Nursing care of the child with hemotalogic disorder

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  1. 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
  2. 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
  3. 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
  4. Variations in Pediatric A&P
    • 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
  5. 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 
  6. 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/ml
    • when 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 
  7. 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
  8. 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 
  9. 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 
  10. 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 
  11. Age related normal values CBC
    • RBC value
    • 2-6mon 5.6
    • 6-1yr 5.2 drops might be them going into physiological anemia
  12. 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 
  13. 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
  14. Anemia
    • Iron Deficiency
    • Lead Poisoning
  15. 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
  16. 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
  17. 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 
  18. 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
    • CV: tachycardia, or murmur cardiomegaly (enlarge heart)
    • 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
  19. 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
  20. 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
  21. 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
  22. 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 
  23. 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)
  24. 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
  25. 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 
  26. 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
  27. 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
  28. Intervention
    start monitoring them after 5 anything more than 45 chelating agent
  29. 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)
  30. 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
  31. 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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Nursing care of the child with hemotalogic disorder
2015-01-31 00:31:37

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