MB4 Peds Hematology Forsythe

rare before age 4 yo 6 mo.  b/c still have maternal Fe stores.  These become depleted around age 9-24 months which is when Fe deficiency anemia is most often seen

compromised tissue oxygenation

cannot make HgB -> RBC are broken down -> exceeds ability of the body to make new RBC

premature and low birth weight

they did not store as much Fe from the mothers

• 1. premature/low birth weight
• 2. excessive intake of cows milk
• 3. decreased Fe intake
• 4. increased Fe lost
• 5. periods of increased growth rate

start on Fe supplements around 2 months of age

causes diarrhea and GI bleeding

no cow's milk before 12 mo.

CH < 12 mo - no more than 24 oz per day

adolescents - poor habits

adol. girls - periods

• 1. adolescents
• 2. premature and low birth weight babies

growing rapidly = increased need for Fe

• 1. skin - pallor, pale/porcelain skin tone
• 2. sclera pale
• 3. MM - pale
• 4. tachycardia
• 5. cardiomegaly
• 6. heart murmur (severe anemia)
• 7. poor school performance
• 8. koilonychias (spoon nail)
• 9. lethargy & fatigue
• 10. slow, strained movements
• 11. difficulty sucking/too tired to eat
• 12. sagging, limp posture

to compensate for RBC and decreased O2 to tissues

will reverse when anemia is treated

Tell parents murmur whould go away

no energy...no energy to eat

ask the pt if they act tired a lot or take a lot of naps or play differently or not at all

ask parent about the infant's movements and how much it sleeps

• 1. 24 h dietary Hx
• 2. HgB
• 3. RBC
• 4. reticulocyte (immature RBC)
• 5. Fe
• 6. Fe binding capacity (Fe carrying proteins)

• 1. foods eaten
• 2. description of CH activities during that time

• 1. HgB
• 2. morphologic changes in RBC
• 3. Fe concentration

6-11 g/dL

microcytic and hypochromic (small and pale/decreased HgB content)

immature RBC

normal or slightly reduced due to decreased Fe stores

may be elevated in severe anemia r/t hypoxia -> erythropeietic response

binding capacity/measurement of all proteins available for binding Fe

increased in IDA b/c decreased Fe levels

• 1. increase Fe in diet
• 2. Fe supplements

b/c absorption of Fe from foods is unreliable

• 1. spinach
• 2. sweet potatos
• 3. coffee
• 4. tea

• 1. egg yolks
• 2. dried beans
• 3. cream of wheat
• 4. dried fruit
• 5. liver
• 6. iron-fortified cereal
• 7. green leafy veggies

• 1. broccoli
• 2. romaine lettuce
• 3. collard greens
• 4. mustard greens

• 1. admin tid
• 2. use a straw, dropper, or syringe
• 3. place at back of mouth and away from the teeth
• 4. brush teeth or wipe with wash cloth
• 5. Admin with Vit C

stains the teeth

• 1. orange
• 2. tomato
• 3. grapefruit

• 1. pear
• 2. apple
• 3. peach

sickle cell anemia

sickle cell disease

• 1. SCDs are broad category of hemoglobineamias
• 2. SCA is one of the groups of SCDs

a genetic disease caused by or ass. with abnormal HgB

• 1. sickle cell anemia - homozygous HgBS
• 2. sickle C disease - HbC
• 3. sickle beta-thalassemia syndrome

1. inherited autosomeal recessive - 2 copies of an abnormal gene present

2. life-long disease

HgBA

HgBS

autosomal recessive

• 1. AA
• 2. mediteranean-Italians & Greeks
• 3. East Indian
• 4. Middle Eastern
• 5. Caribbean
• 6. Central and South American

3 in 1000

1 in 12

sickled or elongated RBC -> stasis of RBC & increased blood viscosity -> obstructed blood flow -> tissue ischemia DISTAL to obstruction -> acute pain & cell infarction/death

at bifurcations in BV

• 1. HgB electrophoresis
• 2. sickledex (sickle solubility test)
• 3. reticulocyte count

HgB electrophoresis - shows have SCA trait

detects presence of HgBS but not the trait that causes SCA

elevated

normal RBC lives for 120 days/4months.  Sickled RBC dies withing 12 days so need increased RBC production

1. obstruction caused by the sickled RBCs and tissue ischemia/infarction distal to obstruction

2. increased RBC destruction

a broad term for several acute or exacerbating conditions occurring with sickle cell anemia

• 1. infection
• 2. dehydration
• 3. hypoxia
• 4. trauma/phys stress
• 5. general stress

• 1. vaso-occlusive crisis
• 2. acute sequestration crisis
• 3. aplastic crisis

• 1. normally has no s/s
• 2. under extreme stress may have s/s:  extreme vigorous exercise, high altitudes

Denver/Colorado mountains

vaso-occlusive

• 1. bone and joint pain
• 2. dactylitis
• 3. acute chest syndrome
• 4. priapism
• 5. CVA

CVA

• 1. in bone and joints
• 2. can occur anywhere in body
• 3. mild to severe

inflammed/red & edematous hands and feet

• 1. chest pain
• 2. fever
• 3. cough if occluded in chest area
• 4. severe abd pain

erection that won't go down

• 1. altered LOC
• 2. vision changes
• 3. headache
• 4. hemi or monoparesis
• 5. aphasia/dysphasia
• 6. ataxia
• 7. seizures

weakness on one side of the body

one limb is very weak but not paralyzed

• 1. splenic enlargement
• 2. hypovolemic shock

blood volume r/t risk for hypovolemic shock

6 to 9 g/dL

aplastic crisis

abnormal decrease in reticulocytes

• 1. pallor
• 2. fatigue
• 3. lethargy
• 4. fainting
• 5. dyspnea/breathlessness
• 6. HA

• 1. monitor V/S q 4 h or more prn
• 2. bed rest
• 3. maintain O2 sat >95% (96 or above)
• 4. PAIN CONTROL
• 5. age-app. non-pharmacologic pain relief
• 6. apply heat to painful joints prn
• 7. HYDRATION
• 8. strict I&O
• 9. perform passive ROM
• 10. Meds
• 11. admin of RBC
• 12. instruct in foods high in folic acid

CH with SCA more at risk for infection r/t splenic damage due to crisis of acute sequestration

5

to reduce O2 expenditure

ease work of breathing & facilitate tissue oxygenation

prevent more sickling - hypoxia can cause crisis

Morphine around the clock

maintain cont analgesic blood level & reduce met. demand for O2 to help end sickling

distraction!!

vasodilation - increase blood flow & O2 to the tissues

D5W1/2NS 1 to 1/2 X the maintenance rate


reduces viscosity of blood (water will go into cells) & provides glucose and fluid

alleviating sites of vascular occlusion and preventing further sickling caused by dehydration

hypotonic

cells hydrated, blood hydrated with free water, and gives glucose for energy

monitor for kidney failure r/t infarction of renal artery

1mL/kg/h

weigh diapers or tell parents to catch it in hat/urinal

• 1. hydroxyurea - increases HgB levels
• 2. folic acid - needed for RBC production
• 3. RBC transfusion - last resort

to maintain HgB and increase O2 carrying capacity and decrease further sickling & microvascular ischemia

immunosuppresors

more at risk for transfusion reactions b/c receive so many of them & may dev hypersensitivity

• 1. green leafy veggies
• 2. broccoli
• 3. oranges, canteloupe
• 4. nuts
• 5. peanut butter
• 6. dried beans/peas
• 7. enriched breads & breakfast cereal
• 8. enriched pasta and grain
• 9. probably need a suppement

• 1. give immunosuppressants before
• 2. give leukocyte-poor/washed RBC to decrease reactions

infection r/t splenic dysfunction

starts as early as 6 mo.

5 years old

daily penicillin starting at 2 months and ont. until at least 5 years or throughout childhood

• 1. pneumococcal
• 2. meningococcal
• 3. influenza

• 1. encourage fluids & increase for hot weather etc
• 2. expect frequent urination
• 3. provide rest
• 4. avoid cold (crisis) & extreme heat(dehydration)
• 5. avoid infectin
• 6. avoid prolonged sun exposure
• 7. monitor body temp & notify MD if increases - do not use antypyretics without talking to MD
• 8. Admin penicillin qd
• 9. avoid use of aspirin - acet/ibu alternatives
• 10. avoid high altitudes
• 11. call primary caregiver if s/s of infection
• 12.know MD phone #

autosomal recessive disorder char by abnormal HgB synthesis

mediterranean, Asian, AA

insidious

late infancy or early toddlerhood & lasts whole life

usually causes heart and/or liver failure by 20 to 30

• 1. severe anemia
• 2. growth maturation retardation
• 3. facial features changed
• 4. hepatosplenomegaly

excessive erythropoiesis

• 1. enlarge head
• 2. frontal & parietal bossing(enlargement)
• 3. severe maxiallary hyperplasia (top bucked)
• 4. malocclusion - teeth not aligned
• 5. bronze skin tone

• 1. erythrocyte transfusion
• 2. chelation therapy
• 3. splenectomy

have build up of Fe from blood transfusions

Xplinked autosomal recessive disorder with a dificiency in one of blood coagulation factors - lifelong and genetic

• A - factor VII deficient
• B- factor IX deficient
• factor XI

• 1. mild - bleeding occurs with severe trauma
• 2. moderate - bleeding occurs with minor trauma
• 3. severe - bleeding occurs with NO traum.  Bleeds spontaneously!

men

mostly carriers

no it is X-linked

• 1. abnormal bleeding from umbilical cord
• 2. prolonged bleeding after circumcision

• 1. bruise easliy
• 2. epistaxis
• 3. bleeding anywhere

1. muscles and joints - hemarthrosis

• 1. Hx of bleeding in infants/toddlers
• 2. test of hemostasis
• 3. test blood clotting
• 4. test clotting factor function

1. platelet aggretation- will be prolonged

2. bleeding time - increased

• 1. PT - increased
• 2. PTT - increased

fatory assays - will tell which clotting factor is affected

factory assays that show if clotting factors are affected

vitamin C

no cure

• 1. classification of bleeding
• 2. type of deficient clotting factor

occurs spontaneously

• 1. factor replacement w/ human blood or recombinant clotting factors
• 2. RICE

admin IV over 5 minutes

8 years old

100-200,000

• rest 24-48 hours
• ice
• compression
• elevation

DDAVP/desmopressin - stimultes release of factor VIII

route:  intranasal

• 1. Avoid toys with sharp edges, sharp scissors, straight razors, IM/IV meds, aspirin/ibup/finger sticks
• 2. use soft toothbrusha dn water irrigating device
• 3. use elc razor
• 4. wear helmet with bike
• 5. wear joint pads when playing
• 6. padded crib sides
• 7. med ID bracelet or necklace
• 8. keep on hand at all times bags of ice or cold packs

venipuncture b/c causes less bleeding

low platelet count

idiopathic thrombocytopenic purpura or hemorrhagic disorder

• 1. thrombocytopenia - <150,000
• 2. pupuric rash, petechiae, echymoses
• 3. normal bone marrow
• 4. absence of identifiable cause
• 5. autoimmune reesponse to disease

any rash in which blood cells leak into the skin or MM

pinpoint purpuric lesions

larger hemorrhages into the skin=- bruising

after upper resp infections or viral infections:  measles, rubella, mumps, chickenpox, 5th disease or parvovirus B19/slapped face

1. acute - self-limiting course (1 to 3 months)

2. chronic - lasts longer than 6 months

1. thorough Hx including:  fam Hx, recent meds, recent vaccination w/ liv virus, recent viral infections o upper resp infection

2. low serum platelet count - below 150,000

• 1. bruising, petechiae and/or ecchymosis
• 2. bleeding gums
• 3. heavy menstrual flow

begin abruptly

• 1. prevent rare, life-threatening bleeding events:  intracranial bleeding
• 2. restore platelet count to above 200,000

• 1. assess for s/s of bleeding from any body system
• 2. monitor platelet count
• 3. no IM injections, aspiring or aspirin containing products, NSAIDS
• 4. restrict activity at onset:  no bikes, contact sports, etc

• 1. acute ITP:  watch and wait - monitor bleeding and monitor platelets
• 2. oral or IV corticosteroids :  block autoimmune destruction of platelets
• 3. IV admin of immunoglobulins (IVIG)

splenectomy

will try to wait until at least 5 years old