The coagulation cascade, or secondary hemostasis, is a series of steps in response

to bleeding caused by tissue injury, where each step activates the next and
ultimately produces a blood clot. The term hemostasis is derived from “hem-”,
which means “blood”, and “-stasis”, which means “to stop.” Therefore,
hemostasis means to stop bleeding. There are two phases of hemostasis.
First, primary hemostasis forms an unstable platelet plug at the site of injury.
Then, the coagulation cascade is activated to stabilize the plug, stopping blood
flow and allowing increased time to make necessary repairs. This process
minimizes blood loss after injuries.

The coagulation cascade involves the activation of a series of clotting factors,

which are proteins that are involved in blood clotting. Each clotting factor is a
serine protease, an enzyme that speeds up the breakdown of another protein.
The clotting factors are initially in an inactive form called zymogens. When placed
with its glycoprotein co-factor, the clotting factor is activated and is then able to
catalyze the next reaction. When a clotting factor becomes activated, it is denoted
with an “a” following its respective Roman numeral (e.g. when activated, Factor V
becomes Factor Va).

Coagulation consists of three pathways, the extrinsic, intrinsic, and common

pathways, that interact together to form a stable blood clot. The extrinsic and
intrinsic coagulation pathways both lead into the final common pathway by
independently activating factor X. The extrinsic pathway involves initiation by
factor III (i.e., tissue factor) and its interaction with factor VII. Whereas, factors XII,
XI, IX, and VIII are utilized in the intrinsic pathway. Then, the common pathway
uses factors X, V, II, I, and XIII.

The extrinsic pathway begins when there is injury to the endothelial tissue (i.e.,
skin tissue), exposing tissue factor (factor III) to the blood. Tissue factor then
becomes bound with calcium and factor VIIa to activate factor X. Factor VII is
present in the blood and requires vitamin K to be activated.

Meanwhile, the intrinsic pathway begins when factor XII or the Hageman factor is
exposed to collagen, kallikrein, and high molecular weight kininogen (HMWK) and
is subsequently activated. Factor XIIa activates factor XI into XIa. With a calcium
ion, factor XIa activates factor IX. Then, factor IXa, factor VIIIa, and calcium form a
complex to activate factor X. Factor VIII is found in the blood and is often
activated by thrombin (factor IIa).

The common pathway may result after the activation of factor X at the end of
either pathway. The common pathway begins when factor Xa, Va, and calcium
bind together, forming a prothrombinase complex. The prothrombinase complex
then activates prothrombin (factor II) into thrombin (factor IIa). Next, thrombin
cleaves fibrinogen (factor I) into fibrin (factor Ia). Afterwards, thrombin cleaves
the stabilizing factor (factor XIII) into XIIIa. Factor XIIIa binds with calcium to then
create fibrin crosslinks to stabilize the clot. Thrombin has several functions,
including activating platelets (cell fragments involved in clot formation) and
activating factors V, VIII, and IX.
Hemophilia

Hemophilia are the most common hereditary clotting defects, occurring as x-

linked recessive disorders. This bleeding disorder occurs due to deficiency of
plasma coagulation factors. It is primarily found in males and transferred by
female carriers. Affected male may pass the gene to female offspring making
them carrier. Female baby may be affected rarely only when the female carrier
bears the child with a male hemophiliac partner.

Classification :

Hemophilia is classified In 3 groups based on factors of coagulation.

A. Hemophilia A (classical hemophilia) – it occurs due to deficiency of plasma

factor VIII (antihemophilic factor). It accounts for 80 to 85 percent of all
hemophilics.
B. Hemophilia B (Christmas hemophilia) – it results from deficiency of plasma
factor IX (plasma thromboplastin component). It accounts for 15 to 20
percent of cases.
C. Hemophilia C –It results from deficiency of factor XI( plasma
thromboplastin antedecent). It accounts for few cases only.

Hemophilia A can be classified based upon the factor VIII level in plasma.they are
as follows:

a) Severe hemophilia – in this type,factor level is found less than 1% of normal

value. Patients have spontaneous and severe bleeding.
b) Moderate hemophilia – factors level remain between 1 to 5 % of normal.
Patients have no spontaneous bleeding and may not have severe bleeding
until any trauma occurs.
c) Mild hemophilia – factor level is in between 6 to 30% of normal.patient
may lead normal life. Bleeding may occur in severe injury and surgical
interventions.

Pathophysiology:
 Hemophilia is concerned with absence or decrease functions of blood
coagulation factors leading to excessive prolonged or delayed
bleeding.
  The basic defect is in intrinsic and extrinsic phase of coagulation
process.
 The blood clotting factors are essential for the formation of
prothrombin activators which converts prothrombin to thrombin.
 The rate of thrombin formation is almost directly proportional to the
amount of prothrombin activator available.
 The rate of clotting is proportional to the amount of formed
thrombin.
 Clinical manifestations depend upon plasma level involved deficient
coagulation factors.

Probability of transmission of hemophilia:

Mother’s Father’s Female child Male child

genotype genotype
Normal Carrier Hemophilic Normal Hemophilic
Carrier Normal 50% 50% 0 50% 50%
Non Hemophilic 0 100% 0 100% 0
carrier
Carrier Hemophilic 0 50% 50% 50% 50%

Clinical manifestations:

 Child with mild hemophilia may be usually asymptomatic. Bleeding may

occur after a severe injury or surgery.
 In severe hemophilia excessive bleeding may occur from neonates from
umbilical cord. But usually the diagnosis is made after the child becomes
active.
 The child may present with easy bruising, spontaneous soft tissue
hematomas, prolonged bleeding from lacerations in the nasal mucosa or
oral cavity.
  The important cause of hospitalization is hemarthrosis (hemorrhages in
joint) especially in elbow, knee or ankles. It can also come associated with
pain, swelling and limited movements.
 The child may have spontaneous hematuria, GI bleeding , intracranial
hemorrhage. But petechial do not occur in hemophilia.

Diagnostic evaluation:
1. Blood tests shows findings such as
 Prolonged clotting time and partial thromboplastin time.
 Reduced protrombin consumption
 Increased thromboplastin level
 Bleeding time and prothrombin time are normal
 Assay of specific clotting factors are indicated deficient level.
2. X-ray if the affected joints helps to defect severity and complications
of hemarthrosis
3. Gene analysis of antenatal detection of hemophilia.
4. Detection of carrier of hemophilia and DNA studies.

Management

Specific management include the following:

1. Replacing the missing coagulation factors is the important aspect of

management
 Factor VIII made from cryoprecipitate or factor VIII concentrate is
transfused in hemophilia A
 Factor IX made from fresh frozen plasma is administered for
hemophilia B
 Fresh frozen plasma is given to supply factor XI.
2. Mild to moderate factor VIII deficient may respond to desmopressin which
causes release of factor VIII from the endothelial stores.
3. Fresh whole blood transfusion can be given if commercially prepared
coagulation factors are not available.
 4. Antifibrinolytics such as tranxemic acid are given for mucosal bleeding to
prevent clot break down by salivary proteins and to prevent hemostasis.
5. Supportive management in case of hemarthosis may include rest,
immobilization, application of ice pack and pressure bandage with local
application of thrombin powder or foam. Pain can be relieved by
anaelgesics like paracetamol or NSAIDs. Aspirin,indomethacin are not used
as they inhibit platelets functions and promote bleeding. Steroids can be
used in cases of hemarhtrosis.
Ambulation and exercise can be allowed after local phase is over. Later
local heat,physiotherapy ahould be given to prevent ankylosis of the joint.
6. Synovectomy can be done to remove damaged synovium in chronically
involved joints.
7. Orthotics can be used to prevent injury to affected joint and help to resolve
hemorrhages.

Nursing management

1. Nursing assessment:
 Detail history of illness
 Family history
 History of prolonged bleeding after injury or surgery
 Thorough physical examination to be done to rule out bruises
bleeding,hemarthrosis,pain and swelling.
2. Nursing diagnosis:
 Risk for volume deficit related to hemorrhage
 Pain related to bleeding joints
 Potential for bleeding related to deficiency of clotting factors
 Impaired physical mobility related to hemarthrosis
 Ineffective family coping related to life – threatening illness
3. Nursing interventions
I. Assessing the child in frequent intervals and recording the findings.
II. Preventing hypovolemia by following measures:
 Applying pressure and cold on the area for 10 to 15 minutes even after
injection and venipuncture.
  Applying absorbable gelatin or fibrinolytic foam on wound or oral bleeding.
 Avoiding suturing or cauterization during surgery.
 Immobilizing and elevating the affected part.
 Providing rest, comfort and quiet environment .
 Monitoring vital signs and signs of shock.
III. Administering drugs as prescribed with necessary precautions. Missing
factors or blood should be given slowly to minimize transfusion reaction ( 2-
3ml/min).
IV. Preserving joint mobility by treating hemarthrosis with prescribed drugs,
immobilization in a slight flexion position, applying cold and following other
instructions of supportive care. Affected joint can be treated with plaster
cast if necessary. Preventing weight bearing on affected joint.
V. Providing protection against bleeding and injury.
VI. Teaching the parent and family members about home care, safety
measures, regular follow- up, continuation of school education, avoidance
of injury and overweight
VII. Providing emotional support to cope with the problem and involving
parents in routine care of the child.

Complications :

 Airway obstruction caused by bleeding into neck and pharynx

 Intracranial bleeding may lead to neurological impairment
 Intestinal obstruction due to GI bleeding
 Compartment syndrome due to compression of nerves by bleeding into
deep tissues
 Degenerative joint disease,osteoporosis and muscle atrophy
 Chronic hepatitis and cirrhosis due to contaminated cryoprecipitate
 Physical,psychological and social handicaps.