PAT I E N T S C E N A R I O ,

CHAPTER 44,
NURSING CARE OF A
F A M I LY
WHEN A CHILD HAS A
H E M AT O L O G I C
DISORDER

A B R A H A M , A S H A R Y, C A I L I N G ,
DELA CRUZ, LUMASAG,
M A N L A N G I T, M O N T E R O ,
PA N G A N D O N G A N , S I A H A A N ,
VA C A L A R E S
 C A S E 01
P R E S E N TAT I O N
GENERAL OBJECTIVES
After
O B J2-3
E hours
C T IofVcase
E Spresentation the learners will be able to foster the care, management and better understanding
of hemophilia and inherited bleeding disorders by making available support, treatment and basic education to people
with hemophilia and their families. The presentation also seeks to demonstrate the student’s knowledge regarding the
general health and disease condition of a patient with diagnosis, its disease process, possible complications, treatment
plan and nursing interventions.

SPECIFIC
O B J2-3Ehours
After C T of
I Vcase
ES presentation the learners will be able to:
1. Review the different causes of Hemophilia
2. Describe the diagnostic test used, its results and how it is performed.
3. Identify the different signs and symptoms that may be manifested by the patient with Hemophilia.
4. Recognize the contributing risk factors associated to children with Hemophilia, epistaxis and active bleeding
5. Summarize treatments of a hemophilia to include, the goals of treatment, discuss blood borne diseases and their
impact on an individual with hemophilia, describe how gene therapy may become a cure for hemophilia.
6. Define how an individual with hemophilia can stop the transmission of the disease
7. Determine proper treatment options to prevent and treat bleeding episodes
8. dentify potential complications associated with hemophilia
 INTRODUC
02 TION
• Hemophilia is an inherited bleeding disorder. Children
with hemophilia can’t stop bleeding because they don’t
have enough clotting factor in their blood. Clotting
factors are needed for blood to clot. Blood clots to
prevent excessive bleeding.
• There are many blood clotting factors involved in the
forming of clots to stop bleeding. Two common factors
that affect blood clotting are factor VIII and factor IX.
• How severe your child’s hemophilia is depends on the
level of blood clotting factors in his or her blood.
 The 3 main forms of hemophilia include:

A B C
HEMOPH HEMOPH HEMOPH
ILIA ILIA ILIA
This is caused by a This is caused by a Some doctors use this
lack of the blood
clotting factor VIII. deficiency of factor term to refer to a lack
About 9 out of 10 IX. This is also called of clotting factor XI. 
people with
hemophilia have type Christmas disease or
A disease. This is also factor IX deficiency. 
referred to as classic
hemophilia or factor
VIII deficiency. 
Impact of hemophilia on quality of life of affected children and their parents, a hospital based
cross sectional study
V. Manikandasamy, Shanmugam Arumugasamy, G. Mathevan https://dx.doi.org/10.18203/2349-3291.ijcp20173196
Abstract

• Background: Hemophilia can have a huge negative impact not only on the physical health but also
on the psychological, economic and social well-being of the affected children and their family.
Documentation of this negative impact on quality of life is vital in drawing the attention of
relevant stakeholders to design and implement appropriate interventions.
• Methods: This was a cross sectional study, Children with hemophilia aged 4 to 12 years, and their
parents who were attending the Pediatric Hematology Clinic of the Advanced Pediatric Centre,
PGIMER, Chandigarh, between July 2009 and June 2010 were included in the study. The Hemo-
QoL questionnaire was used to assess the quality of life after translation into local language and
validation.
• Results: The study included 51 children with 10 children in 4-7 years age group and 41 children in
8-12 years age group and their parents. Perceived impact on family (77.3±14.7), poor physical
health (62.9±29.8), sports and school (53.8±22.8) had the highest negative impact on QoL. Support
from friends, family, and other persons seemed to have contributed positively towards the quality
of life. Parents of older children had higher mean subscale scores in View of yourself subscale as
compared to parents of younger children (40.8±14.2 vs 23.7±33.0, p value 0.018). Under Sports
and school subscale also similar pattern was observed (48.2±20.7 Vs 34.1±13.8, P value 0.045).
• Conclusions: The Quality of life was poor both among children affected with hemophilia and their
parents. Perceived impact on family, poor physical health and inability to participate in
school/sports activities are the major contributors to poor QoL. Support from family, friends, and
03 DEFINITION
OF TERMS
• Accelerator globulin (AcG) is a globulin in serum that promotes the conversion of prothrombin to
thrombin in the presence of thromboplastin and ionized calcium.

• Human antihemophilic factor is used to treat or prevent bleeding episodes in people with hemophilia
A. It is also used to control bleeding related to surgery or dentistry in a person with hemophilia. Human
antihemophilic factor is not for use in people with von Willebrand disease.

• Arthropathy means any disease of the joints.

• Basophils are a type of white blood cell. Although they're produced in the bone marrow, they're found
in many tissues throughout your body.

• Bilirubin is a yellowish substance in your blood. It forms after red blood cells break down, and it
travels through liver, gallbladder, and digestive tract before being excreted

• Blood dyscrasias are medical conditions (hematologic disorders) that may affect the cellular or plasma
components of the blood, the bone marrow, or the lymph tissue.

• Clot retraction is the "shrinking" of a blood clot over a number of days.

• Coagulation is also known as clotting, is the process by which blood changes from a liquid to a gel, forming a
blood clot.

• Cryoprecipitate, BIOCHEMISTRY; a substance precipitated from a solution, especially from the blood, at low
temperatures. MEDICINE; an extract rich in a blood-clotting factor obtained as a residue when frozen blood
plasma is thawed.

• Epistaxis is defined as acute hemorrhage from the nostril, nasal cavity, or nasopharynx. 

• Erythrocytes, a red blood cell that (in humans) is typically a biconcave disc without a nucleus. Erythrocytes
contain the pigment hemoglobin, which imparts the red color to blood, and transport oxygen and carbon
dioxide to and from the tissues.

• Eosinophils are a type of disease-fighting white blood cell. This condition most often indicates a parasitic
infection, an allergic reaction or cancer.

• Esophageal varices are abnormal, enlarged veins in the tube that connects the throat and stomach (esophagus).
This condition occurs most often in people with serious liver diseases. It develop when normal blood flow to
the liver is blocked by a clot or scar tissue in the liver.

• Fibrinogen is a glycoprotein complex, made in the liver, that circulates in the blood of all vertebrates. During
tissue and vascular injury, it is converted enzymatically by thrombin to fibrin and then to a fibrin-based blood
clot.
• Hemarthrosis is a condition of articular bleeding, that is into the joint cavity. This can occur after an injury or,
more commonly, in bleeding disorders such as hemophilia.

• Hemocytoblast, a stem cell for blood-cellular elements especially : one considered competent to produce all
types of blood cell, also lymphoidocyte.

• Hemophilia is a rare disorder in which the blood doesn't clot normally because it lacks sufficient blood-
clotting proteins (clotting factors).

• Hematopoietic Stem Cells give rise to different types of blood cells, in lines called myeloid and lymphoid.
Myeloid and lymphoid lineages both are involved in dendritic cell formation.

• Intracranial Bleeding occurs when a blood vessel within the skull is ruptured or leaks. Leukocytes-a colorless
cell that circulates in the blood and body fluids and involved in counteracting foreign substances and disease; a
white (blood) cell.

• Lyophilized is a water removal process typically used to preserve perishable materials to extend shelf life or
make the material more convenient for transport.

• Monocytes are a type of leukocyte, or white blood cell. They are the largest type of leukocyte and can
differentiate into macrophages and myeloid lineage dendritic cells.

• Myeloid and Lymphoid Cells are two types of multipotent, hematopoietic progenitor cells, which give rise to
the cells in the blood. Furthermore myeloid cells refer to the cells derived from the bone marrow
while lymphoid cells refer to the cells 
• Neutrophils are a type of white blood cell (WBC or granulocyte) that protect us from infections, among other
functions. 

• Normocephalic is a medical term referring to a person whose head and all major organs of the head are in a normal
condition and without significant abnormalities.

• Partial thromboplastin time (PTT) is a blood test that measures the time it takes the blood to clot. A PTT test can be
used to check for bleeding problems. Blood clotting factors are needed for blood to clot (coagulation).

• Plasma thromboplastin component (PTC), a hitherto unrecognized blood coagulation factor; case report
of PTC deficiency Blood.

• Plasma thromboplastin antecedent (PTA), or Factor XI, is the zymogen form of factor XIa, one of the enzymes of
the coagulation cascade.

• Prosthesis in medicine (plural: prostheses; from Ancient Greek prosthesis, "addition, application, attachment")

or prosthetic implant is an artificial device that replaces a missing body part, which may be lost through trauma, disease,
or a condition present at birth (congenital disorder).

• Prothrombin is a protein made by the liver. It is one of several substances known as clotting factors.

• 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. PT is also used to check whether medicine to prevent blood clots is working. A PT test
• Recombinant Factor products offer a safer option than plasma-derived products because they avoid
potential blood-borne transmission of infectious diseases.

• Serum Prothrombin Conversion Accelerator (SPCA), an agent or apparatus that increases the rate
at which something occurs or progresses.

• Shirodkar Sutures is a suture or stitch which is placed around the cervix and tied in order to prevent
the cervix opening too early in pregnancy.

• Stuart Factor or a Clotting factor X, is a vitamin K–dependent serine protease that serves as the first
enzyme in the common pathway of thrombus formation.

• Thrombocytes, another term for Platelets.

• Thromboplastin (TPL) or thrombokinase is a mixture of both phospholipids and tissue factor found in
plasma aiding blood coagulation through catalyzing the conversion of prothrombin to thrombin.

• Tourniquet is a device that is used to apply pressure to a limb or extremity in order to limit – but not stop
– the flow of blood.

• Vasular Spasm develops in the coronary artery. It is a temporary tightening (constriction) of the muscles
in the wall of one of the arteries that sends blood to your heart
 04
ASSESSME
NT
 FAMILY PROFILE:
The family is intact. The father, age 24 years, works as
a parole officer. Mother, age 23 years, is homemaker
A CHILD WITH HEMOPHILIA while attending evening classes to become an
Brian Winston is an 18-month-old with hemophilia. accountant. The family lives in upstairs apartment at
He is seen in the emergency room following a fall the top of the house of father’s parents. Finances are
from a 2-ft high wall at a shopping mall. rated as “doing all right.”

PREGNANCY HISTORY:
The pregnancy is planned, following loss of first
CHIEF CONCERN: pregnancy because of incompetent cervix. Shirodkar
“He fell and his nose is bleeding and his knee is sutures placed at 14 weeks; it was removed at
swelling.” pregnancy’s term. The child is born by vertex vaginal
birth; there is no excessive bruising noticed at birth.
Apgar scores are “good.
HISTORY OF CHIEF CONCERN:
Brian was diagnosed as having hemophilia at birth
because of the history of disease in the family. His
HISTORY OF PAST ILLNESSES:
mother has factor VIII replacement at home and is The infant was not circumcised at birth because of
knowledgeable in how to administer it. Today, she history of disease in family. He was hospitalized at 6
only applied a cold compress to nose and knee and months for bleeding on hand that would not stop after
brought child directly to emergency room because mother clipped fingernail too short. The child fell at 9,
this was closer than going home. Child’s nose is still 12, and 14 months while learning to walk; factor
bleeding after 20 minutes; knee has discolored to a replacement administered each time at home with no
purplish blue and is swollen to twice the size of apparent long term effect. There are no hospitalizations;
opposite knee. No loss of consciousness after injury. no other major illnesses or childhood communicable
diseases.
HISTORY OF FAMILY ILLNESSES:
The mother’s brother, age 24 years, has hemophilia; he is a college student with little
difficulty with illness. An uncle of mother had died of disease of cerebral hemorrhage at
age 12 years. The paternal grandfather had left leg amputated for injury in World War
II; he is ambulatory with prosthesis. The maternal grandmother has alcoholism and liver
and esophageal varices.
DAY HISTORY:
Nutrition: The child eats at table with parents; no special foods prepared for him.
Sleep: He sleeps 10 hours at night; he takes 1 hour nap in afternoon.
Play/recreation: The child participates in preschool play group from church three afternoons a
week.
Growth and development: The child met infant developmental milestones: He sat at 8 months
and walked at 14 months. He speaks many words; his mother is not certain he actually forms
noun–verb combinations yet.
REVIEW OF SYSTEMS:
Overall health: Good
Eyes: Eyes crossed frequently at birth; were straight without therapy by 3 months; no eye
infections
Ears: Hearing never formally tested but mother feels it is adequate; no ear infections.
PHYSICAL EXAMINATION:
General appearance: Crying 18-month-old male with a bleeding nose and swollen; discolored left knee; weight:
23.5 lb (25th percentile); height: 32 in. (50th percentile); BP: 80/50 mmHg
Head: Normocephalic; posterior fontanelle closed; anterior fontanelle still palpable; head circumference: 47.5 cm
(50%)
Eyes: Red reflexes present bilaterally; child refused to follow light to test extraocular muscles; eyes in straight
alignment by Hirschberg test
Ears: TMs pink, landmarks prominent; child responds to whispered word
Nose: Midline septum; point in left nares actively bleeding; bleeding does not halt in response to normal pressure
Mouth and throat: 18 teeth; no caries; midline uvula; no erythema
Neck: Full range of motion; no palpable lymph nodes; midline trachea
Lungs: Clear to auscultation and percussion; no adventitious sounds; respiratory rate: 24 breaths/min (crying)
Heart: Rate: 110 beats/min; no murmurs
Abdomen: Bowel sounds heard in all four quadrants; no masses, not tender to touch
Genitalia: Normal male; testes descended bilaterally; uncircumcised
Extremities: Full range of motion in all but left knee; child has limited motion in knee from pain and swelling;
knee increased in size to twice that of opposite knee; has discolored to purple and is warm to touch
Neurologic: Child is aware of surroundings; answers to name; reflexes not tested to avoid pressure near joints

Brian is diagnosed as a child with hemophilia with epistaxis and active bleeding
into his right knee joint.
 ASSOCIATED
CHARACTER ONSET LOCATIO DURATI SEVERI PATTERN SYMPTOMS
N ON TY
“He fell and his nose is bleeding and his knee is His mother only applied a cold Hemophilia at
swelling.” compress to nose and knee and birth.
brought child directly to
emergency room because this was
closer than going home.
Child’s nose is still bleeding. Nose After 20 Continuous
minutes
Knee has discolored to a purplish blue and is swollen Left knee Twice the size of opposite knee.
to twice the size of opposite knee.
He was hospitalized for bleeding on hand that would At 6
not stop after mother clipped fingernail too short. months

Eyes crossed frequently; were straight without At birth Eyes Were straight without therapy by 3
therapy by 3 months. months.

Actively bleeding; bleeding does not halt in response Point in left Active
to normal pressure. nares
Infant was not circumcised. At birth Genitalia History of disease
in family
Full range of motion in all but left knee; child has Knee increased in size to twice
limited motion in knee from pain and swelling; knee that of opposite knee.
increased in size to twice that of opposite knee; has
discolored to purple and is warm to touch.
DIAGNO
05 STIC
TEST
 TEST
Measures the action of prothrombin; reveals deficiencies in prothrombin,
PROTHROMBIN factors V, VII, and X
TIME (PT) NORMAL VALUE:
11–13 s (PT) or 2.0–3.0 international normalized ratio (INR)

PARTIAL Measures activity of thromboplastin; reveals deficiencies in thromboplastin,

factors VIII to XII
THROMBOPLASTIN NORMAL VALUE:
TIME (PTT) 30–45 s

Measures the time required for bleeding at a stab wound on the earlobe to stop;
BLEEDING reveals deficiencies in platelet formation and vasoconstrictive ability
TIME NORMAL VALUE:
3–10 min

Measures platelet function; interval from placement of blood in a tube to the

CLOT point clot shrinks and expels serum
RETRACTION NORMAL VALUE:
Retraction at side of test tube should be present by 1 hr; complete in 24 hr
 TEST
Measures capillary fragility and platelet function; response of tissue to
application of tourniquet to forearm for 5–10 min
TOURNIQUET NORMAL VALUE:
0–2 petechiae per 2-cm area

Evaluates thromboplastin function; if clot formation used a great deal of

PROTHROMBIN prothrombin (as it should), serum prothrombin time will be brief; prolongation
CONSUMPTION denotes defects in thromboplastin function
TIME NORMAL VALUE:
Approximately 20s

THROMBOPLASTIN Tests basic ability to form thromboplastin; distinguishes factor VIII from factor
IX disorders
GENERATION NORMAL VALUE:
TIME 12 s or less

Measures stage 4 clotting process or level of fibrinogen in blood

PLASMA
NORMAL VALUE:
FIBRINOGEN 200–400 mg/100 ml plasma

VENOUS CLOTTING Measures factor deficits in stages 2 and 4

TIME (LEE-WHITE NORMAL VALUE:
CLOTTING TIME) 9–12 min
 NORMAL
A N AT O M Y
AND
PHYSIOLO
G 0Y6
 45%

95%

5%
● When a blood vessel is damaged, blood
can leak into other tissues and interfere
with normal tissue function, or blood
can be lost from the body. Although the
body can tolerate a small amount of
blood loss and can produce new blood to
replace it. Still, a large amount of blood
loss can lead to death.
 P L AT E L E
T PLUG

VA S C U L
AR
S PA S M
 BLOOD
COAGULA
TION

B L O O D C L O T = P L AT E L E T P L U G
+ FIBRIN
 INTRIN
SIC
FA C T O R
F AVCITI O
I R
● F AXCITI O
EXT R IX
RIN
F A CSTI O
C
F ARCITI O
I R
F AVCITI O
R X

FA C T O
R II

FA C T O
R I
FA C T O R
XIII
Blood clots prevent blood loss during wound
healing, but once the vessels are repaired,
they must be dissolved to restore blood flow.
This process, called fibrinolysis, a small
cascade that produces the enzyme plasmin.
Plasmin cleaves fibrin and dissolves the clot.
●At each step of the clotting process, each clotting factor
acti-vates many additional clotting factors, resulting in the
formation of a clot. Most clotting factors are manufactured
in the liver, and many of them require vitamin K for their
synthesis. In addition, many of the chemical reactions of clot
formation require Ca2+ and the chemicals released from
platelets. The clotting process can be severely impaired by
low levels of vitamin K, low levels of Ca2+, low numbers of
platelets, or reduced synthesis of clotting factors because of
liver dysfunction.

●Humans rely on two sources of vitamin K. About half

comes from the diet, and the other half comes from bacteria
within the large intestine. Antibiotics taken to fight bacterial
infections sometimes kill these intestinal bacteria, reducing
vitamin K levels and causing bleeding problems. Vitamin K
supplements may be necessary for patients on prolonged
antibiotic therapy. Newborns lack these intestinal bacteria
and thus routinely receive a vita-min K injection at birth.
Infants can also obtain vitamin K from food, such as milk.
Because cow’s milk contains more vitamin K than does
human milk, breast-fed infants are more susceptible to
bleeding than are bottle-fed infants. However, maternal
supple-mentation with vitamin K, such as with oral vitamins,
07
RISK FACTOR
AND
PATHOPHYSIO
LOGY
●The most common inherited bleeding disorders associated with epistaxis are
hemophilia A, hemophilia B, and von Willebrand disease. Hemophilia A and B are
caused by deficiencies in factor VIII and factor IX, respectively; both are integral
components in the coagulation cascade.

RISK FACTORS FOR A CHILD

WITH
HEMOPHILIA WITH EPISTAXIS
Non-Modifiable Risk Modifiable Risk Factors:
Factors: • Intensity of treatment
• Genotype • Type of clotting factor
• Ethnicity administered
• Family history (The mother’s • Lifestyle
brother, age 24 years, has • Contact sports
haemophilia) • Sharing personal items
• Gender (Male) • Body piercing, tattoos etc.
• New gene mutation
 PATHOPHYSIOLOGY OF A
CHILD WITH
HEMOPHILIA WITH EPISTAXIS
Trauma

Blood vessels constrict (vasoconstriction)

Formation of platelet plug

Primary hemostatic response Secondary hemostatic response

Platelet adhesion and adp release Active blood coagulation

Disruption of endothelial and part of Fibrin

blood vessels in endothelium
 Platelet plug and
Von willebrand factor stabilize hemostatis

Collagen and platelet and F8 and F9 deficiency

platelet aggregation

Attracts platelet and PTT elevated in

attached to vwf using intrinsic pathway
glycoprotein GP1b

Platelets activate and PT level remain normal

in extrinsic pathway
aggregate

Fibrinogen act as crosslink

bet Delayed fibrin clot

Platelets Prolonged bleeding

Platelet plug and stabilize

hemostatis
 General
appearance Cardiovascular Respiratory Neuro Musculoskeletal
Crying 18- Clear to Full range of
month-old male auscultation and Child is aware of motion in all but
110 beats/min; percussion; no surroundings; left knee; child
with a bleeding adventitious
nose and no murmurs answers to name; has limited
sounds; motion in knee
swollen; respiratory rate: reflexes not
discolored left 24 breaths/min tested to avoid from pain and
knee; weight: (crying) pressure near swelling; knee
23.5 lb (25th joints increased in size
percentile); to twice that of
height: 32 in. opposite knee;
(50th has discolored to
percentile); BP: purple and is
80/50 mmHg warm to touch
MANAGEM
ENT
0
8
 THERAPEUTIC
MANAGEMENT
• Hemophilias, with even minor abrasions, bleeding can be controlled by the
administration of factor VIII, supplied by fresh whole blood, fresh or frozen
plasma, or a concentrate of factor VIII. The concentrate is supplied as a powdered
form that can be stored at home and reconstituted as needed. For most bleeding
episodes, one bag of concentrate per 5kg of body weight is usually sufficient to
provide protection for approximately 12 hours; another transfusion may be
necessary after that time. In some children, the administration of (DDAVP) which
stimulates the release of factor VIII, may also help prevent bleeding.

• In a few children antibodies ( termed inhibitors) to factor VIII develop. If these

happen, e-aminocaproic acid , a fibrinolytic enzyme that helps to stabilize clot
formation and promote wound healing, can be administered every 6 hours if
needed. Children with inhibitors to factor VIII can also help concentrate ( proflex
or konyne). This concentrate enters the coagulation cascade after factor VIII and
halts bleeding at that point.
 ANTICOAGUL
A ANTI- CARDIOVASCUL
TION AND INFLAMMATOR AR/RESPIRATOR
PHARMACO ANTIPLATELE Y AGENTS Y DRUGS
LOGY T AGENTS (NONSTEROIDA • Angiotensin-
ANESTHETIC • Abciximab L) converting enzyme
AGENTS (ReoPro) • Aspirin inhibitors
• Halothane • Antithrombin • Colchicine • Angiotensin
(Fluothane) agents (Colcrys) receptor blockers
• Local anesthetic • Clopidogrel • Ibuprofen (Advil, • Beta-blockers
agents (Plavi Motrin) • Calcium channel
• Dipyridamole • Indomethacin blockers
ANTIBIOTICS (Persantine) (Indocin) • Cilostazol (Pleta)
• Aminoglycoside • Fibrinolytic • Naproxen (Aleve) • Clofibrate
s agents (Atromid-S)
• Beta-lactam • Heparin • Diuretics
• Unfractionated ANTINEOPLAS • Ethacrynic acid
antibiotics TIC AGENTS
• Penicillin heparin (Edecrin)
• Low-molecular • Carmustine • Furosemide (Lasbd)
• Cephalosporins (BCNU)
• Nitrofurantoin weight heparins • Hydralazine
• Ticlopidine • Daunorubicin (Apresoline)
• Sulfonamides (Cerubidine)
(Tielid) • Methylxanthines
• Tirolban • Mithramycin • Aminophylline
(Aggrastat) (Mithracin) • Theophylline (heo-
 PLASMA
EXPANDERS
NITRATES • Dextrans
• Inonorbilde • Hydroxyethyl
(sordid starch (HES)
• Phentolamine • Psychotropic
(legitine) Agents MISCELLANEOU
FOOD AND • Phenothiazines
• Prasugel FOOD S
(Effient) • Serotonin
ADDITIVES Reuptake • Antihistamines
• Prostacyclin • Contrast agents
• Caffeine inhibitors
• Quinidine • Glyceryl
• Chinese black • Fluoxetine
tree fungus (Prozac) guaiacolate (cough
• Clove • Fluvoxamine syrup base)
• Cumin (Luvox) • Hepatitis B vaccine
• Ethanol (.e., • Paroxetine • Heroin
alcohol) (Pladt) • Hydroxychloroquin
• Fish oils • Sertraline e (Plaquenil;
• Garlic (Zoloft) Quineprox)
• Ginger • Tricyclic • Phenytoin
• Onion extract antidepressants (Dilantin)
• Turmeric-1 • Doxepin • Vitamin E
(Sinequan) • Herbal Supplements
• Imipramine • Feverfew
(Tofranit) • Gingko
• Valproic acid • Ginseng
• Kava kava
 Most patients with hemophilia are diagnosed as children. They often require assistance in coping with the condition because
it is chronic, places restrictions on their lives, and is an inherited disorder that can be passed to future generations. From
childhood, patients are helped to cope with the disease and to identify the positive aspects of their lives.

 They are encouraged to be self-sufficient and to maintain independence by preventing unnecessary trauma that can cause
acute bleeding episodes and temporarily interfere with normal activities. As they work through their feelings about the
condition and progress to accepting it, they can assume more and more responsibility for maintaining optimal health.

 Patients with mild factor deficiency may not be diagnosed until adulthood if they do not experience significant trauma or
surgery as children. These patients need extensive education about activity restrictions and self-care measures to diminish
the chance of hemorrhage and complications of bleeding.

 The nurse should emphasize safety at home and in the workplace. Patients and family members are instructed how to
administer the factor concentrate at home at the first sign of bleeding so that bleeding is minimized and complications
avoided. The use of prophylactic factor replacement can be very effective in diminishing the morbidity associated with
repeated bleeding. However, this method requires administration of these factors as often as two to three times each week.
Adherence to prophylactic infusions can be problematic.

 Nurses can assist patients to consider that the potential benefits of prophylactic therapy outweigh the disadvantages (i.e.,
time-consuming), particularly early in prophylaxis therapy. Patients with hemophilia are instructed to avoid agents that
interfere with platelet aggregation, such as aspirin, NSAIDs, some herbal and nutritional supplements (e.g., chamomile,
nettle, alfalfa), and alcohol. This restriction applies to OTC medications such as cold remedies.
NURSING
MANAGEMENT
 Oral hygiene is very important as a preventive measure because dental extractions are hazardous. Applying pressure to a
minor wound may be sufficient to control bleeding if the factor deficiency is not severe. Nasal packing should be avoided,
because bleeding frequently resumes when the packing is removed. Splints and other orthopedic devices may be useful in
patients with joint or muscle hemorrhages.

 All injections should be avoided; invasive procedures (e.g., endoscopy, lumbar puncture) should be minimized or performed
after administration of appropriate factor replacement. Patients with hemophilia should carry or wear medical identification
(e.g., Medic-Alert bracelets).

 In addition, patients or families should have a written emergency plan that includes what to do in specific situations as well
as names and phone numbers of emergency contacts.

 During hemorrhagic episodes, the extent of bleeding must be assessed carefully. Patients who are at risk for significant
compromise (e.g., bleeding into the respiratory tract or brain) warrant close observation and systematic assessment for
emergent complications (e.g., respiratory distress, altered level of consciousness).

 If the patient has had recent surgery, the nurse frequently and carefully assesses the surgical site for bleeding.

 Frequent monitoring of vital signs is needed until the nurse is certain that there is no excessive postoperative bleeding.
Analgesic agents are commonly required to alleviate the pain associated with hematomas and hemorrhage into joints. Many
patients report that warm baths promote relaxation, improve mobility, and lessen pain. However, during bleeding episodes,
heat is avoided because it can accentuate bleeding applications of cold are used instead. Although
the formulation of heat-solvent or detergent treated factor concentrates has rendered factor VIII and IX
preparations free of viruses such as HIV and hepatitis C, many patients treated prior to 1985 have already
NURSI
NG 0
9
CARE
PLANS
 NURSING PLANNING NURSING
ASSESSMENT DIAGNOSI / INTERVENTION RATIONALE EVALUATION
S OBJECTIV
E
Subjective Data: Risk for After 8 hours of Independent: After 8 hours of
“He fell and his nose is bleeding Bleeding nursing Monitor vital signs. Hypovolemic shock can nursing
and his knee is swelling.” related to intervention, happen due to decreased intervention, the
inadequate or the patient will circulatory volume with blood patient was able to
Objective Data: total lack of display loss. Signs include display
• 18 month old, Male-Diagnosed the clotting homeostasis as Elevate and immobilize hypotension and tachycardia. homeostasis as
as having hemophilia at birth factor VIII evidenced by affected limb. Apply ice Repeated hemarthrosis can evidenced by
because of the history of absence of packs to control bleeding. result in severe and crippling absence of
disease in the family. bleeding deformity. ice can help by bleeding
• Mother has factor VIII Apply manual or causing the blood vessels to
replacement at home mechanical pressure to the constrict.
• Child’s nose is still bleeding nose or apply topical Controlling bleeding is a
after 20 minutes. bleeding does coagulant if need. nursing priority. Nasal packing
not halt in response to normal should be avoided, because the
pressure subsequent removal of the
• Knee has discolored to a Collaborative: packing may precipitate further
purplish blue and is swollen to Monitor the coagulation bleeding.
twice the size of opposite knee. assays for factor VIII AND
• The child fell at 9, 12, and 14 IX. Decreased value indicate that
months while learning to walk; factor replacement therapy is
factor replacement Monitor hemoglobin and subtherapeutic.
administered each time at home hematocrit levels. Hgb and Hct are monitored as
with no apparent long term Provide replacement indicators of blood loss.
effect. therapy of deficient clotting Replacement of factors is the
BP: 80/50 factors. primary treatment for bleeding.
mmHgHR: 110 beats/min Treatment includes factor VIII,
RR: 24 breaths/min which is an essential clotting
factor needed to convert
 NURSING PLANNING NURSING
ASSESSMENT DIAGNOSI / INTERVENTION RATIONALE EVALUATION
S OBJECTIV
E
Subjective Data: Acute pain After a series of Independent: Client
“He fell and his nose is bleeding related to joint nursing Assess the location, Hemarthrosis (bleeding into demonstrated
and his knee is swelling.” infiltration by intervention, characteristics, and rate of joint) is the main manifestation evidence of pain
blood Child’s pain pain. of the disease. Common sites reduction to
Objective Data: will be at include the elbows, shoulders, tolerable levels by
BP: 80/50 tolerable levels hips, knees, and ankle joints. reduction in
mmHgHR: 110 beats/min evidenced by crying.
RR: 24 breaths/min reduction in Immobilization promotes
Infant is crying crying. Immobilize joints and apply comfort and decreases joint
elastic bandages to the damage; elastic bandage most
affected joint; elevate and often avoids muscle bleeding;
apply a cold compress to elevation of affected extremity/
active bleeding sites, but joint will minimize swelling;
must be used cautiously in cold application will promote
young children to prevent vasoconstriction.
skin breakdown. `

Collaborative: Acetaminophen (Tylenol),

Administer medications as propoxyphene, corticosteroids,
indicated. and codeine are given for pain
management; NSAIDs such
aspirin are contraindicated
since they impair clotting and
can cause gastric bleeding.
 NURSING PLANNING NURSING
ASSESSMENT DIAGNOSI / INTERVENTION RATIONALE EVALUATION
S OBJECTIV
E
Subjective Data: Impaired After nursing Independent: After the nursing
physical intervention Assess for limited ROM, Recurrent bleeding of the intervention the
Objective Data: mobility child will contractures, and bony joints can lead to bone child has
weight: 23.5 lb (25th percentile); related to maintain changes in the joints when destruction, permanent demonstrated
height: 32 in. (50th percentile); swollen left optimal bleeding has stopped. deformities, and crippling. optimal physical
• Full range of motion in all knee physical This data gives the baseline for mobility as
extremities but left knee; child mobility as evaluating further constraints evidenced by
has limited motion in knee evidenced by Provide gentle, passive from bleeding. normal range of
from pain and swelling. normal range of ROM exercise when the motion
• Swollen knee is warm to touch. motion child’s condition is stable. Clients who are experiencing
active bleeding have restricted
mobility or splinting. However,
later ROM exercise can help
Collaborative: maintain optimal muscle and
Refer for physical therapy, joint movement.
occupational therapy, and
orthopedic consultations, as
required. Electrical stimulation of the
muscles around the joints
prevents muscle atrophy.
Active physiotherapy helps in
regaining joint movement and
preventing fibrous build up.
 NURSING PLANNING NURSING
ASSESSMENT DIAGNOSI / INTERVENTION RATIONALE EVALUATION
S OBJECTIV
E
Subjective Data: Risk for injury After 15 Independent: After 15 minutes of
related to minutes of Teach care givers to Utilize This will minimize and/or discussion the
Objective Data: abnormal discussion, the appropriate toys (soft, not prevent the risk of bleeding caregivers has
• The child fell at 9, 12, and 14 blood profile caregivers will pointedor small sharp due to trauma. understood the
months while learning to walk; understand the objects); the infant, may importance of
factor replacement importance of need to use padded rail preventing injury
administered each time at home preventing sides on his crib and the by means of doing
with no apparent long term injury by means edges of the table where the activities that are
effect. of doing family eats. less risky and by
• Child eats at table with parents. activities that modifying
• The child participates in are less risky As the child grows Contact sports will predispose environment for
preschool play group from and by recommend non-contact the child to injury and bleeding the child’s safety
church three afternoons a modifying sports activities such as episodes. Daily use of padding by correctly
week. environment for swimming, hiking,Also and helmets may cause the verbalizing
• The child met infant the child’s recommend the use of child to feel ostracized and understanding
developmental mile¬stones: He safety as helmets and padding if may create emotional
sat at 8 months and walked at evidenced by needed but do not overuse. discomfort.
14 months. correct
verbalization of Maintain close supervision
understanding during play time to This will prevent bleeding
minimize injuries. related to trauma in the child’s
environment. Also allow the
parents to apply appropriate
interventions in case bleeding
occurs.
Collaborative:
Instruct parents to have a
home meal plan that include To maintain healthy iron levels
 NURSING PLANNING NURSING
ASSESSMENT DIAGNOSI / INTERVENTION RATIONALE EVALUATION
S OBJECTIV
E
Subjective Data: Risk for After 30 Independent: After 30 minutes of
Finances are rated as “doing all interrupted minutes of Assess family’s coping Identifying these areas will discussion the
right.” family discussion the methods and their help the nurse to help the care caregivers has
processes caregiver will effectiveness; family givers in establishing workable demonstrate
Objective Data: regarding to cope effectively interactions and coping methods.child with effective coping
• Family is intact. fears and child with child’s expectations related to long- special long-term needs either with child’s illness,
• The father, age 24 years, works prognosis that illness, by term care knowledge and strengthen or distort family by verbalizing and
as a parole officer. exhausts the verbalizing and use of support systems and relationships and an undue demonstrating
• Mother, age 23 years, is physical and demonstrating resources; presence of guilt degree of overprotection may readiness as
homemaker while attending emotional readiness as and anxiety, overprotection be detrimental to child’s evidenced by calm
evening classes to become an supportive evidenced by and/or overindulgent growth and development such appearance and
accountant. capacity of calm behaviors. as disallowing school sense of control.
• The family lives in upstairs caretakers appearance and attendance or peer activities,
apartment at the top of the sense of avoiding discipline of child,
house of father’s parents. control. and disallowing child to
• The paternal grandfather had assume responsibility.
left leg amputated for injury in
World War II. Encourage family members This decreases anxiety and
• The maternal grandmother has to verbalize problem areas enhances understanding;
alcoholism and liver and and develop solutions on provides the family an
esophageal varices. their own. opportunity to recognize
• An uncle of mother had died of problems and create problem-
disease of cerebral hemorrhage solving strategies.
at age 12 years.
Collaborative:
Provide assistance of social Gives support to the family
worker, counselor, or other faced with long-term care of a
as needed. child with a serious illness.
 1
0
MEDICAL
MANAGEMENT
(DRUG STUDY
AND
TREATMENTS)
 DRUG DRUG DOSAGE & SIDE NURSING
NAME ACTION INDICATION ROUTE EFFECTS INTERVENTION
S
Classification: This product contains Factor VIII is used to Administration of Allergic reactions • Relieve pain.
Coagulation Factors a man-made form of prevent or control Factor VIII can be like skin rash, itching Immobilize joints and
factor VIII, also called bleeding in patients supplied by fresh or hives, swelling of apply elastic
Generic: antihe- mophilic with hemophilia A. whole blood, fresh the face, lips, or bandages to the
Antihemophillic Factor factor. This product is This medicine is also or frozen plasma, tonguechest pain, affected joint if
used to temporarily used in these patients or a concentrate of irregular heartbeat, indicated; elevate
replace the missing during surgery. Some factor VIII. The feeling faint or affected and apply a
Brand: Monoclate-P, factor VIII, a protein products are used to concentrate is lightheaded, cold compress to
Hemofil M, Anti (clotting factor) that is treat von Willebrand's supplied as a fallsfever or chills, active bleeding sites.
Hemophellic Factor normally in the blood, disease. powedered form. 1 numbness or tingling • Prevent bleeding.
(Human) so that the blood can bag of concentrate in hands or feet, pain, Monitor hemoglobin
clot and the bleeding per 5kh of body redness or swelling at and hematocrit levels
can stop. weight. This the injection • Once the solution has
medicine is for sitestomach been prepared, use it
injection into a painunusually weak within 1 to 3 hours.
vein. yellowing of the eyes Throw away any
or skin. unused medicine.
 DRUG DRUG DOSAGE & SIDE NURSING
NAME ACTION INDICATION ROUTE EFFECTS INTERVENTION
S
Classification: Desmopressin is a Desmopressin is used Desmopressin ADVERSE SIDE • Monitor I&O ratio and
Antidiuretic Hormones man-made analogue of to control the comes as a tablet to EFFECTS: pattern (intervals).
vasopressin which is symptoms of a certain take by mouth. It is • Diarrhea • Weigh patient daily
Generic: Desmopressin the body's natural anti- type of diabetes usually taken two • Abnormal thinking and observe for edema.
diuretic hormone insipidus ('water to three times a • Seizure Severe water retention
Brand: DDAVP released by the diabetes'; condition in day.Dosage Forms: • Nasal congestion may require reduction
pituitary. It works by which the body oral tablet (0.1 mg; in dosage and use of a
increasing water produces an 0.2 mg); sublingual COMMON SIDE diuretic.
permeability in renal abnormally large tablet (27.7 mcg; EFFECTS: • Monitor BP during
tubular cells, which amount of urine). 55.3 mcg) • Weight gain dosage-regulating
decreases urine • Loss of appetite period and whenever
volume and increases • Headache drug is administered
urine concentration. • Irritability parenterally.
• Nausea • Monitor urine and
plasma osmolality.
 DRUG DRUG DOSAGE & SIDE NURSING
NAME ACTION INDICATION ROUTE EFFECTS INTERVENTION
S
Classification: Aminocaproic acid For use in the IV Compatibilities • Confusion • Check IV site at
Antifibrinolytics binds reversibly to the treatment of Solution: D5W, NS • Vision decrease frequent intervals for
kringle domain of excessive Ringer's Additive: • Vomiting extravasation.
Generic: plasminogen and postoperative netilmicin • Headache • Observe for signs of
E-Aminocaproic acid blocks the binding of bleeding. Y-site: fenoldopam • Convulsions thrombophlebitis.
plasminogen to fibrin IV Administration • Malaise • Change site
Brand: Amicar and its activation to Initial 5 g in 250 mL • Muscle weakness immediately if
plasmin. With NO over 1 hr, each • Dizziness extravasation or
activation of plasmin, subsequent gram in • Tinnitus thrombophlebitis
there is a reduction in 50-100 mL at 1 • Nausea occurs
fibrinolysis. This g/hrRapid injection • Bradycardia • Monitor & report S&S
consequently will undiluted into a vein • Thrombosis of myopathy: muscle
reduce the amount of is not recommended • Edema weakness, myalgia,
bleeding post surgery. Continue for about 8 • Hypotension diaphoresis, fever,
hr or until bleeding • Stroke reddish-brown urine,
has been controlled. • Syncope oliguria, as well as
• Intracranial thrombotic
• Hypertension complications.
• Monitor vital signs and
urine output.
 DRUG DRUG DOSAGE & SIDE NURSING
NAME ACTION INDICATION ROUTE EFFECTS INTERVENTION
S
Classification: Coagulation Factor IX • Factor IX 1.0 unit/kg x body • Fever • Take the dry medicine
Coagulation Factors is an important protein deficiency weight (in kg) x • Nausea and the liquid (diluent)
in the process of (Christmas desired increase (% • Pain at injection out of the refrigerator
Generic: Factor IX hemostasis and normal disease). of normal)If a 70 kg site and bring them to
Human blood clotting as it • Bleeding (154 lb) patient with • Chills room temperature, as
plays a key role within episodes in a Factor IX level of • Vomiting directed by your
Brands: Proplex, the coagulation patients with 0% needs to be • Dizziness doctor.
Konyne cascade. inhibitors to. elevated to 25%, give • Headache • When injecting the
• Factor VII 1.0 unit/kg x 70 kg x liquid (diluent) into the
deficiency. 25 = 1750 unitsIt dry medicine, aim the
should be infused stream of liquid
slowly, at a rate of (diluent) against the
approximately two to wall of the container of
three mL per minute. dry medicine to
prevent foaming.
• Swirl the container
gently to dissolve the
medicine. Do not
shake the container
 DRUG DRUG DOSAGE & SIDE NURSING
NAME ACTION INDICATION ROUTE EFFECTS INTERVENTION
S
Classification: It may reduce the Like other OTC Children under 12 ADVERSE • Avoid using multiple
Analgesic production of pain relievers, years: 10-15 EFFECTS: preparations
prostaglandins in the acetaminophen mg/kg/dose taken • Liver failure containing
Generic: brain. Prostaglandins temporarily orally once every 4- • Stevens-Johnson acetaminophen.
Acetaminophen are chemicals that relieves minor 6 hours; not to syndrome Carefully check all
cause inflammation aches and pains due exceed 5 doses in 24 • Gastrointestina OTC products.
Brand: Tylenol and swelling. to headache, hours. • Hemorrhage • Give drug with food if
backache, the • Laryngeal edema GI upset occurs.
common cold, • Kidney toxicity • Discontinue drug if
minor pain of hypersensitivity
arthritis, toothache, SIDE EFFECTS: reactions occur.
premenstrual and • Skin swelling
menstrual cramps, • Disorientation
and muscular • Dizziness 
aches, and • Hives
temporarily reduces • Low levels of red
fever. blood cells,
white blood cells,
and/or platelets
• Shortness of breath
 11
PROGNOS
The life expectancy of people who had severe hemophilia in the 1950s and 1960s before the development of factor

IS
concentrates was only 11 years. Most people who had severe hemophilia died in early childhood or adolescence from
intracranial bleeds or bleeding inside the vital organs. In 1964, Judith Pool found the fraction cryoprecipitate from the
plasma, which had large quantities of factor VIII concentrate, and that improved treatment for hemophilia significantly.
Before that, patients with hemophilia could only have treatment with whole blood or fresh plasma, which lacked
sufficient quantities of factor VIII or IX proteins. In the 1970s, lyophilized plasma concentrates of coagulation factors
became available, and this improved treatment significantly.

Primary prophylaxis began in Sweden before being adopted by other countries, which ended up preventing major
bleeding episodes and complications of arthropathies. In 1977, researchers discovered desmopressin. With that, patients
were able to get a better, safer, and relatively inexpensive option for treatment and risks of blood-borne infections from
repeated use of plasma-derived products were minimized. After patients with severe hemophilia got infected with HIV
and hepatitis C from contaminated coagulation factors in the 1980s, methods to screen and inactivate viruses in blood
were developed, and this improved the safety of plasma-derived products significantly. Eventually, the advancement in
DNA technology allowed industrial production of recombinant factor VIII and IX.

The widespread availability of replacement therapy to prevent and treat active bleeding, advancement in viral
inactivation techniques, management of blood-borne infections through surveillance, and availability of newer treatment
options for hepatitis C and HIV treatment have significantly improved the life-style of patients with hemophilia. Today,
life expectancy for patients is almost the same as the general population in developed countries, provided those patients
respond well to the treatment and do not have other health conditions. But in developing countries, where healthcare
access and treatment resources are scarce, the mortality rate remains almost twice that of the general population
 Silbert-Flagg, J., & Pillitteri, A; Maternal & Child Health Nursing Care of the Childbearing &
Childrearing Family Eighth Edition Volume I, 2018

Silbert-Flagg, J., & Pillitteri, A; Maternal & Child Health Nursing Care of the Childbearing &
Childrearing Family Eighth Edition Volume II, 2018

VanPutte, C., Regan, J., & Russo, A; Seeley’s Essentials of Anatomy & Physiology Tenth Edition,
2018

Mehta P..,Kumar A..,Reddivari R.., (2021) Hemophilia retrieved from https://www.ncbi.nlm.nih.gov.

1 Preventing Blood Loss (2021) from https://www.brainkart.com/article/Preventing-Blood-Loss_21869/

LeMone, Burke and Bauldoff (2011), Medical-Surgical Nursing Critical Thinking in Patient Care
2
REFERE Antihemophilic Factor, AHF, Factor VIII injection from
https://my.clevelandclinic.org/health/drugs/19031-antihemophilic-factor-ahf-factor-viii-
NCES injection#:~:text=ANTIHEMOPHILIC%20FACTOR%20(AHF%20or%20FACTOR,to%20treat
%20von%20Willebrand's%20disease.

Schwartz, R., & Nagalla, S., (2021); Factor IX Deficiency (Hemophilia B) Treatment & Management
from https://emedicine.medscape.com/article/199088-treatment

Aminocaproic Acid (Rx) from https://reference.medscape.com/drug/amicar-aminocaproic-acid-

342139#11

Acetaminophen (N-acetyl-p-aminophenol) Nursing Considerations & Management from

https://www.rnpedia.com/nursing-notes/pharmacology-drug-study-notes/acetaminophen-n-acetyl-p-
aminophenol/

Aminocaproic Acid from https://go.drugbank.com/drugs/DB00513

 THA
NK
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