Week 11 - Hemophilia

Question 1

what is hemophilia

Answer 1

• an X-linked recessive genetic disorder caused by defective or deficient coagulation factor

Question 2

what are manifestations of hemophilia (10)

Answer 2

• slow, persistent, prolonged bleeding from minor trauma & small cutes
• delayed bleeding after minor injuries (several hrs-days)
• uncontrollable hemorrhage after dental extractions or irritation to the gums w a hard bristles toothbrush
• epistaxis
• GI bleeding from ulcers & gastritis
• hematuria from GU trauma after falls
• neuro signs
• bruising
• subcut hematomas
• hemarthrosis

all relate to bleeding

Question 3

what is hemarthrosis

Answer 3

• bleeding into joints

Question 4

why do neuro signs occur w hemophilia? what are some? (3)

Answer 4

• d/t nerve compression from hematoma formation
• pain
• anasthesia
• paralysis

Question 5

what is a risk associated w hemophilia

Answer 5

• life-threatening hemorrhage

Question 6

what is included in diagnostic studies for hemophilia (6)

Answer 6

• lab studies
• PT time
• plts
• bleeding time
• thrombin time
• factor assays

Question 6

what is included in diagnostic studies for hemophilia (6)

Answer 6

• lab studies
• PT time
• plts
• bleeding time
• thrombin time
• factor assays

Question 7

what is the goal of collaborative care for hemophilia

Answer 7

• prevent & treat bleeding

Question 8

what is included in acute intervention of hemophilia r/t controlling bleeding (6)

Answer 8

• apply direct pressure or ice to site
• pack area w gelfoam or fibrin foam (hemostatic agents)
• admin of specific coagulation factor
• interventions for joint bleeding
• prevent or treat airway obstruction r/t hemorrhage into the neck or pharynx
• assess & treat intracranial bleeding

Question 9

what are nursing interventions for joint bleeding (4)

Answer 9

• complete rest to prevent permanent damage (ROM after bleeding ceases)
• admin replacement factors
• pack w ice
• analgesics (no aspirin !)

Question 10

why is it imp to rest the joint w joint bleeding r/t hemophilia

Answer 10

• to prevent premanent damage

Question 11

what is treatment for hemophilia (3)

Answer 11

• clotting factor replacement therapy **primary means
• DDAVP (desmopressin) –> minor bleeding and dental procedures
• antifibrinolytic therapy –> stabilizes the clot

Question 12

when can clotting factor replacement theray be used/given

Answer 12

• prophylactically

- treat acute crisis

Question 13

what is the most common difficulties w acute mngmt of hemophilia

Answer 13

• starting factor replacement too late

- stopping replacement too soon

Question 14

generally, minor bleeding episodes should be treated for?

Answer 14

• at least 72 hrs

Question 15

what is imp pt teaching r/t hemophilia (7)

Answer 15

prevention:

• engage in non-contact sports
• wear gloves when doing household chores (protect from tools & cuts)
• wear a medical alert bracelet
• encourage genetic counselling
• should receive coagulation factors before surgery & dental work
• when medical attention is needed
• what to do if bleeding (pressure, ice, etc.)

Question 16

what things may indicate need for immediate medical attention r/t hemophilia (8)

Answer 16

• severe pain
• swelling of a muscle or joint that restricts movement or inhibits sleep
• head injuries
• swelling in neck or mouth
• abdominal pain
• melena
• hematuria
• skin wounds in need of suturing