HEMOPHILIA

A Case Report
Presented to the College of Nursing

In Partial Fulfillment
of the Requirement in
NCM 98: INTENSIVE PRACTICUM

DOROTHY PEARL L. PALABRICA, SN IV

JANUARY 2017
 Table of Contents
I. INTRODUCTION....................................................................................................2

II. ANATOMY AND PHYSIOLOGY.............................................................................4

III. PATHOPHYSIOLOGY........................................................................................6

Pathophysiology of Hemophilia: A narrative.................................................................8

IV. DIAGNOSTIC TESTS.........................................................................................9

V. INTERVENTIONS................................................................................................11

A. General Nursing Interventions................................................................................11

B. Medical Interventions..............................................................................................11

2. Surgical and Special Procedures...........................................................................15

VI. NURSING CARE PLANS.................................................................................16

REFERENCES...........................................................................................................25

2
 I. INTRODUCTION
Hemophilia is usually an inherited, congenital bleeding disorder characterized
by a lack of blood clotting factors, especially factors VIII and IX. It is an X-linked
disorder primarily affecting males; females act as carriers. It occurs in 1 in 5,000
males. There is no racial predilection, and hemophilia is found in all ethnic groups
(Riske, 2005). Identifying the specific coagulation deficiency is important so that
definitive treatment with the specific replacement agent can be implemented;
aggressive replacement therapy is initiated to prevent the chronic crippling effects
from joint bleeding (Behrman, Kliegman, & Jenson, 2004).
There are three common types including factor VIII deficiency (hemophilia A
or classic hemophilia) and factor IX deficiency (hemophilia B or Christmas disease)
and Hemophilia C which is deficient of factor XI.
The worldwide incidence of haemophilia is not well known, but estimated at
more than 400,000 people. Hemophilia A (factor VIII deficiency) affects 1 in 5000
male live births. Hemophilia B (factor IX deficiency) occurs in approximately 1 in
20,000 people, accounting for 15% of people with hemophilia (Valdez R., Zutter M.,
Florea A. D., et al., 2012). Locally, there are only about 1,200 cases registered by
Philippine Hemophilia Foundation.
Hemophilia is highly inherited, it is then expected that most factors are non-
modifiable. This includes: Age (not specified, but occurs mostly in children), family
history of haemophilia, race/ethnicity (African American, Hispanics), gender (Male),
and gene mutation (there are ongoing studies that indicate the type of genetic
mutation one has may indicate a higher risk factor in developing haemophilia). Some
known modifiable factors include: intensive factor therapy related to surgery and
trauma.
The disease, which can be severe, is manifested by hemorrhages into various
parts of the body. Hemorrhage can occur even after minimal trauma. The frequency
and severity of the bleeding depend on the degree of factor deficiency as well as the
intensity of the precipitating trauma. About 75% of all bleeding in patients with
hemophilia occurs into joints. The most commonly affected joints are the knees,
elbows, ankles, shoulders, wrists, and hips. Patients often note pain in a joint before
they are aware of swelling and limitation of motion. Recurrent joint hemorrhages can
result in damage so severe that chronic pain or ankylosis (fixation) of the joint
occurs. Many patients with severe factor deficiency are crippled by the joint damage
before they become adults. Hematomas can be superficial or deep hemorrhages into
muscle or subcutaneous tissue. With severe factor deficiency, they can occur without
known trauma and progressively extend in all directions. When the hematomas
occur within muscle, particularly in the extremities, peripheral nerves can be
compressed. Over time, this compression results in decreased sensation, weakness,
and atrophy of the area involved. Spontaneous hematuria and gastrointestinal
bleeding can occur. Bleeding is also common in other mucous membranes, such as
the nasal passages. The most dangerous site of hemorrhage is in the head
(intracranial or extracranial). Any head trauma requires prompt evaluation and
treatment. Surgical procedures typically result in excessive bleeding at the surgical

2
site. Because clot formation is poor, wound healing is also poor. Such bleeding is
most commonly associated with dental extraction (Boyer, 2010).
The prognosis of patients with hemophilia has been transformed by the
availability of factor VIII replacement. Gene therapy is currently in the developmental
phase, but could further transform the outlook for these patients. The major limiting
factor is disability from recurrent joint bleeding. Hepatitis B and C and HIV infection
from recurrent transfusion are diminishing in incidence.
At present, the National Hemophilia Federation and Hemophilia Association of
the Philippines for Love and Science exert efforts in providing comprehensive care to
promote physical and psychosocial health and quality of life while decreasing
morbidity and mortality for all hemophilic patients.

3
 II. ANATOMY AND PHYSIOLOGY

Platelets, or
thrombocytes, are large fragments from the cytoplasm of bone marrow cells called
megakaryocytes. The platelet has a half-life of approximately 8 to 12 days, and then
it is broken down and eliminated by macrophages. The normal serum concentration
is about 150,000 to 400,000 platelets per microliter (L) of blood. Platelet production
is controlled by a protein called thrombopoietin that causes proliferation and
maturation of megakaryocytes. The sources of thrombopoietin include the liver,
kidney, smooth muscle, and bone marrow. Platelets have a cell membrane but no
nucleus, and cannot reproduce. The cell membrane has phospholipids that assist
with the coagulation process. Although they lack a nucleus, they have many of the
characteristics of a whole cell. The outer cell membrane is covered with a coat of
glycoproteins, glycosaminoglycans, and coagulation proteins. One of the
important glycoproteins is GPIIb/IIIa, which binds fibrinogen and bridges platelets to
one another. The platelet shape is maintained by microtubules and actin and
myosin filaments that support the cell membrane. Platelets have mitochondria
and enzyme systems capable of producing adenosine triphosphate (ATP) and
adenosine diphosphate (ADP). They also have the enzymes needed for synthesis of
the prostaglandin, TXA, required for their function in hemostasis.
Platelets contain two specific types of granules (- and -granules) that
release mediators for hemostasis. The -granules express the P selectin, an
adhesive protein, on their surface and contain fibrinogen, von Willebrand factor
(vWF), fibronectin, factors V and VIII, platelet factor 4 (a heparin-binding chemokine),
platelet-derived growth factor (PDGF), transforming growth factor-alpha (TGF-),

4
and thrombospondin. The release of growth factors results in the proliferation and
growth of vascular endothelial cells, smooth muscle cells, and fibroblasts and is
important in vessel repair. The -granules, or dense granules, contain ADP and
ATP, ionized calcium, histamine, serotonin, and epinephrine, which contribute to
vasoconstriction.
During the process of hemostasis,
hair-like fibrin strands glue the
aggregated platelets together to form the
structural basis of the blood clot. In the
presence of fibrin, plasma becomes gel-
like and traps red blood cells and other
formed elements in the blood.
Hemostasis is divided into three stages:
Vascular constriction, Formation of the
platelet plug and Blood coagulation.
Platelet plug formation involves
adhesion and aggregation of platelets.
Platelets are attracted to a damaged
vessel wall, become activated, and
change from smooth disks to spiny
spheres, exposing glycoprotein
receptors on their surfaces. Platelet
adhesion requires a protein molecule
called von Willebrand factor, which leaks
into the injured tissue from the plasma.
This factor is produced by the
endothelial cells of blood vessels and
circulates in the blood as a carrier
protein for coagulation factor VIII.
Adhesion to the vessel subendothelial
layer occurs when the platelet receptor binds to vWF at the injury site, linking the
platelet to exposed collagen fibers. injury site, linking the platelet to exposed
collagen fibers.
Platelet aggregation occurs soon after adhesion. The secretion of the
contents of the platelet granules mediates it. The release of the dense body contents
is particularly important because calcium is required for the coagulation component
of hemostasis, and ADP is a mediator of platelet aggregation. ADP release also
facilitates the release of ADP from other platelets, leading to amplification of the
aggregation process. Besides ADP, platelets secrete the prostaglandin TXA2, which
is an important stimulus for platelet aggregation. The combined actions of ADP and
TXA2 lead to the expansion of the enlarging platelet aggregate, which becomes the
primary hemostatic plug. Stabilization of the platelet plug occurs as the coagulation
pathway is activated on the platelet surface and fibrinogen is converted to fibrin.
The platelet membrane plays an important role in platelet adhesion and the
coagulation process. The coat of glycoproteins on its surface controls interactions
with the vessel endothelium. Platelets normally avoid adherence to the endothelium.
but interact with injured areas of the vessel wall and the deeper exposed collagen.

5
Glycoprotein (GPIIb/IIIa) receptors on the platelet membrane bind fibrinogen and link
platelets together. Defective platelet plug formation causes bleeding in people who
are deficient in platelets or vWF.

6
 III. PATHOPHYSIOLOGY

Predisposing Factors: Precipitating Factors:

Genetics Trauma
Ethnicity/Race: African Injuury
American, Hispanics Surgery
Gender: Male
Age: mostly children

Genetic changes: abnormal X-chromosome

Legend:

Pathway Genetic mutation in X-chromosomes

Signs/Symptoms

Nursing Diagnosis Mutation causes factor VIII and IX deficiency

Treatment
Precipitation of bleeding due to
Diagnostics
inadequate activation of clotting factors

Limited formation of Prothrombin

activators

7
 Failure of fibrinogen
Vulnerable to convert
to development into fibrin
of bleeding

Unstable platelet formation

Cerebral hemorrhage Seepage of blood to other Excessive and spontaneous Headache
parts of the system bleeding
Hemarthosis Melena
CT scan, MRI Assays for clotting factors
Warm and stiffness on joints Large skin bruises

Partial thromboplastin time PTT Vomiting, lethargy

Hemoptysis
Prothrombin for consumption Epistaxis

Cryoprecipitate Analgesics Apply pressure using soft

Factor concentrates
administration bandages

Administration of antifibrinolytics
Cold compress Elbow pads, helmets

Ineffective peripheral tissue perfusion related to impaired blood flow Activity Intolerance related to bleeding episodes.
through a major vessel caused by bleeding

Risk for fluid volume deficit related to spontaneous

Ineffective protection related to abnormal blood profile secondary to
bleeding.
Hemophilia

Risk for injury related to weakness of the defense secondary to

hemophilia. 8
Pathophysiology of Hemophilia: A narrative
The coagulation cascade is part of the hemostatic process. It is a stepwise
process resulting in the conversion of the soluble plasma protein, fibrinogen, into
fibrin. The insoluble fibrin strands create a meshwork that cements platelets and
other blood components together to form the clot.
Many substances that promote clotting (pro-coagulation factors) or inhibit it
(anticoagulation factors) control the coagulation process. Each of the pro-coagulation
or coagulation factors, identified by Roman numerals, performs a specific step in the
coagulation process. The activation of one pro-coagulation factor or proenzyme is
designed to activate the next factor in the sequence (cascade effect). Because most
of the inactive pro-coagulation factors are present in the blood at all times, the
multistep process ensures that a massive episode of intravascular clotting does not
occur. It also means that abnormalities of the clotting process occur when one or
more of the factors are deficient or when conditions lead to inappropriate activation
of any of the steps. Most of the coagulation factors are proteins synthesized in the
liver. Vitamin K is necessary for the synthesis of factors II, VII, IX, and X,
prothrombin, and protein C.
From the above diagram, a defect in gene X marks the start of the disorder.
With the mutation in gene, the percentage of clotting factor becomes deficient
causing bleeding and low levels of Prothrombin factors to be activated. This then will
lead to the failure of fibrinogen to change into fibrin which is an essential component
for platelet formation. The vulnerability now of bruising and bleeding becomes more
prevalent allowing for the symptoms shown in the diagram to be apparent in
hemophilic children.

9
IV. DIAGNOSTIC TESTS

1. Partial Thromboplastin time

- The partial thromboplastin time (PTT) test is used to evaluate the intrinsic
and common pathways of the coagulation sequence. It represents the time
required for a firm fibrin clot to form after phospholipid reagents similar to
thromboplastin reagent are added to the specimen. In this disorder PTT is
usually prolonged.

Preparation:

-Explain to the child and parents:

The purpose of the test
The procedure, including the site from which the blood sample is
likely to be obtained
That momentary discomfort may be experienced when the skin Is
pierced
That food, fluids, and drugs are to be withheld before to the test
- For children, a doll may be used as the patient for demonstration
purposes. A laboratory technicians equipment basket may hold the
childs attention during the actual procedure.
- For all clients, encourage questions and verbalization of concerns
about the procedure, and provide calm, reassuring environment and
manner.

Nursing considerations:
- Because the client may have a coagulation deficiency, maintain digital
pressure directly on the puncture site for 3 to 5 minutes after the
needle is withdrawn.
-Inspect the site for excessive bruising after the procedure

2. Prothrombin consumption
- The prothrombin consumption time (PCT, serum prothrombin time) test
measures utilization of prothrombin when a blood clot forms. Normally, the
formation of a clot consumes Prothrombin by converting it to thrombin.
Individuals with deficiencies in platelets or factors involved in the intrinsic
coagulation pathway are not able to convert as much prothrombin to thrombin.
In such cases like hemophilia, excess prothrombin remains in the serum after
the clot is formed, thus shortening the PCT

Preparation:
- If the individual is receiving anticoagulant therapy, the time and the
amount of the last dose should be noted.
-Explain to the child and parents:
The purpose of the test
The procedure, including the site from which the blood sample is
likely to be obtained
That momentary discomfort may be experienced when the skin Is
pierced

10
 That food, fluids, and drugs are to be withheld before to the test
- For children, a doll may be used as the patient for demonstration
purposes. A laboratory technicians equipment basket may hold the
childs attention during the actual procedure.
- For all clients, encourage questions and verbalization of concerns
about the procedure, and provide calm, reassuring environment and
manner.

Nursing considerations:
- Ensure infant safety while performing the test.
- Maintain digital pressure directly on the puncture site for 3 to 5
minutes after the needle is withdrawn.
-Inspect the site for excessive bruising after the procedure

3. Assays for clotting factors VIII and IX

- If the PT or PTT/aPTT is abnormal, but the nature of the factor deficiency is
unknown, specific coagulation factors may be measured. Factor assays are
used to discriminate to follow the course of acquired factor inhibitors.

Preparation:
-Explain the test to the parents
- Cover the genital area with a lead apron.
Nursing considerations:
- Remove any articles from the infants body and protect vital areas
- Ensure safety and comfort after the test.
4. CT scan, MRI
- Computed tomography (CT) and Magnetic resonance imaging can also be
helpful in determining the site of bleeding including its extent (Zaiden, 2016).
Preparation:
- Explain to the parents:
That the procedure requires from 45 minutes to 2 hours,
depending on the extent of the imaging and whether a contrast
medium is used. Assess for any allergies when contrast is to be
used.
That foods and fluids are withheld for 4 hours before the
procedure if a contrast medium is used.
That a sedative may be used.
Nursing considerations
-Advise parents to resume food intake and increase fluid intake to
eliminate contrast medium, if one was used
- Note and report nausea, skin rash, sweating, palpitations, respiratory
changes, and changes in vital signs.

11
 V. INTERVENTIONS
A. General Nursing Interventions
1) Monitor for bleeding and maintain bleeding
precautions.
2) Prepare to administer replacement factors as
prescribed.
3) DDAVP (1-deamino-8-D-arginine vasopressin), a
synthetic form of vasopressin, increases plasma
factor VIII and may be prescribed to treat mild
hemophilia.
4) Monitor for joint pain; immobilize the affected
extremity if joint pain occurs.
5) Assess neurological status (child is at risk for
intracranial hemorrhage).
6) Monitor urine for hematuria.
7) Control joint bleeding by immobilization,
elevation, and application of ice; apply pressure
(15 minutes) for superficial bleeding.
8) Instruct the child and parents about the signs of internal bleeding.
9) Immobilize the affected part and elevate above the level of the heart
10)Instruct parents regarding activities for the child, emphasizing the avoidance of
contact sports and the need for protective devices while learning to walk; assist in
developing an appropriate exercise plan.
11) Instruct the child to wear protective devices such as helmets and knee and elbow
pads when participating in sports such as bicycling and skating.
B. Medical Interventions
1. Pharmacological Interventions
a) Administration of recombinant factor VIII or factor IX coagulation
concentrate
Nursing Interventions:
Avoid rapid administration to minimize the possibility of transfusion reaction;
usually 2 to 3 mL/minute; consult package inserts.
Stay with the client for the first 15 minutes of the transfusion and monitor
vital signs and reactions.
Cryoprecipitate and fresh frozen plasma are not recommended because of
their lack of viral inactivation treatment.
Stop the transfusion if hives, headaches, tingling, chills, flushing, or fever
occurs.
Keep child quiet during treatment to decrease pulse and rate of bleeding

b) Aminocaproic Acid
- is a fibrinolytic enzyme inhibitor that can slow the dissolution of blood
clots that do form.
c) Desmopressin (DDAVP)
-induces a transient rise in factor VIII levels.

12
d) Analgesics
- are commonly required to alleviate the pain associated with hematomas
and hemorrhage into joints.
(See drug study below of each medication mentioned above).

Generic Name:
Classification: Contraindication: Side/Adverse
Aminocaproic Acid

Brand Name: Hypersensitive to CNS: dizzi

Amicar Aminocaproic acid malaise
Antifibrinolytic Signs of active EENT: nas
Pharmacologic class: Antihemorrhagic intravascular clotting, stuffiness,
Aminohexanoic acid as in DIC CV: arrhyth
Fibrinolysis inhibitor Upper urinary tract hypotensio
Dosage, timing & route Mechanism of Action bleeding only).
50100 mg/kg/dose Do not use products GI: anorex
administered IV every Inhibits breakdown of containing benzyl bloating, c
6 hr for 2-3 days. After blood clots by interfering alcohol with neonates diarrhea, n
completion, Amicar with plasminogen GU: diures
should be given orally activator substances and
as 50-100 mg/kg q 6H producing antiplasmin
sfor 5-7 days activity.

Generic Name:
Classification: Contraindication: Side/Adverse
Desmopressin (DDAVP)
Brand Name: Antidiuretic Hypersensitive to CNS: drow
Rhinyle drops, Antihemorrhagic Desmopressin or its headache,
Octostim components listlessnes
Pharmacologic class: Renal impairment EENT: intr
Synthetic ADH analogue Previous or current nasal cong
hyponatremia

13
Dosage, timing & route Mechanism of Action

0.3 mcg/kg diluted in 50 As an antihemorrhagic,

ml of normal saline drug increases blood
infused over 15-30 mins. level of clotting factor VIII
and activity of von
Nasal solution: 0.25 Willebrand factor. It also rhinitis.
mcg/kg daily as a single may increase platelet CV: hypert
dose aggregation and hypotensio
adhesion at injury sites tachycardi
by directly affecting blood GI: mild ab
vessel walls. cramps an

Generic Name:
Classification: Contraindication: Side/Adverse
Ibuprofen
Brand Name: Hypersensitivity to CNS: head
Childrens Advil, hydrmorphone dizziness,
Dolgesic, Childrens Acute asthma drowsiness
Motrin Analgesic Increased ICP EENT: blur
vision
Pharmacologic class:
GI: constip
Phenanthrene derivative
dyspepsia,
Dosage, timing & route Mechanism of Action vomiting

30-70 mg/kg daily in May bind with opioid

divided doses three receptors in the spinal
times daily PO cord and higher levels in
the CNS. In this way,
5-10 mg/kg every 4-6 hydromorphone is
hours IVTT believed to stimulate mu
and kappa receptors,
thus altering the
perception of and
emotional response to
pain.

14
2. Surgical and Special Procedures

a) Radiosynovectomy. In patients who develop synovitis from joint bleeds,

intraarticular injection of radioisotopes to ablate the synovium (radiosynovectomy)
can be used to decrease bleeding, slow progression of cartilage and bone
damage, and prevent arthropathy
Nursing Interventions:

Parents of the child should be told that response is unlikely within 14

days of injection and may be delayed until up to 1 month.
Patients should be warned of the risk of a temporary increase in
synovitis following treatment.
Absolute immobilisation of the treated joint(s) fo 48 h using splints or
bed rest is recommended as this will reduce transport of particles
through the lymphatics to the regional lymph nodes.

15
 VI. NURSING CARE PLANS
Ineffective peripheral tissue perfusion related to impaired blood flow through a major vessel caused by bleeding

ASSESSMENT OBJECTIVES INTERVENTION RATIONALE

May exhibit: Short term: Independent: 1. These are indicators of

At the end of 1 hour, the client ineffective peripheral
will be able to: perfusion.
Excessive bleeding 1. Evaluate involved
Maintain moderate activity 2. To promote collateral
Large skin bruises extremity for clinical signs
level to promote circulation. blood flow.
Pain and swelling on Rest comfortably
(pain, decreased
3. To avoid constriction of
joints temperature, pallor,
veins.
Fatigue Long term: delayed capillary refill,
4. Infection or ulcer
Hematuria At the end of 6 hours, the client weak or absent pulse,
formation may develop
Blood in stool will be able to: decreased sensation, and
more easily because of
Experience adequate decreased pulse
perfusion as evidenced by decreased blood supply.
oximetry) 5. Determines the
palpable pulse, normal skin 2. Protect the extremity from
color and temperature at adequacy of systemic
injury using sheepskin or
and beyond the bleeding circulation.
bed cradle and position 6. Dietary aspects may help
site.
extremity at or lower than and contribute to the
level of heart alleviation of symptoms.
3. Teach child and parents 7. This accompanies
to avoid crossing legs or diminished peripheral
keeping legs in a circulation.
dependent position
4. Encourage parents t to
8. To determine probability,
protect extremity from
location, and degree of

16
ASSESSMENT OBJECTIVES INTERVENTION RATIONALE
injury or extreme hot. impairment.
5. Measure capillary refill 9. To promote optimal blood
ofently. flow, organ
6. Note clients nutritional
perfusion, and function.
and fluid status. 10. To maximize systemic
7. Inspect lower extremities circulation and organ
for skin texture that often. perfusion.

Collaborative:

8. Review laboratory
studies.
9. Administer fluids,
electrolytes, nutrients,
and oxygen, as indicated.
10. Collaborate in treatment
of underlying conditions.

17
Ineffective protection related to abnormal blood profile secondary to Hemophilia

ASSESSMENT OBJECTIVES INTERVENTION RATIONALE

Independent:
May exhibit: Short term:
At the end of 1 hour, the client 1. Avoid injections, rectal 11. Protects the child from
will be able to: procedure related
Altered blood clotting temperatures and any
Display relief of fatigue and causes of bleeding.
Bleeding other rectal tubes.
discomfort 12. Reduces accidental
Fatigue 2. Maintain a safe
Demonstrate use of injury, which would result
Impaired healing environment. Keep all
protective measures, in bleeding
conservation of energy and necessary objects within
13. Prompt detection of
attainment of adequate rest reach and keep bed in
bleeding or initiation of
low position. Raise side
Long term: therapy may prevent
rails always.
At the end of 6 hours, the client 3. Test body fluids like urine, critical hemorrhage.
will be able to: 14. Presence of bleeding or
stool and vomitus for
Achieve comfort in a safe hemorrhage may lead to
occult blood.
environment 4. Monitor for changes in circulatory failure or
Display homeostasis as vital signs and skin color shock
evidenced by absence of 15. May reflect cerebral
like blood pressure,
bleeding. bleeding
pulse, perspiration, skin
16. These medications
pallor or discoloration.
reduce platelet
5. Evaluate change in the
aggregation.
level of consciousness.
17. Adequate sleep and
6. Avoid use of aspirin

18
ASSESSMENT OBJECTIVES INTERVENTION RATIONALE
products and NSAIDS nutrition enhance
7. Teach protective immune function. Energy
measures, including the conservation can help
need to conserve energy, decrease the weakness
obtain adequate rest, and caused by anemia.
eat a balanced diet. 18. To decrease threat from
8. Promote personal and microorganisms.
environmental 19. Discomfort interferes
cleanliness. with rest, interferes with
Collaborative: nutritional intake, and
9. Administer medications places added stress on
as ordered for symptoms. the patient.

19
Activity Intolerance related to bleeding episodes.
ASSESSMENT OBJECTIVES
Possibly evidenced by: Short term:
At the end of 30 minutes, the
Excessive bleeding client will be able to:
Large skin bruises Maintain position of function.
Pain and swelling on Increase strength/function of
joints affected and compensatory
Fatigue body parts
Hematuria Maintain maximum joint
Blood in stool range of motion (ROM).
Impaired wound healing
Long term:
At the end of 12 hours, the client
will be able to:
Demonstrate a decrease in
physiological signs of
intolerance
Demonstrate improvement
INTERVENTION
Independent:
1. Monitor physiologic
responses to increased
activity level, including
respirations, heart rate and
rhythm, and blood
pressure.
2. Perform active or passive
ROM exercises to all
extremities every 24 hr.
as tolerated.
3. Turn and reposition patient
at least every 2 hr.
Establish a turning
schedule for the dependent
patient. Post schedule at
RATIONALE
1. To ensure that these return
to normal within 25 min
after stopping exercise.
2. These exercises foster
muscle strength and tone,
maintain joint mobility, and
prevent contractures.
3. Turning and repositioning
prevent skin breakdown
and improve lung
expansion and prevent
atelectasis.
4. To avoid contractures and
maintain optimal
musculoskeletal balance
and physiologic function.
5. To allow child to maintain
or increase muscle tone
20
ASSESSMENT OBJECTIVES INTERVENTION
in activity intolerance bedside and monitor
Participate willingly in frequency.
necessary/desired play 4. Maintain proper body
alignment at all times.
5. Teach about isometric
exercises. But plan to
carefully balance rest
periods with activities.
6. Teach parents to assist
child with ADLs in a way
that maximizes childs
potential.
7. Involve child and parents in
planning and decision
making.
RATIONALE
and joint mobility and to
reduce fatigue.
6. This enables caregivers to
participate in patients care
and encourages them to
support patients
independence.
7. Having the ability to
participate will encourage
greater compliance with
the plan for activity.
21
Risk for fluid volume deficit related to spontaneous bleeding.
ASSESSMENT
Possibly evidenced by:
Excessive bleeding
Large skin bruises
Pain and swelling on
joints
Fatigue
Hematuria
Blood in stool
Impaired wound healing
Dry skin and mucous
memebranes
Poor skin turgor
Low blood pressure
OBJECTIVES
Short term:
At the end of 30 minutes, the
client will be able to:
Maintain stable vital signs
Have normal skin color
Maintain and adequate fluid
and urine volume
Long term:
At the end of 12 hours, the client
will be able to:
Have normal skin turgor and
moist mucous membranes
Have normal fluid and
INTERVENTION RATIONALE
1. Tachycardia, dyspnea, or
Independent: hypotension may indicate
fluid volume deficit or
1. Monitor and record vital electrolyte imbalance
signs every 2 hr or as 2. Low urine output and
often as necessary until high specific gravity
stable. Then monitor and indicate hypovolemia.
record vital signs every 4 3. Weight is a good
hr. indicator of fluid status.
4. To avoid dehydrating
2. Measure intake and mucous membranes.
output every 14 hr. 5. To prevent vasodilation,
Record and report blood pooling in
significant changes. extremities, and reduced
22
ASSESSMENT OBJECTIVES INTERVENTION RATIONALE
blood volume circulating blood volume.
Include urine, stools, 6. To avoid orthostatic
vomitus, wound drainage. hypotension and possible
syncope.
3. Weigh patient daily at 7. To replace fluids and
same time to give more whole blood loss and
accurate and consistent facilitate fluid movement
data into intravascular space
8. To prevent further fluid
4. Assess skin turgor and loss.
oral mucous membranes
every 8 hr and give
meticulous mouth care
every 4 hr.

5. Cover patient lightly.

Avoid overheating.

6. Dont allow patient to sit

or stand up quickly as
long as circulation is
compromised

Collaborative:

7. Administer fluids, blood

or blood products, or

23
 ASSESSMENT OBJECTIVES INTERVENTION RATIONALE

plasma expanders
8. Administer and monitor
medications

Risk for injury related to weakness of the defense secondary to hemophilia.

ASSESSMENT OBJECTIVES INTERVENTION RATIONALE

Possibly evidenced by: Short term: 1. To increase patients

24
 ASSESSMENT OBJECTIVES INTERVENTION RATIONALE
At the end of 1 hour, the client awareness of potential
Vulnerability to injury will be able to: Independent: dangers
Remain safe and 2. Poor lighting is a major
comfortable 1. Help patient identify cause of falls.
Long term: situations and hazards 3. This will foster child
that can cause accidents. household safety.
At the end of 1 week, the client
2. Maintain lighting at all 4. To decrease possibility of
will be able to:
times injury.
Practice safety 3. Encourage adult patient
precautions in and out to discuss safety rules
the house with children to foster
Parents are able to household safety. For
instruct the child in safety example:
habits Dont play with
Childproof house to matches.
ensure safety of young Use electrical
children and cognitively equipment carefully.
impaired adults. Know location of the
fire escape route.
Dont speak to
strangers.
Dial 911 in an
emergency.
4. Encourage parents to
make repairs and remove
potential safety hazards
from environment.

25
 REFERENCES
A. Books

Pillitteri, A. (2010). Maternal and Child Health Nursing. Philadelphia: Lippincott

Williams & Wilkins.

Sparks, S., Taylor, C. (2011). Nursing Diagnosis Pocket Guide. Wolters

Kluwer, Lippincott Williams and Wilkins; Philadelphia

Boyer, M. (2010). Brunner and Suddarth's Textbook of Medical-Surgical

Nursing. Philadelphia: Lippincott Williams & Wilkins.

Riske, B. (2005). Hemophilia Nursing Handbook. New York: Hemophilia

Foundation.

Behrman, R., Kliegman, R., & Jenson, H. (2004). Nelson textbook of

Pediatrics. St. Louis: Saunders.

B. Electronic Sources

Zaiden, R. (2016). Hemophilia: Treatment and Management. MedScape, 12-

24.

World Federation of Hemophilia.org (2017). The Basics of Hemophilia. 45

Retrieved January 27, 2017.

C. Journals

Rickard KA. Guidelines for therapy and optimal dosages of coagulation

factors for treatment of bleeding and surgery in haemophilia.
Haemophilia 1995;1(S1):813.

26