1
 it is transmitted as a sex-linked recessive trait.
Hemophilia
 are inherited disorder of blood coagulation.
 it is a rare disorder in which your blood doesn't clot
normally because it lacks sufficient blood-clotting
proteins.
TYPES OF HEMOPHILIA
 HEMOPHILIA A
 also known as the classic form of hemophilia
which caused by the deficiency of the
coagulation component factor VIII, which is the
antihemophilic factor or AHF
 factor VIII, is an intrinsic factor of coagulation;
its absence cause the intrinsic system for
manufacturing thromboplastin to be
incomplete.
 in which if you don’t have enough AHF, and
you become injured, your blood will not form a
clot properly.
 in child, coagulation ability is not totally absent
if their intrinsic system is affected by the AHF,
because the extrinsic or tissue system remains
intact.
 the baseline level of factor determines the
occurrence of bleeding episodes and can
range from mild to severe.
Mode of transmission
Mode of transmission
-transmitted as a autosomal recessive trait. It not present
on sex chromosome and the condition, therefore, occurs
in both sexes.
Diagnostic Evaluation
 Usually made from a history of bleeding episodes
 Evidence of X-linked inheritance (only one third of
cases are new mutations)
 Laboratory findings
JESURA, JOVER, LACUESTA, LAPORNO, LASAT
 incidence in US shows that 1 in 5,000 White males
have this kind of deficiency.
 a female carrier may have slightly lowered but
sufficient levels of the factor VIII component so that
she does not manifest a bleeding disorder.
 HEMOPHILIA B
 also called factor IX deficiency or Christmas disease,
is a genetic disorder caused by missing or defected
factor IX, a clotting protein in blood. .
 first noted in 1952 in a patient named Stephen
Christmas.
 hemophilia B is four times less common the
hemophilia A.
 only approximately 15% people with hemophilia
have this form.
 it is transmitted also through sex-linked recessive
trait.
 HEMOPHILIA C
 or the plasma thromboplastin antecedent deficiency,
is caused by factor XI deficiency, it is a rare genetic
disorder.
 symptoms are mild compared with those children
with factor VIII or factor IX deficiencies.
 it can be distinguished from hemophilia A and B by
the fact that it does not lead to bleeding into the
joints.
To understand the significance of various tests of
homeostasis, it is helpful to recall the usual mechanisms
to control bleeding (e.g., the function of platelets and
clotting factors). The results of tests that measure
platelet function may be abnormal.
CLINICAL SEVERITY OF HEMOPHILIA
Clinical Severity Factor VIII Bleeding Tendency
Activity
Severe <1% Spontaneous
bleeding without
trauma
Moderate 1%-5% Bleeding with
 2

trauma  Other tests are those that depend on specific

factors for a reason to occur, especially the partial
Mild >5%-40% Bleeding with thromboplastin time.
severe trauma or
surgery  Carrier detection is possible in classic hemophilia
using DNA testing and is an important
consideration in families in which female offspring
may have inherited the trait.
 The tests specific for hemophilia include factor VIII
and IX assays, procedures normally done by
specialized laboratories.

Therapeutic Management
Objectives for Quality Patient Outcome only) if the child shows an appropriate
response;
• Early recognition of signs and symptoms of hemophilia

• Bleeding episodes prevented • Cortecoids- given for hematuria, acute

hemarthrosis, and chronic synovitis;
• Bleeding episodes treated early with factor replacement
- recommended to patients with
• Adherence to prophylactic factor replacement program when
indicated hemophilia avoid aspirin and
nonsteroidal antiinflammatory drugs
• Hemarthrosis prevented when possible with limited joint
damage
(NSAIDs): because they inhibit
platelet function
• Exercise program and physical therapy ongoing
• Ibuprofen (NSAIDs)- effective in relieving pain
caused by synovitis and are occasionally used
with caution
The primary therapy for hemophilia is replacement
of the missing clotting factor. The products currently • Ꜫ -aminocaproic acid (EACA, Amicar)- oral use
available are factor VIII concentrates, either produced prevents clot destruction
through genetic engineering (recombinant form) or
- limited to mouth trauma or surgery,
derived from pooled plasma, which are reconstituted
with a dose of factor concentrate
with sterile water immediately before use.
given first.
 Aggressive factor concentrate replacement
- The child may rinse the mouth with
therapy is initiated to prevent chronic crippling
this medication and then swallow.
effects from joint bleeding.
 Regular program of exercise and physical therapy
 If replacement therapy begins immediately, local
(an important aspect of management;
measures such as ice applications and splinting are
Individualized physical activity strengthens muscles
seldom needed. Other drugs may be included in
around joints and may decrease the number of
therapy plan:
spontaneous bleeding episodes;more rapid
• 1-deamino-8-D-arginine vasopressin (DDAVP) - recovery and a decreased likelihood of
synthetic form of vasopressin treatment of complications)
choice in mild hemophilia and von
 Most children are treated at home(The family is
Willebrand disease (vWD) (types I and IIA
taught how to perform venipuncture and how to
administer factor VIII to children over 2 to 3 years
JESURA, JOVER, LACUESTA, LAPORNO, LASAT

of age. The child learns the procedure for self
administration at 8 to 12 years of age.
 Home treatment is highly successful, and the
rewards, in addition to the immediacy, are less
disruption of family life, fewer school or work days
missed, and enhancement of the child’s
self-esteem and independence.
 Prophylactic therapy is periodic factor
replacement for children with severe hemophilia
to prevent bleeding complications (including
arthropathy and spontaneous and life-threatening
bleeding events proved to be effective in
preventing naturopathy and spontaneous and
life-threatening bleeding events).
SITE OF BLEED
Joint
NURSING ALERT! Passive
range-of-motion exercises should
never be part of an exercise regimen Soft tissue
after an acute episode because the
joint capsule could easily be stretched
and bleeding could recur. Active
range-of-motion exercises are best so
that the patient can gauge his or her Muscle
own pain tolerance.
Various Therapies employed when
bleeding occurs or is anticipated:
Mucous membrane (e.g.,
nose, mouth)
JESURA, JOVER, LACUESTA, LAPORNO, LASAT
3
 Primary prophylaxis (in patients with severe
hemophilia; factor VIII concentrate is infused on a
regular basis before the onset of joint damage.
 Secondary prophylaxis involves the infusion of
factor VIII concentrate on a regular basis after the
child experiences his or her first joint bleed.
(Infusions are given every other day or three times
a week for several weeks to promote healing.
Prompt appropriate treatment of hemorrhage and
prophylactic therapy are key to excellent care and
prevention of long-term morbidity in patients with
hemophilia).
Adjunct Therapies for Hemophilia A
TREATMENT
Rest, ice, elevation
Splint, elastic wrap, crutches
Physical therapy
Ice, elevation
Splint or elastic wrap
Rest, ice, elevation
Splint, elastic wrap, crutches
Physical therapy
Complete bed rest for iliopsoas muscle bleed
Pressure to nares (for nosebleed)
Topical antifibrinolytic agent (ε-aminocaproic
acid)
Nasal pack (sometimes necessary)
 4

Laboratory Tests to Assess Hemophilia

Test Description Comments

Platelet Function

 Platelet function − Measures platelet Results are altered with

analyzer (PFA 100) function and interaction intake of NSAIDs,
with Von Willebrand anticoagulants, ASA, or
factor ASA-containing products

− Has replaced bleeding

time in some centers

− Measures time it takes

for bleeding from small Function depends on
superficial wound to platelet aggregation and
 Bleeding time vasoconstriction; two
cease
common methods used are
Ivy (incision made on the
forearm) and Duka (incision
made on the earlobe)

Normal response is
absence of petechiae or
− Measures platelet <10 petechiae
function and capillary
fragility; pressure Abnormal in platelet and
applied to forearm with connective tissue disorders
to forearm with
 Tourniquet test tourniquet for 5-10
minutes

− Measures degree to
which clot shrinks and Depends on platelet
expresses serum function

JESURA, JOVER, LACUESTA, LAPORNO, LASAT

 5

 Clot retraction test

Blood-clotting
Mechanisms

 Whole blood clotting − Measures time it takes Prolonged clotting time

time for clot to form within indicates problem in
blood thrombin-to-fibrin phase or
in any factor in intrinsic
clotting mechanism, difficult
test to standardized;
therefore often unreliable
results

Actually measures not

prothombin levels but
activity; because it
bypasses intrinsic-extrinsic
− Measures activity of mechanism, detects
 Prothombin time (PT) prothombin and factors deficiencies of factors V,
necessary for its VII, X and fibrogen as well
conversion to thrombin as prothombin
and fibrogen

Specific for factor

deficiencies, except factor
VII, which results in a
normal PTT but prolonged
PT

− Similar to PT but
measures activity of Allows for determination of
thromboplastin, which specific factor deficiencies,
depends on intrinsic
 Partial thromboplastin clotting factors especially distinguishing
time (PPT) between factors VII and IX

 Thromboplastin
generation test
− Measures blood’s
ability to generate Normally, as blood clots,
thromboplastin prothrombin is converted to
thrombin so that serum is
depleted of prothrombin; if
thromboplastin is
decreased (as a result of
extrinsic factor
− Indirectly measures deficiencies), not all
thromboplastin prothrombin will be
generation and converted and removed
prothrombin response from serum

 Prothombin Not dependent on phase I

JESURA, JOVER, LACUESTA, LAPORNO, LASAT

consumption test
− Directly
fibrinogen
blood
 Fibrinogen level
Prognosis
− Early recognition of joint and muscle bleeds is
emphasized, since immediate adequate
treatment with clotting factor is possible using
home infusion therapy. Early treatment has
significantly reduced the morbidity formerly
associated with hemophilia.
− The availability of comprehensive hemophilia
treatment centers offers the child with hemophilia
and the family a coordinated multidisciplinary
approach to meeting their needs and improving
the child’s health and well-being.
− Although there is no cure for hemophilia, its
symptoms can be controlled and its potentially
crippling deformities markedly reduced or even
avoided.
− Today many children with hemophilia function
with minimal or no joint damage. They have an
average life expectancy and are normal in every
aspect but one: they have a tendency to bleed,
which is a significant inconvenience but not
necessarily a life-threatening event.
− Unfortunately, those individuals with hemophilia
who were treated before the development of
current purification techniques for factor VIII
concentrate (between 1979 and 1985) may have
been exposed to HIV.
− It is estimated that more than 50% of these
patients seroconverted to HIV-positive status,
and 30% developed acquired immunodeficiency
syndrome (AIDS)
JESURA, JOVER, LACUESTA, LAPORNO, LASAT
6
or II defeciencies
measures
levels in
− Individuals with hemophilia diagnosed since the
1990s and treated with recombinant factor
products are at virtually no risk for developing HIV
infection from treatment.
Nursing Care Management
− The earlier a bleeding episode is recognized, the
more effectively it can be treated.
− Signs that indicate internal bleeding are
especially important to recognize. Children are
aware of internal bleeding and are reliable in
telling the examiner the location of an internal
bleed.
− In addition, the nurse maintains a high level of
suspicion when a child with hemophilia shows
signs such as headache; slurred speech; loss of
consciousness (from cerebral bleeding); and
black, tarry stools (from gastrointestinal
bleeding).
Prevent bleeding
 The goal of prevention of bleeding episodes is
directed toward decreasing the risk of injury.
 Prevention of bleeding episodes is geared mostly
toward appropriate exercises to strengthen
muscles and joints and to allow age-appropriate
activity.

 the environment should be made as safe as
possible, with close supervision maintained
during playtime to minimize incidental injuries.
 For older children the family usually needs
assistance in preparing the child for school. A
nurse who knows the family can be instrumental
in discussing the situation with the school nurse
and in joint planning of an appropriate activity
schedule.
 Because almost all individuals with hemophilia
are boys, the physical limitations in regard to
active sports may be a difficult adjustment, and
activity restrictions must be tempered with
sensitivity to the child’s emotional and physical
needs.
 Use of protective equipment, such as helmets,
face masks, shin/wrist/forearm guards, kneepads,
and other equipment appropriate for the type of
athletic activity, are encouraged to prevent injury.
 Children and adolescents with severe hemophilia
can participate in noncontact sports such as
swimming, golf, walking, jogging, fishing, and
bowling.
 Contact sports such as football, boxing, hockey,
soccer, and rugby are strongly discouraged
because the risk of injury outweighs the physical
and psycho social benefits of participating in
these sport
 studies have reported that the absolute increase
in risk of bleeds associated with regular
participation in high-impact athletic activities (e.g.,
basketball, football, hockey, gymnastics, karate,
soccer, skateboarding) that is supported by adult
coaching and supervision of school-age
hemophiliac children who received routine
prophylactic factor replacement is small
 To prevent oral bleeding, some readjustment in
terms of dental hygiene may be needed to
minimize trauma to the gums, such as using a
water irrigating device, softening the toothbrush in
warm water before brushing, or using a
sponge-tipped disposable toothbrush. A regular
toothbrush should be soft bristled and small.
 Adolescents also need to be advised of the
dangers of using safety razors with blades and
should use an electric shaver.
 Because any trauma can lead to a bleeding
episode, all persons caring for these children
must be aware of their disorder.
 The subcutaneous route is substituted for
intramuscular injection whenever possible.
JESURA, JOVER, LACUESTA, LAPORNO, LASAT
7
Venipunctures for blood samples are usually
preferred by children. There is usually less
bleeding after venipuncture than after finger or
heel puncture.
 Neither aspirin nor any aspirin containing
compound should be used. Acetaminophen is a
suitable aspirin substitute, especially for use
during control of pain at home.
Recognize and Control Bleeding
 Factor replacement therapy should be instituted
according to established medical protocol, and
supportive measures may be implemented, such
as RICE, which is the mnemonic for rest, ice,
compression, and elevation
 When parents and older children are taught such
measures beforehand, they can be prepared to
initiate immediate treatment.
 Plastic bags of ice or cold packs should be kept in
the freezer for such emergencies. However, such
measures do not take the place of factor
replacement.
Prevent Crippling Effects of Bleeding
 During bleeding episodes the joint is elevated and
immobilized. Active range-of-motion exercises
are usually instituted after the acute phase. This
allows the child to control the degree of exercise
according to the level of discomfort.
 Physical therapy is beneficial to promote
maximum function of the joint and unaffected
body parts. Success of a physical therapy plan
involves control of pain by administering
analgesics before therapy and adjusting the dose
to provide maximum benefit.
 If an exercise program is initiated in the home, a
physical therapist or public health nurse may
need to supervise compliance with the regimen.
 Rarely, orthopedic intervention, such as casting,
application of traction, or aspiration of blood, may
be necessary to preserve joint function.
 Diet is also an important consideration because
excessive body weight can increase the strain on
affected joints, especially the knees, and
predispose the child to hemarthrosis.
Consequently, children need calories that meet
their energy requirements
Support the Family and Prepare for Home Care

Factor concentrates have greatly changed the
outlook for these children by minimizing the bleeding
and allowing the child to live a normal, unrestricted life.
(Children are taught to take responsibility for their
disease at an early age. They learn their limitations,
preventive measures, and self-administration of the
factor replacement).
The needs of families who have children with
hemophilia are best met through a comprehensive
team approach of physicians (e.g., pediatrician,
hematologist, orthopedist),nurse practitioner, nurse,
social worker, and physical therapist.
Parent-group discussions are beneficial in meeting
the needs of similarly affected families.
Children who have become infected with HIV
through transfusions and factor replacement products
Retrieved from:
Hockenberry, M. J., & Wilson, D. (2015). Wongs nursing care of infants and children. St. Louis, MO:
Elsevier/Mosby.
JESURA, JOVER, LACUESTA, LAPORNO, LASAT
8
face the consequences of this dreaded disease.
Consequently, they need the support of health
professionals, especially in areas of safe sexual practices
to avoid disease transmission and public education
regarding AIDS and ways to deal with public reactions
to those who have AIDS.
Identify Persons at Risk
 Genetic counseling is essential as soon as possible
after diagnosis.
 The feeling of responsibility for this condition
usually rests with the mother. (Unless she has an
opportunity to discuss her feelings, the couple’s
relationship may suffer).
 Prenatal DNA testing can identify affected fetuses
and identify carriers in most cases.

VON WILLEBRAND DISEASE
Is an inherited autosomal dominant disorder
that affects both sexes and is most common
among whites. It is a hereditary bleeding disorder
characterized by a deficiency of or protein called
von Willebrand factor, there is an inability of the
platelets to aggregate and the blood vessels to
constrict to aid in coagulation and bleeding time is
prolonged. Epistaxis is a major problem and
childbirth is obviously a risk for a women with a
certain disease. The vWF protein contributes to the
adherence of platelets to damaged endothelium
and serves as a carrier protein for factor VIII. This
results to prolonged bleeding time because
platelets fail to adhere to the walls of the ruptured
vessel to form a platelet plug.
ASSESSMENT
To find out if a person has von Willebrand
disease (VWD), the doctor will ask questions about
personal and family histories of bleeding. The
doctor also will check for unusual bruising or other
signs of recent bleeding and order some blood
tests that will measure how the blood clots. The
tests will provide information about the amount of
clotting proteins present in the blood and if the
clotting proteins are working properly. Because
certain medications can cause bleeding, even
among people without a bleeding disorder, the
doctor will ask about recent or routine medications
taken that could cause bleeding or make bleeding
symptoms worse.
SCREENING TESTS:
 Complete Blood Count (CBC)
 Activated Partial Thromboplastin Time
(APTT) Test
 Prothrombin Time (PT) Test
 Fibrinogen Test
DIAGNOSIS
Mild forms of von Willebrand disease can
be difficult to diagnose because bleeding is
JESURA, JOVER, LACUESTA, LAPORNO, LASAT
9
common, However, if your doctor suspects
you have a bleeding disorder, he or she
might refer you to a blood disorders
specialist (hematologist).
Your doctor will also likely recommend the
following blood tests:
 Von Willebrand factor antigen. This
determines the level of von Willebrand
factor in your blood by measuring a
particular protein.
 Von Willebrand factor activity. There are a
variety of tests to measure how well the von
Willebrand factor works in your clotting
process.
 Factor VIII clotting activity. This shows
whether you have abnormally low levels
and activity of factor VIII.
 Von Willebrand factor multimers. This
evaluates the structure of von Willebrand
factor in your blood, its protein complexes
and how its molecules break down. This
information helps identify the type of von
Willebrand disease you have.
TREATMENT
Von Willebrand disease has no cure, treatment
can help prevent or stop bleeding episodes.
Treatments depends on the type and severity of
the condition and how the body responded to the
previous therapy and the other medications and
conditions that the physician might suggest one or
more of the following treatments to increase your
von Willebrand factor, strengthen blood clots or, in
women, control heavy menstrual bleeding.
Desmopressin- This medication is available as an
injection (DDAVP) or nasal spray (Minirin). It is a
synthetic hormone that controls bleeding by
stimulating your body to release more of the von

Willebrand factor stored in the lining of your blood
vessels.
Replacement therapies- These include infusions of
concentrated blood-clotting factors containing von
Willebrand factor and factor VIII. Doctors might
recommend patients if DDAVP isn't an option for
the patient or was ineffective. Another
replacement therapy approved by the FDA for
treating adults 18 and older is a genetically
engineered (recombinant) von Willebrand factor
product. Because recombinant factor is made
without plasma, it can reduce the risk of a viral
infection or allergic reaction.
Oral contraceptives. For women, these can be
useful for controlling heavy bleeding during
menstrual periods. The estrogen hormones in birth
control pills can boost von Willebrand factor and
factor VIII activity. This effect is likely available with
birth control patches, though further study is
needed to confirm it.
Clot-stabilizing medications- These anti-fibrinolytic
medications such as aminocaproic acid (Amicar)
and tranexamic acid (Cyklokapron, Lysteda) can
help stop bleeding by slowing the breakdown of
blood clots. Doctors often prescribe these drugs
before or after a surgical procedure or tooth
extraction. Drugs applied to cuts. A fibrin sealant
(Tisseel VHSD) placed on a cut helps curtail
bleeding. This is applied like glue using a syringe.
There are also over-the-counter products to stop
nosebleeds.
Lifestyle and home remedies- These self-care tips
can help patient manage his/her condition.
Switch pain relievers. To help prevent bleeding
episodes, talk with your doctor before taking
blood-thinning medications such as aspirin,
ibuprofen (Advil, Motrin IB, others) or naproxen
sodium (Aleve). Your doctor might instead
recommend pain and fever relievers such as
acetaminophen (Tylenol, others). Tell your doctors
JESURA, JOVER, LACUESTA, LAPORNO, LASAT
10
and dentist. Let your doctors or dentist know that
you have von Willebrand disease before you have
surgery, start a new medication or give birth. Also
mention whether anyone in your family has a
history of excessive bleeding. Consider wearing a
medical ID bracelet. Make sure it notes that you
have von Willebrand disease, which will be helpful
to medical personnel if you're in an accident or
taken to an emergency room. Also carry a medical
alert card in your wallet. Be active and safe.
Exercise as part of attaining or maintaining a
healthy weight. Avoid activities that could cause
bruising, such as football, wrestling and hockey.
Emergency Treatment
 Have the child sit up and lean forward
(not lie down)
 Apply continuous pressure to the nose
with thumb and forefinger for at
least 10 minutes.
 Insert cotton or wadded tissue into
the nostril and apply ice or cold
cloth to bridge of nose if bleeding
persists.
 Keep the child calm and quiet.
MANAGEMENT
Nursing Care Management
The nursing goals are similar to those for
hemophilia, with special considerations related to
epistaxis. Nosebleeds are often a frightening
experience for the child and the parents. A calm
reassuring manner can alleviate anxiety and
promote the child‘s cooperation. Because the
nosebleeding originates in the anterior part of the
nasal septum, bleeding can be controlled by
applying pressure to the nose with the thumb and
forefinger. During this time the child breathes
through the mouth. If local measures are not
successful at stopping the bleeding, DDAVP is used
to treat mild and moderate vWD. DDAVP increases
vWF and factor VIII secretion from storage in the
endothelial cells.
 11

For menorrhagia, factor replacement these females frequently do not experience

therapy or the administration of DDAVP may be
beneficial on the first day of the menstrual cycle to
lessen the flow. Teaching the adolescent methods
to prevent embarrassing accidents during
menstruation, such as using double sanitary pads,
helps her adjust to the inconvenience. Interestingly,
excessive bleeding during childbirth. This is
thought to be due to the increased levels of factor
VIII during pregnancy. Decision regarding
childbearing are difficult because of the dominant
pattern of inheritance.

Retrieved from:

Pillitteri, Adele. (2019) Maternal & child health nursing :care of the childbearing & childrearing
family Philadelphia, PA : JoAnne Silbert-Flagg, Adele Pillitteri, Vol. 2

Hockenberry, M. J., & Wilson, D. (2015). Wong's nursing care of infants and children (10th edition.). St.
Louis, Missouri: Elsevier.

MayoClinic.org (2019). von Willebrand disease,

https://www.mayoclinic.org/diseases-conditions/von-willebrand-disease/symptoms-causes/syc-20354978

JESURA, JOVER, LACUESTA, LAPORNO, LASAT

 12

Hemophilia
1. The parents of a client with hemophilia are taking their child home. Which assessment indicates a need for further
education regarding hemophilia?

A. “We should ensure our child has regular dental appointments.”

B. “We need to wrap our child’s limbs daily to prevent bleeding.”

C. “We should help our child select activities that minimize the risk of injury.”

D. “We should not give our child aspirin.”

Wrapping up limbs does not prevent bleeding and does not stop bleeding during acute episodes. Regular dental
appointments are necessary to prevent dental problems in a child with hemophilia. Children with hemophilia should be
encouraged to participate in activities that are not contact sports and have minimal risks of injury. Aspirin, NSAIDs, and
other blood thinners should be avoided.

2. The physician has ordered several laboratory tests to help diagnose an infant’s bleeding disorder. Which of the
following tests, if abnormal, would the nurse interpret as most likely to indicate hemophilia?

A. Bleeding time

B. Tourniquet test

C. Clot retraction test

D. Partial thromboplastin time (PTT)

PTT measures the activity of thromboplastin, which is dependent on intrinsic clotting factors. In hemophilia, the intrinsic
clotting factor VIII (antihemophilic factor) is deficient, resulting in a prolonged PTT. Bleeding time reflects platelet
function; the tourniquet test measures vasoconstriction and platelet function; and the clot retraction test measures
capillary fragility. All of these are unaffected in people with hemophilia.

3. Which of the following assessments in a child with hemophilia would lead the nurse to suspect early hemarthrosis?

A. Child’s reluctancy to move a body part

B. Cool, pale, clammy extremity

C. Eccymosis formation around a joint

D. Instability of a long bone in passive movement

JESURA, JOVER, LACUESTA, LAPORNO, LASAT

 13

Bleeding into the joints in the child with hemophilia leads to pain and tenderness, resulting in restricted movement.
Therefore, an early sign of hemarthrosis would be the child’s reluctance to move a body part. If the bleeding into the
joint continues, the area becomes hot, swollen, and immobile—not cool, pale, and clammy. Ecchymosis formation
around a joint would be difficult to assess. Instability of a long bone on passive movement is not associated with joint
hemarthrosis.

4. A pediatric nurse health educator provides a teaching session to the nursing staff regarding hemophilia. Which of
the following information regarding this disorder would the nurse plan to include in the discussion?

A. Hemophilia is a Y linked hereditary disorder

b. Males inherit hemophilia from their fathers

C. Females inherit hemophilia from their mothers

D. Hemophilia A results from a deficiency of factor VIII

Males inherit hemophilia from their mothers, and females inherit the carrier status from their fathers. Hemophilia is
inherited in a recessive manner via a genetic defect on the X-chromosome. Hemophilia A results from a deficiency of
factor VIII. Hemophilia B (Christmas disease) is a deficiency of factor IX.

5. Which of the following tests is most effective in diagnosing hemophilia?

A. Bleeding time

B. Complete blood count (CBC)

C. Partial thromboplastic test (PTT)

D. Platelet count

Option C: PTT is abnormal in hemophilia. Therefore, this test will be the most helpful in diagnosing the disorder.
Options A and D: Bleeding time and platelet count are normal in hemophilia. Option B: The CBC is not affected in
hemophilia.

Questions: von Willebrand disease

1. An autosomal dominant disorder that affects both sexes and is common among white people?

A.Thrombocytopenia
B. Hypertension
C. von Willebrand disease
D. Galactosemia

2. It is a hereditary bleeding disorder characterized by a deficiency of protein called?

JESURA, JOVER, LACUESTA, LAPORNO, LASAT

 14

A. Sickle cell Anemia

B. von Willebrand factor
C. Phenylalanine
D. Propanolol

3. You are asked to consult on a newborn female with purpura fulminans upon taking the health history you learn that this child
had a male sibling who died in the neonatal period after presenting with purpura fulminas. She has 3 other sibling who are
healthy, this type of condition leads to which physiologic sequence?

A. Excess von Willebrand factor high molecular weight multimers

B. Decreased fibrinogen
C. Inability to inactivate factor VIII
D. Thrombocytopenia

The differential diagnosis of neonatal purpura fulminans include disseminated intravascular coagulation as well as deficiencies of
proteins. The child had an older sibling who presented in the same manner and while DIC can occur to any newborn, this
scenario presents the presence of an autosomal recessive disorder. The proteins c/s complex is responsible for inactivating
factors V and VIII thus the correct answer is C.

4. A 3 day old infant is bought to the ER due to a seizure. A CT scan demonstrates massive intracranial hemorrhage. On your
examination the child has numerous bruises on the abdomen and the trunk. Which of the most scenarios is most likely:

A. The baby is born to an infant of a diabetic mother

B. The baby was born at home
C. The baby is exclusively breastfed
D. The baby has craniosynostosis

This is a vitamin K deficiency. From the above choices only B suggests that this is the diagnosis, infants born at home are at
highest risk for not receiving prophylactic vitamin K at birth

5. An 8 year old female with recurrent tonsillitis is referred for tonsillectomy. Her surgeon order pre-operative laboratory tests
and the results demonstrates a PT of 10.2 seconds (normal 9.7-11.2) and a PTT of 58 seconds (normal 22-36). The most
appropriate next test to order is:

A. Factor VIII activity

B. Factor IX activity
C. Factor XI activity
D. Mixing study

This is a typical scenario for the presence of lupus anticoagulant in a child and therefore ordering a mixing study is the most
appropriate test to order.

JESURA, JOVER, LACUESTA, LAPORNO, LASAT