TABLE OF CONTENTS

I. Introduction……………………………………………………………………………1
II. Concept Map……………………………………………………………….……........2
III. Medical Management……………………………………….……………………3 - 6
IV. Nursing Management……………………………………………….…………….8 - 14
V. Discharge Planning………………………………………….............................15 - 16
VI. Bibliography…………………………………………………………………………17

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 INTRODUCTION

Hemophilia A is referred to as classic hemophilia and was first recognized in the second
century AD. The disease is an X-linked bleeding disorder caused by defects in the
clotting cascade enzyme factor VIII. Factor VIII serves as a cofactor in the activation of
factor X to Xa in a reaction referred to as the "tenase" complex.

The factor VIII gene (symbol = F8) resides near the telomeric end of the long arm of the
X chromosome. Multiple mutations have been identified leading to hemophilia A such as
frameshift mutations, missense mutations, nonsense mutations, gene inversions, large
deletions and splicing errors. Patients with hemophilia A suffer from joint and muscle
hemorrhage, easy bruising and prolonged bleeding time from wounds. Almost all
patients with hemophilia A have normal platelet function thus bleeding is generally not
severe. Hemophilia A can be divided into severe or moderate disease. Since it is an X-
linked disease almost all patients are male with 1 in 5,000 male births. It is also possible
that a female can be affected but only a few.

In the Philippines, there are about 8,000 persons with hemophilia, but only 1,000 of
them are registered with the Philippine Hemophilia Foundation (PHF). There are
hemophiliac patients out there who do not even know they have the disease, and so
they are not being treated. Treatment for hemophilia is extremely expensive, and anti-
hemophilic clotting factors are not readily available. People suffering from this disease
now rely heavily on donations through Project Share, a humanitarian program that
donates clotting factors to developing countries.

Patient X is a 7 year old, male who was confined at Madonna and Child Hospital and
was diagnosed with Intracranial bleeding secondary to Hemophilia A. 2 days PTA there
was an onset of on and off fever associated with headache, left orbital pain which is
later associated with episodes of vomiting and inability to urinate. He was then admitted
at Maria Reyna Hospital in which pain medications given. CT scan showed the
presence of intracranial bleeding, hence referred to Madonna and Child Hospital for ICU
admission

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3
 Medical Management
Medical intervention
HEMOPHILIA
1. Demand Therapy
2. Continuous infusion
3. Replacement therapy:
-Plasma
-Cryoprecipitate
Factor Concentrates
1. FVIII infusion
Adjuvant treatment option
1.Desmopressin
Antifibrinolytic Therapy
1. Tranexamic acid
Oral:25 mg/kg/dose every 6-8hrs
IV: 10 mg/kg/dose every 6-8hrs
2.EACA
Oral: 100-200mg/kg initially followed
by 50-100 mg/kg/dose every 6hrs
IV: 100 mg/kg/dose every 6hrs
Rationale
-Infusion of a factor at the time of a bleed.
-It is usually given before or after elective
surgery or after major trauma.
-Replacement of FVII or IX to hemostatically
adequate plasma level for prevention or
treatment of acute bleeding is the basis of the
management of hemophilia.
- 1 U FFP contains about 160-
250ml plasma with activity of
80%.
- Cryo prepared from 200ml of
FFP contains 80-100 U of FVIII,
250mg fibrinogen and useful
amounts of FXIII and vWF per
10-15 ml of precipitate
-by slow IV push at a rate not to exceed 100 U
per minute in children
-increase plasma FVIII level
-inhibits fibrinolysis of thrombus by plasmin.
- a competitive inhibitor of plasminogen
activation, and at much higher concentrations,
a noncompetitive inhibitor of plasmin. Indicated
for hemophilia for short-term use (two to eight
days) to reduce or prevent hemorrhage
- used in numerous clinical situations to control
bleeding and has been claimed to be an
effective agent in subarachnoid hemorrhage,
genitourinary bleeding from many causes, and
in dental surgery in hemophiliacs.
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Newer Tx Modalities:

1. Activated Prothrombin complex -have increased amounts of activated FVIIa, a

concentrates factor X & thrombin

2.Polyethylene glycol conjugation -increases size, decreases renal excretion,

(Pegylation) extends half life.

3.Polysialicacid polymers -Forms a “watery cloud” around the target

molecule

4.Recombinant factor VIIa -by passes FVIII-dependent stepin factor X

activation
-primary use: hemophilia with inhibitors.

5. Gene Therapy -involves a transfer of genes that express a

particular gene product into human cell.

SUBARACHNOID HEMORRHAGE

Imaging Studies:

1.CT Scan -the location and mass effect of hemorrhage

2. LP (Lumbar Puncture) -to rule out ICP

D-dimer Assay -can discriminate SAH from traumatic LP

3. Cerebral Angiography For:

a. Cerebrovascular anatomy
b. aneurysm location and source of bleeding
c. aneurysm size and shape

4. Intracranial Doppler studies -detection and monitoring of arterial

vasospasm

5. Chest Radiograph -evaluation of possible pulmonary

complications

6. Echocardiogram - evaluation of ventricular wall motion

7. ECG -evaluation of evaluation of non-specific ST

and T wave changes, decrease PR intervals,
increase QRS intervals, increase QT intervals,
presence of U waves
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1. Patient stabilization
-assess level of consciousness,
airway, breathing and circulation.
2. Endotracheal Intubation
3, IV access should be obtained
4. Benzodiazepines (Midazolam)
5. Surgery
Surgical Clipping
Pharmacolgic intervention
1. Mannitol 100cc q4h
2. Furosemide 20mg q4h
3. Ranitidine 20mg IVTT q8h
4. Lactulose (Lilac) 10ml OD HS
5. Paracetamol 250mg/5ml 5ml
q4h RTC
6. Tranexamic acid 250mg IVTT
q8h
7. Factor 8 1 vial q8h
8. Midazolam drip 6 amps 20cc
-initial treatment.
-for patients presenting with coma, depressed
level of consciousness, inability to protect their
airway, increase ICP
-including central and arterial lines.
-administered prior to all procedures for
sedation purposes.
- recommended because the risk of SAH
complications greatly exceed the risk of
surgical complications.
Rationale
For raised intracranial pressure
For acute pulmonary edema
Prophylaxis of stress ulceration and recurrent
hemorrhage from peptic ulcer.
For constipation.
Relief of mild-to-moderate pain.
Temporary reduction of fever.
-a competitive inhibitor of plasminogen
activation, and at much higher concentrations,
a noncompetitive inhibitor of plasmin. Indicated
for hemophilia for short-term use (two to eight
days) to reduce or prevent hemorrhage
-replacement is used for acute bleeding, for
perioperative prevention of bleeding during
planned surgical procedures, for prophylaxis to
prevent recurrent bleeding of target joints, in
early institution of childhood prophylactic
therapy to preserve long-term joint function, or
for immune tolerance induction (ITI) regimens.
-Continuous sedation of intubated
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 D5W @ 1occ/hr And mechanically ventilated patients as
a component of anesthesia or during
treatment in the critical care setting.

9. Nubain 20mg q4h IVTT Moderate to severe pain, adjunct to balanced

anesthesia.

10. Ampicillin 1g q6h IVTT ANST (-) Used for treating bacterial infection.

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 NURSING CARE PLAN
Cues Nursing Goal/Plan Intervention/Plan Rationale Evaluation
Diagnosis
Cues: Impaired Short term: Independent: Evaluation will
Subjective: spontaneous At the end of 8 hours, 1. Verify that - to synchronize be done upon
- Dyspnea ventilation patient will be able to: patient's respirations and delivery of these
related to respirations are in reduce work of interventions. It
respiratory 1. Maintain effective will be based
phase with breathing/
Objective: muscle respiratory pattern via upon the
ventilator. energy
- Tachycardia (HR: weakness ventilator with absence goals/objectives
expenditure
109 bpm) of retractions/ use of and will be
accessory muscles 2. Elevate head - physically and
-TCO2: 16.2 (23-27 psychologically categorized as:
mmol/L) 2. Maximize of bed
beneficial.
-Ph: 7.66 (7.35-7.45) respiratory function - ensure • Fully met –
-PCO2: 17.5 (35-45 adequate if the
mmHg) ventilation or patient/
Long term: S.O.
-PO2: 180.5 (80-105 delivery of
mmHg) At the end of 2 days, desired tidal verbalizes
-HCO3: 19.5 (22-26 patient will be able to: volume understandi
mmol/L) 1. Demonstrate ng as well
3. Inflate ET as agrees to
-SO2: 99.7 (95-98%) behaviors necessary cuff properly using
to maintain - Ventilators participate
minimal leak/ in such
respiratory function. have a series of
occlusive intervention
visual and
2. Maintain ABGs and technique. Check s
audible alarms.
oxygen saturation cuff inflation every Turning off or
within acceptable 4-8 hours and failure to reset • Partially met
range. whenever cuff is alarms places – if the
deflated/ reinflated
3. Participates on patient at risk for patient/ S.O
efforts to wean (as unobserved verbalizes
appropriate) within ventilator failure understandi
individual ability. or respiratory ng but
distress. refuses to
4. Check tubing

8
 for obstruction - Kinks in the participate
such as kinking or tubing prevent in such
accumulation of adequate volume intervention
water. Check delivery and s or vice
ventilator alarms increase airway versa
for proper pressure. Water
functioning. Do prevents proper • Not met – if
not turn off gas distribution the patient /
alarms. Ascertain and predisposes S.O.
that alarms can be to bacterial verbalizes
heard growth no
understandi
5. Keep ng as well
resuscitation bag at as refuses
- restores to
bedside and adequate
ventilate manually participate
ventilation when in any
whenever indicated patient or intervention
problems require s stated in
patient to be this care
temporarily plan.
removed from
the ventilator
Dependent:
1. Assess ventilator - to maintain
settings routinely parameters
and readjust as within
indicated. appropriate
limits.

Collaborative:
Monitor ABG
values. Note - Values are
alterations. affected by
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 capillaries. matching of
ventilation in lung
with perfusion of
pulmonary

10
Subjective: Ineffective Short term: Independent: Evaluation will
- “Wala may tarong cerebral At the end of 8 hours, 1. Regulate - Increased be done upon
iyang katulog katong tissue patient/ SO will be able environmental metabolic needs delivery of these
wala pa siya gipa- perfusion to: temperature as and oxygen interventions. It
tulog sa doctor, sige related to indicated consumption will be based
1. Verbalize
ra man siyag wild sa space understanding of
occur which can upon the
kasakit,” as occupying further increase goals/objectives
condition, therapy
verbalized by mother. lesions regimen, side effects of
ICP. and will be
(hemorrhage) categorized as:
- “sa wala pa na- medications and when
admit, dili siya to contact health care - compresses
2. Maintain head/ the jugular veins • Fully met – if
ganahan ug kaon kay team. neck in midline or and inhibits the patient/
isuka man niya, wala 2. Maintain vital signs neutral position. cerebral venous S.O.
pud siyay gana kay within client's normal Support with small drainage. verbalizes
sakit daw iyang ulo,” range. towel rolls and understandi
as verbalized by 3. Stay alert/ oriented pillows. Avoid ng as well
mother. and without change in placing head on as agrees to
pupillary reaction. large pillows. participate in
- continual such
Objective:
3. Provide rest activity interventions
-altered level of Long term produces a
periods between
consciousness At the end of 2 days, care activities and cumulative • Partially met
-restlessness patient will be able to: limit duration of stimulant effect. – if the
-change in vital signs 1. Maintain usual/ procedures. patient/ S.O
improved level of - Provides verbalizes
-on and off fever calming effect, understandi
consciousness, 4. Decrease
-vomiting cognition and motor/ extraneous stimuli reduces ng but
-Intracranial bleeding sensory function. and provide comfort adverse refuses to
shown on CT scan measures such as physiological participate in
2. Demonstrate stable
quiet environment, response and such
vital signs and promotes rest to interventions
absence of signs of soft voice, gentle
touch. maintain and or vice versa
increased ICP.

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Maintain stable mental lower ICP.
status. • Not met – if
the patient /
5. Help patient avoid - These S.O.
or limit coughing, activities verbalizes
vomiting, straining at increase no
stool or bearing intrathoracic understandi
down when and intra- ng as well
possible. Reposition abdominal as refuses to
patient slowly. pressures which participate in
can increase any
ICP. interventions
stated in this
Dependent: care plan.
1. Administer
supplemental
oxygen as indicated.
- Reduces
hypoxia which
may increase
cerebral
vasodilation and
2. Administer blood volume
medications as elevating ICP.
indicated such as
diuretics, -to draw water
analgesics, from brain cells;
to relieve pain;
to control fever;
to control
restlessness
Collaborative: and agitation
Monitor ABGs/

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 pulseoximetry. - Determines
respiratory
sufficiency and
indicates
therapy needs.

13
Subjective: Pain related Short term: Independent: Evaluation will
-“Katong wala pa to At the end of 8 hours, 1. Provide - To promote be done upon
siya gitagaan ug pain intracranial patient will be able to: comfort non delivery of these
reliever grabe jud bleeding 1. Follow prescribed measures (touch, pharmacological interventions. It
iyang wild tungod sa secondary to pharmacologic repositioning, pain will be based
kalabad sa ulo,” as Hemophilia A regimen. presence of SO) management. upon the
verbalized by mother. quiet goals/objectives
2. Report control and
environment and and will be
- “Halos dili mawala alleviation of pain to
calm activities. categorized as:
kung tambalan,” as 4/10.
verbalized by mother. 3. Demonstrate use of
2. Encourage - To distract • Fully met – if
relaxation skills and
- “Pag mag hilanat use of relaxation attention and the patient/
diversional activities.
siya ug taas techniques. reduce tension. S.O.
(frequency/ verbalizes
precipitating factor),” Long term: understandi
as verbalized by At the end of 2 days, 3. Suggest ng as well
- To comfort
mother. patient (with the help of parent be as agrees to
child.
SO) will be able to: present during participate in
1. Verbalize non procedures such
Objective: - To prevent
pharmacologic interventions
-Left orbital pain with fatigue.
methods that provide 4. Encourage
8/10 intensity
relief. adequate rest • Partially met
-Patient displays - To determine – if the
2. Keep down periods
facial grimacing response to patient/ S.O
expressive behaviors
such as restlessness, 5. Observe non pain since verbalizes
crying, irritability. verbal cues/ pain patient cannot understandi
Be free of pain. behaviors. communicate ng but
verbally. refuses to
participate in
such
interventions
or vice versa

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 Dependent: - To explore
1. Collaborate in methods for • Not met – if
treatment of control of pain. the patient /
underlying S.O.
condition/ verbalizes
disease process no
causing pain. understandi
- To alleviate ng as well
pain as refuses to
2. Administer participate in
analgesics as any
indicated. interventions
stated in this
care plan.
Collaborative:

1. Assist in thorough - To determine

evaluation, including contributory
neurological and factors.
psychological
factors as
appropriate, when
pain persists

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16
 DISCHARGE PLAN
Medication • Encourage the patient of strict compliance to the
medication or factor concentrate and to take
medication/factor as directed to attain therapeutic effects
 Mannitol
 Lactulose
 Furosemide
• Give adequate instructions about the importance of the
medication/ factor concentrate and dietary regimens so
that the patient’s condition will improve and recover to its
healthy state as soon as possible
• The patient must be taught about his medication regarding
its action, indication, right dose, contraindication and its
side effects
• Immediate notification on physician for presence of
adverse reaction in medication, homecare complication
and severe bleeding should also be encouraged
Exercise • Instruct patient to have a moderate exercise and avoid
strenuous exercise which might cause injury.
Treatment • Proper compliance of home medication/ factor concentrate
should be followed as prescribed by the doctor
• Encourage the client to relax and have adequate rest to
prevent stress and promote healing
Health teaching • Provide patient with relative written and verbal information
regarding the ff:
1. Proper hygiene should always be observed to
prevent infection
2. Lifestyle changes. Preventive measures may reduce
symptoms and prevent recurrence of the problem
• compliance to follow-up examinations or check- ups
• teach patient about giving first aid when adverse reactions

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 to the medication arise
3. diet modification to enhance chances for
recuperation
4. Avoidance of using heat for it will cause vasodilation
and may exacerbate a bleeding episode
Avoidance of aspirin or any anticoagulant medications and
IM injections for it could exacerbate bleeding episodes.
Out patient follow- • instruct patient to comply with further examinations or
up follow-up check-ups
encourage patient/SO to ask the physicians regarding the
test results and on any matters of concern
Diet • instruct patient to follow the diet prescribed by the doctor if
there are any
• avoid foods and factors, such as alcohol, spicy foods and
coffee and smoking to avoid gastric irritation thus
preventing GIT bleeding
Eat a high-fiber diet to increase the bulk of the stool, which
helps prevent diverticulosis and hemorrhoids
Spirituality • patient should provide a continuous and appropriate
outook on health to promote development of faith
• having established a strong foundation in following faith
development includes encouraging patient to go to mass,
receive the holy Eucharist and attend confessions

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 BIBLIOGRAPHY

Smeltzer. s.c., et.al. (2009). Brunner & Suddarth’s Textbook of Medical – Surgical

Nursing 11th ed. Singapore: Saunders Publication

Bullock, b.l., & Henze. r.l. (2000). Focus on Pathophysiology. New York: Lippincott

Williams & Wilkins.

Black, j. m., & hawks, j. j. (2009). medical - surgical nursing 8th ed. Singapore:

Saunders Publication.

Doenges,m.r., et.al.(2009). Nurse’s Pocket Guide: Diagnosis, Prioritized Interventions,

and Rationales 11th ed. Philadelphia: F.A. Davis Company.

http://stroke.ahajournals.org/cgi/content/full/30/4/905#SEC4

http://bmb.oxfordjournals.org/cgi/reprint/56/2/444.pdf

http://emedicine.medscape.com/article/1163977-treatment

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