M4 &

Introduction

3. Discuss the etiology, anatomy and

physiology of the blood and the coagulation
cascade;
4. Trace the pathophysiology of Hemophilia
and identify clinical manifestations and risk
factors of the said disease; and
5. Identify the medical and nursing
management appropriate for the disease.

Hemophilia
is an inherited blood clotting disorder,
changes in the Factor VIII and IX gene.
These genes provide instruction for
coagulation factor. Mutation in the F8 or F9
gene lead to the production of an abnormal
amount of coagulation factors or reduce the
amount of one of these clotting proteins.

Many famous people that were afflicted

with hemophilia such as Alexander ,
Abraham Lincoln and Queen Victoria
(carrier of the disease) (because of these
this disease is known as ROYAL
DISEASE)
Dr. JOHN
CONRAD OTTO ( Philadelphian physician)
discovered hemophilia
- He recognized that the condition was hereditary and
affected males. And passed down by healthy females.

- He traced disease back through three

generation to a women ,in 1720

There are several types of hemophilia.

They are classified according to which
clotting factor is deficient:
Hemophilia A, the most common type, is
caused by insufficient clotting factor VIII.
Hemophilia B, the second most
common type, is caused by insufficient
clotting factor IX.

Objectives

General:
At the end of the case presentation, the
participants will be able to acquire the
necessary knowledge, skills and attitudes
in delivering compassionate and quality
nursing care for patients with Hemophilia.

Specific:
At the end of the case presentation, the
participants will be able to:
1. Define and familiarize Hemophilia and its
types;
2. Know the different drugs and their actions
and perform necessary nursing
responsibilities for each drug;

Incidence

About 18,000 people in the United States.

Hemophilia A Represents 80%
Hemophilia B In 4000 to 1 in 5000 males
worldwide.
Hemophilia usually occurs in males (with
rare exceptions). About 1 in 5,000 males are
born with hemophilia each year.

Patients
Profile

Name:
Patient X
Age:
4 y/o
Gender:
Male
Nationality: Pakistani
Date and Time of Admission: 04/08/1435 @ 9:56
PM
Date and Time of Discharge: 10/08/1435 @ 6:52
PM
Chief Complaint: Bleeding from the mouth
Admitting Diagnosis: Hemophilia A
Attending Physician: Dr. Ahmed Al Rashdan/ Dra.
Rafah

History of
Illness

PAST ILLNESS:
This patient was diagnosed as a known
case of Hemophilia. He was diagnosed
with this disease since he was 2 years old.
It was first detected when the patient had
excessive bleeding after a trauma. The
patient was frequently admitted in our
institution for management of bleeding.

10 days prior to admission, the patient

was admitted in PICU due to history of
foreign body ingestion (battery). Upper GI
endoscopy was done for foreign body
removal from the esophagus. No upper GI
bleeding noted.

PRESENT ILLNESS:
The condition started two days ago,
when the patient developed bleeding from
the mouth (soft palate) after trauma from a
lollipop stick. No history of bleeding from
any other site noted. Because of that, the
patient went to ER for consultation and
was received Factor VIII BID for 2 days.
However the bleeding did not stop, so they
went back to ER again. Routine blood
investigations were done.

In initial CBC showed that Hemoglobin level

was only 6.5 (Low Abnormal). After seeing
the reports, Dr. Qadeer advised admission
to M4. Normal Saline 150 ml was given also
from there. From ER, blood requests for 200
ml PRBC transfusion and 150 ml FFP as
standby were sent.
Upon admission to M4, the patient was
conscious, coherent, not in respiratory
distress. However the child looks pale in

color and weak in appearance. Vital signs

taken and recorded. IVF and medications
started as ordered. Due blood works done
and collected. After 2 hours stay in the
ward, continuous and active bleeding from
the mouth still noted. Because of that, the
patient became irritable and mildly in
distress. Hence, Dr. Qadeer ordered to
shift the patient to PICU for more close
and intensive care and monitoring.

During his stay in PICU, the patient

received 200 ml PRBC transfusion twice.
The patient also received Tranexamic Acid
140 mg I.V every 8 hourly, Factor VII 700
mcg I.V every 2 hours, Factor VIII 700 mg
I.V TID and Zantac 13 mg I.V TID as
ordered. Routine blood investigations also
done from there. Blood levels were closely
monitored specially CBC.

The patient was maintained on NPO until

there's episode of bleeding noted. The
patient stayed in PICU for 3 days, and
when the bleeding was controlled and the
patient was stable, he was started on liquid
to soft diet and was transferred back again
to M4. From then, no more bleeding was
noted until the patient was discharged.

Family History
Positive family history of hemophilia. His
elder brother also has the disease and the
their mother is a carrier of the disease.

Developmental
Tasks

Ericksons Psychosocial
Theory

Erik Eriksons
developmental theory:
Erikson is a germen psychologist
He told development consist of the impact of
external factors
According to Eriksons theory, each person must
pass through a serious of interrelated stage over
the entire life cycle

Stage 3: Initiative Vs Guilt

During this period children show desire to
copy the adults around them and take
initiative in creating play situation.
They also starts to use the word WHY in
this stage.
If we may frustrated there natural desires
and goals then they experience guilt.

Success in this stage will lead to the virtue

of PURPOSE.
Interpretation:
The patient passed this stage and
achieved INITIATIVE. During his stay in
the ward, he was active and was regularly
interacting with other patient and health
care team specially to nurses.

Physical
Assessment

General Condition Conscious and Oriented, Weak

looking
ENT
With oozing of blood from throat
(palate)
CVS
S1, S2 are normal, no murmurs
noted, tachycardic, palpitations
Chest
Clear; bilateral air entry are equal,
tachypneic
Abdomen
Soft, no abdominal pain noted, no
organomegaly, not distended
Skin
Pallor noted, No rashes noted, cold
clammy skin
Joints
Normal, no swelling noted,

Vital Signs
Temperature:
Pulse Rate:
Respiratory Rate:
BP:
SPO2:
Head Circumference:
Height:
Weight:

37
156 bpm
46
87/48
95%
56 cm
118 cm
23 kg

Anatomy
and
Physiology

Normally, 7-8% of human body weight

is from blood. This essential fluid carries
out the critical functions of transporting
oxygen and nutrients to our cells and
getting rid of carbon dioxide, ammonia,
and other waste products. In addition, it
plays a vital role in our immune system
and in maintaining a relatively constant
body temperature.

Red Cells/Erythrocytes
are relatively large microscopic cells without
nuclei
make up 40-50% of the total blood volume
transport oxygen from the lungs to all of the
living tissues of the body and carry away carbon
dioxide
Hemoglobin is the gas transporting protein
molecule that makes up 95% of a red cell. Each
red cell has about 270,000,000 iron-rich
hemoglobin molecules.

White cells/ leukocytes

exist in variable numbers and types but
make up a very small part of blood's
volume--normally only about 1% in healthy
people.
the first responders for our immune
system. They seek out, identify, and bind
to alien protein on bacteria, viruses, and
fungi so that they can be removed.

 They also have the function of getting rid of

dead or dying blood cells as well as
foreign matter such as dust and asbestos.
Platelets/ thrombocytes
work with blood clotting chemicals at the
site of wounds. They do this by adhering
to the walls of blood vessels, thereby
plugging the rupture in the vascular wall.

 They also can release coagulating

chemicals which cause clots to form in the
blood that can plug up narrowed blood
vessels. Thirteen different blood clotting
factors, in addition to platelets, need to
interact for clotting to occur

Plasma
clear, yellow tinted water (92+%), sugar,
fat, protein and salt solution which carries
the red cells, white cells, and platelets.
normally, 55% of our blood's volume is
made up of plasma
it contains blood clotting factors, sugars,
lipids, vitamins, minerals, hormones,
enzymes, antibodies, and other proteins.

Clotting factors
Substances in the blood that act in
sequence to stop bleeding by forming a
clot.
factor I (fibrinogen)
factor II (prothrombin)
factor III (tissue thromboplastin)
factor IV (calcium)
factor V (proaccelerin)
factor VI (no longer considered active in
hemostasis)

 factor VII (proconvertin)

factor VIII (antihemophilic factor)
factor IX (plasma thromboplastin
component; Christmas factor)
factor X (stuart factor)
factor XI (plasma thromboplastin
antecedent)
factor XII (hageman factor)
factor XIII (fibrin stabilizing factor)

Coagulation factor VIII (antihemophilic

factor)
is made chiefly by cells in the liver. This
protein circulates in the bloodstream in an
inactive form, bound to another molecule
called von Willebrand factor, until an injury
that damages blood vessels occurs. In
response to injury, coagulation factor VIII
is activated and separates from von
Willebrand factor.

The active protein (sometimes written as

coagulation factor VIIIa) interacts with
another coagulation factor called factor IX.
This interaction sets off a chain of
additional chemical reactions that form a
blood clot.

VIDEO ON HEMOPHILIA

PATHOPHYSIOLOGY
OF
HEMOPHILIA

Risk Factors
Predisposing Factors:
Male
Mother is carrier of Hemophilia

Ethiological factor: heredity

FLOW CHART
PATHOPHYSIOLOGY
Due to etiological factors: Heredity, spontaneous gene
mutation
Abnormal X- chromosomes
Mutation of genes in X- chromosomes
Deficiency of factor VIII & IX

Cont....

limited formation of prothrombin activators

Limited thrombin production
Fibrinogen is not converted into fibrin
Limited clots formed
Uncontrollable bleeding
Signs of hemophilia

Signs of hemophilia
General - Weakness and lethargic
Musculoskeletal (joints) Hemarthrosis,
warmth, pain, stiffness.
CNS - Headache, stiff neck, vomiting,
lethargy, irritability.
GI - Hematemesis, melena, frank red
blood per rectum, and abdominal pain.
Genitourinary - Hematuria, renal colic,
and postcircumcision bleeding

Cont...
GI - Hematemesis, melena, frank red
blood per rectum, and abdominal
pain.
Genitourinary - Hematuria, renal
colic, and postcircumcision bleeding
Other - Epistaxis, oral mucosal
hemorrhage, hemoptysis, dyspnea,
compartment syndrome and
contusions

Laboratory
Works

A complete blood count (CBC) is a

blood test used to evaluate your overall
health and detect a wide range of
disorders, including anemia, infection and
leukemia.
A complete blood count test measures
several components and features of your
blood, including:
Red blood cells, which carry oxygen
White blood cells, which fight infection
Hemoglobin, the oxygen-carrying protein
in red blood cells

 Hematocrit, the proportion of red blood

cells to the fluid component, or plasma, in
your blood
Platelets, which help with blood
clottingAbnormal increases or decreases
in cell counts as revealed in a complete
blood count may indicate that you have an
underlying medical condition that calls for
further evaluation.

NORMAL
RANGE

02-062014
@1500

03-062014
@20:39

08-062014
@05:48

W.B.C

3.5 - 10

8.68

9.32

8.14

R.B.C

4.5 - 6.5

2.26
LO AB

1.88
LO AB

3.02
LO AB

HEMOGLOBIN

13 - 18

6.5
LO AB

5.28
LO AB

11.3
LO AB

HEMATOCRIT

42 - 52

18.5
LO AB

15.3
LO AB

28.7
LO AB

PLT. COUNT

130- 400

221

183

281

Blood (Serum) Chemistry

Sometimes a specific diagnosis may be made
on the basis of a blood chemistry profile
alone. More often than not, however, the
profile provides information on a variety of
body organs and systems, giving the doctor
an indication of where a problem might be
located. The profile can be extremely helpful
in determining which of the many other
diagnostic tests might be beneficial.

NORMAL
RANGE

02-062014
@1500

03-062014
@20:39

08-062014
@05:48

GLUCOSE
FASTINGFBS
CREATININ
E SERUM

4.2 6.1

5.1

53 115

27

48

39.99

URIC ACID
SERUM
SODIUM
SERUM

155 428

LO AB

LO AB
305

LO AB
314.2

132 145

134

136

136

POTASSIUM
SERUM
CHLORIDE
SERUM
UREA

3.2 5.1

3.7

3.2

98 107

104

105

106

2.5 6.4

8.5

3.92

6.61

HI AB

HI AB

ESR
ESR stands for erythrocyte
sedimentation rate. It is a test that
indirectly measures how much inflammation
is in the body.
03-06-2014@O2:00

ESR

RESULT

UNIT

17

MM/HR

HI AB

NORMAL
RANGE
4- 10

CRP (C-reactive protein)

is a non-specific test. It is used by a doctor to
detect inflammation if there is a high suspicion of
tissue injury or infection somewhere in the body,
but the test cannot tell where the inflammation is
or what condition is causing it. CRP is not
diagnostic of any condition, but it can be used
together with signs and symptoms and other
tests to evaluate an individual for
an acute or chronic inflammatory condition.

CRP
03-06-2014@02:00

CRP

NEGATIVE

HIV
The human immunodeficiency
virus (HIV) is a lentivirus (a subgroup of
retrovirus) that causes the acquired
immunodeficiency syndrome (AIDS).
03-06-2014@02:00

HIV 1, II, &P24

NEGATIVE

OCCULT BLOOD SCREEN

Fecal occult blood testing, as its name
implies, aims to detect subtle blood loss in
the gastrointestinal tract, anywhere from
the mouth to the colon.

06-06-

08-06-

2014@04:37

2014@13:25

POSITIVE

NEGATIVE

PTT PROFILE
Prothrombin time (PT) is a blood test that
measures how long it takes blood to clot. A
prothrombin time test can be used to check for
bleeding problems.
INR (international normalized ratio) stands for a
way of standardizing the results of prothrombin
time tests,
Partial thromboplastin time (PTT) is a
blood test that looks at how long it takes for blood
to clot. It can help tell if you have bleeding or
clotting problems.

02-06-2014@20:05
RESULT

UNIT

NORMAL
RANGE

PT-

12.2

SEC

11.6- 12.2

PROTHROMBIN
TIME
INR

1.1

PTT TEST

73 HI AB

.8- 1.2
SECS

30.6- 38.2

Factor VIII Level

19-05-2014 @ 13:41
Normal Range
Factor VIII Level
70-140
Factor IX Level

70-120

Result
5.1
Low Abnormal
96.7

Drug Study

Generic Name

Tranexamic Acid

Brand Name

Cyklokapron

Classification

Anti-fibrinolytic, antihemorrhagic

Indication

Used to stop or reduce bleeding and it works by stopping the clots

from breaking down too quickly so it will reduces the excessive
bleeding.

Prescribed Dose

140 mg

Route

Intravenously

Frequency

TID

Adverse
Reactions:

Nursing
Responsibilities:

Assess for hypersensitivity reaction and monitor vital signs.

Administer the medication after feeding, or administer H2
antagonists as ordered by the physician.
Provide safety for the patient, always raise the side rails of the
crib at all times.
Observe for unusual occurrence of bleeding and report
immediately to the doctor.
It should be used with extreme caution for the children.

Hypersensitivity
stomach pain and vomiting
Headache
Risk for thrombosis/ having blood clots

Generic Name

Ranitidine

Brand Name

Rantag/ Zantac

Classification

Histamine H2 antagonists

Indication

Treatment and prevention of heartburn, acid indigestion, and sour

stomach.

Prescribed Dose

13 mg

Route

Intravenously

Frequency

TID

Adverse
Reactions:

Nursing
Responsibilities:

Assess for hypersensitivity reaction and monitor vital signs.

Advised to increase oral fluid intake and fiber intake which may
minimize constipation.
Administer the medications slowly with the burette.
Provide safety for the patient, always raise the side rails of the
crib at all times.
Inform the mother that medication may temporarily cause tongue
to appear gray black.

Hypersensitivity
Constipation, dark stools
Nausea
Dizziness and drowsiness,
Altered taste, gray black tongue

Generic Name

Factor VII

Brand Name

NovoSeven

Classification

Recombinant Human Coagulation Factor VIIa (rFVIIa)

Indication

To promote hemostasis by activating the extrinsic pathway of the

coagulation cascade.

Prescribed Dose

700 mcg

Route

Intravenously

Frequency

Every 2 hours

Adverse
Reactions:

Nursing
Responsibilities:

Assess for hypersensitivity reaction and monitor vital signs.

Provide safety for the patient, always raise the side rails of the
crib at all times. The nurse should also assess pain level with the
help of pain scale.
Observe for rashes and notify to the physician.
Observe for signs of complications and should educate the
mother regarding the complications.
Administer the medication after feeding, or administer with H2
antagonists as ordered by the physician

Hypersensitivity
Dizziness and headache
Rashes ( including allergic dermatitis and rash erythematous
Arterial and Venous Thromboembolitic events
Upset stomach, nausea & vomiting

Generic Name

Factor VIII

Brand Name

Octonative

Classification

Recombinant Human Coagulation Factor VIIa (rFVIIa)

Indication

It activates substances in your blood to form clots and decrease

bleeding episodes.

Prescribed Dose

700 mcg

Route

Intravenously

Frequency

TID

Adverse
Reactions:

Nursing
Responsibilities:

Assess for hypersensitivity reaction and monitor vital signs.

Provide safety for the patient, always raise the side rails of the
crib at all times. The nurse should also assess pain level with the
help of pain scale.
Observe for rashes and notify to the physician.
Observe for signs of complications and should educate the
mother regarding the complications.
Administer the medication after feeding, or administer with H2
antagonists as ordered by the physician

Hypersensitivity
Dizziness and headache
Rashes ( including allergic dermatitis and rash erythematous
Arterial and Venous Thromboembolitic events
Upset stomach, nausea & vomiting

Nursing Care
Plan

Assessment

Nursing
Diagnosis

Goal

Subjective Data:
The mother
verbalized that
her son had
bleeding from
the mouth (soft
palate) since 2
days.
Objective Data:
Active bleeding
noted
Patient is
irritable
Pale and weak
looking
Hemoglobin
level is low
abnormal (6.5)
Fatigue
Body Malaise

Ineffective
Tissue
Perfusion
related to
active
bleeding
secondary
to
Hemophilia

Short Term:
After 15-30
minutes of
nursing
interventions,
the patient will
not manifest
signs and
symptoms of
respiratory
distress and
no impairment
in
consciousness
.

Nursing
Interventions
Independent:
1. Monitored &
recorded vital
signs
accordingly.
2. Assessed the
cause of
bleeding.

3. Assessed
skin color and
capillary refill.

Rationale

Evaluation

Short Term
>To have a
After 30
baseline data
minutes of
interventions,
the patient
did not
>By knowing the manifest any
cause of
signs of
bleeding it will
respiratory
assist in
distress and
determining
theres no
appropriate
alteration in
interventions for consciousness
patients
.
GOAL MET
>To provide
information
about the
degree /
adequacy of
tissue perfusion
and assist in
determining
appropriate
intervention.

Assessment

Capillary
refill is
decreased
Cold
clammy
skin
Pulse
Rate: 156
bpm
Respirator
y Rate: 46
BP- 87/48
SPO2:
95%

Goal

Long Term:
After 1-3
days of
nursing
interventions,
the patient
will not
manifest
bleeding
episodes.

Nursing Interventions

4. Provided adequate
rest and comfort
measures.

Rationale

>To decrease oxygen

consumption in the
system.

Evaluation

Long Term

After 3 days of
interventions,
Dependent:
>To maintain fluid and bleeding was
1. Started IVF and
electrolyte.
controlled as
regulated well as
evidenced by
ordered by the
the patient was
physician.
stable, active,
>To treat hemorrhage not in distress.
2. Packed RBC 200 ml
and to improve
Pale to pinkish
over 4 hours given twice oxygen delivery to the in color, latest
as ordered by the
tissues.
HB was 11.3.
physician.
>Medications which
GOAL MET
helps to control and
3. Due medications
manage bleeding.
given as ordered by the
physician.

Assessment

Patient is
irritable
Pale and
weak
looking
Fatigue
Body
Malaise
Patient is 4
y/o only, so
he is
unaware of
his
limitations
Humpy
Dumpty
Score is 14
(High Risk)

Nursing
Diagnosis

Goal

Nursing
Interventions

Rationale

Risk for injury

related to
imbalance
between
oxygen
supply and
demand as
evidence by
excessive
blood loss
secondary to
Hemophilia

After 30
minutes to 1
hour of
nursing
interventions,
the mother will
able to
understand
the health
educations
taught to her
as manifested
by the patient
will exhibit no
further signs of
bleeding; and
injury and
complications
can be
avoided.

1. Maintained
security of
client's crib by
maintaining its
side rails up at
all time. Placed
a high risk for
fall sign in the
crib.

Fragile tissue
and impaired
clotting
mechanisms
boost the risk
of bleeding
despite the
injury / mild
trauma

Evaluation

After 1 hour
of health
education,
the mother
verbalized
understandin
g regarding
prevention of
injury and
complications
2. Advised the
Early
, as
mother to never identification
manifested by
leave the patient and treatment the patient
unattended.
can limit the
did not
Instructed to
severity of
developed
always to keep
complications any bleeding
an eye on every
from other
move that
sites.
allows the
occurrence of
GOAL MET
injury.

Assessment

Nursing
Diagnos
is

Goal

Nursing Interventions

Rationale

3. Emphasized to the mother

the importance of using
elbow pads, helmets and
safety belts and to keep their
home free of furniture with
sharp corners.

It may help prevent

injuries from falls
and other accidents.

4. Advised the mother to give

soft foods to the child and
avoid such foods like potato
chips, lollipop, hard candies.

To prevent minor
injury from the
mouth which can
result to bleeding
afterwards.

5. Educated the mother

about 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.

Early management
can be given at
home and to prevent
further
complications.

Evalua
tion

Nursing Discharge
Plan

M- edications
No take home medication prescribed to the
patient upon discharge.

E- xercise
Exercise regularly. Activities such as swimming
,bicycle riding, walking can build up muscles, while
protect joints. Contact sports such as, foot ball,
hockey or wrestling are not safe for people with
hemophilia.

T- reatment
Factor VII and Factor VIII Replacement Therapy

H- ealth Teachings
AVOID CERTAIN PAIN MEDICATIONS- Drugs that
can aggravate bleeding include ASPIRIN and
IBUPROFEN. Instead of that use Acetaminophen or
Paracetamol which is a safe alternative for mild
pain relief.
PRACTICE GOOD DENTAL HYGIENE- To prevent
tooth extraction

 PROTECT CHILD FROM INJURIES THAT COULD

CAUSE BLEEDING- Use elbow pads, helmets,
safety shoes, all may prevent injuries from
falls and other accident. Keep the room free of
furniture with sharp corners.
Keep out of reach or locked away small and sharp
objects and other items that could cause bleeding or
harm.
Check play equipment and outdoor play areas for
possible hazards.
Tell all of your childs health care providerssuch
as doctor, dentist, and pharmacistthat your child
has hemophilia.

O- PD Follow Up
Have regular checkups and vaccinations as
recommended.
Follow your treatment plan exactly as your doctor
prescribes.
Follow up on Pedia Clinic after 2 weeks.

D- iet
Advised soft foods and to give nutritious foods to boost
the childs immune system and to increase intake of
Iron rich foods like liver, egg, red meat, green leafy
vegetables.