Chapter 32: Hemophilia (Children)

Question 1

Hemophilia

Answer 1

caused by a deficiency or absence of factor VIII (hemophilia A) or factory IX (hemophilia B) and plasma proteins required for normal blood clotting

Question 2

How can this happen in the child?

Answer 2

this group of bleeding disorders is inherited sex-link recessive
-the mother is the carrier of the X-linked deficiency, and the sons are those affected with the disorder

Question 3

The coagulation process in children with hemophilia

Answer 3

the coagulation process cannot be completed, so bleeding is prolonged
-the prolonged bleeding is what causes the clinical manifestations to be evident

Question 4

How is Hemophilia classified?

Answer 4

• mild, moderate, or severe based on the degree of deficiency present
• the child with a greater degree of factor deficiency experiences more bleeding episodes than the child with mild deficiency

Question 5

Signs and Symptoms

Answer 5

-bruising
-excessive bleeding (circumcision, tooth loss)
-oozing after a circumcision
-intracranial hemorrhage in the neonate as a result of childbirth
-soft tissue bleeding
-swelling or stiffness of joints, especially knees
-decreased range of motion of extremities
-painful joints
>most are free of symptoms until they crawl or walk
>in infancy a bleeding disorder may be discovered at the time of circumcision
>in older children, may occur with a tooth extraction or tooth loss
>for a child with hemophilia who experiences frequent bleeds in the joints, there may be long-term consequences including mobility limitations, bony changes, and crippling deformities

Question 6

Diagnosis

Answer 6

usually diagnosed after presenting with bleeding or there is a known family hx of bleeding disorders
>Diagnostic Tests:
-prothrombin time (PT)
-partial thromboplastin time (PTT)
-most important: direct assay of plasma factor activity level for hemophilia A and B

Question 7

Prothrombin Time (PT)

Answer 7

Range: 10-14 seconds

• significance: measures the extrinsic pathway for bleeding, requires fibrinogen, prothrombin, and factors V, VII, and X
• prolonged times may indicate deficiencies of vitamin k liver factors, malabsorption, and liver disease

Question 8

Partial Thromboplastin Time (PTT)

Answer 8

Range: 22-35 seconds

• Significance: measures the intrinsic pathway for bleeding, requires factors V, VIII, IX, X, XI, and XII and fibrinogen and prothrombin
• prolonged times may indicate a bleeding disorder

Question 9

Seeking Medical Attention`

Answer 9

-children with bleeding disorders often experience hemarthrosis (a bloody effusion within a joint) and soft tissue bleeding
>hemarthrosis= often seek medical attention after a minor injury in which there is swelling, or pain in the affected joint

Question 10

Prevention

Answer 10

is a genetically transmitted condition, therefore, the primary strategy is genetic counseling to decrease the possible transmission of the blood disorder

• patients with hemophilia require education on how to determine if a bleed is present and methods to treat and prevent further episodes
• avoid aspirin and aspirin-containing products, IM injections, and avoidance of activities that may cause injuries
• safety precautions at home
• use safety equipment such as helmets, car seats, and seat belts
• avoidance of bunk beds, ladders, and play toys such as skate boards, trampolines, and other high-risk items
• patients that require factor supplementation are taught how to properly administer them at home safely

Question 11

Things to Avoid When a Child has Hemophilia

Answer 11

• aspirin and aspirin-containing products
• IM injections
• activities that may cause injuries
• bunk beds
• ladders
• play toys such as skateboards, trampolines, and other-high risk items

Question 12

Nursing Care: Interdisciplinary Approach

Answer 12

a collaborative interdisciplinary approach is essential for a child with hemophilia

• caregivers, school nurses etc. must be aware of the condition and taught about emergency care; parents must be notified by whoever is working with the child so proper care can be sought
• nurse initiates prompt treatment of bleeding episodes
• identification of the deficient factor is important to administer the proper replacement factor
• Nurse Priority= patient safety, prevention of additional complications, and promoting wellness and quality of life

Question 13

Number One Nursing Priority when Caring for A Child with Hemophilia

Answer 13

• Safety
• Prevention of additional complications
• Promoting wellness and quality of life

Question 14

Hemophilia: Managed in the Home or Hospital?

Answer 14

• can be safely managed at home by informed and educated family members
• depending on the severity of the bleed, such as a bleed into a joint, head injury, or internal trauma, patients may require close observation and inpatient hospitalization

Question 15

Medical Care

Answer 15

-recombinant factor products are main treatment
>these manufactured clotting factors are genetically engineered, thus reducing the transmission of various infectious diseases

Question 16

Education/ Discharge

Answer 16

• with severe hemophilia, prophylactic doses of recombinant factor products may be administered in the home by caretakers to prevent bleeding episodes
• nurse teaches how to administer by IV
• important step for the adolescents with hemophilia is learning how to self-administer medications; proper administration improves quality of life, decreases hospitalizations, reduces missed days in school, and prevents long-term complications for children with this chronic illness
• for children who have experienced bleeding into the joints, physical therapy may be necessary to preserve and maintain functional status
• in the instance that the child has a soft tissue injury or bleeding into a joint, before seeking medical attention the family initiates supportive measures (e.g. application of pressure to bleeding site, ice, elevation, and rest)
• any trauma to the head or a change in the level of consciousness is a medical emergency

Question 17

How to Administer Replacement Factor Products

Answer 17

1. Prepare the setting: gather supplies, wash hands, and have environment ready for medication administration
2. Medication preparation: mix the factor concentrate with sterile water per instructions
3. Set up: draw contents of the reconstituted drug into a syringe to be ready to administer IV
4. Prepare the child for treatment to be given
5. IV access: insert a butterfly needle into a vein so you can infuse factor medication
6. Injection: infuse the factor concentrate to treat bleeding episode or to administer s prescribed for preventative measures
7. Dispose of sharps and syringe in appropriate containers