Child w/Blood disorders

Question 1

What clotting factor does hemophilia A have

Answer 1

Low clotting factor 8

Question 2

What is hemophilia

Answer 2

a hereditary bleeding disorder, specifically x-linked recessive trait (females may carry affected gene on one X chromosome)

Question 3

Which lab indicates Hemophilia A

Answer 3

prolonged PTT, low factor 8 or 9

Question 4

what is the likelyhood of a baby (boy or girl) not getting Hemophilia A allele at all if mom is a carrier

Answer 4

50%

Question 5

Blood disorders: physical assessment (7)

Answer 5

*Look in mouth aka mucous membrane for PALLOR
*Skin bruising or hemosiderosis/bronzing
*ABD for enlarged liver or spleen (Big for sickle cell)
*sceras for jaundice
*hematuria (Big w/ sickle cell)
*Fever/illness (Sickle cell)
*increased pulse (anemia), systolic flow murmur (sickle cell)

Question 6

Why do people with hemophilia bleed so much

Answer 6

bc they lack protein in their blood that is essential in clotting

Question 7

Which med route is used for hemophilia

Answer 7

IV

Question 8

Management of hemarthrosis (6)

Answer 8

*elevate and immobilize joints
*ice
*analgesics
*ROM exercises after bleeding stops to prevent contractures
*PT
*avoid obesity to minimize joint stress

Question 9

Sickle cell disease (SCD): cause

Answer 9

*cause: presence of hemoglobin S (HbS) which is flawed hemoglobin

Question 10

When do HbS work abnormally

Answer 10

when there is low-oxygen

Question 11

what can SCD lead to

Answer 11

obstruction which leads to engorgement and tissue ischemia which causes PAIN

Question 12

why do newborns with SCD not display symptoms usually

Answer 12

fetal hbg is not prone to sickling like adult hbg is

Question 13

common prognosis for SCD

Answer 13

*pt’s are prone to bacterial infections which is due to immunocompromise

Question 14

leading cause of death in kids w/ SCD

Answer 14

bacterial infections (they lead to sepsis)

Question 15

What med should be avoided in SCD pts and why

Answer 15

Demerol: it can lead to bleeding which is critical for SCD pts

Question 16

Is B-Thalassemia major a recessive or dominant disease?

Answer 16

Recessive: both parents must be carriers

Question 17

Who is most at risk for having B-Thalassemia Major

Answer 17

Mediterranean: Italy, Greece, Syria

Question 18

What is the type of treatment for B-Thalassemia Major

Answer 18

supportive: goal is to maintain norm hemoglobin levels by blood transfusions

Question 19

most common hematologic disease in children

Answer 19

Anemia

Question 20

Anemia: Cause

Answer 20

*decreased production of RBCs (iron-deficiency, decreased reticulocyte count)
*increased destruction or loss of RBCs (heavy mensural periods)

Question 21

Iron deficiency anemia: cause

Answer 21

decreased production of hemoglobin-carrying RBCs as result of inadequate dietary intake

Question 22

Iron deficiency anemia: blood appearance

Answer 22

microcytic (small) and hypochromic (less red colored)

Question 23

Iron deficiency anemia: who is at risk (3)

Answer 23

*older infants and toddlers who drink too much milk (>32oz daily)
*adolescent females with heavy periods
*children having growth spurts

Question 24

why does milk drinking cause lead to low iron anemia

Answer 24

milk interferes w/ body’s ability to absorb iron from food and supplements