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Review of Hematologic system
- Consists of blood and blood forming tissues of body (bone marrow tissue form blood cells)
- - three cell categories
- * Erythocytes or red blood cells (RBCs)
- * Leukocytes or white blood cell: type neutrophils, lymphocytes, monocytes, eosinophils, basophils
All blood cells originate from a single type of cell called a multipotent hematopoietic stem cell
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hematologic problems
- results from different causes:
- Nutritional deficiency: malabsorption of nutrients (vitamins: b12, folic acid, other iron)
- Injury: trauma (hemorrahage), ingestion/exposure of toxins, radiation or drug exposure
- Infections: can lead to autoimmune (immune system attack itself) response idiopathic thrombocytopenia purpura (clotting disorder)
- genetics: sickle cell, hemophilia thalassemia
- Chronic bleeding: GI, GU, heavy menses
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Variations in Pediatric anatomy and physiology
- RBC production: begins at 8 weeks gestation, primarily from the liver but when the baby is born the kidneys take over
- - Erythopoieton (EPO):
- * Derived from liver in fetus and kidney after birth
- * Hormone that regulates RBC production
- * chemotherapy can effect this production or is someone has kidney problems. can cause anemia
- - Hemoglobin
- * three types: Hgb A, hgb F (fetal), Hgb A2
- * Hgb F is found in the fetus and over time it converts to hgb A
- * hgb A is the predominate type (after 6m of age)
- * Hbg F is protective for some people esp with sickle cell helps cells to not sickle
- * hgb A2- abnormal??
- mom has transferred iron to the baby so baby has a good supply but after 6m if baby not getting enough from food then they can have physiological anemia
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Variations in Pediatric A&P
IRON
- Fetus: iron received from the placenta
- Preterm: at risk for anemia as the missed final weeks or months of transplacental iron transfer from mother (nurse- make sure mom is getting enough iron)
- Term: physiologic anemia occurs between 2-6 mons. iron stores depleted about 4-6 of life
- Sufficient iron intake is critical:
- breast milk or iron fortified formula (iron rich foods)
- Possible iron supplements
- Adolescents: need iron due to rapid growth, menses, they dont eat very well
- if baby ends up with anemia assess how mom is feeding. make sure mom isn't watering milk down found mostly in poor families
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Common Medical treatments
- Require a physicians order:Blood transfusions
- - PRBCs Packed red blood cells (admin for severe anemia to get the blood up also sickle cell thalessmia)
- - Whole blood: acute hemmorhage nothing wrong with they way the manufacture blood it is just they had a terrible injury
- - Fresh frozen plasma: given to hemaphiliacs to help with clotting factors that these people are difficient in
- Supplement oxygen: bc hypoxia O2 stat low
- Splenectomy: bc spleen enlarged and isn't working as well (kids with sickle cell, hemophillia)
- Bone marrow transplant: sickle cell, aplastic anemia, thalssemia
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Common medical treatments: Medications
- Iron supplements (ferrous sulfate): iron deficiency anemia (IDA)
- Deferoxamine: Iron toxicity (chelating agent)
- Chelation: that this medication will bind itself to a metal in the body and excrete it thru the kidneys)
- some people have multiple transfusions and this is how they get too much iron in the body.
- Succimer: chelating agents- binds to lead used for blood lead levels > 45 mcg/mlwhen u give these chelating agents monitor I&O
- Factor (VIII/IX) replacement: focusing more on factor VIII bc it is the clotting factor that is necessary to form a clot. hemophillacs are deficient in this clotting factor. replacement so they don't bleed
- Penicillin VK: used as infection prophylaxis. kids with sickle cell are prone to infection. kids will get this for the 1st 5-6 yrs of life. PO everyday
- folic acid: folic acid deficiency/some kids with sickle cell
- Hydroxyurea: sickle cell pt helps to stimulate and promote hgb F production. helps to reduce the incidence of sickle cell
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Common medical treatments: nursing implications of medications
- iron supplements:
- - no milk or milk products
- -give 1 hr before or 2 hr a meal better absorbed on an empty stomach
- - liquid iron: rinse mouth can stain teeth (use straw behind teeth, mix in juice)
- - stool/urine turn dark colors, dark green/black
- - give with Vitamin C to help absorb
- - constipation: fluid/fibers
- Deferoxamine: rotate site of injection given sc. sometimes they may have some reaction when u give this so put hydrocortizone on it
- Factor VIII/IX:
- - glass vial. u have to use filter needle
- - admin IV: when pt is experiencing bleed
- - Admin at home or ER: or given prophylactically to help bleed not to happen
- Penicillian VK:
- - check for allergies
- - monitor renal and hematologic function
- Folic acid supplements: admin anytime no meal required
- - encourage foods high folic acid: asparus, brussel sprouts, spinach, dark leafy green
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Assessing a child with hematologic disorder
- Always begins with Hx
- chief concerns: kids are fatigue, platelet disorder u may have bruising, nose bleed
- Preterm baby? was Vitamin K given at birth? nutrition- picky eater?, Fhx genetics, taking in too much milk, pica (non food substances)
- ice cubes: if anemic
- Obese infant/fatigue- IDA, not much milk and not enough iron
- eye: retinal hemorrahage/yellow eye jaundice- sickle
- frontal/maxillary bone boss (enlarged) bc bone marrow is overworking and it causes changes in the bone
- mouth: mucus membrane- anemia it will be pale. bleeding gum: anemia and coagulation problems
- petechia: little red spots..they don't blanch
- echymosis:
- abdomen: maybe tenderness liver enlargements
- extremities: nails spooning/clubbing bc of lack of oxygen
- iron def weak muscles
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Nursing Dx
- Fatigue: decrease oxygen supplement, cluster care as a nurse, observe activity
- Pain: sickle cell, hemophilic, use approa pain scale
- Impaired physical mobility: sickle cell, hemophilliac, encourage mobility, manage pain
- ineffective health maintenance: education about iron rich food, cow milk kids should be taken more than 24oz/day. vitamin supple
- anxiety: confusion bc of decr oxygenation to the brain
- ineffective family coping: needs support
- risk for injury: aviod rectal temps, perforated rectum, becareful
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laboratory and diagnostic testingq
- Complete blood count (CBC): test the following
- RBC total count: if u have too much it is called polycethemia, erythrocytosis- if too much blood gets to thick puts u at risk for clotting.
- sometimes if a person is dehydrated can look like they have this. just give fluids
- - hbg: hem and globin in the blood
- - hematocrit: indirect measure RBC
- both use together to evaluate anemia
- RBC indices:
- - MCV: size of the RBC (microcystic cell if anemia, if deficient in B12, folic acid, macrocystic cell)
- - MCH: O2 carrying capacity of HGB
- - MCHC: concentration of hgb in the RBC
- - RDW: width of RBC (when bone marrow is making more blood cells u will see variability with different sizes)
- WBC total count: fight infection
- - neutrophils: fight bacterial infection
- -lymphocytes: fight viral infection
- - monocytes: kids with mono, eusophil: allergic/parasitic
- PLT count: 150-450 normal range clot
- differential determines types and numbers of each WBC in 100
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Age related normal values CBC
- RBC value
- 2-6mon 5.6
- 6-1yr 5.2 drops might be them going into physiological anemia
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other labs and Dx
- Blood type and cross match
- - determine ABO blood type and antigens
- - used for trauma or a person with suspected blood loss
- - nurse: only good for 48-72hrs expired
- Clotting studies
- - PT, INR, PTT all done for clotting to make sure person is clotting
- Coagulating factor concentration
- - measure concentration of coagulating factors
- - indicated for: hemophillia, DJC
- Hemoglobin electrophoresis
- - measures normal and abnormal hemoglobin in blood
- - indicated for: confirms sickle cell anemia, thalassemia
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other labs dx iron, lead, reticulocytes, serum ferratin
- iron
- - evaluates iron metabolism
- - indicated for: IDA, hemosiderosis, hemoglodbinpathies
- lead:
- - lead in blood
- - screen or confirm lead poisoning
- Reticulocyte count
- - measures reticulocytes (immature RBCs)
- - indicate bone marrow function
- - use this to see if the iron therapy are working: a week 5-8 days u will see these cells increasing (incre in count)
- Serum ferratin
- - measures ferratin level (major iron storage protein)
- - most sensitive test for diagonosing iron deficiency anemia
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Anemia
- Iron Deficiency
- Lead Poisoning
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Disorder of red blood cells: anemia
- Anemia: level of RBC's and Hgb are lower than normal
- Causes:
- - nutrional deficiency
- - RBC structure
- - kids living in high altitude
- - other chronic condition cystic fribrosis
- - RBC structure is altered
- - bone marrow malfunction
- - exposure to toxin
- - adverse reaction to medication
- - trauma or surgery
- - increased rbc dustruction- hemolysis breaking down really fast
- In children (pediatrics) the most commons cause is nutritional deficiency
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Anemia
- classification: according to change in number of RBC's configuration or cause
- s/s: symptoms are observed when hgb <7-8 g/100ml
- - baby normal: birth- 2wks 14.5-24.5
- - 1-6yr: 9.5-14.1
- - think about it we won't see symptoms until it drops way lower than it is suppose to be. overtime child will get paler
- Normocytic (normal size cell), normochromic (normal color) anemia
- - this type means decr production of bone marrow due to hemorrahage blood loss
- Microcytic, hypchromic anemia: small pale rbc's due to stunted hgb
- - r/t iron deficiency anemia, lead poisoning
- Macrocytuc anemia: rbc's abnormally large, immature
- - megaloblastic anemia's, folic acid, or vita b12 deficiency
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Iron Deficiency Anemia (IDA) nutrition
cause, symptoms, dx/labs
- cause:
- - low iron diet (6-15ml needed a day)
- - diet too rich in milk or dairy
- - malabsorption (GI disease ie celiac disease)
- - lead poisoning
- - heavy menstruation
- Symptoms:
- - pallor/pale mucus membrane
- - poor muscle tone
- - decre activity/irritable
- - heart murmur- harder for the heart to pump blood due to the lack of oxygen
- - splenomegaly?
- - spoon shape/clubbing fingernails
- - pica
- Diagnostic lab
- Decreased:
- - hgb and hct- hgb < 11, hct< 33
- - reticulocyte count
- - serum iron and ferratin levels
- - microcytic, hypochromic RBC's
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IDA: assessment,
- Hx/dietary hx: PMH, FHX, diet incr milk consumption, picky eater, 24hr diet hx
- Physical examination:
- - neuro: fatigue, lethargy, irritable
- - skin: pale skin, conjuctibae, oral mucosa, palms and soles Nails: spooning
- Abd: splenomegaly (enlarged spleen)
- MSK: poor muscle tone, activity decre
- Infants: may show muscle tome and reduced activity. AAP recommends screening at 9-12 mons of age. if child is high risk u can start 6m
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IDA therapeutic/nursing management
- therapeutic management: PO iron supplements. dose depends on severity of iron deficiency 1-6 mg/kg/day
- severe cases: blood transfusion
- Reticult count will increase
- Nursing management:
- - Patient/fam education
- - Iron supplement admin
- - correct measurement
- - do not give w/milk or milk product
- - admin behind teeth, with straw, dilute juice, rinse mouth
- - control constipation: fiber, inc fluids
- Dietary intervention:
- - consume food high in iron
- - vitamin c
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Providing dietary interventions
- For iron deficient infants:
- - breast fed: start iron supplements at 4-5 (bc of physiological anemia). encourage moms to increase iron rich foods in diet/supplements
- - formula fed- use only iron fortied formula/cereals
- For children over 1 year of age:
- - limit cow's milk to 24oz/day
- - limit fast food consumption
- - encourage iron rich foods. provide variety of foods
refer to wic if family is struggling
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Foods Rich in Iron
- red meats- most easiest to absorb
- tuna and salmon
- eggs
- tofu
- enriched grains
- dried beans, peas, fruits
- leafy green vegatables
- iron-fortified breakfast cereals
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Lead poisoning
microcystic anemia
- Definition: increased level of lead (heavy metal) in the body (>5 ug/dl). due to chronic inhalation or ingestion of lead (can inhale this from dust in home if doing renovation) containing materials
- - results in hypochromic, microcystic anemia
- Complications: behavioral and learning difficulties, cognitive impairments, possible seizures, and brain damage
- treatment: for > 45 mg/dl Chelation therapy med succimer other than this get rid of exposure
- Symptoms: abd pain, headache, pallor (bc of anemia), irrability,
- severe cases: seizures, coma, death
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Risk factors: lead exposure (mayo clinic)
- Age: 1-5 infants and young children
- living in an older home: lead paint banned since 1978
- hobbies: use of lead solder, refinishing old funiture
- Country of origin: developing countries at high risk (adopted children from other country)
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Source of lead
- Paint: homes built before 1978, dust from windowsill, walls, and plaster in older homes
- Pica or eating non food items
- soil: leaded gas or car battery recycling
- glazed pottery and stained glass products
- lead pipes sypplying water to the home
- on the clothing: of certain parents/caregivers that work in certain manufacturer jobs
- Folk remedies: greta and arazon (spanish meds fine red power taken for upset stomach and teething babies) need to find these out in hx
- old painted toys or funiture
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Nursing assessments: lead
- History: source of lead, inhaled or ingested, pica
- PE: Sx anemia, pallor, abd pain, vomiting, constipation, anorexia, headache, fever, lethargy
- CNS sign:
- - hyperactivity, impulsiveness, irrability, loss of developmental progress, hearing impairment, learning difficulties
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lead poisoning medical
- well check ups:
- - start universal screening 9-12m 6m if high risk
- - ensure high risk children have blood lead levels measured
- Labs:
- - finger stick, confirm with venous blood sample
- - CBC, chemistry profile
- - radiology: abdominal x-ray, head CT or MRI
- lead will land in the bone
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Nursing Lead
- Education and prevention is key
- screen for risk factos
- ensure lead is being removed from child's home
- administer chelating agents:
- - monitor I&O bc of kidneys
- - hydrate: PO or IV
- - Monitor VS
- - Have epinephrine available (just in case they go into a anaphylatic reaction
- If they have lead: Referral early intervention or further evaluation
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Intervention
start monitoring them after 5 anything more than 45 chelating agent
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Anemia Bone Marrow dyfunction
Aplastic Anemia
- Bone dysfunction
- def: bone marrow fails to produce blood cells. Pancytopenia: reduce RBC, WBC, platelets
- at risk for alot of different problems ie anemia, infection, bleeding
different types ie congential
- Acquired aplastic anemia: excessive exposure to radiation, drugs, or chemicals that are known to cause bone marrow damage. most causes unknown
- classified as severe or non severe
- Drugs: chloramphenicoL (lots of lawsuits), sulfonamides (to treat uti), arsenic, hydantoin, benezne, quinine, felbamate
- other: radiation, inspecticide, chemotherapy drugs and serious infections(menigococcal pneumonia)
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Aplastic anemia
Nursing assessment
- Hx: exposure to drugs or radiation therapy
- FHx: enviroment, infectious disease hx
- PE: pallor, fatigue, anorexia, easy bruising, petechiae, frequent nose bleeds, heavy menses, gi bleeding, incr infection
- CV: incr HR, incr RR, SOB, cyanosis
- comes on quickly
- Therapeutic management: bone marrow transplant, PRBC's, and platelet transfusion, EPO (to help stimulate bone marrow to produce cell), corticosteroids
- Note: bone marrow transplant offers possible complete recovery
- any drug/chemical suspected of causing the bone marrow dysfunction be discontined at one and the child must never be expose to it again
- offer parental support
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aplastic anemia
nursing management
- safety: prevent injury, avoid hemorrhage, prevent re exposure to offending substance
- prevent infection
- stool softners: strain, develop rectal fissure which they can bleed
- PRBC, or platelet transfusions: administer only irradiated and leukocyte depleted units bc u don't want any reaction, if they are having a bone marrow transplant they cant have any of these antibodies in their blood
- refer families:
- aplastic anemia and myelodysplastic syndrome international foundation
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