Chapter 32: Hemophilia (Children) Flashcards
Hemophilia
caused by a deficiency or absence of factor VIII (hemophilia A) or factory IX (hemophilia B) and plasma proteins required for normal blood clotting
How can this happen in the child?
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
The coagulation process in children with hemophilia
the coagulation process cannot be completed, so bleeding is prolonged
-the prolonged bleeding is what causes the clinical manifestations to be evident
How is Hemophilia classified?
- 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
Signs and Symptoms
-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
Diagnosis
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
Prothrombin Time (PT)
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
Partial Thromboplastin Time (PTT)
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
Seeking Medical Attention`
-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
Prevention
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
Things to Avoid When a Child has Hemophilia
- 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
Nursing Care: Interdisciplinary Approach
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
Number One Nursing Priority when Caring for A Child with Hemophilia
- Safety
- Prevention of additional complications
- Promoting wellness and quality of life
Hemophilia: Managed in the Home or Hospital?
- 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
Medical Care
-recombinant factor products are main treatment
>these manufactured clotting factors are genetically engineered, thus reducing the transmission of various infectious diseases
Education/ Discharge
- 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
How to Administer Replacement Factor Products
- Prepare the setting: gather supplies, wash hands, and have environment ready for medication administration
- Medication preparation: mix the factor concentrate with sterile water per instructions
- Set up: draw contents of the reconstituted drug into a syringe to be ready to administer IV
- Prepare the child for treatment to be given
- IV access: insert a butterfly needle into a vein so you can infuse factor medication
- Injection: infuse the factor concentrate to treat bleeding episode or to administer s prescribed for preventative measures
- Dispose of sharps and syringe in appropriate containers
