PROBLEMS WITH

PLASMA

COAGULATION & BLEEDING

DISORDERS
COAGULATION & BLEEDING DISORDERS

poor blood clotting - Coagulopathy

 continuous & excessive bleeding - Hemorrhage
COAGULATION & BLEEDING DISORDERS

HEMOPHILIA
HEMOPHILIA

 A deficiency of coagulation resulting from a disorder in x linked recessive trait and deficit in clotting factors
(VIII,IX,XI).

 Males inherit hemophilia from their mothers

 Females inherit the carrier status from their fathers
HEMOPHILIA

a. Hemophilia A (HA) or the classic form - due to the deficiency

in factor VIII (Anti Hemophilic globulin)
b. Hemophilia B (HB) or Christmas disease – due to deficiency
in factor IX (Plasma Thromboplastin Component)
c. Hemophilia C – due to factor XI (Plasma Thromboplastin
Antecedent) deficiency (Autosomal recessive)
HEMOPHILIA
Pathophysiology and Etiology:
1. Hereditary
a. Sex – linked recessive trait (X
chromosome)
b. Transmitted by asymptomatic female
c. Appears in males with hemophilic
gene on their only X chromosome
d. Affected males can pass the gene
to female offspring (carriers)
HEMOPHILIA
Typical Clinical Manifestations

1. Slow persistent bleeding

 after minor injury ( cuts, scratches)

 At birth after cutting cord
 following circumcision
 following IM immunization
2. increase bruising as child learns to crawl and walk
HEMOPHILIA
Typical Clinical Manifestations

3. Abnormal bleeding in response to trauma

- Bleeding gums after dental extraction or even brushing the teeth with a had
toothbrush
- Severe sometimes fatal epistaxis after injury to the nose
 HEMOPHILIA

 Clinical Manifestations
Severe
1. Hemarthrosis – hallmark; bleeding swollen joints, painful
and can lead to deterioration of the joints – deformed and
permanently crippled.
HEMOPHILIA

2. Synovial hypertrophy
3. Hemosiderin deposition
4. Fibrosis
5. Damage to the cartilage
6. Subchondral cyst
HEMOPHILIA

5. Overwhelming gastric hemorrhage – liked to gastritis and ulcers

6. Recurrent hematoma formation in deep SQ tissue and intramuscular tissue (muscular atrophy)
7. Hematoma around peripheral nerve causing compression which may result to severe pain anesthesia of
the innervated part, nerve damage and paralysis.
HEMOPHILIA

8. Hematuria r/t genitourinary trauma

9. Splenic rupture from falls or abdominal trauma

10. Intracranial bleeding – leading cause of hemorrhagic death.

HEMOPHILIA

 Diagnostic Evaluation
1. Platelet ct, Prothrombin time and bleeding time are normal
2. PTT – prolonged
HEMOPHILIA
MANAGEMENT

 Stop topical bleeding as quickly as possible

1. Apply pressure or ice pack to the injured site.

2. Pack the area with fibrin foam
3. Apply topical hemostatic agents
(thrombin)
HEMOPHILIA
MANAGEMENT

 Supply the missing factor causing the hemorrhage

1. Transfusion of factor VIII or IX concentrate

- Administer Factor VIII concentrate / cryoprecipitate

- Thaw slowly; Gently rotate bottle; Infuse immediately;
deteriorates at room temperature
HEMOPHILIA
MANAGEMENT

 Prevent complications leading to and cause by bleeding

1. Discuss the need to curtail physical activities such as contact sport and minor invasive procedures, falls
and cuts that may precipitate and injury.
2. Teach client and family early manifestations of bleeding.
HEMOPHILIA

 Complication
1. Hemorrhage
2. Airway obstruction due to compression caused by hemorrhage
3. Nerve compression
4. Intracranial bleeding
5. Acquiring blood borne viruses
(Hepatitis and HIV)
HEMOPHILIA
NURSING INTERVENTION

 Control hypovolemia through control of bleeding

1. Provide emergency care for bleeding
a. Apply pressure or cold on area
for 10 -15 mins to allow clot
formation – venipuncture or
injection
b. Place firbin foam or absorbable
gelatin foam in the wound.
HEMOPHILIA
NURSING INTERVENTION

2. Immobilize the affected part

and elevate above the level of the heart
3. Keep child quiet during the treatment to decrease pulse and rate of bleeding
4. Monitor bleeding, joint pain, neurological status, urine for hematuria
5. Monitor VS , treat shock if child becomes hypotensive.
HEMOPHILIA
NURSING INTERVENTION

 Providing protection against bleeding

1. Avoid rectal temperature

2. Avoid injections if possible
a. Administer medications orally
whenever possible
b. SQ rather than IM
c. Apply pressure on the injection
site.
HEMOPHILIA
NURSING INTERVENTION

3. Maintain a safe environment and teach parents safety measures.

a. Pad crib or bedrails
b. Inspect toys for sharp or rough edges.
c. Offer finger foods and fluids in plastic or paper containers
d. Supervise small children when the are ambulatory
e. Continually assess environment for potential hazards
f. No straws or sharp eating utensils
g. No hard candy
h. avoidance of contact sports
HEMOPHILIA
NURSING INTERVENTION

 Preserving Mobility
1. Treat hemarthrosis or muscle bleed as soon as possible
2. Supportive management for hemarthrosis
a. Immobilize joint – slight flexion
b. Elevate the affected part above
the level of the heart
c. Apply ice packs then after active
bleeding has stopped apply heat
HEMOPHILIA
NURSING INTERVENTION

3. For severe cases: continue

immobilization and casting, prevent
weight bearing

4. For less severe hemathrosis: begin gentle passive exercise 48 hours

after the acute phase – prevent stiffness and fibrosis. Progress to
active exercise

5. Administer short course of corticosteroids as ordered

HEMOPHILIA
NURSING INTERVENTION

 Relieve Pain
1. Be aware the increase pain means bleeding continues and further
replacement therapy may be needed.
2. Assess for further swelling of joints and limitation of movement.
3. NSAID – acute phase. Cautious in chronic pain – interferes with
platelet function.
4. Alternative treatment: TENS, hypnosis, relaxation techniques
COAGULATION & BLEEDING DISORDERS

VON WILLEBRAND DISEASE

VON WILLEBRAND DISEASE

 Von Willebrand factor is a protein in blood that helps blood to clot.

 In von Willebrand disease, the body either produces too little of the
protein, or produces a protein that doesn't work well.
 The condition is inherited, but most people with Von Willebrand
disease have no symptoms and don't know they have it. Some people
with Von Willebrand disease will have excessive bleeding after an
injury or during surgery
HYPERCOAGUABLE STATE

 A tendency for the blood to clot too easily;

 most affected people have only a mild excess tendency to clot, and may never be diagnosed.
 Some people develop repeated episodes of blood clotting throughout life, requiring them to take a daily
blood thinning medicine.
COAGULATION & BLEEDING DISORDERS

DEEP VENOUS THROMBOSIS

DEEP VENOUS THROMBOSIS

 A blood clot in a deep vein, usually in the leg;

 a deep venous thrombosis can dislodge and travel through the heart to the lungs, causing a pulmonary
embolism.
COAGULATION & BLEEDING
DISORDERS

DISSEMINATED
INTRAVASCULAR
COAGULATION
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)

 Acquired Thrombotic And Hemorrhagic Syndrome

 results in tiny blood clots or bleeding tendency or both
 characterized by abnormal intravascular activation of the coagulation cascade and
accelerated fibrinolysis and consumption of clotting factors.
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)

Etiology (unknown)
A syndrome arising with an underlying disorder or event:
a. Overwhelming infections,
particularly bacterial sepsis
b. Obstetric complications:
abruptio placenta, eclampsia,
amniotic fluid embolism,
retention of dead fetus
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)

Etiology

c. Massive tissue injury, burns, trauma, fracture, major surgery and fat embolism
d. Vascular and circulatory collapse -shock
e. Hemolytic transfusion reaction
f. Malignancy: particularly lung, colon,
stomach and pancreas.
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
Pathophysiology
* Abnormal activation of the clotting cascade and
accelerated fibrinolysis
*Results in widespread clotting of small vessel of
the body with consumption of clotting factors and
platelets.
* At the same time forming an unstable
clot resulting to bleeding and thrombosis
occurring simultaneously.
 DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)

2 CONFLICTING SETS OF MANIFESTATIONS:

 Diffuse fibrin deposition within arterioles and capillaries throughout the body
 Bleeding into the kidneys, brain, adrenal, heart and other organs
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
Clinical Manifestations

1. Signs of abnormal clotting

a. Coolness and mottling of extremities
b. Acrocyanosis (cold, mottled extremities with clear demarcation from normal
tissues)
c. Petechiae, ecchymosis
Mottled skin
Acrocyanosis
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
Clinical Manifestations

2. Signs of abnormal bleeding

a. Oozing, prolonged bleeding from sites of procedures, IV catheter
insertion sites, suture lines, mucous membranes, orifices.
b. Internal bleeding leading to changes in vital organ function.
c. Altered VS
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
DIAGNOTIC EVALUATION

1. Platelet count – diminished

2. PT and PTT – prolonged
3. Fibrinogen – decreased level
4. Fibrin split (degradation) products – increased level
5. D – Dimer fibrin degradation product – increased level
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
MANAGEMENT

1. Treat underlying disorder

2. Replacement therapy for serious hemorrhagic complications
a. Fresh frozen plasma replaces clotting factors
b. Platelet transfusions
c. Cryoprecipitate replaces clotting factor and fibrinogen
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
MANAGEMENT

3. Supportive measures including fluid replacement, oxygenation, maintenance of BP

and renal perfusion.

4. Heparin therapy (controversial) – inhibits clotting component of DIC

DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
COMPLICATIONS

1. Thromboembolic: pulmonary embolism, cerebral, myocardial, splenic or bowel

infarction, acute renal failure, tissue necrosis or gangrene
2. Hemorrhagic: cerebral hemorrhage is most common cause of death in DIC.
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
NURSING INTERVENTIONS
1. Minimize bleeding
- Monitor pad count/amount of saturation during menses
- Monitor platelet count
- Avoid dislodging clots.
- Apply pressure to site of bleeding
- Assist with platelet transfusions
 cryoprecipitate - increases fibrinogen
 fresh frozen plasma -increases clotting factors
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
NURSING INTERVENTIONS

4. Maintain bed rest during bleeding episodes

5. If internal bleeding is suspected – assess abdominal girth and bowel sounds
6. Evaluate fluid status and bleeding by frequent measurement of VS, CVP and I & O.
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
NURSING INTERVENTIONS

 Promoting Tissue Perfusion

1. Keep patient warm
2. Change patient’s position frequently and perform ROM exercises
3. Monitor ECG and laboratory test for dysfunction of vital organs caused by
ischemia.
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
NURSING INTERVENTIONS

4. Monitor for signs of vascular occlusion and report immediately

Brain decrease LOC,
a.
sensory and motor deficits
b. Eyes – visual defects
c. Bone – bone pain
d. Pulmonary vasculature – chest pain,
shortness of breath
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
NURSING INTERVENTIONS

e. Extremities – cold, mottling,

numbness
f. Coronary arteries – chest pain,
arrhythmias
g. Bowel – pain, tenderness and
decrease bowel sound.