations in the F9 gene cause hemophilia B (OMIM 306900). The F8
gene provides instructions for making a protein called coagulation factor VIII. A related protein,
coagulation factor IX, is produced from the F9 gene. Coagulation factors are proteins that work
together in the blood clotting process. After an injury, blood clots protect the body by sealing off
damaged blood vessels and preventing excessive blood loss. Mutations in the F8 or F9 gene lead
to the production of an abnormal version of coagulation factor VIII or coagulation factor IX, or
reduce the amount of one of these proteins. The altered or missing protein cannot participate
effectively in the blood clotting process. As a result, blood clots cannot form properly in
response to injury. These problems with blood clotting lead to continuous bleeding that can be
difficult to control (Hoffman, 2003, Ahmad et al., 2003, Genetic Home Reference, 2012).

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Hemophilia is a bleeding problem among human beings. People with hemophilia do not bleed
any faster than normal; they can bleed for a longer time. Their blood does not have enough
clotting factors. Clotting factor is a protein in blood that controls bleeding (Mannuci et al., 2004,
WFH, 2012).
Hemophilia is sex linked hereditary bleeding disorder caused by a complete or partial deficiency
of clotting factor VIII (Rosendaal et al., 2006) transmitted through the X-chromosomes. The
usual transmission is form mother to son. The mother has two X-chromosomes as a part of her
forty six (46) chromosomes and at conception gives one to her son. He gets a Y- chromosome
from his father. If the X- chromosome the boy gets from his mother is defective, he will have
hemophilia (Francine et al., 2008, Manuel, 2008).
Hemophilia is usually inherited, meaning that it passes on through a parent’s gene. Genes carry
message about the way the body cells will develop as a baby grows in to an adult. Sometimes
hemophilia can occur when there is no family history of it. This is called sporadic hemophilia.
About 30% of people with hemophilia didn’t get it through their parents’ genes. It was caused by
a change in the person’s own gene (Stephal et al., 2003, Lewis, 2007, Shrimati et al., 2012).
According to Franchini et al. (2008), hemophilia always affects male because it is inherited with
the x0 chromosome and males have only one x, while female have two. Rare cases of female
inheriting the disease do exist, through and rare cases of people acquiring the disease without
having been born with it.
The gene for hemophilia-A is located at Xq28 while the gene for hemophilia-B is located at
Xq27.1-q27.2. There are about thirty other disorders associated with the Xq28 area including
manic depression and favism. This must indicate that Xq28 includes many different genes which
have not yet been isolated (Stephal et al., 2003, Lewis, 2007).

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 Symptoms of Hemophilia

According to Gitschier et al.,(1984) and Rangietal, (2011), the severity of symptom can vary and
the severe form becomes apparent early on. Bleeding is the main symptom. It is often first seen
when the infant is circumcised. Additional bleeding problems usually show up when the infant
starts crawling and walking. The symptoms can include: bleeding in to joints and associated pain
and swelling, blood in the urine and stool, bruising, excessive bleeding following circumcision,
gastrointestinal tract and urinary tract hemorrhage, nose bleeds, prolonged bleeding from cuts,
tooth extraction and surgery and spontaneous bleeding.

Diagnosis and Treatment

Diagnosis of Hemophilia

Many people who have or have had family members with hemophilia will ask that their baby
boys get tested soon after birth. About one-third of babies who are diagnosed with hemophilia
have a new mutation not present in other family members. In these cases, a doctor might check
for hemophilia if a newborn is showing certain signs of hemophilia. To make a diagnosis,
doctors would perform certain blood tests to show if the blood is clotting properly. If it does not,
then they would do clotting factor tests, also called factor assays, to diagnose the cause of the
bleeding disorder. These blood tests would show the type of hemophilia and the severity
(Aledort et al., 1994, Stephal et al., 2003, Baker et al., 2005). Hemophilia is diagnosed by taking
a blood sample and measuring the level of factor activity in the blood. Hemophilia A is
diagnosed by taking the level of factor VIII activity. Hemophilia B is
diagnosed by measuring the level of factor IX activity. If the mother is a know carrier of
hemophilia, testing can be done before a baby is born. Parental diagnosis for hemophilia can be
carried out between 9 to 11 weeks of pregnancy using chorionic villi sampling (CVS) or by using
fetal blood sampling (FBS) at a later stage of pregnancy, that is at 18 or more weeks (Escuriola
et al., 2001, Baker et al., 2005, WFH, 2012).

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 Treatment of Hemophilia

Hemophilia is not curable [4], but in the more severe cases of hemophilia
there are effective treatment options available. Unfortunately only 25% of people receive
adequate treatment. Without treatment hemophiliacs can experience crippling pain, severe joint
damage and disability (Escuriola et al., 2001). Clotting factor replacement therapy which uses
plasma derived clotting factor concentrates is often used as treatment. (World book, 2003).They
are taken from the plasma that is pooled from thousands of blood donors. The plasma has to be
pooled and collected and processed to separate the clotting factors
(http/www.medprorx.com/multimidia.htm). The blood donors have to be very carefully screened
so that blood borne diseases would not be transmitted (Escuriola et al., 2001, CDC, 2011).
Prior to 1960, treatment of hemophilia involved massive blood transfusions, which were
largely ineffective and even dangerous; because of the huge volumes of blood needed to give the
patient enough clotting factor VIII. In the sixties and seventies techniques were developed to
give a concentrated form of clotting factor by isolating the protein from the blood plasma of
numerous donors. Unfortunately this contributed to the HIV infection among hemophiliacs, since
one infected donor could infect an entire batch of clotting factor. The purification processes
which are now performed, along with the high cost of producing the clotting factor have driven
the price of the drug to astronomical levels. A year’s supply of clotting factor for a severe
hemophiliac can easily run $100,000 dollars a year (Mannucci et al., 2004).
Treatment includes replacing the defective clotting factor (Ghosh et al., 2001, Medline, 2012).

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 CHAPTER THREE

RESEARCH METHODOLOGY

3.1 RESEARCH DESIGN

The study's design used a cross-sectional method to examine hemophilia cases at Black Lion
Hospital from 2005 to 2014 across a ten-year period. In order to ascertain the prevalence, trends,
and distributions of the illness at the study hospital, all patients, regardless of sex, who were
diagnosed as positive for hemophilia cases both clinically and in laboratory over the preceding
10 years, are planned to be used.

3.2 POPULATION OF THE STUDY

All the data of the patients including their age, sex and the type of Hemophilia they are caught of
whose records were available regardless of the sex of the patients from the hospital Record
Office over ten years (2005-2014) are used as study subjects in this project based research. The
recorded data in the hospital record office for the past ten years totally were 3,020,875 and the
data also is going to be grouped according to Ethiopian population age structure in to three.
These are 0-14 children, 15-64 working age, 64 and above older.

3.3 SAMPLE SIZE AND SAMPLING TECHNIQUE

Sample size for patients based on the parameter with the greatest standard deviation in relation to

its size, . A formula for difference between means used (5)

A total of 379 paired subject were included giving 90% power to detect a difference in mean

percentage of 10%, given a standard deviation of 10.0 (36).

n=2(SD)2 (Z1-α/2 +Z1-β)2

2(10)2 7.9 = 316, 20 % non response rate =379

50

α --- 0.05

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β --- 0.20

Power= 1- β = 0.80

◊ -- Clinically significant difference

Total sample size=379

Generally 379 individuals with hemophilia cases who had enrolled at Black Lion hospital ART unit
from September 1 2005 to August 31 2010 are planned to be included in the study using Convenience
sampling method.

3.4 Data Type and Source

All secondary data on hemophilia cases at Black Lion Hospital from 2005 to 2014 are the
data types used in this investigation. In addition to being the Federal, Referral, and Teaching
hospital, the hospital was purposefully chosen because of the significant patient traffic from
diverse urban and rural areas of the country. The prevalence of hemophilia cases among patients
visiting Black Lion Hospital was ascertained through systematic document analysis using the
patient's medical file. All cases of hemophilia were counted in the records (collectively
exhaustive) and each case of hemophilia was counted only once (mutually exclusive).
Hemophilia cases are routinely tracked, and it is then intended that they would be evaluated and
summarized using a health record review format that was created by principal investigator.

3.5 METHOD OF DATA ANALYSIS

The collected is expected to be organized and analyzed by using descriptive statistical tools such
as frequencies and percentages and the qualitative part will be systematically documented.

15
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international medicine, 236:391-399.
3. Escuriola, E., Halimeh,S., Kumik, K. Schobess, R., Wermes, C. 2001. Symptomatic on
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