Child w/Blood disorders Flashcards
What clotting factor does hemophilia A have
Low clotting factor 8
What is hemophilia
a hereditary bleeding disorder, specifically x-linked recessive trait (females may carry affected gene on one X chromosome)
Which lab indicates Hemophilia A
prolonged PTT, low factor 8 or 9
what is the likelyhood of a baby (boy or girl) not getting Hemophilia A allele at all if mom is a carrier
50%
Blood disorders: physical assessment (7)
*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)
Why do people with hemophilia bleed so much
bc they lack protein in their blood that is essential in clotting
Which med route is used for hemophilia
IV
Management of hemarthrosis (6)
*elevate and immobilize joints
*ice
*analgesics
*ROM exercises after bleeding stops to prevent contractures
*PT
*avoid obesity to minimize joint stress
Sickle cell disease (SCD): cause
*cause: presence of hemoglobin S (HbS) which is flawed hemoglobin
When do HbS work abnormally
when there is low-oxygen
what can SCD lead to
obstruction which leads to engorgement and tissue ischemia which causes PAIN
why do newborns with SCD not display symptoms usually
fetal hbg is not prone to sickling like adult hbg is
common prognosis for SCD
*pt’s are prone to bacterial infections which is due to immunocompromise
leading cause of death in kids w/ SCD
bacterial infections (they lead to sepsis)
What med should be avoided in SCD pts and why
Demerol: it can lead to bleeding which is critical for SCD pts
Is B-Thalassemia major a recessive or dominant disease?
Recessive: both parents must be carriers
Who is most at risk for having B-Thalassemia Major
Mediterranean: Italy, Greece, Syria
What is the type of treatment for B-Thalassemia Major
supportive: goal is to maintain norm hemoglobin levels by blood transfusions
most common hematologic disease in children
Anemia
Anemia: Cause
*decreased production of RBCs (iron-deficiency, decreased reticulocyte count)
*increased destruction or loss of RBCs (heavy mensural periods)
Iron deficiency anemia: cause
decreased production of hemoglobin-carrying RBCs as result of inadequate dietary intake
Iron deficiency anemia: blood appearance
microcytic (small) and hypochromic (less red colored)
Iron deficiency anemia: who is at risk (3)
*older infants and toddlers who drink too much milk (>32oz daily)
*adolescent females with heavy periods
*children having growth spurts
why does milk drinking cause lead to low iron anemia
milk interferes w/ body’s ability to absorb iron from food and supplements
