@medical_surgical_nursing @child-health_nursing

1. UNIT = I
 HEMATOLOGICAL CONDITIONS:
 CONGENITAL: HAEMOPHILIA

2. INTEGRAL INSTITUTE OF
NURSING SCIENCES &
RESEARCH
TOPIC:HAEMOPHILIA
PRESENTED BY:SALMAN KHAN
LECTURER
(IINSR)
LUCKNOW

3. CONTENTS
 INTRODUCTION
 DEFINITION
 CAUSESE
 CLINICAL MANIFESTATION
 DIAGNOSTIC EVALUATION
 MANAGEMENT: 1. MEDICAL
 2.NURSING

4. INTRODUCTION
 Haemophilia, or hemophilia[6] (from Ancient Greek αἷμα (haîma) 'blood',
and φιλία (philía) 'love of'),[7] is a mostly inherited genetic disorder that impairs the
body's ability to make blood clots, a process needed to stop bleeding.[2][3]
 This results in people bleeding for a longer time after an injury, easy bruising, and an
increased risk of bleeding inside joints or the brain.[1] Those with a mild case of the
disease may have symptoms only after an accident or during surgery.[1]
 Bleeding into a joint can result in permanent damage while bleeding in the brain can
result in long term headaches, seizures, or a decreased level of consciousness.[1]
 Haemophilia A affects about 1 in 5,000–10,000, while haemophilia B affects
about 1 in 40,000, males at birth.As haemophilia A and B are both X-linked
recessive disorders, females are rarely severely affected.Some females with a
nonfunctional gene on one of the X chromosomes may be mildly symptomatic.

5. Definition
 1.Haemophilia is a genetically transmitted blood clotting disorder, caused by deficiency
of coagulation factor VIII(antihemophilic factor) or factor IX (Christmas factor).
 2.haemophilia is an inherited abnormality of blood coagulation characterized by a
tendency of haemorrhage from trauma.it is due to the deficiency of plasma factor VIII,
factor IV and factor XI.

6. CAUSES:
The causes of both hemophilia A and B are
apparently from a genetic form.
 Genetics. Hemophilia A is caused by an inherited or acquired a
genetic mutation that results in dysfunction or deficiency of factor
VIII, or by an acquired inhibitor that binds factor
VIII; Hemophilia B is an X-linked recessive disease caused by an
inherited or acquired mutation in the factor IX gene or by an
acquired factor IX inhibitor.

7. CLINICAL MANIFESTATIONS
 Hemophilia is suggested by a history of hemorrhage disproportionate to trauma or of
spontaneous hemorrhage, or a family history of bleeding problems.
• Spontaneous hemorrhage. Approximately 30-50% of patients with severe hemophilia
present with manifestations of neonatal bleeding (eg, after circumcision); other
neonates may present with severe hematoma and prolonged bleeding from the cord or
umbilical area or at sites of blood draws or immunizations.
• Hematuria. In the genitourinary tract, gross hematuria may occur in as many as 90% of
patients.
• General symptoms. Weakness and orthostasis may occur.
• Musculoskeletal. Tingling, cracking, warmth, pain, stiffness, and refusal to use the
joint among children are common.
• Central nervous system. Headache, stiff neck, vomiting, lethargy, irritability,
and spinal cord syndromes may occur.
• Genitourinary. Symptoms may be painless; there may be hepatic/splenic tenderness
and peritoneal signs.

8. DIAGNOSTIC FINDINGS
 History collection
 Genetic history
 CBC
 Platelet function test

9. Diagnostic Findings
• Chromogenic assay. This assay is considered by some to be more accurate,
as it measures the level of plasma factor VIII activity but it is less widely
available in clinical laboratories in the United States.
• Laboratory studies. Laboratory studies for suspected hemophilia include a
complete blood cell count, coagulation studies, and a factor VIII (FVIII) assay.
• CT scans. Head CT scans without contrast are used to assess for spontaneous
or traumatic intracranial hemorrhage.
• MRI. Perform magnetic resonance imaging (MRI) on the head and spinal
column for further assessment of spontaneous or traumatic hemorrhage; MRI is
also useful in the evaluation of the cartilage, synovium, and joint space.
• Ultrasonography. Ultrasonography is useful in the evaluation of joints affected
by acute or chronic effusions.

10. Conti………………..
• Testing for inhibitors. Laboratory confirmation of a FVIII inhibitor is clinically
important when a bleeding episode is not controlled despite infusion of
adequate amounts of factor concentrate.
• Carrier testing. Screening for carrier status can be performed by measuring
the ratio of FVIII coagulant activity to the concentration of von Willebrand factor
(vWF) antigen; a ratio that is less than 0.7 suggests carrier status.
• Radiography. Radiography for joint assessment is of limited value in acute
hemarthrosis; evidence of chronic degenerative joint disease may be visible on
radiographs in patients who have been untreated or inadequately treated or in
those with recurrent joint hemorrhages.

11. PHARMACOLOGIC MANAGEMENT
• Factor VIII. Factor VIII (FVIII) is the treatment of choice for acute or potential
hemorrhage in hemophilia A; recombinant FVIII concentrate is generally the preferred
source of factor VIII; prophylactic administration of FVIII is often recommended for
pediatric patients with severe disease.
• Antifibrinolytic agents. Antifibrinolytic agents, such as aminocaproic acid and
tranexamic acid, are especially useful for oral mucosal bleeds but are contraindicated as
initial therapies for hemophilia-related hematuria originating from the upper urinary
tract because they can cause obstructive uropathy or anuria.
• Factor IX. Factor IX is the treatment of choice for acute hemorrhage or presumed acute
hemorrhage in hemophilia B. Recombinant factor IX is the preferred source for
replacement therapy.

12. Conti……………………………..
• Coagulation factor VIIa. These agents can activate coagulation factor X to factor Xa as well as
coagulation factor IX to IXa.
• Coagulation factors. FVIII concentrates replace deficient FVIII in patients with hemophilia A,
with the goal of achieving a normal hematologic response to hemorrhage or preventing
hemorrhage; recombinant products should be used initially and subsequently in all newly
diagnosed cases of hemophilia that require factor replacement; agents that bypass FVIII activity in
the clotting cascade (eg, activated FVII) are used in patients with FVIII inhibitors.
• Antihemophilic agents. These agents are used to control bleeding in hemophilia B or FIX
deficiency and to prevent and/or control bleeding in patients with hemophilia A and inhibitors to
FVIII.
• Monoclonal antibodies. Monoclonal antibodies are used to bind to one specific substance in the
body (eg, molecules, antigens); this binding is very versatile and can mimic, block, or cause
changes to enact precise mechanisms (eg, bridging molecules, replacing or activating enzymes or
cofactors, immune system stimulation).
• Vasopressin-related. Desmopressin transiently increases the FVIII plasma level in patients with
mild hemophilia A.

13. NURSING MANAGEMENT
 NURSING ASSESSMENT
 ASSESSMENT IN A CHILD WITH HEMOPHILIA INCLUDE THE FOLLOWING:
• HISTORY. FOR PATIENTS IN WHOM HEMOPHILIA IS SUSPECTED,
INQUIRE ABOUT THE HISTORY OF HEMORRHAGE DISPROPORTIONATE
TO TRAUMA, HISTORY OF SPONTANEOUS HEMORRHAGE, BLEEDING
DISORDERS IN THE FAMILY, AND CONCOMITANT ILLNESS (ESPECIALLY
THOSE ASSOCIATED WITH ACQUIRED HEMOPHILIA, SUCH AS CHRONIC
INFLAMMATORY DISORDERS, AUTOIMMUNE DISEASES, HEMATOLOGIC
MALIGNANCIES, AND ALLERGIC DRUG REACTIONS).
• PHYSICAL EXAMINATION. ASSESS FOR JOINT SWELLING AND ABILITY
TO MOVE AFFECTED LIMB; ASSESS FOR LIMITED ROM,
CONTRACTURES, AND BONY CHANGES IN THE JOINTS WHEN BLEEDING
HAS STOPPED.

14. NURSING DIAGNOSES
 Based on the assessment data, the major nursing diagnoses are:
• Acute pain related to traumatic injury to the muscles.
• Impaired physical injury related pain and discomfort with the onset of bleeding
episodes.
• Compromised family coping related to incorrect and inadequate information or
understanding.
• Risk for bleeding related to decreased concentration of clotting factors circulating in
the blood (factor VIII and factor IX).
• Risk for injury related to decreased clotting factor (VIII or IX).

15. Practice Quiz: Hemophilia
 1. Mr. and Mrs. Smith’s child has hemophilia; which of the following actions
would you instruct them to avoid?
 A. Lowering the injured area.
B. Immobilizing the joint.
C. Applying pressure.
D. Applying cold to the area.
 1. Answer: A. Lowering the injured area.
• Option A: With hemophilia, the injured area must be elevated, not lowered.
• Options B, C, and D: Immobilizing the joint and applying cold or pressure to the area
are appropriate measures to control bleeding.

16. Conti……………
 2. Which of the following laboratory tests is most effective in diagnosing
hemophilia?
 A. Complete blood count (CBC).
B. Bleeding time (BT).
C. Platelet count (PC).
D. Partial thromboplastin time (PTT).
 2. Answer: D. Partial thromboplastin time (PTT).
• Option D: PTT is abnormal in hemophilia. Therefore, this test will be the most helpful
in diagnosing the disorder.
• Option A: The CBC is not affected in hemophilia.
• Options B and C: Bleeding time and platelet count are normal in hemophilia.

17. Conti…………….
 3. A child with known hemophilia A was brought to the emergency room with
complaints of nose bleeding and some bruises in the joints. Which of the following
should the nurse anticipate to be given to the child?
 A. Cyclosporine.
B. Oral iron supplement.
C. Factor VIII.
D. Factor X.
 3. Answer: C. Factor VIII.
• Option C: Hemophilia A, also called factor VIII (FVIII) deficiency or classic
hemophilia, is a genetic disorder caused by missing or defective factor VIII, a clotting
protein. The initial treatment is the administration of factor VIII to replace the missing
factor and decreases the bleeding episode.
• Options A, B, D: These medications are not used in this case.

18. Conti……..
 4. The mother of a child with hemophilia asks the nurse which over-the-counter
medication is suitable for her child’s joint discomfort. The nurse should tell the
mother to purchase:
 A. Aspirin (Acetylsalicylic acid).
B. Naproxen (Naprosyn).
C. Tylenol (Acetaminophen).
D. Advil (Ibuprofen).
 4. Answer: C. Tylenol (acetaminophen).
• Option C: The nurse should recommend acetaminophen for the child’s joint discomfort
because it will have no effect on the bleeding time.
• Options A, B, D: Answers A, C, and D are all nonsteroidal anti-inflammatory
medications that can prolong bleeding time; therefore, they are not suitable for the child
with hem

19. Conti………..
 5. Which of the following disorders results from a deficiency of factor VIII?
 A. Sickle cell disease.
B. Christmas disease.
C. Hemophilia A.
D. Hemophilia B.
 5. Answer: C. Hemophilia A.
• Option C: Hemophilia A results from a deficiency of factor VIII.
• Option A: Sickle cell disease is caused by a defective hemoglobin molecule.
• Options B & D: Christmas disease, also called hemophilia B, results in a factor IX
deficiency.

20. REFRENCES
 Hemophilia Nursing Care Planning and Management Study Guide – Nurseslabs
 Haemophilia – Wikipedia
 Pankaj sharma,Dr. Bhuvanesh Shukla “A text Book of Pediatric Nursing,lotus
publishers,page no.202 to 204.
 Sharma rimple, “A text book essential of pediatric nursing” published by
japee brothers,page no.264 to 266.