child health nursing

1. Hemophilia
Presented by,
Mrs. Arifa T N
Child Health Nursing
Second year M.Sc Nursing
MIMS CON

2. Introduction
• The term hemophilia refers to a group of bleeding disorders resulting
from congenital deficiency or dysfunction or absence of specific coagulation
proteins or factors.
• Mainly two classification A and B.
• Other classification
▫ Hemophilia A, B and C
▫ Hemophilia A, B and Von Willebrand disease (vWD)
• Symptomatology is similar regardless of which clotting factor is deficient.

3. Definition
Hemophilia refers to a group of hereditary bleeding disorders that
result from a deficiency in specific clotting factors.
Hemophilia is not one disease but rather one of a group of
inherited bleeding disorders that cause abnormal or exaggerated
bleeding and poor blood clotting.
The term is most commonly used to refer to two specific conditions
known as hemophilia A and hemophilia B.

4. Classification
• Hemophilia A, or classic hemophilia:
▫ Factor VIII deficiency
▫ Prevalence is approximately 1 in 5000
• Hemophilia B, or Christmas disease
▫ Factor IX deficiency
▫ 1 in 20,000 to 30,000 live births
• Von Willebrand disease (vWD)
▫ Another hereditary bleeding disorder characterized by a
deficiency, abnormality, or absence of the protein called von
Willebrand factor (vWF).

5. Hemophilia A (Classical Hemophilia)
▫ It results from deficiency of factor VIII, the antihemophilic factor
(AHF).
▫ It is X-linked recessive, occurring almost exclusively in the males*.
▫ The females act as the carriers without manifesting the disease.
▫ Hemophilia A accounts for 98% of all the hemophilics.
▫ The incidence in the population is 1 in 10 thousands

6. Hemophilia B (Christmas Disease)
▫ It results from deficiency of factor IX, the plasma thromboplastin
component (PTC).
▫ Hemophilia B accounts about 10% to 15%
Hemophilia C
▫ It results from deficiency of factor XI, the plasma thromboplastin
antecedent (PTA).

7. Mode of transmission
• Even though it is X-linked recessive, one third of hemophilia
may be caused by gene mutation.
• Usual pattern is a cross between an unaffected male and trait
carrier female
• The chances of its occurrence are 25% or 1:4

10. Etiology
• Hemophilias A and B are X-linked recessive disorders, which manifest almost
exclusively as affected males and carrier females.
• Genes for clotting factors VIII and IX are located near the terminal
long arm of the X chromosome
• A daughter will inherit the gene from her father with hemophilia and may
transmit it to her sons
• Some children affected by hemophilia do not have a family member with a
history of a clotting disorder.
• In these cases, the disorder is caused by a new mutation
• The degree of bleeding is related to the amount of clotting factor and
the severity of the injury

11. Pathophysiology

12. Pathophysiology
• The basic defect of hemophilia A is a deficiency of factor VIII
(antihemophilic factor [AHF]).
• Factor VIII is produced by the liver and is necessary for the formation of
thromboplastin in phase of blood coagulation
• The less factor VIII that is found in the blood, the more severe the
disease.
• Individuals with hemophilia have two of the three factors required for
coagulation: Vascular influence and platelets. Therefore, they may bleed for
longer periods but not at a faster rate.

13. Clinical Manifestations
• Prolonged bleeding anywhere from or in the body
• Hemorrhage from any trauma: Loss of deciduous teeth,
circumcision, cuts, epistaxis, injections
• Excessive bruising, even from a slight injury, such as a fall
• Subcutaneous and intramuscular (IM) hemorrhages
• Hemarthrosis (bleeding into the joint cavities), especially the knees,
ankles, and elbows hematomas

14. Clinical Manifestations
• Pain, swelling, and limited motion
• Spontaneous hematuria
• Intracranial bleeding occurs in 10% of patients with severe
hemophilia and has a mortality rate of 30%.
• Females who carry the trait for hemophilia do not usually
manifest symptoms of the disease

15. Diagnosis
• Diagnosis of affected children and carriers can be done before birth through
chorionic villus sampling or amniocentesis
• Genetic testing of family members is increasingly being used to identify
carriers
• History
• Physical examination
• laboratory data.
▫ Low levels of factor VIII or IX, and prolonged activated partial
thromboplastin time (aPPT).
▫ Prothrombin time (PT), thrombin time (TT), fibrinogen, and platelet
count are normal.

16. Management
• The goal of medical management is to control bleeding by replacing
the missing clotting factor.
• Desmopressin (DDAVP),
▫ An analog of vasopressin, stimulates the release of factor VIII stored in the
blood vessels, thereby increasing the percentage of available factor by
approximately threefold.
▫ DDAVP is effective in some patients with mild and moderate hemophilia A.
• Many recombinant factor VIII concentrates are available as
replacement therapy for hemophilia A
• Factor IX concentrates are also available to treat hemophilia B

17. • The child with severe hemophilia may be on a prophylactic
regimen of replacement therapy, whereas the child with mild to
moderate hemophilia may only receive episodic therapy.
• Prophylaxis decreases bleeding episodes and joint damage that may
result from repeated episodes of hemarthrosis
• Prompt and adequate treatment is needed to prevent serious bleeding
episodes and their sequelae.
• Gene therapy is being explored for treatment of hemophilia in
hopes that an eventual cure will be found

18. Treatment in detail………..
• The products available are factor VIII concentrates, either
produced through genetically engineering (recombinant form) or
derived from pooled plasma, which are reconstituted with sterile
water immediately before use.
• A synthetic form of vasopressin, 1-deamino-8-d-arginine
vasopressin (DDAVP), increases plasma factor VIII activity and is
the treatment of choice in mild hemophilia and vWD types I and II A
only if the child shows an appropriate response.

19. • After DDAVP administration, a threefold to fourfold rise in factor VIII
level activity should occur.
• It is not effective in the treatment of severe hemophilia A, severe
vWD, or any form of hemophilia B.
• Aggressive factor concentrate replacement therapy is initiated to
prevent chronic crippling effects from joint bleeding

20. Other drugs therapy,
• Corticosteroids are given for hematuria, acute hemarthrosis, and chronic
synovitis
• Nonsteroidal antiinflammatory drugs (NSAIDs), such as ibuprofen, are
effective in relieving pain
• Oral administration of ε-aminocaproic acid (Amicar) prevents clot
destruction. Its use is limited to mouth trauma or surgery with a dose of factor
concentrate given first.

21. • Regular program of exercise and physical therapy. Regular program of
exercise and physical therapy
• Treatment without delay results in more rapid recovery and a decreased
likelihood of complications
• Most children are treated at home
• The family is taught the technique of venipuncture and to administer the AHF to
children older than 2 to 3 years old. The child learns the procedure for self-
administration at 8 to 12 years old.

23. Prophylactic therapy
• Prophylactic therapy is periodic factor replacement for children with severe
hemophilia to prevent bleeding complications, including arthropathy and
spontaneous life-threatening bleeding events
• Primary prophylaxis involves the infusion of factor VIII concentrate on a
regular basis before the onset of joint damage.
• Secondary prophylaxis involves the infusion of factor VIII concentrate on a
regular basis after the child experiences his or her first joint bleed..

24. • The administration of infusions differs among treatment centers and may
range from every other day to three times a week for several weeks to
promote healing.
• On-demand factor replacement may be a cost-effective alternative to
primary prophylaxis, but prophylaxis decreases the development of joint
disease and preserves joint function compared with on-demand factor
replacement treatment
• Prompt appropriate treatment of hemorrhage and prophylactic therapy are key
to excellent care and prevention of long-term morbidity in patients with
hemophilia.

25. Nursing management

26. Nursing Assessment and Diagnosis
• Physiologic Assessment
▫ Obtain a complete medical history from the parents or child
▫ Previous episodes of bleeding
▫ The occurrence of hemophilia or any other bleeding disorders in family members.
▫ Assess the child for any joint pain, swelling, or permanent deformity, particularly
around the knees, elbows, ankles, and shoulders.
▫ Observe for prolonged bleeding or oozing of blood. Note the presence of hematuria
and mild flank pain
▫ Conduct a neurologic assessment because the risk for intracranial hemorrhage and
bleeding can lead to peripheral neuropathies

27. • Psychosocial Assessment
▫ Difficulty for families to manage care of the child with hemophilia, especially if the
disease is severe.
▫ Assess the family’s coping mechanisms and support systems.
▫ Determine the family’s ability to manage procedures and treatments; the factor
concentrates and infusion equipment are costly.
▫ Assess older children’s understanding of the disease, limitations, and their
adaptation to the disease.

28. • Developmental Assessment
▫ Physical activity restrictions
▫ Physical skills may be delayed.
▫ Perform frequent developmental assessments, being particularly attentive to fine
and gross motor skills.

29. Nursing diagnosis
• Acute Pain related to bleeding episodes
• Impaired physical mobility related to joint stiffness or
contractures
• Impaired Home Maintenance related to challenges o f hemophilia
• Interrupted Family Processes related to family role shift required
to care for a child with a chronic illness
• Risk for Injury related to bleeding disorder
• Risk for delayed growth and development
As per child’s condition……..

30. Planning and Implementation
• Nursing care focuses on
▫ Preventing and controlling bleeding episodes,
▫ Limiting joint involvement and managing pain, and
▫ Providing emotional support.
*Both short-term interventions and long-term management are necessary

31. Prevent and Control Bleeding Episodes
• Advise to parents for the need of close supervision and a safe
environment.
• Parents should encourage children to play with safe, age-appropriate toys.
• When the child is hospitalized, use nursing approaches to minimize the
possibility of bleeding.
• Ensure that the hospital environment is safe by orienting the child to the
room and keeping the floor and room clear of hazards as much as
possible.

32. Prevent and Control Bleeding Episodes
• If significant bleeding does occur, offer supportive measures and assist with
factor replacement therapy.
• Carefully monitor the child’s condition for any side effects when factor
replacement therapy is administered.
• Control any superficial bleeding by applying pressure to the area for at
least 15 minutes.
• Immobilize and elevate the affected area, and apply ice packs to
promote vasoconstriction

33. Tips to control bleeding***
• Avoid taking temperatures rectally or giving suppositories.
• Avoid intramuscular injections unless absolutely necessary and only after factor
replacement has been given.
• Children should receive recommended immunizations subcutaneously with firm
pressure to the injection site for 5 minutes following the injection.
• Apply firm, continuous pressure to venipuncture sites 5 minutes after any
venipuncture procedure.
• Do not give aspirin or aspirin-containing products

34. Limit Joint Involvement and Manage Pain
• Administration of factor replacement as quickly as possible
• Elevating and immobilizing the joint and applying ice packs
• Administering analgesics for pain.
• Once bleeding has been Controlled, range-of-motion exercises strengthen
muscles and Joints and prevent flexion contractures.
• Physical therapy may be needed.
• Encourage the child to maintain an appropriate weight, because excessive
weight can place added stress on joints.

35. Provide Emotional Support
• Comprehensive team approach.
• Refer the parents for genetic counseling as soon as possible after diagnosis.
• It is important to identify family members who carry the trait because they
may suffer excessive bleeding during surgery.
• Encourage the parents to verbalize their feelings.
• Be understanding and sensitive to their needs.
• Teach the parents about hemophilia and explain how the disorder affects both
the child and other family members.
• Refer the parents and child to organizations such as the National Hemophilia
Foundation for further information.

36. Discharge Planning and Home Care Teaching
• Most care will subsequently take place in the home.
• Home care needs should be identified and addressed well in advance of
discharge.
• Advise parents to have the child wear a medical identification bracelet.
• Dentists and all other healthcare providers should be aware of the
diagnosis.
• Explain the cause of bleeding so both the child and parents understand the
disease process.

37. Discharge Planning and Home Care Teaching
• Teach the child and family how to identify internal bleeding.
• Signs and symptoms such as joint pain, abdominal pain, and obvious
bleeding are indicators for immediate factor infusion.
• Make sure the child and parents know what situations could cause
bleeding to occur.
• **Teach parents to give acetaminophen instead of aspirin and
aspirin-containing products

38. Discharge Planning and Home Care Teaching
• Instruct the parents and the child, when appropriate, to prepare and
administer factor concentrate.
• If infusion of the missing factor is scheduled regularly, bleeding episodes can be
controlled or avoided.
• Have the parents demonstrate the procedure and make sure they can
administer the product correctly.
• Ensure that parents know where they can get the factor concentrate.

39. Discharge Planning and Home Care Teaching
• The child will need an individualized school health plan
• Members of the school staff should be instructed in management of
emergencies.
• The nurse can identify key staff members in the school and teach them
the actions that need to be taken
• Help the family and school in planning an appropriate schedule of activities for
the child.
• Provide ongoing case management, assisting the family to take on this task if
able.

40. Discharge Planning and Home Care Teaching
• Identify Family’s resources (financial burden)
• Refer families to appropriate social services
• Encourage sharing experiences with other families of children with hemophilia
can provide support.

41. Tips
• ***Use the acronym RICE (rest, ice, compression, elevation) to help you
remember important measures to control a bleeding episode.

42. Evaluation
• Injury to the child is prevented.
• Pain is managed to promote comfort level.
• The nurse and family promote normal growth and development for the child.
• The child and family have adequate knowledge of disease management,
including recognition of bleeding and prompt initiation of infusion