Hemophilia

All of the above

The nurse is conducting staff in-service on Von-willebrand's disease. Which should the nurse include as characteristics of von williebrand's disease? Select all that apply: 1. easy bruising occurs 2. gum bleeding occurs 3.it is a hereditary bleeding disorder 4. treatment and care are similar to that for hemophilia 5. it is characterized by extremely high creatnine levels. 6. The disorder cause platelets to adere to damaged endothelium

1,2,5

A child with hemophilia A fell and injured a knee while playing outside. The knee is swollen adn painful. WHich of the following measures should be taken to stop the bleeding? Select all of the following. 1. the extremity shoudl be immoblized 2. the extremity should be elevsted 3. warm compress should be applied to decrese pain 4. passive range of motion excercise should be administred to the extremity 5. factor 8 should be administered.

Place the child on bleeding precautions and monitor for bleeding. monitor vs and joint pain, joint bleeding should be controlled by immobilization, elevation, and application of ice. apply pressure for 15 minutes for any superficial bleeding. Monitor loc for brain bleed, monitor urine for heaturia.

A child with hemophilia who has been in a motor vehicle crash is admitted to the pediatric unit. what should the nurse do in care of this child?

A,B,C,D

A nurse is caring for a child with von williebrand disease. The nurse is aware that that which of the following is a/are clincial manifestations of von williebrand disease? A. Bleeding of the mucous membranes B. The child brusies easily C. excessive menustration D. the child has frequent nosebleeds E. elevated creatnine levels F. the child has a factor IX deficiency

Von Williebrand disease

Characterized by epitaxis, gum bleeding, easy bruising, and menstural bleeding

moderate

Classification of hemo A: bleeding with trauma

B,C,D Avoideance of IM injections, acetaminophen( tylenol) for mild pain control, soft tooth for dental hygiene

The nurse is planning care for a school-age child admitted to the hospital with hemophilia. Which interventions should the nurse plan to implement for this child? (Select all that apply.) a. Finger sticks for blood work instead of venipunctures b. Avoidance of IM injections c. Acetaminophen (Tylenol) for mild pain control d. Soft tooth brush for dental hygiene e. Administration of packed red blood cells

B,D,E

Parents of a school-age child with hemophilia ask the nurse, "Which sports are recommended for children with hemophilia?" Which sports should the nurse recommend? (Select all that apply.) a. Soccer b. Swimming c. Basketball d. Golf e. Bowling

true

Pt with hemophilia will have a prolonged ptt

rest, ice, compression and elevation

RICE

hemo A

Rare for a female to have this type of hemophilia

severe hemophilia

Spontaneous bleeding/ umbilical cord or circumcision

half of activity is gone in 8-12 hours, give it daily.

DDVAP factor 8 deficient mild to moderate

prolonged PTT

DX hemophilia: history ( family) lab findings??

Can affect girls and boys equally

Difference in Von Williebrand Disease and the other hemophilias.

tightness with no real pain

Hemarthrosis

Classic hemophilia

Hemophilia A also known as

hemophilia A

Hemophilia deficient in factor *

Hemophilia B

Hemophilia deficient in factor 9

Von Willebrand Disease

Hereditary bleeding disorder in males females

hemophilia type A

Most common hemophilia

prophylaxis, during acute crisis( hemarthosis)

Name 2 situation for factor replacement

3. swimming

The nurse is providing home care instructions for the parents of a 10 year old child with hemophilia. which sport activity should the nurse suggests for this child? 1. soccer 2.basketbll 3.swimming 4. field hockey

2. avoid contact sports

The nurse is teaching a client with hemophilia A about home management. Which strategy should the nurse incluse in the teaching plan? 1. increase iron rich foods 2. avoid contact sports 3. use aspirin when severe pain occurs 4. minimize joint pain by walking and weight-bearing

4

Which of the following measures shoudl the nurse teach the parent of a child with hemophilia to do if the child sustains an injury to a oint causing bleeding? 1. give the child a dose of tylenol 2. immobilize the joint, and elevate the extremity 3. apply heat to the area 4. administer factor per the home-care protocol

hemo A

X-linked recessive disorder

hemarthrosis

redness, edema, hot to touch are all symptoms of

demand therapy

replacement therapy used for active bleeding

seizure patients

risk for injury

use a disposable sponge

severe hemophiliac patient dental care would need to do what?

desmopressin( DDAVP)

synthetic form of vasopressin for mild of moderate. stimulates release of factor VIII

liver

where is AHF produced

female

50% carrier 50% fine with no problems Hemo A

rest, ice, compression, elevation

What does RICE stand for?

soft tooth brush, soak in warm water b4 brushing or use a water pick

What should be done for dental hygiene in the patient with hemophilia?

Rice therapy

What should be done in acute stages of hemarthrosis

elevate, and immobilze joint.

What should be done in the early stages of hemarthrosis?

1,2,3,4

Which of the following activites should a nurse suggest for a client diagnosed with hemophilia? select all that apply 1. swimming 2. golf 3. hiking 4. fishing 5. soccer

1. Care should be taken to avoid bleeding of gums, and softening of toothbrush in warm water before brushing or using a sponge-tipped disposable toothbrush may be helpful. 2. signs of internal bleeding should be recognized, such as headache, slurred speech, and black tarry stools. 3. all of my sons teachers need to be aware of what to do if he gets a bloody nose. ALL OF THE ABOVE

You are discharging a patient with hempphilia. Which of the following responses by the parents indicate an understanding of this disorder?

hemophilia B

a disease also known as the christmas disease

hemophilia A

also known as classic hemophilia

one she wants us to be responsibel for

carrier mother with a noral father

Von Williebrand Disease

characterized by increase tendency to bleed from mucous mebranes.

mild

classification of hemo A: bleeding with severe surgery or trauma

severe

classification of hemo A; Spontaneous bleeding( 60-70%) of cases

keep clotting factors enough so bleeding doesnt occur

goal of replacement therapy

false

high level of AHF will have more bleeding tue or false

lean forward, stay calm, apply bridge of nose pressure, apply ice ot bridge for 2 minutes

hemo pt with epitaxis, what teaching should be done?

AHF- anti hemophillic factor

hemophilia A is deficient in factor 8 which is what?

unaffected male and trait carrier female

most common transmission of hmophilia A

after the acute phase

Active ROM should be done when?

bruising

Prolonged ________ can be an early sign of hemophilia

circumcision, umbilical cord bleeding, hep B vaccine.

At birth how may one know they have hemophilia

NO

Can desmopressin be administered for severe cases of hemo?

factor 8

hemo A needs what replacement

factor 9 replacement

hemo b needs what replacement

male

50% normal or 50% they have it hemo A

3. IV infusion of factor VIII

A 10 year old with hemophiia A has slipped on the ice and bumped his knee. The nurse should prepare to administer which prescription? 1. injection of x factor 2. IV infusion of iron 3. iv infusion of factor vIII 4. IM injection of iron using the Z-track method

C- X-linked recessive inherited disorder in which blood-clotting factor is deficient

Parents of a hemophiliac child ask the nurse, "Can you describe hemophilia to us?" Which response by the nurse is descriptive of most cases of hemophilia? a. Autosomal dominant disorder causing deficiency in a factor involved in the blood-clotting reaction b. X-linked recessive inherited disorder causing deficiency of platelets and prolonged bleeding c. X-linked recessive inherited disorder in which a blood-clotting factor is deficient d. Y-linked recessive inherited disorder in which the red blood cells become moon-shaped

4. PTT

The nurse analyzes the labratory results of a child with hemophilia. The nurse understands that which result will most likely be abnormal in this child? 1. platelet count 2. hematocrit level 3. hemoglobin level 4. partial thromboplastin time

1

Which of the following measures should be implemented for a child with von willebrand disease who has a nosebleed? 1. apply pressure to the nose for at least 10 minutes 2. have the child lie supine and quiet 3. avoid packing the nostrils 4. encourage the child to swallow frequently

1,2,3,4

Which of the following will be abnormal in a chil with diagnosis of hemophilia? 1. platelet count 2. HgB level 3. white blood cell count 4. partial thromboplastin time

being overweight will increase pressure on the joints

Why should the hemophilia pt be on dietary restrcitions?

intracranial bleed

headache, vomitting, change in level of conciousness, could all be early signs of what in the hemophiliac patient

2-3 times a week

preventative therapy of factor infusion

hemarthrosis

usually the first symptom of hemophilia

females

very rare for _____ to have hemophila

factor 8 concentrate

what can be infused for a hemo A patient?

aspirin, ibuprofen, and other NAIDS.

what drugs need it be avoided with hemo?

blood clotting- formation of thromboplatin

what is anti hemophillic factor important for

golf, band, swimming, cross country

what sports can a hemophiliac participate in??

true

when a child looses their first tooth may be when a parent learns they have mild hemophilia t/f