Hematologic/immunilogical Function Flashcards
1
Q
Pediatric variation on blood
A
- RBC production shifts from the liver to the bone marrow as you age
- infants have hemoglobin F predominantly for first 6M (F has shorter life span—it is produced when we give erythromycin)
- fetus gets iron thru placenta and stores iron for 4-6M until fetus starts storing adult iron (hemoglobin A)
2
Q
Assessment of hemoglobin fxn
A
- CBC, hx, and assessment findings
- child’s energy and activity level
- child’s illness and healing patterns
- growth patterns
3
Q
Precautions for platelets under 100k
A
- No contact sports
- protective equipment like bike helmets
4
Q
Precautions for platelets 50-100k
A
- padding with activity
- protective equipment like bike helmets when riding a bike
5
Q
Precautions for platelets under 50k
A
- extreme caution as spontaneously bleeds can occur in their head
- quiet activities
6
Q
At what platelet level can kids go back to school
A
Over 20k
7
Q
Inc WBCs indicates
A
Infection
8
Q
Who might have low WBCs
A
Chemo, newborn
9
Q
Increased lymphocytes indicates
A
Viral infection
10
Q
Monocytes
A
Second line of defense, acute phase
11
Q
Neutrophils
A
1st line of defense, elevated in bacterial infection
- segs and bands
- segs are more mature
12
Q
Basophils are elevated with…
A
Chronic inflammation
13
Q
Eosinophils are elevated with…
A
Allergies
14
Q
Absolute neutrophil count (ANC)
A
Tells us the body’s ability to fight infection
- under 500, severe risk of getting infection
- should be above 1000
Calculated by adding % segs + % bands, convert to a decimal and multiply by WBC count
15
Q
Interventions for neutropenic people (ANC <1000 for infants, ANC <1500 in older kids)
A
- monitor VS est temp (TEMP IS EMERGENCY)
- handwashing
- inspect skin for breaks and redness
- inspect mouth for ulcers
- no flowers and plants in room
- low bac diet (somewhat controversial), no grapes, cook meat well, keep cold food cold and hot food hot
- chx dressings and lines sterilely
- no live-virus vax (MMR, varicella, flu mist)
- avoid contact with people who carry diseases—screen visitors
16
Q
Erythrocytes
A
RBCs; tissue oxygenation
- abnormalities are polycythemia (excess) or anemia (low)
17
Q
Erythropoietin
A
Stim RBC production
- Made by kidneys; kidneys can be affected if normal is abnormal
18
Q
Anemia
A
Dec in RBCs and/or hemoglobin concentration below normal; oxygen carrying capacity of blood is too low
- most common hematologic disorder of childhood
19
Q
Causes of anemia
A
- hemorrhage
- hemolysis
- dec production from bone marrow suppression, absence of subs needed for production like iron, B complex vits, erythropoitein
20
Q
CM of anemia
A
- anorexia
- pallor
- skin b/d
- jaundice
- tachy and tachy
- altered neuro status/behavior
- weak or low exercise tolerance
- gum hypertrophy
- smooth tongue
- blood in urine or stool
- infection
- cold intolerance
21
Q
Effects of anemia on circ system
A
- hemodilution—dec conc of cells in blood bc inc amount of fluid
- dec peripheral resistance
- inc cardiac circ and turbulence (can lead to murmur or cardiac failure)
- cyanosis
- growth retardation
22
Q
Therapeutic management of anemia
A
- treat underlying cause
- transfusion after hemorrhage if needed
- nutritional intervention for deficiency anemias
- supportive care like IVF, oxygen, bed rest
23
Q
NC for anemia
A
- prep kid and fam for labs
- dec oxy demands
- safety
- good hand wash and mouth care
- maintain normal body temp
- prevent complications
- support family
- avoid vigorous exercise
24
Q
Production anemias
A
Body not making enough
- Iron deficiency anemia—lack iron to make RBCs
- Aplastic anemia
25
Anemia etiologies for production anemia
Bone marrow fails to produce RBCs
- leukemia or other malignancy
- chronic renal disease
- collagen diseases
- hypothyroidism
- nutritional deficiencies
26
Iron deficiency anemia
- most prevalent nutritional dx in US
- incidence dec with WIC (women infant children supportive program—get foods rich in iron)
- body lacks enough iron to make HgB
27
Hemolytic anemias
RBCs are rupturing or there is a destruction
- sickle cell anemia
- beta-thalassemia
28
CM in iron deficiency anemia
- irritability, anorexia
- pallor of skin and mucus membranes
- mild growth retardation
- exercise intolerance
- frequent infections and weakened immune sys
- cognitive delays and behavior changes (long-term anemia)
29
Etiologies for iron deficiency anemia
- inadequate iron stores at birth
- deficiency dietary intake (rapid growth rate—infancy, toddler, adolescence, excessive milk intake, poor general eating habits, exclusive breastfeeding after 6M)
- impaired iron absorption (presence of iron inhibitors, malabsorption dx, chronic diarrhea)
30
Therapeutic management for IDA
- Prevent by switching to whole milk by 12M, limit formula to < 1L/day (32 oz), limit milk to <24oz/day
- add iron fortified formula and cereal by age 6M
- iron supplements like ferrous sulfate
- blood transfusions for severe cases
31
NC for IDA
- assess—milk and iron intake
- determine and eliminate cause
- give iron-rich foods (pb, meat, grain, eggs)
- teach parents to admin supplements
- administer parenteral iron safely (can come in multivitamins for kids—out of reach for child bc looks like candy)
- follow up care
- keep warm
- hands on heart
32
How to administer oral iron
- best btwn meals
- give with straw or back of mouth
- best abs in acidic environments
- teach parents—measure well, inc fluids and fiber in diet (can be constipating)
- avoid antacids, coffee, teac, dairy, egg or whole grains one hour before admin
33
Adverse effects of giving iron
Constipation, nausea, gastric irritation, diarrhea, anorexia, stained teeth, tarry stools, OD is lethal
34
Aplastic anemia
Bone marrow failure to make all elements of the blood
- pancytopenia—all elements of the blood are low
- patho: red bone marrow converted to yellow fatty marrow which doesn’t make the elements
35
Aplastic anemia etiologies
- primary (congenital)
- secondary (acquired)
36
Therapeutic management of aplastic anemia
- bone marrow transplant
- stem cells transplant (HSCT)
- immunosuppressive therapy
- follow leukemia protocols (mimicks leukemia bc cell counts are low)
37
Anemias caused by inc destruction of RBCs
- hemolytic
- dec life span of RBC
- hereditary spherocytosis (HS)—spleen destroying RBCs; splenectomy/partial plenectomy can correct hemolysis but does not fix underlying disease
- aplastic crisis—bone marrow and hematopoietic stem cells that reside there are damaged
38
Sickle cell anemia
Most common destructive anemia
- RBCs don’t carry normal adult hemoglobin; instead carry HgB S
- auto recessive
- most common in AfrAm, Mediterranean, middle eastern and Indian descent
- asymp until 6M when fetal HgB declines
39
SC trait
- By definition have less than 50% HgB that is sickle shaped; different than having the anemia
- usually not symptoms
40
Sickle cell patho
Mutation in Beta-globulin gene that causes glutamic acid to be changed to valine, creating Hgb S that is prone to polymerization of other HgB molecules under conditions of low oxygen tension.
- normal Hgb replaced with Hgb S
- Hgb S is less soluble than Hgb A
- when deoxygenated Hgb S becomes viscous and precipitates into long crystal shapes
- when RBCs are sickled, they are more rigid, fragile, and rapidly destroyed. Can obstruct capillary blood flow.
- microscopic obstructions—engorgement and ischemia
- RBCs with Hgb S live under 20 days
41
Multi-organ effects of sickled hemoglobin
Stroke, retinopathy, paralysis, death, blindness, hemorrhage, avascular necrosis (shoulder), hepatomegaly, gallstones, splenomegaly (often spleen must be removed), large amounts of dilute urine (Hyposthenuria), avascular necrosis, ab pain, dactylitis (erection that won’t go down), chronic ulcers, osteomyelitis
42
Complications of sickle cell anemia
- acute painful episodes (sickle cell crises)—when RBCs get lodged up
- stroke (need head scan every 6M-1Y)
- sepsis
- acute chest syndrome
- dec visual acuity
- chronic leg ulcers
- delayed G & D
- delayed puberty
- priapism—extended erection
- enuresis
43
Sickle cell crisis
Acute painful episode
- acute exacerbations that vary in severity and frequency
- precipitating factors—anything that increases the body’s need for oxygen, trauma, stress, infection, fever, dehydration, hypoxia—from high altitude, poorly pressurized airplanes (chopper or small plane), vasoconstriction due to hypothermia
44
Vaso-occlusive thrombotic episode (VOC)
Blood clumps up and gets lodged in vein
- very painful
- stasis of blood with clumping of cells in microcirculation—ischemia
- s/s—fever, pain, tissue engorgement
45
Therapies for sickle cell anemia
- been in chronic pain—may need stronger meds
- heat
- hydration
46
Splenic sequestration
Life-threatening—death can occur w/i hours
- blood pools in spleen
- signs include profound anemia, hypovolemia, shock, palpable spleen
47
Aplastic crisis
- dec production of RBCs and inc demand
- triggered by viral infx or depletion
- s/s—profound anemia, pallor
48
Acute chest syndrome
- sim to pneumonia
- VOC or infx causing sickling in lungs
- chest pain, fever, cough, tachypnea, wheeze, hypoxia
- repeat episodes—hypertension
49
Prognosis for sickle cell anemia
- varies—expectancy is mid-40s
- no cure but bone marrow transplant might help
- freq bacterial infx may occur (big cause of death)
- strokes in 5-10% kids with disease (neurodev delay)
50
SCA medical management
- diagnosed with Hgb electrophoresis
- palliative tx—(erythropoietin or hydroxyuria inc Hgb F production)
- penicillin prophylaxis daily
- transcranial Doppler annually
- prevent crisis
- crisis management—hydration is key
- hypertransfusion
- bone marrow transplant
51
Crisis management/NC
- maintain hydration—open the vasculature to get things flowing and helps with pain
- maintain oxygenation
- pain management—ATC good for chronic, may need morphine
- support for child and family—scary
52
Thalassemia
Auto recessive disorder with varying degrees of severity
- both parents must be carriers for parents to have
53
Thalassemia Patho
- alpha or beta-globulin chain in Hgb synthesis is dec or absent causing RBCs to be rigid and easily hemolyzed
- normal RBC size with dec amt Hgb
- chronic hypoxia—headache, irritable, precordial and bone pain, exercise intolerance, anorexia, epistaxis
- detected in infants or toddlerhood—pallor, FTT, hepatosplenomegaly, severe anemia
54
Alpha-thalassemia
- mild
55
Beta-thalassemia major
Severe anemia from a lack of hemoglobin
- bronze skin bc high iron levels from transfusions
- delayed puberty and poor growth
- cardiac enlargement with flow murmur—heart failure
- bone pain, skeletal deformities—frontal bossing
- death usually related to heart failure or iron overload
56
Medical management of beta-thalassemia
- Diagnosed with Hgb electrophoresis
- blood transfusion to keep Hgb normal
- SE—hemosiderosis—excess iron builds in body
- give oral vit C to help bind to iron
57
Hemosiderosis tx
Treat with iron-chelating drugs like deferoxamin which binds to excess iron and secrete by kidneys
- given IV or SQ over 8-10h multiple times/week
- may be given at home with IV pump by parents
- new oral chelation drugs—deferasirox
58
NC for beta thalassemia
- administer RBCs safely
- general measures for anemia
- monitor chelation therapy (N/V/D, edc appetite, rash, inc liver enzymes, neutropenia)
- ADEQUATE HYDRATION bc excreted via kidneys
- enhance self-esteem and body image
- enhance home health maintenance
59
Clotting Review
- Intrinsic path--slower; factor XI and VII cause cascade that leads to activation of factor X
- Extrinsic path--initiated when damage occurs, factor III released which activates VII with help of calcium ions
- Activated factor X with help of calcium ions, factor III, factor V, and PF3 activate prothrombin activator which then converts prothrombin in thrombin
- thrombin converts fibrinogen to fibrin which forms a loose mesh
- fibrin and factor III form a denser network of mesh fibers which can trap RBC and PLT forming a successful clot
60
Brief review of clotting process
1. BV get smaller
2. platelet plug is made; platelets adhere to damage wall, change shape (activate) and connect with other platelets, and aggregate--stick to each other and to the BV
3. clotting factor--fibrin clot made from clotting factors is needed to make a fibrin weave to make a strong seal
61
Idiopathic thrombocytopenia purpura
Abnormal destruction of platelets
- acquired hemorrhagic disorder
- acute episodes or chronic
- most often caucasian kids 2-8Y
- spontaneously recovery often
62
ITP CM
- sudden onset of easy bruising, purpura, petechia
- epistaxis
- plt count under 20k
- prolonged bleeding time
63
ITP Medical management
- primarily supportive
- antiD antibody--plasma derived immunoglobulin. causes transient anemia by clearing the antibody coated RBCs and breaking them down--Can cause TRANSIENT ANEMIA (don't give if kid is anemic)
- IV immune globulin (IVIG)--might be autoimmune dx
- steroids (1-2W course)
- splenectomy and IV gamma globulin if chronic
64
ITP Nursing interventions
- educate parents
- assess for bleeding (soft toothbrush, use electric razor)
- safety measures to prevent trauma
- Check for neuro chx like slurred speech, irritability, LOC chx for intracranial bleeds
- avoid unnecessary procedures, needle sticks, suppositories, rectal temps, (contact HCP to get all labs done at once)
- apply pressure for 5 min after venipuncture
- follow up care in home and clinic
65
Epistaxis NC
- seek med attn for bleed longer than 30 min
- sit up leaning forward
- apply pressure for 10 minutes
- apply ice bag to bridge of nose
66
Hemophilia
Group of hereditary bleeding dx from deficiencies of specific clotting factors (affects clotting factors, not plts )
67
Hemophilia A
- classic hemophilia
- deficiency of factor VIII
- most cases
- males affected but F can carry
- degree of bleeding depends on amt of clotting and severity of injury
- hereditary of mutation
68
Hemophilia B
- Christmas disease
- deficiency of factor IX
69
Hemophilia sx
- bleeding range from mild to severe
- sx appear around 6M when start bleeding from teeth
- mobility cause injuries and falls
- hemarthrosis--bleeding into joint spaces of knee, ankle, elbow, leading to impaired mobility
- ecchymosis—discoloration under the skin from bleeding
- epistaxis
- bleeding after procedures (minor trauma, tooth extraction, minor surg, SQ or IM hemorrhage)
70
Hemophilia labs
- normal plt
- normal PT
- hemoglobin normal or low
- prolonged PTT
71
Hemarthrosis signs
Stiff, tingle, ache, dec mobility, warmth, redness, swelling, severe pain
72
Management of hemophilia
- prevent bleeding--avoid contact sports, wear helmet, no puppy :(
- replacement of deficient factor (factor VIII concentrate or DDAVP to inc VIII production (desmopressin)) to stop bleeding
- regular exercise and PT
73
Hemophilia NC
- safe enviro
- teach protective equipment use
- avoid contact sports
- avoid aspirin
- SQ instead of IM, venipuncture instead of finger stick, no suppositories
- soft toothbrush and electric razor
74
Bleeding control
- pressure for 15 min
- RICE (rest, immobilize, ice, compression, elevation)
- kids can tell if have internal bleeding (elbow getting bigger and tingling)
- be sus of HA, slurred speech, altered stools (black, tarry)
75
Hemophilia Nursing interventions
- prevent SE of bleeding (active ROM after acute phase, PT, nutrition)
- avoid extra wt--extra wt on joints
- family support and home care
- med alert bracelet
- notify school nurse and teacher
76
Henoch-Schonlein Purpura (HSP)
- often follow URI
- general inflam of vascular sys of capillaries
- extravasalation of RBCs, makes petechial skin lesions
- inflam and hemorrhage in GI tract
- may lead to ongoing nephrotic syndrome
77
HPS management
- most cases resolve w/o tx
- steroids and anticoags for severe cases
- symptomatic tx
- maintain hydration
- monitor renal fxn
78
Variations in peds anatomy (immune)
- immature immune sys
- dec response to invading orgs, inc sus to infx
- cellular immunity is generally fxnal at birth (T cells); humoral immunity dev
79
Severe combined immunodeficiency disease (SCID)
Defect char by absence of humoral and cell mediated immunity
- B and T affected
80
SCID CM
- early freq infx
- chronic diarrhea
- thrush
- FTT
- adventitious signs bc pneumonia
- very low immunoglobulin levels
- graft vs host rxn to foreign subs
81
SCID management
- IVG
- sterile enviro
- bone marrow transplant
- prevent exposure to infx
- support and edu family
82
SCID diagnosis
By r/o
83
HIV in kids
- acquired cell-mediated immunodeficiency dx caused by wide spectrum of illness in kids
- T cell affected mostly but also B cell, NK cells, macrophage/monocyte fxn. HIV infects CD4 (helper T cells)
- invades CNS--progressive encephalopathy; microcephaly, motor defects, loss of previously achieved milestones
84
Difference with HIV in kids and adults
Kids haven't had much exposure to be protected and already have impaired immune response so more vulnerable. Mode of transmission different too
85
HIV etiology in kids
- transmit mother to infant; prenatal, perinatal, breast milk
- blood transfusion
- unprotected sex
86
HIV CM
- lymphadenopathy
- oral thrush
- hepatosplenomegaly
- chronic or recurrent diarrhea
- FTT
- dev delay
- parotitis--infx in salivary glands
- more severe sicknesses
87
AIDS CM
- serious recurrent bac infx
- lymphoid interstitial pneumonia
- PCP
- candidal esophagitis
- cytogealovirus (CMV)
- Herpes simplex disease
- HIV encephalopathy
- wasting sx
- cryptoporidiosis--water-borne parasite causing diarrhea
- often fussy
88
HIV diagnosis
PCR (can tell if pos w/i a month)
- can't do antibodies bc kids don't have many antibodies
89
HIV tx
- antiretroviral therapy--all infants born to HIV+ moms for first few months at least (until test results back)
- aggressive abx therapy for infx
- modified immunization schedule
- prophylaxis against opportunistic infx
90
HIV NC
- focus on prevention
- safe sex
- no needle sharing
- no breast feed with pos mom
- mom on antiretroviral therapy (can damage fetus so if mom doing ok, take her off for first tri)
- educate on safety, storage of meds and equip, transmission and prevention
- altered nut; less than body requirements (inc pro and cal, provide preferred foods, nut supplements
- universal precautions; clean bod fluids with 10% bleach, properly dispose
- don't need to tell school if following universal precautions
- encourage to go to school
- pregnancy inc rate of HIV progress
- caregiver anxiety
91
Which VS is most important to monitor with anemia?
Temperature—indicates emergency
92
When do symptoms for sickle cell first appear?
Around 6M when fetal HgB decline
93
Cause of sickle cell crisis
Anything that increases the body’s demand for oxygen like trauma and illness
