Hemophilia Flashcards
Pathophysiology of Hemophilia
A group of X-linked recessive disorders that result in a deficiency in one of the coagulation factors in the blood.
When bleeding occurs, the vessels constrict and a platelet plug forms, but because of the deficient factor the fibrin will not solidify, and thus the bleeding continues
Hemophilia A: A deficient in Factor VIII and Hemophilia B deficient in Factor IX
Hemophilia A tends to be more severe and prevalent
Therapeutic Management
PRIMARY GOAL: PREVENT BLEEDING
- Instruct children to avoid activities with a high potential for injury (football, riding motorcycle, skateboarding)
- Encourage the child to participate in activities with the least amount of contact (swimming, running, tennis)
- If bleeding or injury occurs, factor administration is prescribed
- Factor replacement should be given before any surgeries or other procedures that can lead to bleed (intramuscular injections, dental care)
Risk Factors of Hemophilia
- Acquired immunologic process
- Spontaneous mutation
- Inherited X-linked recessive trait (hemophilia A and B)
Signs and Symptoms of Hemophilia
- Hematomas
- Joint Swelling
- Limited/painful ROM
- Prolonged nosebleeds
- Excessive bleeding from cuts
- Bleeding from gums
- Blood in urine or stool
- Increased PTT and bleeding time
- PT and platelet count normal
- Low factor assay levels
Complications of Hemophilia
- Infection with blood-borne viruses
- Pain, swelling, extreme tenderness, and permanent joint and muscle deformity
- Peripheral neuropathy
- Paresthesia
- Muscle atrophy
- Fractures
- Chronic synovitis
- Ischemia and gangrene
- Hematomas
- Thrombosis
- Shock and death
Nursing Assessment: Health History
- Determine the nature of the bleeding episode or bruise
- Include any hemorrhagic episodes in other systems, such as GI (black, tarry stools, hematemesis) or as a result in joint hemorrhage or hematuria
- Inquire about the length of bleeding and amount of blood loss
Nursing Assessment: Physical Examination
- Assess circulation (heart sounds, pulses if severe prolonged bleeding occurs)
- Note chest pain or abdominal pain
Without intervention, hypovolemia could follow, leading to shock
Lab and Diagnostic
- Decrease hemoglobin and hematocrit if bleeding is prolonged or severe
- Factor levels may be quantified with blood testing
Nursing Management: Preventing Bleeding Episodes
- Teach children and families that regular physical activity or exercise help to keep the muscles and joint stronger and children with stronger joints and muscles have fewer bleeding episodes
- Refer the child with moderate to severe hemophilia to a pediatric hematologist and/or comprehensive hemophilia treatment center
Preventing Bleeding in the Child with Hemophilia
- Protect toddlers with soft helmets, padding of the knees, carpet in the home, and softened or covered corners
- Children should stay active: swimming, baseball, basketball and bicycling (wearing a helmet) are good physical activities
- Avoid intense contact sports such as football, wrestling, soccer and high diving
- Avoid trampoline use and riding ATVs
- Arrange premedication with Amicar if oral surgery is indicated
Nursing Management: Managing a Bleeding Episode
- Administer factor VIII replacement as prescribed
- Educate that transmission of viruses via the product is not a risk
- Administer factor replacement slowly by IV push
- Document the product name, number of units, lot number and expiration date
- If external bleeding occurs, apply pressure to the area until bleeding stops
- If bleeding is inside the joint, apply ice or cold compress to the area and elevate any injured extremities
- Make sure all cases of bleeding are followed up to identify whether factor replacement is necessary
Nursing Assessment: Providing Education
- Child should wear medical alert bracelet
- Notify school nurse and teachers of child’s diagnosis
- Instruct all school personnel to call parents immediately if the child sustains a head, abdominal or orbit injury at school
- Teach parents and caregiver how to administer IV factor VIII
- Involve children as developmentally appropriate in the infusion process
> Young child hold and apply band-aid
> Older children may assist with dilution and mixing of the factor
> Teach teenagers to administer their own factor infusions - Teach the family access, care and flushing of the implanted port
Nursing Management: Providing Support
- Reassure parents that since factor replacement began to be treated there have been no reports of HIV transmission from factor infusion
- Educate and support parents
- Refer families to NHF which offer support, education, youth leadership, scholarships and a directory for children with hemophilia and other bleeding disorders
