Heme

Question 1

proper administration of iron supplements

Answer 1

• give in divided doses for max absorption
• give in b/w meals to inc absorption
• administer w/ fruit juice or multivitamin preparation
  
  • vit C facilitates absorption
• do not administer w/ milk or antacids–dec absorption
• do not give w/ tea–b/c tannin in tea binds iron to form an insoluble complex
• may stain teeth–administer to infants via a syringe, older children via a straw
  
  • wash off teeth after administration
• only keep a month supply in the home b/c it is a poison

Question 2

therapeutic mgmt to prevent iron deficiency anemia

Answer 2

• breast milk or commercial formula should be used for the first 12 mos of life
• begin iron supplements
  
  • full term: by 4-6 mos via iron fortified formula or rice cereal
  • preterm: by 2 mos via iron drops initially, then iron fortified cereal
    
    • administer ferrous sulfate at 2-3 mg/kg to breast fed preterm infants after 2 mos
• limit amt of milk to <1 L/day
• universal screening for iron deficiency starts at 12 mos

Question 3

causes of anemia

Answer 3

• bone marrow fails to produce RBCs
• leukemia or other malignancy
• chronic renal dz
• collagen dz
• hypothyroidism

Question 4

aplastic anemia

Answer 4

• all formed elements in the blood are depressed
  
  • pancytopenia–low platelets (so dec clotting), WBC (so dec ability to fight infection), low RBC (so dec O2 carry capacity)

Question 5

hypoplastic anemia

Answer 5

• profound depression of RBCs, but normal WBCs and platelet
• this is how iron deficiency anemia works

Question 6

Sickle Cell Anemia

Answer 6

• hereditary hemoglobinopathy
• ethnicity:
  
  • primarily in African Americans
  • occasionally in ppl of Mediterranean descent
    
    • also seen in South American, Arabian, and East Indian descent

Question 7

etiology of Sickle Cell

Answer 7

• in areas where malaria is common, individuals w/ sickle cell trait tend to have a survival advantage over those w/o the trait
• autosomal recessive disorder (so both parents have it)

Question 8

patho of sickle cell anemia

Answer 8

• partial or complete replacement of normal hgb with hgb S
  
  • hgb in the RBCs takes on an elongated/sickle shape
    
    • sickled cells are rigid and obstruct blood flow–>engorgement and tissue ischemia
    • when hgb is deprived of O2–>hypoxia–>clumping and agglutination of the sickling cells
• dz not normally seen until 6 mos of age b/c hgb F does not sickle
• large tissue infarctions occur–>damaged tissues in organs lead to impaired fcn

Question 9

splenic sequestration

Answer 9

• occurs with sickle cell anemia
• blood pools in the spleen which can cause a splenic rupture and can look like hypovolemic shock (tachycardia, hypoTN)
• may require splenectomy at an early age
  
  • results in dec immunity
  • need prophylactic abx and MUST have immunizations

Question 10

dx of sickle cell

Answer 10

• cord blood in newborns is tested
  
  • newborn screening is done in most states
• genetic testing is done to identify carriers and children who have dz
• sickle turbidity test: quick screening purposes in children older than 6 mos

Question 11

prognosis of sickle cell

Answer 11

• no cure (except possibly a bone marrow transplant)
• supportive care and prevent sickling episodes
• frequent bacterial infections may occur due to immunocompromise–>due to lack of splenic involvement
  
  • bacterial infection is leading cause of death to yg kids w/ sickle cell dz
• strokes occur in children
  
  • result in neurodevelopmental delay, cognitive disabilities

Question 12

precipitating factors of a sickle cell crisis

Answer 12

• anything that inc the body’s need for O2 or alters transport of O2
• trauma
• infection, fever
• physical and emotional stress
• inc blood viscosity due to dehydration
• hypoxia
  
  • from high altitude, poorly pressurized airplanes, hypoventilation, vasoconstriction due to hypothermia

Question 13

vaso occlusive/thrombotic sickle cell crisis

Answer 13

• preferably called a painful event/episode
• most common type
• very painful
• stasis of blood w/ clumping of cells in microcirculation–>ischemia–>infarction
• signs: fever, pain, tissue engorgement

Question 14

splenic sequestration: sickle cell crisis

Answer 14

• life threatening–death can occur w/in hours
• blood pools in the spleen and sometimes the liver
• S/S:
  
  • profound anemia: pallor, fatigue, low O2, tachycardia, tachypnea
  • hypovolemia
  • shock

Question 15

aplastic crises: sickle cell crisis

Answer 15

• diminished production and inc destruction of RBCs
• triggered by viral infection or depletion of folic acid
• S/S:
  
  • pallor
  • profound anemia: low O2, tachycardia, tachypnea

Question 16

acute chest syndrome

Answer 16

• clinically similar to pneumonia
• vaso-occlusive crisis or infection results in sickling in the lungs b/c the lungs are being deprived of adequate perfusion
• need to reverse ASAP or they die
• S/S: chest pain, fever, cough, tachypnea, wheezing, hypoxia, may have inc WOC w/ retractions
• repeated episodes may lead to pulmonary HTN and restrictive lung dz

Question 17

tx of acute chest syndrome

Answer 17

• O2 administration based on ABGs and/or pulse ox reading
• transfusion
• IS to prevent atelectasis
• pain meds
• prevent fluid overload

Question 18

psychosocial needs with Sickle Cell Anemia

Answer 18

• coping mechanisms
• support w/ genetic counseling
• financial needs
• caregiver role strain
• living with chronic illness in the family

Question 19

medical tx of SCD

Answer 19

• IV should be started immediately
• transfusion:
  
  • prevention of stroke
  • monthly transfusions to keep hgb S at only 20-30%
  • risks:
    
    • iron overload
    • development of abs to foreign RBCs
    • contamination w/ infective agents hyperviscosity of blood
    • transfusion rxns
    • time consuming

Question 20

medication to tx SCD

Answer 20

• hydroxyurea: medication that increases the formation of Hgb F b/c it doesn’t sickle

Question 21

how to tx a pt in vaso occlusive crisis

Answer 21

• fluids: usually 2x maintenance
• O2
• pain meds:
  
  • must have opioids!
    
    • need large doses, so should be on PCA–to prevent breakthrough pain
    • demerol (meperidine) should not be used b/c the metabolites it breaks down to are toxic to CNS
    • hydromorphone (dilaudid) and morphine are considered the best drugs for pain mgmt
  • NSAIDs: ketorolac–is used if serum Cr is <1.5
  • No ASA: may inc acidosis

Question 22

nursing mgmt of SCD

Answer 22

• monitor child’s growth–watch for failure to thrive
• careful multisystem assessment
• assess pain
• observe for presence of inflammation or possible infection
  
  • need to tx infection promptly
• maintain hydration (oral or other as needed)
• carefully monitor for S/S of shock

Question 23

HTSC transplant

Answer 23

• human tumor stem cell (HTSC)
• potential cure for pts with SCD
• difficult decision
  
  • child may face death w/o transplant
  • preparing child for transplant places them at risk
• no “rescue” procedure if complications follow

Question 24

labs w/ hemophilia

Answer 24

• prolonged PTT
• normal PT
• normal bleeding time
• normal platelet count
• deficiency of factor VIII or IX

Question 25

what is hemophilia

Answer 25

• group of hereditary bleeding disorders that result from deficiencies of specific clotting factors

Question 26

Hemophilia A

Answer 26

• classic hemophilia
• deficiency of factor VIII
• most common type of hemophilia
• occurrence: 1 in 5000 males

Question 27

Hemophilia B

Answer 27

• also known as Christmas Disease
• caused by deficiency in factor IX
• only 15% of cases of hemophilia

Question 28

etiology of hemophilia A

Answer 28

• X linked recessive trait
  
  • mom is carrier–>boys are affected
• degree of bleeding depends on amount of clotting factor and severity of a given injury
• 33% have NO family hx
  
  • dz caused by new mutation

Question 29

manifestations of hemophilia

Answer 29

• bleeding tendencies range from mild to severe
• symptoms may not occur until 6 mos of age b/c not may injuries before then
  
  • mobility leads to injuries from falls and accidents
• hemoarthrosis: bleeding into joint spaces of knee, ankle, elbow–>leads to impaired mobility
• ecchymosis: bruising
• epistaxis
• bleeding after procedures:
  
  • minor trauma, tooth extraction, minor surgeries
  • large subQ and IM hemorrhages may occur
  • bleeding into neck, chest, mouth may compromised airway

Question 30

hemophilia clinical therapy

Answer 30

• can be dx thru amniocentesis
• genetic testing of family members to identify carriers
• dx on basis of hx, labs, exam

Question 31

medical mgmt of hemophilia

Answer 31

• DDAVP (AKA desmopressin/vasopressin)
  
  • IV
  • causes 2-4x inc in factor VIII activity
  • used for mild hemophilia
• replace missing clotting factors
• transfusions
  
  • at home w/ prompt intervention to dec complications
  • following major or minor hemorrhages

Question 32

prognosis of hemophilia

Answer 32

• mild to moderate hemophilia: live near normal lives
• gene therapy for future:
  
  • infuse carrier organisms into pt
  • these act on target cells to promote manufacture of deficient clotting factors
• most often die from head injuries b/c have internal bleeding w/ no external problems

Question 33

interventions w/ hemophilia

Answer 33

• close supervision and safe environment
• dental procedures in controlled situation
• shave only w/ electric razor
• superficial bleeding: apply pressure for at least 15 min and ice to vasoconstrict
• if significant bleeding occurs, transfuse for factor replacement

Question 34

mgmt of hemoarthrosis

Answer 34

• during bleeding episodes, elevate and immobilize the joint
• ice–>vasoconstrict
• analgesics–>avoid ASA
• ROM after bleeding stops to prevent contractures–>do active ROM
• PT
• avoid obesity to minimize joint stress