MB4 hematology in children
Card Set Information
MB4 hematology in children
pediatric hematologic disorders nursing
Hematologic disorders in Peds for mother baby test 4
When is Fe-deficiency anemia rarely seen?
before age 4 to 6 mo b/c of maternal Fe stores
When does a baby become at risk for Fe deficiency anemia?
age 9-24 months mo when maternal iron stores are depleted
this is when parents start to notice it
Main problem of IDA?
Why does this occur?
iron deficiency anemia
compromised tissue oxygenation - esp to the brain
B/D of RBC exceeds ability to make new RBC's -> decreased synth of HgB -> decreased ability to carry O2
Why may premature and low birth weight babies have iron deficiency anemia?
How is this treated?
b/c they may not have iron stores built up like normal babies do
put them on iron around 2 mo of age
Why can excessive intake of cow's milk cause?
iron deficiency anemia
protein in the milk can cause diarrhea and GI bleeding; also Ca in the milk binds with iron and interferes with its absorption
At what age can a child have cow's milk?
How much is OK?
12 mo and over
should not have more than 24oz per day
Who is most at risk for decreased iron intake?
adol b/c of poor dietary habits
Who is most at risk for iron deficiency anemia due to iron loss?
adol girls - menstruation + increased growth rate
2 periods of increased growth rate that may contribute to iron deficiency anemia?
2. premature and low birth weight infants
At what age do they start preemies on iron supplements?
Why do periods of increased growth cause iron deficiency anemia?
as they grow their need for RBC's increases
5 things that cause iron deficiency anemia?
1. premature/low birth weight
2. excessive intake of cow's milk
3. decreased Fe intake
4. increased Fe lost (menstruation)
5. periods of increased growth rate
S/S of iron deficiency anemia?
1. Skin - pallor, pale, porcelain skin
2. conjunctiva - sclera is pale
3. MM - pale
4. CV - tachycardia, cardiomegaly, heart murmur
5. Respiratory - tachypnea, dyspnea upon minimal exertion
6. CNS - poor school performance
7. Nails - koilonychias/spoon nail (not same as clubbing with COPD)
8. lethargy, fatigue
9. slow, strained movement
11. difficulty sucking b/c no energy
12. sagging limp posture
Why does tachycardia occur in anemia?
to compensate for decreased RBC & decreased tissue perfusion
When may a heart murmur occur with anemia?
How is this resolved?
Teaching for parents?
with severe anemia
will go away when anemia is reversed
tell parents that it is not permanent or a defective heart
Why may a baby/child lose weight due to anemia?
they don't have enough energy to eat/suck bottle
spoon nail that may occur with long-term anemia
How to assess fatigue and other S/S of anemia?
Ask parents about the child's:
1. eating habits
2. crying and movememnts
3. irritability and crying
5. how much the sleep/nap
Dx evaluation of Fe deficiency anemia?
1. have them keep a 24 h food dietary Hx including foods eaten & description of child's activities during that day
2. lab tests: HgB, morphologic changes in RBC (color, size, etc), Fe concentration
Level of HgB considered low?
6 - 11
RBC appearance in anemia?
microcytic and hypochromic
Changes in reticulocytes in anemia?
may be normal or slightly reduced due to decreased stores of iron or could be elevated in severe cases r/t hypoxia eliciting an erythropoietic response
Serum iron level in iron deficiency anemia?
What happens to proteins available to bind iron in iron deficiency anemia?
What does this do?
they will increase -> increased iron binding capacity r/t decrease serum iron levels
2 Tx for iron deficiency anemia?
1. dietary intake
Why are iron supplements necessary in iron deficiency anemia?
dietary intake is unreliable
4 foods that inhibit iron absorption?
2. sweet potato
7 foods that are iron rich?
1. egg yolks
2. dried beans & peas
3. cream of wheat
4. dried fruit
6. Fe-fortified cereal
7. green leafy veggies: broccoli; romaine/green leaf lettuce, collard greens, mustard greens
Consideration with children and dried fruits?
good for iron but can get stuck in teeth and cause cavities - if you can use another source of iron it will be better
How is Fe supplement given?
tid through a straw, dropper, or syringe
place at back of mouth and away from teeth
Brush or wipe off teeth
Consideration with liquid iron supplements?
can stain teeth- keep away from them and wipe off after admin
Way to increase iron absorption?
admin with vit C
What vitamin enhances Fe absorption?
What juices have a lot of Vit C?
1. orange juice
2. tomato juice
3. grapefruit juice
Juices with low Vit C juices?
pear, apple, and peach
What is SCA?
sickle cell anemia - 1 of the groups of SCD's (sickle cell diseases)
genetic disease caused by or associated with the presence of one of several forms of abnormal hemoglobin in the blood: SCD, SCA, etc
sickle cell disease - broad category of hemoglobanemias
3 common forms of SCD?
1. sickle cell anemia
2. sickle C disease
3. sickle beta-thalassemia syndrome
SCA caused by?
abnormality in Hgb -> HgbS
Cause of sickle C disease?
Most common SCD in children?
sickle cell anemia
2 things all 3 forms of SCD have in common?
1. all are inheritied autosomal recessive
2. all are life-long diseases
Normal HgB is HgB ____ and abnormal HgB is _____.
normal - HgB A
abnormal - HgbS
What is caused by sickled cells?
occlusion of vessels -> tissue ischemia distal to the occlusion
if occlusion is in renal artery will cause renal failure
Who has most incidence of SCA?
AA - 3 in 1000 have SCA and 1 in 12 have the trait
Ppl at risk for sickle cell anemia?
1. african americans
2. mediteranean-italians and greeks
3. east indian
4. middle eastern
6. central and south american
Patho of SCA?
sickled/elongated cell -> stasis of RBC's & increased blood viscosity -> obstructed blood flow (esp. in bifurcations) -> tissue ischemia distal to the obstruction -> acute pain & cell infarction/cell death
Where is obstruction due to SCA likely to occur?
in bifurcations of vessels
Pain with sickle cell anemia?
will be extreme and occur wherever the occlusion is located
Where does ischemia with sickle cell occur?
distal to the occlusion site
3 tests for SCA?
1. HgB electrophoresis
2. Sickledex - sickle solubility test
3. Reticulocyte count will be elevated
Definitive test for SCA?
HgB electrophoresis - shows have SCA trait
What does the Sickledex test show?
sickle solubitily test- detects presence of HgbS but not the trait for SCA
Why is reticulocyte count elevated in SCA?
a normal RBC lives 120 days/4 months and a sickled RBC lives for 12 days -> need more RBC production
2 factors that cause the manifestations of SCA?
1. obstructin caused by the sickled RBC leads to tissue ischemia and infarction of cell death distal to where the obstruction is (can be anywhere)
2. increased RBC destruction
What is sickle cell crisis?
a broad term for several acute/exacerbating conditions that occur in with sickle cell anemia
5 causes of sickle cell crisis?
2. dehydration (overwork)
3. hypoxia (increased elevation)
5. general stress
3 types of sickle cell crisis or complications?
Which is most common?
1. vaso-occlusive crisis - most common
2. acute sequestration crisis
3. aplastic crisis
6 S/S and complications of vaso-occlusive crisis?
1. pain - usually in bone and joint but can occur anywhere and is mild to severe
2. dactylitis - inflammation of fingers and toes
3. acute chest syndrome - chest pain, cough
5. severe abd pain
Most serious complication of vaso-occlusive crisis?
1. altered LOC
2. vision changes
4. hemi or monoparesis
6. ataxia- can't coordinate muscles
weak or paralyzed on 1 side of the body
What is aphasia where pt can talk clear, understand what you said, but does not make any sense?
do not understand what is being said
What occurs in carrier state of SCA?
usually do not have S/S but in extreme stress may have some: extreme vigorous exercise & high altitudes
2 characteristics of acute sequestration crisis?
all of the blood is sequestered in the spleen -> splenomegaly & hypovolemic shock due to all the blood being in spleen
6 S/S of aplastic crisis?
6. short of breath or breathlessness
What occurs in aplastic crisis?
hemolytic anemia (hemolysis) -> decreased HgB-> aplastic crisis
HgB level in aplastic crisis?
6 to 9 g/dL
9 Tx management of SCA crisis of any kind?
1. monitor vital signs q 4 h and more frequently prn
2. bed rest
3. O2 sat 96 and up (above 95%)
4. PAIN CONTROL - around the clock
5. incorporate the use of age-appropriate non-pharmacologic pain relief measures
6. Apply heat to painful joints prn
7. HYDRATION - D5W1/2NS 1 to 1/2 times the maintenance rate: need calories and cells to be hydrated & bloodstream hydrated
8. Strict I&O: maintain urine output of at least 1mL/kg/h
9. Perform passive ROM
10. Meds: hydroxyrea, folic acid
11. RBC transfusion last resort
12. instruct to eat foods high in folic acid
Pt with SCA are at risk for ______.
risk for infection r/t alteration in splenic function from crisis of acute sequestration
What may occur in acute sequestration?
total splenic dysfunction by age 5 years -> risk for infection
Why are pt's put on bedrest with SCA crisis?
reduce O2 expenditure
O2 sat for SCA crisis?
above 95% (96% and up)
D5W 1/2 NS is a _____ solution.
hypotonic - brain will use the dextrose immediately & will have 1/2 NS left
Why does O2 sat in SCA crisis need to be above 95%?
to ease WOB & facilitate tissue perfusion & to prevent further sickling
will not reverse sickling that has already occured
How is SCA crisis pain usually controlled?
morphine around the clock - maintain a cont. analgesic blood level & reduce metabolic demand for O2 & help to end sickling
Good non-pharm pain relief measures?
1. distraction - age appropriate
2. apply heat to painful joints prn - microwave a wet towel
3. be sympathetic
Why may heat be applied for SCA crisis?
will increase blood flow (vasodilation) & perfusion to the ischemic tissues
Why is hydration important in SCA crisis?
reduce the viscosity of blood and alleviate sites of vascular occlusion and prevent further sickling caused by dehydration
Urine output in SCA crisis should be maintained at______.
to monitor kidney failure that can result from infarction of the renal artery
How are I&O's measured in babies and children?
1. weigh diapers
2. have parents catch it in a hat or urinal
Why is passive ROM performed for SCA crisis pt?
to promote circulation without exacerbating fatigue and increasing O2 needs
What does hydroxyrea do?
increases HgB levels
Why may anemia and SCA pt be given folic acid?
folic acid is necessary for the formation of RBC's
What type of RBC transfusions may be given to SCA crisis pt?
leukocyte-poor or washed RBC's
blood that has the WBC's removed as much as possible to try and prevent a transfusion reaction r/t SCA more at risk for transfusion reactions b/c of their repeated exposure to blood transfusions
Why may blood transfusions be done in SCA crisis?
to maintain adequsate HgB level which increases O2 carrying capacity to aid in further sickling and microvascular ischemia
Nursing consideration for a pt who is receiving a blood transfusion and has had them in the past?
at risk for transfusion reaction - RN MUST do v/s and stay in room
8 foods high in folic acid?
1. green leafy veggies
3. oranges, cantaloupe
5. peanut butter
6. dried beans and peas
7. enriched breads and cereals
8. enriched pastas and grains
When does risk for infection with SCA start?
What will eventually occur?
risk for infection starts as early as 6 mo of age and may have total dysfunction by 5 years old - r/t chronic immunocompromised state due to splenic damage
Tx for risk for infection with SCA?
daily penicillin therapy starting at 2 mo old and cont. until at least 5 years old or throughout childhood
3 immunizations need with SCA?
12 things to teach pt of child with SCA for home care?
1. prevent dehydration with fluids
2. expect frequent urination
3. provide rest
4. avoid cold and hot
5. avoid infections
6. avoid prolonged exposure to the sun
7. monitor body temp & notify MD of fevers & do not use antipyretics until talked to someone
8. Admin penicillin qd as ordered
9. avoid high elevations
10. S/S of infections - call MD
11. know MD phone number
Why do SCA anemia pt need to avoid cold and hot temperatures?
cold can cause sickling
hot can cause dehydration which can lead to sickling
inherited disorder/autosomal recessive that is char by abnormal HgB synth
3 groups that get beta-thalassemia the most?
3. African American
Onset of beta-thalassemia?
When does beta-thalassemia normally occur/get caught?
late infancy or early toddlerhood
How long does beta-thalassemia last?
Major complication of beta-thalassemia?
heart and/or liver failure by 20 to 30 years of age
3 differences in SCA and beta-thalassemia?
1. heart/liver failure most common in beta-thalassemia
2. growth retardation in beta-thalassemia
3. head morphological changes in beta-thalassemia
Facial char of pt with beta-thalassemia?
1. enlarged head
2. frontal and parietal bossing - enlargement
3. severe maxillary hyperplasia - top bucked
4. malocclusion - misalignment of teeth
5. bronze skin tone
4 clinical manifestations of beta thalassemia?
1. severe anemia
2. growth maturation retardation
3. char face
thalassemia major & Cooley's anemia
3 Tx for beta-thalassemia?
1. erythrocyte transfusion
2. chelation therapy - remove heavy metals/iron
Why is chelation therapy needed for beta-thalassemia?
leach off Fe b/c will have overload from too many transfusions
Pt is at risk for _____ if they have had a splenectomy.
X-linked autosomal recessive disorder that occurs predominantly in men and women are mostly carriers
Cure for hemophilia?
What does hemophilia cause?
deficiency in one of factors for blood coagulation
3 types of hemophilia?
1. hemophilia A - classic - factor VII
2. hemophilia B - factor IX
3. factor XI
Classifications of hemophilia?
1. mild - bleeding occurs with severe trauma
2. moderate - bleeding occurs with minor trauma
3. severe - bleeding occurs with no trauma/SPONTANEOUS bleeding
With each pregnancy a woman carrier of hemophilia has a __% chance of having a hemophilic son.
If a father has hemophilia what will his daughters have?
What about his sons?
all of his daughters will be carriers and none of his sons will be affected
4 clinical manifestations of hemophilia?
1. newborn - umbilical bleeding or prolonged bleeding with circumcision
2. bruises easily
3. epistaxis - nose bleeds
4. bleeding anywhere - most common sites: muscles and joints
bleeding in joints that will cause swelling of the joint
4 components of Dx of hemophilia?
1. Hx of bleeding episodes in newborn or toddler
2. tests of hemostasis
3. tests for blood clotting
4. tests of clotting factor function
2 tests for hemostasis and results that indicate hemophilia?
platelet aggregation - prolonged
bleeding time - increased
2 tests for blood clotting and results that indicate hemophilia?
1. prothrombin time - PT- increased
2. partial thromboplastin time - PTT- increased
Definitive test for hemophilia?
What is tested?
factor assays- test for clotting factor function & will show what factor is being affected
2 components in therapeutic management of hemophilia?
1. classification of bleeding will det Tx: mild, moderate, severe
2. type of deficient clotting factor - will give factor replacement
Factor replacement therapy in hemophilia?
replace factor with human blood and/or recombinant clotting factors through IV over 5 minutes
very expensive 100,000 to 200,000 dollars per year
Therapy for severe bleeding in hemophilia?
factor replacement IV over 5 min.
Children may do this themselves at 8 years and above - may go to hemophilia camps
* RICE - rest for 24 to 48 h, ice, compression, elevation
Tx for mild to moderate bleeding in hemophilia?
desmospressin/DDAVP - stimulates release of factor VIII for hemophilia A
route - intranasal - need to keep up with how many sprays used
Why do you need to specify how long child should rest if bleeding occurs?
don't want them to get stiff
Things to avoid with hemophilia child?
1. toys with sharp edges
2. sharp scissors
3. straight razors
4. NO IM or IV unless necessary
5. no ASA or ibuprofen
6. finger sticks (use venipuncture instead b/c will bleed less)
7 nursing instructions for hemophilia pt?
1. use soft toothbrush and water irrigating device
2. electric razor
3. wear helmut on bike
4. wear joint pads when playing
5. padded crib sides
7. medical ID bracelet
8. keep on hand at all times
: bags of ice or cold packs in freezer
Immune thrombocytopenic purpura?
idiopathic thrombocyopenic purpura/hemorrhagic disorder
5 char of immune thrombocytopenic purpura?
1. thrombocytopenia - platelet count below 150,000/mm3
2. purpuric rash
3. normal bone marrow
4. absence of identifiable cause of thrombocytopenia
5. autoimmune response to disease-related antigens
any rash in wheich blood cells leak into the skin or mucus membranes, usually at mult sites
2 types of purpuric skin lesions?
1. petechiae- pinpoint purpuric lesions
2. ecchymoses- larger hemorrhages into the skin
What usually occurs right before occurrence of immune thrombocytopenic purpura?
upper resp infections and/or viral infections: measels, rubella, mumps, chickenpox, 5th disease or parvovirus B12 (slapped face)
2 classifications of ITP?
acute - self - limiting course (1 to 3 mo)
chronic - lasts longer than 6 mo
Dx eval of ITP?
1. thorough history: family Hx, recent meds, recent vaccination w/live virus, recent viral infections
2. low serum platelet count - <150,000
3 clinical manifestations of ITP?
1. bruising, petechiae and/or ecchymoses
2. bleeding gums
3. heavy menstrual flow
2 goals in Tx of ITP?
1. prevent rare, life threatening bleeding events : IC bleeding
2. restore platelet count to above 20,000
4 nursing interventions for ITP?
1. assess for s/s of bleeding from any body system
2. monitor platelet count
3. no IM injections, ASA, or NSAIDS
4 restrict activity at onset: no bikes, roller blading etc
3 possible TX for ITP?
1. for acute may just watch and wait - monitor bleeding and platelet count
2. oral or IV corticosteroids: block autoimmune destruction of platelets
3. IV admin of immunoglobulins (IVIG)
When my splenectomy be performed for ITP?
if chronic 1 year or longer and doesn't respond to drug therapy
will try to wait until at least 5 years old